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2022 Southern Medical Research Conference
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Cardiovascular club I

11:00 AM

Thursday, February 10, 2022

#1  IGF-1 reduces atheroscelrosis by reducing CXCL12

P Snarski1*

S Sukhanov1

T Yoshida1

Y Higashi1

S Danchuk1

S Shai1

C Bysani2

P Delafontaine1

1Tulane University School of Medicine, New Orleans, LA

2University of Missouri System, Columbia, MO

Purpose of Study

Atherosclerosis is the leading cause of Cardiovascular Disease, which is still the global leader of mortality. Insulin Like Growth Factor I (IGF1) has been shown to reduce cardiovascular events. IGF1 administration in ApoE deficient (Apoe-/-) mice fed a high fat diet reduced atherosclerosis and reduced plaque macrophages. Results of our previous in vitro experiments suggest that macrophages play a predominant role in mediating IGF1 effects in atherosclerotic plaque, but exact mechanisms remain unclear. We hypothesized that increasing IGF1 levels strictly in macrophages will prevent atherosclerosis.

Methods Used

After breeding a novel macrophage-specific IGF1 overexpressing transgenic mouse to an Apoe-/- background (MF-IGF1 mice), we assessed atherosclerotic plaque burden, stability, and monocyte recruitment. We accelerated atherosclerotic development by feeding animal a high fat diet for three months. We also assessed cholesterol efflux and foam cell formation in vivo and in vitro.

Summary of Results

Macrophage IGF1 overexpression downregulated plaque burden by 30%, reduced plaque macrophages by 47%, and promoted features of a stable plaque phenotype. Monocyte recruitment was reduced by 70% in MF-IGF1 mice and was associated with a 27% reduction in circulating levels of CXC Chemokine Ligand 12 (CXCL12). CXCL12 protein levels were reduced in plaque and peritoneal macrophages in MF-IGF1 mice. IGF1 completely blocked oxidized low-density lipoprotein (oxLDL)-dependent increase of CXCL12 mRNA transcription (98% reduction, P<0.01) and IGF1 treatment reduced CXCL12 protein (56% decrease, P<0.001) in vitro.

ATP-binding cassette transporter A1 (ABCA1) is the key cholesterol transporter mediating macrophage cholesterol efflux and CXCL12 reduces its expression. We found that peritoneal macrophages isolated from MF-IGF1 mice have a 2 fold increase in ABCA1 protein levels. We loaded peritoneal macrophages with oxLDL to measure changes in cholesterol efflux and found that MF-IGF1 mice have a 42% increase in efflux. We also found a 27% increase in cholesterol efflux in IGF1 (100 ng/mL) treated THP-1 cells with Apolipoprotein AI as a cholesterol acceptor.

Conclusions

Our results indicate that macrophage IGF1 reduces atherosclerosis and decreases CXCL12, a chemokine newly implicated in atheroprogression. IGF1 potentially exerts its atheroprotective effect via this reduction in CXCL12 by reducing monocyte recruitment and by increasing ABCA1, thereby increasing cholesterol efflux capacity.

#2  Association of transthyretin Val122Ile variant with incident heart failure and mortality among Black Americans: insights from the regards study

V Parcha1*

G Malla1

M Irvin1

ND Armstrong1

S Judd1

L Lange2

M Maurer3

P Goyal4

E Levitan1

G Arora1

P Arora1

1University of Alabama at Birmingham, Birmingham, AL

2University of Colorado, Denver, CO

3Columbia University, New York, NY

4Weil Cornell Medical Center, New York, NY

Purpose of Study

Genetic mutation in the TTR gene (rs76992529; Val122Ile) seen exclusively in individuals with African ancestry (population frequency: 3–4%) causes misfolding of the tetrameric transthyretin protein complex that accumulates as extracellular amyloid fibrils seen in hereditary transthyretin amyloidosis (hATTR). Estimation of the effect of this amyloidogenic TTR variant on the risk of heart failure (HF) and all-cause mortality in a large, geographically diverse cohort of Black Americans may provide insight into the clinical significance of this variant. We evaluated the Black participants from the Reasons for Geographic and Racial Differences in Stroke (REGARDS) study to examine the association of TTR Val122Ile mutation with HF and all-cause mortality.

Methods Used

We evaluated self-reported Black American participants of the REGARDS study without HF at baseline. Poisson regression was used to estimate the rates of incident HF and all-cause mortality. We used multivariable-adjusted Cox regression models accounting for demographic, clinical, and social factors, and genetic African ancestry to assess the risk of incident HF and all-cause mortality among those carrying TTR Val122Ile genetic variation compared with those without the variation.

Summary of Results

Among 7,514 Black participants (median age: 64 years; 61% females), the population frequency of the TTR Val122Ile variant was 3.1% (232 carriers; 7,282 non-carriers). The incidence of HF (per 1000 person-years) was 15.9 (95% CI: 11.5–21.9) among variant carriers and 7.2 (95% CI: 6.6–7.9) among variant non-carriers. Val122Ile variant carriers had a higher risk of incident HF (HR: 2.46 [95% CI: 1.72–3.53]; P<0.0001) compared with non-carriers. The incidence of all-cause mortality (per 1000 person-years) was 41.5 (95% CI: 34.6–49.7) among variant carriers and 33.9 (95% CI: 32.7–35.2) among variant non-carriers. Val122Ile variant carrier had a higher risk of all-cause mortality (HR: 1.44 [95% CI: 1.18–1.76]; P=0.0004) compared with non-carriers. There was no interaction between TTR variant carrier status and sex on HF and all-cause mortality outcome.

Conclusions

In a large cohort of Black Americans, we demonstrate that the amyloidogenic Val122Ile mutation in the TTR gene is associated with a ~2.5-fold higher risk of HF and a ~40% higher risk of all-cause mortality. With the advent of numerous hATTR therapies, the presence of TTR Val122Ile mutation seen commonly in those with African ancestry may be deemed clinically actionable and prompt an early access to therapy.

#3  Vitamin D modulates histone modifications governing the natriuretic peptide receptor-a gene

P Kumar*

K Pandya

CN Nguyen

KN Pandey

Tulane University Health Sciences Center, New Orleans, LA

Purpose of Study

Activation of guanylyl cyclase/natriuretic peptide receptor-A (GC-A/NPRA) by cardiac hormones atrial and brain natriuretic peptides (ANP and BNP) produces the second messenger cGMP. cGMP activates downstream signaling and biological effects of ANP/NPRA natriuresis, diuresis, vasorelaxation, antimitogenic responses, and cardiac anti-hypertrophic effects. The expression of Npr1 gene (coding for GC-A/NPRA) is regulated by several external and internal stimuli, but the hormonal and epigenetic mechanisms mediating Npr1 regulation is not well understood. The objective of present study was to examine the effect of Vitamin D (vitD) in the regulation of Npr1 gene transcription and expression via modulation of epigenetic factors.

Methods Used

Our bioinformatics study with the murine Npr1 promoter has shown presence of 4 vitD response elements (VDRE) in the region -583 to -495 from the transcription start site having perfect and VDRE-like consensus sequence. To delineate the mechanisms of the regulation of the promoter activity of Npr1 promoter deletion constructs were transiently transfected in cultured rat thoracic aortic smooth muscle cells (RTASMCs) and mouse mesangial cells (MMCs) and the transcriptional activity was measured dual luciferase assay kit.

Summary of Results

Luciferase assays demonstrated that treatment with Vitamin D3 (1α, 25-dihydroxy; VD3) enhances Npr1 promoter activity by more than 6-fold in a dose-dependent manner. Western blot and densitometry analysis showed increasing concentrations of VD3 significantly induced NPRA protein levels by 3.5-fold in MMCs and 4.7-fold in RTASMCs and maximum effect was observed at 100 nM. VD3 increased protein levels of vitD receptor (VDR) in a dose-dependent manner. There was 50% inhibition of histone deacetylase (HDAC) activity in presence of VD3 as measured by HDAC activity/inhibition ELISA kit. Moreover, treatment with VD3 reduced class I HDAC enzymes, HDAC1 and HDAC3 protein levels and dose-dependently enhanced acetylation level of histones, H3 at lysine residues 9 and 14 (H3-K9/14 ac) and H4 at lysine residue 12 (H4-K14ac).

Conclusions

The results demonstrate that VD3 regulate Npr1 gene expression epigenetically by modulation of histone modifications. The identification of epigenetic targets of vitamin D signaling as a regulator of Npr1 gene transcription and protein expression will have important implications in hypertension and cardiovascular regulation.

#4  Cell to cell communication through entanglement and superconductivity improving left ventricular function in an uncoupled state

PI Altieri*

W De Mello

N Escobales

University of Puerto Rico, School of Medicine, San Juan, Puerto Rico

Purpose of Study

Show that entanglement and superconductivity improves intracellular conduction in paired isolated heart myocites, improving coupling and left ventricular function.

Methods Used

Using the quantum concepts of entanglement and superconductivity experiments were done, intracellularly, using artificial intelligence; measuring intracellular electrical conduction across junction gaps (G.I.), induced by Enalapril (E.) and Angiotensin II (Ang II). E. was (25 ug/ml) injected at a rate of 1 ug/ml in 4 minutes. A plateau was reached at a valve of 106% from the bag. Ang II. was injected at 1 ug/min with a reduction of GI (55%) without a plateau.

Summary of Results

We think the plateau was reached after a reduction of entanglement, but not with Ang II. In a superconductivity state, E. is more effective in improving coupling in failing myocites, improving left ventricular function.

Conclusions

Further studies are needed to fully understand the hemodynamics changes done in a quantum state

#5  Association of serum lipid levels with covid-19 infection, severity and mortality

V Chidambaram1*

H Shanmugavel2

A Kumar1

D Voruganti1

JL Mehta1

1University of Arkansas for Medical Sciences, Little Rock, AR

2Saint Vincent Hospital, Worcester, MA

Purpose of Study

Coronavirus disease (COVID-19) can range from asymptomatic infection to severe illness with multiorgan failure. Recent studies demonstrated an association between lower serum lipid levels namely high-density lipoprotein (HDL), low-density lipoprotein (LDL), and total cholesterol (TC) and COVID-19 disease severity. But the results lack consistency, and the magnitude of the association is currently unknown.

Methods Used

We conducted a systematic review and meta-analysis on the difference in HDL, LDL, TC, and triglycerides(TG) levels between 1) COVID-19 patients and healthy controls 2) COVID-19 patients with and without severe disease 3) COVID-19 patients who died and who survived. We included articles from PubMed and Embase up to September 1, 2021. We analyzed the pooled mean differences (pMD) in lipid levels (mg/dL) for the above-mentioned groups using random effects meta-analysis and assessed publication bias using funnel plots.

Summary of Results

Among the 441 articles retrieved, 29 articles (26 retrospective and 3 prospective cohorts) met the inclusion criteria with an aggregate of 256,721 participants. Patients with COVID-19 had lower HDL (pMD = -6.95), and TC (pMD = -14.9) levels (table 1 and figure 1). There was no difference in LDL and TG levels among patients with and without COVID-19. Patients with severe COVID-19 had lower HDL (pMD = -4.4), LDL (pMD = -4.4), and TC (pMD = -10.4) levels compared to non-severe COVID-19 patients. Patients who died had lower HDL (pMD = -2.5), LDL (pMD = -10.6) and TC (pMD = -14.9) levels. TG levels did not differ with COVID-19 severity or mortality. None of the above analyses showed statistically significant publication bias.

Conclusions

Our analysis demonstrated lower lipid levels in COVID-19 patients compared to healthy controls. Among COVID-19 patients, lower HDL, LDL, and TC levels were associated with severity and mortality. We believe that reduced lipoprotein levels are secondary to systemic inflammation and hepatic dysfunction. Lipid levels could be explored as potential prognostic factors in patients with COVID-19.

#6  Impaired glucose tolerance in guanylyl cyclase/natriuretic peptide receptor-a gene-knockout and gene-duplication mutant mice

C Ramasamy*

K Neelamegam

M Bloodworth

KN Pandey

Tulane University School of Medicine, New Orleans, LA

Purpose of Study

Atrial and brain natriuretic peptides (ANP and BNP) are circulating hormones of cardiac origin that play pivotal roles in regulating blood pressure and fluid homeostasis through vasodilatory and diuretic actions and improve cardiac remodeling. Both ANP and BNP act by binding to the transmembrane guanylyl cyclase/natriuretic peptide receptor-A (GC-A/NPR-A). Systemic disruption of Npr1 gene (encoding GC-A/NPRA) leads to volume overload, high blood pressure, and congestive heart failure. However, the underlying mechanisms are not yet precisely determined. The aim of this study was to investigate whether Npr1 plays a critical role in regulating glucose homeostasis in Npr1 gene-disrupted mice.

Methods Used

The adult male and female (16–18 weeks) Npr1 gene-knockout haplotype (Npr1+/-, 1-copy), wild-type (Npr1+/+ , 2-copy), and gene-duplicated (Npr1+ +/+ + , 4-copy) mice were fasted for 16 hours and given free access to water. The administrated of glucose was done both orally and intraperitoneally (2 g/kg body weight) in mice to determine oral glucose tolerance test (OGTT) and intraperitoneal glucose tolerance test (IPGTT). The glucose levels in blood were determined by performing tail bleeds at 0, 15, 30, 60, 90, and 120 min using the AlphaTRAK blood glucose monitoring system (Zoetis Inc, Kalamazoo, MI). Systolic blood pressure (SBP) was determined by non-invasive computerized tail-cuff method (Visitech 2000).

Summary of Results

The results showed that the blood glucose level was elevated to the maximum value at 15 min after glucose (2 g/kg body weight) administration and declined to near basal level at 120 mins in 2 copy mice (OGTT: 101 ± 4 mg/dL in male and 98 ± 3 mg/dL in female, IPGT: 100 ± 3 mg/dL in male and 97 ± 4 mg/dL in female), whereas in 1-copy mice, the blood glucose levels remained elevated even after 120 mins (OGTT: 244 ± 6 mg/dL in male and 220 ± 4 mg/dL in female, IPGT: 250 ± 5 mg/dL in male and 225 ± 6 mg/dL in female) when compared with 2-copy mice. The blood glucose levels were also significantly lower at 120 mins in 4-copy mice (OGTT: 78 ± 3 mg/dL in male and 73 ± 2 mg/dL in female, IPGT: 76 ± 4 mg/dL in male and 70 ± 3 mg/dL in female) compared with 2-copy mice. SBP was significantly greater in 1-copy mice (134 ± 3 mmHg in male and 125 ± 3 mmHg in female) than 2-copy mice (101 ± 2 mmHg in male and 92 ± 2 mmHg in female). Similarly, SBP was also significantly lower in 4-copy mice (85 ± 3 mmHg in male and 78 ± 2 mmHg in female) than 2-copy mice. OGTT showed a significantly lower level of maximal blood glucose compared with IPGTT.

Conclusions

The present findings showed that Npr1 markedly prevented a steep rise of blood glucose levels after glucose challenge and ameliorated glucose intolerance in wild-type and gene-duplicated mice, suggesting that Npr1 plays a critical role in the regulation of glucose levels and the loss of Npr1 exerts detrimental effects on renal and cardiac functions in mutant mice. This work was supported by NIH grant (HL062147).

#7  Meta-analysis of randomized vs observational studies of the effects of invasive therapy in patients with non-st-elevation myocardial infarction and chronic kidney disease

JK Bissett*

Central Arkansas Veterans Healthcare System John L MClellan Memorial Veterans Hospital, Little Rock, AR

Purpose of Study

Patients with chronic kidney disease (CKD) and non-st-elevation myocardial infarction (NSTEMI) are a significant clinical challenge. Agreement between randomized and observational studies is uncertain. (1) Do both randomized and observational studies support the use of invasive therapy to the same extent (2) Are the results affected by the level of renal function? (3) Is the mortality with medical therapy alone equal in randomized and observational studies?

Methods Used

Studies were selected according to the criteria of: (1) randomized or observational report of patients with NSTEMI and CKD (2) numbers of patients and mortality with invasive and conservative therapy available for each level of renal function including estimated glomerular filtration rate (eGFR) 30–60 and <30. Meta-analysis with subgroup comparison was completed with calculation of odds ratios for death with invasive therapy vs death with conservative therapy.

Summary of Results

(1) Five randomized studies and four observational studies met the selection criteria with a total of 362486 patients treated between 1994 and 2020 with either invasive or conservative therapy

(2) The odds ratio for death with invasive therapy in patients with eGFR 30–60 in the randomized studies was 0.739 with confidence interval (C.I.) 0.382–1.431 , p = 0.370. The odds ratio for death with invasive therapy in the observational studies with eGFR 30–60 was 0.144 with C.I. 0.012–0.892, p=0.037.

(3) The odds ratio for death with invasive therapy in patients with eGFR <30 in the randomized studies was 0.790 with C.I. 0.135–4.63, p=.794. The odds ratio for death in patients with eGFR <30 in the observational studies was 0.384 with C.I. 0.281–0.552, p<.05.

(4) The mean risk for death with conservative therapy alone in patients with eGFR 30–60 was 0.128 (CI -0.001–0.227) in the group of randomized studies and 0.44 (CI 0.227–0.6525) in the observational studies, p<0.01. The mean risk for death with conservative therapy alone in patients with eGFR <30 was 0.345 (C.I. -0.103–0.794) in randomized studies and 0.463 (CI 0.00–0.926) in the observational studies, p=0.579.

Conclusions

(1) Although a favorable effect of invasive therapy was present in both randomized and interventional studies, the odds ratio for death was statistically significant in the observational studies.

(2) The observational studies showed a significant reduction in the odds ratio for death with invasive therapy in both patients with eGFR 30–60, and patients with eGFR <30.

(3) The risk of death with conservative therapy alone tended to be higher in patients in the observational group.

(4) Additional studies are needed to develop a model for selection of patients who will derive the most benefit from invasive or conservative therapy.

(5) Limitations of this study include the disparities in the numbers of patients in the study groups, the absence of hemodynamic and angiographic data according to eGFR, and the possibility that some studies could include patients with unstable angina in addition to NSTEMI.

#8  A retrospective analysis of mortality in adult patients with acute coronary syndrome and cardiogenic shock requiring temporary mechanical circulatory support

G Del Rio-Pertuz*

C Morataya

K Nugent

K Parmar

E Argueta-Sosa

Texas Tech University Health Sciences Center, Lubbock, TX

Purpose of Study

Despite the technical advances in cardiology, cardiogenic shock as a complication of acute myocardial infarction remains a medical challenge. Recently there has been a movement to standardize cardiogenic shock management across the United States with the National Cardiogenic Shock Initiative aiming to improve survival, particularly in acute coronary syndrome patients (ACS). We aim to determine how cardiogenic shock secondary to ACS that required mechanical circulatory support is managed in our institution and compare the clinical characteristics between survivors and non-survivors.

Methods Used

A retrospective study of patients 18–89 years old who required temporary mechanical circulatory support in the setting of ACS from August 2018 to August 2019 at University Medical Center at Lubbock, Texas. A comparison between survivors and non-survivors to discharge was done. Fisher’s Exact test and Wilcoxon Rank-Sum test were used for categorical and continues variables.

Summary of Results

A total of 39 patients were included, 90% males, the mean age was 62 years, 62% had diabetes, and the mean body mass index was 29.01± 5.84 kg/m2. Intra-aortic ballon pump was the most commonly used mechanical support device, followed by Impella (92% vs 8%). Total mortality was 18%. Elevated heart rates during mechanical support use and lactate at admission were associated to mortality (105 bpm vs 83.91 bpm, p=0.02) (6.85 mmol/l vs 2.55 mmol/l p,0.003. Mechanical support prior to percutaneous coronary intervention (PCI) or coronary artery bypass graft (CABG) was done in 44% of the patient, and its presence was associated to survival (53% vs 0% p=0.01).

Conclusions

Elevated heart rates during mechanical support placement and lactate levels are associated with mortality in patients with cardiogenic shock secondary to an acute coronary syndrome. The initiation of mechanical support prior to PCI was associated to survival. Larger more rigorous studies are needed to clarify these association.

Adult clinical symposium

12:00 PM

Thursday, February 10, 2022

#9  Refractory hidradenitis suppurativa, clinical response using superficial radiation therapy

KJ Pithadia2*

H Vildibill1

L Dismuke1

W Montgomery1

W McAfee1

DA Jones1

1Phoebe Putney Memorial Hospital, Albany, GA

2Augusta University Medical College of Georgia, Augusta, GA

Case Report

Managing hidradenitis suppurativa (HS) can be challenging. In many cases after initial conservative intervention, patients experience improvement in symptoms. Unfortunately, some cases become refractory and result in cosmetically challenging and painful recurrences. Surgery is often used to debride or excise affected tissue which can facilitate healing. We present the case of a patient refractory to surgical intervention who was approached with superficial electron beam radiation therapy.

Case

A 44 year-old male presented with diffusely thickened buttocks, gluteal cleft, perineum, and bilateral thigh HS. The patient was refractory to surgical debridement and medical management via antibiotics and corticosteroids. He was treated with split course electron beam radiotherapy, using a total dose of 30 Gray in 10 fractions and sustained a partial response within 2 weeks from initiation of treatment. Objective physical exam within one month of treatment showed a 25% decrease in total area of inflammation, with significant flattening of raised areas. At that time, the patient reported subjective decrease in pain and drainage. The response was deemed durable at 6 and 12 months post-treatment.

Discussion

Radiation therapy has anecdotal benefit for a variety of benign conditions and has been studied in low doses, sometimes single fraction delivery in the management of HS. We opted to use a split course which we believed was safest in mitigating side effects and potentially most durable.

Abstract #9 Figure 1

Treatment field for patient, displaying pre-treatment hidradenitis suppurativa in buttocks, gluteal cleft, perineum, and bilateral thighs

Conclusion

Superficial electron beam radiotherapy is effective in the management of benign conditions and has promise for refractory HS. Total dose and fractionation schedules need to be studied to optimize and guide future use.

#10  Mitochondrial myopathy mimicking guillain-barre syndrome in a 21-year-old graduate student

K Bharathidasan*

A Evans

FM Fernandez

A Motes

K Nugent

Texas Tech University, Lubbock, TX

Case Report

Mitochondrial myopathies occur in one out of 5000 people in the general population of the United States. Clinical manifestations can be broadly classified into three categories: chronic progressive external ophthalmoplegia, skeletal muscle-CNS syndromes, or pure myopathy. Cardiac abnormalities occur in 30–32% of cases, mostly in the form of hypertrophic cardiomyopathy, dilated cardiomyopathy, or conduction abnormalities. We present a case of bilateral lower limb weakness, pain, and swelling diagnosed with mitochondrial myopathy on muscle biopsy. CASE DESCRIPTION: A 21-year-old male graduate student was referred to our hospital with a 3-week history of weakness, pain, and swelling in both legs which began after he arrived in the United States from India. Examination revealed tachycardia, bilateral 2+ pitting edema to his knees, 4/5 MRC grade weakness and mild tenderness of his upper and lower extremity proximal and distal muscle groups, absent deep tendon reflexes, foot drop, bilateral ptosis and limited extraocular movements. Initial laboratory results revealed elevated creatinine kinase of 691 IU/L, brain natriuretic peptide of 3437 pg/mL, troponin of 47.1 ng/L, myoglobin of 195 ng/mL, lactic acid of 7.7 mmol/L, and reduced serum bicarbonate of 12 mmol/L. Due to a traumatic tap, the lumbar puncture results for suspected Guillain-Barre syndrome were unreliable. An electrocardiogram showed left axis deviation with left anterior fascicular block. Chest x-ray and CT angiography of the chest/abdomen/pelvis showed cardiomegaly and volume overload. His bedside ECHO revealed mild left global hypokinesis, with reduced ejection fraction of 40–44%, and mild pulmonary hypertension. Due to declining maximal inspiratory pressures, the patient was admitted to the medical intensive care unit. Ophthalmology confirmed ophthalmoparesis and ruled out cranial nerve palsy, myasthenia gravis, and retinitis pigmentosa. Gq1b antibody was negative. Extensive autoimmune and infectious work-up was non-contributory. A muscle biopsy from the patient’s rectus femoris revealed scattered ragged-blue and cytochrome-c oxidase negative fibers with increased perimysial and endomysial connective tissue, consistent with active and chronic primary mitochondrial myopathy. Endomyocardial biopsy results revealed active lymphocytic myocarditis. The patient has been treated successfully with furosemide, metoprolol, and methylprednisolone.

DISCUSSION

Myopathies should be considered in the differential diagnosis of patients in whom Guillain Barre syndrome is suspected. We report an interesting case of myopathy with prominent cardiac manifestations. Myositis presenting with myocarditis should raise suspicion for mitochondrial disease. Our experience stresses the importance of using an inter-disciplinary team approach to diagnose uncommon pathologies with widely variable multi-systemic involvement.

#11  Gaisbock in 2021

A Evans*

P Southerland

B Songtanin

K Nugent

Texas Tech University Health Sciences Center, Lubbock, TX

Case Report

The purpose of the study is to explore the possible diagnosis of Gaisbock in a patient with long-standing erythrocytosis and hypertension.

Methods Used

Case Study

Summary of Results

A 40-year-old Caucasian man with obesity was admitted with recurrent leg swelling and increasing oxygen requirements two weeks after hospitalization with COVID-19 pneumonia. Upon review of the patient’s history, he was found to have untreated hypertension over several medical encounters and an erythrocytosis spanning ten years. Recent medical history included a diagnosis of deep vein thrombosis (DVT) in the same leg two and a half months prior and was treated with Xarelto.

The patient reported a history of low testosterone for 12 years. However, he had not used any testosterone supplementation for the last nine months. He reported daytime fatigue, frequent bouts of nighttime awakenings, and frequent snoring. The patient never had a sleep study or used a CPAP. The patient used half a can of chewing tobacco daily for thirteen years, and he smoked one pack per day for ten years but quit 12 years ago. He worked strenuous jobs in the construction industry most of his life.

On this admission, the patient’s lab work was notable for hemoglobin of 18.7 gm/dL (13.7–17.5) and a normal erythropoietin level of 5.7 MIU/mL (2.6–18.5) without thrombocytosis or leukocytosis and a positive factor V Leiden mutation. His blood pressure was 132/91 mmHg. On review of previous records, the patient was found to have consistently elevated hemoglobin The patient had a stocky, ruddy appearance without hepatosplenomegaly.

Abstract #11 Table 1

Hemoglobin and blood pressure trends in case subject

Conclusion

Erythrocytosis can be categorized as primary, secondary, or relative. Patients with relative erythrocytosis have a decreased plasma volume with a relative increase in hemoglobin. Additionally, elevated hemoglobin levels have been associated with hypertension. Gaisbock’s syndrome, first described in 1905, is characterized by hypertension and erythrocytosis without splenomegaly, leukocytosis, or thrombocytosis. It is associated with mild obesity, elevated blood pressure, and increased blood viscosity, which may explain why these patients often develop cardiovascular complications. Patients with relative erythrocytosis are at a higher risk for thromboembolic complications. In this case, Gaisbock’s syndrome was suspected because the patient had had a stocky, plethoric appearance with persistently elevated hemoglobin and blood pressure with a normal erythropoietin level. Gaisbock’s syndrome establishes a relationship between benign erythrocytosis, hypertension, and an increased risk for cardiovascular events.

#12  Sarcoma of the hand

R Mathew

A Sethi*

A Flint

J House

University of Florida Health Science Center Jacksonville, Jacksonville, FL

Case Report

A 62-year-old female with a past medical history of hypertension presented to the emergency department with a large, ulcerating mass on the dorsal aspect of her left hand. The patient reported the mass first appeared about two months prior to presentation and had been rapidly enlarging within the past week. The patient also reported an associated 50- pound weight loss over the past two months. Vital signs were stable. Labs were remarkable for iron deficiency anemia and thrombocytosis. MRI showed a large 9.6 cm heterogeneous soft tissue mass at the dorsal aspect of the fifth digit, abutting the fourth digit. MRA showed patent radial and ulnar arteries. There was evidence of vasculature feeding into the mass from the common palmar digital artery branches of the ulnar artery and deep palmar branches of the radial artery. On physical examination, the large mass on the dorsal aspect of the left hand was friable and bleeding (figure 1). The patient reported minimal pain. Sensation remained intact in the median, ulnar, radial and axillary distributions. The patient underwent excisional biopsy of the mass with amputation of the 4th and 5th digits. Negative margins were achieved per frozen pathology. Pathology of the postsurgical specimen showed high- grade undifferentiated pleomorphic sarcoma (UPS).

Clinical suspicion for soft-tissue malignancy of the hand is usually low because most tumors of the hand are small and benign. However, soft-tissue sarcomas of the hand are rapidly growing tumors with a high metastatic potential. UPS have no specific line of differentiation and are usually a diagnosis of exclusion. Patients with UPS are typically older in age compared to those with other soft tissue sarcomas. The use of adjuvant chemotherapy for patients with resectable soft -tissue sarcoma remains controversial. Prior studies have shown a very small efficacy of chemotherapy in regards to recurrence and overall survival. Although our patient had no evidence of metastatic disease at presentation, close follow-up with oncology will be necessary for surveillance of recurrence.

Abstract #12 Figure 1

Ulcerating soft-tissue mass of left hand (measuring 8.2 x 9.6 x 8.9 cm)

#13  A challenging case of marjolin ulcer obscured by chronic osteomyelitis

SR Edwards1*

B Googe2

B McIntyre2

1University of Mississippi School of Medicine, Jackson, MS

2The University of Mississippi Medical Center, Jackson, MS

Case Report

The presence of chronic osteomyelitis may obscure the diagnosis of Marjolin ulcer. The present case report highlights this unique presentation to raise reasonable clinical suspicion and prevent missed diagnoses.

A 67-year-old woman with a past medical history of Crohn’s disease and multiple skin neoplasms was initially seen by dermatology for a nonhealing tibial wound. While an initial shave biopsy was nonmalignant, a repeat biopsy six months later revealed indeterminant pathology. At this time, she was referred to plastic surgery for an excisional biopsy. At presentation to plastic surgery, a nonhealing tibial wound with bone visible through a draining sinus tract was apparent within the boundaries of a previously healed scar. She reported a remote history of an open compound fracture requiring multiple surgical operations in the vicinity of the lesion. An X-ray obtained to assess any residual hardware demonstrated findings consistent with osteomyelitis.

Excisional biopsy revealed superficially invasive squamous cell carcinoma, consistent with a Marjolin ulcer. At the time of her initial operation, a satellite lesion was identified and biopsied, revealing squamous cell carcinoma in situ. Marrow edema was observed in the screw path associated with hardware from her prior operations. Integra dermal substitute was placed while awaiting pathology results. Biopsy and culture of the affected bone revealed chronic osteomyelitis growing Staphylococcus lugdunensis. The patient returned to the OR for full excision of the satellite lesion along with corticotomy and sequestrectomy of the affected bony tissue. A cement spacer with vancomycin and tobramycin was also placed at this time. Definitive coverage of the tibial defect was obtained with a soleus myocutaneous flap and split-thickness skin graft. Six weeks of outpatient cefazolin administration was arranged following discharge due to her chronic osteomyelitis.

Chronic immunosuppression, chronic infection, and multiple cutaneous neoplasms may be independently associated with the development of Marjolin ulcers. In the setting of atypical wound behavior, particularly when associated with these factors, clinical suspicion of secondary malignancy must remain high – even with prior negative biopsies and strong clinical evidence of an alternative diagnosis.

#14  A case of herpes zoster neuropathy with limb paralysis

J Garza*

LF Castro

MA Elmassry

J Sekhon

J Lalmuanpuii

Texas Tech University Health Sciences Center School of Medicine, Lubbock, TX

Case Report

Varicella zoster virus lies dormant in the spinal dorsal root ganglia until reactivation occurs and causes Herpes zoster. With pain being the most common complication of Herpes zoster, other, more rare manifestations can be looked over. Segmental zoster paresis occurs in around 3% of patients. VZV can spread from the dorsal root to the neighboring ventral root and cause subsequent motor weakness. This usually occurs in the same anatomical region as the presenting rash, but the two can rarely be dissociated. The majority of patient who develop segmental zoster paresis have a good prognosis and will recover all function after three to six months.

Case

In this case report, we present a 78-year-old female who came to the hospital with altered mental status that was found to have right arm paralysis after resolution of her encephalopathy. Magnetic resonance imaging of the brain showed no acute infarction. The patient had been diagnosed with Herpes zoster and prescribed gabapentin three days prior to admission with characteristic vesicular rash eruption present on the right upper extremity. Segmental zoster paresis was suspected at this point. She was started on IV acyclovir, and physical therapy was consulted. The patient remained hospitalized for 3 weeks awaiting discharge to inpatient rehab in which time she performed daily strength exercises for her arm. IV acyclovir was given for 21 days. The rash resolved, and the patient was discharged to inpatient rehab for two weeks where she was recommended by PT to perform three hours of intensive therapy per day. After 3 months of physical therapy, the patient’s motor function had improved significantly and full motor function was noted a year after her presentation on follow up.

Discussion

Herpes zoster is a common disorder seen in older adults. Although we tend to focus on the associated pain, we must remember other possible manifestations that can occur during or after the initial rash presentation. Segmental zoster paresis should be suspected in any patient with paralysis and recent diagnosis of shingles.

#15  Sugar also rots your muscles, not just your teeth: a 27-year-old female with poorly controlled diabetes with leg pain

S Duangkham*

A Wichmann

U Sharma

M Phy

Texas Tech University Health Sciences Center, Lubbock, TX

Case Report

Diabetes mellitus is a chronic disease with the potential for significant morbidity and mortality. Here we present spontaneous diabetic myonecrosis, a rare complication of diabetes mellitus, in a patient with CF.

A 27-year-old female with past medical history of CF, cystic fibrosis-related diabetes mellitus, diabetic retinopathy, exocrine pancreatic insufficiency presented to the hospital with severe right calf pain for 2 months associated with swelling. She denied trauma or fever. Physical examination revealed right calf swelling and exquisite tenderness to palpation, no erythema or warmth, 2+ dorsalis pedis and posterior tibial artery pulses with intact sensation. Labs revealed blood sugar at 785 mg/dL, moderate acetone, pH 7.389, and glycated hemoglobin 14.9%. Creatinine kinase and thyroid stimulating hormone were unremarkable. Ultrasound of lower extremities showed no evidence of deep vein thrombosis, and X-ray of the right leg was unremarkable. Magnetic resonance imaging (MRI) right leg showed nonspecific myositis noted throughout the calf with areas of nonenhancement and soft tissue edema. The Patient was diagnosed with mild diabetic ketoacidosis and diabetic myonecrosis of the right calf. DKA was treated per protocol, low-dose aspirin was started, and symtomatic treatment was given with pain control for diabetic myonecrosis. Patient improved with no further complications.

Spontaneous diabetic myonecrosis is a rare complication of diabetic mellitus. The pathogenesis is uncertain. Patients usually present with swelling and pain. The most common affected area is the front of the thigh, followed by the back of thigh or calf. Awareness of the syndrome will frequently suggest the diagnosis and laboratory and imaging studies can be used to exclude other diagnoses. Interestingly, creatinine kinase is normal in many patients. MRI may show high intensity in the involved muscle on T2-weighted sequences as well as subcutaneous edema and subfascial fluid. MRI with contrast is the diagnostic tool of choice which can distinguish nonenhancing infarcted muscle from surrounding inflammation or edema. Muscle biopsy is not necessary; it is indicated only when the diagnosis remains in doubt or when infection cannot be excluded by other investigative techniques. Treatment includes rest, analgesia, low-dose aspirin, and optimal glycemic control. Complications including compartment syndrome and secondary infections are reported in some patients. Resolution usually takes weeks to months in most patients with recurrence reported in approximately 40% of patients and often involves the contralateral side.

Spontaneous diabetic myonecrosis is a rare, debilitating complication of diabetes. The treatments are symptomatic treatment with analgesia, aspirin and optimize glycemic control. Without a high clinical suspicion for this condition, patients may be at risk for life- and limb-threatening progression to compartment syndrome and superimposed infection.

#16  A rare case of henochschonlein purpura in 80 years old man

KN Abdelmalek*

A Roy

T Naguib

I Mohammed

Texas Tech University Health Sciences Center School of Medicine, Amarillo, TX

Case Report

A Rare case of Henoch-Schonlein purpura in 80 years old man

Introduction

Henoch-Schonlein Purpura (HSP) is a small vessel vasculitis mediated by IgA-immune complex deposition. It is characterized by non-thrombocytopenic palpable purpura, abdominal pain, arthritis, and renal involvement. Pathologically, it can be considered a form of immune complex-mediated leukocytoclastic vasculitis (LCV) involving the skin and other organs. Though it primarily affects children (over 90% of cases), the occurrence in adults has been rarely reported.

Case

This is an 80-year-old gentleman with CKD stage IV, coronary artery disease, status post stent placement, who presented withupper extremities pain, mild swelling, and a subsequent development of palpable rash that spread on the lower extremities while being nonpainful and with no itch. He had mild proteinuria and microscopic hematuria notable in his urinalysis. Further deterioration of his kidney function was noted with normal complement levels and ANCA panel. TEE ruled out infective endocarditis vegetations. The patient developed NSTEMI during his hospital stay prompting a percutaneous coronary intervention of the right coronary and left circumflex arteries. A kidney biopsy was positive for IGA nephropathy with less than 50% interstitial fibrosis and more than 50% glomerular sclerosis. , High dose steroids were initiated with intermittent hemodialysis and plasmapheresis. The rash and joint pain improved completely while his residual kidney function remained stable. There was no immediate improvement of kidney function after the plasmapheresis course of 5 days.

Discussion and Conclusion

HSP is a heterogeneous disorder manifesting in adults with palpable purpura/skin vasculitis, hematuria, and proteinuria. The diagnosis can be easily missed. Accordingly, a high degree of suspicion and attention to noninvasive laboratory work culminating into kidney biopsy with immuno-fluorescence studies is mandatory to establish the diagnosis. Skin biopsy and immunofluorescence confirm the presence of LCV with IgA deposition which is the pathognomonic finding in HSP. Adults with HSP carry a different prognosis, and the development of hematuria may be a harbinger for more serious complications such as nephritic or nephrotic syndrome. Malignancy is common in adult-onset HSP and imaging should be done to exclude this possibility.

Physicians should be aware of the possibility of HSP in patients who present with vasculitic rash and kidney disease. Normal complement level can distinguish between IgA nephropathy and vasculitis like granulomatosis with angiitis and lupus erythematosus. Early diagnosis of HSP with kidney biopsy may improve the outcome.

#17  Splinter hemorrhages in the presence of pasteurella multocida bacteremia

A Nguyen*

G Dharmarpandi

A Dweik

J Dharmarpandi

T Naguib

Texas Tech University Health Sciences Center School of Medicine Amarillo, Amarillo, TX

Case Report

To describe an unusual case of Pasteurella bacteremia

Methods Used

Literature Review and Case Report

Summary of Results

71-year-old Caucasian male with end stage renal disease on peritoneal dialysis, atrial flutter, type 2 diabetes, ischemic cardiomyopathy with implanted CRT-D presented to the hospital with altered mental status with GCS 12. He had tachycardia, tachypnea, and fever with an erythematous, tender wound on left index finger. He had lacerated his finger 2 weeks prior with a pocket knife while cleaning dirt under his fingernail and failed cephalexin and clindamycin oral therapies prior to admission. The patient pointed to the splinter hemorrhages seen on his right index finger as the ‘dirt’ he was attempting to remove on his left index finger with his pocket knife.

Intravenous broad spectrum vancomycin and piperacillin-tazobactam were initiated and subsequently blood cultures grew Pasteurella multocida. He lived on a farm with a cat. In view of the implanted cardiac device, fever and positive blood cultures, infective endocarditis was a concern. However, transthoracic echocardiogram did not reveal any vegetations. XR and bone scan imaging of his finger also ruled out osteomyelitis. During his four days in the hospital, his condition and laboratory results improved and he was asymptomatic. Follow up blood cultures were negative and patient was discharged home on oral amoxicillin clavulanate for another week duration.

Conclusions

Pasteurella multocida is a commensal gram-negative bacteria seen in the mouths of many domesticated animals that is the most common cause of cellulitis after an animal bite. While it typically presents as soft tissue infections, it can more rarely cause meningitis, endocarditis, and bacteremia. It seems that contamination of the self-inflicted subungual minor wounds with cat saliva is what caused Pateurella multocida bacteremia. The patient also presented with splinter hemorrhages. This physical exam finding is caused by rupture of capillaries underneath the nail plate. Blood attaches to the nail plate and moves distally as the nail grows, creating the characteristic linear pattern. While the association between splinter hemorrhages and endocarditis is often emphasized in medical training, they are present in other pathologies as well and in and of themselves do not constitute a criteria for endocarditis. They most commonly appear due to trauma, but are also seen in endocarditis, vasculitis, medications, and renal failure due to platelet dysfunction from buildup of uremic toxins as was likely the case in this patient. The patient was unlikely to have endocarditis since he became asymptomatic in 2–3 days and tested negative in repeat blood culture. Although a transesophageal echocardiogram would have been beneficial in definitively ruling it out infective endocarditis, a transthoracic echo of good quality is adequate especially in persons with prompt clinical improvement.

#18  Sars-cov-2 induced pancreatitis

A Bonner*

F Ibrahim

W Walker

The University of Alabama at Birmingham School of Medicine Huntsville, Huntsville, AL

Case Report

SARS-CoV-2 Induced Pancreatitis

Introduction

SARS-CoV-2 is responsible for the ongoing pandemic and has been the cause of 4.7 million deaths and 233 million infections worldwide. Although it primarily attacks the respiratory system, the extrapulmonary targets of the virus include the gastrointestinal tract and hepatobiliary system, among others. We present a case of acute pancreatitis in a patient with SARS-CoV-2 infection without any other known causes.

Case presentation

A 55-year-old male with a past medical history of coronary artery disease, hypertension, 0.5 pack per day tobacco use for 30 years, and hyperlipidemia presents to the emergency department with a chief complaint of left precordial and epigastric sharp pain radiating to the back associated with nausea and vomiting. He denied shortness of breath, cough, fever, or chills. COVID PCR testing was positive on admission, and the patient had not received the COVID vaccine. Heart Rate- 82 beats per minute, Respiratory Rate- 22 per minute, Blood Pressure- 154/95 mmHg, 100% Oxygen Saturation on Room Air. Physical examination was significant for epigastric tenderness. Laboratory results: Lipase- 395U/L, Triglycerides-152 mg/dL, LDL-103 mg/dL, D-dimer- 0.67 mcg/mL. Normal troponin. CXR on admission showed mild patchy bibasilar infiltrates suggestive of developing pneumonia. Right upper quadrant abdominal ultrasound showed no gallstones or other abnormalities within the pancreatobiliary system. CT abdomen and chest with and without contrast showed evidence of acute pancreatitis and no other abnormalities. He was managed with intravenous fluids, pain management, and DVT prophylaxis and transitioned to a full liquid diet on hospital day four with lipase at 37U/L. He was discharged on hospital day five with full resolution of pancreatitis and COVID symptoms.

Conclusion

There is a limited but growing amount of literature supporting the diagnosis of SARS-CoV-2 induced viral pancreatitis. We worked up common causes of pancreatitis, and we do believe the patient presented with SARS-CoV-2 induced pancreatitis. About 1–2% of non-severe and 17% of severe cases of COVID have exhibited pancreatic injury. Angiotensin-converting enzyme 2 (ACE2) is the main target receptor of SARS-CoV-2. ACE2 is most abundantly expressed in the pulmonary sytem, but is also expressed in pancreatic cells as well as other cells in the gastrointestinal tract. This could explain a correlation between the virus and pancreatitis and other gastrointestinal symptoms. Although there is growing evidence of COVID-induced pancreatitis, the causal relationship is still debated between the two presentations.

Neonatal case reports

12:00 PM

Thursday, February 10, 2022

#19  Defying the definition of perinatal lethality for achondrogenesis type II

L Weaver*

AF Kane

The University of Alabama at Birmingham, Birmingham, AL

Case Report

Achondrogenesis Type II is an autosomal dominant lethal collagen disorder characterized by prominent forehead, recessed chin, small chest and ribs with resulting lung hypoplasia, enlarged abdomen, shortened limbs, and decreased ossification of the pelvis and spine due to pathologic variants of the COL2A1 gene. The gene encodes for the pro-alpha1 chain, a key component of type II collagen which is necessary in developing the framework for embryologic skeletal development. The phenotype of achondrogenesis is variable, and that variation remains poorly understood.

Case presentation

Infant and mother presented after a detailed sonographic exam showed micromelia and polyhydramnios concerning for skeletal dysplasia. An amniocentesis was performed at 22 weeks gestation age (wga) was notable for a heterozygous missense mutation on the COL2A1 gene concerning for a collagenopathy. The infant was born at 31.4wga due to premature amniotic rupture. Upon delivery infant was noted to have high palate, protuberant abdomen, rhizomelic shortening of all extremities, sacral dysgenesis, and small thoracic cavity. He required intubation and mechanical ventilation immediately following delivery due to respiratory failure. He has failed multiple attempts at extubation and has remained ventilator dependent since birth. He was initially considered to have a perinatally lethal disease phenotype due to upper airway obstruction and severe lung hypoplasia, however, after 6 months he may be considered as a tracheostomy candidate due to improved amount of lung tissue and continued survival.

Discussion

The clinical and radiographic phenotype of achondrogenesis are widely varied and may place a significant burden on a family as the definition of perinatal lethality remains challenging. The definition of lethality could be altered as a phenotype progresses with age. The number of skeletal dysplasias is growing rapidly due to technological advancements in genetic testing. A detailed description of this patient in the medical literature will aid the ongoing differentiation of the collagenopathy types. Skeletal dysplasias may be inherited via autosomal dominant, autosomal recessive, X-linked dominant, X-linked recessive, and Y-linked fashions. Mutation can occur de novo or inherited. Early prenatal diagnosis helps to guide genetic and palliative discussions with families to determine postnatal treatment options and recurrence risk.

#20  Congenital dislocation of spine in a neonate: a case report

AS Surti*

K Dolma

University of South Alabama, Mobile, AL

Introduction

Congenital dislocation of the spine (CDS) is a rare spinal malformation due to defective embryogenesis of the spine and spinal cord at a single level, that results in abrupt angulation of the spinal cord. We report a rare case of congenital displacement of the spine in a neonate which was missed at first admission.

Case Report

An 8-day old Ex-37-week old female infant was admitted to our neonatal intensive care unit due to concerns about a bump in the back. The infant was born to a 30-year-old female via spontaneous vaginal delivery at an outside hospital. The prenatal course was unremarkable, all prenatal ultrasounds were reported as normal, and the mother was not on any medications during pregnancy except prenatal vitamins. Delivery was uncomplicated – total duration of labor was 8 hrs, APGAR assigned were 8/10 and 9/10 at 1-minute and 5-minutes respectively. She was discharged from the delivery hospital after routine care. On follow-up exam with the primary care pediatrician, she was found to have a lump on the lower back. Spinal imaging including ultrasonography and magnetic resonance imaging were done which revealed posterior dislocation of L3 vertebral body relative to L2 with spinal cord compression. She was admitted to the neonatal intensive care unit after neurosurgery consultation. On physical exam, the infant was noted to have bilateral talipes valgus deformity with an otherwise unremarkable neurological exam. On spinal exam, she had midline bony swelling (step-up defect) at L3 level with no other neurocutaneous findings. Further workup did not show any evidence of VACTERL. Chromosomal microarray and whole-exome sequencing were normal. The infant’s spine was stabilized with custom-built thoracic lumbar sacral orthosis with the eventual plan to attain surgical intervention when infant attains 10 kg.

Abstract #20 Figure 1

Posterior dislocation of L3 vertebral body relative to L2 with spinal cord compression

Conclusion

Although rare, congenital dislocation of the spine when diagnosed needs an immediate stabilization of the spine. Our case stresses the importance of a thorough spinal examination for appropriate diagnosis.

#21  A slippery slope – incidental detection of hhv6 in meningitis/encephalitis panel – to treat or not to treat?

M Lakshmi Narasimhan*

A Kharbat

S Bhaskaran

S Parat

Texas Tech University Health Sciences Center, Amarillo, TX

Introduction

Human herpesvirus-6 (HHV-6) is a beta herpesvirus found ubiquitously. Bio Fire’s Filmarray meningitis/encephalitis panel (FDA approved test of 14 bacteria, fungi and viruses commonly found in encephalitis) is used in many laboratories for diagnosis of central nervous system infections and HHV-6 is found to be frequently positive. However, establishing HHV-6 as a cause of meningitis or encephalitis may be challenging as a polymerase chain reaction (PCR) test alone does not imply causality.

Case Description

A female neonate was born at 34+5 weeks gestation to a 21-year-old with active genital HSV lesions which was positive for HSV-2. Examination revealed a clinically stable neonate. Workup per Redbook guidelines showed negative HSV surface culture and blood PCR. The Biofire meningitis panel was negative for HSV-DNA-PCR, however, was positive for HHV-6 virus. HHV-6 blood PCR on the neonate was also positive. There was no CSF pleocytosis. Both mother and neonate had a negative HHV-6 IgM antibody and a positive HHV-6 IgG antibody, indicating possible vertical transmission and latent infection. After consult with infectious disease specialist, baby received IV Ganciclovir and repeat Biofire meningitis panel at 7 days continued to remain positive for HHV-6. A decision was made to continue IV Ganciclovir for total of 21 days. WBC counts were serially monitored, and on day 10 of therapy, she developed significant neutropenia (800 cells/mm3,). As the positive HHV6 was a serendipitous finding and the literature review showed possibility of lifelong latent infection the decision was made to discontinue Ganciclovir. Neonate was closely monitored as she was in the NICU with feeding issues and her counts recovered. Baby remained asymptomatic and had an uneventful hospital course.

Discussion

A unique feature of HHV-6 is establishment of lifelong latency by integration into human chromosomes. If such integration occurs in a germ cell, it can be vertically transmitted to the fetus through chromosomal integration (ciHHV-6). With the advent of newer diagnostic tests like Biofire meningitis panel, there has been an increasing rate of detection of HHV-6 virus, but majority are not associated with encephalitis. A positive test may either be due to chromosomal integration or subclinical reactivation of latent virus. A viral load or qPCR test on whole blood can easily determine ciHHV-6 status in almost all cases. One should be vigilant when initiating antiviral agents and decision to treat should be based on the patient’s clinical condition, immune status, and laboratory results to avoid serious adverse effects from antiviral agents.

Conclusion

Physicians caring for patients who test positive for HHV-6 on the Biofire panel should approach therapeutic decision making using all available information to minimize significant morbidity due to unnecessary use of antiviral agents, and emotional and financial burden associated with unwanted hospital stay.

#22  A preterm infant with congenital disseminated herpes simplex virus following maternal covid-19 infection

SG Engel*

S Yazdi

VE Duncan

B Sims

N Arora

University of Alabama at Birmingham, Birmingham, AL

Case Report

Disseminated Herpes Simplex Virus (HSV) is a feared neonatal infection typically presenting after the first week of life with sepsis-like features and encephalopathy. Congenitally acquired HSV infection represents a rare, serious variety of HSV in the neonatal period, providing a unique diagnostic challenge with significant morbidity and mortality.

A female infant was delivered at 29.2 weeks gestational age via cesarean section in the setting of non-reassuring fetal heart tracings, maternal preeclampsia, gestational diabetes, and Sars-COV2 infection. Physical exam at 1 hour of life demonstrated erosive lesions of the knee, foot, and cheek. Dermatology was consulted and favored infectious source of lesions, so a sepsis evaluation including HSV, VZV, and CMV studies was performed and ampicillin, gentamicin, acyclovir, and amphotericin B were started. Given high concern for HSV vs. varicella, ophthalmology was consulted, finding bilateral, likely viral, retinitis. Laboratory evaluation revealed transaminitis, thrombocytosis, and CSF pleocytosis with elevated protein. HSV PCR was positive in blood, CSF, and cutaneous lesion, as well as HSV2 positive on surface culture, yielding the diagnosis of congenital disseminated HSV with meningoencephalitis. The remainder of infectious studies were negative. There was no known maternal HSV history, although placental pathology revealed positive immunohistochemical staining for HSV 1/2 in addition to Sars-COV2. Patient’s serial CSF and blood HSV remained positive despite treatment with acyclovir. Serial HUS showed initially normal findings that progressively worsened to feature bihemispheric cystic encephalomalacia, periventricular leukomalacia with ex vacuo dilation of lateral and third ventricles. She developed central diabetes insipidus and was started on desmopressin. Ocular involvement subsequently included retinal necrosis and diffuse retinal hemorrhage. She developed severe myoclonic jerks in the absence of electrographic correlate on EEG. Levetiracetam and phenobarbital alleviated jerks, although she developed progressive hypotonia as neurologic status continued to deteriorate. Considering persistently positive HSV studies, foscarnet was added to acyclovir. However, at 3 weeks of life, she was intubated for apnea and respiratory failure, and given clinical trajectory and devastating prognosis, mother asked to compassionately withdraw support and allow natural death on day of life 25.

This case of congenital, disseminated HSV is particularly unique in that it occurred in a premature infant of 29 weeks gestation and had significantly elevated copy numbers in the blood and CSF as well as skin lesions, indicating likely longstanding infection at the time of delivery. Additionally, it is unknown how concurrent placental viral infections with SARS-CoV2 may have contributed to this patient’s course, or if the recent maternal SARS-CoV2 infection may triggered HSV reactivation and subsequent congenital HSV.

#23  Transient myeloproliferative disorder in trisomy 21 complicated by tumor lysis syndrome and multi-organ failure

KE Ordemann*

L Yu

M Knecht

M Espanol- Mendez

J Robinson

J Patrick

LSU Health New Orleans, New Orleans, LA

Background

Transient Myeloproliferative Disorder (TMD) is diagnosed in 10–30% of newborns and young infants with Trisomy 21. This disorder often results from a GATA1 mutation, causing uncontrolled proliferation of blast cells, with spontaneous regression in most cases. Complications include thrombocytopenia, hepatosplenomegaly, pericardial and pleural effusions, and rarely tumor lysis syndrome (TLS).

Case

We present the case of a late pre-term male infant, who at delivery had phenotypic features consistent with Trisomy 21, hepatosplenomegaly, and respiratory distress, prompting transfer to our facility. Laboratory examination showed hyperleukocytosis with a leukocyte count >440,000 (103/uL) and 97% blasts, hyperkalemia, hyperuricemia, and disseminated intravascular coagulation (DIC). Echocardiography revealed an atrial septal defect and pulmonary hypertension. The patient received supportive care with mechanical ventilation, intravenous hydration, Allopurinol, Rasburicase, packed red blood cells, cryoprecipitate, and fresh frozen plasma. A double volume exchange transfusion was also performed. Despite aggressive management, he remained hypotensive with metabolic acidosis, hyperkalemia, and multi-organ failure succumbing to his disease, on day 2 of life, prior to chemotherapy.

Discussion

We describe the case of a newborn with Trisomy 21 and TMD that developed TLS and multiorgan failure prior to chemotherapy. Most patients with TMD do not need cytotoxic drugs, as the process spontaneously resolves. However, symptomatic babies with high blast counts and organ dysfunction benefit from early interventions, including exchange transfusion and cytotoxic agents. TLS is a complication of TMD which usually results from the rapid degradation of malignant cells after initiation of chemotherapy. Our case indicates that TLS can occur before the initiation of chemotherapy and can be fatal.

#24  Unusual cause of respiratory distress in a term newborn

A Mirza*

S Kilaikode

N Walyat

LSU Health Shreveport, Shreveport, LA

Introduction

Respiratory distress is a common clinical presentation seen in neonates. Common causes of respiratory distress in term neonates include, but are not limited to, transient tachypnea of newborn, infections, aspiration, or cardiac etiologies. We present the rare case of a term infant who presented with an unusual cause of respiratory distress in newborn.

Case Report

Our patient was born at an outside facility to a 27-year-old female who had poor prenatal care. Infant was delivered at 37 weeks gestation via emergent C-Section due to non-reassuring fetal heart rates. On delivery, his APGARs were 8 and 9 at 1 and 5 minutes. He was noted to have an obvious chest wall deformity with a concavity to the right anterior chest and slight lateral curvature of thoracic vertebra. He was unable to maintain saturations on room air and had persistent tachypnea so was placed on supplemental oxygen and transferred to NICU. Chest X-ray obtained at birth revealed multiple mid upper thoracic developmental vertebral body anomalies, diminished left thoracic volume and multiple bilateral rib fusion abnormalities. Blood work obtained was unremarkable with no concern for sepsis, metabolic conditions, or hematological issues. He failed multiple attempts to wean off oxygen and would drop his saturations mostly when feeding. He was therefore transferred to our facility for higher level of care at two weeks of life. On arrival to our NICU, Pulmonology was consulted, and further workup was obtained per their recommendations including a CT Chest which showed the known thoracic abnormality but no parenchymal or intrapulmonary processes. Modified Barium Swallow Study was negative for aspiration. Echocardiogram showed a Patent Foramen Ovale but was otherwise normal. Cranial Ultrasound was normal and Abdominal Ultrasound showed a right pelvic kidney. Genetics was consulted, and genetic tests were obtained including chromosomal microarray which was normal and the working diagnosis was possible variant of Skeletal Dysplasia. Since all other causes of respiratory distress were ruled out, his continual oxygen requirement was attributed to restrictive lung disease secondary to congenital chest wall deformity. He was unable to be weaned to room air and continued to be tachypneic on nasal cannula. Since non-invasive ventilation (BiPAP/CPAP) is not practical in this age group, decision was made to discharge home on high flow nasal cannula. He was eventually weaned off oxygen and at his last follow up at 9 months of age, he had been doing well on room air. He was referred to Pediatric Orthopedics and is scheduled for Expansion Thoracoplasty when he turns 1 year old.

Conclusion

Restrictive Lung Disease secondary to chest wall deformities can be a cause of respiratory distress in newborns. High flow nasal cannula is a newer means of home oxygen delivery and its use in this population is still evolving.

#25  A rare case of hybrid ELS/CPAM without a systemic arterial supply

Y Cao1*

B Weidner2

1University of Florida, Pensacola, FL

2Ascension Florida, Pensacola, FL

Case Report

A newborn female was delivered via elective C-section at 34 weeks due to maternal preeclampsia, growth restriction and a suspected right lung mass. Two doses of prenatal steroids were given to mother prior to delivery. The chest mass was noticed on a prenatal ultrasound which was followed by a fetal MRI. However, the MRI did not reveal an abnormal chest mass but it was somewhat limited by motion of the fetus.

Infant developed respiratory distress shortly after delivery and required CPAP for poor color and perfusion.Stat x-ray confirmed immature lungs with right lower chest mass. She was admittedto NICU and was promptly intubated due to increasing oxygen requirement. The series of CXR revealed the likelihood of lung origin was high, suggesting extra-pulmonary sequestration or CPAM. Emergent chest CT with IV contrast was done which showed the right lung mass was likely CPAM type II or type III vs. sequestration that seems compressing the R Lung with mediastinal shift to the Left.

Due to persistent respiratory distress and the size of the mass, decision was made to perform right upper lobectomy to release the compression. During the surgery, the patient was noticed to have total of 6 lobes of lung on the right side and 3 lobes looks abnormal. The abnormal lobes were intimately attached to the normal lobes. The patient had normal appearing upper, middle and lower lobes once the abnormal tissue was removed. No aberrant vessel from any of the abnormal lobes to the aorta was seen. From the middle abnormal lung a frozen section biopsy was taken which showed cystic appearance close to the pleural surface up to 1.2 cm consistent with CPAM T2.

Thoracotomy and resection of triple lobectomy was successfully performed on the 3-day old female and two chest tube was placed on the right side. Patient was remained to be on Jet Ventilation with chest tube to suction after surgery. She was then extubated and weaned to NIPPV one week later and both chest tubes were removed 2 weeks after surgery. Extra-lobar sequestration was confirmed presenting as three abnormal lobes, with histologic feature of CPAM type 2, but no aberrant vessel from any of the abnormal lobes to a systolic artery was seen.

#26  Preterm infant with tuberous sclerosis complex

ME Franco Fuenmayor*

A Williams

AM Aly

S Jain

M Huff

The University of Texas Medical Branch at Galveston, Galveston, TX

Case Report

A male infant was born at 28 weeks gestation to a G2P2 mother due to non-reassuring fetal status. Pregnancy was complicated by intractable seizures requiring induced coma, suspected chorioamnionitis, and insulin-dependent diabetes mellitus. Mother had epilepsy requiring multiple anti-epileptics. At delivery, the baby was limp, edematous, and apneic requiring mechanical ventilation. He received surfactant for respiratory distress syndrome, dopamine for hypotension and antibiotics for presumed sepsis. On the third day of life, he was noted to have a systolic ejection murmur with widened pulse pressures. Echocardiogram showed multiple non-obstructive echogenic masses scattered over the myocardium of the ventricles, septum, and papillary muscles suggestive of rhabdomyomas. Due to maternal epilepsy and suspected cardiac rhabdomyomas, the diagnosis of familial tuberous sclerosis complex (TSC) was considered. Neither mother nor infant had cutaneous findings. Mother had unremarkable head imaging. His cranial ultrasound showed nodular intraparenchymal echogenicity in bilateral subcortical frontal lobes suspicious for subcortical tubers and left lateral ventricle prominence with ependymal nodularity suspicious for a subependymal hamartoma. Renal ultrasound and ophthalmologic exam were unremarkable.

TSC is a genetic disorder featuring the growth of benign tumors in multiple organ systems. Most cases are caused by mutations in either TSC1 or TSC2. Disease severity is variable and specific physical manifestations appear at progressive ages. Often, mutations in TSC2 are associated with a more severe clinical course and developmental delay. Cardiac rhabdomyomas may lead to arrhythmias or outflow obstruction. Seizures typically present in infancy and can be difficult to control. A clinical diagnosis is made when 2 major and 1 minor criteria or 1 major and >2 minor criteria are fulfilled. Major criteria include hypomelatonic macules, angiobriomas, ungual fibromas, shagreen patch, retinal hamartomas, cortical tubers, subependymal nodules, subependymal giant cell astrocytoma, cardiac rhabdomyomas, lymphagioleiomyomatosis or angiomyolipmas. Minor criteria include confetti skin lesions, dental enamel pits, intraoral fibromas, retinal achromatic patch, multiple renal cysts, nonrenal harmatomas and sclerotic bone lesions.

Genetic testing revealed normal maternal TSC1 and TSC2, however the infant’s testing was abnormal and revealed a variant of unknown significance (VUS) in TSC2. If paternal testing is negative for TSC changes, the infant’s VUS can be considered a de novo pathogenic mutation. At near-term, the infant’s brain MRI demonstrated multiple subependymal nodules and subcortical tubers. The baby was diagnosed with TSC given his clinical findings. He was discharged at term without cutaneous findings or evidence of seizures. He will be followed by cardiology, genetics, ophthalmology, and the premature developmental follow-up clinic.

#27  Congenital ectropion of bilateral upper eyelids in a newborn

M Lakshmi Narasimhan*

S Parat

S Herrick

M Naqvi

Texas Tech University, Amarillo, TX

Introduction

Congenital ectropion is an eversion of eyelid, where the skin of the lid is folded on itself, and conjunctival surface is exposed externally. The incidence is higher in black infants, Trisomy 21 syndrome, and in infants born with collodion skin disease.

Case Description

An early term female newborn was born to a 25 year old G3P2 mother of African ethnicity by vaginal delivery. Mother had adequate prenatal care with negative infectious prenatal labs. Mother had spontaneous rupture of membranes lasting for 14 hours and a healthy-looking female baby was delivered without any complications. Examination revealed a normal baby not in any obvious distress, but on eye exam, an erythematous and edematous upper palpebral conjunctiva with mild eversion of bilateral upper eyelids were noted on crying (Grade II as per Pico’s classification). The underlying pupils or sclerae could not be visualized, and red reflex could not be performed. The eyeballs were palpated in the socket. Edematous upper palpebral conjunctiva was separated using eyelid retractors and underlying pupils and conjunctivae were noted to be normal in appearance. Condition was diagnosed as bilateral congenital ectropion with severe chemosis. Hypertonic saline compresses over the eyelids were given for edema. On day of life 4, there was complete resolution of ectropion and normally appearing pupils and conjunctivae were noted.

Discussion

Congenital ectropion is rarely reported in literature. Venous stasis, birth trauma and various abnormalities of muscles and ligaments of orbit including hypotonia of orbicularis muscle, vertical shortening of anterior lamella or vertical elongation of posterior lamella, failure of fusion of orbital septum with levator aponeurosis have all been proposed as possible mechanisms of congenital eversion. Initially, eversion occurs only when the infant squeezes the eyelids shut during crying and resolve when the infant is calm. As edema increases, the eversion persists and may be difficult to reposition. The chemosed conjunctiva protects the underlying cornea and therefore, corneal complications are uncommon. Management strategies include both conservative and surgical approaches. Conservative management includes 5% hypertonic saline and lubricants which prevents desiccation of exposed conjunctiva and decreases edema allowing for spontaneous inversion of eyelids. Surgical treatment includes tarsorrhaphy, fornix sutures and full thickness skin grafts to the upper eye lid. The condition resolves without any sequalae with prompt management, but delay in treatment can lead to complications including amblyopia, corneal ulcers, and permanent blindness.

Conclusion

Congenital eyelid eversion is a rare condition. A favorable clinical outcome may be expected with prompt diagnosis and intervention of this condition. Knowledge of this condition with early intervention by clinicians may help in the prevention of complications that arise from poorly treated cases.

#28  Juvenile myelomonocytic leukemia associated with noonan syndrome

A Gurung*

R Ramakrishnan

K Dolma

P Maertens

P Marri

BM Vamesu

University of South Alabama Health System, Mobile, AL

Introduction

Juvenile Myelomonocytic Leukemia (JMML) is an aggressive myeloproliferative disorder associated with RASopathies due to selective mutations in RAS, NF1, PTPN11, and CBL. Patients with Noonan syndrome (NS) with germ-line PTPN11 mutations have an increased risk of developing JMML or JMML-like myeloproliferative disorder. JMML associated with NS is less aggressive and transient compared to isolated JMML caused by somatic mutation in PTPN11. We are reporting the case of a newborn female with NS and JMML

Case summary

At 35 6/7 weeks of gestation, a female infant was born to a 35-year-old G6P2124 via spontaneous vaginal delivery. Cystic hygroma was noted on the fetal ultrasound at 14 weeks. On physical exam at birth, she had pterygium colli and facial dysmorphism (hypertelorism, downslanting palpebral fissures, posteriorly rotated low set ears). She was admitted to the neonatal intensive care unit (NICU) due to respiratory distress. The patient was initially started on Ampicillin and Gentamicin for suspected sepsis. Initial CBC revealed WBC 36,000/μL with 21% monocytes and platelet count 46,000/μL. She had seizure-like activity on day two of life. CSF analysis showed WBC 138 cells/μL with 91% monocytes, RBC 1000 cells/μL, protein 95 mg/dl, and glucose 63 mg/dL. Gentamicin was discontinued and a total of 14-day course with Ampicillin and Ceftazidime was completed. Blood and CSF cultures were negative. EEG showed no seizure activity. Cranial ultrasound revealed hypoplastic corpus callosum, paucity of gyri, mild colpocephaly, cerebellar vermis hypoplasia, and wide extra-axial space. ASD (3 mm) and perimembranous VSD (3 mm) were found on echocardiogram. RASopathy panel identified a pathogenic variant c.211T>C (p.Phe71Leu) in PTPN11 that confirmed the diagnosis of NS.

The infant had persistent leukocytosis (40–60x103/μL) with monocytosis and thrombocytopenia. She also had splenomegaly. Flow cytometry revealed 3% myeloblasts and 23% monocytes with mild monocyte abnormalities. She was diagnosed with JMML and the treatment with 6-mercaptopurine (6-MP) was initiated. WBC count decreased and thrombocytopenia resolved after starting the treatment with 6-MP.

Conclusion

JMML should be suspected in any patient with NS who has persistent leukocytosis with monocytosis and thrombocytopenia without evidence of an infectious process. Even though JMML associated with NS is transient, we decided to treat this patient with 6-MP due to hyperleukocytosis.

Pediatric clinical symposium

12:00 PM

Thursday, February 10, 2022

#29  A rare presentation of peritoneal chlamydial infection mimicking peritoneal carcinomatosis

S Muneer1*

AI Kamil2

E Whittingham1

1University of Florida, Pensacola, FL

2Phoenix Children’s Hospital, Phoenix, AZ

Case Report

A 17-year-old sexually active female, presented with diffuse abdominal pain and distension. The symptoms were waxing and waning for a year. She denied recent travel. Family history of a breast canacer was reported.

On examination: Patient was vitally stable but appeared uncomfortable. Abdomen was markedly distended, dull to percussion with positive fluid thrill but bowel sounds were appreciable. Pelvic exam was negative for cervical tenderness or discharge.

Labs

CRP 5.36 mg/dL, serum albumin 2.9 g/dL, CA125 198.4 U/L. Liver enzymes, LDH and uric acid were normal. CBC unremarkable. CT scan abdomen-pelvis showed severe ascites with mesenteric omental caking. Peritoneal fluid showed SAAG <1.1, WBC 950, 53% lymphocytes, LDH 186 U/L, protein 6.4 g/dL.

Working diagnosis was peritoneal carcinomatosis vs tuberculous peritonitis. TB workup was negative. Laparoscopy showed pelvic inflammation around the uterus with abscess and benign lymph nodes.

STIs as possible cause of patient’s ascites were investigated. Hep-B and C,HIV,syphilis,gonorrhea workup was negative. Endocervical NAAT was positive for Chlamydia. Pelvic inflammatory disease (PID) was presumed as the cause of ascites. Patient was started on intravenous antibiotics. Symptoms resolved and the patient was discharged on Doxycycline and Metronidazole.

Discussion

Chlamydia Trachomatis (CT) is the most commonly reported STI in the US, with nearly 1.8 million cases reported in 2018. Although the majority of females with CT infection are asymptomatic, CT known to cause urethritis, salpingitis, and cervicitis. 10 -15% of untreated women may develop PID. Ascites was rarely reported as the only presenting symptom of CT. Diagnosis of CT infection is commonly made by NAAT which is the most sensitive test. Exudative ascetic fluid and predominance of lymphocytes were noted in all reported CT cases with ascites. Hospitalization with parenteral antibiotic for 1–2 days is recommended for severe PID. Clindamycin or metronidazole should be used when tubo-ovarian abscess present.

Abstract #29 Figure 1

CT abdomen-pelvis: White arrow pointing towards fluid collection in the abdominal cavity

Conclusion

Despite the paucity of reported cases with ascites as a presenting symptom of CT infection, CT & other STIs should be in the DDx for sexually active adolescent with abdominal pain and ascites. Appropriate testing should be pursued early in the workup of these patients.

#30  Transverse myelitis in a pediatric patient with covid-19

A Jagadish*

M Benjamin

P Pichilingue Reto

LSU Health Shreveport, Shreveport, LA

Case Report

Transverse myelitis is the segmental inflammation of the spinal cord with motor and sensory abnormalities at and below the level of the lesion. Often, the etiology is unknown but may be attributed to autoimmune conditions or viruses. Here we describe a rare case of transverse myelitis secondary to severe acute respiratory syndrome coronavirus 2 [SARS-CoV-2]/coronavirus disease (COVID-19).

Case

A 5-year-old male with a history of asthma presented for vomiting and altered mental status. The patient was noted to be altered, lethargic, and in respiratory distress. Intubation was performed. After family collateral was obtained, it was revealed that patient possibly ingested Sertraline and/or Risperidone at an unknown time prior to arrival. History also revealed that he had slurred speech, ataxia, and a fall with trauma to forehead 1 day prior to arrival. He tested positive for COVID-19 via PCR and chest x-ray revealed RLL consolidation. Dexamethasone was started.

When sedation was weaned in hopes of extubation, patient was noted to be alert, but not moving extremities and had minimal gag and cough reflex. MRI of Brain and Spine were conducted and revealed findings suggestive of long segment transverse myelitis involving C2 to C3. LP was performed with unremarkable CSF studies and IV Solumedrol was started. In light of active COVID-19 infection, and worsening respiratory status, patient started on 5 days Remdesivir. Further, patient underwent ten sessions of plasmapheresis. Repeat MRI was consistent with previous. Physical and occupational therapy initiated at the onset of illness in hopes of achieving musculoskeletal improvement.

Patient had some minimal musculoskeletal improvement, however, given his condition, decision was made for patient to undergo placement of gastrostomy and tracheostomy tubes. Patient was weaned off of sedatives and withdrawal was treated with a clonidine taper. Once stabilized, patient was transferred to neurological inpatient rehabilitation center.

Discussion

Neurological manifestations in children affected by SARS-CoV-2 are relatively common but are often non-specific. Worldwide data reports only 1% of children with COVID-19 present with severe symptoms of encephalopathy, seizures, and meningeal signs. Pathophysiology is multifactorial, including direct invasion of the CNS, vascular insufficiency, immune dysregulation and autoimmunity. Imaging is paramount in the diagnosis of transverse myelitis. Treatments are emerging and may include steroids, immunoglobulin, plasmapheresis, and monoclonal antibodies.

Conclusion

Much is unknown about COVID-19. Information is emerging and evolving daily. Cases of transverse myelitis in COVID-19 have been reported in few adult patients and minimal pediatric patients. Practitioners should keep transverse myelitis on their list of differentials for neurological complications of SARS-CoV-2 infections and initiate aggressive treatment with a multidisciplinary approach.

#31  Sudden peripheral vision loss: a case of partial retinal artery occlusion in an adolescent

M Bahavar*

Y Nathani

The University of Oklahoma Health Sciences Center, Oklahoma City, OK

Case Report

This is a case of partial retinal artery occlusion in an adolescent. A 16-year-old female with history of ASD/VSD repair at age one, anxiety, and hyperhidrosis presented to the Emergency Department with sudden onset painless peripheral vision loss in the right eye. She denied any trauma, redness, excessive tearing, discharge, floaters, vomiting, fever or headaches. Her family history was unknown as patient was an adopted child. Her initial external ocular, including intraocular pressure, and remainder of the neurologic exams were normal. A few hours later, the patient’s vision loss gradually regressed to an inferonasal defect. Retinal edema along the superotemporal arcade on the right was noted during a dilated fundoscopic examination by Ophthalmology, concerning for branched retinal artery occlusion (BRAO). MRI/MRA of the brain, carotid duplex ultrasound, lipid panel, bartonella and toxoplasmosis titers, coagulation profile, ANA, and a hemoglobin A1C were normal. Transthoracic echocardiogram showed trivial tricuspid and mitral valves insufficiency, but was otherwise normal with an intact interventricular septum, no atrial septal defect, and no thrombus. She was discharged home on daily Aspirin with close outpatient follow-up with Ophthalmology and Neurology.

The incidence of central retinal artery occlusion (CRAO) is rare, with about 1 to 10 in 100,000; symptomatic BRAO is even less common. The mean age of patients is usually between 60–65 years, with more than 90% of patients over the age of 40, making this condition especially uncommon in children and adolescents. The central retinal artery is a branch of the ophthalmic artery, which itself is a branch of the internal carotid artery. It enters the eye at the optic disc and divides into smaller branches to supply the inner layers of the retina. An occlusion of the central artery or any of its branches leading to retinal ischemia is defined by sudden painless monocular vision loss. Men are more commonly affected than women. Potential etiologies include carotid artery atherosclerosis, cardiogenic embolism, vascular disease, hematologic disease such as hypercoagulable state, inflammatory disease such as giant cell arteritis and polyarteritis nodosa, and infection. Although carotid artery atherosclerotic disease is the most common cause overall, it is unusual under the age of 40, when cardiogenic embolism is the most common cause. Patients with BRAO typically complain of monocular visual loss, which may be restricted to just part of the visual field. Diagnostic work-up focuses on identifying the underlying etiology. It includes carotid artery imaging, cardiac evaluation, and hypercoagulable testing. About 80% of patients recover their normal vision, and therefore acute treatments are generally not offered. In the case of our patient, her BRAO remains of unknown etiology as all of her inpatient work-up yielded normal results. Further testing is currently ongoing.

#32  A modern conundrum: chickenpox in a vaccinated child

ML Evers1*

A Buttaci1

I Hill2

A Messer1

1LSU Health New Orleans, New Orleans, LA

2LCMC Health, New Orleans, LA

Case Report

Chickenpox is a highly contagious viral infection due to the varicella zoster virus. Primary infection in young children typically presents as a cutaneous, self-limited disease. Older patients and immunocompromised individuals are at higher risk for severe sequelae including superimposed bacterial infections, pneumonia, hepatitis, meningitis, encephalitis, hepatitis, and Reye’s Syndrome. Prior to the introduction of the varicella vaccine in 1995, roughly 4 million people contracted chickenpox each year. The vaccine led to a reduction in rates of infection, hospitalizations, and mortality. Today, fewer than 350,000 people contract chickenpox yearly. However, breakthrough infections in vaccinated individuals still occurs in approximately 13 per 100,000 individuals. Below, we discuss the case of a vaccinated 9 year old female with a superimposed bacterial infection secondary to chickenpox.

A fully vaccinated 9 year old female was transferred from an outside hospital for worsening cellulitis of her right upper extremity. Two weeks prior to admission she was treated with 10 days of oral clindamycin for cellulitis of her face, chest, and right arm with clinical improvement. Four days after resolution of the cellulitis she developed a new pruritic papule on her right arm. Her sister had several similar lesions which resolved within 48 hours. Our patient developed progressive erythema of her right antecubital fossa with evolution to a large, serum crusted plaque. Pruritic vesicles developed on her face, abdomen, and legs. She was transferred to our hospital for worsening cellulitis and started on intravenous vancomycin while wound cultures were pending. Though she experienced moderate improvement in the right arm plaque after several days of vancomycin, she developed progressive vesicles, facial swelling, eye pain, and severe pruritus. Varicella infection was confirmed by direct fluorescence antibody testing. The bacterial culture grew methicillin sensitive Staphylococcus aureus. She was discharged home with oral acyclovir and cephalexin.

Despite previous varicella vaccination, our patient developed disseminated infection consistent with chickenpox, which was complicated by a superimposed bacterial infection. Due to the high efficacy of the varicella vaccine, many clinicians lack firsthand experience with varicella infections and are unfamiliar with the natural progression of the virus. Prompt recognition of varicella infection is necessary to prevent and manage significant complications and to ensure appropriate isolation precautions both inpatient and outpatient settings. Despite reduced rates of this infection, the varicella virus remains a significant pathogen in both the unvaccinated and the immunocompromised populations. This case highlights the importance of familiarity with both typical and atypical presentations of varicella, as well as the importance of maintaining a high index of suspicion for varicella even in the vaccinated patient.

#33  A rare case of adenoid cystic carcinoma of the soft palate in an adolescent female presenting as chronic pharyngitis

J Doss1*

J D’Souza1

E Zakris2

S Collier3

C Morrison1

1LSU Health New Orleans, New Orleans, LA

2Touro Infirmary, New Orleans, LA

3LCMC Health, New Orleans, LA

Case Report

Adenoid cystic carcinoma is a rare malignant tumor of the secretory glands, most often affecting the salivary glands. It is known for taking a prolonged course with late local recurrences, distant metastases, and poor response to systemic chemotherapy. This case report highlights this rare malignancy’s presentation masquerading as a common pediatric complaint and our experience with the diagnosis, treatment and management of an adolescent patient.

Methods Used

A case review of the presentation and the multi-disciplinary management of an adolescent female patient with adenoid cystic carcinoma of the soft palate.

Summary of Results

A 17-year-old female presented to Otolaryngology for evaluation of chronic pharyngitis. She endorsed a year-long history of recurring sore throat and a growing lump on the roof of her mouth. On physical exam, a 1 cm submucosal lesion on the soft palate was noted. Surgical excision revealed adenoid cystic carcinoma with perineural invasion and positive margins. She was referred to Hematology/Oncology for concurrent management. Imaging revealed localized disease. Due to increased risk of recurrence with positive margins, the patient subsequently underwent a repeat excision with negative margins. She was then treated with 60 Gy of adjuvant radiotherapy over 6 weeks with minor complications. She remains without local recurrence 6 months after presentation.

Conclusions

Chronic pharyngitis is an uncommon pediatric complaint and should prompt concern for malignancy. Salivary gland tumors account for 0.5% of pediatric malignancies but are the most common pediatric malignancy exclusive to the head and neck. The incidence of head and neck cancer amongst the pediatric population has risen, warranting greater awareness of these cancers amongst general pediatricians and more treatment standardization.

As a rare tumor, studies of optimal treatment for adenoid cystic carcinoma have been limited and there is no agreed upon set of prognostic factors to predict recurrence. Up-front treatment with surgery and radiotherapy has remained the standard of care for decades, as no effective systemic chemotherapy has been identified. Complete tumor excision must be balanced with the risk of functional deficits and the consequent morbidity of dysfunction in speech, swallowing or nerve injury. In children undergoing radiation therapy, late effects may occur at a much earlier age and cause lifelong morbidity.

This patient has a predicted 5-year event-free survival of 75% with a 10–20% chance she will remain disease-free at 15 years. Recurrence can be locoregional but often presents as distant metastases to the lung or liver. The lack of effective systemic treatments during recurrence presents a dim prospect for a pediatric patient looking towards young adulthood. Further research is needed to determine optimal treatment, including targeted therapy, in pediatric patients to improve long-term event-free survival.

#34  Fibrosing mediastinitis secondary to histoplasmosis: a unique presentation in an adolescent

B Wilmore*

MA Jacob

JA McClain

K Sagdeo

MS Sagdeo

The University of Oklahoma Health Sciences Center, Oklahoma City, OK

Case Report

A 15-year-old female presented to our hospital with cough, weight loss, and a mediastinal mass. She initially developed a cough after moving from Oklahoma to Florida in 2020. In January 2021, she sought medical care for vomiting, vaginal bleeding, and a persistent cough. She was found to have a miscarriage, and her cough was not addressed. A month later, she sought medical evaluation due to persistent cough and new onset chest pain, and was diagnosed with an upper respiratory infection. Her symptoms failed to improve so she presented to the ER. X-rays of the chest and abdomen were negative. Her labs were significant for an elevated d-dimer and a microcytic anemia. A chest CT was done to rule out pulmonary embolism and revealed right hilar and subcarinal adenopathy, and a right middle lobe pulmonary nodule. She underwent a VATS procedure and debulking. On further testing, her histoplasma antibody was positive. She was treated with a 14-day course of Amphotericin B with plans for a 12-month course of itraconazole. Over the next month she developed worsening cough, vomiting, dysphagia, and a 10 kg weight loss. She was subsequently readmitted and repeat chest CT showed an infiltrative mediastinal and right hilar mass that had increased in size, with the pulmonary nodule in her right middle lobe now causing mass effect on the pulmonary artery and left atrium. She was given a steroid burst with a steroid taper prior to transfer to our hospital for higher level of care. Pediatric infectious disease, pulmonology, rheumatology, and hematology/oncology were all consulted. Differential diagnosis included: lymphoma, histoplasmosis, sarcoidosis. Right thoracotomy with mass biopsy was performed by pediatric cardiothoracic surgery. Pathology was consistent with fibrosing mediastinitis, likely secondary to histoplasmosis. She was continued on oral steroids and itraconazole. She was discharged home with repeat imaging and follow up arranged. At most recent follow up her mass has decreased in size, though she continues to deal with a chronic cough and has some night-time sweats. She has minimal limitations in physical activity. Currently, her specialists are debating initiation of Rituximab as the next best step in her treatment.

Fibrosing mediastinitis is a rare condition of fibrosing on the mediastinum from an abnormal immune reaction. This condition is typically seen in adult patients. Histoplasmosis, tuberculosis, sarcoidosis, other autoimmune disorders, and mediastinal radiation are all known causes of fibrosing mediastinitis, though many cases are idiopathic. Symptomatic therapies include vascular stents, airway dilation, esophageal stenting, and surgery. Targeted therapies include antifungals, glucocorticoids, and rituximab. Our patient received both antifungals and steroids. This case is unique given her presentation, young age, and the relative rarity of this diagnosis. She will require life-long therapy though currently is stable and overall doing well.

#35  Bilateral optic neuritis following Epstein-Barr virus infection

T Philip*

K Mather

L Campion

The University of Oklahoma School of Community Medicine, Tulsa, OK

Case Report

Optic neuritis (ON) is typically caused by demyelination of the optic nerves. Etiologies include autoimmune, inflammatory, or infectious causes. Epstein-Barr virus (EBV) is a rare cause of ON and should be included in the differential diagnosis as its presentation in children can be variable and difficult to distinguish.

Case

A 5-year-old female presented to the emergency department with a history of headache, vision changes, and fatigue with initial symptom resolution with acetaminophen seven days prior to presentation. Over the next several days, her parents noticed symptom recurrence leading to worsening visual and ambulation impairment. History revealed no recent infectious or toxic exposures and no family history of neurological disorders. On physical exam the patient was somnolent with bilateral vision loss, mydriasis, and sluggish pupillary light responses. Funduscopic exam performed by ophthalmology revealed bilateral mild optic disc edema. Initial laboratory results included an erythrocyte sedimentation rate of 64 mm/h, white blood cell count of 13.5 thousand, normal cerebrospinal fluid studies, and negative immune studies. Further findings included a positive mononuclear spot test, elevated EBV IgG early (D) antigen (anti-EA), and markedly elevated EBV antibody to nuclear antigen IgG (anti-EBNA) consistent with recent EBV infection. Additionally, a <1 cm focus of subcortical heterotopia was noted on brain MRI. Intravenous methylprednisolone was administered which led to marked improvement with ability to track objects by day two of treatment. The patient was eventually discharged on a 10-day oral prednisolone taper and referred for follow-up with outpatient ophthalmology.

Discussion

Epstein-Barr virus is a rare etiology of optic neuritis in children and should be considered in the primary workup in addition to MRI, lumbar puncture, autoimmune studies, and other infectious panels. A combination of visual and central nervous system changes can be observed, but these findings may be complicated in the pediatric population, due to difficulties obtaining a detailed history. It is important to inquire about symptoms such as somnolence, headache, and ataxia in combination with ocular findings such as papilledema, mydriasis, and vision loss. The combination of a positive anti-EA and anti-EBNA confirms the diagnosis of recent infection with EBV, which further supports the etiology of ON as it typically does not present during the acute infection period. The current standard treatment includes corticosteroids, which have been shown to accelerate symptom resolution. This case highlights the important role of EBV workup in pediatric optic neuritis and the wide variety of history and physical findings present as well as the importance of serologic studies and role of treatment in recovery.

#36  Mysterious cause of hypotension in MIS-C

SS Wang*

C Lares

C Marbrey

The University of Mississippi Medical Center, Jackson, MS

Case Report

Multisystem inflammatory syndrome (MIS-C) involves severe multi-organ inflammatory injury 2–6 weeks after COVID-19 infection. Seventy to 85% of patients have cardiovascular involvement, including diminished left ventricular ejection fraction (EF), coronary aneurysm, arrhythmias, valvular dysfunction, and pericardial effusion. Here we present a patient who arrived to the pediatric emergency department (ED) with MIS-C and suspected cardiogenic shock, though without the echocardiogram abnormalities commonly associated with MIS-C.

A 7 year old African American male presented for a third time to our ED over the course of 4 days of febrile illness and was found to have MIS-C. During this time, he had no chest pain, palpitations, shortness of breath, or abnormal cardiopulmonary exam. At the first 2 ED visits, he was generally well appearing and after treating fever, had vital signs normal for his age. At his third visit, his vital signs were notable for borderline hypotension 86/48 (threshold 83/39 for his height of 1.25 meters). Troponins, chest X-ray, and EKG were normal. Bedside ultrasound was normal, with EF 55–60% so the hypotension was presumed to be secondary to hypovolemia and sepsis. However, despite 40 mL/kg of fluid boluses and maintenance fluid x1.5, his blood pressure continued to downtrend to a nadir of 79/39. He soon developed an S3 gallop and facial edema indicating fluid overload. His proBNP 4986 pg/mL also resulted at this time, suggesting cardiac injury was present.

A formal cardiology echocardiogram confirmed the bedside ultrasound findings, noting normal ventricular size and motion, trivial pericardial effusion, and normal coronary artery size. However, it also detected diastolic dysfunction evident in mildly elevated E/e’ of 10.86 of lateral mitral annulus, and 12.7 at medial mitral annulus. Three hours after starting solumedrol for treatment of MIS-C, his blood pressure improved to 110/52. The patient had no further episodes of hypotension, though it is unclear if steroids had resolved this by alleviating the underlying inflammation or as a secondary effect.

We present a case of MIS-C that led to diastolic heart failure detected by mild hypotension, elevated proBNP, and subtle findings on formal echocardiogram. Although less common than systolic dysfunction in MIS-C, early recognition of diastolic heart failure is important for effective fluid management and initiation of vasoactive agents in criticallly `ill patients. Diastolic heart failure with preserved systolic function has been seen on echo of MIS-C patients, and is hypothesized to be the subacute period after recovery of systolic function. However, we did not find clinical symptoms of systolic heart failure prior to the patient‘s development of diastolic heart failure. It is therefore essential to recognize that a patient with MIS-C may present with diastolic heart failure without preceding symptoms or echo findings of other cardiac anomalies.

#37  Presentation and etiology of mediastinal masses in children: a case series

A Jagadish*

S Kilaikode

LSU Health Shreveport, Shreveport, LA

Case Report

Mediastinal masses can be difficult to diagnose and may be an incidental finding on imaging or present with diverse symptoms. Etiology differs based on location in the mediastinum. Here we see three cases of mediastinal masses in children having diverse etiology and varying presentations.

Case 1: A 2 yo male with history of G6PD deficiency presented with fever and cough for one week. On exam, patient was noted to have an abnormal lung exam, warranting chest x-ray which exhibited pneumonia and collapsed RUL. CT chest showed a large mediastinal and hilar mass. Given family history of death secondary to granulomatous disease, DHR was done and was low at 66.4%, concerning for CGD. Patient underwent bone marrow biopsy at a higher center which was negative for malignancy. Unable to obtain a biopsy of the mass as the family was concerned about consequences of anesthesia. Genetic testing revealed CYBB mutation, consistent with CGD. Patient was started on Interferon Gamma 1b injections and prophylactic Trimethoprim and Itraconazole. Follow-up CT chest done at 7 months showed significant improvement. Patient is well and continues to follow with multiple specialties.

Case 2: A 12 yo male with no significant PMH presented to the ED with dyspnea and left-sided chest pain. Chest x-ray revealed a focal left suprahilar opacity and CT chest reported a mediastinal lesion with multilocular fluid collection. Patient was diagnosed with pneumonia and discharged on antibiotics. Patient returned 3 months later with similar complaints. Repeat chest x-ray revealed persistence left suprahilar opacity. CT chest showed mass and multilocular fluid collections in the anterior mediastinum. Work-up was done to rule out malignancy, fungal etiology, and TB, all of which were unremarkable. Patient followed up with pediatric surgery and underwent robotic-assisted excision of the mediastinal mass. Pathology reported thymic hyperplasia with benign cystic changes. Currently, patient is well.

Case 3: A 12 yo female with history of asthma hospitalized for right upper back pain in the scapular region and acute worsening of chronic cough. Chest x-ray showed right-sided hilar lesion, pleural effusion and middle lobe atelectasis. CT chest revealed middle mediastinal soft tissue conglomerate mass with mediastinal lymphadenopathy. Leukemia, lymphoma, and TB were ruled out. Biopsy of the mass was negative for malignancy. Serology revealed histoplasmosis. She was treated with itraconazole and is currently stable.

Conclusion

Etiology and presentation of mediastinal masses in children differ based on location, which may be anterior, middle, or posterior mediastinum. When patients present with respiratory symptoms such as cough, we are inclined to assume the presence of a pulmonary infection. There must be a high index of suspicion if any abnormal mediastinal findings are noted in primary imaging. Further workup, including CT and biopsy, must be conducted in a timely manner and treated.

#38  Welcome to the club: the wild subtleties of hypersensitivity pneumonitis

C Braun1*

M Gunnett2

S Poore2

1The University of Alabama at Birmingham, Birmingham, AL

2Benjamin Russell Hospital for Children, Birmingham, AL

Case Report

Hypersensitivity pneumonitis (HP) is a rare cause of lung disease in children with an estimated prevalence of 4 per million cases.1 It presents with dyspnea, cough, and weight loss due to an inflammatory reaction following antigenic exposure, avian being the most common.1 Grise et. al. found that 96% of the CT scans of patients with HP had classical nodular appearance.2 The mainstay of treatment is elimination of exposure and immune modulation with corticosteroids. Inadequate or delayed treatment can result in irreversible fibrosis.2 We present an 8-year-old female initially misdiagnosed with recurrent pneumonia due to lack of characteristic CT findings, who eventually had HP confirmed by history and bronchoalveolar lavage (BAL).

Case presentation

An 8-year-old Hispanic female presented to pulmonary clinic due to recurrent pneumonia, anemia, and failure to thrive (FTT). For two years prior to admission, she had multiple episodes of a cough with associated pneumonia in various lung lobes that would improve with different courses of antibiotics (amoxicillin-clavulanate, ceftriaxone, azithromycin, doxycycline, and ceftazidime), but would eventually recur. Cystic fibrosis, primary ciliary dyskinesia, and immune deficiency workup was reassuring. A CT scan obtained during one of her acute illnesses revealed bilateral lower lobe consolidation, yet no ground glass opacification or nodular pattern. Given her course, she was admitted for intravenous antibiotics and further work up. Pulmonary function tests (PFT) revealed FEV1 49% and an x-ray revealed right lower lobe consolidation. Physical exam was notable for weight in the 1%ile, digital clubbing, and focal crackles. She underwent bronchoscopy with BAL revealing a 72% lymphocytic predominance. Further history revealed direct exposure to birds. Despite non-classic CT findings, the avian exposure, BAL results, digital clubbing, and FTT confirmed HP diagnosis. During her hospitalization, she had improvement of her cough, weight, and FEV1 to 68%. She received pulse dose of methylprednisolone and was discharged home on a 2 week steroid taper with instruction to avoid avian triggers.

Conclusion

This patient represents a unique presentation of HP without classical imaging findings likely due to her sporadic exposure to birds. Although rare, HP should be included in the differential for chronic cough, FTT, or digital clubbing. Our case highlights that patients can have HP without classical imaging findings and the importance of discussing certain exposures such as hay, plastic, or animals. Treatment with antigen avoidance and steroids is effective and prevents the complication of pulmonary fibrosis.1,2

References

1. Soumagne T, Dalphin ML, Dalphin JC. Hypersensitivity pneumonitis in children. Revue Des Maladies Respiratoires 2019;36(4).

2. Griese M, Haug M, Hartl D, et al. Hypersensitivity pneumonitis: lessons for diagnosis and treatment of a rare entity in children. Orphanet journal of rare diseases. 2013;8.

Case reports in cardiovascular medicine

2:00 PM

Thursday, February 10, 2022

#39  Respiratory symptoms of the 2019 novel coronavirus when the source of the symptoms is intra-cardiac thrombus not the pneumonia

M Abohelwa*

N Mittal

M Elmassry

S El Nawaa

Z Elharabi

G Del Rio-Pertuz

K Nugent

Texas Tech University System, Lubbock, TX

Case Report

The 2019 Novel Coronavirus (COVID-19) is currently causing a global pandemic. Common symptoms are fever, cough, myalgia, fatigue, headache, dyspnea, sore throat, vomiting, and diarrhea. Patients may present with end-organ failure, ARDS, shock, acute kidney injury, or even death. We present a case of COVID-19 with shortness of breath caused by an intra-cardiac thrombus.

Case presentation

An 84-year-old woman with COPD and diastolic heart failure presented with shortness of breath. She had hypoxemia on room air upon presentation. Lungs were clear on physical examination. COVID-19 PCR was positive. Her chest radiograph demonstrated no pulmonary infiltrates. Transthoracic echocardiography (TTE) demonstrated a large, irregularly shaped echogenic mass in both the right atrium and right ventricle consistent with a large thrombus. The mass in the right atrium was 3.9*3.6 cm; the portion in the ventricle was 3.2*2.2 cm. A previous TTE study in this patient did not reveal an intra-cardiac thrombus. No deep venous thrombosis was found. She was begun on anticoagulation and refused catheter-directed therapy. She improved and was discharged to her home.

Discussion

Thromboembolic complications of COVID-19 have been described in the literature. The most common are deep venous thrombosis and pulmonary embolism in critically ill patients despite the use of prophylactic anticoagulation. Several studies have reported post-mortem biopsies with widespread microthrombi. Arterial thrombosis with stroke and limb ischemia has also been described. Our case had an unusual presentation since the cause of her shortness of breath was the intra-cardiac thrombus. The pathogenesis beyond the hypercoagulability in COVID is not well understood. Some studies propose direct endothelial injury by the COVID-19 virus, causing microvascular inflammation, endothelial exocytosis, and endothelitis. Some experts propose a hypercoagulable state in COVID-19 patients based on elevated factor VIII, elevated fibrinogen, circulating prothrombotic microparticles, and neutrophil extracellular traps (NETs). Yet, no definitive mechanism has been identified.

#40  Post-covid myalgic encephalomyelitis in chronic heart disease patient: a case series

KG Holder*

V Vemulapalli

B Daines

A Kankam

B Galvan

R Nambiar

Texas Tech University System, Amarillo, TX

Purpose of Study

Myalgic encephalomyelitis (ME), also called chronic fatigue syndrome, is a condition characterized by severe fatigue that impairs a patient’s ability to perform common daily activities. Criteria for ME include 6 months of fatigue-limited daily activities, unrefreshing sleep, and symptom exacerbation following physical or mental strain, and orthostatic intolerance. New reports indicate that ME incidence may be higher in specific patient populations. This study was designed to investigate the association between ME and Cardiovascular disease in patients recovering from COVID-19 infection.

Methods Used

The patient population used for this study includes 19 patients that were referred to the Amarillo Heart Group in Amarillo, TX who also tested positive for Covid-19 at least 6 months prior to September 1, 2021. The patients that fit this timeline were asked a series of standardized questions and rate the severity of their symptoms on a scale of 0 to 5, with 0 being the absence of symptoms and 5 being the most severe. Two sets of questions were created and named Life Spheres Criteria (4 questions) and Symptoms Criteria (3 questions) based on the 2015 IOM Diagnostic Criteria for CFS. Rating more than 1 Life Spheres question as a 3 or higher or rating all 3 Symptoms Criteria questions as a 3 or higher indicated Chronic Fatigue Syndrome. Information from the survey, including time since infection, demographics, and question scores, were analyzed.

Summary of Results

Our study included 10 women and 10 men, with the average amount of time since Covid-19 infection being 328.17 ± 41.36 days. Worsening of symptoms with mild exertion was the most commonly endorsed criteria(3.58 ± 1.64) and the least common criterion was fatigue reducing activity in school (2.00 ± 1.94). Women scored higher in every category except reduced activity in school when compared to men. However, there was no significant difference in symptom scores between the two groups with the Combined Fatigue Score being 2.89 ± 1.47 for women and 2.67 ± 1.59 for men. Nearly all symptom scores significantly positively correlated with one another, meaning if one category was high it was likely for other categories to be high as well. Ultimately, when looking at the Cumulative Pearson Correlation Scores, reduced social life, difficulty concentrating, and symptoms worsening with mild exertion were found to be most predictive of a high Combined Fatigue Score.

Conclusions

In this case series, over 80% of patients met the criteria for Post-COVID Myalgic Encephalomyelitis. While the link between ME and both COVID-19 and cardiovascular disease has been established, little is known about the severity of ME in patients who have a history of both cardiovascular disease and COVID-19 infection. To our knowledge, this is the first study to examine ME in patients with both of these predisposing conditions. A high degree of clinical suspicion for ME should be used when screening and treating cardiac patients who have been infected with COVID-19.

#41  An unusual case of multiple idiopathic coronary artery aneurysms

T Fradella-Doucet*

A Ito

M Bank

CH Harris

N Jain

S Sanne

LSU Health New Orleans, New Orleans, LA

Case Report

Coronary artery aneurysms (CAAs), especially multiple, are a rare cardiac pathology and an unusual cause of cardiac chest pain.

Case

A 39-year-old African American woman with hypertension and non-insulin dependent type II diabetes mellitus presented with one day of chest pain. The pain was exertional, pressure-like, and 10/10 in intensity, but also aggravated by lying flat and with deep inspiration. She was evaluated five months prior for a similar presentation and had negative cardiac stress test and normal echocardiogram at that time. She followed with cardiology and was compliant with a regimen that included aspirin, statin, beta blocker, calcium channel blocker, thiazide and metformin. On evaluation in the ER, BP was 162/92 mmHg and physical exam revealed clear lungs, a regular heart rate and normal rhythm without murmur, no lower extremity edema and some chest wall tenderness with palpation. Troponin was elevated to 0.9 ng/mL and EKG showed diffuse, dynamic T wave changes. A transthoracic echocardiogram revealed new LVEF of 35–40% and severe inferolateral hypokinesis. A subsequent coronary angiogram revealed no atherosclerotic disease but multiple saccular coronary artery aneurysms: 6 mm in the left anterior descending artery (segment D1) and two 6–8 mm aneurysms in the left circumflex artery (OM1 lower pole and OM2 upper pole). The patient was discharged on long-term clopidogrel. Subsequent rheumatologic workup has been unrevealing and she continues to follow with cardiology.

Discussion

Coronary artery aneurysms involving multiple vessels is an extremely rare finding, particularly in this patient without any other significant risk factors. CAAs should be considered as a rare differential diagnosis in a patient with cardiac chest pain who does not fit the classic ACS illness script. CAA require close follow up, and can be managed percutaneously, surgically, or medically, depending on factors such as size, location, and if they are causing cardiac ischemia. Further reporting and study of this rare condition is crucial for better understanding and delineation of best management.

#42  Reverse takatsubo from profound hypokalemia

P Dhruva*

S Mikulic

MB Omar

P Reddy

University of Florida Health Science Center Jacksonville, Jacksonville, FL

Case Report

Reverse takotsubo cardiomyopathy is a rare variant of takotsubo cardiomyopathy which is characterized by basal wall ballooning and apical hyperkinesis. In this case, we present the first documented incidence of reverse takotsubo cardiomyopathy caused by profound hypokalemia.

A 62 year old male was resuscitated following cardiac arrest secondary to ventricular fibrillation in the field. After resuscitation, initial lab work was notable for hypokalemia with a potassium level of 2.5 and elevated cardiac biomarkers. The patient underwent emergent left heart catheterization which demonstrated angiographically normal coronary arteries. Interestingly, the cause of the patient’s cardiac arrest was determined by left ventricular angiography to be reverse takotsubo with a depressed EF of 20% (figure 1). Given that the patient had no recent physical or emotional stressors and no other significant lab findings, the etiology of the reverse takotsubo was determined to be hypokalemia caused by the paitent’s only home medication, hydrochlorothiazide. The patient’s hypokalemia was corrected by standard electrolyte replacement. The patient’s reverse takotsubo was managed with standard therapy of a beta blocker and ACE inhibitor. Repeat imaging 6 months later demonstrated resolution of the patient’s reverse takotsubo and a normal EF. It should be noted that, takotsubo cardiomyopathy is a rare syndrome characterised by temporary and reversible left ventricular dysfunction that is provoked by a stressor that can be either physical or emotional. There are 4 types of takotsubo cardiomyopathy; reverse takotsubo is unique in that it causes ballooning of the left ventricular base rather than the apex. As evidenced in this case, takotsubo cardiomyopathy is generally considered a diagnosis of exclusion and paitents must undergo detailed evaluation in order to exclude alternative causes of left ventricular dysfunction prior to diagnosis.

Abstract #42 Figure 1

Hyperdynamic left ventricular apex (circle) relative to ballooned base on transthoracic echocardiogram (A-diastole, B-systole) and left vetriculogram (C-diastole, D-systole)

#43  Heart failure secondary to non-compaction cardiomyopathy

AA Ali*

N Maaliki

J Ruiz-Morales

K Esmail

University of Florida College of Medicine – Jacksonville, Jacksonville, FL

Case Report

Left Ventricular Non-Compaction (LVNC) is a rare congenital cardiomyopathy which carries a high risk of malignant arrhythmias, thromboembolic phenomenon and left ventricular dysfunction. This form of cardiomyopathy remains unclassified by the WHO and only recently the American Heart Association’s 2006 classification of cardiomyopathies considers noncompaction cardiomyopathy a genetic cardiomyopathy.

A 48-year-old male with no known comorbidities presented with new onset heart failure, NYHA Class III, ACC/AHA Stage C. TTE revealed severe global hypokinesis, EF 20%. Prominent trabeculations were noted in the LV apical and anterolateral segments. Stress cardiac MRI revealed non-compaction of the LV with a dilated cardiomyopathy. There was no evidence of stress induced myocardial ischemia. The patient responded to intravenous diuresis and was counselled on the diagnosis of LVNC cardiomyopathy, fitted with a LifeVest and discharged on warfarin anticoagulation.

LVNC is congenital cardiomyopathy characterized by extensive endomyocardial trabeculations and recesses within the ventricular cavity. These trabeculations are the result of an aberrant embryonic development, in which the initially ‘spongy’ myocardial fibrils fail to condense into the smooth, well-vascularized, and mature myocardium. The clinical sequelae of LVNC mainly involve congestive heart failure (CHF), arrhythmogenesis, thromboembolism, and a small percentage of patients may remain asymptomatic. The diagnosis is made through imaging, with echocardiography as the first-line method. Cardiac Magnetic Resonance Imaging has also emerged as a potentially superior method due to its 3-dimensional nature and higher image quality. After the diagnosis of LVNC is made, treatment is directed at the different elements of disease. Goal-directed medical therapy with Beta-blockers and Angiotensin-converting enzyme inhibitors/angiotensin receptor blockers is indicated in LVNC patients with CHF, with most strategies deduced from the regular treatment of Heart failure with reduced ejection fraction. Due to the increased thromboembolism risk of LVNC, the strategy of anticoagulation has been a subject of debate.

Due to the genetic nature of the disease, it is recommended that a thorough 3 generation family history is obtained and genetic testing be done in appropriate relatives if a specific genetic mutation was determined. Genetic cardiomyopathies should be considered in the differential diagnosis of young patients presenting with symptoms of heart failure with no known comorbidities or prior history of cardiac disease.

Abstract #43 Figure 1

Cardiac MRI with prominent trabeculations

#44  Gender differences in cardiac implantable electronic devices (cieds) and systolic heart failure hospitalizations: data from the national inpatient sample 2016–2018

DC Voruganti*

A Kumar

V Chidambaram

JL Mehta

University of Arkansas for Medical Sciences, Little Rock, AR

Purpose of Study

Gender differences in systolic heart failure (HF) patients for the implantation of various cardiac implantable electronic devices (CIEDs) using ICD-10 have not been studied. We aim to explore the gender differences for each type of procedure.

Methods Used

The National Inpatient Sample (NIS) 2016–2018 was used to obtain the hospitalizations with HF (ICD 10 CM codes I5020, I5021, I5022, I5023). Pacemaker/Defibrillator procedures were obtained using ICD 10 procedure codes. Demographic data were obtained using the variables provided in the NIS

Summary of Results

We identified 4,341,313 HF hospitalizations from January 2016 to December 2018. Overall, two third of patients were male (63.15%). Majority of hospitalizations were in the Whites (66% males & 63% females). Females were substantially greater Medicare beneficiaries (74% vs. 67% males). Among the CIEDs, males had a higher rates of procedure utilization compared to females : Percutaneous insertion of defibrillator in right ventricle (1.61% in males vs. 1.05% in females); Insertion of defibrillator generator, open surgery (1.15% in males vs. 0.72% in females); Percutaneous insertion of defibrillator lead in right atrium (1.13% in males vs. 0.75% in females); Cardiac resynchronization therapy-pulse generator (0.88% in males vs. 0.57% in females). Reword to support the conclusion.

Conclusions

Despite minimal differences in baseline characteristics, implantation of CIEDs appear to be utilized less often in women than in men, less often in blacks than in white, and more often in urban hospitals. Further studies are required to confirm these findings and further explore gender differences.

#45  Shock that: a retrospective study of the safety and effectiveness of intravascular lithotripsy for in-stent restenosis

JC Holbert*

MK Teshome

RN Khouzam

The University of Tennessee Health Science Center College of Medicine, Memphis, TN

Purpose of Study

Coronary calcium is an independent risk factor for adverse outcomes in coronary artery intervention. Modification of this calcium via intravascular lithotripsy is accomplished through acoustic pressure waves that disrupt the calcium and improve vessel compliance. Studies have shown intravascular lithotripsy is effective in the management of heavily calcified de novo coronary lesions. Evidence for use in in-stent restenosis is limited and is still off-label. The purpose of this study is to evaluate the effectiveness and safety of intravascular lithotripsy for management of calcium-mediated in-stent restenosis.

Methods Used

A retrospective, single-center study was performed for four cases of in-stent restenosis with evidence of significant underlying calcium burden resulting in stent under-expansion and probable calcium intimal neoplasia. Lesions were treated with intravascular lithotripsy (Shockwave Medical). Angiographic success was defined as residual luminal stenosis of less than 30% and improvement in Thrombolysis in Myocardial Infarction (TIMI) flow. Complications were defined as vessel dissection, decrease in Thrombolysis in Myocardial Infarction flow, recurrent anginal symptoms, or death.

Summary of Results

Four patients presented with symptomatic in-stent restenosis (60 to 80%) with previous angiography demonstrating significant calcium burden at site of involved stent placement. In-stent intravascular lithotripsy followed by angioplasty with non-compliant balloon inflations at high pressures was performed. In all cases, there was improvement in luminal stenosis to less than 30% as well as improvement in Thrombolysis in Myocardial Infarction flow to at least 2 (N = 3, TIMI 3; N = 1, TIMI 2). There were no complications identified in the study group.

Conclusions

The role of intravascular lithotripsy has been established in patients with de novo calcified lesions. This study demonstrates the effectiveness and safety of intravascular lithotripsy for calcium-mediated coronary in-stent restenosis. Given the challenges of in-stent restenosis, particularly associated with underlying calcium, additional studies are warranted.

#46  Acute nonischemic myocardial injury with high sensitivity troponin and its prognostic implications

B Mohanakrishnan1*

M Khan1

B Mohammed1

R Nambiar2

1Texas Tech University Health Sciences Center, Amarillo, TX

2Amarillo Heart Group, Amarillo, TX

Case Report

Approach to acute nonischemic myocardial injury with high sensitivity troponin

Introduction

62-year-old male with a past medical history of CVA, hypertension was admitted following recurrent episode of substernal chest pain, provoked by exertion, not relieved by rest or nitroglycerine, and reproducible. Other risk factors include smoking half a pack-year for 45 years, hyperlipidemia, BMI 34, no family history of heart disease, carvedilol, aspirin, and statin. On admission, his BP was 158/113, HR 94/min, examination showed chest wall tenderness. EKG shows no acute ischemic changes, and Troponin was 103ng/l. His urine toxicology was positive for amphetamine and cannabinoids with serial troponins of 110 ng/l and 105 ng/l. Previous records show a chronically elevated troponin average of 93 in the past two months and 124ng/l last year. The nuclear stress test showed normal LV function, and TTE confirms no motion wall abnormalities with an EF of 60%. The BP was controlled with IV labetalol and amlodipine. He was observed for two days without any further events. Chest pain was more suggestive of costochondritis than cardiac. He was recommended to avoid weight lifting one week, smoking and drug cessation, and follow up with a cardiologist. In all three admissions, the patient was admitted and discharged with elevated troponins with no definite diagnosis.

Discussion

Potential reasons a patient with clinically suspected acute MI may be misdiagnosed can be a) Test-related issues, b) myocardial injury not related to coronary artery atherothrombosis, and c) acute myocardial injury not related to the coronary circulation. In most high-sensitivity cardiac troponin (hs-cTn) assays, the 99th percentile URL values are higher in men than in women. The United States recommends sex-specific cut-off values; however, not adopted in Europe. A subgroup of these patients have a Type 2 MI, consequent to increased oxygen demand or decreased supply, and will not have significant epicardial coronary artery disease when coronary angiography is performed.

Conclusion

The patients with acute ischemia and elevated troponin benefit from diagnostic coronary angiography and possible percutaneous coronary intervention. This may almost be true with hs-cTn assays, as the increased sensitivity means that more significant numbers of patients with type 2 MI will also be detected. Hence, these patients may not benefit from an invasive approach.

In most studies, short- and long-term mortality rates were higher in type 2 MI than in type 1 MI patients. In a multivariable model accounted for competing risk of death between subgroups, the adjusted 5-year risk of MACE was lower in type 2 MI versus type 1 MI with risk ratio, 0.74 [95% CI, 0.62–0.88]). Higher mortality but similar or lower MACE rate among type 2 MI and nonischemic myocardial injury versus type 1 MI advocates this risk of death is from comorbidities rather than by complications of ischemia or necrosis.

#47  Resynchronizing a systemic right ventricle with left ventricular septal pacing

R Derenbecker*

S Dunn

M Cribbs

W Maddox

UAB Hospital, Birmingham, AL

Case Report

The patient is a 38-year-old man with a history of dextro-transposition of the great arteries for which he underwent an atrial switch (Senning) operation with VSD closure at 3 years of age. As an adult, he required pacemaker and ICD implantation for complete heart block and ventricular tachycardia. He endorsed functional class II symptoms in clinic. Cardiac CT demonstrated a hypertrophic and dilated systemic right ventricle (SRV) with an end-diastolic volume of 312 ml and ejection fraction of 32%. Right and left heart catheterizations demonstrated no coronary disease, normal filling pressures, and a preserved cardiac index. ICD interrogation revealed that the subpulmonic LV threshold was elevated and the battery was at replacement time. He had a 100% left atrial and left ventricular pacing burden without an underlying escape rhythm. ECG showed a QRS duration of 188 ms with a right bundle branch block (RBBB) pattern consistent with LV apical pacing.

He was referred for generator replacement and ventricular resynchronization. A plan was made to attempt His bundle pacing, and if that failed, to implant a new pacing lead in the LV septum. Left axillary venous access was obtained and an Agilis HisPro (Abbott, Inc.) steerable catheter was advanced to the subpulmonic LV via the superior systemic venous baffle. The His bundle could not be located, so we proceeded with placement of a lead in the interventricular septum. We first attempted placement of a Medtronic 3830 pacing lead; however, due to the tortuosity of the baffle, we could not affix the lead. We advanced a Medtronic 5076 pacing lead and, using a primary and secondary curved stylette, successfully positioned and affixed the lead to the LV septum. Pacing from this location demonstrated appropriate pacing and sensing parameters. ECG demonstrated persistent RBBB QRS morphology but with a narrower QRS duration of 140 ms, suggesting that more physiologic pacing was accomplished. Three months later, the patient reported functional class I symptoms. Repeat cardiac CTA imaging showed a reduction in RV end-diastolic volume from 312 mL to 279 mL and improvement in RV ejection fraction to 40.5% from 32%.

Abstract #47 Figure 1

Leads seen: preexisting LA and ICD lead, new LV septal lead

#48  Too young for heavy metal: environmental toxin diagnosis and the value of the social history

E Mertens1*

B Kirkwood2

N Soyster3

J Joseph3

A Molina1

EO Schmit1

W Rushton2

1The University of Alabama at Birmingham, Birmingham, AL

2The University of Alabama at Birmingham School of Medicine, Birmingham, AL

3The University of Alabama at Birmingham Department of Pediatrics, Birmingham, AL

Case Report

A 2-year-old male presented to the Emergency Department (ED) for the 3rd time in 2 months with staring spells and mild encephalopathy. Parents had also noted irritability, headaches, emesis, diarrhea, generalized erythematous rash, lymphadenopathy, and intermittent fevers. Symptoms started shortly after the family moved into a new home from out of state 2 months prior to presentation but had worsened in the preceding 2 weeks. Lab results during the prior two ED visits included microcytic anemia and elevated ESR; investigation for Kawasaki disease including TTE was negative and on both occasions was diagnosed with a presumed viral infection.

On this visit to the ED, he was afebrile and tachycardic. Exam showed a tired, irritable, but consolable male with tachycardia with regular rhythm, faint bibasilar crackles, occipital and inguinal lymphadenopathy, excoriations in various stages of healing over torso and legs. Workup revealed mild hyponatremia, elevated ESR, and reassuring head CT. He had an episode concerning for seizure while in the emergency department. Continuous EEG demonstrated focal seizures and MRI was unremarkable. He was started on oxcarbazepine and discharged home. Following discharge, patient continued to be sleepier than normal and re-presented to the ED. At this fourth visit, he had an unremarkable LP and persistently elevated ESR. He was noted to be hypertensive and tachycardic, for which EKG, BNP, and echo were unremarkable. A detailed social history was obtained and revealed the unexpected deaths of two family cats who died shortly after moving to the area, raising concern for environmental toxins. A CT chest/abdomen/pelvis during admission demonstrated extensive adenopathy but was overall reassuring against malignancy. A heavy metal panel was sent and resulted with a mercury concentration of 50 mcg/L (ref: <10 mcg/L). Patient was subsequently given chelation therapy. Further inquiry and EPA investigation ultimately discovered mercury concentrated in the patient’s room, later determined to be a spill from an old ink bottle found by the prior homeowners.

Discussion

This case highlights several challenges with identification of a subacute pediatric environmental exposure. Acute mercury inhalation injury secondary to a solution being spilled then inhaled over at least a month presented initially as an acute upper respiratory illness. Later, the initial morbilliform rash was recognized as acrodynia, an idiosyncratic hypersensitivity to mercuric salts. Finally, the hypertension encountered in our patient presumably resulted from mercury’s inhibition of enzymes that catabolize circulating endogenous catecholamines, leading to a pseudopheochromocytoma presentation. Unfortunately, each of these symptoms can be found in a spectrum of clinical pathologies. The key to diagnosis in this scenario was the identification of the unexpected death of two pets that ultimately led to the timely detection of a very dangerous environmental toxin.

#49  Closure of chronic wound in a patient with sickle cell disease using serial continuous peripheral nerve block infusions

JP Brandt1*

M Herr1

J Jones2

A Chandrashekar2

1The University of Tennessee Health Science Center College of Medicine, Memphis, TN

2The University of Tennessee Health Science Center, Memphis, TN

Case Report

The management of pain in vaso-occlusive crises (VOC) in patients with sickle cell disease (SCD) often involves opioids, putting patients at risk for adverse effects. However, the management of VOC-related pain with continuous peripheral nerve blocks (CPNBs) shows potential as a viable alternative to opioids. The purpose of this study is to examine the outcome of a patient with a chronic non-healing medial malleolar ulcer related to repeated VOC which was effectively managed with a series of CPNBs.

Methods Used

A 37-year-old female patient with SCD and acute-on-chronic pain due to a VOC-related chronic ulcer on her right medial malleolus was referred to the Acute Pain Service. The patient required daily oral opioids for pain control and had been hospitalized 15 times for pain control since the wound developed. With informed consent, a CPNB of the saphenous nerve was performed midthigh with an 18-gauge Touhy needle. An elastomeric pump was attached, and an infusion of 0.2% ropivacaine was initiated at 4 mL/hr. The patient was allowed to adjust the rate as needed after discharge. One week later, the pump was removed with no complications. These techniques were repeated periodically over a span of 176 days. Wound healing progress was documented with photos.

Summary of Results

The wound was reduced to a small scab, and the patient no longer required opioids to manage her chronic wound pain. Hospital admissions for pain control decreased during the period of CPNB infusions and the average length of stay decreased by 2.6 days. Pain scores and opioid dosage also decreased. These metrics support the use of CPNBs for their ability to decrease costs and reduce opioid dependence.

Conclusions

CPNBs can be used to effectively eliminate acute or acute-on-chronic pain and reduce opioid use, healthcare costs, and hospital visits in patients with VOC related to SCD. The degree to which the serial CPNBs contributed to the closure of the chronic wound in this case requires further study, however, there are plausible mechanisms by which a CPNB might contribute to wound closure. For example, the sympathectomy-induced vasodilation by perineural local anesthetic infusions increases blood flow and oxygen delivery to wounds, counteracting the peripheral vasoconstriction observed in reaction to acute pain.

Abstract #49 Figure 1

Top: day 10, 18, 43; Bottom: day 80, 148, 739

Adolescent Medicine and Pediatrics

Joint Plenary Poster Session and Reception

4:30 PM

Thursday, February 10, 2022
#50  Acute food refusal in 12 year old with anorexia nervosa requiring sedation and mechanical ventilation for enteral feedings

A Andrucioli*

JJ Burns

C Garrison

University of Florida, Pensacola, FL

Case Report

Anorexia Nervosa is a mental health disorder with significant morbidity and mortality. Acute food refusal is one of the indications for admission. We present a patient who went to extreme lengths to restrict food intake, requiring intensive care sedation and ventilation to enable enteral feedings.

12 year old male, was admitted with symptoms of anorexia nervosa and BMI of 12.0, (<1%ile) with baseline BMI of 16 (25%ile), K of 3.3 and glucose of 54. He was treated with supervised eating on an inpatient pediatric floor with no need for enteral feeding. Psychiatry consultation confirmed the diagnosis of anorexia nervosa and recommended the addition of Olanzapine to his Sertraline. He was discharged pending placement in an eating disorder center after 21 days of hospitalization with discharge BMI of 14.

He was followed as an outpatient by his pediatrician, dietician and counselor but unfortunately, he required readmission 11 days after discharge due to acute food refusal, with BMI that had dropped to 13.1. Patient was readmitted and started on nasogastric (NG) feeds but he became severely agitated, pulling NG out multiple times and continued to lose weight with BMI dropping to 12. Sedation was attempted to facilitate maintenance of NG feedings, with Benadryl, Haldol and Ativan, but was ineffective at levels deemed safe without compromising his airway and breathing.

Due to severe malnourishment and unsuccessful NG feeds he was transferred to PICU for sedation, endotracheal intubation and continuous nasoduodenal (ND) tube feedings on two separate occasions while inpatient. He was able to wean from the ventilator but once awake he found ways to manipulate delivery of his calories, even finding scissors and cutting the ND tube. The patient ultimately agreed to eat in order to avoid replacement of the feeding tube.

He was finally transferred to an eating disorder facility, with a BMI of 13.9 and persistent anorexia thinking with restriction of eating anything but pizza. Patient completed three months of an inpatient program and had significant improvement in BMI to 19.3 (70%ile). He was subsequently discharged for continued outpatient follow-up and since discharge from the eating disorder center, his BMI has shown steady improvement in outpatient follow-up. He shows no signs of food refusal and is doing well with Family Based Therapy.

This case highlights several unique characteristics in management of eating disorder patients. The age and being male along with extreme food refusal and resistance to enteral feeding that led to the requirement of deep sedation are quite unusual and not well described in the medical literature. The severity of his illness was a significant barrier to inpatient placement. In addition, despite a nationwide attempt to find an inpatient facility for him, which took several weeks, we identified shortages in eating disorder beds that have been exacerbated by the COVID-19 pandemic.

#51  Impact of obesity on hospital admission and length of stay in children with status asthmaticus (SA)

S Battle*

K Lasota

R Mehta

J Chen

Augusta University, Augusta, GA

Purpose of Study

Asthma is an obstructive, inflammatory lung disease which is often recognized by wheezing and increased work of breathing. Although mild exacerbations may be controlled in an outpatient setting, an episode of significant exacerbation may lead to status asthmaticus (SA). With increased prevalence in pediatric populations, SA generates concern for hypoxic injury and mortality. Obesity is also associated with adverse outcomes leading to significant morbidity in asthmatic populations. However, the association in children, particularly those with acute asthma has remained poorly defined. This study aims to investigate the relationship between obesity and SA in children requiring hospital admission.

Methods Used

The charts of patients aged 2–18 years with SA admitted to the Children’s Hospital of Georgia (CHOG) from July 2020 to August 2021 were reviewed in a prospective manner. The data collected included the following: age, which was divided into 3 groups ( 2–5, 6–12, >12), gender, severity of asthma, insurance type (none, medicaid or private), risk factors (smoking exposure, split family), comorbidities, clinical asthma score, inpatient medical management, discharge medications, weight percentile for their age ( < 50th , > 50th, and > 85th), and length of stay (LOS) in Pediatric Intensive care unit (PICU) and or hospital. Patient with weight percentile greater than 50% were considered obese and weighing more than the 85th percentile were designated significantly obese. Prolonged LOS was considered greater than 48 hours in the PICU and or inpatient. Patients with chronic lung disease ( e.g. Cystic Fibrosis) were excluded from the study.

Summary of Results

A total of 163 (female 67, male 96) charts were analyzed. Multivariable contingency analysis using Fisher’s exact test was performed with response to assess p- values. Nominal logistic regression was used to assess the covariates. The number of patients in the 3 age categories were 59, 75, 29 respectively. There was a difference in the 3 age categories for LOS with a p-value of 0.27. Patients with significant obesity were admitted more often and had prolonged LOS in comparison to non obese patient in all the 3 age categories, p-value: 0.048. Patients with mild obesity had similar results. Patients with no insurance or medicaid had more admissions and prolonged stays but the difference was not statistically significant. ( p-value 0.06). The logistic regression model analysis revealed no difference in hospital admission rate LOS when compared with with gender (p-0.24), severity of asthma (p-0.16), or social factors (p-0.16)

Conclusions

Childhood asthma is a common illness within the pediatric population. In our study it appears that obesity is a major morbidity factor in childhood asthma. It plays a significant role in exacerbation of acute asthma leading to hospital admission and increased LOS. Further studies are needed to explore the relationship of obesity and acute asthma.

#52  Coexistence of child maltreatment and intimate partner violence: a retrospective look at outcomes

C Beeson1*

AD Hendrix1

J Jenkins1

R Munoz2

M Baxter1

S Passmore1

L Conway1

1OU/TU School of Community Medicine, Tulsa, OK

2The University of Oklahoma Anne and Henry Zarrow School of Social Work, Norman, OK

Purpose of Study

The term intimate partner violence (IPV) describes physical or sexual violence, stalking, or psychological harm by a current or former intimate partner or spouse. The literature establishes a strong association between IPV and childhood maltreatment, which often co-occur. Long term consequences of childhood IPV exposure include increased risk of future victimization, perpetration of violence, and higher risk for sexual abuse. It is still unclear if screening for other forms of violence in the home during a child maltreatment evaluation is beneficial. We hypothesized children experiencing both maltreatment and IPV at their index medical exam are more likely to have medical exams for additional abusive events in the future.

Methods Used

A retrospective cohort study was conducted following children in Oklahoma who were diagnosed with one or more forms of confirmed or suspected child maltreatment and assessed at the Tulsa Children’s Advocacy Center (CAC) from October 1, 2016-September 30, 2019. Each child’s first visit during the study timeframe acted as the index encounter for IPV screening. Hospital consults were excluded from analysis, however; follow-up visits at the CAC were included as index visits. Demographic, medical, and social history data were collected from the electronic medical record for visit with a suspected or confirmed child maltreatment ICD-10 code. Analysis included descriptive statistics and Welch’s ANOVA comparing the mean number of events for children with documented IPV exposure vs children without documented IPV exposure.

Summary of Results

A total of 1370 children met study inclusion criteria with most being white (n=811, 59.2%), non-hispanic or Latino (n=810, 59.1%), and female (n=724, 52.8%) and a mean age of 6.99+4.59 years. The top diagnosis codes used were Child Physical Abuse, Suspected (n=612, 42.68%), Child Physical Abuse, Confirmed (n=359, 25.03%) and Child Sexual Abuse, Suspected (n=298, 20.78%). Two hundred and eighty-nine children (21.1%) had a history of IPV documented in their initial medical exam. Of those children with known IPV history, only 11 had additional medical exams prompted by new allegations of abuse compared to 26 children with no-IPV. There was not a statistically significant difference in the mean number of medical exams between the IPV and no-IPV cohorts (p=0.336).

Conclusions

While this analysis did not yield statistically significant results, it does create additional questions regarding the effectiveness of screening, and if the presence of IPV influences the decision to remove children from the home, thus mitigating the chance for future abusive events. We postulate that IPV screening is inadequate and fails to catch many cases due to the multifactorial nature of disclosing violence in the home, as well as the hidden and taboo nature of family conflict. We plan to expand our data to include non-medical visits related to new abusive events in our future analyses.

#53  Decision-making and reported condom use of juvenile justice-involved youth

D Yang1*

A Huang1

A Davidson1

LJ Benjamins1,2

1The University of Texas Health Science Center at Houston John P and Katherine G McGovern Medical School, Houston, TX

2Wayne State University, Detroit, MI

Purpose of Study

Prior studies have demonstrated patterns of risky decision-making and sexual health practices among juvenile justice-involved youth. However, there is limited research on the relationship between these two behaviors. The purpose of the study is to examine reported condom use and decision-making skills among juvenile justice-involved youth.

Methods Used

Youth at a large, urban juvenile detention facility in the Southwestern US completed a four-part survey assessing: education and goals; condom use; the Decision-Making Scale (DMS); and the Post-Detention Likelihood to Succeed Scale (PDLSS). Within the DMS, four constructs were tested: generating options, considering consequences, evaluating decisions, and decision-making efficacy. Both the DMS and PDLSS used a four point Likert scale.

Summary of Results

91 youth participated; 89 completed both the DMS and PDLSS. 82.4% were male and 86 (94.5%) were sexually active. Of the eighty-six, 52.3% reported using condoms during sexual activities ≥50% of the time. However, only 33.7% reported using a condom during their most recent sexual encounter. Most common reasons given for not using condoms included knowing the status of their partner, disliking the feel of condoms, and not having a condom available at the time. Respondents who reported ≥50% condom usage scored higher on the DMS than those reporting <50% condom usage. The ≥50% condom group scored higher in all four of the DMS constructs, however this did not reach statistical significance. Additionally, no difference was observed for the PDLSS.

Abstract #53 Figure 1

Decision-making scores by reported condom use

Conclusions

Youth in this study who reported more frequent condom use scored higher on the DMS compared to their peers who reported <50% condom use. Although the findings were not statistically significant, there was a trend among all four of the DMS constructs. Results from this study can be used to shape educational programs designed to foster healthy decision-making among juvenile justice involved adolescents. Future study plans involve recruiting a larger sample of female participants and assessing relationships between short-term/long-term goals and sexual behaviors.

#54  Acute paralysis as an initial presentation of graves’ disease thyrotoxicosis

S Conner*

EW Larsen

L Campion

L Stuemky

American Academy of Pediatrics, Tulsa, OK

Case Report

Graves’ disease is an autoimmune disease that affects multiple systems, including the thyroid, skin, and eyes. The most common cause of Graves’ disease is hyperthyroidism, which affects almost all patients. It is caused by thyrotropin stimulating receptor autoantibodies, which activate the receptor and stimulate excessive thyroid hormone synthesis and secretion. Thyrotoxicosis is when the excess thyroid hormone synthesis and secretion causes clinical manifestations, including tachycardia, weight loss, diarrhea, pretibial myxedema, and proptosis. An overactive thyroid can also cause electrolyte abnormalities, such as hypokalemia, which can lead to acute paralysis. Initial treatment includes symptomatic care with beta blockers, such as atenolol until thionamides can be started, or radioiodine ablation or surgery.

Case Description

A 16-year-old previously healthy male with no significant past medical history presented from an outlying emergency department with tachycardia, acute paralysis upon waking, and hypertension. Thorough history revealed he had several missed school days due to diarrhea and chills. He also had a family history of Brugada syndrome in his older brother. Physical exam revealed a well-appearing male with tachycardia, without proptosis, periorbital edema, or pretibial myxedema. Initial testing revealed significant hypokalemia of 2.8, hypertension (146/90), and sinus tachycardia of 130 bpm. Further work up revealed a decreased TSH of <0.01, elevated Free T4 of 2.8, and increased Anti-Thyroglobulin antibody of >1000 and anti-TPO antibodies of 378. Pediatric Endocrinology was consulted and recommended thyroid imaging and starting atenolol 100 mg daily. His hypokalemia was corrected with 20 mEq intravenous potassium chloride, and thyroid uptake scan showed increased uptake concerning for Graves’ disease.. During outpatient follow up, he was continued on atenolol 100 mg and methimazole 10 mg BID by Pediatric Endocrinology, and referred to Pediatric Cardiology for persistent tachycardia and family history of Brugada syndrome. He was also referred to Electrophysiology for further evaluation.

Discussion

Graves’ disease thyrotoxicosis can have varying clinical manifestations, including electrolyte disturbances such as hypokalemia, which can cause acute paralysis. Although the cause of hypokalemia in Graves’ disease is unknown, it is hypothesized that elevated levels of thyroxine increase Na+/K+ ATPase activity, which results in an increased shift of potassium into cells. Thus, causing decreased potassium in the circulation and leading to hypokalemia. While hypokalemia may not be the first presenting symptom of Graves’ disease, any patient with complaints of acute paralysis upon waking should be evaluated and treated for hypokalemia. Persistent symptoms of tachycardia, should be further evaluated to rule out other pathologies.

#55  A non-infectious cause of chronic sinusitis

J Coren*

LSU Health New Orleans, New Orleans, LA

Case Report

Granulomatosis with polyangiitis is a microscopic vasculitis that can cause sinusitis (as well as other ear nose and throat issues), disease of the lung parenchyma, glomerulonephritis, and signs and symptoms of systemic inflammation. A 12 year old girl presented from ENT clinic following a 9 month history of chronic recurrent sinusitis. She had failed multiple antibiotic regimens for presumed bacterial sinusitis and culture of sinuses had consistently shown no growth. She had a 10 pound weight loss in the two weeks prior to admission. Other symptoms included headache and chest wall pain with deep inspiration. 1 week prior to presentation, she was seen in the emergency room where a maxillofacial CT was obtained and showed pan-sinusitis. On admission, Vital signs were within normal limits and physical exam was unremarkable other than the patient having dried blood around the inside of both nares. Laboratory workup showed leukocytosis and thrombocytosis as well as elevated ESR and CRP. ALT was mildly elevated but CMP was otherwise within normal limits. UA was significant for microscopic hematuria. Repeat CT confirmed previous finding of pansinusitis. A chest xray was obtained which showed an apical lung nodule. Diagnostic workup showed elevated c-ANCA titer and biopsy of sinuses showed vasculitis with necrosis and granulomas consistent with a diagnosis of granulomatosis with polyangiitis. Although this patient presented with sinusitis, she was also found to have lung and kidney involvement which is often seen with this type of vasculitis. The patient was started on a course of prednisone and azathioprine as induction therapy and is following with rheumatology. This case presents us with typical findings associated with granulomatosis with polyangiitis and reminds us of the importance of reevaluating a patient’s diagnosis when therapeutic interventions fail. While GPA is usually seen in older patients, it is important to be aware that it can present at any age.

#56  Testosterone levels in adolescent males hospitalized for malnutrition

M Duarte*

H Baker

M Anderson

AB Middleman

The University of Oklahoma, Oklahoma City, OK

Purpose of Study

Previous studies among malnourished disordered eating patients have shown differences in menstrual patterns between female patients with Avoidant Restrictive Food Intake Disorder (ARFID) and Anorexia Nervosa (AN), potentially related to the fat content of recently consumed food. Little has been written about testosterone levels and testosterone level recovery among malnourished males. The purpose of this study is to describe testosterone levels in adolescent males at both presentation and later in hospitalization for refeeding.

Methods Used

Patient data of all males admitted to a disordered eating unit at a children’s hospital between January 2014 and December 2020 were reviewed. Of these, 40 males had a testosterone level drawn, of whom 16 had two or more testosterone levels recorded during a single inpatient stay. Data extracted during retrospective chart review included BMI on admission;%mean estimated BMI (MEBMI) was calculated per CDC growth charts. Additional data points collected included eating disorder (ED) diagnosis, sexual maturity rating (SMR) , demographic variables including age, and whether the patient had eaten ‘junk food’ defined as fried foods, chips, pizza, traditional fast food, sugared soda, sweets or desserts in the reported diet history from the 24 hours prior to admission. Analysis included frequencies, determinations of normality for continuous variables, T-test or Wilcoxon-Mann-Whitney test (as appropriate), and robust regression.

Summary of Results

34 male patients were included in the initial analysis. Their mean age was 16.3 years. 17 patients had a diagnosis of AN; 17 patients had a diagnosis of ARFID. The mean age (16.7 for ARFID, 15.9 years for AN) and median SMR (5, for the patients for whom it was available) were not significantly different between groups, nor were testosterone levels on admission to the hospital. Patients with ARFID (72.0%) had a lower%MEBMI (p=0.003) at presentation than those with AN (82.9%). Regression analysis using testosterone as the dependent variable and controlling for%mean estimated BMI did not show a significant association of ED diagnosis with initial testosterone level. The proportion of subjects reporting junk food consumption 24 hours before admission was not significantly different among those with ARFID (88%) and those with AN (69%). Among the 16 patients with more than one testosterone value, mean age was 15.8 years, median BMI was 15.3 kg/m2,%MEBMI was 77.9%, and median time to second measured testosterone value was 11.5 days (range of 3–30.5 days). Paired t-test showed significantly greater testosterone levels at the second evaluation than the first. The mean difference between testosterone levels 1 and 2 was 156 ng/dL (p=0.01).

Conclusions

Testosterone levels for malnourished adolescent males rebound quickly with refeeding. ED diagnosis and consumption of ‘junk food’ do not appear to be independently associated with testosterone levels at admission.

#57  A case of orbital cellulitis complicated by mucormycosis in a diabetic teen

NP Glynn*

J Collette

C Sandlin

LSU, Louisiana State University, New Orleans, LA, New Orleans, LA

Case Report

Patients with diabetes mellitus may experience increased infection frequency and severity, particularly those with poor glucose control. The immunocompromised state in poorly controlled diabetic patients is caused by the hyperglycemic environment that favors immune dysfunction. Mucormycosis is a rare invasive fungal infection which occurs primarily in immunocompromised individuals, mostly in patients with diabetes. Invasive mycosis is associated with high mortality and morbidity.

An 18-year-old male with a past medical history of poorly controlled diabetes mellitus and multiple prior admissions for diabetic ketoacidosis (DKA) presented with left-sided eye pain and headaches for three days. Initial examination revealed mild periorbital swelling and pain, intact extraocular movements, and normal vision. He soon progressed to have limited upward gaze (-3 to -4) and adduction (-0.5) along with blurred vision in the left eye. Laboratory results were significant for a hemoglobin A1C of 13.7, baseline creatinine of 1.10, and no evidence of concurrent DKA. Admission computed tomography (CT) showed orbital apex inflammation with a 1 cm subperiosteal-appearing abscess along the orbit, lamina papyracea erosion, and ethmoidal air cell mucosa thickening.

The patient was started on meningeal doses of vancomycin and pipercillin/tazobactam. Initial fiberoptic endoscopic sinus surgery to drain the abscess revealed healthy appearing tissue and no purulent drainage, with resultant negative cultures and histopathology. Several surgical procedures were necessary to provide a definitive histopathological diagnosis and containment of the fungal infection. Ultimately, sp. rhizomucor was identified. The patient was treated with three doses of intra-orbital amphotericin B and three weeks of intravenous liposomal amphotericin B. He transitioned to oral isavuconazole for an additional three weeks upon discharge. Subsequent magnetic resonance imaging (MRI) showed concern for thrombophlebitis of the superior ophthalmic vein, necessitating prophylactic dosing of Lovenox during his inpatient course. His insulin regimen was closely monitored and adjusted during hospitalization, as tight blood glucose control was important to his clinical progression.

This case of non-classic orbital cellulitis highlights the importance of recognizing the increased risk of rhinocerebral mucormycosis in patients with poorly controlled diabetes mellitus. Suspicion for mucormycosis should prompt surgical debridement with tissue biopsies adequate for histopathology and initiation of systemic antifungals if the course appears rapidly progressive. In our patient, aggressive techniques were used to successfully contain and treat the fungal infection thus limiting morbidity and mortality associated with his diagnosis.

#58  Improving oral health in primary care through fluoride varnish application

D Hicks1,2*

J Yaun1,2

1The University of Tennessee Health Science Center, Memphis, TN

2Le Bonheur Children’s Hospital, Memphis, TN

Purpose of Study

Dental caries is one of the most common chronic diseases of childhood. USPSTF and Bright Futures guidelines recommend fluoride varnish application in the medical home to the primary teeth of all infants and children starting at primary tooth eruption as part of health maintenance and preventative care. The resident continuity clinic at UTHSC is the medical home to a large number of patients at risk for dental caries. Prior to this project, providers were not routinely conducting dental screenings and fluoride varnish was not being applied in clinic. This project was implemented to improve the oral health of the patients seen in the resident clinic by routinely performing dental screenings and applying fluoride varnish to eligible patients.

Methods Used

A multidisciplinary team was created to establish a clinic-based screening and application program following a traditional PDSA model. An oral risk assessment tool and screening form was created using AAP and Bright Future oral health guidelines. These assessed a child’s risk for caries and access to dental care. Dental screenings and fluoride varnish applications were implemented at routine well child checks (every 6 months from 6–60 months of age). Completed fluoride varnish applications were recorded weekly for review.

Summary of Results

Thus far, the project has been implemented for a total of eight weeks with one PDSA cycle completed. Of eligible patients screened, a total of 39 patients at high risk for dental caries have received a fluoride varnish application.

Conclusions

Dental screening and fluoride varnish are an important part of primary care, are part of the Bright Futures guidelines, and can be implemented in resident pediatric clinics to help improve the oral health of high-risk pediatric populations. Further PDSA cycles are needed to improve dental screening and fluoride varnish application in this ongoing QI study.

#59  Don’t do drugs: a case of levamisole- induced leukocytoclastic vasculitis

A Llorens Bonilla1*

CM Casas Loyola1

M Hernandez Puig1

JJ Nieves1,2

1Universidad de Puerto Rico Recinto de Ciencias Medicas, San Juan, Puerto Rico

2Centro Cardiovascular de Puerto Rico y del Caribe, Rio Piedras, Puerto Rico

Case Report

Cocaine is a highly addictive stimulant drug that has surged in use and mortality in the United States in the past five years. Approximately 70% of street cocaine is contaminated with levamisole, a synthetic agent used to potentiate its effect by increasing endogenous opioids and dopamine levels in cerebral reward pathways. In medicine, cocaine has been the culprit of many diseases. Nevertheless, its ability to mimic other etiologies can make the diagnosis a challenge. This is the case of a young female whose history and physical findings were vital for diagnosing a rare drug-induced vasculitis.

A 30-year-old female with chronic cocaine abuse was admitted with bilateral lower extremity ulcers for IV antibiotics and surgical cleansing and debridement. Upon evaluation, she also complained of tender black lesions throughout her body that had worsened in the past week after snorting cocaine. Associated symptoms included subjective fever, general weakness, and pain. She reported the use of intranasal and IV cocaine and smoked a pack of cigarettes daily. She was hemodynamically stable and afebrile. On physical examination, dry gangrene was observed on her left second finger. Multiple tender retiform purpuric lesions were observed on arms, abdomen, distal digits, and bilateral toes. Her ears had a painful, dusky purple rash with erythematous borders and necrosis, mainly on helix/antihelix. Lower extremity ulcers were treated with debridement and antibiotics. Labs were remarkable for leukopenia and iron deficiency anemia. Renal function was preserved. Hepatitis panel, RPR, and HIV were negative. However, she had +cryoglobulins, +p-ANCA, +cardiolipin IgG/IgM, +ANA(1:320, homogenous), and +DS-DNA (1:20). No imaging evidence suggestive of osteomyelitis. Up to this point, the differential diagnosis included autoimmune connective diseases, septic vasculitis, antiphospholipid syndrome, blood dyscrasias, and drug-induced etiologies. Skin punch biopsy was remarkable for leukocytoclastic vasculitis with intraluminal thrombi. The patient‘s history of cocaine use, clinical morphology of lesions, and anatomic distribution, as well as histopathologic findings, were consistent with levamisole-induced leukocytoclastic vasculitis (LIV). The patient was treated with supportive management given no end-organ damage was identified. Cessation of cocaine was advised to avoid recurrences.

Levamisole-induced leukocytoclastic vasculitis is associated with 3 clinical syndromes: destructive skin lesions, cutaneous vasculitis, and agranulocytosis. Therefore, diagnosis is achieved based on clinical presentation linked to histopathological findings. Interestingly, LIV can mimic other rheumatologic etiologies by presenting +ANA, +DS-DNA, + cardiolipins, and +cryoglobulins. Knowing about LIV clinical presentation, histopathology, and laboratory findings is imperative to establish an accurate diagnosis and determine if management is supportive or more invasive (immunosuppressive therapy).

#60  Pediatric case of covid-19 associated rhabdomyolysis

M Markow*

CG Lares Romero

MN Frascogna

The University of Mississippi Medical Center, Jackson, MS

Case Report

According to the Center for Disease Control, Severe Acute Respiratory Syndrome Coronavirus 2 (COVID-19) may present with a wide range of symptoms. Among those, fever, cough, and shortness of breath are commonly present. While COVID-19 associated myalgias is reported as a symptom, we present a case of COVID-19 related rhabdomyolysis.

This is a previously healthy 13-year-old obese male who arrived to an outside hospital in respiratory distress after a 1-week history of nasal congestion, productive cough, shortness of breath, emesis and diarrhea associated with malaise and muscle weakness sensation. On presentation he was hypoxic (O2 sat 87%), tachypneic and had increased work of breathing for which was initially treated with 2 liters per minute of oxygen by nasal cannula and fluid resuscitation. The patient had a rapid COVID antigen test that was negative, a chest x-ray that showed bilateral infiltrates (worse on the right), normal complete blood count and normal electrolytes. However, he was found to have an elevated serum creatinine (1.2), an elevated creatinine kinase level (1264 U/L) and serum myoglobin (97.2 ng/ml) leading to him being diagnosed with acute kidney injury secondary to viral induced rhabdomyolysis and pneumonia. He was treated with antibiotics (Azithromycin and Ceftriaxone) and aggressive fluid resuscitation and transferred to our hospital for further care. At our facility, antibiotics were discontinued after a COVID-19 PCR testing resulted positive. Aggressive fluid resuscitation was continued to treat rhabdomyolysis and he received a course of Remdesivir and Decadron to treat COVID-19 infection. The patient then recovered and was discharged home on the 5th day.

Rhabdomyolysis is a life-threatening condition that occurs when muscle necrosis results in the release of its intracellular contents into circulation. In the pediatric population, viral myositis has been reported as a leading cause. It is commonly diagnosed by elevation of serum creatine kinase (CK) and presence of myoglobinuria. Clinically, patients with rhabdomyolysis may be asymptomatic or present with severe disease characterized by myalgias, massive CK enzyme elevations, severe electrolyte imbalances and acute kidney injury. While rhabdomyolysis has previously been reported as a rare complication of COVID-19 infection, there are fewer reports of rhabdomyolysis secondary to COVID-19 in the pediatric population. This case illustrates the importance of suspecting this life threating condition in patients with COVID-19 infection complaining of myalgia or muscle weakness to avoid the severe complications. Of note, while we did not test specifically for the Delta variant, given the time frame of the patient’s presentation, we may consider the possibility of COVID-19 Delta variant related rhabdomyolysis.

#61  Influence of sex, ethnicity, socioeconomic status, and breastfeeding status on development of childhood obesity

N Tantivit1*

D Preud’homme1

M Mulekar1

N Nasomyont2

1University of South Alabama, Mobile, AL

2Cincinnati Children’s Hospital Medical Center, Cincinnati, OH

Purpose of Study

To identify influence of male sex, African American, low socioeconomic status, and non-breastfeeding status on development of overweight and obesity on children age 2, 3 and 4 years old.

Methods Used

A retrospective cohort study of children born at University of South Alabama Children’s and Women’s Hospital from May 2011 to October 2013. Included participants had at least one well child visit between ages 2 years to 4 years old. Preterm birth, small for gestational age, and patient with comorbidities were excluded from the study.

All collected data were organized in an Excel spreadsheet and analyzed using statistical software JMP 15.2.0. All categorical data was summarized using percentages. Association between categorical variables was studied using Fisher’s test or Chi square test.

Summary of Results

There were 1,178 children were identified by ICD codes. A total 612 children met inclusion and exclusion criteria for this study.

At 2 years of age, there were 401 children included. No significant association were observed between overweight or obesity and sex (p = 0.0596), race (p = 0.078), socioeconomic status (p = 1.000) or breastfeeding status (p = 0.3643).

At 3 years of age, there were a total 329 children included. No significant association were observed between overweight or obesity and sex (p = 0.8374), race (p = 0.2229), socioeconomic status (p = 0.8462) or breastfeeding status (p = 0.6035).

At 4 years of age, there were a total 328 children included. No significant association were observe between overweight or obesity and sex (p = 0.8448), race (p = 0.1864), socioeconomic status (p = 0.6852) or breastfeeding status (p = 0.3050)

Abstract #61 Table 1

Conclusions

No association between sex, race, socioeconomic status or breastfeeding status and childhood overweight or obesity at age 2, 3 and 4 years.

Environmental factors and lifestyle, rather than biological factors, may play importance role in childhood obesity. Further studies are warranted.

#62  Screening for extragenital sexually transmitted infections during the COVID-19 pandemic

AC Ybarra1*

LJ Benjamins1,2

1The University of Texas Health Science Center at Houston John P and Katherine G McGovern Medical School, Houston, TX

2Wayne State University School of Medicine, Detroit, MI

Purpose of Study

A prior study conducted at a large, Southwestern juvenile detention facility in 2019 revealed that only 33.5% of youth reporting oral sex and 25.9% of youth reporting anal sex were tested for extragenital sexually transmitted infections (STIs). Since many extragenital infections are asymptomatic, testing is crucial for diagnosis and treatment. For the past year and a half, the COVID-19 pandemic has created new challenges for medical care, especially in detention centers where large numbers of youth reside close together. While several studies have found recent decreased rates of STI screening, little is known about how the pandemic affected screening for extragenital STIs for juvenile justice involved youth.

Methods Used

A retrospective chart review from March 1, 2020 to June 31, 2021 was conducted; only youth seen by a physician for an intake physical exam were included. During the intake physical exam, youth are asked if they have ever put their tongue or mouth on someone’s penis, vagina or anus and if anyone has put their penis in their anus. The answers to these questions determine which youth are eligible for screening of extragenital infections. The answers to these intake screening questions, the completion of extragenital testing, and lab results were input into an excel spreadsheet. This data was analyzed using Excel. This study was approved by the University of Texas Health Science Center and the Juvenile Justice Center internal review boards.

Summary of Results

A total of 574 youth were seen for their intake physical by a physician during this time. 86% were male, the mean age was 15.4 (range 11–17), and 48.6% were black. Of these youth, 224 (39%) reported performing oral sex and 128 of those youth (57%) were tested for oropharyngeal STIs. 7 had unsatisfactory samples, while 1 (0.78%) had oral gonorrhea and 1 (0.78%) had oral chlamydia. There were 20 (3.48%) of all youth attesting to having received anal sex. Of these, 4 (20%) were tested for anal STIs and all were negative for anal gonorrhea or chlamydia.

Conclusions

The testing for oropharyngeal STIs significantly increased despite the COVID-19 pandemic, from 33.5% to 57%. However, the testing for rectal STIs decreased from 25.9% to 20%. While the prevalence of extragenital STIs at our facility also decreased during the pandemic, it is still necessary to continue testing since many extragenital STIS are largely asymptomatic. As providers offer more testing, it will be important for them to appropriately educate patients and decrease stigma of extragenital testing so that screening rates can improve.

Adult case reports, aging and geriatrics

Joint plenary poster session and reception

4:30 PM

Thursday, February 10, 2022
#63  A case of necrolytic acral erythema associated with chronic hepatitis C

L Abusalem*

S Matlock

HK Wong

University of Arkansas for Medical Sciences, Little Rock, AR

Case Report

A 50-year-old gentleman known to have chronic hepatitis C and polysubstance use disorder presented with a skin rash of several months’ duration involving the soles of both feet and the palmer aspect of both hands with sparing of the hand joints. Labs were notable for negative antinuclear antibody panel, and serum Zinc level of 24.2 (normal reference range of 60–120 ug/dL). Skin biopsy showed an interface dermatitis with prominent basilar as well as superficial dyskeratosis, confluent parakeratosis, and a vacuolar interface pattern suggestive of Necrolytic Acral Erythema. This is a pruritic skin disease that’s a rare extrahepatic manifestation of hepatitis C and is strongly associated with zinc deficiency.

The patient was offered zinc replacement therapy (which should be given regardless of serum levels as epidermal levels can be low and zinc plays a role in necrolysis) in addition to antiviral therapy which he could not afford due to socio-economic barriers

On the left, the dorsal aspect of the right hand shows erythematous plaques with variable scaling and erosions. On the right, palmer aspects of both hands show erythematous and violaceous plaques with desquamation and sparing of the metacarpophalangeal, proximal and distal interphalangeal joints

The histologic features in this skin biopsy light microscopy slide show an interface dermatitis with prominent basilar as well as superficial dyskeratosis. In accordance with these features and in light of the patient‘s lab work showing a very low serum zinc level, and in conjunction with his known history of hepatitis C virus (HCV), the possibility of this rash representing a nutritional deficiency is somewhat favored, specifically necrolytic acral erythema.

#64  A case of cefiderocol-induced posterior reversible encephalopathy syndrome

L Abusalem*

T Sharma

H Salah

JC Rico-Crescencio

R Jagana

University of Arkansas for Medical Sciences, Little Rock, AR

Case Report

Posterior Reversible Encephalopathy Syndrome (PRES) is a constellation of neurological symptoms associated with characteristic neuroimaging findings in the parietal and occipital lobes most sensitively diagnosed using Magnetic Resonance Imaging (MRI). It has been recognized as a potential complication of allogenic stem cell transplantation as it is associated with several immunosuppressants and requires the exclusion of underlying infectious and metabolic causes. We present a unique case of a young female who developed seizures while being treated with Cefiderocol for Acinetobacter bacteremia & urinary tract infection (UTI).

This is a 43-year-old female with myelodysplastic syndrome who underwent allogenic stem cell transplantation complicated by Graft-Versus- Host-Disease (GVHD) for which she received steroid therapy & Tacrolimus. She presented to the hospital with nausea, abdominal pain, diarrhea and was admitted for management of Central Line Associated Bloodstream Infection & UTI due to Acinetobacter Baumanii. Her Tacrolimus was stopped on admission due to active infection and thrombocytopenia. She was started on intravenous Cefiderocol then developed generalized tonic clonic seizures within 24 hours of drug initiation that were eventually controlled with two antiepileptic medications. Patient was not found to have metabolic or electrolytes disturbances to account for her seizures. Her serum Tacrolimus level was 13.6 (normal reference range 5–20 ng/ml) prior to the onset of seizures. Brain MRI showed multiple areas of T2 and FLAIR hyperintensity involving the cortical subcortical region of bilateral parieto-occipital lobes consistent with PRES. Cefiderocol was discontinued, her blood pressure was controlled with a titratable Nicardipine infusion with subsequent improvement in her mental status. Foley catheter was inserted after transfer to the medical intensive care unit and patient’s urine was incidentally found to be purple in color.

It’s often challenging to make the diagnosis of drug-induced PRES in stem cell transplantation recipients as they’re often on multiple immunosuppressants and are more susceptible to drug resistant microorganisms. Her seizures & encephalopathy persisted despite discontinuation of Tacrolimus and strict control of her hypertension. In this case we believe Cefiderocol, a cephalosporin used to treat multi-drug resistant gram negative pathogens, is the culprit as patient’s symptoms started to improve with its discontinuation. Her Purple Urine Bag Syndrome was only an incidental finding likely due to high bacterial load in her urine. More research is needed about the potential neurotoxicity of Cefiderocol

#65  Assessment of depression and anxiety in residents of a continuing care retirement community during the covid-19 pandemic quarantine

EA Aguilar1

S Barry1*

M Chavez1

A Chapple2,3

R Ducote1

A Johnson3

L Ali1

R Pattabhi1

P Poudel1

TM Reske1

1LSU Health New Orleans, New Orleans, LA

2Louisiana State University, Baton Rouge, LA

3LSUHSC, LSU Health New Orleans, New Orleans, LA, US

Purpose of Study

During the COVID-19 pandemic, the State of Louisiana implemented a quarantine to decrease the risk of infection. This situation led to a decrease in social interaction which is a risk factor for anxiety and depression, among the elderly population confined in residences. The objective was to determine whether being quarantined while living in a residential community would negatively affect the mental health of the elderly.

Methods Used

A short longitudinal assessment and analysis to assess anxiety and depression. Data was initially collected, then at 6 weeks, and 12 weeks during the quarantine. We used the Geriatric Depression Scale (GDS) and Geriatric Anxiety Inventory (GAI) as these screening tools are designed for the elderly, to assess the incidence and severity during the quarantine.

Summary of Results

57 residents of a Continuous Care Retirement Community (CCRC) in the New Orleans metropolitan area, stratified as nursing home residents, assisted living, and independent living of a 37 (80%) of the 46 patients were females, with a mean age of 86.1 (SD 9.1) years old; 25 (54.3%) were nursing home residents, 13 (28.3%) were in assisted living, and 8 (17.4%) were in an independent living community.

16 (34.8%) patients were diagnosed with depression before taking the survey, and five (10.9%) had previously diagnosed anxiety. Anxiety scores decreased significantly from baseline (average 4.35 vs. 3.28, p-value =0.045) at 6 weeks but did not change from time 0 to week 12 or week 6 to week 12. Depression, scores did not change significantly between the time periods.

Conclusions

Our data indicate a trend toward an increase in depression and anxiety during periods of pandemic quarantine isolation. Because the elderly are particularly susceptible to loneliness and resulting depression assessing these indicators may help mitigate the economic burden and cognitive decline resulting from the complications of depression and anxiety in the elderly population residing in community centers during periods of unanticipated extended quarantine isolation.

#66  Dress syndrome with reactivation of epstein-barr virus in patient taking lamotrigine

LF Castro*

J Garza

AL Bell

MA Elmassry

J Sekhon

J Lalmuanpuii

Texas Tech University Health Sciences Center, Lubbock, TX

Case Report

Drug Reaction with Eosiniphilia and Systemic Symptoms (DRESS) syndrome is a cell-mediated hypersensitivity reaction involving CD4+ and CD8+ cells that produce tumor necrosis factor-alpha and interferon-gamma. It is often triggered by high-risk drug exposure that includes aromatic anticonvulsants, allopurinol, and sulfonamide-containing antibiotics. The disease presentation commonly includes systemic symptoms, hematologic abnormalities, diffuse maculopapular rash, and organ involvement. In this case report we will present a case of DRESS syndrome and EBV reactivation in a patient taking lamotrigine.

Case

A 20-year-old female with a history of bipolar type 2 disorder was admitted for a diffuse maculopapular rash. Four weeks earlier, she had been prescribed lamotrigine and was instructed to titrate the dose by 50 mg every week until she reached 200 mg twice daily. The following day she started experiencing a rash on her right hand. During the following 72 hours, the rash spread to the rest of the body with associated fever (103.6° F), nausea, and vomiting. She was advised to go to the emergency center, where she was diagnosed with an allergic reaction and discharged with ondansetron and methylprednisone dose-pack. Despite taking her medication, the fever and rash persisted over the following 2 days, and she also started having trouble producing urine despite adequate fluid intake. On readmission, the patient presented with a fever of 105.6° F and a maculopapular rash on greater than 80% of body surface area with areas of erythroderma but without oral mucosa or genital involvement. Dermatology started her on topical triamcinolone and hydrocortisone due to suspicion of DRESS syndrome and she was admitted for observation

On day one of hospitalization, abdominal examination was remarkable for hepatosplenomegaly. Complete blood count (CBC) with differential was remarkable for leukocytosis of 14.96 k/mL and eosinophilia (1.08%). Complete metabolic panel was remarkable for transaminitis. A monoSpot test was positive for EBV. Patient was also oliguric despite having normal BUN and creatinine. Over the following days, the patient developed anuria, acute kidney injury, and transient thrombocytopenia. She was treated with pulse-dose steroids. At the time of discharge, the patient’s laboratory results normalized, and her urine output increased. She was discharged with a 10-week taper of corticosteroids, and she was doing well at her one-week outpatient appointment.

Discussion

There is evidence that reactivation of latent herpesviridae infection is common in patients with DRESS syndrome. It is unclear whether the culprit drug can also cause reactivation of EBV or whether reactivation of EBV in a patient taking a high-risk drug can cause DRESS syndrome. More research is needed to elucidate the pathophysiology behind EBV reactivation in patients with DRESS syndrome.

#67  Superior syndrome, poor prognosis

RM Covington*

R Patel

JA Puckett

R Noor

The University of Mississippi Medical Center, Jackson, MS

Case Report

Superior vena cava (SVC) syndrome is a potentially life-threatening condition that results from compression or obstruction of the SVC. This can cause a variety of symptoms such as jugular venous distension, facial plethora, hoarseness, cough, neurologic symptoms, syncope, and swelling of the face, neck, or upper extremities. SVC syndrome is commonly due to a malignant mass and can potentially be a medical emergency depending on severity. We present an interesting case of SVC syndrome due to a newly diagnosed malignant mediastinal small cell carcinoma.

A 69-year-old African American male presented to the Emergency Department (ED) with complaints of neck swelling and shortness of breath over the previous week. He first noticed the swelling after waking up in the morning six days prior to presentation. He reports noticing his neck was more swollen on the right than left, non-painful, and had progressively increased in size over the week before arrival to the ED. He also reported a non-exertional shortness of breath that began around this same time. Other noted symptoms included significant hoarseness of his voice and an unintentional 12-pound weight loss over the previous month. Upon arrival in the ED, he was hemodynamically stable and breathing comfortably on room air. A chest x-ray in the ED showed widening of the mediastinum, and subsequent Computed Tomography (CT) imaging showed mediastinal lymphadenopathy with significant narrowing of the SVC and right pulmonary artery. Interestingly, this patient had no facial or upper extremity swelling, no facial plethora, and a negative Pemberton’s sign on exam. Exam findings were significant for neck swelling and palpable, swollen anterior cervical and supraclavicular lymph nodes. Other lab work was significant for a mild acute kidney injury which improved with IV fluid resuscitation. Due to symptoms and imaging findings concerning for malignancy, the patient underwent endobronchial ultrasound bronchoscopy with transbronchial needle aspiration and biopsy of 4 right mediastinal lymph nodes with Interventional Pulmonology the next morning. He was also evaluated by Cardiothoracic Surgery during admission which recommended no urgent surgical intervention for SVC compression at the time. The patient was discharged with follow-up with oncology to discuss treatment options. Unfortunately, pathology from biopsy of the nodes later showed a new diagnosis of malignant, metastatic small cell carcinoma.

In summary, SVC syndrome is a potentially emergent and dangerous condition that presents in various ways. In this case, the clinical exam findings were consistent with mild to moderate SVC syndrome, since the patient was stable and no emergent vascular intervention was required. Although SVC syndrome is often thought of as an emergent condition, recent literature shows us that it can often be a relatively insidious, minimally symptomatic complication that still strongly raises suspicion for an underlying malignancy.

#68  Esophagopleural fistula in the setting of pneumotosis intestinalis and oropharyngeal carcinoma

G Dharmarpandi*

J Dharmarpandi

D Harper

Texas Tech University Health Sciences Center School of Medicine Amarillo, Amarillo, TX

Case Report

A 64-year-old Caucasian male with chronic obstructive pulmonary disease (COPD), gastroesophageal reflux disease (GERD), and stage IV oropharyngeal cancer diagnosed within the past few months and G-tube placement two weeks prior presented with a one day history of abdominal pain, nausea, and coffee-ground emesis. He denied diarrhea, constipation, hematochezia, fever, and dyspnea. CT abdomen revealed pneumobilia and pneumatosis intestinalis. Vitals showed hypotension, hypothermia, tachypnea, and tachycardia. After 1 liter of crystalloid bolus was administered, repeat radiographic imaging revealed right-sided pneumothorax with effusion. A chest tube was placed and dark brown fluid containing amylase was drained.

Four hours after initial presentation, the patient underwent awake tracheostomy followed by exploratory laparotomy. Intraoperatively, a large air leak was noted through the chest tube with the initiation of mechanical ventilation. The following day, a re-exploratory laparotomy was conducted in addition to a bronchoscopy, which revealed no abnormalities despite continuous leakage through the tracheostomy tube. Four days later, an esophagogastroduodenoscopy was performed and an abnormality was found 35 cm from the teeth mark along the right lateral wall of the esophagus. Further examination revealed an esophagopleural fistula (EPF), confirmed with the administration of methylene blue through the defect and the return of the dye through the right thoracostomy tube.

An impediment in the diagnosis of EPF is the subtlety with which it can present in a patient with serious illnesses. The patient’s initial complaint of abdominal pain as a result of pneumobilia and pneumatosis intestinalis masked the diagnosis of EPF. In addition, the symptoms most commonly associated with EPF, such as cough, dyspnea, and regurgitation can be easily misdiagnosed as or easily explained by other diseases like COPD or GERD. The problem with reaching this diagnosis is further compounded by the inadequacy of imaging to visualize the defect, which can be small enough to evade radiological inspection but large enough to induce inflammation and infection of mediastinum resulting in sepsis, multiorgan dysfunction, and/or death. In this patient’s initial CT, the large pneumothorax with effusion obscured the pathologic connection between the esophagus and the pleura. EPF is primarily caused by thoracic esophageal perforation due to surgery, trauma, malignancy, or radiation. In this case, the cause is potentially due to G tube insertion, prior endoscopic instrumentation, and/or oropharyngeal carcinoma causing erosive changes in adjacent tissue leading to fistula formation. Treatment of the esophagopleural fistula would involve surgical debridement of the affected portion of lung, irrigation of the pleural cavity, and closure of the pathological orifices, typically with through endoscopic placement of a stent or through the use of a over-the-scope-clip.

#69  Different kind of aura: migraine presenting as transient ischemic attack

G Dharmarpandi

Y Tawfeeq*

T Shihab

T Naguib

Texas Tech University Health Sciences Center School of Medicine Amarillo, Amarillo, TX

Case Report

A 64-year-old male with transient ischemic attack, type 2 diabetes, dyslipidemia, symptomatic bradycardia with pacemaker placement, and migraine on aspirin/paracetamol/caffeine presented with headache, aphasia, perioral numbness, and right hand and foot weakness for one hour. CT head was unremarkable. The patient had an NIH stroke scale of 11 and was administered tissue plasminogen activator. CT angiogram revealed 50% stenosis of the vertebral artery with hypoplasia noted on past imaging. Within 24 hours, his symptoms completely resolved. MRI was not done due to pacemaker. Upon investigation, the patient had a history of similar episodes five times in the past five years. Patient’s last admission presented as syncope and blurry vision prior to the onset of headache, left face numbness, and perioral tingling that all resolved spontaneously within 24 hours without administration of tissue plasminogen activator. Carotid doppler showed 40% stenosis of bilateral internal carotid arteries. Pacemaker interrogation revealed normal sinus rhythm with no paroxysms or episodes of atrial fibrillation. Family history is negative for migraines and positive for stroke leading to hemiplegia in grandfather. Due to a suspected cerebrovascular accident on antiplatelet therapy, he was advanced to dual antiplatelet therapy and he reported compliance. Additionally, it was thought that the patient’s right vertebral artery stenosis may have contributed to the patient‘s symptoms and he was advised to avoid tilting his head and bending his neck to the right.

During the most recent visit, workup did not reveal any imaging evidence of stroke, significant vascular stenosis, arrhythmia, cardiac embolic source, patent foramen ovale, or significant risk factors for recurrent strokes on dual antiplatelet therapy. In addition, the patient‘s history of five migraines with auras that include fully-reversible motor weakness and language symptoms that are not accounted for by another diagnosis make hemiplegic migraine the most likely diagnosis. The leading theory for hemiplegic migraines is spreading depression of neuronal and glial depolarization across the cerebral cortex, but may also involve alterations in cranial blood flow and production of factors such as calcitonin gene-related peptide that can cause vasodilatory changes leading to neurogenic inflammation. This patient has underlying vascular abnormalities, but it is unlikely they were significant enough to cause symptoms. During an initial evaluation of headache and hemiplegia, it is necessary to rule out intracranial hemorrhage, mass, infection, ischemic stroke, seizure with postictal paralysis, metabolic disturbances, and inherited vascular disorders. However, when a patient has repeated episodes of hemiplegic migraines, it is important to make the diagnosis and reserve stroke workup and treatment for deviations in presentation.

#70  Air in the wrong place: a case of pulmonary artery air embolism after intravenous contrast injection

S Duangkham*

A Cavazos

U Sharma

H Mezughi

K Nugent

Texas Tech University Health Sciences Center, Lubbock, TX

Case Report

Venous air embolism is an uncommon but potentially catastrophic event that occurs when air enters the systemic venous circulation and travels to the right ventricle and pulmonary circulation.Here we present a case of pulmonary artery air embolism after intravenous contrast injection.

A 59-year-old female patient with Marfan syndrome, complete heart block status post BiVentricular pacemaker, Protein S deficiency, history of pulmonary embolism, chronic combined systolic and diastolic heart failure, and nephrolithiasis with recent right-sided percutaneous nephrolithotomy who presents to ER with right flank pain. Patient was initially found to be hypotensive with with mean arterial pressure of 48 and was found to be hypoxic at her arrival saturating 86% on room air. Computed tomography angiogram of chest, abdomen and pelvis showed no acute findings in the arterial system, no aortic dissection, prominent volume of air within the central pulmonary artery and right ventricle. Right perinephric hematoma with active arterial extravasation. Patient was also found to be anemic. Hemodynamic status improved with blood transfusion, oxygen support and fluid resuscitation. Due to her stable hemodynamic status, and lack of symptoms, patient was treated with conservative management. Urology was consulted for further management regarding right perinephric hematoma. Patient continued to improve with no further complications.

Surgery, trauma, vascular interventions, and barotrauma from mechanical ventilation and diving are the most common causes of air embolism.Small amounts of air can be removed from pulmonary vascular bed by gas diffusion into the alveolar space. Air bubbles in the pulmonary microcirculation can cause endothelial damage, activation of complement and release of medicators from inflammatory cells resulting in noncardiogenic pulmonary edema, bronchoconstriction, and hypoxemia. Clinical spectrum can range from no symptoms to cardiac arrest depending on the degree of severity of embolism, end-organs affected, and underlying comorbidities. Dyspnea is the most common symptom that may be accompanied by chest pain, dizziness. Air embolism should be suspected in patient who has sudden onset cardiopulmonary decompensation in the setting of a known risk factor. Diagnosis is made based upon a high index of suspicion, exclusion of other life-threatening processes and best by demonstrating air in the intravascular space. Definitive therapy, including hyperbaric oxygen, manual removal of embolized air is indicated in patient with evidence of cardiopulmonary compromise or end-organ damage due to air embolism.

Venous air embolism is uncommon, potential life-threatening condition.There are no pathognomonic features of air embolism and many patients have no symptoms. Airway, breathing, circulation should first be assessed and maintained. Definitive therapy is indicated in patient with hemodynamically unstable. Ultimately, efforts should be made to prevent air embolism.

#71  Shock value vt storm in mssa bacteremia

S Duangkham*

A Wichmann

U Sharma

M Abohelwa

K Nugent

Texas Tech University Health Sciences Center, Lubbock, TX

Case Report

Ventricular arrhythmias are common in patients with heart failure (HF).Ventricular tachycardia (VT) storm represents a life-threatening event refractory to ICDs. We present case of VT storm in a patient with heart failure with reduced ejection fraction (HFrEF).

A 57-year-old male with history of coronary artery disease status post 3 vessel bypass, ischemic cardiomyopathy, HFrEF status post single chamber ICD, paroxysmal atrial fibrillation, peripheral artery disease, hypertension, and type 2 diabetes mellitus presented to the hospital after his ICD fired multiple times. Device interrogation revealed appropriate shock times for likely atrial tachycardia-induced VT. Electrocardiogram on arrival showed atrial tachycardia with right bundle branch block. He was started on amiodarone and lidocaine drips and piperacillin-tazobactam for an incidental finding of superficial phlebitis. He continued to have several runs of VT with ICD discharges throughout the evening despite the drips, so the patient was intubated and sedated with fentanyl and propofol to decrease sympathetic drive. Patient was stable without further ventricular tachycardia and was extubated the next day. Blood cultures grew methicillin-susceptible Staphylococcus aureus (MSSA). Transesophageal echocardiogram showed echogenic structure attached to the right ventricular lead, which was subsequently extracted. He was discharged with wearable defibrillator and intravenous nafcillin with plans to reimplant the ICD after resolution of bacteremia.

In patients with an ICD, the most widely accepted definition of electrical storm is three or more appropriate therapies for ventricular tachyarrhythmias, including antitachycardia pacing, within 24 hours. Potential triggers can be new or worsened heart failure, changes in antiarrhythmic medication, context with other illness, psychological stress, diarrhea, and hypokalemia. In the hemodynamically stable patient, electrical storm is treated with IV anti-arrhythmics therapy. Our patient’s VT storm was likely caused by endocarditis and MSSA bacteremia and was refractory to initial therapies. While catheter ablation of ventricular tachyarrhythmias is an effective therapy in persistent storm, general anesthesia was chosen in this case due to its speed and availability as well as the severity of storm being experienced by an alert patient. Sympathetic activation has been implicated in pathogenesis of ventricular arrythmias and evidence has shown that sympathetic blockade may also have a beneficial effect. Propofol, a general anesthetic agent, suppresses sympathetic stimulation without prolonging the QT interval can be effective on electrical storm treatment.

VT storm is a serious, traumatic experience for a patient. This patient’s storm was likely brought on by an otherwise mild case of MSSA bacteremia, which reinforces the importance of carefully managing patients with ICDs

#72  The diversity of presentations in hydralazine-induced anti-neutrophil cytoplasmic antibody associated vasculitis

E Embry*

I Soliman

E Lam

J Russe

P Anand

Nassau University Medical Center, East Meadow, NY

Case Report

Hydralazine-induced anti-neutrophil cytoplasmic antibody (ANCA) associated vasculitis (HAAV) is a rare but rapidly progressive disease. HAAV is associated with renal involvement, antibodies to double-stranded DNA (dsDNA) and myeloperoxidase-ANCA (MPO-ANCA). Diagnosis is challenging due to nonspecific and variable symptoms. We report three cases of HAAV with diverse presentations.

CASE 1: 53-year-old African-American female with hypertension (HTN) on hydralazine for 5 years presented with dizziness and red-tinged urine. Lab tests revealed acute kidney injury (AKI), anemia, hematuria, nephrotic-range proteinuria, and elevated inflammatory markers. Glomerulonephritis workup revealed elevated antinuclear (ANA), antihistone, and MPO-ANCA antibodies. Renal biopsy revealed crescentic pauci-immune glomerulonephritis consistent with drug-induced vasculitis. Treatment included cessation of hydralazine, initiation of pulse steroids, hemodialysis, plasma exchange and immunosuppressive therapy. Patient’s was discharged after symptomatic improvement.

CASE 2: 59-year-old Filipino male with HTN on hydralazine for 10 years and chronic kidney disease (CKD) presented to the hospital with hematuria and symptomatic anemia. Urinalysis revealed hematuria and proteinuria. At the hospital, he developed new hemoptysis and diffuse purpuric rash. Immunological test was positive for MPO-ANCA. Kidney biopsy was consistent with ANCA glomerulonephritis. CT thorax revealed ground glass opacity and diffuse alveolar hemorrhage. Patient was admitted to the ICU and started on immunosuppressive therapy with plasmapheresis. He succumbed to a massive pulmonary hemorrhage.

CASE 3: 46-year-old African-American male presented with abdominal pain, fever, and dark urine. Home meds were hydrochlorothiazide, aldactone, and hydralazine. In the hospital, he developed progressive dyspnea and AKI. Positive labs included ANA, complement, PR3-ANCA and lupus anticoagulant, but negative for dsDNA and MPO-ANCA. Chest imaging showed bibasilar pleural effusion with atelectasis. Renal biopsy revealed pauci-immune diffuse necrotizing and crescentic glomerulonephritis, suggesting drug-induced etiology. He was treated with corticosteroids and immunosuppressive therapy with improvement. The patient continued to have good functional capacity with stable CKD Stage 3B disease over the next five years.

Discussion

We present three cases of HAAV with markedly diverse presentations, but the common findings of AKI with hematuria and/or proteinuria, pulmonary infiltrates, and elevated inflammatory markers, prompted for further immunological and renal biopsy workup. All labs, images, biopsy findings, and history of hydralazine use, hint at the diagnosis of HAAV. Hydralazine was discontinued for all patients, followed by corticosteroid and immunosuppressive therapy. Many recovered patients exhibit irreversible kidney damage.

#73  A case report of restless leg syndrome worsened after covid-19 vaccine

M Abdelnabi

N Eshak*

J Benjanuwattra

BL Mora

M Elmassry

K Nugent

Texas Tech University Health Sciences Center, Lubbock, TX

Case Report

Restless legs syndrome (RLS) is a poorly understood underdiagnosed neurological, sensorimotor disorder. RLS arises from central nervous system dysfunction leading to both sensory and motor symptoms. Limited cases of COVID-19 vaccines related neurological sequelae, such as Guillain-Barré syndrome (GBS), have been reported.

Case presentation

A 77-year-old male patient with a past medical history of well-controlled hypertension, diabetes mellitus, hypothyroidism, coronary artery disease status post percutaneous coronary intervention, obstructive sleep apnea on CPAP at night, and restless leg syndrome diagnosed 20 years ago, presented complaining of a 3-month history of worsening of his restless leg symptoms although being compliant with his medications 2 weeks after his 2nd dose of Moderna vaccine. He stated that the frequency and severity of his symptoms had increased from 3–4 times a week lasting for minutes to a daily basis lasting for hours at night, had improved partially with exercise, and affected his sleep hygiene and daily morning activities. He requested several refills of his previously prescribed ropinirole. A comprehensive evaluation, including clinical examination, laboratory workup, brain computed tomography, and polysomnography was unremarkable. He was commenced on pramipexole 0.5 mg daily and instructed to follow up in the clinic in 3 months and call back with no improvement or worsening of his symptoms.

Conclusion

This case fulfilled the four essential features of RLS, urge to move, worsening with rest, improvement with exercise, and worsening in the evening. To date, no case of RLS associated with COVID-19 vaccines has been previously reported. Although COVID-19 vaccines are relatively safe, long-term complications should be monitored closely.

#74  Glycosuria induced hypernatremia secondary to covid-19

A Evans*

P Southerland

K Nugent

Texas Tech University Health Sciences Center, Lubbock, TX

Case Report

The purpose of this study is to describe a case study of possible glycosuria-induced hypernatremia in a patient hospitalized with COVID-19 acute respiratory distress syndrome.

Methods Used

Case study and literature review

Summary of Results

A 55-year-old man with a past medical history of non-insulin-dependent type II diabetes and hypertension developed hypernatremia, glycosuria, and acute kidney injury in the setting of COVID-19 pneumonia after resolution of diabetic ketoacidosis. The patient was initially admitted with a positive SARS-COVID-19 screening, a creatinine of 1.1 mg/dL (0.5–1.2) with glycosuria, and sodium of 137 mmol/L (136–145). Seventeen days into his hospital admission for severe acute respiratory distress syndrome, he developed hypernatremia (147 mmol/L). Over the subsequent twenty-two days, the patient continued to have hypernatremia up to 153 mmol/L refractory to treatment. In addition, the patient had persistent glycosuria and an elevated creatinine of 2.3 mg/dL (greater than thirty percent above his baseline). His total fluid balance was +1444 mL during this phase of hospitalization. The patient’s electrolyte derangements concomitant with his worsening renal function suggests possible Fanconi syndrome. We hypothesize this is secondary to COVID-19.

Conclusion

COVID-19 has been shown to be associated with renal dysfunction, including acute tubular injury, such as membranous nephropathy and Fanconi syndrome. Experimental data have suggested that COVID-19 can infect renal proximal tubular cells via the Angiotensin Converting Enzyme 2 with subsequent development of incomplete Fanconi syndrome preceding acute kidney injury. Studies have also shown that glycosuria, proteinuria, pyuria, and hematuria may occur with COVID-19 regardless of comorbidities. We concluded our patient developed refractory hypernatremia secondary to glycosuria induced incomplete Fanconi syndrome due to COVID-19.

#75  Combined central retinal artery and vein occlusion in methicillin-resistant staphylococcus aureus sepsis – a case report

P Kositangool1*

DN Damani2

AM Armato1

DM Salazar1

F Dihowm2

1Texas Tech University Health Sciences Center El Paso Paul L Foster School of Medicine, El Paso, TX

2Texas Tech University Health Sciences Center El Paso, El Paso, TX

Case

Community associated Methicillin Resistant Staphylococcus Aureus (CA-MRSA) has been increasing in incidence leading to challenges with treatment, complications and adding to the morbidity, mortality and healthcare costs. This case reports a rare complication of MRSA orbital cellulitis and sepsis resulting in unilateral blindness due to combined central retinal vein and artery occlusion (CCRAVO).

Case Report

A 51-year-old male with a history of intravenous drug use presented with a 4-day history of abscess over the right knee; swelling of the nose, upper lip, bilateral periorbital region with no visual loss; purulent nasal discharge and necrotizing fasciitis of right arm. Examination of both eyes revealed chemosis, conjunctival injection, edema and erythema. Extra ocular movements were limited–right more than left with an intact visual field. Fundoscopic exam was deferred at this time due to intense pain. CT scan demonstrated swelling of the face and periorbital region but no fractures or abscess. The patient had blood culture positive for MRSA and was started on Vancomycin. He underwent incision-drainage and debridement of the bilateral periorbital regions, upper lip, nose, right upper and lower extremity; lateral canthotomy and catholysis. The patient developed septic shock requiring intubation and pressor support for a brief period of time. On day 3 of admission, the patient had a gastrointestinal bleed and was managed by placing 4 clips on the gastric ulcers. After ex-tubation, a complete eye exam revealed perception of light with complete scotoma, relative afferent pupillary defect, pain on abduction in the right eye and an acuity of 20/60 with normal pupil and eye movements in the left eye. The intraocular pressure was 15 and 19 mmHg. Indirect ophthalmoscopy showed blurred margins with retinal whitening, attenuated arteries, cart wheel appearance, focal arterial sheathing, aneurysms, flame shaped hemorrhages, roth spots and cherry red spot whereas the left eye showed mild A-V nicking and vein tortuosity, fundus exam findings are characteristic of CCRAVO. Labs: increased platelet count with normal function consistent with reactive thrombocytosis. Hypercoagulability workup showed normal PT/APTT/INR and was negative for RF, ANA, Lupus, anticardiolipin, anti-dsDNA, protein C and S, protein C resistance, factor V leiden, antithrombin III, prothrombin mutation, hyperhomocysteinemia, and leukocyte alkaline phosphatase deficiency. Infectious panel was negative for RPR, HIV and hepatitis. Protein immunoelectrophoresis was normal. Bilateral carotid duplex was normal.

Conclusion

CCRAVO are seen with vasculitis and thromboembolic disorders, and it is rare in adults. In the absence of risk factors, the CCRAVO can be attributed to MRSA sepsis-induced hypercoagulopathy. Early detection and treatment are essential to prevent debilitating long-term complications like blindness.

#76  Follow-up characteristics of hospitalized COVID-19 patients: a single-center retrospective chart review

D Landis*

D Le

C DeWare

C Conde

D Payne

Texas Tech University Health Sciences Center School of Medicine, Lubbock, TX

Purpose of Study

As of October of 2021, there have been over 43 million cases of COVID-19 and nearly 700,000 deaths in the U.S. alone. Many of those who have survived COVID-19 have been left with long-lasting symptoms such as fatigue, dyspnea, loss of smell or taste, and depression or anxiety. Mechanisms for these lasting symptoms are not fully known, so monitoring of patients after their infection has been cleared is important to determine both the rates and the mechanisms of the aptly named ‘Long COVID.’ The purpose of our study was to monitor the status of COVID-19 patients that were previously hospitalized at the UMC Medical Center in Lubbock, Texas after discharge.

Methods Used

We conducted a comprehensive chart review of 128 patients that were hospitalized for COVID-19 at any time between April 1, 2020 and April 1, 2021, and reviewed follow-up data for these patients after discharge.

Summary of Results

In our cohort of patients, 46% (n = 59) were men and 54% were women (n = 69) with an average age of 59.7 ± 14.8 years old. 60.9% of patients identified their race as Hispanic or of Latino origin (n = 78), with the next largest group being Caucasian at 22.65% (n = 29). The average number of days until post-hospitalization follow-up was 36 ± 38 days. Notable findings included a 50% rate of telehealth follow-up (n = 64), a 65.6% (n = 84) rate of diabetes, and a 73.4% (n = 94) rate of hypertension. 26.56% (n = 34) of patients reported respiratory symptoms at their follow-up appointments, 18.75% (n = 24) of patients reported constitutional symptoms, 9.37% (n = 12) of patients reported GI symptoms, and 19.5% (n = 25) of patients reported other symptoms such as paresthesia, lower extremity edema, or psychological symptoms. After discharge, 54 patients received follow-up X-rays. 75.9% (41/54) were found to still have abnormal findings consistent with COVID-19 imaging characteristics. During their follow-up appointment, 57 patients had a D-dimer lab value, 56 patients had a Ferretin value, and 59 patients had a Troponin T HS value. Of the follow-up patients with labs, 77.2% (44/57) had an elevated D-Dimer value, 78.6% (44/56) had an elevated Ferretin value, and 35.6% (21/59) had an elevated Troponin T HS value.

Conclusions

Our findings are consistent with pre-existing literature concerning higher rates of diabetes and hypertension in hospitalized COVID-19 patients, as well as reports of lasting symptoms after viral clearance. The CXR findings indicating lasting lung damage have some explanatory power in regard to respiratory symptoms at follow-up. Furthermore, our findings of elevated lab values in patients who received lab testing after discharge align with the emerging literature on D-dimer and Long COVID. Our lab findings should be considered limited due to pre-existing conditions or unrelated hospital visits that would predispose some these lab findings to be elevated. More research should be conducted to further elucidate mechanisms and treatment options for patients with Long COVID.

#77  A curious case of diarrhea, abdominal pain, and recurrent intestinal infection

AQ Nguyen*

K Le

S Subbiah

B Boulmay

LSU Health New Orleans, New Orleans, LA

Case Report

Enteropathy-associated T-cell lymphoma (EATL) is a rare and aggressive type of non-Hodgkin lymphoma that affects the small intestine. Its median age at diagnosis is 60 years old. It is strongly associated with Celiac Disease (CD). However, without previously known history of CD, diagnosis is usually delayed. Here, we present a case of a 57-year-old healthy man with acute onset of abdominal pain and diarrhea, found to have recurrent Clostridium difficile colitis. His symptoms initially improved with treatment of colitis, however recurred. He eventually developed enteroenteric fistula requiring resection and EATL was diagnosed on pathology. Prompt diagnosis and treatment of EATL with multidrug chemotherapy is crucial to achieve optimal outcome.

#78  Normolipemic xanthelasma palpebrum in a case of acute limb ischemia

A Nguyen*

G Dharmarpandi

A Dweik

J Dharmarpandi

T Naguib

Texas Tech University Health Sciences Center School of Medicine Amarillo, Amarillo, TX

Case Report

To discuss a case of acute limb ischemia in a patient with xanthelasmas and normal lipid panel

Methods Used

Literature Review and Case Report

Summary of Results

43-year-old Caucasian male with 20 pack year history of tobacco use presented to with left leg pain associated with numbness, swelling, and poikilothermia. He had no history of obesity, diabetes, hypertension, coronary artery disease, or thyroid disease. He denied medications or supplements usage but had poor diet and limited physical activity. He reported prevalent heart disease in his family but was unable to be more specific. He had a six-month history of cholelithiasis and underwent emergent cholecystectomy one month prior.

On physical exam of the patient there were noticeable xanthelasmas to both eyelids, which patient he stated first appeared three years ago. Fasting lipid panel was within normal range (cholesterol 162, triglyceride 120, HDL 41, LDL 97).

CT angiogram of the leg showed a complete occlusion of the left external iliac artery and patient was taken in by vascular surgery for thrombectomy, stent placement, and fasciotomy. His hospital course was complicated by partial occlusion of the stent, requiring repeat angioplasty. Repeat lipid panels were obtained four days after initial testing to rule out possible lab error and patient’s results were largely unchanged. Apolipoprotein B was also collected and yielded a result of 90 mg/dL (normal levels in adults are less than 100 mg/dL). Patient continuously improved during the 6 days he was in the hospital and was cleared for discharge home on clopidogrel and rivaroxaban with wound care and rehabilitation services.

Conclusions

The presence of xanthelasma palpebrum, recent cholelithiasis, acute limb ischemia, and significant family history suggest underlying dyslipidemia, which is not reflected in laboratory results. Apolipoprotein B, which has a strong correlation with atherosclerotic disease, was also collected and yielded results below the threshold for concern. The patient was not taking any medications, including statins, which would have suppressed lipid values. However, a documented side effect of cholecystectomy is the lowering of lipid levels, which is possible in this patient. Notably, normolipemic xanthelasma palpebrum is not an uncommon phenomenon. Studies have found that patients with xanthelasmas are at higher risk of atherosclerosis independent of lipid concentrations. While lipid levels were within normal range, patients with visible xanthelasmas had an increased intima-media thickness of the carotid compared to patients without xanthelasmas. Our patient stated that his eyelid lesions had been present for three years, and this was likely a harbinger of his proclivity for atherosclerotic disease which resulted in his eventual limb ischemia. This vignette describes an unusual case of a patient who presented with a strong clinical picture of dyslipidemia yet had normal lipid panels on laboratory testing.

#79  A presentation of profound (8200 ml) urinary retention

T Nguyen*

M Johnson

E Clement

S Hardy

LS Engel

LSU Health New Orleans, New Orleans, LA

Case Report

Acute urinary retention (AUR) often presents in men as the inability to urinate coupled with acute lower abdominal/suprapubic pain. AUR in men is most commonly due to outflow obstruction secondary to BPH, but other etiologies including neurologic dysfunction, medication adverse effects, and infection can also be attributed to this. In the case of chronic urinary retention (CUR), however, patients often present without any pain symptoms. Here, we describe the presentation of painless urinary retention of a significant volume.

Case

A 60-year-old man with past medical history of hypertension and an abdominal lipoma presented with a 1.5-month history of nausea, vomiting, and fatigue associated with oral intake intolerance and a reported 10-lb weight loss. The patient had no abdominal pain or any urinary complaints including no decreased urination or reported incontinence. Upon presentation, he was afebrile and hemodynamically stable. His exam was very concerning for a firm, non-tender, and grossly distended abdomen with protuberance that stretched from his xiphoid process to his pubic symphysis. Review of his medical record showed CT imaging of his abdomen/pelvis from four years prior notable for moderate left hydronephrosis/hydroureter secondary to a distended urinary bladder and markedly enlarged prostate causing bladder outlet obstruction. Initial bloodwork was notable for elevated renal indices (BUN 136 mg/dL and creatinine 10.3 mg/dL); his urine studies did not show evidence for infection. CT imaging performed this presentation was concerning for a large intraabdominal mass (measuring approximately 31 cm in diameter) with bilateral hydronephrosis and hydroureter. A foley was placed and initially 1L of urine was drained before the foley was clamped. Urology was consulted and cleared the patient for complete bladder emptying: in total, 8.2 liters of urine was drained. He was discharged approximately one week thereafter following resolution of his post-obstructive diuresis with improvement in his post-renal acute kidney injury due to his obstructive uropathy. A foley was left in place upon discharge for close outpatient follow-up with Urology.

#80  Prevalence and factors associated with advance health directives in frail older inpatients

JJ O’Leary*

The University of Queensland Ochsner Clinical School New Orleans, New Orleans, LA

Purpose of Study

Advance health directives (AHDs) can be used to explore and document patient preferences for treatment and are therefore an important aspect of care planning. In this study, we aimed to investigate the prevalence and associations of AHDs in a large cohort of older inpatients over a decade of evolving advance care planning strategies.

Methods Used

This retrospective study included 6449 patients, aged ≥ 65 years referred for specialist geriatric consultation between 2007 and 2018 across 27 centres in Queensland, Australia. Sociodemographic and health data were analysed from the Comprehensive electronic Geriatric Assessment online database. The interRAI-Acute Care Comprehensive Geriatric Assessment tool was used to calculate a frailty index (FI), based on the model of accumulated deficits.

Summary of Results

Patients’ mean (SD) age was 80.7 (7.7) years, 3489 (54.1%) were female and mean (SD) FI was 0.46 (0.15). An AHD was present in 1032 (16.0%) patients. Prevalence of AHD increased over time, from 7.6% in 2008 to 35.4% in 2017. In logistic regression models, higher frailty (OR:1.34 [1.27–1.40]), older age (OR:1.04 [1.03–1.05]), living in an institution (OR:1.33 [1.01–1.73]), and recent hospitalisation (OR:1.42 [1.23–1.62]) were significantly associated with higher prevalence of AHDs.

Conclusions

The presence of AHDs is associated with sociodemographic factors, as well as higher frailty levels. Much of the focus on improving advance care planning has been on increasing AHDs. Prevalence of AHDs among inpatients has increased over the past decade but remains modest. Clinicians have a responsibility to promote AHDs to ensure patients’ own goals of care are documented and respected.

#81  Penile calciphylaxis

C Rathore*

A Loghmani

J Larrazolo

LS Engel

S Walvekar

LSU Health New Orleans, New Orleans, LA

Case Report

Calciphylaxis, or calcific uremic arteriolopathy, is a rare skin disorder that presents as intensely painful areas of skin ischemia and necrosis and carries a high morbidity and mortality rate. The skin lesions of calciphylaxis normally involve adipose-rich areas such as abdomen and thighs, though in much rarer cases have involved the penis. Calciphylaxis is most commonly associated with end-stage renal disease (ESRD). Other associated risk factors include hyperphosphatemia, hypercoagulable states, long-term dialysis use, and warfarin use.

Case

A 50-year-old man with past medical history of ESRD on HD and mechanical mitral valve replacement on warfarin presented with one month of progressive penile pain and swelling, which worsened to an ulcerated lesion at the glans penis and phimosis. CT scan of the pelvis demonstrated marked diffuse atherosclerotic disease with wall calcification of all major vessels, as well as questionable mild soft tissue edema and skin thickening of the penis. Infectious workup was negative for chlamydia/gonorrhea, HIV, HSV, and syphilis. The patient was evaluated by urology and taken to the operating room for circumcision and penile biopsy given concern for malignancy. Frozen section for cancer was negative. Final surgical pathology results revealed necrotic ulcer with thrombosis and intravascular calcification at the ulcer base. Dermatology reviewed the biopsy findings and felt this was most consistent with calciphylaxis. The treatment plan was coordinated with nephrology to begin sodium thiosulfate with dialysis. The patient continued to complain of intense pain despite scheduled and prn oral and IV pain medications. Palliative care and wound care were consulted for symptom management. The patient was offered penectomy given refractory pain despite medication adjustment, however he declined. And although it is recommended to discontinue warfarin in the setting of calciphylaxis as it can worsen skin necrosis, the decision was made to continue warfarin given presence of his mechanical valve and otherwise limited options due to ESRD.

Discussion

Penile calciphylaxis is particularly rare because of the rich vascular network in the area. Microvascular calcification leads to a cascade of vascular endothelial injury and consequent narrowing and thrombosis of the vessels, which ultimately leads to tissue necrosis from reduced blood flow. There is no approved treatment for calciphylaxis. A collaborative approach involving nephrology, dermatology, wound care, and palliative care is necessary to try and reduce risk factors..

#82  Cryptogenic organizing pneumonia during the COVID-19 pandemic: a missed diagnosis

DM Salazar1*

DN Damani2

P Kositangool1

AM Armato1

F Dihowm2

1Texas Tech University Health Sciences Center El Paso Paul L Foster School of Medicine, El Paso, TX

2Texas Tech University Health Sciences Center El Paso, El Paso, TX

Case Report

Purpose of Study

Interstitial lung disease (ILD) is a group of pulmonary disorders that cause varying degrees of inflammation and fibrosis of pulmonary architecture. The diagnosis requires good clinical history, examination, appropriate workup, and a high degree of suspicion. This case report draws attention toward a unique case of cryptogenic organizing pneumonia after mold exposure.

Methods Used

Not applicable.

Summary of Results

A 36-year-old nonsmoker male with no comorbidities presented with worsening shortness of breath after cleaning a walk-in cooler room contaminated with mold. He was seen at multiple facilities for presumed diagnosis of COVID-19 despite being vaccinated and 4 negative COVID-19 results. He was discharged with 2 liters of supplemental home oxygen and a 7-day course of Levofloxacin, with no resolution of symptoms. The patient presented to our hospital 2 months after initial onset of symptoms. On examination, the patient had bronchial breath sounds with fine crepitations, egophony, and increased vocal resonance. Chest x-ray revealed bilateral airspace consolidation with scattered ground-glass opacities in the apices. Computed Tomography (CT) of the thorax showed peripheral upper lobe ground-glass opacities with interstitial thickening in a ‘crazy-paving’ pattern. A chest CT angiogram showed patchy ground-glass pulmonary infiltrates with peripheral predominance consistent with severe COVID pneumonia. PCR for SARS-CoV-2 was negative. The patient’s oxygen demand increased progressively from 4L on nasal cannula to 40L on high flow nasal cannula to maintain an oxygen saturation of 90%. Labs showed normal leukocyte count, ESR, ALT, and AST with a mildly elevated CRP. Workup for infectious etiology was negative for S. pneumoniae, legionella, coccidioides, HIV, hepatitis panel, QuantiFERON gold and blood culture. Autoimmune workup was negative for ANA, RF, CCP, ANCA, anti-centromere Antibody, anti-ds DNA. The patient underwent a bronchoalveolar lavage with culture negative for acid fast bacilli, fungi, and P. jirovecii. Bronchoscopic biopsy was subsequently performed and revealed lung parenchyma with foci of mild chronic inflammation with focal fibroblastic proliferation and fibrosis, suggestive of an organizing pneumonia. The patient was started on steroids 1 mg/kg resulting in significant clinical improvement requiring only 3L on nasal cannula on day 5 of treatment. He was then discharged with high dose steroid therapy for 3 months.

Conclusions

The prognosis and treatment of ILD depends on accurate diagnosis and its subtype. Hence appropriate workup is essential to guide therapy. In the setting of the current pandemic, relatively uncommon causes of ILD like cryptogenic organizing pneumonia may go undiagnosed due to the unconscious bias among health care providers resulting in delayed treatment. This report highlights the importance of considering alternative diagnosis when a disease does not follow an expected course.

#83  Group b streptococcal bacteremia possibly due to genitourinary invasion from sexual encounter: a case study

A Falco*

V Vemulapalli

T Naguib

Texas Tech University Health Sciences Center School of Medicine, Lubbock, TX

Case Report

Case report to elucidate the importance of Group B Streptococcal infection acquired in a middle-aged woman possibly due to genitourinary invasion from a sexual encounter.

Case presentation

A 63-year-old hispanic female was admitted with fever, shortness of breath, epigastric and chest pain that started earlier in the day. She presents with a history of cirrhosis, pancytopenia, obesity (BMI 38 kg/m2), esophageal varices, and colon cancer that was removed by chemotherapy. Chest pain was unrelieved by nitroglycerin. She mentioned a failed trial of sexual intercourse with her husband one week before presenting to the hospital. Patient stated she felt ‘ripping’ despite using lubricants and had been sexually inactive for at least 5 months prior. On physical exam she does not present with notable vulvar or vaginal discharge or bleeding. Vitals on admission are significant for a fever of 101.3 F. Heart Rate, respiratory rate, and SpO2 were normal. EKG showed normal sinus rhythm. CTA chest with contrast and echo reported no acute abnormalities. CT of abdomen and pelvis with contrast reported fatty cirrhotic liver without lesions, splenomegaly, and mildly thickened urinary bladder wall. Labs show mildly elevated troponin, stable over 3 measurements. CBC reported low hemoglobin, platelet, and WBC counts. Ceftriaxone 1 g daily was started on admission. Blood cultures came back positive for Group B Streptococcal bacteremia and coagulase-negative staphylococcus. In addition Enterobacter cloacae was found in urine culture. Pancytopenia was possibly caused by both bacteria and liver failure, and her cirrhosis etiology was unclear. IV ceftriaxone was changed to IV vancomycin on day 3. Symptoms gradually improved and she was discharged after day 10 with plans for outpatient IV vancomycin for a total of 2 weeks.

Discussion

Due to the high disease burden of invasive Group B Streptococcus in non-pregnant women and men and associated mortality rate of 25%, invasive GBS has been a topic of research over the past 50 years. GBS bacteremia in non-pregnant adults have been reported as hospital-acquired or due to an unidentified source. coagulase-negative staphylococcus blood growth of unclear significance however could have a similar portal of entry. Liver cirrhosis has been reported to significantly increase the risk of GBS bacteremia as seen in this patient due to decreased lymphocyte production. This patient’s history of a sexual encounter, impaired immunity due to liver cirrhosis, and symptoms five days before admission suggest a possible hematologic invasion of bacteria from the genitourinary tract. This may shed light on many cases of unidentified sources since sexual history is not always forthcoming.

Conclusion

We conclude that although Group B Streptococcus bacteremia due to genitourinary invasion is rare, the genitourinary system must be always considered as a possible source. Sexual history is imperative in these cases.

Cardiovascular

Joint plenary poster session and reception

4:30 PM

Thursday, February 10, 2022
#84  Hypertensive emergency reveals the diagnosis of cardiac amyloidosis

M Abohelwa*

E Elgwairi

N Mittal

K Parmar

J Abdelmalek

M Botros

P Khalil

P Sethi

Texas Tech University Health Sciences Center, Lubbock, TX

Case Report

The clinical manifestations of cardiac amyloidosis are diverse and vary based on the organs that are involved. Cardiac amyloidosis usually presents with symptoms and signs similar to congestive heart failures such as lower extremity edema, elevated jugular venous pressure, hepatic congestion, ascites, and dyspnea. These are usually caused by restrictive cardiomyopathy with predominantly right ventricular failure.

Case presentation

A 37-year-old male patient has a history of hypertension, DM type 2, ESRD on hemodialysis three times weekly, heart failure with preserved EF, and severe concentric left ventricular hypertrophy. He presented for evaluation of a 3-day history of gradually worsening generalized fatigue with dyspnea on exertion. The patient was hypertensive at 207/95, tachypneic at 32, and saturating 72% on room air in the ED. He was placed on 4L NC with improvement to 99% saturation and given Labetalol 10 mg IV with BP improvement to 186/83. At that time, he denied chest pain, dyspnea, or abdominal pain. On physical exam, he had bilateral basal crackles and + 1 lower extremity edema. The patient was admitted to the hospital and started on hemodialysis for fluid removal and blood pressure control. The medical team thought that the infiltrative cardiomyopathy/disease might be possible with his severe LVH and restrictive diastology. The presence of ESKD before DM diagnosis raised the possibility of possible amyloidosis, which prompted the medical team to order NM PYP scan that came back positive and suggestive of myocardial transthyretin amyloidosis ATTR. Beta-2 globulin was ordered, and it came back high relative to beta-1 globulins. The presence of a monoclonal protein could not be ruled out from the lab interpretation.

Discussion

The diagnosis of cardiac amyloidosis may be challenging in a low-resource setting. If there’s suspicion for cardiac amyloidosis, transthoracic echocardiography is the initial cardiac imaging to be done with some findings suggestive of cardiac amyloidosis like relative apical sparing of longitudinal strain. In addition, cardiovascular magnetic resonance (CMR) imaging is recommended for patients with unexplained LVH, aortic stenosis with features associated with cardiac amyloidosis, or HF with symptoms or signs typical of amyloidosis. Features of cardiac amyloidosis on CMR are typical late gadolinium enhancement (LGE) patterns. If the features are consistent with amyloidosis, then tests for monoclonal proteins should be ordered: serum kappa/lambda free light chain ratio analysis, serum protein immunofixation, and urine protein immunofixation. If monoclonal proteins are not detected, then bone tracer cardiac scintigraphy should be performed with 99mtechnetium pyrophosphate (99mTc-pyrophosphate [PYP]).

#85  Hispanics puerto rico (U.S.A. Island) with a higher LDL lipoprotein levels shows a lower percent of coronary artery disease than the U.S.A

PI Altieri1,2*

JE Muñoz1

N Escobales1

LE Barreto1

1University of Puerto Rico, Medical Sciences Campus, San Juan, Puerto Rico

2Cardiovascular Center of Puerto Rico and the Caribbean, San Juan, Puerto Rico

Purpose of Study

Coronary artery disease (C.A.D.) is one of the highest causes of death in the world. The purpose of this report is to demonstrate a lower incidence of C.A.D. (20–30%) in Puerto Rico (P.R.) (U.S.A. Island) compared with the mainland, U.S.A.

Methods Used

The study of population was 1000 consecutive Puertorrican patients and the U.S.A. health statistics reported by the Department of Health and the U.S.A. Department of Health. They were compared statistically, and a significant difference was found. Investigators in P.R. has reported the genetic admixture of the Puertorrican population (CYP2C9, VXORCI, VKORCI-1639> A Allele in sector 1), which we call ‘protective genes’.

Summary of Results

A report showed a genetic admixture of 3 genes which we named protective against severe-aggressive C.A.D. in the P.R. population when compared with the U.S.A. population. This admixture has a diffuse distribution in our population, reducing the inflammatory effect of several factors. These are statistics of the most of European population. C.A.D. is an inflammatory process of the coronaries which includes the endothelial cells, monocytes and macrophages. This inflammation is induced by angiotensin II, oxidize LDL monocyte-macrophage axis, inducing plaque formation, especially in the coronaries.

Conclusions

Probably, this gene admixture protects these cells against an aggressive inflammatory process, which will reduce the expression of C.A.D. Again, cultures ethnicity and evolution are all important part in this reduction of C.A.D. in our population. We will compare our data with the U.S.A. and European data.

#86  Coronavirus at the heart center of Puerto Rico incidence-death: the role of genetics

CM Díaz1*

LE Barreto1

PI Altieri1,2

HL Banchs1,2

1University of Puerto Rico, Medical Sciences Campus, San Juan, Puerto Rico

2Cardiovascular Center of Puerto Rico and the Caribbean, San Juan, Puerto Rico

Purpose of Study

Coronavirus disease, caused by a beta-coronavirus, mostly affects the respiratory system. Since it is a novel disease, very little is known about the connection between heart involvement and COVID-19. This study will strengthen the current literature and demonstrate to what extent the coronavirus affects the cardiac system. This is a leap forward towards understanding how the heart responds to the virus; based on a cross sectional study of a Hispanic population.

Methods Used

In total, 50 patient records with positive PCR for SARS-CoV-2 were collected from the Heart Center Hospital. These 50 patients (P.) were admitted after coming into the emergency room. We studied age, sex, race, cardiac involvement, medications, EKGs, Chest Plates, X-rays, CT-Scans, and previous and current health problems. Within the medication section, our prime focus was to observe whether these P. were currently taking or took Losartan in the past, because this drug reduces the penetration intracellularly of the virus. For the chronically ill P., we analyzed underlying diseases, intubation and their role in complications or even death.

Summary of Results

All of the 50 P. were from Puerto Rico (P.R.), a Hispanic population. None of the P. was taking Losartan. According to the records 96% had severe health problems previously to being contaminated by the virus. Some had atherosclerosis, while others had cardiomyopathy or diabetes mellitus, not related to an acute viral infection. Ten percent of these P. died; however, their cause of death was not a result of a clear correlation between COVID-19 and other comorbidities. These P. were chronically ill and probably the virus further complicated their medical condition.

Conclusions

In P.R., and possibly other Hispanic countries, there are genes which we call ‘protective genes’ (P.G.) that control the incidence and degree of heart disease, especially atherosclerotic heart transmitted by evolution. We believe P.G. are crucial in reducing the risk of contracting severe complications by the COVID-19 virus. In addition, since none of the 50 P. was not taking Losartan, we also think this is a factor that will increase the incidence of getting the virus intracellularly, increasing the incidence of death.

#87  Estimating risk of diabetic foot disease progression among spanish-speaking patients

A Giakas1

KG Holder2*

B Galvan2

M Torres1

1University of South Carolina System, Columbia, SC

2Texas Tech University System, Amarillo, TX

Purpose of Study

This study aims to implement a Spanish-language foot evaluation for assessing the prevalence of foot pathology among the Hogar Carlos María Ulloa’s (HCMU) patient population with type II diabetes mellitus and identify each patient’s risk of developing serious foot complications.

Methods Used

A Spanish-language evaluation form was developed to obtain demographic factors, assess the prevalence of diabetic foot disease, and estimate the risk of developing severe foot disease amongst all diabetic patients at HCMU, a state-sponsored nursing home in San Jose, Costa Rica. Foot evaluations were completed by the first author and three licensed HCMU physical therapists. Statistical Analysis Software (SAS) was used to assess the prevalence of foot disease among diabetic patients and estimate the risk of progression to severe foot disease, including foot ulcers and amputation.

Summary of Results

Of the 43 patients with type II diabetes mellitus assessed, 61.9% of patients experienced a diminished posterior tibial pulse, while pulse was absent in 16.67% of patients. 66.67% of patients presented with claw or hammer toe deformities, 54.76% experienced hallux valgus deformity, and 30.95% presented with pes cavus or pes equinus deformities. Strikingly, over half 54.76% of patients had developed peripheral neuropathy within their feet. Of the patients examined, 64.29% of patients had a moderate risk of progressing to more severe diabetic foot disease, including ulcers requiring amputation. Two of the patients examined had already developed diabetic foot ulcers, and an additional two patients had already undergone single-foot amputations. On average, the evaluation form took two minutes and fifty-three seconds to administer.

Conclusions

The American Diabetes Association (ADA) states that a comprehensive foot examination should be conducted annually to assess skin, neurologic, vascular, and biomechanical status, but foot examinations are documented in only 6% to 12% of HMO/private practice settings. Although the incidence of diabetes is increasing globally, diabetes and diabetic foot care play an especially large role in the healthcare management of patients in patients living in Latin American countries, where the frequency of diabetes is expected to increase by 38% over the next 10 years. A specific barrier to proper preventative management in these countries includes lower rates of diabetic foot examinations at primary care visits due to a dearth of Spanish-language forms to evaluate the prevalence and progression risk of foot pathologies commonly seen in patients with diabetes. This study implemented a Spanish-language evaluation form to record the results of physical examinations and estimate the risk of progression to more severe diabetic foot disease. This evaluation can be adopted in healthcare settings looking to improve screening efficiency and preventative care quality for their Spanish-speaking patients with diabetes.

#88  Diagnosis and robotic-assisted, minimally invasive, autologous pericardial patch repair of a sinus venosus atrial septal defect (ASD) with repair of partial anomalous pulmonary venous connection

LN Joseph*

J Barrios

S McElwee

University of Alabama at Birmingham, Birmingham, AL

Case Report

Sinus venosus ASDs are usually associated with one or more anomalous right sided pulmonary veins. Diagnosis by transthoracic echocardiogram (TTE) and confirmation with transesophageal echocardiogram (TEE) and right heart catherization can lead to a multidisciplinary approach for appropriate surgical correction.

Case

A 21-year-old male with no PMH presented to clinic for a routine physical to return to collegiate athletics post COVID-19 infection. His only complaint during this time was residual dyspnea (NYHA Class 1). An ECG was obtained and showed an incomplete right bundle branch block and TTE revealed an ASD with moderate RV dilation. Repeat TTE at our institution showed an interatrial shunt on injection of agitated saline via the right arm within three beats after injection. Subsequent right heart catheterization with shunt series revealed a step in oxygen saturation from 75% in the superior vena cava (SVC) to 88% in the right atrium. Additional imaging obtained with TEE confirmed a sinus venosus ASD. Cardiovascular surgery was engaged and further imaging with computed tomography angiography (CTA) of the chest confirmed a large superior sinus venosus ASD measuring 16 mm in diameter as well as partial anomalous right pulmonary venous drainage into the SVC.

The heart team decided on a minimally invasive robotic approach and performed an autologous pericardial patch repair of the ASD with redirection of the right and superior pulmonary veins into the left atrium. Intra-op TEE showed no residual shunt across the interatrial septum. The patient had an uncomplicated post-operative course and was discharged home on day 4.

Decision-Making

Sinus venosus ASDs and associated anomalous pulmonary veins are often missed on TTE. In our patient, TEE and CTA assisted in the detection of anomalous pulmonary venous connection. A multidisciplinary heart team approach helped determine and tailor the best option for surgical correction in our patient’s case.

Abstract #88 Figure 1

A) Transesophageal echocardiogram with red arrow depiciting superior sinus venosus atrial septal defect. Color doopler shows ASD with left to right shunt. LA (left atrium), RA (right atrium); B) Transesophageal echocardiogram with blue arrow showing anomalous right pulmonary vein with drainage into the superior vena cava. Red dotted line and arrow depict atrial septal defect measuring 16 mm in diameter. LA (left atrium), SVC (Superior vena cava)

Conclusion

Sinus venosus defects account for up to 10% of ASDs and can lead to pulmonary hypertension if left uncorrected. TTE remains the first imaging modality in assessing for ASDs, but TEE, RHC, and CTA can assist in comprehensive diagnosis and planning for procedural correction. Surgical closure in patients less than 25 years old without pulmonary hypertension is associated with low postoperative mortality, and a multidisciplinary approach can help ensure the most optimal method of surgical correction.

#89  Are direct oral anticoagulants going to replace the rat poison?

SM Kayali1*

S Roman2

A Asbeutah1

M Oberoi3

C Stadnick4

RN Khouzam1

1The University of Tennessee Health Science Center, Memphis, TN

2St Joseph’s University Medical Center, Paterson, NJ

3University of South Dakota, Vermillion, SD

4The University of Tennessee Health Science Center College of Medicine, Memphis, TN

Purpose of Study

This abstract aims to discuss the current guidelines and trials shaping the evolving landscape of direct oral anticoagulant (DOAC) and vitamin-K antagonist use for various cardiovascular diseases and patient groups.

Methods Used

We conducted a Medline search of ‘direct oral anticoagulants,’ ‘vitamin K antagonists,’ ‘left ventricular thrombus,’ ‘atrial fibrillation,’ and ’prosthetic heart valves’ to identify landmark trials published before June 22, 2021, for inclusion in this review. Trial bibliographies, important practice guidelines, and relevant reviews were examined to ensure the inclusion of relevant trials. The following section discusses the trials and emerging evidence for the use of either DOACs or VKAs in different patient groups requiring anticoagulation for cardiovascular disease.

Summary of Results

DOAC use has grown dramatically in recent years. Large, multicenter randomized controlled trials have affirmed the superiority of DOACs over VKAs for patients with nonvalvular atrial fibrillation (AF) and venous thromboembolism (VTE) when body-mass-index (BMI) and hepatorenal function are relatively normal. In patients with BMI extremes, renal or hepatic dysfunction, guidelines are driven by expert opinion, retrospective data, and small randomized trials, and thus, the efficacy and safety of DOACs in these patients are less clear. VKA therapy is the standard of care for patients with mechanical prosthetic valves and left ventricular (LV) thrombi, however, further investigation of DOAC therapy in patients with LV thrombi is under away. In patients with a bioprosthetic mitral valve and comorbid AF, the RIVER trial demonstrated that rivaroxaban was non-inferior to warfarin in preventing VTE.

Conclusions

While VKAs have traditionally reigned as the oral anticoagulant of choice for over half a century for many cardiovascular diseases, DOACs have been recently shown to possess a non-inferior antithrombotic benefit in addition to a reduced bleeding profile as compared to VKAs for patients with nonvalvular AF and VTE. However, these studies do not address the ideal anticoagulation strategy for underrepresented populations such as those with hepatic or renal impairment. Randomized controlled trials are needed to investigate these specific populations. Although recommendations for left ventricular thrombus treatment have traditionally supported VKAs, the first randomized controlled trial to compare VKAs with DOACs in patients with LV thrombi has revealed promising results in favor of DOACs. Lastly, whilst VKAs remain the preferred anticoagulants in patients with mechanical prosthetic valves, the RIVER trial has revealed the role of DOACs in treating patients with a bioprosthetic valve and concomitant atrial fibrillation. We anticipate that more trials will address this issue, and DOACs may eventually be all affordable, and perhaps replace VKAs or the rat poison, as a non-inferior alternative while avoiding its side effects and the need for periodic INR checks.

#90  Synthetic cannabinoids as a cause of coronary vasospasm mimicking myocardial infarction

B Mohanakrishnan1*

M Khan1

M Amin1

R Anand1

J Rahesh1

F Khan2

W Rasheed1

A Zakir3

D brabham1

1Texas Tech University Health Sciences Center, Amarillo, TX

2Khyber Medical University, Peshawar, Pakistan

3King Edward Medical University, Lahore, Pakistan

Case Report

Marijuana has long since been popular as an illicit substance in the United States & the rest of the world. MI is a particularly lethal sequela of marijuana use. We present a case of diffuse coronary vasospasm secondary to marijuana, mimicking myocardial infarction.

Case presentation

53-year-old male with a past medical history of hypertension, hyperlipidemia, and COPD woke up at 3 AM with severe shortness of breath unresponsive to albuterol inhalers. Three hours later, the patient reported severe chest pain and was found pulseless. EMS was called and reported the patient to be in ventricular fibrillation. CPR was initiated, and the patient was intubated. The patient was shocked a total of 5 times, 4 rounds of epinephrine and one round of amiodarone. Return of spontaneous circulation was achieved after 30 minutes. Labs were significant for creatinine 1.5 mg/dl, HCO3 13 mmol/L, and lactic acidosis with pH 7.04. Troponins and BNP were within the normal range. EKG showed no dynamic ST-segment changes on admission. Urine drug screen was positive for marijuana. Left Heart cath. showed severe spasms involving all 3 coronary vessels responsive to intra-coronary nitroglycerin, but otherwise no significant obstructive coronary disease. Six hours after LHC, the patient was found to have ST elevation in leads V3-V6 & wide QRS rhythm lasting for 20 minutes along with hypotension. At this point, the patient was intubated with good oxygen saturation and stable electrolytes. He was not on pressor support. EKG changes resolved with increasing the rate of nitroglycerin drip & hypotension responded to IV fluids. Troponin HS drawn at the time was found to be elevated at 197ng/L. Repeat LHC was not deemed necessary, but the patient was switched to diltiazem from carvedilol. However, there were no further notable events. The patient was discharged 8 days later & counseled to quit marijuana.

Discussion

Marijuana is one of the most widely abused substances in the US. When smoked, THC results in a rapid, dose-dependent tachycardia by 20–100%, an increase in blood pressure, and an increase in cardiac output by > 30%. MI is a rare complication from marijuana use. Our patient was unusual in that his MI symptoms were due to global vasospasm of the coronary arteries, which we theorize was contributed to by marijuana toxicity. It is important to note that hundreds of chemical variants are likely in circulation relative to the few that clinicians are capable of testing for. The workup is identical to the workup for any patient suspected of ACS. The diagnosis of substance-induced angina is a diagnosis of exclusion, with the particular substance identified through careful patient history, UDS, and the clinician’s awareness of current commonly abused substances.

Conclusion

In patients with low risk for cardiovascular events, particularly pediatric and young adult patients, presenting with symptoms of MI, substance-induced MI should be suspected

#91  Description of dexmedetomidine dosing in neonates and its impact on cardiovascular system

IE Sierra-Strum

MM Makoni*

The University of Oklahoma Health Sciences Center, Oklahoma City, OK

Purpose of Study

Dexmedetomidine (DEX) is gaining popularity as an off-label sedative and anxiolytic for neonates. DEX does not cause gastrointestinal or respiratory effects, however does have bradycardic and hypotensive effects. There is no evidence of cardiovascular patterns associated with these side effects. Currently, there is no evidence-based practice recommendation regarding DEX dosage in neonates.The objective of the study is to retrospectively evaluate the cardiovascular effects of DEX infusion in neonates admitted to the NICU.

Methods Used

Retrospective study of NICU patients with an order for DEX infusion between Jan 2016 and Dec 2020. MBP-mean blood pressure, DBP-diastolic blood pressure and SBP-systolic blood pressure, vasoactive inotrope core, were collected at baseline, pre-DEX infusion and at 1 – 4-hour intervals in the first 24 hours after starting DEX. Total sample is 228; at the time of abstract submission, we completed 60% of data input. Data was recorded into REDCap™ and descriptive statistics were calculated using SAS 9.4. Baseline data was compared to post DEX infusion at 1–4 hour intervals in the first 24 hours and the patients were divided into GA groups.

Summary of Results

160 neonates had a mean GA of 33 weeks (SD 5.7) at DEX initiation. All age groups had a decreased heart rate 12 hrs after DEX initiation and the largest mean reduction in heart rate (12%) in the 28–32 wks GA group. Changes in BP were largest for newborns <28 wks at 9–12 hrs with a 13.7% decrease in mean DBP, 12.4% decrease in MBP and 11.4% decrease in mean SBP associated with a mean DEX dose of 0.4 mcg/kg/hr. In the 28–32 wks GA age group, largest decrease in BP was at 3–4 hour interval with 10.7% decrease in mean DBP, 8.9% decrease in MBP and 4.6% decrease in mean SBP associated with a mean DEX dose of 0.5 mcg/kg/hr.

Abstract #91 Figure 1

Diastolic, Mean, and Systolic Blood Pressure

Conclusions

Bradycardia was noted at intervals corresponding to the half-life associated with GA. There was more hypotension in preterm infants <28weeks GA. Newborns less than 28 weeks had the largest decrease in BP associated with a mean DEX dose of 0.4 mcg/kg/hr. Additional studies are needed in this population to better assess clinical significance of such hemodynamic changes.*

#92  Cardiovascular sequelae of psychiatric disorders

S Minhas1,2*

F Hassan3

M Malik4

JR Patel3

D Hana5

RN Khouzam3

1Baptist Memorial Hospital Desoto, Southaven, MS

2Emory University School of Public Health, Atlanta, GA

3The University of Tennessee Health Science Center, Memphis, TN

4East Tennessee State University James H Quillen College of Medicine, Johnson City, TN

5West Virginia University, Morgantown, WV

Purpose of Study

To review the current literature on the effect of psychiatric disorders on the cardiovascular system, recognize the damaging impact of psychological stress and disorders on the physical health, and adopt screening and intervention modalities to decrease the treatment cost.

Methods Used

This is a review on the effect of psychiatric disorders on the cardiovascular system. Psychiatric conditions such as depression, anxiety, bipolar disorder, schizophrenia, stress, type A and D personalty disorders, and obsessive-compulsive disorder are presented.

Summary of Results

Cardiovascular disease (CVD) is the leading cause of morbidity and mortality worldwide. Many psychiatric conditions pose an independent risk factor in development of coronary artery disease and can lead to detrimental cardiovascular outcomes. Etiology of CVD is multifactorial, including genetic, biological, and environmental factors. Many of these mental health disorders result in dysregulation of the hypothalamic-pituitary-adrenal (HPA) axis and the sympathetic nervous system, thus contributing to hypertension, left ventricular hypertrophy, coronary vasoconstriction, endothelial and platelet dysfunction, and production of pro-inflammatory cytokines.

Major depressive disorder, bipolar disorder, and schizophrenia are associated with a 2–3 fold increased mortality rate and poor prognosis especially from CVD. Mortality rate ratio secondary to CVD can be as high as 8-fold in bipolar patients less than 40 years. Surprisingly, treatment with lithium in bipolar patients blunts the CVD risk in comparison to the second-generation antipsychotic medications which increase the risk of CVD. Initial presentation of CVD in schizophrenic patients can manifest as sudden cardiac death due to undetected myocardial infarction, as indicated by Q waves on the electrocardiogram.

People with hostility (a peculiar trait of type A behavior pattern) and type D personality disorder experience stress-related adverse effects and have an increased risk of CVD. Social support deprivation and social isolation are associated with a higher risk of CVD like atherosclerosis, cardiovascular mortality, and myocardial infarction. Obsessive-compulsive disorder also increases the risk of cardiovascular or metabolic complications. Patients with PTSD have a 2-fold increased risk of coronary heart disease, irrespective of the traditional CVD risk factors. Takotsubo cardiomyopathy, or stress-induced cardiomyopathy, is associated with significant emotional or physical stress. Numerous studies have supported incorporating psychosocial interventions such as cognitive behavioral therapy and transcendental meditation in the traditional cardiovascular rehabilitation programs that may help in reducing the risk of CVD events.

Conclusions

Our review suggests that many mental health disorders can contribute to detrimental cardiovascular outcomes. Providers should remain vigilant and keep a low threshold in screening patients presenting with mental health disorders for CVD.

#93  Remote pulmonary artery hemodynamic monitoring as outpatient in heart failure

B Mohanakrishnan1*

R Nambiar1

B Reyes2

1Texas Tech University Health Sciences Center, Amarillo, TX

2Amarillo Heart Group, Amarillo, TX

Case Report

Role of PA pressure monitoring in preventing recurrent hospital admissions in HF

Case presentation

A 67-year-old male with a past medical history of nonischemic cardiomyopathy, HFrEF NYHAIII StageC, hypertension, and DM with recent LHC in 2019 showed mild diffuse atherosclerosis was managed conservatively. He received an implantable PA monitoring device (CardioMEMS) in March 2021 to remotely monitor his home PA pressures on a daily basis. The average diastolic PA pressures were in the range of 5–10 mmHg since insertion of the ambulatory device with routine HF medication and lifestyle modifications. The daily monitoring system began to sense gradually raising diastolic PA from 19–22 mmg Hg. The patient was contacted to screen for any acute worsening of heart failure. He reported worsening of shortness of breath and a decrease in appetite corresponding to raising End DP PA pressure by the CardioMEMS. The patient was advised to 2X Lasix and report to the outpatient clinic. In the next 3–4 days, Diastolic PA began trending upward, peaking in the range of 25–30 mmg Hg. The patient was scheduled for an urgent outpatient visit; at this, we noted that there were no signs of fluid overload in the peripheries or any significant weight gain but worsening of his shortness of breath with all other examinations appearing normal. The patient‘s diuretics regiment was further intensified in this encounter. Subsequent ambulatory pulmonary diastolic pressure begins to trend down towards his usual range of 10 mmHg with the improvement of the patient‘s symptom of shortness of breath. His diuretics were gradually stepped down, and he continued to maintain his usual state health with improvement in his clinical outcomes.*

Discussion

In clinical practice, ambulatory hemodynamic monitoring of a patient with cardioMEMS made clinicians take medicine one step closer to the patient‘s home and intercept treatment earlier, even before any worsening clinical signs, helping avoid hospitalization and at the same time improve patients quality of life in HF. GUIDE-HF study of 1000 patients reported that hemodynamics-guided management of heart failure did not result in lowering the composite endpoint rate of mortality but indicated a possible benefit primarily driven by a lower heart failure hospitalization rate compared with the control group.2 In this new era of COVID, we can mitigate the need for our patients to come to the medical facility frequently and to be able to keep our advanced HF patients safe and healthy at home.

Conclusion

Ambulatory hemodynamic monitoring based on pulmonary pressure guided therapy for HF has shown beyond doubt that lower PA pressure, lower rates of heart failure are associated with hospital admission. These devices can sense very early changes in patient clinical conditions even before any early signs of fluid overload appear. Above all, it builds a huge patient-provider trust by knowing your patients‘ hemodynamics the best.

#94  Impact of interstage home monitoring between the first and second stages of surgical palliation on quality markers of morbidity and mortality in in high-risk neonates

R Mehta

H Sthole*

J Chen

Augusta University, Augusta, GA

Purpose of Study

Congenital Heart Defects occur in 1:100 births in the United States. The most critical and complicated are ‘single ventricle pathology’, which occur in about 5:100,000 live births. These children require multi-step surgeries and close monitoring due to high risk of sudden death related to shunt thrombosis or cardiac arrhythmia. The purpose of this retrospective observational study is to evaluate whether interstage home monitoring between the first and second stages of surgical palliation can reduce urgent cardiac interventions, unplanned admissions and mortality in such high-risk infants.

Methods Used

A retrospective review of infants after completion of 1st stage surgical palliation admitted to pediatric intensive care between June 2018- December 2021 was done. Parents were provided with an infant scale, pulse oximeter, home oxygen, and a heart rate monitor upon discharge. Parents recorded daily measurements of heart rate, oxygen saturation, all medications administered, intake and output (amount of urine and stool in diapers measured by weight), and daily body weight, which were collected and recorded at weekly clinic visits. This continued until the second stage of surgical palliation. Multivariable contingency analysis was performed using Fishers exact test with response variables defined as unplanned admissions, urgent cardiac intervention required, and mortality during interstage monitoring. Covariate was defined as compliance with home monitoring program with 75% overall participation defined as compliance and <50% overall participation defined as non-compliance.

Summary of Results

Of the 35 infants who participated in the Interstage Home Monitoring program, 68.5% were compliant with the program. 65.7% of infants did not require unplanned hospital admissions. 14.2% had unplanned admission secondary effects related to cardiac defects, 14.2% had unplanned admission due to infection. Only 34.2% patients required cardiac intervention. Compliance to home monitoring led to fewer unplanned hospital admissions (p- 0.06). but was insignificant for cardiac intervention (p- 0.5). and for mortality at home (p-0.5). Mortality was low in our study with 94.3% of infants surviving to the second stage of surgical palliation.

Conclusions

Infants who enrolled in a home monitoring program had fewer unplanned admissions and had increased surveillance compared to infants historically not enrolled in a monitoring program. Though larger study is needed, our observation suggests even minimal compliance in the program may give a protective benefit for surviving the interstage period.

#95  Utilization of electrophysiological procedures based on current procedure terminology (CPT) codes in the united states 2016–2017. a national ambulatory surgery sample study 2016–2017

DC Voruganti*

V Chidambaram

A Kumar

JL Mehta

University of Arkansas for Medical Sciences, Little Rock, AR

Purpose of Study

Catheter based ablation related electrophysiological (EP) procedural reporting using current procedural terminology (CPT) codes has not been described before.

Methods Used

The National Ambulatory Surgery Sample database from the years 2016–2017 was used to identify encounters with CPT codes. Encounters with first CPT code with 93650 (Atrioventricular AV node ablation), 93653 (supra-ventricular tachycardia SVT ablation), 93654 (Ventricular ablation), 93656 (Atrial fibrillation AF ablation) were used for the purpose of this study.

Summary of Results

In the United States, highest number of catheter-based ablation related EP procedures were performed in the south (77,740) followed by mid-west (75,029), west (56,819) and then the north-east (37,101). The highest number of procedures were AF ablation (112,389), followed by SVT ablation (105,623), ventricular ablation (17,925) and AV nodal ablation (10,723). Higher number of procedures were performed in the fourth quarter October to December (63,147) and lowest in the first quarter January-March (56,343). Largest number of procedures were in the private insurance group (111,063) followed by Medicare (107,014), and Medicaid (14,913). 99.3% were discharged to home after their procedure. 83.26% procedures were performed in the urban teaching hospitals. 78.28% procedures were performed in hospitals with bed size more than 300 beds and 20.36% were performed in hospitals with bed size 100–299 beds. 82.66% were performed in private non-for-profit hospitals.

Conclusions

This study sheds light on the utilization of EP procedures in the United States. Further studies are needed to explore the regional, seasonal, and the payer-based disparities in the utilization of these procedures.

#96  Cardiac tamponade as a presenting symptom of tuberculosis

M Abohelwa1*

P Khalil1

M Botros2

G Del Rio-Pertuz1

N Mittal1

K Parmar1

K Nugent1

1Texas Tech University System, Lubbock, TX

2Cairo University Kasr Alainy Faculty of Medicine, Cairo, Egypt

Case Report

Introduction

Tuberculous pericarditis is a rare complication of tuberculosis (TB); the diagnosis is usually difficult to establish and may even be delayed or missed. Mortality is very high if the diagnosis is missed and difficult to treat.

Hospital Course: 66-year-old male patient known to have high blood pressure presented to the ED with 2–3 hours of chest pain. He described his substernal chest pain as dull, pressure-like, unrelated to exertion, none radiating, and associated with dizziness. He described similar pain three months before. He also described chronic cough, night sweats, and weight loss that was attributed to allergy/sinusitis and was not investigated. The physical exam was unremarkable except for being in distress. EKG in the ED showed a new right bundle branch block. The patient oxygen saturation started to drop to 89%. He began to develop hypotension in the ED, nonresponsive to fluids and requiring vasopressors. He had a computed tomography pulmonary embolism protocol in the ED that showed probably exudative pericardial effusion with increased density compared with simple fluid. The largest pockets were along the under surface of the heart and posterior to the left atrium, where there was also some mass-effect along the posterior left atrium with coronary artery disease, mitral annulus, and aortic valve calcifications with pericardial calcifications. It also showed bilateral ground-glass alveolitis and dependent atelectasis along with the posterior lungs bilaterally. Transthoracic echocardiography showed possible intracardiac lesion, RV failure, loculated pericardial effusion. In the morning, the patient was emergently taken to OR for emergent thoracotomy. Soon after transfer to the OR table, the patient went into cardiac arrest. Thorax was opened, and the patient was put on the bypass in around 5–10 minutes. He was safely able to come off bypass. The patient was found to have a perforated PDA rupture which was ligated, and the pericardial effusion evacuated. The patient was critically ill, requiring multiple pressors, and unfortunately passed away. Pericardial fluid analysis/PCR was suggestive of tuberculosis.

Discussion

The symptoms of tuberculous pericarditis are usually nonspecific, like fever, weight loss, cough, dyspnea, chest pain, and night sweats. The symptoms usually depend upon the stage of infection, the extent of tuberculous disease outside the pericardium, and the degree of pericardial involvement. In addition, patients may present with clinical constrictive, effusive constrictive pericarditis, myopericarditis, and/or cardiac tamponade. Pericardiocentesis with fluid analysis is required for diagnosis; however, the sensitivity for detection by PCR is even low as 15%, with tissue diagnosis required with a sensitivity of 81% to detect TB by PCR.

#97  Chronic diarrhea with significant weight loss due to olmesartan induced enteropathy

A Kankam*

B Daines

SA Tanami

KG Holder

V Vemulapalli

R Nambiar

Texas Tech University Health Sciences Center School of Medicine Amarillo, Amarillo, TX

Case Report

Olmesartan induced enteropathy is rare but should always be considered as a differential diagnosis of sprue-like enteropathy. Early diagnosis is important since replacing olmesartan with an alternative antihypertensive drug can simplify the diagnostic workup and can provide clinical improvement.

A 54-year-old male with a past medical history of hypertension, diabetes mellitus, and gout presented with the complaint of constant passing of loose watery stools, 5–6 times daily for about a month. He endorsed 35-pound weight loss, nausea, chills, and low-grade fever without any abdominal pain during this period. Additionally, he had constant chest pain, which worsened with inspiration, radiating to the right shoulder without any relieving factor for the past one week. The patient also mentioned that he was admitted to the hospital two weeks earlier with the same presentation, but now with chest pain. Initial evaluation, including the basic lab tests, was unremarkable except low bicarbonate (11) and vitals were stable. Chest x-ray revealed left lower lung opacities indicative of pneumonia which was treated with doxycycline. Imaging showed some diverticulosis without colitis and the stool pathogen panel was negative. Home medications included allopurinol, colesevelam, cyclobenzaprine, dapagliflozin, dulaglutide, gemfibrozil, hydrochlorothiazide, olmesartan, pregabalin, rosuvastatin, and sitagliptin. Colonoscopy and subsequent biopsy were unremarkable. Due to persistent diarrhea without any identifiable trigger, olmesartan was discontinued to rule out potential medication-induced enteropathy and the patient was discharged.

The patient presented 3 days after being discharged due to persistent diarrhea and chest pain. Work-up included amyloidosis, carcinoid syndrome, intestinal tuberculosis, celiac disease, VIPoma, and Zollinger-Ellison syndrome which were all negative. Chest x-ray revealed pericardial effusion which resolved shortly after admission with supportive care. However, without further gastroenterology intervention, the patient’s diarrhea began to resolve over the course of this hospital stay and the patient was experiencing one normal stool per day. The patient was discharged and, in his follow-up visit with the cardiologist two weeks later, there were no more reported symptoms of diarrhea.

Discussion

Olmesartan-induced enteropathy was first reported in 2012 with the FDA publishing an adverse event warning of sprue-like enteropathy in 2013. Enteropathy most often appears after months to years of olmesartan exposure resulting in diarrhea and weight loss. Drug cessation is associated with symptom improvement within days and weight gain within weeks.

Conclusion

Although rare, olmesartan-induced enteropathy is an important consideration to make when prescribing olmesartan or in patients who present with unexplained sprue-like enteropathy. Cessation of the drug can lead to rapid improvement of symptoms and quality of life.

#98  Intracardiac myxoma presenting with embolic stroke

G Daniels*

C Hunley

C Hebson

The University of Alabama at Birmingham, Birmingham, AL

Case Report

Strokes affect approximately 5–10 per 100,000 children each year with higher incidence at neonatal and young adult ages and significant resultant morbidity. Etiology is broad and multisystemic, classically divided into ischemic and hemorrhagic origins. This case recounts diagnosis of a cardiac myxoma, a rare cause of embolic stroke.

Case

An 11-year-old male with a history of intermittent asthma developed acute difficulty walking with an inability to lift his right foot. In the school nurse’s office, he was unable to sign in using his right hand and developed a right facial droop. His mom took him to a local ED where non-contrast CT did not show acute findings. With a presentation still concerning for stroke, he was transferred to our hospital where his exam revealed slurred but comprehensible speech, right facial droop and asymmetric smile, right arm and leg hemiparesis, right hemibody paresthesia, right ankle clonus, and positive right Babinski. He denied recent fever or illness, previous episodes, or family history of strokes and seizures. A fast sequence stroke MRI identified acute infarcts in the left middle cerebral artery territory secondary to likely embolic phenomena. He was started on aspirin and had gradual near complete resolution of symptoms. Extensive hematologic, neurologic, and rheumatologic workup were unrevealing; however, an echocardiogram revealed large, heterogenous, friable, left atrial mass extending from the posterior wall and abutting the mitral valve. Surgical excision produced a 3.5 x 3.0 x 1.2 cm cardiac myxoma. Retroactive detailed family history revealed a maternal grandmother with a history of cardiac myxomas requiring surgical excision. Genetic testing for Carney complex was thus completed prior to discharge.

Discussion

Cardiac myxomas are rare in the pediatric population, though incidence increases with age such that they are the most common primary cardiac tumor of adulthood. Such tumors are most commonly found in the left atrium along the atrial septum near the fossa ovalis. Obstructive, embolic, and constitutional presentations are possible with the most critical being cardiac or cerebral infarction. That said, early findings such as skin manifestations (lentigines, large birthmarks), non-specific constitutional symptoms, and the rare auscultatory ‘tumor plop’ are more often recognized retroactively. Diagnostic concern is raised via echocardiogram and confirmed with pathology after surgical excision. Recurrence rate is usually low; however, follow up screening echocardiogram is prudent and patients with multisystem findings or family history should be screened for Carney Complex – an autosomal dominant disorder that increases the recurrence rate of myxomas. This report highlights a subtly indolent disease that has the potential for significant morbidity, but once recognized can be treated with cardiac surgery.

#99  Non st elevation myocardial infarction secondary to dexamethasone; a case report

G Del Rio-Pertuz*

J Rios

D Davis

K Parmar

K Nugent

Texas Tech University Health Sciences Center J T and Margaret Talkington Department of Internal Medicine, Texas Tech University Health Sciences Center J T and Margaret Talkington Department of Internal Medicine, Lubbock, TX, US, academic/meddep, Lubbock, TX

Case Report

There are well known risk factors for coronary artery disease (CAD) including diabetes mellitus, hypertension, hyperlipidemia, and/or family history. However, it has been shown that nontraditional risk factors also have a significant role in more than 50% of CAD cases. For example, many drugs have been reported to trigger an acute coronary artery syndrome. We report a case of Non ST Elevation Myocardial Infarction (NSTEMI) associated with hyperglycemia due to dexamethasone use.

Case

70-year-old woman with a past medical history of coronary artery disease, end stage renal disease, diabetes mellitus, hypertension and chronic back pain presented to the emergency department complaining of neck pain, palpitations, and significant hyperglycemia status post a dexamethasone injection for her chronic back pain. Patient states that the glucometer read blood glucose levels from 650 to 700 mg/dL directly after the shot was given. During the first assessment, her vital signs showed HR:75 bpm, RR: 16 rpm BP: 200/89 mmhg O2 Sat:98% on room air and labs of Hb: 12 g/dl WBC: 11.11 k/dl, PLT: 299 k/dl, Glucose:697 mg/dL, Serum osmolality:319 mOsm/kg, K:4.7 MMOL/L HCO3:21 MMOL/L, troponin T High Sensitivity:147.7 ng/l. Electrocardiogram showed sinus rhythm with left ventricular hypertrophy. Hyperglycemia and blood pressure were controlled in the emergency room and the patient was managed as a NSTEMI. Anti-ischemic treatment was started with heparin drip, dual antiplatelet therapy and high dose statin. She was transferred to the catheterization lab where the angiogram revealed obstructive proximal right coronary artery stenosis, where one stent was placed. Patient was discharged the following day on guideline directed medical therapy: dual antiplatelet therapy, beta-blocker, and angiotensin converting enzyme inhibitor.

Discussion

Glucocorticoids impact traditional risk factors for CAD, but also influence vascular functions, atherogenesis, and cardiac remodeling following ischemia or intravascular injury. Dexamethasone can promote an atherogenic and hypercoagulable state through multiple pathways, by increasing levels of Von Willebrand Factor, P-selectin, Fibrinogen, VII, VIII, or XI. Physicians should be aware of this possible association, especially because of the wide use of corticosteroids for a variety of indications. Cautious use should be considered particularly in patients with risk factors for CAD.

#100  Complete heart block in mitochondrial myopathy, encephalomyopathy, lactic acidosis and stroke-like episodes syndrome in the absence of structural heart changes

G Del Rio-Pertuz*

M Abohelwa

J Rios

Z Elharabi

K Parmar

E Elgwairi

K Nugent

Texas Tech University Health Sciences Center J T and Margaret Talkington Department of Internal Medicine, Texas Tech University Health Sciences Center J T and Margaret Talkington Department of Internal Medicine, Lubbock, TX

Case Report

The heart is an organ with high metabolic demand, making mitochondrial function a key determinant of myocardial performance. Mitochondrial myopathy, encephalomyopathy, lactic acidosis and stroke-like episodes (MELAS) syndrome is a maternally inherited multisystemic disorder caused by mutations of mitochondrial DNA. Cardiac dysfunction occurs in approximately a third of these patients. The most common cardiac abnormality is ventricular hypertrophy. Conduction abnormalities have been reported but are not well elucidated. We report a case of a 23-year-old woman with MELAS syndrome who presented with complete heart block without structural changes of the heart mimicking a seizure-like episode.

Case

23-year-old woman with past medical history of diabetes mellitus and mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS) syndrome came to the emergency department accompanied by her mother due to a seizure-like episode. The patient’s history was limited to patient’s mother due to a baseline non-communicative state. The mother inferred witnessing an episode of tonic-clonic movements lasting 10 minutes with no apparent conscious responsiveness. First assessment showed vital signs and labs of HR: 41 bpm, RR: 17 rpm, BP: 130/92 mmHg, O2 Sat: 98% on room air, Hb: 17.1 g/d l WBC: 8.9 k/dl, PLT: 100 k/dl, lactic acid 5.6 MMOL/L, K+: 5.3 MMOL/L, and TSH: 1.84 mcIntUnit/m. Electrocardiogram demonstrated a complete heart block (figure 1), and transthoracic echocardiogram showed a left ventricular apical akinesis with normal wall motion of the mid and basal segments with left ventricular ejection fraction of 55%. Her mother did not consent to temporary transvenous pacing, so the patient was started on dopamine drip preceding by implantation of a permanent pacemaker. The patient was discharged after two days of hospitalization.

Discussion

The hallmark abnormality of MELAS is the occurrence of stroke-like episodes and seizures in the presence of hyperlactatemia. Cardiac abnormalities are common, occurring in 30–56% of patients. Hypertrophic cardiomyopathy is the most common phenotype, followed by dilated cardiomyopathy, and pulmonary arterial hypertension respectively. Electrophysiological abnormalities such as complete heart block have been reported in the presence of structural abnormalities such as chambers dilation and hypertrophy. However, the case we describe did not have any structural heart disease, which raises the hypothesis that complete heart block can occur even in the absence of structural changes, and more studies are needed to confirm this association.

#101  Thrombus or vegetation? the importance of following the duke criteria for infective endocarditis before proceeding with advanced cardiac imaging

G Del Rio-Pertuz*

J Rios

K Parmar

M Abohelwa

A Cavazos

Z Elharabi

E Elgwairi

K Nugent

Texas Tech University Health Sciences Center J T and Margaret Talkington Department of Internal Medicine, Texas Tech University Health Sciences Center J T and Margaret Talkington Department of Internal Medicine, Lubbock, TX

Case Report

We present a case of a middle-age male who based on the Duke criteria (DC), we were able to rule out infective endocarditis (IE) before proceeding with advanced imaging.

Case

We present a case of a 51-year-old man with past medical history of heroin and cocaine use disorder, that was admitted to the intensive care unit (ICU) becouse COVID-19 pneumonia. On day 1 of admission, transthoracic echocardiogram (TTE) showed moderate tricuspid regurgitation. In the ICU, blood cultures (BC) were positive for Staphylococcus coagulase-negative species, patient received a course of vancomycin for 7 days. On day 17, the patient had repeat TTE, which showed new nodular thickening of the tricuspid valve associated with tricuspid regurgitation. BC were sent that same day, and they grew out Staphylococcus coagulase-negative species. At that time, it was not clear if new tricuspid valve findings were secondary to vegetation or a thrombus. The DC for infective endocarditis was applied to determine whether the findings on the TTE were due to IE. Major criteria include positive blood cultures and findings of vascular vegetation on echocardiography. After analyzing the BC and meeting with the microbiology personnel, it was found that the Staphylococcus coagulase-negative species found were different from each other. However, there was a possible vegetation present, shown on echocardiography. The patient had predisposing heart condition of tricuspid regurgitation and a history of IV drug abuse. Fever was absent. As for vascular phenomena, the patient did not have skin changes and no pulmonary embolism was found following chest CT protocol. As for immunologic phenomena, the patient did not have Roth spots or glomerulonephritis. Lastly, for microbiological evidence, the patient’s blood cultures were possibly positive due to contamination. Based on the DC the patient had a possible diagnosis of IC but it was rejected because a patient had a firm alternative diagnosis, mainly a ventricular thrombus. The patient received anticoagulation treatment for thrombus, and he was discharged on his 24th day of hospitalization without complications.

Discussion

The diagnosis of IE remains difficult due to low specificity of the laboratory and imaging tests. Our patient had all the risk factors to believe it was endocarditis instead of a thrombus. Based on the DC, endocarditis was ruled out and the patient responded well to anticoagulation. More studies are needed to clarify the utility of the DC as a method to differentiate IE from thrombus before proceeding with advanced imaging.

#102  Idiopathic cardiomyopathy associated with delayed gadolinium enhancement and fibrosis found on endomyocardial biopsies, can endomyocardial fibrosis be possible in west texas?

G Del Rio-Pertuz*

K Nugent

E Argueta-Sosa

Texas Tech University Health Sciences Center J T and Margaret Talkington Department of Internal Medicine, Texas Tech University Health Sciences Center J T and Margaret Talkington Department of Internal Medicine, Lubbock, TX

Case Report

Endomyocardial fibrosis (EMF) is a disease of rural poverty that is characterized by fibrosis of the apical endocardium. It is thought that eosinophils infiltrate the heart due to transient episodes of eosinophilia secondary to parasitic infections.

Case 1: A 58-year-old man with past medical history of asthma and atrial fibrillation (AFIB) was admitted to hospital with dyspnea and elevated troponins. During this admission he was also diagnosed with allergic bronchopulmonary aspergillosis. He met criteria based on eosinophils (6.26 K/µL), chest imaging, and positive IgG, and IgE levels for aspergillosis. He received treatment with prednisone. Transthoracic echocardiogram (TTE) was done and showed a left ventricular ejection fraction (LVEF) of 30%. Coronary angiography did not reveal epicardial coronary artery disease (CAD), and CMR imaging showed subendocardial delayed gadolinium enhancement suggestive of scarring in the septal and left inferoseptal walls with LV EF improvement (47%). The posibility of eosinophilic myocarditis was considered and the patient was scheduled for EMB that demonstrated moderate myocyte hypertrophy and moderate scattered focal interstitial fibrosis. The day when the EMB was done, eosinophils were within normal limits (0.145K/µL) and the patient had already completed the course of steroids.

Case 2: A 65-year-old man with PMH of AFIB was evaluated due to new-onset systolic heart failure found during a previous hospitalization where he had an AFIB ablation. TTE demonstrated a LVEF of 34% without wall motion abnormalities. On coronary angiography no significant CAD was found. CMR was done and demonstrated delayed gadolinium enhancement suggestive of scarring in the septal and anteroseptal mid-myocardial wall. The differential diagnosis was either sarcoidosis or amyloidosis. EMB was done and showed moderate myocyte hypertrophy and moderate interstitial fibrosis. The patient was approached as idiopathic dilated cardiomyopathy and is currently stable on guidelines directed medical therapy.

Discussion

EMF is a disease from the tropics and primarily in poor rural areas from several countries of eastern Africa. This disease can be secondary to late scarring due to eosinophilic deposition and/or chronic infections. West Texas is considered a rural area, where autochthonous transmission of neglected parasitic diseases has become increasingly reported. We consider that the first case most likely had scarring due to previous eosinophilic infiltrate secondary to the bronchopulmonary aspergillosis. In contrast case 2 does not have a clear etiology for endomyocardial fibrosis and appears to be more advanced.

#103  The forgotten findings on the chest x-ray and transthoracic echocardiogram suggestive of pulmonary embolism; co-occurrence of mcconnell’s, palla’s, westermark’s, and hampton’s hump in a single patient

G Del Rio-Pertuz*

K Parmar

K Nugent

E Elgwairi

M Ansari

Texas Tech University Health Sciences Center School of Medicine, Lubbock, TX

Case Report

We present a case of a woman with a PE whose imaging showed the co-occurrence of all four signs, McConnell’s, Palla’s, Westermark’s, and Hampton’s Hump, a scenario that has rarely been reported in the literature.

Case presentation

59-year-old woman with a past medical history of migraines, fibromyalgia, hypertension, psoriatic arthritis, and hypothyroidism who presented to emergency department complaining of shortness of breath, retrosternal chest pain for six days, and right leg swelling for 3 days. During the first assessment she was hemodynamically stable, her vital signs and labs were HR:78 bpm, RR: 17 rpm, BP: 165/109 mmhg, O2 Sats:98% on room air, Hb: 12.7 g/d l, WBC: 8.4 k/dl, PLT: 230 k/dl, BNP: 2240 pg/mL, and Troponin T High Sensitivity:25.4 ng/L. Acute pulmonary embolism was suspected; thus, a bedside transthoracic echocardiography was performed, which showed moderate to severe enlargement of the right ventricle (RV) and severe hypokinesis of the mid RV free wall with the apical segment contracting normally (McConnell’s Sign). Initial CXR(figure 1) showed a wedge-shaped opacity (black arrow) in the lung’s right middle lobe (Hampton’s hump), a focal area of oligemia (area between white arrow heads) in the right lower zone (Westermark’s sign) and a prominent right descending pulmonary artery (red arrow)(Palla’s sign). Suspicion for pulmonary embolism increased, so the patient underwent CT angiogram of the chest for confirmation. It showed bilateral segmental PE involving all lobes causing a considerable burden.

Conclusion

CXR and echocardiogram findings of PE are generally nonspecific. However, there are certain signs that have a higher specificity; they are Palla’s sign, Westermark sign, and Hampton’s Hump in CXR and McConnell’s sign in the echocardiogram. Westermark’s sign is attributed to a focal area of enhanced translucency due to oligemia. Palla’s sign refers to an enlarged right descending pulmonary artery. Hampton’s hump is a wedge shaped opacity with a rounded convex apex directed towards the hilum, indicating a pulmonary infarction distal to the thrombus. McConnell’s sign is defined as right ventricular free wall akinesis with sparing of the apex, it is caused due to the tethering of the right ventricular apex with a hyperdynamic contracting left ventricle.

#104  Clinical and hemodynamic improvement guided by a cardiomems pulmonary arterial pressure monitoring system in a patient with heart failure with preserved ejection fraction; a case report from the first patient implanted in our center

G Del Rio-Pertuz*

K Nugent

E Argueta-Sosa

Texas Tech University Health Sciences Center, Lubbock, TX

Case Report

Among all patients with heart failure, nearly half have heart failure with preserved ejection fraction (HFpEF). Its prevalence is increasing by about 1% annually relative to that of heart failure with reduced ejection fraction. A pulmonary artery pressure hemodynamic monitoring system, CardioMEMS, has been shown to improve quality of life and decrease hospitalizations in patients with HFpEF. We report the first patient implanted with a CardioMEMS hemodynamic monitoring system done in our institution to guide heart failure therapy.

Case

60-year-old man with a past medical history of chronic kidney disease, atrial fibrillation, and obstructive sleep apnea, was diagnosed with HFpEF associated to coronary artery disease and valvular heart disease. He was New York Heart Association class III, had one hospitalization for congestion at our institution, and multiple prior admissions at outside hospitals. With the prior history he met criteria and agreed to undergo CardioMEMS implantation. Right heart catheterization hemodynamics were: Right atrial pressure:18 mmHg, Right ventricular systolic pressure: 92 mmHg, Right ventricular diastolic pressure: 2 mmHg, Pulmonary artery systolic pressure (PASP):90 mmHg, Pulmonary artery diastolic pressure (PADP): 21 mmhg, mean pulmonary artery pressure: 52 mmHg, Pulmonary artery wedge pressure: 33 mmHg, Cardiac output by thermodilution 5.61 L/min, cardiac index:2.47 L/min/m2. He met criteria for combined pulmonary hypertension by the revised ESC criteria. Despite increasing diuretics after implant, he required an additional hospitalization, where he was also noted to be in atrial fibrillation. After IV diuresis, escalating diuretics and a cardioversion, he was able to be discharged. During this time he learned about dietary habits that were harmful to him, and improved CPAP compliance by using a chin strap (figure 1).

Discussion

The use of an implantable device to provide daily pulmonary artery hemodynamic information has been shown to reduce heart failure hospitalizations and all-cause hospitalization either in heart failure with reduced ejection fraction or HFpEF. Once implanted, the device can detect rising pressures in the pulmonary artery, which can be an early warning of fluid backing up in the lungs and pending onset of congestion before symptoms are reported. Because the patients have to measures the pressures on their own, it is highly recommended that the devices are implanted in compliant patients. After pressures are measured, they are automatically transmitted electronically to providers, in that way they can review the readings and proactively adjust medical therapy which helps with symptoms which include edema and dyspnea.

#105  Takotsubo cardiomyopathy due to hemophagocytic lymphohistocytosis secondary to covid-19 pneumonia

G Del Rio-Pertuz*

D Davis

J Rios

K Parmar

K Nugent

Texas Tech University Health Sciences Center J T and Margaret Talkington Department of Internal Medicine, Texas Tech University Health Sciences Center J T and Margaret Talkington Department of Internal Medicine, Lubbock, TX

Case Report

Hemophagocytic lymphohistiocytosis (HLH) is a potentially fatal disease characterized by excessive immune response and cytopenia. Severe COVID-19 infection induces a life-threatening inflammatory syndrome associated with intense cytokine release that similar to HLH. We present a patient who developed takotsubo cardiomyopathy due to HLH.

Case

24-year olrd man with a past medical history of obesity was admitted at the medical intensive care unit (MICU) due to acute respiratory distress syndrome secondary to COVID-19 pneumonia. During the MICU stay, the patient required a high dose of vasopressors and ventilatory support. For Covid management, the patient received tocilizumab, high dose steroids (20 mg daily of dexamethasone), and empiric antibiotic coverage with vancomycin and cefepime. On day six of MICU admission, the patient developed hypertriglyceridemia (TGL) that was initially thought to be secondary to propofol, but after discontinuing propofol the patient continued to have increasing TGL levels. On day 8 of MICU admission, the suspicion of HLH increased, HSscore was calculated, and the patient had a 70–80% probability of having HLH (181 points: Temperature of 103 °F, ferritin 2580 ng/ml, TGL:771 mg/dl, Fibrinogen 220 mg/dl, AST:116 u/L). On day 10 of MICU admission, troponins increased from 7.5 to 2,966 ng/L, telemetry showed diffuse ST elevations, but ECG did not show any ischemic changes. At that time, his clinical parameters included HR: 96 bpm, BP: 92/42 mmHg, O2 Sat:93% on mechanical ventilation with pressure support FIO2: 100%, Hb: 11.6 g/dl, WBC:10.36 k/dl, Plt: 210 k/dl. Acute stress cardiomyopathy secondary to HLH was suspected. Transthoracic echocardiogram demonstrated preserved ejection fraction and inferoapical akinesia consistan as takotsubo cardiomyopathy. On day 11 of MICU admission, the patient had a cardiac arrest and after 30 minutes of cardiopulmonary resuscitation no return of spontaneous circulation was achieved.

Discussion

HLH induces a cytokine cascade that causes an excessive inflammatory response and multi-organ dysfunction that can be secondary to infections such as Covid-19. Takotsubo cardiomyopathy also known as stress cardiomyopathy, is a reversible dysfunction characterized by acute hypokinesia/akinesia of the apical and middle segments of the left ventricle that extends beyond a unique coronary territory. We conclude that the trigger for takotsubo cardiomyopathy in this case was related to excess catecholamine release secondary to HLH.

#106  An unusual case of postpartum cardiac tamponade

E Elgwairi1*

A Abdalla2

Z Elharabi1

M Abohelwa1

G Del Rio-Pertuz1

S El Nawaa1*

L Jenkins1

1Texas Tech University Health Sciences Center, Lubbock, TX

2University of Benghazi Faculty of Medicine, Benghazi, Libya

Case Report

Cardiac tamponade is an uncommon but life-threatening condition. Its occurrence in pregnant women or the puerperium may be somewhat confused with peripartum cardiac disease. Although diseases of the pericardium may occur sporadically during pregnancy, there is no evidence that pregnancy increases the susceptibility to pericardial diseases. However, recognition of other associated extracardiac manifestations coincident with this serious condition may lead to detection of the underlying etiology and consequently, the appropriate treatment. We report a rare presentation of Systemic Lupus erythematosus (SLE) with cardiac tamponade in a postpartum lady.

Case presentation

A 24-year-old female at 36th week of gestation was hospitalized due to seizure-like episodes. She has unremarkable past medical, family, and social history. On presentation, she was alert and oriented with no distress. Her physical exam was unremarkable. She underwent labor induction on the day of admission. Her vaginal delivery was complicated by postpartum hemorrhage. Two days after delivery, she developed shortness of breath and hemoptysis. She was normotensive, tachycardic with pulsus paradoxus, tachypneic, and required supplemental oxygen. The cardiac exam revealed JVD and distant heart sounds. Chest exam revealed decreased breath sounds. CXR showed patchy infiltrates. EKG showed electrical alternanas. Transthoracic echocardiography showed large pericardial effusion with signs of tamponade. Labs showed normocytic anemia with positive Coomb’s test, IgG, and hypoalbuminemia. She underwent pericardiocentesis. Based on clinical manifestations, a lupus panel was ordered. It was positive for ANA, Anti-dsDNA, and Anti-Smith antibodies with low C3 and C4. She was started on high-dose steroids in addition to mycophenolate Mofetil and hydroxychloroquine. She improved on SLE medications.

Discussion

There are no published data indicating the incidence of pericardial disease in pregnancy. Acute pericardial disease is usually viral. Infectious causes other than viral are less common. Noninfectious causes include trauma, iatrogenic, neoplastic, and autoimmune diseases. SLE is the most common autoimmune disease associated with pericarditis, and pericarditis is the most frequent cardiac manifestation of SLE. It is found in 62% of autopsies. However, pericardial effusion causing cardiac tamponade is an uncommon complication in lupus. Its incidence is estimated at 1- 2%. The diagnosis of SLE in our case was based on the combined picture of pericardial effusion, alveolar hemorrhage, anemia, neurologic manifestation, and subsequent serology. These patients are usually treated with pericardial drainage and immunosuppressant therapy. Physicians should be vigilant to recognize cardiac tamponade in a postpartum woman who presents with suggestive signs and symptoms, using a holistic approach to detect the etiology behind it. Recognition of this rare presentation of SLE may be lifesaving.

#107  Do you know that brugada syndrome is more prevalent in schizophrenic patients?

E Elgwairi*

A Abdalla

K Parmar

J Benjanuwattra

G Del Rio-Pertuz

Z Elharabi

M Abohelwa

Texas Tech University System, Lubbock, TX

Case Report

Brugada syndrome (BrS) is an autosomal dominant genetic disorder presented with abnormal findings on electrocardiogram (ECG). It increases risk of ventricular tachyarrhythmias and sudden cardiac death (SCD). Brugada ECG pattern characterized by a pseudo-right bundle branch block and persistent ST segment elevation in leads V1 to V2. It is more common among schizophrenic patient than in general population. Here we report a case of schizophrenia diagnosed with Brugada syndrome.

Case presentation

This is a 25-year-old male who presented to ER after a syncopal attack. He was told that he was found unconscious, no seizure, no urine incontinence. He stated he only remember having shortness of breath and dizziness while he was sitting just before the event. He reported similar syncopal episode 2 days ago. Denied SOB, chest pain, palpitation, fever, chills, cough, or other signs of infection. He had history of similar episodes last year. He was diagnosed with schizophrenia and bipolar disorder many years ago, as well anxiety. He was admitted to psychiatric facility at the age of 14–15 due to suicide attempt. He has history of Asthma. He takes Sertraline and aripiprazole and reported compliance. No family history of heart problem or sudden death He used to be heavy smoker, Meth and Marijuana user, but quit in 4 years ago. He denies EtOH intake. Vital signs were normal and physical exam was unremarkable. EKG showed Biphasic T wave inversion in V1 & V2 consistent with Brugada pattern type A. Lab results including urine screening test were unremarkable. Transthoracic echocardiography did not show any abnormalities. He was evaluated by Electrophysiology team and underwent after AICD placement without any complications. No further syncopal episodes occurred, and he was discharged in good conditions.

Discussion

Patients with schizophrenia have been found to have Brugada pattern ECGs more than the general population. Blom et al analyzed ECGs of a cohort of 275 patients with schizophrenia compared the findings with nonschizophrenia individuals of similar age. They found out Brugada pattern was more common in the schizophrenia cohort (11.6%) compared with non-schizophrenic cohorts at 1.1% and 2.4%. This result of 11.6% is significantly higher than the prevalence in general population estimated at 0.05–1%. Rastogi et al illustrated in his review article some similarities in pathophysiology of Brugada and schizophrenia. Genetic Mutations in calcium channels that have been found in schizophrenia are also present in 1–3% of BrS patients. Postrema et al presented a detailed list of drugs that induce or unmask Brugada pattern. ICD is the first line treatment for BrS. It reduces mortality rates in symptomatic patients with BrS. Providers should be vigilant for Brugada ECG pattern in schizophrenic patients and aware of the antipsychotic drugs that unmask or induce this fatal condition.

#108  Superficial femoral artery occlusion complicated by stent fracture

B Galvan1*

KG Holder1

A Tran2

B Boeger2

A Giakas3

M Ansari2

1Texas Tech University Health Sciences Center School of Medicine Amarillo, Amarillo, TX

2Texas Tech University Health Sciences Center School of Medicine, Lubbock, TX

3University of South Carolina School of Medicine, Columbia, SC

Case Report

Endovascular intervention for clogged arteries have shifted towards minimally invasive options such as angioplasty, atherectomy, or placement of stents. Stents are constantly being improved to increase durability and lifetime. Stent fracture, although uncommon, is associated with potentially severe consequences such as in-stent restenosis (ISR) or stent thrombosis (ST). Thus, repeated vessel revascularization is needed. We report a peculiar case of a fractured stent involving the distal superficial femoral artery (SFA).

Case presentation

Female patient age 63 with a PMH of peripheral artery disease with prior bilateral peripheral vascular interventions including the most recent being the placement of an iliac stent, hypertension, dihydrolipoamide dehydrogenase deficiency, paroxysmal atrial fibrillation, cerebrovascular accident, and an active smoker who presents with severe right lower leg resting pain. Patient was referred for peripheral angiography which showed chronic total occlusion (CTO) of the common femoral artery (CFA) and SFA. Decision was reached to treat percutaneously and for that purpose crossing catheter and guide wire was utilized to cross CTO. Balloon angioplasty of the CFA and SFA were performed and revascularization was achieved. It then became clear that the reason for occlusion was a stent fracture of a prior placed stent. At this point, flow was achieved but the struts of the stent fracture appeared to be the nidus for obstruction. The utilization of a covered stent to isolate the struts from the lumen wall seem to be a good option and henceforth, a Viabahn self-expanding stent was placed within the prior fractured stent. Significant improvement in the lesion was noted on angiography. Post-operative course was unremarkable, and patient was discharged on hospital the next with instruction for follow-up with the cardiologist.

Conclusion

Our case proves that Viabahn covered stent is a good option to treat occlusion due to a prior stent fracture. This helps isolate the struts from the vessel lumen which could act as nidus for future occlusion.

#109  EKG changes in severe hypercalcemia mimicking brugada’s syndrome

B Galvan*

KG Holder

K Moseley

M Diab

R Nambiar

Texas Tech University Health Sciences Center School of Medicine Amarillo, Amarillo, TX

Case Report

A 62-year-old Caucasian, female patient with history of celiac disease and chronic pain s/p spinal cord stimulator presented to our institution to follow up on abnormal lab findings. The patient presented to her PCP with complaints of worsening weakness, nausea, vomiting, constipation, polydipsia, and occasional palpitations. Labs resulted a severely elevated serum calcium level (17 mg/dL), increased BUN (32), and elevated Cr (1.8) indicating acute kidney injury. Full workup was initiated. Vitamin D, 25-Hydroxy level returned greater than 209 and PTH resulted in a normal range of 22. Detailed history revealed that the patient was taking 50,000 units of vitamin D3 by mouth six times/week for six months. Fear surrounding the current COVID-19 pandemic prompted the exorbitant intake of vitamin D supplementation in hopes of immune improvement. Bisphosphonate were contraindicated due to AKI.Volume expansion with normal saline and calcitonin successfully decreased the patient’s serum calcium.

Discussion

The diagnostic criteria for reversible Brugada pattern, recently classified as Brugada phenocopy, includes four mandatory components. Primarily, an ECG tracing delineating type 1 or type 2 Brugada morphology. Secondarily, the presence of an underlying condition that is identifiable and reversible. Third, complete resolution of the ECG pattern upon elimination or correction of the underlying condition. Fourth, a low probability for Brugada syndrome determined by the lack of symptoms, clinical history, and family history. Our patient experienced severe hypercalcemia with palpitations that prompted an ECG. The abnormal ECG produced was read independently by two interventional cardiologists and a cardiac electrophysiologist who all concluded the ST segment and T wave deviations were consistent with Brugada pattern type 1. Importantly, the ECG was compared to one from a year prior which showed a normal rate and rhythm. There was complete resolution on repeat ECG once serum calcium was returned to reference range. The patient did not experience Brugada specific symptoms of syncope, seizures, nocturnal agonal breathing, or sudden cardiac death. No family history suggested Brugada syndrome or cardiac issues. Electronic medical record documentation tracked over the last 5 years showed no concerns for prior arrhythmias or syncope. Additionally, the patient does not fit the epidemiological profile of a male of Southeast Asian decent which is classically associated with Brugada syndrome.

To our knowledge, this is the first documented presentation of Brugada phenocopy induced by severe hypercalcemia secondary to vitamin D toxicity.

Conclusion

Although the mechanism is not completely understood, severe hypercalcemia can cause a reversible type 1 Brugada pattern on ECG. Careful consideration of vitamin supplementation must be discussed with patients to avoid potentially fatal cardiac outcomes.

#110  Running healthy with arcapa? – a brief report of an adult athlete with anomalous right coronary artery of the pulmonary artery

LT Jaynes*

E Varney

K Miller

CM Howard

University of Mississippi School of Medicine, Jackson, MS

Case Report

A 17-year-old, healthy, and normally developed female with a family history of rheumatologic disease, presented with multiple daily episodes of sudden fatigue; however, she did not complain of dizziness, syncope, chest pain or palpitations. She denied exercise intolerance and stated that she had always been a successful track athlete with no limitations.

Initially, the patient underwent a full rheumatologic, endocrinologic, and metabolic work-up revealing a positive ANA and Sjogrens Syndrome A-nuclear antibody. Full cardiac work-up revealed normal segmental anatomy with diffuse dilation of both coronaries and anomalous origin of the right coronary and prominent collateral flow within the septum. Duplex ultrasound revealed retrograde flow in the right coronary artery suspect of a steal phenomenon from the pulmonary arterial system. As a result, the patient was referred for a Cardiac CT and Cardiac MRI for further evaluation.

Cardiac computed tomography confirmed severe dilation of both right (6 mm, proximally) and left (left main 7 mm and proximal LAD 6 mm) coronary systems with significant collateral blood flow, likely stemming from the left anterior descending artery to provide retrograde flow. Additionally, the right coronary artery was found to have anomalous origin from the main pulmonary artery.

Quantitative functional analysis using cardiac magnetic resonance imaging indicated good left ventricular function with no signs of ischemia or infarction, confirmed extensive collateralization, and increased delayed signal along the septum confirmed to be septal collaterals rather than delayed septal enhancement from scarring or infiltrative process.

The patient was advised to cease all sports and major physical activity. Patient then proceeded with successful surgical correction with re-implantation of the anomalous R coronary artery to the aorta above the sinotubular junction. Two years status post-correction, the patient is well and has been cleared for light physical activity.

Anomalous coronary arteries are rare abnormalities usually caused by abnormal embryogenesis; however, an anomalous right coronary artery from the pulmonary artery (ARCAPA) is considered the rarest with nearly 100 reported cases and a prevalence of 0.002%. This anomaly is considered to be isolated, benign and asymptomatic; however, sequalae have been reported. Collateralization of septal and LAD perforators is essential in order to prevent infarction and SCD. With retrograde flow in the right coronary system and the high probability of a steal phenomenon occurring in the pulmonary arterial system, septal perforators and LAD collaterals adequately compensated for the loss of significant coronary flow. Although much less severe with fewer complications than ALCAPA, understanding this rare pathology, its detection, and its management are essential.

#111  Immune checkpoint inhibitor associated pericardial effusion with serial transthoracic echocardiogram used for monitoring of disease progression

LN Joseph1*

C Lenneman2

1University of Alabama at Birmingham Sc, Birmingham, AL

2University of Alabama at Birmingham, Birmingham, AL

Case Report

Immune checkpoint inhibitors (ICIs) including Nivolumab, a PD-1 inhibitor, have been used for treatment in a variety of different cancers. Pericardial effusions are a rare, but potentially dangerous side effect of ICIs and warrant echocardiographic monitoring to prevent the development of cardiac tamponade.

Case

A thirty-one-year-old male with squamous cell carcinoma of the right tonsil with metastasis to the lungs presented to the Emergency Department with chest pain and shortness of breath. The patient had a heart rate of 110 but was normotensive on exam with ECG showing sinus tachycardia, normal voltage, and no ST changes. He was found to have worsening pleural metastases and a new moderately sized pericardial effusion on CT chest having started treatment with Nivolumab, a PD-1 inhibitor, one week prior to presentation. He had undergone CT pulmonary angiography two months prior to starting Nivolumab with no pericardial effusion seen on imaging. Transthoracic echocardiogram (TTE) confirmed a moderate to large pericardial effusion with no sign of tamponade physiology.

The Cardiology consult team evaluated the patient and recommended obtaining serial TTEs every two to four weeks to monitor size of the effusion and prevent development of tamponade. The patient came for each of these appointments with stability in effusion size until five months later during which he presented to the Emergency Department for weakness and dehydration. He was tachycardic with heart rate of 120 and blood pressure of 100/75 and was administered one liter of fluids. TTE was obtained which showed enlargement of the pericardial effusion with tamponade physiology. The patient was brought to the operating room with pericardial window performed by Cardiovascular Surgery with removal of 1.1 liters of serous fluid and intrapericardial drain placement. The pericardial fluid was sent for culture and flow cytometry and showed negative gram stain, fungal culture, with no malignant cells observed. Therefore, the development of the patient’s pericardial effusion was deemed likely due to initiation of Nivolumab therapy.

Decision-Making

ICIs have been associated with cardiac immune related adverse events (irAEs) including myocarditis, vasculitis, and pericardial effusion. The Cardiology team used serial TTEs to assess pericardial effusion size and stability for this patient. This approach enabled the medical team to expedite treatment when the patient developed signs concerning for cardiac tamponade.

Conclusion

Pericardial disease is a rare, but serious side effect of ICIs, with fatalities occurring in over 21% of cases. Patients with symptoms concerning for cardiac irAEs should seek immediate medical care. TTE can be used to evaluate for pericardial disease and serial TTEs can be used to monitor and treat enlarging pericardial effusions with concern for cardiac tamponade.

#112  A pseudoaneurysm with true consequences

SI Liles*

JA Puckett

R Patel

H Nannapaneni

R Noor

The University of Mississippi Medical Center, Jackson, MS

Case Report

Left ventricular aneurysms are serious, potentially fatal cardiac complications that rarely occur after myocardial infarction. General classification distinguishes true aneurysms from pseudoaneurysms based on involvement of vessel wall layers. The latter forms when pooled blood from cardiac rupture is contained by the pericardium. Pseudoaneurysm diagnosis is challenging due to nonspecific symptom presentation, including congestive heart failure, chest pain, dyspnea, or even asymptomatic. With the high propensity for pseudoaneurysms to rupture and result in sudden death, rapid diagnosis and treatment are essential to prevent significant morbidity and mortality. Currently, surgical intervention is the favored management, though medical therapy with afterload reduction, anticoagulation, and antiplatelet agents is used in patients not deemed surgical candidates. We present a unique case of new onset left ventricular pseudoaneurysm presenting as orthostatic syncope in a patient with recent NSTEMI.

Mr. P is a 64-year-old Caucasian male with recent NSTEMI requiring PCI and subsequent drug-eluting stent, atrial fibrillation, hypertension, and type 2 diabetes admitted for evaluation after a near-syncopal episode. The patient had a prolonged hospitalization 2 months prior for NSTEMI and embolic stroke, during which time he experienced cardiac arrest due to the NSTEMI. He was resuscitated and taken for coronary angiography where a stent was placed to the first diagonal artery, but a distal LAD embolism noted was unable to be stented due to total infarction of the tissue. His hospitalization was complicated by prolonged ICU admission with treatment for sepsis and cardiogenic shock post-PCI. He was noted to have a small LV aneurysm without thrombus during this time. He was discharged to home and began to experience syncopal episodes 1 month later. Thorough workup upon re-admission initially revealed no changes in echocardiography, and the patient’s orthostasis improved with IV fluid resuscitation. He was again discharged for ongoing rehabilitation. However, the patient’s pre-syncopal episodes without chest pain continued even 2 weeks later with profound orthostasis (change in systolic blood pressure >100 mmHg upon standing), and repeat echocardiography revealed an apical pseudoaneurysm. Further evaluation via gated CT and cardiac MRI revealed a left ventricular pseudoaneurysm measuring up to 3.3 x 3.9 cm that had significantly increased in size from two months prior, at which time it was noted to be a small true aneurysm. He is tentatively being evaluated for surgical correction as of the time of this case report.

In summary, LV pseudoaneurysms carry a substantial risk of both morbidity and mortality due to high risk of rupture, especially if un-diagnosed. Symptoms at presentation can be very nonspecific, but the diagnosis should be suspected in patients with recent myocardial infarction, specifically those with major vessel occlusions not amenable to stenting.

#113  Implantable cardioverter defibrillator perforates the right atrium but fails to shock arrhythmia

N Maaliki*

S Streit

A Roemer

P Staiano

KR Green

S Steinberg

V Seeram

University of Florida College of Medicine – Jacksonville, Jacksonville, FL

Case Report

A 51-year-old lady presented after a pulseless electric activity arrest and respiratory failure. She had a history of heart failure with a reduced ejection fraction of 10% with a dual-chamber implantable cardioverter-defibrillator (ICD) placed two years ago. She was found unresponsive and was admitted after ACLS and intubation, followed by resuscitation for shock. ECG showed atrial flutter with a rapid ventricular response. A mediastinal window Chest CT displayed the tip of the ICD’s right atrial lead extending through the right atrial myocardium and the anterior extra-cardiac soft tissue into the right lung middle lobe. Initially, the plan was for urgent surgical correction of the ICD displacement; however, there was no echocardiographic evidence of pericardial effusion and no radiographic evidence of a pneumothorax, suggesting a more chronic process. Device interrogation revealed only noise on the right atrial lead and an 11-minute episode of a supraventricular tachycardia of 170 BPM that was not differentiated due to atrial lead malfunction. The right ventricular lead was functioning normally but programmed to a ventricular fibrillation zone of 180 BPM. The cause of arrest could not be ascertained, with a differential diagnosis of pulseless tachycardia in the setting of undifferentiated shock that was not resuscitated by the ICD due to it being below the set threshold or due to improper sensing by the right atrial lead. She was placed on Amiodarone, but then the family elected for withdrawal of care.

ICD lead perforation rarely occurs, and the process is related to lead stiffness, increased torsion of the lead coil, and myocardial wall thinning, with the right atrium at the highest risk. Predispositions include low BMI, old age, dilated cardiomyopathy, and anticoagulant usage. Diagnosis is by imaging with chest x-ray and CT scan. Clinical manifestations can be acute with pericardial tamponade or pneumothorax, in which treatment includes urgent surgical evaluation. Chronic presentations, however, tend to be less life-threatening and include ICD lead malfunction, persistent chest pain or dyspnea, and a chronic pericardial effusion. In these instances, intervention may be more appropriate in the controlled setting. In our case, while lead malfunction may have contributed to the deterioration, urgent ICD lead repositioning in the shock state would have been detrimental and unnecessary.

#114  The misfit mitral valve

S Mikulic*

H Guo

J Rivas Rios

K Esmail

University of Florida Health at Jacksonville, Jacksonville, FL

Case Report

A 70 year old female with history of rheumatic fever (RF) with resultant surgical mitral valve replacement with bioprosthetic valve 10 years prior presented to the hospital with dyspnea and leg edema. Examination revealed a systolic and diastolic murmur 3/5 in intensity heard best at the apex. Labs significant for an elevated NT-proBNP and chest XR showed bilateral interstitial opacities. The patient was admitted to the cardiac care unit for suspected acute decompensated heart failure. Transthoracic echocardiogram revealed a preserved ejection fraction with normal diastolic function, however it also showed the bioprosthetic mitral valve seated angled toward the left ventricular outflow tract (rather than the left ventricular cavity). A transesophageal echocardiogram (TEE) was then performed and confirmed the valve oriented to the basal interventricular septum and left ventricular outflow tract. The effective regurgitant orifice (ERO) was calculated at 0.54 cm2 with a regurgitant volume of 68 ml/beat, as well as a mean transmitral gradient of 5 mmHG. These findings were consistent with severe mitral regurgitation (MR) and severe mitral stenosis (MS). Structural cardiology was consulted and the patient is currently undergoing evaluation for mitral valve replacement.

RF is the leading cause of MS. Management of such can be either medical or surgical. Individuals with long-standing MS can develop severe complications including low cardiac output, pulmonary congestion, and pulmonary hypertension. Ultimately, this warrants surgical intervention with mitral valvotomy or replacement. The most common complications of a prosthetic valve include thromboembolism, structural valve dysfunction, and endocarditis. In total, serious complications occur in about 2–3% per patient-year. Thus, it is important to recognize these early signs of valve dysfunction. In our patient, she had malposition and stenosis with resulting regurgitation of her bioprosthetic mitral valve.

Echo-TEE with 3D imaging showing a bioprosthetic mitral valve oriented to basal interventriclar septum and LVOT rather than LV cavity. Findings also significant for severe bioprosthetic valve stenosis and regurgitation.

#115  Ethical issues regarding surgical management of intravenous drug use associated endocarditis

HA Nieves Figueroa*

J Vazquez

Z Mirza

CA Cortes

VA Caribbean Healthcare System, San Juan, Puerto Rico

Case Report

Withholding of valve surgery in active, non-compliant IV drug use (IDU) patient’s is a controversial and ethical issue that calls for a rigorous multi-disciplinary assessment of the ability to comply with therapy and the willingness to undergo rehabilitation. We present a case of a 39-year-old male with medical history of IDU brought to the emergency room due to a two-day history of altered mental status with associated fever and night sweats. Physical examination revealed a new holosystolic murmur at the left lower sternal border. Laboratories were remarkable for leukocytosis. Blood cultures were positive for methicillin resistant Staphylococcus aureus. Echocardiogram showed new onset valvular disease and transesophageal echocardiogram showed a perforated aortic valve and a mobile vegetation on the posterior leaflet of the tricuspid valve. Clinical course was complicated by acute decompensated heart failure and pulmonary septic emboli. Due to rapid hemodynamic deterioration patient underwent emergent aortic valve replacement and posterior tricuspid valvectomy after which he completed 42 days of antibiotics. On follow-up outpatient visit he was found compliant with medical therapy and his drug rehabilitation program. Despite antimicrobial agents’ availability, development of advanced diagnostic methods and modern surgical interventions infective endocarditis (IE) incidence remains unchanged. We highlight the importance of a thorough physical examination and diagnostic imaging studies for IE since delay can lead to life-threatening complications such as severe valvular disease, decompensated heart failure, embolic events, sepsis and death. Close outpatient follow-up and monitoring resulted to be essential on improving prognosis and IDU relapse in this case. However, recurrence rates and non-compliance in this patient population raises concern for offering valve surgery therefore a thorough assessment of social support needs to be established for proper management.

#116  Cardiac tamponade: a fatal complication of untreated hypothyroidism

A Nieves-Ortiz1*

A Quilichini2

K Hernandez Moya1

A Rojas Figueroa1

I Rivera-Nazario1

JL Ayala Rivera1

T Rucabado-Bruno1

1Hospital Municipio de San Juan, San Juan, Puerto Rico

2Centro Cardiovascular de Puerto Rico y del Caribe, Rio Piedras

Case Report

Pericarditis is often accompanied by a pericardial effusion and can sometimes progress to cardiac tamponade. Etiologies include infectious processes, inflammatory diseases, metabolic disorders, cardiovascular diseases and idiopathic causes. Some clinical features include retrosternal chest pain, dyspnea, pericardial friction rub and characteristic ECG changes. Pericardial effusion secondary to hypothyroidism is well described; however, rapid accumulation of a large amount of fluid leading to cardiac tamponade is unusual.

A 30 year-old obese man with hypothyroidism presented to the emergency department with retrosternal chest pain. Pain was described as oppressive, worse with inspiration and improved by sitting forward. No fever, chills, illicit drug use or recent illness were reported. Hypothyroidism was diagnosed two years prior and levothyroxine was initiated but patient was non-adherent to regimen. Upon evaluation, distant heart sounds without jugular venous distention were noted on physical exam. Electrocardiogram (ECG) showed diffuse ST segment elevations and low voltages. Cardiac enzymes were negative. Echocardiogram showed a small pericardial effusion measuring 7 mm without hemodynamic compromise. After evaluation by cardiology service, patient was discharged home on high dose non-steroidal anti-inflammatory drugs (NSAIDs). Nevertheless, patient returned three days later with worsening symptoms. Patient was admitted to hospital for close monitoring and Colchicine was added to treatment regimen. Follow up echocardiogram two days later, showed progression of pericardial effusion, now measuring 2.5 cm with physiologic evidence of cardiac tamponade. Telemetry showed characteristic features of electrical alternans. Emergent pericardial window was performed by cardiothoracic surgery with release of 800 ml of serosanguinolent fluid.

Rheumatologic, infectious and malignancy workup was negative. TSH was elevated at 32.17 (n, 0.46–4.6 uIU/L) along with low free T4 of 0.1 (n, 0.78–2/19 ng/dL) and low and free T3 of 1.1 (n, 2.77–5.27pg/mL). Given these findings, pericarditis due to thyroid dysfunction was considered. Levothyroxine dose was optimized and compliance was encouraged. Echocardiogram one week later, revealed no significant re-accumulation of pericardial fluid and due to clinical improvement, patient was discharged home. Follow up with cardiologist revealed complete resolution of symptoms and normalization of thyroid function tests.

This case illustrates an uncommon etiology of a potentially fatal cardiac tamponade. Hypothyroidism leads to decreased albumin production and increased capillary permeability leading to increased interstitial fluid and pericardial fluid accumulation. It is important to consider hypothyroidism as potential cause of pericardial effusion and evaluate all patients for thyroid dysfunction. Early thyroid hormone replacement can prevent the development and progression pericardial effusion and cardiac tamponade.

#117  Arrythmias in cardiac sarcoidosis: a case report

SR Nuthulaganti*

Y Zhang

W Kogler

D Zoltowska

J Su

University of Florida College of Medicine – Jacksonville, Jacksonville, FL

Case Report

As a multi-inflammatory disorder, sarcoidosis can present with multi-organ involvement. Cardiac involvement has been associated with a worse prognosis.

Patient is a 48-year-old female with hypertension, heart failure with preserved ejection fraction, and progressive sarcoidosis who presented with chest pain and atrial flutter. She underwent CTI ablation with amiodarone for rhythm control and placement of an implantable loop recorder for frequent premature ventricular complexes. Patient presented again a few months later for chest pain and subsequent cardiac arrest following an episode of ventricular fibrillation. Echocardiography at the time demonstrated preserved ejection fraction, left heart catheterization was negative for obstructive disease. Cardiac MRI revealed diffuse, patchy late gadolinium enhancement consistent with infiltrative disease. Patient then underwent an ICD placement. During follow up one year later patients repeat echocardiogram demonstrated reduction of ejection fraction to 20%. She was then referred for cardiac transplantation.

Cardiac sarcoidosis most often presents itself with conduction abnormalities, heart failure, and sudden cardiac death. Early diagnosis of cardiac complications is vital for patient survival, with the ejection fraction as the most significant prognostic indicator. Although sarcoidosis itself is seen more commonly in women, cardiac involvement has been shown to be more prevalent in males. The cardiac complications are caused by the formation of granulomas in the endocardium, myocardium, pericardium with patchy multifocal infiltration leading to conduction delays. Cardiac MRI and PET Scan can be used as a diagnostic modality to visualize the patchy hyper-enhancement consistent with cardiac sarcoidosis. Endomyocardial biopsy has been shown to be low yield given the patchy involvement of the disease.These patients will benefit from frequent follow up and placement of implantable cardiac defibrillators to prevent sudden cardiac death.

This case aims to emphasize the importance of early involvement of an electrophysiologist. Close follow up and frequent risk assessment are pivotal for positive outcomes.

Abstract #117 Figure 1

Cardiac MRI with delayed gadolinium enhancement. Patchy enhancement consistent with cardiac sarcoidosis

#118  Elusive myocarditis unfolding as cardiogenic shock

M Oye

N Maaliki*

B Al-Turk

W Aung

University of Florida Health Science Center Jacksonville, Jacksonville, FL

Case Report

A 45-year-old male presented in cardiogenic shock. He was obtunded, hypotensive, and in respiratory distress requiring intubation. Exam revealed bilateral lung crackles and cold extremities with lower limb edema. Echocardiography demonstrated an ejection fraction of 10% with global hypokinesis and a dilated left ventricle of normal thickness. Laboratory analysis showed elevated cardiac and inflammatory biomarkers and electrocardiogram displayed a left bundle branch block with no prior for comparison. A left heart catheterization ruled out coronary artery disease. Intravenous diuretics and Dobutamine were started. Workup was significant for elevated IgG titers to 4 serotypes of Coxsackievirus A. An endomyocardial biopsy was not accessible to confirm the presumed diagnosis of acute myocarditis. Empiric treatment was started with intravenous immunoglobulins (IVIG) and high-dose steroids. Over the ensuing weeks, he was weaned from inotropic support and guideline-directed medical therapy (GDMT) was introduced. Of note, he had suffered from fevers, malaise, and progressive dyspnea for the prior two weeks, which was attributed to COPD exacerbations.

Acute myocarditis can manifest as a cardiovascular disease within three months. Etiology is commonly viral, involving direct cytotoxic damage and antigen recognition inducing an inflammatory response. Symptoms can range from a subclinical presentation to acute heart failure. Laboratory analysis reveals inflammatory and cardiac biomarker elevation. TTE can demonstrate systolic dysfunction and a dilated left ventricle of normal wall thickness. The diagnostic gold standard is an endomyocardial biopsy, which is often not performed due to lack of access to an experienced interventionalist and the invasive nature of the procedure. Cardiac MRI has emerged as a reliable modality. Treatment includes diuresis and hemodynamic support. Immunosuppression has been a contested option and results have varied for myocarditis with positive viral serologies. IVIG has shown promise due to the antiviral and immunomodulatory effects with relatively low risk, but more studies are required.

#119  A case of wide complex tachycardia due to supraventricular tachycardia with accessory pathway

K Parmar*

T Nguyen

G Del Rio-Pertuz

E Elgwairi

M Abohelwa

D Swaminath

Texas Tech University Health Sciences Center, Lubbock, TX

Case Report

Wolff-Parkinson-White syndrome (WPW) is rare, with a prevalence of 0.07 percent. Up to 80% were atrioventricular reentrant tachycardia (AVRT),15–30% were atrial fibrillation (AF) and 5% were atrial flutter.

Case presentation

A 16-year-old man with history of asthma presented to hospital for dizziness and palpitations that started from past one day after he played basketball. It was followed by multiple dizziness episodes throughout the night. Patient had family history of a cousin who passed away at the age of 26 years. Vitals were stable except heart rate 186 beats/min. Laboratory showed elevated troponin with flat trend. Electrocardiogram (EKG) showed irregular wide complex tachycardia concerning for either AF with WPW or ventricular tachycardia (figure 1). He was successfully cardioverted with 360J with reversion to sinus rhythm. Repeat EKG showed sinus bradycardia with delta waves suggestive of WPW. Transthoracic echocardiogram showed normal left ventricle ejection fraction, mild concentric left ventricular hypertrophy, not consistent with hypertrophic cardiomyopathy. Patient underwent successful ablation of the right posterior accessory pathway. On follow up, one month later patient denied dizziness or any other complaints.

Abstract #119 Figure 1

A- Electrocardiography (EKG) of the patient on arrival (left) demonstrating irregular wide complex tachycardia. B-EKG of the patient after cardioversion (right) demonstrating sinus bradycardia

Discussion

This case highlights the importance of cardioversion in patients with AF with WPW. The rate-slowing drugs for atrial fibrillation are not effective, and digoxin and the non-dihydropyridine calcium channel blockers are contraindicated since blocking the AV node will promote conduction down the accessory pathway and may sometimes directly enhance the rate of conduction over the accessory pathway. Drugs that prolong the refractory period of the accessory connection such as procainamide or ibutilide are preferred if cardioversion is not possible in stable patients.

#120  Cardiac tumor presenting as chest pain: a rare presentation

K Parmar*

K Ratheal

K Nugent

G Del Rio-Pertuz

E Elgwairi

M Abohelwa

P Sethi

Texas Tech University Health Sciences Center, Lubbock, TX

Case Report

Primary cardiac tumors are rare found in only 0.02% of autopsies and most are non-malignant (1). Fibroelastomas are the most common valvular tumor with variable clinical presentation.

Case presentation

A 73-year-old man with past medical history of hypertension and hyperlipidemia presented with complaint of chest pain at rest which was sudden onset, substernal, radiating to the left arm and neck, associated with shortness of breath and diaphoresis. Physical examination was unremarkable. Vitals were stable. Electrocardiography and chest-xray were unremarkable. Troponin was normal. A left heart catheterization showed 50% ostial focal stenosis at first obtuse marginal branch of left circumflex and was otherwise normal. An echocardiogram showed normal systolic function without regional wall motion abnormalities. A 1 cm X 0.7 cm lesion seen attached on the left cusp of the aortic valve on the aortic side which appeared to have a stalk (figure 1). The mass was intermittently occluding the left main coronary artery. A TEE was done which showed the well circumscribed nodule measuring 0.8 X 0.7 cm on the left coronary cusp with a stalk. Patient was discharged and asked to follow up in 6–8 weeks, but he was lost to follow up.

Discussion

Papillary fibroelastoma (PFE) range from 2 mm to 40 mm and are usually pedunculated (2). They can arise from the aortic or ventricular surface of the valves. Given the location of the tumor on the valve and echocardiographic features it was suspected that the patient had papillary fibroelastoma. However, a pathologic diagnosis could not be confirmed. Most common presentation is stroke or transient ischemic attack, and other times these are coincidental findings on echocardiography(3).

Abstract #120 Figure 1

Transthoracic echocardiography (TTE) showing possible tumor in systole and diastole (pointed by arrows). It is 1XO.7 cm echogenic lesion attached on the aortic valve on the aortic side. Vegetation and thrombus cannot be excluded

Conclusion

Cardiac tumors are extremely rare malignancies, and this patient presented with an unusual hemodynamic complication, namely occlusion of the coronary artery.

#121  A case of gastrointestinal bleeding and aortic stenosis: heyde syndrome

W Rasheed1*

M Diab1

B Mohanakrishnan1

R Nambiar2

1Texas Tech University Health Sciences Center Amarillo, Amarillo, TX

2Amarillo Heart Group, Amarillo, TX

Case Report

Heyde syndrome is a triad of aortic stenosis, gastrointestinal (GI) bleeding from angiodysplasia, and acquired coagulopathy from relative von Willebrand (VW) factor deficiency (1). It is a rare disease that can be fatal if not promptly recognized and treated (2).

We present a case of a 73-year-old male with a history of severe aortic stenosis who presented with chest pain secondary to severe symptomatic anemia. No source of bleeding was found as he denied any other symptoms however fecal occult blood test was positive. Provided his history of aortic stenosis, Heyde syndrome was suspected and GI bleed workup was started promptly.

Esophagogastroduodenoscopy (EGD) revealed gastric angiodysplasia which was successfully treated with clipping. The patient did well after the procedure and hemoglobin stabilized. Echocardiography revealed aortic valve jet velocity 5.12 m/s consistent with severe aortic stenosis. The diagnosis of Heyde Syndrome was made and he was referred for aortic valve replacement, which is the first-line treatment for Heyde syndrome (2).

Heyde syndrome is a rare but potentially fatal disease and the diagnosis requires a high index of suspicion (1). Acquired VW factor deficiency and GI angiodysplasia are believed to result from shear stress forces of the stenotic aortic valve over large VW factor multimers, which results in cleavage of large VW factor multimers into smaller fragments. This interferes with inhibition of angiogenesis resulting in angiodysplasias. As VW factor normally carries and prolongs the half-life of coagulation factor VIII, this also reduces the half-life of factor VIII resulting in coagulopathy (3 4). Early diagnosis and treatment are very important as Heyde syndrome carries an excellent prognosis following aortic valve replacement (2).

Conclusion

The abstract emphasizes the importance of ruling out Heyde syndrome in elderly patients presenting with anemia, especially with a history of aortic stenosis.

References

1. Chukwudum, Chidinma A, Saul Vera, Munish Sharma, Joseph Varon, and Salim Surani. ‘Heyde Syndrome: A Case Report and Literature Review.’ Cureus 12, no. 4 (n.d.): e7896. https://doi.org/10.7759/cureus.7896.

2. Theis, Samuel R., and Sherry D. Turner. ‘Heyde Syndrome.’ In StatPearls. Treasure Island (FL): StatPearls Publishing, 2021. http://www.ncbi.nlm.nih.gov/books/NBK551625/.

3. Crawley, James T. B., Rens de Groot, Yaozu Xiang, Brenda M. Luken, and David A. Lane. ‘Unraveling the Scissile Bond: How ADAMTS13 Recognizes and Cleaves von Willebrand Factor.’ Blood 118, no. 12 (September 22, 2011): 3212–21. https://doi.org/10.1182/blood-2011-02-306597.

4. Hudzik, Bartosz, Krzysztof Wilczek, and Mariusz Gasior. ‘Heyde Syndrome: Gastrointestinal Bleeding and Aortic Stenosis.’ CMAJ : Canadian Medical Association Journal 188, no. 2 (February 2, 2016): 135–38. https://doi.org/10.1503/cmaj.150194.

#122  Heparin-induced thrombocytopenia presenting with multiple thromboembolic disorder after coronary artery bypass graft surgery

SA Tanami*

N Ray

S Tasnim

MA Tanbir

A Ray

T Naguib

Texas Tech University Health Sciences Center School of Medicine, Lubbock, TX

Case Report

Heparin-induced thrombocytopenia (HIT) with thrombosis after coronary artery bypass graft (CABG) is a rare but serious side effect.

A 64-year-old female with essential hypertension presented to the emergency department with progressive dyspnea at rest, orthopnea, and paroxysmal nocturnal dyspnea after being discharged to home two weeks earlier following three-vessel CABG without any clinical features suggestive of HIT.

An initial evaluation in the emergency department revealed a low platelet count (66,000 mm-3), elevated D-dimer (>20), elevated brain-type natriuretic peptide (1167 pg/mL), and elevated troponin (0.52 ng/mL). Computed tomography angiography demonstrated significant thrombus in the right main pulmonary artery extending into the right upper lobe artery with a moderate to large pulmonary infarction in the right upper lobe. There were also small amounts of thrombus seen within the right lower lobe and trace thrombus seen in the left lower lobe. There was also appreciable cardiomegaly with dilatation of the left ventricle, and findings were compatible with recent CABG. There was no evidence of deep vein thrombosis in the pelvis or thighs. The patient was initiated on high-dose heparin infusion with transitioning to newer anticoagulant therapy for her pulmonary embolism.

The next day, the patient developed sudden onset weakness of her left upper extremity. Magnetic resonance imaging without contrast of the brain showed foci of multiple right cerebral hemisphere embolic infarcts. Transthoracic echocardiography showed markedly reduced left ventricular systolic function with apical hypokinesis and an apical thrombus of 2 cm. Transesophageal echocardiography showed multiple thrombi in the right atrium and a large left ventricular apical thrombus. HIT serology with an optical density of 2.452 and serotonin release assay 100% was consistent with HIT. Heparin was immediately discontinued, and the patient was started on argatroban. The patient was transitioned to apixaban 5 mg twice daily, and thrombocytopenia recovered completely with a platelet count of 220,000 mm-3 within one week.

Extensive arterial and venous thromboembolism is most likely due to heparin use during CABG. HIT should be suspected in any patient presenting with arterial or venous thromboembolic disorders after recent heparin therapy. Even though the heparin exposure occurred more than two weeks before presentation in this case, HIT should be considered and ruled out before initiating heparin therapy.

#123  Tachycardia-induced cardiomyopathy in a patient with newly diagnosed acute myeloid leukemia

J Rios*

G Del Rio-Pertuz

K Parmar

D Davis

K Nugent

Texas Tech University Health Sciences Center, Lubbock, TX

Case Report

Congestive heart failure (CHF) is a rare complication of acute myeloid leukemia (AML) and typically presents after receiving chemotherapy. There are several reasons for which CHF may occur, including tachycardia-induced cardiomyopathy caused by a persistent supraventricular or ventricular tachy-arrhythmia. There are little data on CHF as a presenting feature of AML. We present a case in which a previously healthy patient presented to the emergency department with leukocytosis and concomitant CHF symptoms that resolved after leukapheresis and symptomatic therapy, suggesting tachycardia-induced cardiomyopathy.

Case

A 26-year-old man presented to the emergency department complaining of progressive anasarca, testicular swelling, petechial rash, diaphoresis, fever, and weight loss for the past 4 weeks. First assessment showed vital signs and labs of HR: 140 bpm, BP: 116/71 mmHg, RR: 22 rpm, O2 Sat: 100% on room air, Temp: 101.9 °F, Hb: 11.2 g/dl, WBC: 79 k/µL, PLT: 22 k/µL, and uric acid: 10.3 mg/dl. Peripheral blood smear was remarkable for numerous blasts. Electrocardiogram showed sinus tachycardia. Transthoracic echocardiogram (TTE) showed a left ventricular ejection fraction (LVEF) of 30–35% with moderate global hypokinesis of all myocardial segments. Left heart catheterization ruled out ischemic coronary artery disease. While awaiting bone marrow biopsy results, the patient was started on leukapheresis and hydroxyurea for cytoreduction to achieve WBC <50k/µL. Bone marrow biopsy confirmed diagnosis of acute myeloid leukemia, and the patient was started on 7 days of cytarabine and 3 days of daunorubicin with furosemide as needed. After two weeks, as the patient was clinically improving, a repeat TTE showed a LVEF of 60–65%.

Discussion

In tachycardia-induced cardiomyopathy, noninvasive imaging techniques, such as echocardiography, usually show left or right ventricular dilatation and systolic dysfunction. Acute leukemias are characterized by the proliferation of immature, nonfunctional white blood cells known as blasts in the bone marrow. One of the best-known features of tachycardia-induced cardiomyopathy is that systolic dysfunction may be improved and completely resolve after controlling the arrhythmia. In this case, it is possible that the patient was in a blast crisis that caused hyperviscosity leading to tachycardia and acute heart failure with reduced ejection fraction. Physicians should be aware of this possible scenario, and should monitor cardiac status during acute interventions.

#124  Tackling a deadly duo: a case of thrombotic thrombocytopenic purpura causing heart failure and stroke

T Sharma*

L Abusalem

H Salah

E Askew

A Kumar

MS Cross

University of Arkansas System, Little Rock, AR

Case Report

Thrombotic thrombocytopenic purpura(TTP) is a thrombotic microangiopathy with a 19% mortality despite optimum medical management. Heart failure due to TTP is rare and doubles the mortality rate. We report a case of a 51-year-old female with past medical history of hypertension who presented with right sided headache, left arm weakness and bilateral pedal edema for two days. On examination, vital signs were normal except tachycardia. Neurological exam was significant for mildly decreased grip strength of left hand. 2+ pitting edema was noted bilaterally up to mid-shin. Cardiovascular examination was otherwise normal. Significant laboratory test results and trends are noted in table 1. Computed tomography scan of her brain showed right parietal, frontal and cerebellar infarcts. No evidence of constrictions concerning for vasculitis or large vessel obstruction noted on magnetic resonance angiography of head and neck. Electrocardiogram showed no acute ischemic changes and echocardiogram showed an ejection fraction reduced to 35% without any regional wall motion abnormalities. Myocardial perfusion SPECT did not show evidence of reversible ischemia. In light of worsening anemia and thrombocytopenia, peripheral smear was repeated and demonstrated dacrocytosis and red cell fragmentation. In presence of normal coagulation studies, our suspicion for TTP was high and upon testing, ADAMTS13 activity was remarkably decreased at 8%. No secondary cause of TTP was found and TTP specific therapy was initiated including plasma exchange, Rituximab and Caplacizumab. Patient was discharged after stabilization of platelet count. At subsequent follow up, she reported ongoing symptoms of heart failure and guideline directed medical therapy was optimized with a plan to repeat echocardiogram in three months.

Systolic dysfunction in TTP was initially reported in case reports with evidence of microthrombi in coronary vasculature noted on autopsy. Despite high mortality, a lot is not known about the natural history and pathophysiology of heart failure in TTP. Further investigation into optimum management, especially the role of newer TTP specific therapies in reducing the duration and severity of heart failure could help improve outcomes in this population. In our patient, early recognition of TTP allowed aggressive management with immunomodulators and plasma exchange which proved to be lifesaving.

Abstract #124 Table 1

Trend of significant laboratory values

#125  Global vasospasm treated with simultaneous use of anti-hypertensive and vasopressor

MA Tanbir*

L Bellamkonda

D Brabham

SA Tanami

Texas Tech University Health Sciences Center, Amarillo, TX

Case Report

A Rare Presentation of Multivessel Vasospastic Angina in the Setting of Septic Shock

Background

Prinzmetal or vasospastic angina is an unusual but important consideration when evaluating a patient with chest pain. Unlikely acute coronary syndromes (ACS) which primarily occur as a result of coronary artery occlusion, prinzmetal angina occurs angina occurs mainly as a result of coronary artery vasospasm. We present the unusual case of a patient who suffered cardiac arrest and was found to have >90% occlusion in multiple coronary arteries on a left heart catheterization (LHC) performed within 60 minutes.

Case presentation

Patient is a 70-year-old female who was initially being treated in the hospital for covid-19. She spent a few days in the ICU due to requiring high flow nasal cannula but was transferred to the floor after she was weaned down to 3L/min via regular nasal cannula. On day of arrest, patient had increasing oxygen requirements and was on ventimask immediately prior to the code blue. Patient received 2 rounds of CPR and her initial rhythm was found to be ventricular fibrillation. Pt was defibrillated and ROSC was immediately achieved. EKG showed ST elevations in inferior leads. Patient was, however, alert and oriented x3 on initial evaluation by critical care team. She was not intubated after the arrest. She was transferred to the intensive care unit, given 300 mg intravenous amiodarone and therapeutic dose lovenox. On LHC, her left anterior descending artery (mid/distal portion), distal diagonal vessel, left circumflex artery (mid portion), distal portion of the obtuse marginal and right coronary artery were found to be severely spasmodic. Patient had recurrence of angina after the catheterization which was transiently relieved with nitro. Patient had sustained relief of angina after starting nitro drip. Patient was also started on amiodarone drip upon transfer back to the ICU.

Discussion

The obvious side-effect of our therapeutic treatment was hypotension that was initially responsive to intravenous fluids. Patient, however, became hypoxic a few hours later and needed to be diuresed to return to baseline oxygen requirement. Patient was then started on norepinephrine drip with goal to maintain mean arterial pressure above 65. After patient was loaded with amiodarone, nitro drip was discontinued. She was then transitioned to oral amiodarone. She was started on isosorbide dinitrate prior discontinuing nitro drip. Patient’s blood pressure stabilized as her per oral intake improved and norepinephrine drip soon thereafter. Novel presentations require novel treatment and creative thinking lead to the decision to continue nitro drip to keep her stable, even if it meant the simultaneous use of an anti-hypertensive and a pressor. It is possible that covid-19 served as a trigger for such a global vasospasm event. Patient was restarted on her home medication of long-acting nitrates which were held on admission due to hypotension.

Endocrinology and metabolism

Joint plenary poster session and reception

4:30 PM

Thursday, February 10, 2022
#127  A case of post-roux-en-y gastric bypass (rygb) hypogylcemia associated with adrenal insufficiency

H Aljumaili*

S Usala

Texas Tech University Health Sciences Center, Amarillo, TX

Case Report

Post-RYGB hypoglycemia has become increasingly recognized, as either noninsulinoma pancreatogeneous hypoglycemia syndrome(NIPHS) or late dumping syndrome

Post-RYGB hyperinsulinemic hypoglycemia can be diagnosed with the appropriate clinical criteria: symptoms developing ≥1 year after RYGB and occurrence of hypoglycemia with autonomic and/or neuroglycopenic symptoms after a high carbohydrate mixed meal. In contrast to hyperinsulinemic hypoglycemia (NIPHS/late dumping syndrome), adrenal insufficiency has been reported only rarely post-RYGB. We present a case further documenting the natural history and treatment of adrenal insufficiency in what first appeared to be hyperinsulinemic hypoglycemia. A 52-year-old female with BMI 40 and Type 2 DM underwent a RYGB procedure; six years later she developed hypoglycemic symptoms without neuroglycopenia that occurred mainly after meals. She would record blood sugars as low as 57 by glucometer when she felt shaky, sweaty, and weak. She was initially diagnosed with NIPHS and treated with diet therapy and acarbose, but her symptoms and documented hypoglycemia did not improve. Of note, she had had a Cortrosyn stimulation test two years earlier with 30 min and 60 min cortisol levels of 21.9 mcg/dl and 26.1 mcg/dl, respectively, indicating normal adrenal function at that time when she was asymptomatic in terms of hypoglycemic symptoms. However, because the patient had developed new symptoms of hypoglycemia and increased fatigue, which did not improve with standard therapy for NIPHS, a Cortrosyn test was repeated; hypoadrenalism was found with 30 min and 60 min stimulated cortisol levels of 14.5 mcg/dl and 15.9 mcg/dl, respectively. The diagnosis of hypoadrenalism was confirmed by a third Cortrosyn test six weeks later, with cortisol levels at 30 min and 60 min of 14.4 mcg/dl and 17.0 mcg/dl, respectively. Treatment with hydrocortisone 10 mg twice daily was initiated, and the patient had a significant clinical improvement; she reported more alertness and much more energy with only occasional episodes of hypoglycemic symptoms. NIPHS is increasingly recognized as a problem following gastric bypass surgery. It is a known complication of RYGB and can occur months to years after the procedure. NIPHS is diagnosed in approximately 10 – 15% of individuals who have undergone gastric bypass surgery, and it is more common in women. Adrenal insufficiency associated with NIPHS has not been extensively investigated. A proposed mechanism for hypoadrenalism in NIPHS has been prolonged hypothalamic exposure to severe hypoglycemia resulting in some form of resistance syndrome and secondary hypoadrenalism. Clinicians should be aware of the possible co-existence of hypoadrenalism with NIPHS and the advisability of a Cortrosyn test in this context

#128  Euglycemic diabetic ketoacidosis after the first dose of empagliflozin in acutely-ill patient

J Benjanuwattra

M Abdelnabi

N Eshak

E Elgwairi

MA Elmassry

B Songtanin

BL Mora*

PC Aristimuno

Texas Tech University Health Sciences Center, Lubbock, TX

Introduction

A sodium-glucose cotransporter 2 inhibitor (SGLT2i), empagliflozin, was recently approved for add-on treatment of type II diabetes mellitus (DM) due to its proven cardiovascular benefit. Euglycemic diabetic ketoacidosis (DKA) is a rare life-threatening complication associated with empagliflozin. Glucosuria and subsequent osmotic diuresis, leading to a state of dehydration and decreased insulin production, are the proposed underlying pathogenesis. An imbalance between glucagon and insulin then promotes lipolysis and ketogenesis.

Case presentation

A 32-year-old female patient with morbid obesity was admitted to our facility with tibial plateau abscess and infected hardware which was placed after traumatic tibial plateau fracture three years earlier. She underwent debridement and hardware removal. A new diagnosis of diabetes mellitus was made due to hemoglobin A1c of 10.2%. None of the diabetic symptoms were reported. Random plasma glucose was 263 mg/dL. Insulin therapy was offered, but patient refused. Initially, she was commenced on sitagliptin and empagliflozin. The following day, sitagliptin was withheld as plasma glucose responded well and she was encouraged to lose weight and lifestyle modification. Only empagliflozin was continued due to its weight loss advantage. Unfortunately, she rapidly developed severe euglycemic DKA.

Laboratory investigation revealed leukocytosis (13,250 cells/µL) and thrombocytosis (platelet 523,000/µL). A combination of high anion gap (AG) and normal AG metabolic acidosis was noted as evidenced by serum bicarbonate 5 mmol/L, elevated serum acetone, AG 23, and delta ratio 0.6. Urinalysis revealed ketonuria, and massive glycosuria (>500 mg/dL). Plasma glucose was 120 mg/dL. Empagliflozin was discontinued and treatment for DKA with aggressive fluid replacement and regular insulin drip was initiated. Resolution of DKA was achieved after three days and bridging therapy was successful.

Conclusion

Euglycemic DKA can develop early after initiating the first dose empagliflozin. The administration of SGLT2i in acutely-ill patient is associated with higher risk of this complication; therefore, close monitoring is mandatory. Switching to insulin-based therapy may be appropriate in patients, who chronically taking SGLT2i, with acute illness requiring hospitalization. To date, whether discontinuation of dipeptidyl peptidase 4 inhibitor, which also regulates insulin/glucagon balance, in patients taking SGLT2i contributes to DKA remains unknown.

#129  Acanthosis nigricans and hypoglycemia: a rare case of insulinoma presenting with hyperandrogenism characteristics

M Delgado-Lopez*

Hospital Municipio de San Juan, San Juan, Puerto Rico

Case Report

Insulinomas are pancreatic endocrine tumors most commonly identified at the median age of 51–55 years. Hypersecretion of insulin is characteristically accompanied by neuroglycopenia symptoms and a catecholaminergic response. We present a rare case of a 33 y/o obese female patient with a past medical history of abnormal uterine bleeding until last year that begun having hypoglycemic episodes, as well as irregular menstruation. She mentioned that since 2019, there have been symptoms such as handshake, forehead headache, and fingertip tingling, which are always resolved through food and juice ingestion. This year patient encountered two episodes of altered mental status (loss of consciousness and nonsense speech) witnessed by her husband on two occasions. Both of them were always early in the morning. The patient was taken to the urgency room with dextrose at 29 mg/dl. Denies any current medication. Physical examination was remarkable for obesity, facial acne, hirsutism, and acanthosis nigricans on the neck, armpits, and lower abdomen. Treatment with D5W, continuous infusion, and Diazoxide started, while workup for hypoglycemia was available. Hepatitis panel non-reactive, Insulin antibodies less than 5.0 (negative), Beta-hydroxybutyric acid 0.6 mmol/L, Sulfonylurea screen negative, IGF-1 150 pmol/L, growth hormone 1.13ng/mL, high C-peptide (4.2 nmol/L) and high insulin (58 microU/mL) levels found. Abdomino-pelvic MRI was performed with the finding of a 1.6 cm x 1.10 cm x 1.3 cm lesion on the posterior aspect of the pancreatic body tail. Surgery was performed with pathology consistent with insulinoma. After surgery, hypoglycemic episodes resolved and insulin therapy started due to persistent hyperglycemia. It is curious to have signs and symptoms of hyperandrogenism in patients with insulinomas. Only four cases have been reported previously and only one case mentioned the characteristics of the patient with hirsutism, acne, and acanthosis nigricans.

#130  The challenge of corticosteroid taper in a patient with fungal infection

D Katwal*

N Pant

S Aziz

H Steinberg

The University of Tennessee Health Science Center College of Medicine, Memphis, TN

Introduction

High-dose corticosteroids, used for several diseases, induce iatrogenic adrenal insufficiency (AI) after prolonged exposure. Corticosteroids at varying doses have been used as the first line of treatment for sarcoidosis based on expert consensus. There are very few large randomized controlled trials with long-term follow-up. We present a challenging case of sarcoidosis requiring rapid tapering of prednisone while avoiding AI.

Case Report

64-year-old African American female with status post liver and kidney transplant for ESRD and liver cirrhosis secondary to sarcoidosis on immunosuppressive therapy presented to the hospital for progressive worsening of legs swelling and shortness of breath for two months. She was on 40 mg prednisone daily for a recent diagnosis of biopsy-proven acute tubulointerstitial nephritis. Endocrinology consultation was requested to assist with steroid tapering. On exam, she was afebrile, tachycardic at 107/min, fine crackles at lung bases, and 3+ pitting edema on lower extremities. Laboratory results were remarkable for hemoglobin 9.9 g/dl, WBC 1.9 K/uL, platelets 74 K/uL, potassium 2.4 mmol/L, carbon dioxide 12 mmol/L, corrected calcium 8.5 g/dl, and creatinine 2.95 mg/dl. Transthoracic echocardiogram was only significant for mild pulmonary hypertension (37 mmHg). Despite aggressive medical therapy, her renal function continued to worsen, requiring the initiation of hemodialysis. Her hospital course was complicated by the development of pancytopenia, oral thrush, and oral ulcers. This led to a bone marrow biopsy that showed granulomas, consistent with sarcoid involvement of the bone marrow. Further workup revealed a CD4 count of 7, positive CMV, and a negative HIV test. She was subsequently started on valganciclovir. Blood cultures grew Histoplasma, and Amphotericin was started and later transitioned to itraconazole. Due to the severe immunocompromised status, the decision was made to taper steroids to aid recovery from disseminated histoplasmosis. Therefore, prednisone was tapered to 10 mg once daily over ten days. She tolerated taper and was advised to reduce the prednisone by 1 mg each week upon discharge.

Discussion

Steroid taper can be challenging as there is a lack of evidence regarding standardized corticosteroids dose, tapering regimen, and eventual discontinuation. Literature suggests that for patients on a daily dose of prednisone between 20–40 mg, an initial rapid taper of up to 5–10 mg weekly can be done until 20 mg daily is reached. This is followed by a slow taper of weekly to monthly decreases at 1–2.5 mg to avoid glucocorticoid withdrawal syndrome. However, there is no guidance for steroid taper in terms of a life-threatening infection where a faster reduction is desirable; our case shows that a faster taper can be done without compromising safety, hopefully aiding a quick recovery from a potentially life-threatening infection.

#131  Two coexisting causes of severe hypercalcemia a rare case of severe hypercalcemia caused by multiple myeloma and primary hyperparathyroidism

Z Liu1*

L Amini2

J Lequerica2

H Steinberg1

1The University of Tennessee Health Science Center College of Medicine, Memphis, TN

2Methodist Le Bonheur Healthcare, Memphis, TN

Introduction

Hypercalcemia is a relatively common clinical problem. Among all its causes, primary hyperparathyroidism (PHPT) and malignancy are the most common ones, accounting for >90% of cases. However, the coexistence of the two pathological processes together in one patient is very rare. Here, we present a case with this rare phenomenon as a reminder to search for concomitant causes of hypercalcemia in an elderly population.

Case Report

A 73 year old female with a past medical history of hypertension, hyperlipidemia, and recently diagnosed renal failure presented to the hospital due to one-week symptoms of worsening fatigue, nausea and abdominal pain. She was taking amlodipine, ostercal-D, atorvastatin, aspirin, and omeprazole before admission. Review of systems was positive for fatigue, abdominal pain, nausea, and back pain. On physical exam, abdomen was mildly and diffusely tender to palpate, trace bilateral lower extremity edema. Laboratory results were remarkable for Calcium (Ca)13.7 mg/dL, albumin 3.2 g/dL, Creatinine 3.27 mg/dL, total protein 11.5 g/dL, Phosphorus 2.4 mg/dL, intact parathyroid hormone (PTH) 39.3 pg/mL, 25 hydroxy-vitamin D 18.8 ng/mL, hemoglobin 8.8 g/dL. She was treated with intravenous palmidronate 60 mg, calcitonin 4 units/kg every 12 hours for 4 doses, and fluid hydration. DEXA scan was normal a month prior. Further laboratory evaluation also showed elevated Immunoglobulin A (IgA), with monoclonal protein IgA kappa type with free kappa light chains and elevated IgA kappa in the urine. Skeletal survey showed multiple small focal lytic bone lesions involving the skull. A bone marrow biopsy was markedly hyper-cellular with sheets of kappa restricted plasma cells replacing normal hematopoiesis. She was diagnosed with stage 3 IgA kappa multiple myeloma (MM) and was started on chemotherapy as well as on Cinacalcet 30 mg twice a day for PHPT. Her Ca level normalized to 9.6 mg/dL before discharge with symptoms resolved.

Discussion

While prevalence of PHPT and MM increases with age, increasing the chance of both occurring together in one patient, this is very rare with only a few cases reported in the literature. Moreover, one study suggested that the prevalence of monoclonal gammopathies is higher than expected in patients with surgically proven PHPT. The mechanism is unclear, but it is hypothesized that the elevated PTH may mediate the induction of MM through the downstream biological effects of IL-6. In our case, with profound hypercalcemia, renal failure, and MM, intact parathyroid hormone was not sufficiently suppressed which is consistent with underlying PHPT being at least partially responsible for the high Ca. To conclude, our case demonstrates that two pathological processes causing hypercalcemia do, in fact, sometimes coexist; understanding of the underlying pathologies will help target the appropriate therapeutic approach.

#132  Cyclic hyperthyroidism in nodular thyroid disease

B Mohanakrishnan1*

KG Holder2

R Waqas1

H Werner1

1Texas Tech University Health Sciences Center, Amarillo, TX

2Texas Tech University Health Sciences Center School of Medicine, Amarillo, TX

Learning Objective

To interpret the TSH, fT3/T4 and correlate with symptoms in Cyclic Hyperthyroidism.

Case presentation

A 39-year-old healthy woman presented with anxiety, hair thinning, weight loss, and appetite suppression since her pregnancy in September of 2019. One month after giving birth, she began experiencing occasional hot flashes but denied any other symptoms. In November 2019, she was suspected of hyperthyroidism due to worsening of her initial symptoms, and her preliminary thyroid profile showed a TSH of 0.006 uIU/ml and an fT4 of 1.2 ng/dl. She was referred to an endocrinologist for further care, and her subsequent thyroid profile with symptoms variations can be found in the table. The follow-up clinical and laboratory data interpreted that the patient had cyclic features of hyperthyroidism with sporadic intervals of asymptomatic periods without any medical treatment. I-123 uptake displayed a euthyroid state with uptake in the normal range at 4 and 24 hours. On the scan, multiple nodules, both hot and cold, were observed throughout the gland. This pattern is consistent with multinodular goiter and the possibility of clinical hyperthyroidism from August to October 2020. I131 thyroid ablation was performed in August 2021, and three weeks follow-up revealed that the patient was asymptomatic with a significantly suppressed TSH and a normal range of fT3 1.4 pg/ml and fT4 0.6 ng/dl. All labs were performed in the Texas Tech laboratory.

Discussion

Patient with symptoms of thyrotoxicosis and significantly suppressed TSH with unelevated free T4 immediately makes us think of T3-toxicosis; knowing the very short half-life of T3, the 6–7-day half-life of T4, and the 2–3 months half-life for TSH can explain why the patient‘s thyroid function tests can be confounding in Cyclic Hyperthyroidism compared to the patient‘s clinical picture. A literature review revealed that Cyclic Cushing Syndrome, another cyclic glandular disease affecting the Adrenals with similar hormonal cyclic patterns, has unclear pathophysiology. In this case, the likely explanation for these Cyclic Thyrotoxicosis features may be explained by the inconsistent hormonal secretions from hot nodules. The hot nodules generally overproduce triiodothyronine (T3) relative to T4, causing T3-toxicosis. As the nodules die off and become ’cold’ nodules, free T3/T4 returns to the normal reference range with symptom tenacity in spite of TSH remaining suppressed. By our review of the literature, this is a new phenomenon, and no similar cases of Cyclic Hyperthyroidism have been reported.

Conclusion

Diagnosing a Cyclic Hyperthyroidism depends on thyroid function tests that may exhibit cycling TSH, fT3, and fT4 levels compared to the patient‘s symptoms. T3 toxicosis may likely correspond to the phase of the hot nodular activity and can be confirmed by elevated fT3. Close follow-up is essential for monitoring patient symptoms and the thyroid function test until definite diagnosis.*

#133  A case of pheochromocytoma in a 29-year-old woman with non-classical presentation and neoplastic pathological features

N Mon*

S Usala

Texas Tech University Health Sciences Center School of Medicine Amarillo, Amarillo, TX

Case Report

A 29-year-old woman presented to the ER with a complaint of severe constipation associated with abdominal and back pain. An abdominal CT with contrast revealed an enhancing left adrenal mass, 7.9 x 7.3 x 5.4 cm. An adrenal core and FNA biopsy showed small clusters of modestly pleomorphic cells with evidence of neuroendocrine differentiation in a broadly fibrotic background which were strongly and diffusely positive for chromogranin A, consistent with native adrenal medullary cells vs pheochromocytoma. There was hemodynamic instability following the biopsy. Initial hormonal data was consistent with a pheochromocytoma: urinary metanephrines 10,836 mcg/24 hr (94–604), urinary epinephrine <5 mcg/24 hr, urinary norepinephrine 966 mcg/24 hr (15–100), total urinary epinephrine and norepinephrine 966 mcg/24 hr (26–121), urinary dopamine 422 mcg/24 hr (52–480).

She was referred to the endocrine clinic for further evaluation and preoperative treatment. Her chief complaints were forgetfulness and ‘sleeping and eating’ a lot. She did not report headaches as a problem, but did admit to headaches, sweating and hot flashes at times. She denied any spells. There was no family history of hypercalcemia, parathyroid tumor, pituitary tumor or thyroid cancer. Initially she demonstrated orthostatic changes with HR 66, SBP 128 sitting and HR 102, SBP 112 standing. The labs via the endocrine clinic revealed plasma metanephrine <25pg/ml(<57), plasma normetanephrine 2,469pg/ml(<148), aldosterone/renin ratio 25ng/dl/2.79ng/ml/hr = 9.0. TSH, PTH and calcitonin were normal. She was treated preoperatively with doxazosin until the orthostatic changes in BP resolved, then atenolol was added. She later underwent a left adrenalectomy with uneventful recovery. Pathological diagnosis was consistent with a pheochromocytoma of a ki-67 proliferation index of 5–10% and moderate to focally marked cytologic atypia comprising a Pheochromocytoma of the Adrenal Gland Scaled Score (PASS), which was concerning for high malignancy risk. Given her young age and the neoplastic features on pathology, genetic testing is indicated in this case.

Pheochromocytoma and paraganglioma are rare catecholamine secreting tumors arising from chromaffin cells of the adrenal medulla and the sympathetic ganglia, respectively. It classically presents with episodic headache, sweating, and hypertension. Up to 25% were discovered during imaging for unrelated disorders and 75% after symptoms development. We report an unusual presentation of a norepinephrine secreting pheochromocytoma with neoplastic pathology in a young woman. In addition, although phenoxybenzamine is often touted as the means to alpha receptor blockade preoperatively, it is non-specific in contrast to doxazosin and it has become unaffordable. Doxazosin was quite effective in expanding vascular volume and preparing the patient for surgery.

#134  Primary hyperparathyroidism in a pregnant patient

N Pant*

A Eapen

D James

The University of Tennessee Health Science Center College of Medicine, Memphis, TN

Case Report

Primary hyperparathyroidism in pregnancy is very rare. Acute hypercalcemia in the setting of pregnancy has limited treatment options. This case illustrates the evaluation, workup, and treatment of hypercalcemia related to primary hyperparathyroidism in a pregnant patient.

Case presentation

A 22-year-old African American female G1P0 with chronically elevated calcium presented to ER with nausea, vomiting, and dizziness for two days duration. She was 17 weeks pregnant. She denied constipation, fatigue, polyuria, or polydipsia. Medications only included taking prenatal vitamins. Initial vitals were within normal limits. Her physical examination was unremarkable except for a gravid uterus. Initial lab work revealed hypercalcemia with serum calcium of 13.2 mg/dL and albumin of 3.8 g/dL for a corrected calcium of 13.36 mg/dL, ionized calcium elevated at 1.89 mmol/L, PTH elevated at 101.2 pg/mL, Phosphorous low at 1.9 mg/dL, 25-OH vitamin D low at 7.8 ng/mL. TSH and free T4 were 3.070 mcIU/mL and 1.38 ng/dL respectively. Serum creatinine and liver function tests were within normal limits. She was started on a normal saline IV infusion and Endocrinology was consulted for further recommendations. She received two doses of Ergocalciferol 50,000 IU to replete 25-OH vitamin D. Thyroid ultrasound revealed a hypoechoic lesion abutting the inferior aspect of the left thyroid lobe: etiology favoring an enlarged parathyroid gland. 24-hour urine studies were significant for 24-hour urine calcium of 162 mg/24-hour, random urine calcium of 5.0 mg/dL, random creatinine of 32 mg/dL, 24-hour urine creatinine of 1.94 g/24 hour; calculated fractional excretion of calcium was low at 0.007. Serum calcium improved to 11.8 mg/dL with IV hydration. She was evaluated by obstetrics and gynecology and recommended parathyroidectomy as the mainstay of treatment. She decided to discuss the surgical treatment with her primary obstetrician and opted to be medically managed in the meantime. She was subsequently started on cinacalcet 15 mg daily after explaining its pros and cons in pregnancy with plans to follow-up with Endocrinology for its dose adjustment as medically indicated. On follow-up with us, she reported adherence to Ergocalciferol 50,000 IU weekly but had not taken cinacalcet due to cost issues since hospital discharge (9 days prior to the clinic visit).

Discussion

Primary hyperparathyroidism in pregnancy can result in significant maternal and fetal complications. The reported prevalence of the disease appears to be less than 1%. While surgery is the treatment of choice in primary hyperparathyroidism, this can only be pursued in the second trimester in pregnant patients, if not completed prior to pregnancy. There are very few case reports on cinacalcet usage during pregnancy. Before considering cinacalcet treatment, one should extensively discuss the pros and cons of its use in pregnancy.

#135  Euglycemic diabetic ketoacidosis in a patient with COVID-19 case report

NM Perez-Cruz*

R Ramos-Marquez

F Del Olmo

VA Caribbean Healthcare System, San Juan, Puerto Rico

Purpose of The Study

Awareness of Covid-19 virus infection can precipitate decompensation of chronic diseases such as type 2 diabetes Mellitus. Euglycemic diabetic ketoacidosis (eu-DKA) has been seen in patients using sodium-glucose co-transporter 2 inhibitor (SGLT2i) and with COVID-19 infection.

Methodology

Authors identified the case while providing clinical care of a 61-year-old man with medical history of Diabetes Mellitus Type II using SGLT2i and hypertension presented to the Emergency Room with chief complaint of fever, chills, dry cough, watery diarrhea and general malaise 5 days prior arrival to the hospital.

Summary of Results

A 61-year-old man Puerto Rican male with medical history of Diabetes Mellitus Type II using sodium-glucose co-transporter 2 inhibitor (SGLT2i), and hypertension, already vaccinated against COVID-19, who presented to the Emergency Room with chief complaint of fever, chills, dry cough, watery diarrhea and general malaise 5 days prior arrival to the hospital after returning from a recent family trip to Florida. Home medications include Empagliflozin. Patient referred he had a recent travel to Florida (United States) and was in contact with a family member infected with COVID-19 infection. Physical examination was remarkable for dry oral mucosa and laboratories showed a metabolic acidosis with a high anion gap of 20 mEq/L with a marked increase in plasma b-hydroxybutyrate of 57.8 mg/dL and a central glucose <300 g/dL. Patient tested positive for COVID-19 infection. Chest X-ray showed bilateral scattered peripheral hazy groundglass opacities. Considering mentioned findings patient placed on airborne isolation precautions and was admitted to Medical Intensive Care Unit where he was started on DKA protocol with continuous intravenous regular, D5W and aggressive hydration. Medical therapy also included Remdesivir and Dexamethasone. Patient improved after 2 days with resolved eu-DKA. Patient transferred to Internal Medicine Ward.

Conclusion

Eu-DKA has been seen in patients using SGLT2i and with COVID-19 infection; several cases described in literature are suggestive of a specific association between these factors. Our case also highlights the importance of early recognition and management of euglycemic DKA in patients using SGLT2i infected with COVID-19, both increase the risk of dehydration. Physicians must be aware and identify this patients earlier in outpatient setting and be more aggressive in hydration, maintaining euvolemic status to avoid admission to Intensive Care Unit.

#136  Leydig cell tumor leading to peripheral precocious puberty in a 6-year-old boy

N Tantivit*

W Lim

University of South Alabama, Mobile, AL

Learning Objective

To report a rare case of peripheral precocious puberty in a 6-year-old boy.

Case

6 years old male with no significant past medical history presented with concern of pubic hair, prominent facial hair, voice change, enlargement of testis and growth spurt for 9 months. There was no exposure to any form of testosterone at home. Physical exam demonstrated pubic hair at tanner 3, and testes at tanner 2. Labs were done and showed elevated testosterone with suppressed follicular stimulating hormone and luteinizing hormone level which made the diagnosis of peripheral precocious puberty. Testicular ultrasound revealed enlarged right testicle and CT pelvis showed hypervascular 1 cm right testicular mass without definitive evidence of metastatic disease. Patient underwent right radical orchiectomy and pathology report showed Leydig cell tumor. Due to limited testicular involvement, chemotherapy was not required. Repeated testosterone level post-surgery was low.

Discussion

Precocious puberty is uncommon in males, and peripheral precocious puberty is much less frequent than central precocious puberty. About 1.25–4% of reported peripheral precocious puberty in male patients are due to Leydig cell tumors. Leydig cell tumors (LCT) are rare stromal tumors of the testis. Classically, LCT presents with testicular mass (90%) and precocious puberty (10%) such as sudden external genital growth, pubic hair growth, accelerated skeletal and muscle development, and mature masculine voice. Since LCT are non germ cell tumors, they do not cause elevation of alpha feto-protein or beta-human chorionic gonadotrophin. More than 90% of LCT are benign and these tumors can be treated with surgery. Radical inguinal orchiectomy is the treatment for LCT. Due to the benign nature, testes-sparing surgery with regular follow-up also appears to be safe management.

Abstract #136 Table 1

Laboratory results

Conclusion

Leydig cell tumor is a non germ cell tumor that can lead to peripheral precocious puberty in boys.

#137  A case of adrenal incidentaloma with uncharacteristic imaging findings and subclinical cushing syndrome

J Turner*

The University of Tennessee Health Science Center College of Medicine, Memphis, TN

Case Report

The patient is a 65-year-old Caucasian male with a past medical history of right adrenal incidentaloma, cirrhosis secondary to hepatitis C virus and alcohol abuse, hepatitis C infection status post treatment with sustained viral remission, congestive heart failure with reduced ejection fraction, chronic obstructive pulmonary disease, vitamin D deficiency, gastroesophageal reflux disease, and depression with psychosis who presented to the Endocrinology Clinic for evaluation of a right adrenal incidentaloma. He feels well, and he has no new complaints at this visit. The patient was originally discovered to have an adrenal nodule 6 years ago on an MRI of his abdomen. CT of the Abdomen performed several years later showed that the nodule had grown from 1.8 cm to 2.9 cm. The density of the nodule was heterogeneous and described as greater than 10, and its delayed contrast washout was not greater than 50%. MRI findings described a 50% loss of signal on out of phase as opposed to in phase T1-weighted imaging. The consensus across multiple imaging studies was that this incidentaloma’s imaging characteristics were consistent with but not characteristic of adrenal adenoma. Functional testing was performed in the Endocrinology clinic. Assessment for primary aldosteronism and pheochromocytoma were unremarkable. 1 mg overnight dexamethasone suppression tests on two separate occasions did not suppress AM cortisol levels. Dexamethasone levels were also collected to verify that dexamethasone was taken appropriately by the patient. 24-hour urinary free cortisol was also elevated. The patient does not exhibit signs or symptoms of Cushing Syndrome. He was referred to Endocrine Surgery for evaluation, and he has been scheduled for laparoscopic adrenalectomy.

Abstract #137 Table 1

Results of 1 mg overnight dexamethasone suppression test

Abstract #137 Figure 1

Right Adrenal Incidentaloma

Gastroenterology and nutrition and dietary Supplements

Joint plenary poster session and reception

4:30 PM

Thursday, February 10, 2022
#138  An unusual presentation of small bowel intussusception due to an adenomatous polyp

CI Chinchilla Putzeys*

AM Ponnambalam

University of South Alabama, Mobile, AL

Case Report

The patient is a 11-year-old female with past medical history of type 1 diabetes who presented with nausea, vomiting, constipation, and upper abdominal pain. The symptoms began almost 8 months prior to admission and had been gradually worsening. The symptoms worsened considerably 2 weeks before admission. Her stools were small, hard, and pebble-like. Her abdominal pain was described as tightness. She had nausea and vomiting that occurred up to 6–8 times a day. Seven weeks prior to admission, she was seen by her pediatrician and a KUB showed fecal retention. She was placed on polyethylene glycol and lactulose but did not improve.

Two weeks before admission an upper endoscopy revealed esophagitis, duodenitis, and gastritis, and the biopsies were consistent with the endoscopy findings. She was started on cyproheptadine and pantoprazole for possible cyclic vomiting syndrome but the symptoms persisted. She was admitted for worsening symptoms.

On physical examination, she was dehydrated, tachycardic, with a heart rate of 125–130 bpm. Abdomen was soft, non distended with no guarding or rebound tenderness. Lab investigations were significant for hyponatremia, hyperglycemia, hypomagnesemia, hypophosphatemia, and lactic acidosis. Abdominal ultrasound was within normal limits. She continued to experience abdominal pain and vomiting and a CT abdomen with contrast was obtained. This revealed thickened, long segment intussuscepted loop of small bowel and heavy stool burden. Upper GI series showed a dilated loop of small bowel with contrast not passing through, indicative of partial small bowel obstruction.

She underwent diagnostic laparoscopy with reduction of the proximal jejunal intussusception. Upon reduction, a large intraluminal mass was identified 10 cm distal to the ligament of Treitz. The bowel was run through and no additional masses were palpable. Biopsy of the mass revealed tubulovillous adenoma with focal high-grade dysplasia. A repeat upper endoscopy 3 months later was within normal limits. Genetic testing was negative for any genetic predisposition to intestinal polyps.

Tubulovillous adenoma is a polyp protruding into the digestive tract lumen that has around 50% villous and tubular contents respectively. These polyps are capable of progressing to malignancy. Small bowel intussusception is usually an incidental finding diagosed on imaging and usually reduces spontaneously. Most common sites are ileocolic and ileoileal. Around 75% of all childhood intussusception is considered to be idiopathic. Other causes include rotavirus vaccine, adenovirus infection, bacterial enteritis, and development of a lead point secondary to Meckel diverticulum, polyps, hematomas or vascular malformations. Adenomatous polyps leading to small bowel intussusception are rare in children.

#139  Spontaneous peritoneal drainage following paracentesis in a hospitalized patient

MA Elmassry*

B Songtanin

M Abdelnabi

J Benjanuwattra

S Sanchez

M Faragallah

S Rao

C Peterson

K Nugent

Texas Tech University Health Sciences Center, Lubbock, TX

Introduction

The median survival of decompensated cirrhotic patients without transplantation is approximately two years. Repeated large volume paracentesis with albumin administration is the first line treatment for refractory ascites. Previous studies showed the benefit of long-term peritoneal drainage in minimizing frequent hospitalization and reducing cost.

Case summary

A 36-year-old man with a past history of alcohol abuse with decompensated liver cirrhosis (Child Pugh Class C), hypertension, and depression, presented to the hospital with an altered mental status and was diagnosed with hepatic encephalopathy and acute kidney injury. Vital signs on admission were normal except for tachycardia. Physical examination showed confusion, jaundice, and icteric sclerae. Gastrointestinal examination showed tense ascites with fluid wave, mid-abdominal tenderness, and dullness on percussion. He also had bilateral leg swelling up to mid-tibial level. His MELD-Na on admission was 36 (65–66%, estimated 90-day mortality) and Serum-Ascites Albumin Gradient (SAAG) was 2.7 gm/dL consistent with portal hypertension. Paracentesis was performed, and the ascites fluid was a transudate. A peritoneal drainage catheter was removed after 72 hours of insertion due to blockage in the peritoneal catheter. Surprisingly, peritoneal fluid continued to leak at the insertion site. A urostomy bag was placed over the puncture site, allowing peritoneal fluid to drain for 7 days. His kidney function improved after the continuous drainage started (Table).

Abstract #139 Table 1

Improvement of renal functions during patient‘s hospital course

Conclusion

Continuous peritoneal drainage in this case provided unexpected benefits, including significant removal of ascitic fluid and improvement in renal function. Continuous peritoneal drainage might be considered in other hospitalized patients with large volumes of ascitic fluid causing important clinical effects.

#140  Virtual reality and mindfulness technique in the treatment of rumination syndrome: a case report

M Esteban*

S Cherukuri

TQ Dang

K Espino

C Didia

A Dwivedi

R McCallum

Texas Tech University Health Sciences Center, El Paso, TX

Introduction

Virtual reality (VR) offers immersive, realistic, three-dimensional experiences that lets users experience audio and visual environments of guided relaxation. Multiple studies have proven its effectiveness in reducing pain and anxiety in both adults and children. We report a patient with Rumination Syndrome who underwent a treatment program of VR and Mindfulness technique leading to marked symptom improvement related to abdominal pain, nausea, vomiting.

Case

Patient is a 24 year old Hispanic female with a past medical history of hypothyroidism who initially presented to the GI Motility clinic with a six-month history of epigastric pain and post-prandial nausea and vomiting occurring within 15 minutes of ingesting a meal and even water. Laboratory findings were unremarkable. An upper endoscopy was normal except for mild gastritis, pathology showed H. pylori was negative and a gastric emptying study was normal. Given this rather ‘classic’ clinical presentation, she was diagnosed with Rumination Syndrome. Patient was started on Omeprazole, Promethazine, Dicyclomine and Amitriptyline with some improvement but not resolution of symptoms. After discussion with patient, she decided to be enrolled in our ongoing IRB approved program using VR and Mindfulness techniques in the management of refractory gastrointestinal disorders. Patient completed baseline Patient Assessment of Gastrointestinal Disorders Symptom Severity Index (PAGI-SYM) questionnaire in which her score was 77 out of a possible 110.

She underwent a total of 16 VR (2/Week) and 8 Mindfulness (2/Week) sessions in a span of 8 weeks. She also continued mindfulness exercises at home once the final treatment session was completed. No medication changes were made during the duration of the sessions. Patient was asked to answer the PAGI-SYM questionnaire at 2 week intervals and her scores showed continued improvement: such that her score at 8 weeks was 10 (85% improvement) specifically involving changes in12 symptoms. No adverse side effects from both the VR and mindfulness technique sessions were reported by the patient.

Conclusions

Managing Rumination Syndrome and other functional gastrointestinal disorders can be a challenge even to the most seasoned gastroenterologist. Often, medical pharmacology is insufficient in controlling symptoms. A good working relationship and rapport between the physician and patient is the first key to therapy. We have demonstrated that combining sessions of VR and Mindfulness technique is a very effective and safe tool in managing patients with the Rumination Syndrome symptoms of nausea, vomiting and abdominal pain refractory to standard therapy. We are continuing our study at our institution and we aim to apply this approach to other upper gastrointestinal disorders such as gastroparesis, Irritable Bowel Syndrome, Chronic Functional Abdominal Pain and refractory nausea.

#141  Decompensated liver cirrhosis occurring in heterozygous alpha 1 antitrypsin deficiency

S Gandu*

A Vasikaran

S Pandit

LSU Health Shreveport, Shreveport, LA

Case Report

Alpha 1 Antitrypsin deficiency(AATD), is an autosomal co-dominant disorder occurring in individuals who inherit an abnormal A1AT gene from each parent. Over 120 different alleles have been associated with this gene, of which the three most common are Z, S, and M. The Z and S allele are the abnormal variants, associated with severely and moderately reduced production of normal A1AT protein respectively. The prevalence of the Z allele is increasingly present in Caucasians, with males seemingly more affected than females. The typical presentation of adults with severe AATD associated with liver disease; chronic hepatitis, cryptogenic cirrhosis, and decompensated liver disease is commonly associated with the PiZZ variant. This toxic ‘gain of function’ mutation causes an accumulation of the abnormal A1AT, overwhelming the hepatocytes resulting in an apoptosis-driven liver cell injury. There exists profound variability in the clinical presentation of A1AT deficient patients. As patients with the ZZ variant may present in infancy or adulthood. Additionally, variability may be related to socio-environmental risk factors and disease modifiers like smoking, allele variability, and hormonal effects. Here we have a patient diagnosed with cryptogenic cirrhosis of the liver upon biopsy, incidentally, found to have reduced serum A1AT levels with the MZ phenotype. There are multiple unique aspects to this case. The patient is a 48-year-old Caucasian woman with no past medical history, non-smoker, that denied alcohol consumption, illicit drug use, devoid of any pulmonary symptoms. She presented after a hysterectomy which was complicated by bleeding and was found to have mildly elevated serum aminotransferase levels. While undergoing evaluation for liver transplant the patient developed signs of decompensated liver cirrhosis evidenced by ascites and hepatic encephalopathy, leading to this diagnosis.

#142  Delayed presentation of hirschsprung’s disease

K Glisson*

C LeBlanc

Louisiana State University, Baton Rouge, LA

Case Report

Hirschsprung’s disease (HD) is a disorder characterized by the failure of ganglion cell precursors to migrate into the bowel in utero, leading to aganglionosis in the submucosal and myenteric plexus. 95% of patients with HD fail to pass meconium within the first few days of life. Other typical clinical features include abdominal distension, bilious vomiting, enterocolitis, feeding intolerance, and failure to thrive. Establishing a diagnosis is important as this is a condition with mortality rates of 2%-5% have been reported despite advances in care. The following is a case of HD that lacked many of the classic historical features and exam findings at initial presentation and contributed to a delay of diagnosis and definitive surgical management.

A 16-month-old infant male presented to the pediatric gastrointestinal clinic with constipation. He was seen previously at 8 months of life for the same concern, but failed to attend scheduled follow-ups. At the first visit, parents reported that the patient had a bowel movement within the first 24 hours of life, normal bowel movements for the first 2 months of life, normal newborn screening, a reassuring exam and his weight was in the 70th percentile. Treatment for constipation was recommended at the initial visit. However, at the 16 month visit parents reported minimal symptomatic improvement.

The patient was admitted for further evaluation of his constipation. His initial exam was significant for a distended, non-tender abdomen with a palpable mass in the lower abdomen. Digital rectal examination revealed good rectal tone with no palpable stool and a tight anal sphincter. His initial abdominal plain film showed abundant stool. A subsequent barium enema revealed a narrow rectum that transitioned to a severely enlarged, fecal impacted sigmoid colon. The patient underwent a rectal suction biopsy which confirmed his diagnosis of HD. The patient underwent a diverting colostomy with no post-operative complications.

Constipation is a common general pediatrics issue. It is estimated to be the cause of 3–5% of all visits to pediatricians. Functional constipation is responsible for over 95% of these visits. This case highlights that although constipation in this age group is frequently functional, diseases such as HD can present outside of the classic age range and with a lack of red flag symptoms for gastrointestinal pathology. While 80% of HD patients present in the first few months of life, it should remain on the differential diagnosis for constipation as up to 15% of the cases remain undiagnosed until the age of 5 years.

#143  A rare presentation of proximal small bowel adenocarcinoma

B Mohanakrishnan1*

A Abraham2

R Waqas1

A Islam1

1Texas Tech University Health Sciences Center, Amarillo, TX

2Texas Tech University Health Sciences Center School of Medicine, Amarillo, TX

Introduction

Several tumors can arise within the small bowel, both malignant (adenocarcinomas, neuroendocrine tumors, lymphomas, and sarcomas) and benign (adenomas, leiomyomas, lipomas). However, it is crucial to recognize chronic abdominal pain as a rare proximal small bowel tumor presentation. The specific components of the diagnostic and staging workup include computed tomography scan, small bowel series, enteroclysis, capsule endoscopy, push enteroscopy, double-balloon endoscopy. No single method is best for imaging the small intestine in a patient with a suspected small bowel tumor.

Case presentation

A 43-year male with no significant past medical history presented with symptoms of several months of fatigue, tiredness, and exercise intolerance. He experienced a constant, dull left upper quadrant abdominal pain radiating to the back with no change to food and not improving with time. He also reported taking ibuprofen to lessen the pain and agreed to heavy alcohol use in the past. Denies weight loss, fever, night sweats, chest pain, nausea, vomiting, loss of appetite, hematemesis, hematochezia, bowel habit changes, and tobacco use. At presentation, hemoglobin was 4.7, normal MCV, platelet count, and coagulation panel and received two units of packed red blood cells transfusion. Contrast CT scan of the abdomen showed a mass in the small bowel, in the proximal jejunum. Esophagogastroduodenoscopy and colonoscopy were performed. The EGD showed a large ulcerated mass with stigmata of recent bleeding found in the proximal jejunum. Biopsies of the mass were done, and histopathology showed findings of small bowel adenocarcinoma. Colonoscopy was negative. Hand-assisted laparoscopic resection of malignant small bowel tumor with small bowel resection and anastomosis was performed and sent for histopathological examination. A poorly differentiated adenocarcinoma with mucinous features measuring 8.9 X 8.7 X 6.3 cm, extensive necrosis extending into sub-serosal tissue with bowel perforation, and no positive regional lymph node with a good tumor margin was reported. The adenocarcinoma was staged as pT4N0M0. The patient was discharged on postoperative day 5 with a follow-up in the outpatient oncology clinic.

Discussion

In 2021, the American cancer society reported 11390 cases of small intestine tumors in the United States. According to the National Cancer statistic center in 2018, proximal small bowel cancers have a very low incidence of 0.6% of all diagnosed cancers in the USA. This case demonstrates the potential of missing a rare and deadly gastrointestinal malignancy from vague and nontraditional clinical symptoms.

Conclusion

We conclude that, although rare, a small bowel carcinoma must be kept in mind when working up patients presenting with vague, chronic abdominal pain. As most reported adenocarcinomas with small bowel origin are diagnosed late, and the prognosis is poor, early recognition of small bowel adenocarcinoma is crucial and lifesaving.

#144  Another sherlock holmes mystery: abdominal pain explained by median arcuate ligament syndrome

AJ Ortega*

H Memon

B Davis

R McCallum

Texas Tech University Health Sciences Center El Paso, El Paso, TX

Introduction

Median arcuate ligament syndrome (MALS) also known as celiac artery compression syndrome is a rare gastrointestinal condition with an estimated incidence of 2 per 100,000 population. Predominantly in female patients, it is characterized by the compression of the celiac artery at its origin from the aorta by the median arcuate ligament, also entrapping the celiac plexus resulting in upper abdominal pain, notably postprandial pain, associated with nausea, vomiting, food aversion, and weight loss. An abdominal bruit is present in approximately one-third of patients. MALS is a diagnosis of exclusion relying on more recognized etiologies of abdominal pain to be first investigated.

=We present a case of abdominal pain secondary to MALS that was appropriately diagnosed after requiring narcotic medication and responding to surgical therapy.

Case description

A 48-year-old female with a past medical history significant for diabetes mellitus and hypertension presented with an eight-month history of sharp, non-radiating, intermittent left upper quadrant pain with fluctuating intensity. She denied any association with meals but reported aggravation by positional changes and direct palpation. Review of systems was negative for nausea and vomiting.

Upper endoscopy and colonoscopy were unremarkable. A four-hour nuclear medicine scintigraphy gastric emptying study of a solid meal indicated rapid emptying at 30 and 60 minutes. An abdominal ultrasound to study Doppler blood flow suggested a spectral waveform of the celiac artery suspicious for MALS. On inspiration, peak systolic velocity was 297 cm/s and end-diastolic velocity of 139 cm/s. On expiration, the peak systolic velocity was found to be 607 cm/s and end-diastolic velocity of 109 cm/s. A mesenteric arteriogram confirmed the stenosis of the celiac artery accentuated with end-expiration and reduced by inspiration. The patient underwent a laparoscopic robotic procedure to release the median arcuate ligament combined with intraoperative Doppler ultrasound blood flow measurements. After ligation of the ligament, the expiration peak velocity of 169 cm/s was markedly reduced compared to the preoperative data. Two weeks after the procedure, the patient was seen in the clinic and she reported significant improvement with essentially no abdominal pain.

Discussion

We present an atypical case of MALS presenting as abdominal pain not accentuated by meals and without associated nausea and vomiting. Diagnostic modalities such as Doppler ultrasound blood flow velocity and aortic angiogram were able to make the diagnosis. The goal of treatment of MALS is surgical release of the median arcuate ligament, thus reducing the compression on the celiac ganglion. In this case, we present subjective improvement of abdominal pain following laparoscopic resection of the median arcuate ligament with objective intraoperative findings confirming resolution based on the return of normal blood flow velocity.

#145  A case of wernicke encephalopathy in a patient with autism disorder and restrictive eating

DL Rohlfs Rivera1*

AA Prevot1

N Sharma2

G Hescock1

D Manning1,2,3

1LSU Health New Orleans, New Orleans, LA

2Tulane University Health Sciences Center, New Orleans, LA

3LCMC Health, New Orleans, LA

Purpose

Wernicke encephalopathy (WE) is an acute neurodegenerative condition characterized by confusion, ophthalmoplegia, and gait ataxia. While most frequently seen in chronic alcoholism due to thiamine deficiency, WE can occur in other scenarios where there is poor nutrition. Recognizing WE in other high-risk patients is important as delay in treatment can lead to permanent neurological deficits, coma, and death.

Methods

Retrospective Chart Review. (Case presentation)

Results

The patient is a 16-year-old female with a medical history significant for autism disorder, restrictive eating with weight loss, and epilepsy who presented with intractable nausea and emesis requiring intravenous hydration. The patient was diagnosed and treated for H. pylori with improvement of her symptoms. Several days into her hospital stay, she developed cranial nerve six palsy, confusion, and ataxia. CT head and ophthalmology exam were not suggestive of increased intracranial pressure. MRI brain with and without contrast revealed T2 hyperintensity of the hypothalamus, bilateral mamillary bodies, paramedian thalami, and periaqueductal gray matter consistent with Wernicke-Korsakoff Syndrome. The patient was given high dose parenteral Thiamine with improvement in cranial nerve palsy, confusion, and gait. Thiamine level obtained prior to supplementation was low. Patient was continued on oral thiamine supplementation and was admitted to inpatient rehabilitation for neurorehabilitation.

Conclusions

It is important to recognize children and adolescents who might be at increased risk for thiamine deficiency as WE is an uncommon disease in the pediatric population and dextrose containing intravenous fluids (commonly used in pediatric rehydration protocols) are known to precipitate or exacerbate WE. Identification of high-risk patients can also lead to early diagnosis and treatment.

#146  Primary biliary cholangitis – sherlock’s clue to the fatigued man

P Stueve*

I Pathirana

Dwight David Eisenhower Army Medical Center, Fort Gordon, GA

Case Report

Primary biliary cholangitis (PBC) is a rare disease characterized by intrahepatic destruction of the bile ducts and cholestasis. The diagnosis requires two of the following three criteria: an elevated alkaline phosphatase (ALP), presence of anti-mitochondrial antibodies (AMA), and histologic evidence of destructive cholangitis and interlobular bile duct destruction. The disease has a female to male predilection of 10:1, with diagnosis typically occurring in the fourth through seventh decade of life. Risk factors associated with this condition include family history of PBC, tobacco exposure, history of other autoimmune diseases, and previous pregnancies in females.

Case Description

Our case involves a 74 year-old Caucasian male with iron deficiency anemia, remote 40 pack-year history of tobacco use, and chronic liver masses who presented to the outpatient internal medicine clinic for evaluation of unintentional weight loss and fatigue. He was found to have elevated liver associated enzymes in a cholestatic pattern with ALP of 197 IU/L, AST of 64 IU/L, and ALT IU/L of 177. Repeat testing six months later demonstrated resolution of the transaminase elevations with continued elevation of ALP at 145 IU/L. Computed tomography (CT) of the abdomen and pelvis re-demonstrated pathologically enlarged (stable) lymph nodes, and unchanged liver masses with recommendation for triple phase CT. Subsequent imaging showed indeterminate lesions only seen on arterial phase concerning for hepatocellular carcinoma versus other benign cause. A liver biopsy was then performed demonstrating only a ductulocentric inflammatory process including periportal and periductal inflammation without bridging fibrosis. Laboratory results showed a positive anti-mitochondrial antibody (AMA) of 106 units (0–20 units ref range) with negative anti-smooth muscle antibody, anti-nuclear antibody, IgG and ferritin. He had preserved hepatic synthetic function. His imaging, pathology, and laboratory results were most consistent with a diagnosis of PBC. Treatment with ursodeoxycholic acid (UDCA) was initiated with resolution of symptoms and improvement in ALP levels.

Discussion

This case illustrates the importance of maintaining a broad differential for atypical presentations of common derangements, even in patients who lack typical demographic characteristics. PBC and other autoimmune conditions should be evaluated in patients that have laboratory or tissue abnormalities raising appropriate clinical suspicion for the condition. Accurate diagnosis of these conditions will facilitate early proper treatment and thus improve patient outcomes.

#147  Incidental discovery of a rare esophageal gastrointestinal stromal tumor during cardiac imaging

AL Wilson1,2*

D Velu2

A Ahmed1,2

N Abraham-Philip1,2

1The University of Alabama at Birmingham, Birmingham, AL

2UAB, Huntsville, Huntsville, AL

Case Report

Gastrointestinal stromal tumors (GIST) are mesenchymal neoplasms located within the stroma of the GI tract. Their incidence is rare especially involving the esophagus. They are usually asymptomatic unless metastatic or large, causing compression. We report an interesting case with an incidental diagnosis of GIST delineating the features and management we pursued.

A 69-year-old female with history of symptomatic atrial fibrillation/GERD was planned to undergo pulmonary vein isolation (PVI). Before the procedure, Cardiac CT was done for evaluation of pulmonary veins. Incidentally, she was found to have a large soft tissue posterior mediastinal mass adjacent to the esophagus and above the hiatus. Further questioning revealed symptoms of intermittent pill dysphagia of unknown duration. Thoracic imaging from a year ago showed no evidence of mass. The primary differentials considered are Lymphoma due to the patient’s age and rapid growth of the mass; Epithelial (Squamous vs Adenocarcinoma), and mesenchymal tumors (Leiomyomas >> GIST).

Endoscopic ultrasound with FNA showed spindle cell neoplasm with atypia positive for c-KIT and CD34 confirming the diagnosis of GIST. Prioritizing her symptoms, she successfully underwent PVI, and then further workup was pursued for the mass.

Ideally, the management of GISTs is based on the size, histopathology, and extent of metastasis. In our patient, the mass was around 8 cm, making a complex surgical option inevitable. Successful Ivor Lewis esophago-gastrectomy was pursued removing the mass completely with clear margins. Additionally, the patient was started on adjuvant chemotherapy with Imatinib for three years. Follow-up to date (2 years since surgery) confirmed that the patient continued to remain asymptomatic with no radiological evidence of relapse assuring treatment success.

GIST typically derives from intestinal cells of Cajal and often is positive for c-KIT, a tyrosine kinase that encodes stem cell factor receptor, which undergoes a point mutation to cause malignancy. Surgical resection is a potentially curative treatment for localized GISTs. Smaller tumors may permit enucleation, while esophagectomy may be needed in larger or high-risk tumors with a high mitotic rate. Recurrence and metastasis are common with aggressive c-KIT mutations. Tyrosine kinase inhibitors such as Imatinib are recommended by National Comprehensive Cancer Network (NCCN) for mass greater than 3 cm even if they are localized, to minimize the risk of relapse.

Health care research, quality improvement & patient safety

Joint plenary poster session and reception

4:30 PM

Thursday, February 10, 2022
#149  Standardization of the management of necrotizing enterocolitis at a single-center neonatal intensive care unit: a quality improvement initiative

NB Akahara*

M Arbuthnot

M Chang

R Pandey

K Tsao

C Domonoske

W Li

C Ault

AL Speer

M Forbes

C Aneji

The University of Texas Health Science Center at Houston John P and Katherine G MGovern Medical School, Houston, TX

Purpose of Study

Necrotizing enterocolitis (NEC) is an inflammatory disease that affects the small or large bowel. NEC is a leading cause of death in neonatal intensive care units (NICU).

It occurs in 1 to 3 per 1000 live births, with more than 90% occurring in infants < 32 weeks gestational age and < 1500 g birth weight. The etiology of NEC is unknown but available evidence supports a multiple-factorial mechanism. There is significant variation in the care provided within and between different NICUs. There is evidence from other patient populations like congenital diaphragmatic hernia patients, that standardizing care leads to better outcomes. The purpose of this quality improvement (QI) initiative is to standardize the care of NEC patients from diagnosis to discharge. This initial stage involved evaluating 6.4 years of patients who were managed for NEC at our single-center to establish baseline data.

Methods Used

We conducted a retrospective chart review on all patients diagnosed and treated for NEC from January 1, 2015, through April 30, 2021, at Children’s Memorial Hermann Hospital (CMHH) to establish our baseline data. The patient outcomes of interest included length of stay, antibiotic utilization, timing of surgery team consultation, enteral and parenteral nutrition practices. A multidisciplinary team is developing a guideline. Two PDSA cycles will be run, and prospective data will be collected to assess 6- and 12-month outcomes. We present the baseline data.

Summary of Results

There were 132 patients diagnosed with NEC in the 5-year period at CMHH; 47% males and 53% females. The average gestational age was 28.8 weeks, average birth weight was 1414.4 g with range of 360 g to 5560 g. Seventy-two percent of cases were managed medically, while 28% required surgery. Of the 26.5% of patients who died in hospital, majority (91.4%) had surgical NEC while 8.6% had medical NEC. There was variability in the length of stay of patients, antibiotic utilization, use of surgery consultation, and nutrition practices.

Conclusions

There was a higher incidence of NEC in preterm, very low birth weight patients. There was variability in management of these patients. We are currently working on a comprehensive NEC management guideline with the goal of standardizing the care of patients diagnosed with NEC at CMHH and decreasing the variability by 50% over 1 year. We anticipate that this will improve antibiotic usage, nutrition and reduce the average LOS of patients.

#150  Pediatric intensive care unit and operating room handoff process improvement

H Bauer1,2,3*

A Creel1,2,3

R Monier1,2,3

1LSU Health New Orleans, New Orleans, LA

2Children’s Hospital New Orleans, New Orleans, LA

3LCMC Health, New Orleans, LA

Purpose of Study

This quality improvement initiative seeks to assess the perception of process change of direct admission to PICU from OR utilizing a nurse driven handoff tool, as well as perceived effects to PICU staffing, OR efficiency, patient safety, and workflow.

Methods Used

Anonymous survey data using Likert 5-point scale of frequency was collected via Survey Monkey. This data was utilized to track baseline team member perception of the PICU-OR Handoff Process prior to implementation of a handoff tool and immediately after its implementation at 1 month. A follow-up survey will be completed 6 months after the process change. Physicians, nurses, and respiratory therapists from the departments of Pediatric Critical Care, Anesthesia, and Surgery were asked to participate in the survey voluntarily. No identifying data was collected or stored during this study.

Summary of Results

Data collection began in April 2021 with baseline and 1 month post hand-off implementation data. Thus far there have been 59 team members from the departments of Anesthesia (n=10), Pediatric Critical Care (n=46), and Surgery (n=3) who participated. Data collection of 6 months post intervention is ongoing. The survey data included Likert 5-point scale of frequency (Never, Sometimes, Often, Usually, and Always). Four survey questions were included which addressed anticipation of patient arrival to PICU, whether comprehensive report was given at the time of admission and whether adequate information was given at the time of handoff to provide excellent care, or whether clarification of treatment plans was required within 1 hour. This was followed by a final qualitative, open ended question to provide any thoughts on the handoff process as well as suggestions for improvement. A comprehensive report given in person was reported as given often or always approximately 22% of the time, respectively (13/59 each, 26/59 total). Team members felt they usually had the information they needed to provide excellent care 37% of the time (n=22/59). Forty seven percent of respondents (n=28/59) felt they sometimes needed to clarify treatment plans within 1 hour of report.

Conclusions

Though limited by small sample size with data collection still in process, this quality improvement initiative highlights the need for further process improvement to provide the safest, timely, organized transition of care of a pediatric patient between the OR and the PICU. With each transfer of information there exists the possibility to introduce error which may lead to adverse events. A dedicated hand-off tool can help to accomplish these goals by bringing all stake holders together, allowing the opportunity for listening and questioning, as well as outlining plans of care.

#151  Caretaker beliefs towards influenza vaccination

E Bolender*

D Kim

S Studebaker

E Knowlton

AD Hendrix

M Condren

The University of Oklahoma – Tulsa, Tulsa, OK

Purpose of Study

An effective vaccine against influenza is available, yet a significant portion of the pediatric population does not receive it. Across the United States during the 2017–2018 influenza season, 68% of children aged 0–4 years and 55% of children aged 5–17 years received their influenza vaccine. The lack of vaccination in the pediatric population causes many patients to be vulnerable to the potentially devastating effects of influenza. We sought to investigate caretaker beliefs towards the influenza vaccine to better understand the possible motivators for, and barriers to, parents vaccinating their children.

Methods Used

De-identified paper surveys were given to caretakers of patients seen at an academic pediatric clinic during the 2019–2020 and 2020–2021 influenza seasons. The survey included demographic questions for both the patient and caretaker. The survey also included questions about the perceived severity of the flu and beliefs regarding influenza and other pediatric vaccinations. Descriptive and inferential statistics were calculated using SPSS®version 28.

Summary of Results

During the study period, 87 completed surveys were collected from caretakers of patients. While most caretakers (82%) considered influenza to be a serious disease, fewer (49%) indicated that the vaccine prevents disease. Few caretakers (28%) believed that it is possible to contract influenza from the vaccine. Caretakers (54%) agreed to vaccinating their child(ren) routinely for influenza while more (74%) indicated vaccinating their children for other diseases. Even fewer (41%) caretakers indicated that they vaccinate themselves against influenza each season.

Conclusions

The preliminary data from this study has shown a difference in caregiver beliefs regarding the influenza vaccine compared to regular childhood immunizations. The majority of the survey respondents indicated support of regular immunizations with some skepticism of the influenza vaccine.

#152  A pilot study evaluating respirator fit based on app-determined and self-determined facial shape

N Buynak*

D Hahn

D Tyungu

C Aston

Oklahoma University Medical Center, Oklahoma City, OK

Purpose of Study

This project was developed to find a quick and effective way for frontline workers to obtain a well-fitting N95 in resource limited settings when a fit test may not be plausible. The goal was to determine if facial shape could be used as a predictor of N95 fit.

Methods Used

Forty volunteers were given a facial shape self-assessment questionnaire which asked them to subjectively determine their own facial shape and then measure several dimensions with a disposable tape measure: half facial height (nasion to menton), full facial height (trichion to menton), and facial width (bizygomatic breadth). Two free facial assessment phone applications, ‘Face Shape’ and ‘Zennioptical’, were used as an additional assessment for facial shape.

Participants were then fit tested with an AccuFIT 9000 Respirator Fit Test Machine using an OSHA standardized technique to assess the quantitative fit of four different N95’s – a small and regular sized duckbill type mask, and a small and regular sized cup style mask. Pass/fail criteria was determined per OSHA standards and was set at a fit factor greater than or equal to 100.

Summary of Results

There was no association between face shape and best fitting mask based on either self-assessment (p= 0.51), the zenni app (p=0.59), or the Face Shape app (p=0.095). Correlation was not seen even when grouping face shapes into curved and angular.

Face shape based on the 3 self-measured objective facial dimensions can be predicted with about 65% accuracy.

Self-assigned face shape correlated with Zenni app face shape 40% of the time and with the Face Shape app 37.5% of the time (p>0.1). All three correlated 25% of the time, however the mutual face shape was ‘oval’ which was the most common facial shape identified in this study by both the applications and self-assessment.

Forty-four percent of the participants did not pass fit testing per OSHA standards with their routinely worn N95’s. The participants in this group also generally had poorer fit testing overall, with 50% of this group failing fit testing for all four masks. In addition, 33% of the entire study group only passed fit testing with one of the available N95’s in this study.

Conclusions

Data from this pilot study shows that there is no correlation between N95 fit and face shape, largely due to the variability and subjectivity in the determination of facial shape by either app, self-assessment, or objective self-measurement. However, the researchers learned that nearly half of the participants did not pass fit testing for their regularly used N95’s during the COVID pandemic. This illustrates a significant concern for the safety of healthcare workers and the inability for them to access appropriate, well-fitting respirators in resource limited settings. Furthermore, it highlights the importance of personalized fit testing prior to exposure to airborne particles and the need for access to multiple styles and sizes of respirators.

#153  Improving pediatric clinical research competency through interactive e-learning modules

R Cantu*

S Chervinskiy

J Snowden

University of Arkansas for Medical Sciences, Little Rock, AR

Purpose of Study

Clinical research has significantly advanced the diagnosis, treatment, and prevention of illness and has provided patients access to ‘state-of-the-art’ care. While the curricula of pediatric trainees often include structured education for interpreting the results of such research, rarely are trainees instructed in conducting research itself. Children have historically been underrepresented as subjects of clinical research, making the ability to expand participation in high-quality pediatrics research essential to improving our knowledge of children’s health in order to provide the best medical care. We designed, implemented, and executed a longitudinal research course that accomplished these goals, containing ten interactive online modules with periodic face-to-face sessions consisting of coaching in use of various tools and problem-based learning sessions.

Methods Used

The PRIME project at Arkansas Children’s and the University of Arkansas for Medical Sciences (UAMS) consisted of online modules as well as face-to-face sessions for trainees in pediatrics including residents and fellows. After completion of the modules, participants were asked to complete an online survey and post-course knowledge check. The survey asked questions about the modules, including whether the trainees felt the modules improved their research competency and whether they preferred synchronous or asynchronous sessions. Participants were also asked to rate the modules and to state three most impactful lessons/skills learned from the modules.

Summary of Results

Of the eight courses evaluated, six of them received at least one rating of ‘excellent’ and all received at least one rating of ‘good’. No course received any poor evaluations. Courses that provided hands-on education for practical, readily available concepts were most favored. All post-survey responses indicated a perceived, self-identified increase in confidence in the ability to identify a research question and design a study. Respondents also indicated that they are more likely to incorporate research into their future careers based upon the knowledge gained from this course. Half of the respondents noted that they preferred live, scheduled sessions as opposed to individual online sessions.

Conclusions

Based on feedback from attendees, this innovative course successfully helped training clinicians gain knowledge and competence in core principles of clinical research, develop their own protocols for pursuing their own studies, and change attitudes toward pursuing future research-related careers. With an increasing number of clinicians with appropriate interests and skills planning to participate in clinical research, a greater number and diversity of children may be engaged in future clinical studies.

#154  Bibliometric analysis of publication trends in the covid-19 pandemic

B Daines*

A Kankam

SA Tanami

KG Holder

V Vemulapalli

R Nambiar

Texas Tech University Health Sciences Center School of Medicine Amarillo, Amarillo, TX

Purpose of Study

The COVID-19 pandemic has presented a major challenge to the global medical community resulting in many necessary changes in worldwide research publication trends. As focus and funding of research have shifted throughout the pandemic, so too have publication types, topics, and country-wide research output. While changes during the pandemic have undoubtedly had an impact on publications, these impacts and their implication for long-term research have been poorly characterized. We aim to illustrate these shifting changes in publications during the COVID-19 pandemic.

Methods Used

Publications were accessed using PubMed Advanced Search Criteria. The beginning of the COVID-19 pandemic was set as the date of the first reported case in Wuhan on 12/31/2019. Searches were conducted on 9/10/2021. We generated searches based on type of publication, country affiliation of the first author, date ranges, and specific topics such as ‘COPD’ or ‘Diabetes’. We analyzed the changes in publications during the 4 months before the first case of COVID-19 and every 4 months thereafter. Results were analyzed using SPSS.

Summary of Results

In the two years before the pandemic, 3541.9 PubMed-indexed articles were published per day, while 4379.9 such articles have been published each day during the pandemic. Covid-19 resulted in net increases relative to baseline in case reports, reviews, and retracted publications with net decreases in clinical trials, multicenter studies, and randomized control trials in most yearly tertiles relative to the 4 months before Covid-19. Meta-analysis and observational studies demonstrated initial net increases followed by subsequent net decreases. The largest percent decrease in publication type relative to baseline has been seen in Phase I (-82.6%) and Phase IV (-80.5%) clinical trials. In the first yearly tertile, China had the largest percent increase in publications relative to baseline (38.4%) followed by Italy in the second tertile (32.0%) and India in the third tertile (43.5%). Increases in daily publications were seen in many research topics including diabetes (32.8/day), asthma (4.6/day), heart disease (2.9/day), and COPD (2.4/day).

Conclusions

COVID-19 has caused a shift in research focus and funds towards pandemic-related research. This has emphasized lower evidence research, such as case reports, and shifted focus away from high evidence studies, such as clinical trials and meta-analysis. Production of high-evidence studies does not appear to be recovering as the pandemic continues. Research output of individual countries appears to coincide with COVID-19 related infection surges in each respective country. Despite new emphasis on Covid-related research, many important topics such as diabetes and heart disease have experienced increases in publications.

#155  Generational differences in covid-19 vaccine hesitancy among college students

AB Davis*

J Rush-Kolodzey

T Davis

C Arnold

D Smith

Louisiana State University in Shreveport, Shreveport, LA

Purpose of Study

The objective of this study was to identify generational differences in COVID-19 vaccine hesitancy among college students.

Methods Used

An online survey was conducted between September and October 2021 to the student body at Louisiana State University Shreveport to compare COVID-19 vaccine hesitancy among the following generations: Baby Boomers (1955–1964), Generation X (1965–1980), Millennials (1981–1996), and Generation Z (1997–2000).

Summary of Results

Among the 339 participants, 66.8% were female, 28.5% male, 2.4% non-binary, and 2.4% other. Regarding their race, 64% were White, 16% African American, 9.8% selected two or more races or other, 6% Middle Eastern or North African, 5% Hispanic/Latinx, 2.7% Asian, and 1.2% Native American or Alaska Native. Among the generations who participated in the study, 43.2% were from Generation Z (GZ), 31.8% Millennials (M), 21.9% Generation X (GX), and 3% Baby Boomer (BB). Of the participants who selected that they had not received the COVID-19 vaccine, Generation Z (53.8%) reported the highest number, followed by Millennials (26.1%). In addition, when asked if participants planned to receive the COVID-19 vaccine, over half of Generation Z reported that they did not plan to get the vaccine compared to 19.7% of Generation X and 1.7% of Baby Boomers. Interestingly, when asked if FDA approval made them feel more confident in the vaccine’s safety, the data suggests no statistical significance between groups. The leading causes for vaccine hesitancy among Generation Z were not trusting the COVID-19 vaccine (56.7%), and concerns about unknown long-term side effects of the vaccine (68.3%).

Conclusions

Of the generational groups, Generation Z college students report low rates of COVID-19 vaccination and intention of receiving the vaccine. This fact may have implications for herd immunity for college campuses across the nation.

#156  Improving firearm safety counseling in primary care clinic

J Bell

A Donahue*

AR Wheeler

CR Hunley

J Joseph

A Martin

M Ladinsky

The University of Alabama at Birmingham, Birmingham, AL

Purpose of Study

Pediatricians can play a major role in the prevention of pediatric firearm injuries. The presence of firearms in the home is associated with increased likelihood of suicide, homicide, and unintentional injuries. Unintentional and self-inflicted firearm injuries are less likely in homes where weapons are stored unloaded and locked. Child health care providers can and should play a key role in preventing gun violence, injury, and death in children. The American Academy of Pediatrics (AAP) recommends that pediatricians educate patients and families about the dangers of firearms in the home. Gun storage that is more secure minimizes injuries, and physician counseling combined with the distribution of cable locks appears to improve gun storage security. How frequently and effectively we provide firearm safety counseling to Primary Care Clinic (PCC) patients is poor. The objectives of this quality improvement (QI) project are to increase the consistency and quality of firearm safety counseling in PCC, as well as to provide patients with gunlocks.

Methods Used

This project has undergone multiple Plan-Do-Study-Act (PDSA) cycles including PCC ‘spotlights’, a didactic lecture, and resident surveys. The current focus is on retrospective chart review of 9-month-old and 24-month-old well checks in PCC and determining if the clinic resident documented that a gunlock was provided and if firearm safety counseling was provided. Charts from June 2018 to present were reviewed with the plan to include all charts from June 2017 to April 2021.

Summary of Results

To date, 1,269 well child visits have been reviewed. Of these, residents documented that 7 gunlocks were distributed (0.6%) and firearm safety education was provided to 237/1269 (18.7%). Manual monthly gunlock counts demonstrate that, in reality, 86 gunlocks have been distributed from Jan 2020–April 2021.

Abstract #156 Figure 1

Proportion control chart of firearm education provided in PCC

Conclusions

The discrepancy in resident documentation and manual gunlock counts is likely secondary to a busy clinic and a difficult to navigate Electronic Medical Record (EMR) system. A PCC ‘spotlight’ was performed August 2020, which might explain the increase in gunlocks distributed the following month. A future PDSA cycle in December 2021 will include creating templates within the EMR to improve documentation. We hope to continue to expand the project to maximize firearm safety counseling and gunlock distribution in PCC.

#157  Evaluating pediatric resident satisfaction with scheduling and curriculum changes to the inpatient night float rotation

AY Hammouda*

JJ Burns

CR Antonetti

University of Florida, Pensacola, Pensacola, FL

Purpose of Study

The purpose of this study is to determine the impact of changes to the night float inpatient resident rotation on resident perceived patient safety, workload/efficiency, resident wellbeing, educational experience and interaction with the attending hospitalist.

Methods Used

Changes to the night float inpatient rotation for pediatric residents at Studer Family Children’s Hospital were made including:

1.expanding duration of the night rotation from 1 to 4 weeks

2.staffing with an additional senior resident

3.changing attending hospitalist coverage to outside the hospital instead of in-house

4.implementation of a night float curriculum with a structed syllabus addressing the most common pediatric clinical presentations encountered.

After these changes were made, a survey was conducted to evaluate the effect of changes in the specific components of night float to several important categories. The categories of questions included patient safety, workload and efficiency, resident well-being, resident education/teaching and interactions with the attending hospitalists. All questions were worded with a positive focus and the mean percent of residents who selected ‘strongly agree’ or ‘agree’ on the specific questions for each category were calculated.

Additionally, a separate questionnaire was given to senior residents only, to compare the current night float with the previous more traditional system.

Summary of Results

Results: Sixteen out of twenty-seven residents responded to the survey. Patient safety and workload/efficiency was rated as improved with the nightfloat changes whereas, wellbeing was very much rated as negatively affected by the changes due to the longer duration of night float with few in agreement that wellbeing was improved. (see Table).

Questions given to senior residents only, compared the new system to previous more traditional night float system. Percent ‘strongly agree’ or ‘agree’ were measured: 32% agreed or strongly agreed overall it was a better call experience for them, 32% agreed or strongly agreed they were more willing to teach with new system, only 32% agreed or strongly agreed a harder adjustment to sleep cycle with new system and 66% agreed or strongly agreed that fatigue was worse with the new system.

Abstract #157 Table 1

Percent of residents who ‘strongly agree’ or ‘agree’ that night float changes were an improvement by category

Conclusions

Changes in the night-float call schedule resulted in improved resident perceived patient safety and workload/efficiency but there were also problems with resident wellbeing. This was also reflected in the questionnaire given to senior residents except for sleep cycles which were less disrupted with longer time on night float block.

#158  Improving delivery of lactation trays to breastfeeding mothers

JB Hirst*

C Montcalm

A O’Connor

A Davis

MD Veters

S Berger

The University of Alabama at Birmingham, Birmingham, AL

Purpose of Study

Breastfeeding mothers of hospitalized infants are vulnerable to gaps in their own nutritional support, which can be detrimental to the growth, development, and overall health of pediatric patients. Our hospital provides meals, ‘lactation trays,’ to mothers breastfeeding their infants. However, an order for this tray must be placed. We noted a significant delay in a tray delivery to one mother and sought out data on how often this occurs and how to make improvements for breastfeeding mothers of infants on the Pediatric Hospital Medicine service.

Methods Used

Our study utilized the Plan Do Study Act method of quality improvement. Baseline data from September 2019 to March 2021, including demographics, was obtained looking at the time difference between admission orders and lactation tray order. If the time difference was <2 hrs, we scored this as a ‘yes.’ If the time was >= 2 hr or not ordered at all, we scored this as a ‘no.’ Data continued to be collected on a monthly basis. Our SMART aim: By December 2021, 75% of lactation trays for breastfeeding mothers on PHM teams will be ordered in <2 hrs from admission.

Interviews were conducted with members of hospital staff involved in admission, bedside care, and lactation consultation. A process map was developed to guide the team for interventions. Interventions to date: education to resident physicians regarding the project (March 2021); modification to infant diet order set (June 2021); education to resident physicians regarding order set changes (July 2021).

Summary of Results

Baseline data showed 45% of lactation trays were ordered in <2 hrs from admission. Demographic data showed no differences between the 2 groups with the exception of time of day (day versus night) with more trays being ordered in < 2 hrs during the day compared to night (p<0.01). Nurses ordered 63% of trays and physicians ordered 36% of trays. Following interventions to date, the% of trays ordered in <2 hrs has improved to an average of 70% with 76% of trays being ordered by physicians and 15% of trays being ordered by nurses. See control chart.

Abstract #158 Figure 1

Control chart illustrating most recently available data

Conclusions

This project is ongoing, but the improvement shown thus far is promising for systemic change that will be sustainable. We are optimistic based on our current trend that we will succeed in our SMART aim.

#159  What resources do parents use prior to their child’s pediatric emergency department visit

D Hutchinson*

J Davis

The University of Mississippi Medical Center, Jackson, MS

Purpose of Study

Among children presenting to a single, urban pediatric emergency department (PED), we sought to determine what information resources were used by parents prior to the visit and how useful these resources were to the child’s parent. We also investigated whether specific resource usage correlated with visit metrics including acuity scores, disposition and return visits.

Methods Used

Parents completed a brief survey during their child’s PED visit, reporting which of the following resources they used up to three days prior to their child’s ED visit: internet searches, health-related websites, social media, friend/family consultation (with and without medical training), and primary care provider (PCP) consultation (with and without an office visit). For each resource a parent used, they were asked to evaluate its influence and usefulness using visual analog scales (VAS [0–100], 0 = no influence/not useful).

Summary of Results

Parents (n=275) used an average of 1.48 (95% CI 1.32 – 1.65) information sources prior to their child’s PED visit. Internet resources (28.4%) and family/friend resources (with medical training: 22.2%, without: 27.6%) were used as commonly as primary care provider consultations (with office visit: 24.4%, without: 24.0%). Higher VAS ratings with positively skewed distributions were observed for parental ratings of family/friend consultations (with medical expertise) and PCP consultations, whereas family/friend consultations (without medical expertise) and web-based resources showed lower VAS ratings with normal or plateaued distributions. No significant correlations were observed between resource usage and visit metrics.

Conclusions

Parents frequently used a variety of resources prior to their child’s PED visit. Parents indicated that medically-trained family/friends as well as PCP consultations were more helpful and had a greater influence on care decisions when compared with other resources.

#160  Transitions for a pediatrics clinic and its patients: the quality improvement story

S Austin*

SA Hashimi

A Joshi

J Yaun

The University of Tennessee Health Science Center College of Medicine, Memphis, TN

Purpose of Study

The American Academy of Pediatrics (AAP), with endorsements from the American Academy of Family Physicians (AAFP) and the American College of Physicians (ACP) in the last twenty years, has emphasized the importance of standardized transitions of adolescent patients from pediatric to adult providers. The purpose of this quality improvement (QI) project is to analyze the current practices of the pediatric care providers at the UTHSC resident teaching practice in Memphis, Tennessee regarding the transition management of adolescent patients. The clinic services newborn to 18-year-old patients in a resident led clinic staffed by senior pediatricians. This data will be used to establish and implement a standardized transition policy for our primary care clinic.

Methods Used

The study is designed based on a traditional plan/do/study/act (PDSA) cycle. Initial chart review included all Well Child Checks (WCC) occurring at LeBonheur Children’s Hospital January – June 2021. A transition worksheet was subsequently created which includes the clinic policy on transition care, instructions for finding an adult provider, space to include medical history and treatment, and a list of local primary care providers. Education was provided to Residents and Attending Physicians in the clinic regarding the QI project goals and implementation. Further chart review was done on a quarterly basis following implementation to determine adherence to the new policy.

Summary of Results

Initial chart review shows significant room for improvement in adolescent transition planning. Initial data analysis shows that only approximately 20% of providers are introducing the topic of transition in WCC from 15 – 17 years of age. Age stratification revealed that even at 17 year old visits, only 51.6% of providers are discussing transition, compared to 0.01% at 15 year old WCC and 11.8% at the 16 year old WCC. We compared follow-up appointments for additional oppertunities to discuss transition. Further chart review will be aimed at analysis of data after implementation of transition documentation to all WCC during this time frame. The goal post-intervention will be 80% adherence to a standardized transition policy.

Abstract #160 Table 1

Initial rates of transition discussion and follow-up

Conclusions

The goal of this QI project is to develop and implement a standardized transition process at an academic pediatric center. Initial data shows significant room for improvement regarding transition discussion and planning. Implementation of a systematic transition policy at this primary care clinic is under further review to determine adherence to the new policy after the creation of the transition worksheet, provider education, and patient counseling.

#161  Delivery room admission of patients with congenital anomalies to expedite care in a level 4 neonatal intensive care unit

AR Karla*

KR Anderson

S Lopez

The University of Texas Health Science Center at Houston John P and Katherine G MGovern Medical School, Houston, TX

Purpose of Study

Admission of patients to a Neonatal Intensive Care Unit (NICU) generally begins after the neonate physically arrives in the NICU from Labor and Delivery (L&D). Similarly, for all patients at Children’s Memorial Hermann Hospital (CMHH), including neonates with congenital anomalies, the electronic health record (EHR) (including the medical record number [MRN]) is not created until the patient’s arrival to the NICU. This may result in delays in patient care outside of basic patient assessment and emergent procedures. To expedite patient care, we developed a quality improvement project to establish a process for creating EHRs in L&D for University of Texas Fetal Center (UTFC) patients. The project aims are to 1) evaluate the current NICU admission process at CMHH, 2) increase NICU patient EHR creation in L&D from <10% to 80%, and 3) expedite patient care after delivery (i.e., physical arrival to the NICU, creation of EHR, and patient care procedures).

Methods Used

This quality improvement project includes neonatal patients delivered at CMHH NICU with a prenatal diagnosis of congenital defects or fetal concerns requiring subspecialty or surgical subspecialty care. Baseline data was collected retrospectively from January 2021 to May 2021 for patients delivered at CMHH with NICU admission. The following data was collected: maternal and patient demographics, delivery information, major timepoints in the early care of the neonate (i.e., times of delivery, MRN creation, physical arrival in the NICU, routine procedures), and neonatal outcomes based on prenatal diagnosis (i.e., echocardiogram for congenital heart abnormalities). The same measures will be collected after each Plan-Do-Study-Act (PDSA) cycle of 3–4 months, and the data will be compared to pre-implementation baseline data. The project timeline includes at least 3–4 PDSA cycles, which will each be followed by focus group discussions for further implementation of improvements. PDSA Cycle #1 began in September 2021, and initial focus groups and surveys with CMHH stakeholders (i.e., NICU staff, L&D staff, transport personnel, charge nurses) involved in the admission process were completed prior to implementation.

Summary of Results

Pre-implementation data included 105 UT Fetal Center patients delivered between January 2021 and May 2021. Examples of fetal diagnoses included in the study were congenital gastroschisis, hydronephrosis, oligohydramnios, and intrauterine growth restriction. Stakeholder small-group discussions and surveys with transport team, NICU staff, and L&D nurses did not suggest barriers prior to PDSA Cycle #1. Subsequent PDSA cycles will include follow-up stakeholder discussions, greater standardization of measures recorded in the patient chart, and other improvements identified from data and feedback from previous cycles.

Conclusions

Conclusions are pending completion of study cycles.

#162  Clinical value of weekend eegs

K Khan*

A Wilner

The University of Tennessee Health Science Center College of Medicine, Memphis, TN

Purpose of Study

Although status epilepticus is often apparent clinically, non-convulsive status epilepticus may occur without clinical accompaniment, requiring an EEG for diagnosis. Lack of access to EEG coverage may result in missed cases or delays in treatment resulting in increased morbidity and mortality. Ideally, hospitals should have 24-hour EEG availability to diagnose NCSE. However, providing EEG services 24/7 can be challenging, especially for small hospitals with limited staff and budgets. In this study, we looked at the results of emergency EEGs performed over a 10-year period to determine the clinical utility of weekend EEGs in a 300-bed acute care hospital with the purpose of finding data in support of increasing availability of stat EEGs in the context of non-convulsive status epilepticus.

Methods Used

This retrospective anonymized study qualified and received IRB exemption. The technologist identified all patients who had weekend EEGs between 2010 to 2020. Patient charts were reviewed for a number of factors, including new diagnosis of NCSE, change in anti-seizure medication, and change in level of care. Demographic and clinical data were compiled in an electronic database.

Summary of Results

Out of 3,464 total EEGs done at our institution between 2010–2020, 45 (1.3%) were weekend EEGs. Of these, 27(60%) patients had records available for review. Two patients (7.4%) had NCSE, and three patients (11.1%) received a new epilepsy diagnosis. EEG results led to medication changes in 16 patients (59.3%). In 14 patients (51.9%), an ASD was added. In two patients (7.4%), ASD dosage was increased. In no case were ASD dosages decreased or discontinued as a result of the EEG. After the EEG, 10 (37.3%) patients were transferred to a different level of care; eight patients (29.6%) received a higher level of care, and two patients (7.4%) a lower level.

Conclusions

Our study demonstrates that stat EEG is clinically useful. As others have observed, the diagnosis of NCSE is essential to effective management. Our study found that weekend EEGs contribute to the diagnosis of NCSE in 7.4% of the patients observed. Our study also found a change of care (ASD change, level of care change) in 59.3% of patients. This suggests that the benefit of a stat EEG goes beyond the diagnosis of NCSE to management assistance that may improve patient outcomes.

#163  Obstructive sleep apnea screening in children: a quality improvement project

S Kilaikode1*

SA Hall2

1Ochsner LSU Health, Shreveport, Shreveport, LA

2LSU Health Shreveport, Shreveport, LA

Purpose of Study

Obstructive sleep apnea (OSA) affects approximately 3% of children. Disrupted sleep presents differently at different ages, making it difficult to recognize. Untreated OSA carries serious morbidity in neurobehavioral, cardiovascular, and somatic growth and development. Early identification and management of OSA is crucial to prevent adverse outcome. We conducted a quality improvement project to spread the awareness and increase the rate of OSA screening in pediatric clinic.

Goal

Increase and Standardize OSA screening in children who snore in the general pediatric clinic at Ochsner LSU Health, Shreveport, Louisiana.

Methods Used

Baseline data over 1 month was collected before any intervention to find rate of OSA screening. After that three PDSA cycles lasting 2 weeks each were performed testing 3 interventions in each cycle. Interventions included (in order performed) weekly emails, weekly slide show presentations, and Wall Posters to communicate with pediatric residents about OSA screening. An EPIC dot phrase containing the ’I’m Sleepy Questionnaire’ and the ’Epworth Sleepiness Scale’ was used for screening OSA. Ongoing data was collected till the end of the project timeline. If screened positive, recommended to refer patients for further evaluation (sleep study) and management.

Summary of Results

Baseline data showed 2.56% of children were screened for OSA. Increase in snorers screened from control data after each intervention is as follows: Emails (7.24%), Presentation (3.89%), Poster (9.44). Percentage increase in snorers referred from emails (2.56%), Presentation (3.89%), Poster (5.44%).

Conclusions

Timely screening and identification of OSA in children is important. However, this is not easy in a busy pediatric clinic. Our interventions were successful in showing improvement in rate of OSA screening among children in our clinic. Further measures will be required to demonstrate enhanced and ongoing improvement.

#164  Assessment of infant sleep habits while hospitalized in a non-birthing children’s hospital

C Kutz*

M Thompson

L DiAngelo

SR Arnold

J Yaun

The University of Tennessee Health Science Center College of Medicine, Memphis, TN

Purpose of Study

Sleep-related deaths account for 18.2% of all infant mortalities in Tennessee. In 2019, Shelby County had the highest number of infant sleep-related deaths in the state of Tennessee with 23 cases (22% of all infant deaths in the county). Most occur while in an unsafe sleep environment, including co-sleeping or from suffocation items in the crib. A committee was formed at our tertiary care children’s hospital to implement and model safe sleep practices. The purpose of this study is to evaluate the effectiveness of our safe sleep interventions by comparing type and proportion of unsafe sleep practices before and after implementation of a safe sleep policy and staff education.

Methods Used

Residents and medical students assessed infant (less than 6 months old) sleep practices with a 6-question survey evaluating sleep location, position, and presence of suffocation items. ICU and NICU patients were excluded. Baseline data was collected from May 2019 to January 2020, with the following interventions implemented thereafter: crib cards explaining safe sleep, [ASLR1] recommendations for removal of extra blankets and other items from the crib, an updated hospital policy based on American Academy of Pediatrics guidelines, and online training for staff. Follow up data was collected from May 2020 to October 2020. Further data collection was limited due to the COVID-19 pandemic until February 2021.

Summary of Results

Data was collected for 105 infants in the baseline group and only 29.5% were sleeping safely: 87.6% were in a safe position, 90.5% were in a safe location, but only 30.5% had no suffocation items in the crib. After safe sleep interventions were implemented, data from May to October 2020 in 46 infants showed a slight decline in safe sleep to 23.9%: 84.8% in a safe position, 89.1% in a safe location, and 26.1% with no suffocation items in the crib. Among 116 infants evaluated from February to August 2021, only 13% were sleeping safely: 83% in a safe position, 77% in a safe location, and 30% with no suffocation items in the crib.

Conclusions

Overall, infants tend to sleep in safe positions and locations in our hospital, but many continue to have suffocation items in the cribs. Differences in results of the two periods of follow up data could be related to a stricter definition of ‘overall safe sleep’ for survey responses. Due to lack of improvement after initial safe sleep policy implementation, new interventions, including requirement for a physician order for head of bed elevation, involvement of nursing staff as ‘Safe Sleep Champions’, and additional education on suffocation items are planned in order to improve safe sleep in our hospital.

#165  Picking up the pace – a quality improvement project

EW Larsen*

S Conner

M Condren

The University of Oklahoma – Tulsa, Tulsa, OK

Purpose of Study

For the 2020–21 academic year, the impact of the coronavirus disease 19 (COVID-19) pandemic on residency programs nationwide not only included decreased connectivity between residents, but also decreased involvement in communities. When restrictions were lifted, we anticipated resident engagement would be slow to return to normal. In response, our Pediatric Chief Resident organized monthly community involvement opportunities to promote engagement, and community service. The purpose of this quality improvement (QI) project was to increase resident engagement in the community through volunteer opportunities.

Methods Used

The goal for this QI project was to achieve 75% resident engagement in community-centered activities in a twelve-month period. To achieve this, the Chief Resident collaborated with community partners to plan monthly community-oriented activities. A total of twenty-five pediatric residents were invited to participate in these events on a voluntary basis. Participation in each event was documented and used to follow overall resident engagement over time. Cycle 1 consisted of 3 identical events in a 3-month period, which took place at a local farmers’ market as a public health initiative to educate families in the community about summer safety. Cycle 2 incorporated other opportunities to accommodate varying interests and schedules. These options included a Teddy Bear Clinic at a local park and a clothing drive for children attending a local summer camp for children who have experienced burns. Cycle 3, which is ongoing, includes new activities as well as a resident sign-up to provide a more formal invitation and commitment to participate.

Summary of Results

Prior to Cycle 1, some residents volunteered in the community on their own volition, but as a residency program, participation in formally organized community service activities was scarce. In Cycle 1, 4/25 (16%) of residents engaged; one PGY2, two PGY3, and the chief resident. With the incorporation of different activities in Cycle 2, resident engagement increased to 9/25 (36%). The same residents who participated in Cycle 1 also participated in Cycle 2, with the addition of two PGY1, two more PGY2, and one more PGY3. Offering a variety of service opportunities has proven to increase resident engagement. Cycle 3 is currently ongoing.

Conclusions

Community service restores the connectivity between residents and the surrounding community we serve. Our program’s resident engagement in the community is improving over time as more opportunities are offered. The goal of 75% resident participation has not yet been reached, but ideas for future community service activities are emerging. Realizing residents have different strengths and motivations has emphasized the importance of offering new activities that are appealing to residents who have yet to participate. Our hope is the overall success of this QI project will result in perpetual community involvement.

#166  A single-center quality improvement project for gastroschisis care

M Law1*

L Dalal1

C Domonoske2

M Forbes2

R Pandey1,2

W Li1

M Chang1,2

M Zambruni1,2

C Aneji1,2

1The University of Texas Health Science Center at Houston John P and Katherine G McGovern Medical School, Houston, TX

2Memorial Hermann Health System, Houston, TX

Purpose of Study

Gastroschisis (GS) is a congenital abdominal wall defect affecting 4 in every 10,000 babies. GS patients are managed in a neonatal intensive care unit (NICU) with surgical repair and medical therapy. However, there is significant variation in GS care provided within and between NICUs. For other patient populations like congenital diaphragmatic hernia, evidence shows that standardizing care improves patient outcomes. This quality improvement (QI) project aims to improve patient outcomes by standardizing multidisciplinary care of GS patients from birth to discharge at a single center.

Methods Used

We conducted a retrospective chart review of all GS patients from December 2008-May 2020 at Children’s Memorial Hermann Hospital (CMHH) in Houston, Texas to establish a baseline group (N=161). The outcomes tracked included type of gastroschisis (simple gastroschisis (SG) vs. complex gastroschisis (CG)), surgical closure type, antibiotic exposure, enteral and total parenteral nutrition (TPN), length of stay (LOS), and growth. Clinical guidelines (not shown here) were developed and implemented in November 2020. In PDSA cycle 1 (N=9), we compared outcomes of babies born 0–6 months after guideline implementation (November 2020-June 2021) to the baseline. Compliance of NICU teams to the new practice guidelines was also studied. Wilcoxon and Fisher’s exact tests were used to compare continuous and categorical variables, respectively.

Summary of Results

In the baseline group, there was variability in IV fluid management (types and total fluid volumes), type of feeding, antibiotic exposure, and laboratory testing schedule.

Cycle 1 showed some improvement in outcomes and a decrease in care variability. Baseline patients had a median TPN duration of 25.5 days (range=7–257), versus a median TPN duration of 23 days (range=12–141) in cycle 1. In the baseline, 97.5% of patients received at least one course of antibiotics and 41.6% received two or more. In cycle 1, 77.8% of patients received at least one course of antibiotics and 33.3% of patients received two or more. In baseline patients, median LOS was 39 days (range=13–291), versus median LOS of 27 days (range=17–180) in cycle 1.

Audit of the cycle 1 group showed variable compliance in some guidelines. Pain control using acetaminophen and initiating enteral feeds with expressed breast milk had 100% compliance. However, limiting days on initial antibiotic therapy had only 33% compliance, and initial type and volume of IV fluids had 67% compliance.

Abstarct #166 Table 1

Conclusions

There was some improvement in care variability after guidelines were implemented, and we revised some processes with this data. In November, we will compare patient outcomes and evaluate team compliance with new data from six months to one-year post-implementation.

#167  Decreasing unnecessary antibiotic exposure in post-surgical neonates: a quality improvement initiative

M McGowan*

T Cerisier

D Davis

C Welch

M Akinola

Wake Forest Baptist Medical Center, Winston-Salem, NC

Purpose of Study

Neonates are sometimes exposed to unnecessary antibiotics due to concerns for increased risk of infection related to immature immune systems. Studies have shown that prolonged, unnecessary antibiotic exposure in the newborn is associated with adverse consequences such as antibiotic resistance and antibiotic-induced microbiome alterations, increasing the risk of immune-related diseases in childhood. Reduction in antibiotic use in neonates with suspected early onset sepsis has been achieved by quality improvement studies but there is a lack of antibiotic stewardship related to type and duration in surgical infants. Our study aim was to decrease unnecessary post-operative antibiotic exposure in surgical neonates by implementing standardized post-operative antibiotic guidelines.

Methods Used

Post-IRB approval, a multidisciplinary healthcare team developed and implemented post-operative antibiotic guidelines. Baseline data was collected through retrospective chart review of neonates undergoing the following surgical procedures: gastroschisis repair, intestinal atresia repair, necrotizing enterocolitis (NEC) procedure, spontaneous intestinal perforation (SIP) procedure, and gastrostomy tube placement, between January 2017 and June 2020. Retrospective and prospective data collection included an infant’s gestational age, sex, birth weight, race, antibiotic type and duration, and reasons for guideline deviation (prospective data). Guideline compliance monitoring is ongoing using Plan-Do-Study-Act (PDSA) cycles.

Summary of Results

Of 134 neonates that had one of the surgical procedures of interest, 23 infants with gastroschisis repair had a median post-operative antibiotic duration (POAD) of 2 days (range 0–7 days); 25 infants with intestinal atresia repair had a median POAD of 1 day (range of 0–5 days); 37 infants had NEC procedures with median POAD of 5.5 days (range 1–14 days); 30 infants had SIP procedures with median POAD of 7 days (range 1–23 days); 49 infants had gastrostomy tubes placed with median POAD of 2 days (range 0–10 days).

Abstract #167 Figure 1

Post-operative antibiotic duration (days) for neonatal surgical procedures

Conclusions

Baseline retrospective data demonstrated variability in antibiotic type (data not supplied) and duration in post-surgical neonates even within the same surgical procedure. Next steps include monitoring guideline compliance to determine the need for any modification and additional provider education.

#168  Outcomes of hepatitis C virus testing at a safety net clinic in Appalachia

PS Means1*

SW McKenzie2

1Belmont Abbey College, Belmont, NC

2Providence Medical Clinic, Johnson City, TN

Purpose of Study

Hepatitis C Virus (HCV) is the most common bloodborne infection in the United States. HCV is a leading cause of liver related morbidity and is heavily prevalent in the Appalachian region. Tennessee in particular is in the top ten states for both prevalence of and population living with HCV infection. In an earlier study (J Invest Med 2019;67 (suppl 2):A74) we demonstrated a 10% incidence of HCV positivity within an unselected population presenting for primary care at safety net clinics. In this study, we present findings from one of those clinics for HCV rapid testing over a seventeen-month period.

Methods Used

Providence Medical Clinic serves many homeless and/or previously incarcerated patients with histories notable for injection and/or intranasal drug use. Over a seventeen-month period from December of 2019 to August of 2021, 97 patients were tested using Orasure© rapid HCV antibody test kits of which 12 were antibody positive (12.3%).

Summary of Results

To date eight of these 12 patients have received confirmatory testing showing active infection and seven of these have followed up for continuing care. Of those who followed up, four patients have begun therapy with glecaprevir/pibrentasvir or sofosbuvir/velpatasvir. One of these has fully completed therapy and cure of HCV verified. One patient has completed therapy but was lost to follow up before confirmation of cure. One patient completed six of twelve week of sofosbuvir/velpatasvir but stopped due to headache and was lost to followup. Another patient had no viremia and did not require treatment. Hepatitis B Virus Core antibodies were detected in two patients (16.7%), with no HBV antigenemia. No co-infection with HIV has been detected in the patient population of the clinic at this time.

Conclusions

In conclusion, rapid testing for HCV has been shown to effectively identify HCV infection among the population of a safety net clinic, providing an opportunity for potentially curative treatment.

#169  Improving pneumococcal vaccination rates among in-patient pediatric diabetic population – a quality improvement project

A Mirza*

A Jagadish

K Trimble

A Olanrewaju

LSU Health Shreveport, Shreveport, LA

Purpose of Study

To increase pneumococcal vaccination rate among pediatric diabetic patients admitted to the inpatient service by 90% in 6 months

Methods Used

Baseline data was collected by reviewing charts from last six months prior to start of the study (September 2020 – February 2021). In-patient diabetic admissions to the Pediatrics Ward and Intensive Care Unit were noted and their pneumococcal vaccination records were reviewed using LaLinks web portal.

Initial intervention was employed in March 2021. Residents were educated about guidelines on pneumococcal vaccination in diabetics via an oral lecture in morning conference and a smart phrase was created on the Electronic Medical Record to ensure uniform documentation that pneumococcal vaccination review was done and offered to patient if not previously vaccinated. Pharmacy also helped remind and prompt physicians to order vaccine for eligible patients.

Second intervention was employed in April 2021 and included a mass email to all residents about the guidelines and eligibility criteria for pneumococcal vaccination. Fliers were also posted in resident team rooms to serve as constant reminders. Third intervention occurred in July 2021 and included sending monthly reminder emails to residents on service.

Data gathering was accomplished by monthly chart review to assess number of diabetics admitted and whether pneumococcal vaccination was completed prior to discharge or not. Vaccination administration rate for each month was calculated by using the total eligible diabetic patients as a denominator to those who received the vaccine during their admission.

Summary of Results

A total of 63 charts were reviewed throughout the study duration. Review of data up to six months prior to initiation of study showed that pneumococcal vaccination rates ranging from 25%-67% per month with exception of November 2020 which was an outlier as only one diabetic patient was admitted who received the vaccine. After the First intervention in March 2021 vaccination rates remained steady at 67%. In April 2021, the second intervention took place and led to an overwhelming response with 100% vaccination rate. In the following months, we noted that our vaccination administration rate was dropping again and so third intervention was started in July 2021. Careful chart review revealed that vaccines were being ordered by residents but not being administered by nursing staff. Therefore, we were able to identify another area for improvement: nursing education. At this point, we are working on this and results for this intervention are yet to be published. After initiation of our project, average vaccination rates improved to 77% in the six months post intervention compared to 6-month average of 45% prior to intervention.

Conclusions

A simple intervention including resident education and liaison with pharmacy led to an increase in pneumococcal vaccination rates among diabetic patients. However, better rates can be attained by educating nursing staff.

#170  Developing a smarter and safer endotracheal tube

S Perez1,2*

T Nienaber1,2

A Abella Villafranca1,3

K Stephenson1,2

J Sanford1,3

K Sexton1,3

1University of Arkansas for Medical Sciences, Little Rock, AR

2Arkansas Children s Hospital, Little Rock, AR

3Institute for Digital Health and Innovation, Little Rock, AR

Purpose of Study

Uncuffed endotracheal tubes have been used in pediatric patients following concern for complications surrounding the endotracheal tube (ETT) cuff. It is postulated that high pressure causes venous blockade, leading to edema and/or necrosis causing airway edema, extubation failure, subglottic stenosis, etc. Current devices have not decreased complications significantly, potentially due to the cuff pressure remaining above central venous pressure. We hypothesize that measuring and decreasing pressure exerted by the cuff will decrease tracheal mucosal injury.

Methods Used

A prototype endotracheal tube was developed, integrating a sensor that can detect changes in pressure of the cuff and leak around the cuff. 16 New Zealand White (NZW) rabbits were equally randomized into 2 groups, comparing standard cuffed ETTs to our prototype. Intubation was performed with video assistance. ETT cuffs were inflated to eliminate leak by auscultation in the control group and based on signal output in the intervention group. Both groups were ventilated for 2 hours and vital signs were monitored. Subsequently the trachea was excised, fixed in formalin, and examined histologically. The percent surface area preserved was evaluated by generating two masks per slide, one covering all the undamaged area and the second one covering only the damaged area, and counting the colored pixels for each mask using a Python script.

Summary of Results

With 8 rabbits in each group, 4 died in the control group, 2 died in the intervention group, due to pre-existing illness. In the control group, 35% surface area of the trachea mucosa was intact, compared to 70% in the intervention group, which is greater than our hypothesized difference of 15%.

Analysis of the waveform data generated yielded the surprising results of our ability to use the sensor to monitor physiologic parameters, including respiratory rate, heart rate, and possibly blood pressure. The data also suggest that cuff shape may be as important as cuff pressure for creating a seal in the trachea and a dampening cuff pressure design is better compared to the traditional cuff design.

Abstract #170 Figure 1

Hematoxylin and eosin stained tracheal sections; preserved mucosa highlighted in red

Conclusions

Our prototype endotracheal tube decreases tracheal mucosal injury and may provide physiological monitoring capabilities. Further work needs to be done to determine the long term outcomes of this device.

#171  Enhanced auscultation with the eko core digital insert to calculate heart rate during neonatal resuscitation

EW Reynolds*

The University of Texas Health Science Center at Houston, Houston, TX

Purpose of Study

Hearing loss can limit a physician’s ability to auscultate sounds. In addition to age-related presbycusis, pathologic hearing loss can occur at younger ages. Devices that increase accessibility for hearing-impaired individuals may also be helpful to those with normal hearing. The Eko CORE Digital Attachment (Eko Devices, Berkley CA) is a device which attaches to a standard stethoscope and is capable of amplifying auscultated sounds and wirelessly connecting to a smartphone or tablet for visual display and automated heart rate (HR) calculation. The objective of this project is to determine if the Eko CORE Digital Attachment HR calculator is not inferior to calculation by other methods and may decrease the time it takes for a neonatal resuscitation team to calculate the infant’s HR during the initial steps of resuscitation when compared to standard auscultation or ECG or pulse oximetry (ECG or PO).

Methods Used

This is a single-center, observational study to compare 3 methods of determining the HR during neonatal resuscitation: 1) Standard Auscultation (SA), 2) Enhanced Auscultation (EA) with the Eko CORE Digital Attachment, and 3) ECG or PO. The study population consists of members of the neonatal resuscitation team when an infant requires more than the initial steps of resuscitation. Encounters are defined as each time the team leader asks for an assessment of the infant’s HR. The primary outcome of this study is time to estimate HR (T) during neonatal resuscitation by the 3 methods. Data collection will include: years of experience of the HR assessor, infant’s gestational age, birthweight and HR, and time to estimate HR by all three methods (SA, EA, ECG or PO) when available at each encounter.

Summary of Results

Of the first 5 encounters, TEA was unmeasurable 3 times. Of two of the 5 encounters with both TSA and TEA measurements, TEA was less than TSA(25 vs 36 and 7 vs 11 seconds). Mean TSA was 20.4 sec (range 11.0–36.0, n=5). Mean TEA was 16.0 sec (range 7–25, n=2). HR reported by SA was somewhat imprecise, with reports of ‘about 50’ or ‘65 to 70’. Whereas, HR reported by EA was precise, being 171 and 162 on the 2 occasions in which the automated system worked and exactly correlated with the HR displayed by ECG both times.

Conclusions

Enrollment is continuing with a goal of 150 encounters. Initial encounters show that the automatic algorithm in the Eko software is subject to interference from vigorous crying, lung sounds and ambient noise. When successful, EA was faster than SA for these few encounters. HR assessors are taking well over the 6 seconds recommended during NRP to calculate a HR. TSA and TEA can be expected to improve with practice. The sound amplification feature of the Eko device during SA is helpful to persons with normal hearing as well as hearing-impaired users. Although, some users found the amplification to be too loud. This project was sponsored by the American Academy of Pediatrics, Human Factors in Neonatal Resuscitation Grant Program.

#172  Pilot assessment of cleft lip and palate patients at a regional cleft center

SN Rimmer*

GK Fulton

LSU Health New Orleans, New Orleans, LA

Purpose of Study

Retrospectively assessed the cleft population at a regional children’s hospital and craniofacial team over a 30 month period using a standardized set of outcomes developed by the cleft team. Assessment characterizes the demographic factors and the distribution of cleft anomalies of our patient population. Surgical data: age at primary cleft lip (CL) repair, primary cleft palate (CP) repair, and alveolar bone graft (ABG) was also analyzed.

Methods Used

Electronic health records were queried for ICD-10 and CPT codes that correlated with cleft anomalies and surgical procedures of the lip and palate to generate a sample of 457 patients evaluated at the hospital over a 30 month period. Demographics, cleft characterization, and surgical procedures was analyzed using Microsoft Excel and compared to national statistics for the epidemiology and care timelines of CL±P.

Summary of Results

The sample identified as 85% Caucasian, 10% African American, 2% Asian, 2% Other, and 1% American Indian. 88% of patients reside in Louisiana. Payor status indicated 66% Medicaid, 32% Commercial, 1% Self Pay, and 1% Medicare. 43% presented with cleft lip and palate (CL/P), 38% cleft palate only (CPO), 12% cleft lip (CL), and 7% cleft lip/alveolus (CLA). 53% of patients were male and 47% female. Male patients presented with 54% CL/P, 28% CPO, 11% CL and 7% CLA. Female patients presented with 49% CPO, 29% CL/P, 14% CL, and 8% CLA. The average age at primary cleft lip repair was 5.2±2.6 months, primary cleft palate repair was 1.4±0.8 years, and alveolar bone grafting was 11.6±3.7 years.

Conclusions

Our population sample does match current national cleft characterization statistics: CP vs. CL±P, male to female ratios for CL±P and CPO.

Standard cleft care timeline recommendations in the US maintain that primary CL repair should be performed at 3–6 months, primary CP repair at 8–12 months, and ABG at 6–10 years. In our sample, time to surgery fell within the recommended timeframe for primary CL repair, but not for primary CP repair or ABG.

This pilot study serves to elucidate areas of delayed care to focus future interventions. Specifically, phenotypic cleft characteristics and demographic factors will be compared to age at surgery to illuminate populations that require more outreach, surveillance, and cleft care coordination.

#173  Predicting cervical spine injury in children

BT Robinson1,2*

R Kink1

T Jones1

R Williams1,2

1The University of Tennessee Health Science Center College of Medicine, Memphis, TN

2LeBonheur Children’s Hospital, Memphis, TN

Purpose of Study

The current trend in pediatric trauma is to minimize radiation exposure from imaging; c-spine injuries remain difficult to diagnose without computed tomography(CT). We sought to identify predictors for cervical spine injuries to determine which pediatric patients would benefit from a CT of the cervical spine for further evaluation.

Methods Used

All pediatric patients <18 years who presented to a tertiary freestanding pediatric level 1 trauma center over 5 years that had a cervical spine CT performed for trauma were reviewed. Descriptive statistics, univariate and bivariate analysis were performed.

Summary of Results

Overall 243 patients were identified with 41 patients diagnosed with a cervical spine injury. Though age and gender were not associated with injury, it was associated with Caucasian race. MVC was the most common mechanism of action (42%) followed by fall from heights and pedestrian versus automobiles (15%). The mechanisms of action (MOA) were also designated high versus low impact per ACLS guidelines. 66% of patients with C spine injuries were high impact. In a multivariable analysis race, transportation from a referring facility, altered mental status, intubation prior to arrival or during resuscitation, and impact of MOA (high vs. low) found to be significant predictors of cervical spine injury.

Conclusions

Cervical spine injury was associated with white race, impact of mechanism of action, intubation, transfer from another facility, neurologic deficit, and altered mental status. This is more consistent with the adult Canadian C spine rule more than the Nexus criteria due to the inclusion of impact of mechanism criteria in the Canadian C spine rule. This study showed that awake children without any neurologic findings who were in a low impact trauma have low risk of injury and might not benefit from a CT of the cervical spine.

#174  Pediatric multidisciplinary care huddle: improving quality, efficacy, and efficiency

C Roth*

S Sarkar

LSU Health New Orleans, New Orleans, LA

Purpose of Study

Multidisciplinary Care Huddle (MCH) serves as an important platform for communication and coordination of patient care. At our institution, MCH occurs twice weekly at 9 am via a virtual platform. During MCH, senior residents on the pediatric hospitalist teams present patient overviews to the multidisciplinary care team. A needs assessment regarding MCH revealed multidisciplinary team member concerns around timeliness and content covered and resident concerns around unclear expectations.

Methods Used

A checklist was created detailing the types of information team members used from MCH to perform their roles. Following collection and analysis of pre-intervention data, outcome measures were set as percent start time by 9:02 am and percent content communication of patient descriptions, plans of care, room numbers, and discharge dates. Balancing measures included average time per patient, resident satisfaction with efficiency of MCH, and resident perception that MCH interferes with their other clinical duties. Three Plan-Do-Study-Act (PDSA) cycles were completed. Interventions included scheduled times for each team to arrive at MCH, resident education on expectations, a posted guideline of information to be presented, phone reminders, and cues for resident start.

Summary of Results

Content coverage improved with increased presentation of room numbers (64% to 87%), patient descriptions (27% to 98%), plans of care (47% to 96%), and discharge dates (28% to 62%). Percent of time MCH started by 9:02 decreased from 62% to 28% for PDSA 1 but improved to 79% for PDSA 2 and PDSA 3. Resident perceptions regarding interference of MCH with clinical duties improved. However, average time per patient presentation increased from 34 sec to 41 sec, and resident satisfaction with efficiency of MCH decreased.

Abstract #174 Figure 1

Percent of time room numbers, patient descriptions, plans of care, and estimated discharge dates were conveyed during Multidisciplinary Care Huddle from April 1, 2021 to August 26, 2021

Conclusions

Resident education, an established and displayed guideline for desired information, and time reminders and cues improve content delivery, start times, and resident perception regarding interference with clinical duties. This process has the potential to be expanded to other subspecialty teams or to other facilities. Opportunities for interventions targeting efficiency of MCH also remain.

#175  Conducting a pediatric randomized clinical trial during a pandemic: a shift to virtual procedures

J Roberts1

S Chervinskiy2

R McCulloh3

J Snowden2*

T Phan4

E Dawley1

PM Darden2

V Reynolds4

C Lim5

L Pyles6

D Hubberd2

J Baldner2

L Lawrence2

A Davis7

1Medical University of South Carolina, Charleston, SC

2University of Arkansas for Medical Sciences, Little Rock, AR

3University of Nebraska Medical Center, Omaha, NE

4Nemours Biomedical Research, Wilmington, DE

5The University of Mississippi Medical Center, Jackson, MS

6West Virginia University, Morgantown, WV

7Kansas University Medical Center, Kansas City, KS

Purpose of Study

Prior to the COVID-19 pandemic, we initiated a randomized clinical trial for childhood obesity. The trial consented 131 and randomized 104; 6–12 year old patients who reside in rural regions in 4 member states (DE, NE, SC, and WV) of the ECHO IDeA States Pediatric Clinical Trials Network (ISPCTN) Approximately 6 weeks into the 10-week recruitment period, the trial was forced to pause all study activity due to the COVID-19 pandemic. This pause necessitated a substantial revision in recruitment and study methods to using virtual procedures. This descriptive paper outlines ways to recruit and manage clinical trial participants using technology to obtain informed consent, obtain height and weight measurements by video, and maintain participant engagement throughout the duration of the trial.

Methods Used

We reviewed multiple data sources to describe the transition to virtual study procedures. These include research electronic data capture (REDCap) surveys conducted both during the pause and at the completion of the study to identify readiness for each site to conduct virtual recruitment and other study procedures as well as at the end of the study to identify issues that each site encountered during the virtual phase of the project. We also reviewed meeting notes and study enrollment figures.

Summary of Results

The IRB approved study changes allowed for variability between clinical sites in terms of virtual communication platforms and methods for participant consent and height/weight assessment. Identified advantages of the study included ability to conduct visits during all times of the day or evening, and reduced travel requirements. Challenges included poor Internet reliability in some rural areas; additional participant contacts for consent and eligibility screening; shipping delays of materials; reliance on family to perform height and weight measures; increased costs for materials and shipping. Despite the added challenges, all sites were able to meet the study enrollment objectives. Flexibility was key in implementation of virtual procedures given the variations in site resources.

Conclusions

While each study site had certain challenges unique to their location during the pandemic, we also identified several common issues with the transition to remote procedures. Lessons learned from this study can assist other study groups in navigating challenges, especially when recruiting and implementing studies with a difficult to reach rural and underserved populations or during challenging events like the pandemic.

#176  Preliminary data collection for forthcoming evidence-based qi for the management of asthma exacerbation in the acute care setting

A Thompson*

S Martin

E Kornfeld

D Hahn

C Aston

M Naifeh

The University of Oklahoma Health Sciences Center, Oklahoma City, OK

Purpose of Study

An estimated 5.5 million children have asthma. Representing about 500,000 emergency department (ED) visits and 2 million office visits; posing a large financial burden on our healthcare system. To examine the quality and equity of asthma care at the Oklahoma Children’s Hospital (OCH).

Methods Used

We included all visits from July 2019 and September 2020 with a primary diagnosis of asthma. Exclusion criteria included another chronic lung disease. To evaluate adherence to EPR-3 NAEPP 2007 guidelines we examined use of: supplemental oxygen, short acting beta agonist (SABA), ipratropium bromide, systemic steroids, close follow up, asthma education, asthma action plan (AAP) and inhaled corticosteroids (ICS). We examined the impact of our asthma order sets and equity of care using race and insurance provider.

Summary of Results

611 unique patients with 731 visits were seen during the study period. The majority of patients had a single visit (85%) with 10% having 2 visits and 4% having more than 2 visits. Patients were more likely to be male (57%) and older than 5 (54%). 49% of patients were black, 27% white, and 24% Hispanic. Of the 731 visits included, 74% were ED only, 16% required supplemental O2, and 6% required PICU care during admission. 79% of patients received a strong recommendation for close follow-up with their PCP, only 54% received an AAP, and 31% received a prescription for ICS. Patients who were admitted to the hospital were more likely to be discharged with ICS (73% vs 16% p<.001), receive an AAP (94% vs 40% p<.001), and a follow-up appointment (96% vs. 73% p<.001). An asthma order set was used 56% of the time, when an asthma order set was used patients were more likely to receive an AAP (p<.001), and follow-up appointment (p<.001). None of the patients seen by the asthma educator had more than one visit during the study time period (p<.001). Of note, patients identified an urgent care center (UCC) as their PCP 53% of the time, these patients were more likely to be seen in the ED only (86% vs 62% P<.001) and have more than one visit (15% vs 10% p=.058). With regards to race/ethnicity, more than 95% of Black and Hispanic children were treated in ED only while 70% of white children were seen in the ED only. There was no difference in AAP and close follow-up by race. Black and Hispanic children were more likely to have more than one visit during the study time period than white children (18%, 16% vs. 6% p=.005).

Conclusions

Our treatment of acute asthma exacerbation was in line with current guidelines. Opportunities exist in assuring appropriate long-term care of asthma including: use of an AAP, establishing close follow-up and prescribing ICS especially in patients only seen in the ED. A large percentage of our population identified an UCC as their PCP suggesting the need to assist patients in finding a PCP. Of note, more frequent use of an asthma educator may decrease our revisit rate for asthma.

#177  Icu de-escalation times pre and post transitionary team addition: insight to a possible quality improvement project

E Villemez*

C Hebert

LS Engel

S Sanne

LSU Health New Orleans, New Orleans, LA

Purpose of Study

On average, an ICU uses three times as many nursing hours per patient compared to hospital wards. Over fifty percent of the direct costs of maintaining an ICU can be attributable to this. When a patient continues to be provided with a resource that is no longer applicable to their needs, medical waste occurs. Nationwide, hospitals are beginning to implement variations of ICU step-down and transitionary care teams.

Methods Used

In 2018, the Section of Hospital Medicine within the Department of Medicine at LSUHSC Health Sciences Center in New Orleans implemented a dedicated medical team for the transition of care of patients being de-escalated from the ICU. The time delay between the request for de-escalated care and the actual de-escalation of care was recorded and analyzed pre and post the addition of a transitionary team with 50 patients in each group.

Summary of Results

After the implementation of a dedicated team, ICU step-down time was reduced by 33%. All delays post addition occurred despite adequate response time from the new care team suggesting outside or non-provider related factors.

Conclusions

The dedicated internal medicine team responsible for ICU transitions clearly reduced length of stay in the MICU. Early discharges from the MICU to the medical ward reduces costs. A previous study demonstrated that Hospital length of stay decreased without effecting mortality when an MICU based transition team continued to follow patients for 24 hours after transfer to the medical ward. The model studied here reduces stress on the MICU physician work force. We suggest further studies in the UMC ICU de-escalation of care process to reduce costs associated with long ICU step-down delays. The effects of this patient care model on other factors, including length of stay, re-admission and mortality, also require further study.

Hematology and oncology

Joint plenary poster session and reception

4:30 PM

Thursday, February 10, 2022
#178  Evaluation of quality of life data reporting in metastatic kidney cancer trials

FG Bell*

JC Henegan

University of Mississippi, University of Mississippi, University Park, MS, US, Jackson, MS

Purpose of Study

Health related quality of life (HRQoL) results provide information as to the general impact on a patient’s life that a therapy may have. Both statistical and clinical significance should be reported for HRQoL but not all studies present both pieces of information.

We sought to identify if there is a difference in the frequency of the presentation of statistical significance of HRQoL versus clinical significance of HRQoL in phase III studies of investigational agents in metastatic renal cell carcinoma reporting longitudinal results of overall HRQoL.

Methods Used

A Medline search using the MESH term ‘Kidney Neoplasms’ and filtering for Phase III clinical trials was conducted. The articles were reviewed and those studies which included longitudinal results of overall HRQoL in phase III studies of investigational agents in metastatic renal cell carcinoma were included in the analysis. The binomial test was used to calculate a z-score, with the frequency of the reporting of statistical significance used as the expected probability of reporting of clinical significance.

Summary of Results

31 phase III trials were able to be included in the analysis. Of these, 26 (83%) reported if a result was statistically significant and 21 (68%) reported if a result was clinically significant. Due to the small sample size the data did not meet the requirements for a z-test.

Conclusions

There is a nominal increase in the frequency of the presentation of statistical significance of HRQoL versus clinical significance of HRQoL in phase III studies of investigational agents in metastatic renal cell carcinoma reporting longitudinal results of overall HRQoL. However, the small sample size prevented the planned statistical analysis from being able to be reliably performed.

#179  Cardiomyopathy after doxorubicin

C Bergeron*

A Garcia

LSU Health New Orleans, New Orleans, LA

Purpose of Study

To highlight the importance of a thorough work-up for new onset heart failure in patients treated with doxorubicin

Methods Used

Review of Electronic Health Records and literature review

Summary of Results

A 59-year-old female with PMH of hypertension was diagnosed with left breast cancer. She underwent a modified radical mastectomy and pathology showed a stage III breast cancer. Transthoracic echocardiogram (ECHO) showed left ventricular (LV) hypertrophy with normal cardiac function (left ventricular ejection fraction, LVEF > 55%). Electrocardiogram (ECG) showed LV hypertrophy and no additional abnormalities. She started adjuvant chemotherapy with dose-dense doxorubicin (D), cyclophosphamide, and paclitaxel. Cumulative dose of D was 240 mg/m2. Two months after completing D, she presented with tachycardia. She reported 5 months of progressive dyspnea since beginning chemotherapy, followed by an acute worsening of dyspnea 2 weeks prior to presentation.

At presentation, brain natriuretic peptide was elevated. CXR showed signs of volume overload. ECG showed anteroseptal infarct of undetermined age. ECHO showed a LVEF of 25–30%. The patient was diagnosed with new onset heart failure (HF) with reduced ejection fraction (HFrEF) secondary to doxorubicin cardiomyopathy (DCMP). Following stabilization, she was discharged on goal-directed medical therapy for HFrEF and referred for follow-up with cardiology. At follow-up, nuclear stress testing revealed a large mid-distal defect in the anteroseptal wall corresponding to left anterior descending artery (LAD) territory infarction. Left heart catheterization and coronary angiography showed 100% occlusion of the LAD and 80% of the left circumflex artery, suggesting ischemic cardiomyopathy as the true cause of this patient’s new onset HFrEF.*

DCMP is a well-described cause of dilated cardiomyopathy. Dilated cardiomyopathy is defined by ventricular dilatation with systolic dysfunction, in the absence of coronary artery disease, hypertension, valvular disease, or congenital heart disease. The absence of these conditions is required for the diagnosis of dilated cardiomyopathy and subsequently DCMP. Risk factors for DCMP include age (> 65 years), female gender, preexisting cardiovascular disorders, hypertension, smoking, obesity, diabetes and high cumulative dose. It is usually classified as acute (occurs during treatment), subacute (detected within 1 year) and chronic (detected years after exposure). The prognosis of DCMP is poor and therefore it is essential to rule out treatable causes of HFrEF.

Conclusions

Cardiomyopathy is a well described, but uncommon side effect of D. Symptomatic HF is seen in approximately 1% of patients. Our patient had several risk factors for DCMP. This case highlights the importance of thorough evaluation for the cause of newly diagnosed HF even when the etiology seems straightforward. While D is a well described caused of dilated cardiomyopathy, ruling out more common causes of LV dysfunction are necessary to make the diagnosis.

#180  Leukemia cutis: an uncommon presentation of acute myeloid leukemia

D Beyer*

M Lieux

B Van Court

C Pham

C Van Dreumel

DA Van

SM Ford

R Foret

LS Engel

S Sanne

LSU Health New Orleans, New Orleans, LA

Case Report

Introduction

Leukemia Cutis is a rare condition characterized by infiltration of neoplastic cells into the epidermis and dermis with a characteristic dermatologic appearance. Leukemia cutis generally portends a poor prognosis in the setting of newly diagnosed blood cancers.

Case

A 31-year-old man without past medical history presented after 1 month of progressive dyspnea on exertion, worsening fevers, thirty pound unintentional weight loss and diffuse rash on all extremities and trunk. Physical exam was significant for fever, tachycardia and scattered petechia and erythematous papules and macules on his bilateral upper and lower extremities. Initial laboratory findings were significant for leukocytosis (222,000) with 94% immature mononuclear cells and thrombocytopenia (8,000). Punch biopsy was performed and pathology returned leukemia cutis with myeloid features. Bone marrow biopsy was significant for acute myeloid leukemia with 86% large blastoid cells. Patient was started on induction chemotherapy with cytarabine and idarubicin resulting in slow resolution of rash.

Discussion

Leukemia cutis is a challenging diagnosis and rare presentation of leukemia. When coupled with a new diagnosis of acute myeloid leukemia, as in this patient, portends a worse prognosis. This patient presented in blast crisis with cutaneous involvement and new diagnosis of acute myeloid leukemia was made promptly based on bone marrow biopsy results. Prompt dermatologic consultation resulted in diagnosis of leukemia cutis based on punch biopsy. After initiation of induction chemotherapy (cytarabine and idarubicin), his cutaneous lesions subsided significantly.

#181  Cobalamin deficiency masquerading as possible thrombotic microangiopathy

L Bradley*

E LeJeune

The University of Mississippi Medical Center, Jackson, MS

Case Report

Prompt recognition of Thrombotic Thrombocytopenic Purpura (TTP) is of utmost importance for clinicians considering the near 100% mortality rate associated with delayed treatment. Cobalamin (B12) deficiency is a rare cause of hemolytic anemia and can sometimes mimic the presentation of TTP. Though an infrequent diagnosis, pseudo-thrombotic microangiopathy (TMA) can in some cases result in inappropriate treatment with plasmapheresis if not distinguished from true TTP early. Here we present a case of severe B12 deficiency causing a TMA like syndrome.

58 year old African American male presented to the emergency department complaining of shortness of breath. His laboratory values were discovered to be white blood cell count 9.89 TH/cmm with normal differential, hemoglobin 5.7 g/dL, hematocrit 18.1%, platelet count 75,000 TH/cmm. He was admitted to the internal medicine service for further evaluation. Further workup revealed MCV of 127 fl, LDH >3500 U/L, Haptoglobin <10, and Total bilirubin of 2.22 mg/dL with indirect bilirubin 1.80 mg/dL. Reticulocyte count 1.2%. Direct antibody coombs test was negative. Serum B12 level was 150 with Methylmalonic acid of 5.52umol/mmol. On peripheral blood smear 1–3 schistocytes per high power field, rare tear drop cells, and numerous hypersegmented neutrophils were observed. ADAMTS-13 activity level was 78%.

Plasmapheresis was held and B12 1000 mcg IM was given daily for 7 days, then weekly for 4 weeks, followed by monthly indefinitely. On hospital follow up 6 weeks later patient had complete resolution of his anemia and thrombocytopenia. Serum B12 level was >500. He was referred to gastroenterology for further evaluation of suspected B12 malabsorption etiology.

Though rare, B12 deficiency causing hemolytic anemia is a known disease process and should be considered in the differential for hemolytic anemia. The pathogenesis of low B12 causing hemolytic anemia is uncertain but it has been demonstrated in vitro that elevated levels of homocysteine can lead to endothelial damage likely resulting in hemolysis. Early recognition of this patient’s pseudo-TMA likely shortened his hospital stay and prevented unnecessary initiation of plasmapheresis.

#182  Correlation between intubation and palliative care: what is the trend?

OH Brunson*

The University of Mississippi Medical Center, Jackson, MS

Purpose of Study

Over the last twenty years there has been an increase in the number of endotracheal intubations.1 In that same time period, palliative care has emerged with a focus on quality of life and alleviating suffering in patients with chronic, severe illness. More recently societal guidelines, including the American Society of Clinical Oncology, have recommended early integration of palliative care. 2,3 Hypothetically, earlier goals of care discussions could lead to less invasive interventions, such as intubation. With this focus on earlier intervention, we aim to study the correlation between inpatient palliative care consultation and intubations at our institution.

Methods Used

Utilizing a function of EPIC electronic medical record SlicerDicer, we were able to identify patients admitted to the University of Mississippi Medical Center with a co-occurrence of intubation and palliative care consult, examine patient demographics and calculate relative risk (RR).

Summary of Results

We first looked at intubations, which increased by 136% from 2012–2016 with the rate of change from 2017–2020 varying by only 1–2% a year. Next, we looked at palliative care consults. Since inpatient palliative care became available in 2017, the number of consults increased by 39% (264 to 367). We then analyzed 99,622 admissions from 2017–2020 to look for co-occurrences of intubation and palliative care consultation during the same admission. In the general population, excluding patients with a cancer diagnosis, co-occurrences increased by 86% from 2017–2020 (23 to 43). In cancer patients, co-occurrences increased initially by 600% from 2017–2019 (2 to 14), but then decreased by 35% in 2020. Based on this data, the RR of intubation and a palliative care consult during an admission was higher in non-cancer patients than cancer patients (RR 5.8, RR 2.3 respectively). The highest RR was seen in non-cancer patients less than 30 and 50–70 years old (RR 13.6, RR 7.8 respectively).

Conclusions

Overall, there was an increase in co-occurrences of intubation and palliative care consultation. This could be attributed to a growing palliative care service, but the excess RR in younger, non-cancer patients may point to the palliative care team being increasingly utilized in the acute, critically ill patient. Notably, co-occurrences in non-cancer patients increased compared to cancer patients in 2020, likely due to Sars-Cov2. Though co-occurrences increased in cancer patients overall, the decreased RR compared to non-cancer patients is promising. This could point to more sub-specialist involvement in inpatient palliative discussions and/or earlier goals of care discussions. There is still work to be done to emphasize earlier goals of care discussions in chronic illnesses, which could ultimately lead to a decrease in the number of co-occurrences of intubation and palliative care over time. Further investigation is needed to follow this trend.

#183  Diagnostic dilemma: primary orbital squamous cell carcinoma or not?

VK Carey1*

J Steyer1

P Goel1

A Ananthula1

SK Halat2

R Walvekar1

R Chowdry1

1LSU Health New Orleans, New Orleans, LA

2Tulane University School of Medicine, New Orleans, LA

Introduction

Primary orbital squamous cell carcinoma (SCC) is extremely rare with only 9 cases reported in literature. Secondary squamous cell carcinoma of the orbit is more common, accounting for 6.8% of orbital tumors and is usually the result of local invasion from a cutaneous primary (eyelid, conjunctiva, lacrimal gland, sino-nasal tract etc.), perineural invasion or distant metastasis. Here, we present a case of SCC of the right orbit which poses a diagnostic challenge as to the origin of the tumor and optimal management strategy.

Case

The patient is a 60-year-old male with a history of right eye injury and subsequent blindness who presented with a large right orbital mass which has been growing for a few months. The patient was struck in his right eye with metal debris several decades ago. Biopsy confirmed a diagnosis of moderately differentiated squamous cell carcinoma. Within a month of presentation, the mass continued to further grow rapidly with involvement of the entirety of the right orbit with proptosis. Ulceration along the medial canthus were noted with intermittent mild bleeding. The patient was unable to move his eyelids. CT of the orbits demonstrated a large right orbital mass extending into orbital apex with intraconal & extraconal involvement extending into the supraorbital & infraorbital fissures. The globes & optic nerve were not identified. MRI of the brain was negative for intracranial involvement. CT Chest, Abdomen, Pelvis was negative for any distant metastasis. Decision was made to proceed with right orbital exenteration, parotidectomy, modified radical neck dissection, and free flap reconstruction. Histopathology showed a 6.1 cm invasive squamous cell carcinoma with tumor invasion into the globe, extraocular muscles, orbital fat and optic nerve. Lymphovascular and perineural invasion was present. All margins were negative for malignancy. He is staged as pT4aN0M0 SCC of the right orbit. He has tolerated surgery well and plan is to start adjuvant chemo-radiation with weekly cisplatin given the risk of recurrence in his case.

Discussion

The orbit does not contain squamous epithelium, which accounts for the rarity of the disease. Case reports have detailed primary orbital SCC from dermoid cysts, lacrimal gland cysts with squamous metaplasia and conjunctival cysts occurring after ocular surgery. Squamous metaplasia is a consideration in this patient secondary to chronic irritation from his eye injury. In further review of existing literature, we inferred that we might not be able to confirm where the SCC originated in such cases with de novo orbital mass. Orbital SCC is usually treated with surgery with radiotherapy or chemoradiation depending on the extent of tumor invasion and risk of recurrence.

#184  Primary pulmonary melanoma presenting as brain metastasis

KJ Clay1*

BS Ross2

F Joiner1,2

1The University of Mississippi Medical Center, Jackson, MS

2G.V. (Sonny) Montgomery VA Medical Center, Jackson, MS

Case Report

Melanoma, a malignant tumor of pigment-producing cells, is a potentially fatal neoplasm most commonly arising from a cutaneous origin. However, cases arising from mucosal, ocular, and visceral sites are described. Here, we report a case of a primary melanoma of the lung with pulmonary and brain metastases.

A 42-year-old male with diabetes mellitus and a tobacco use disorder presented to the emergency department after a five-minute episode of aphasia associated with perioral tingling. Upon arrival, he was able to speak clearly, and he had no focal neurologic deficits. The remainder of his review of symptoms and physical exam was unremarkable. The differential diagnosis included seizure or transient ischemic attack. A non-contrast head CT revealed an acute focal hemorrhage within the left parietal lobe with a small subarachnoid component. MRI imaging further demonstrated a 1.5 cm rim-enhancing mass concerning for hemorrhagic metastasis. He was admitted. An awake craniotomy with tumor resection revealed a high-grade malignant neoplasm positive for MART1 and SOX10 and negative for GFAP, TTF-1, CK7, CK20, EMA, and CD34, which favored metastatic melanoma. This lesion was BRAF V600E positive and NRAS negative. Subsequent PET scanning revealed multiple FDG-avid pulmonary nodules, with an endob ronchial mass amenable to biopsy. Bronchoscopic biopsy of this mass demonstrated malignant cells positive for MART1 and SOX10 consistent with malignant melanoma and suggested that this was the primary lesion. A specialty-conducted dermatologic evaluation failed to reveal other potential primary sites. Initial therapy consisted of gamma knife radiation to the post-resection brain tumor bed and immunotherapy with nivolumab and ipilimumab. Two months later, an MRI showed multiple new intracranial metastases, and a PET scan showed an increase in the size of the endobronchial lesion and an increase in the number and size of the metastatic lung nodules. The patient has completed four cycles of nivolumab/ipilimumab and is being evaluated for dabrafenib/trametinib combination therapy.

Primary malignant melanoma of the lung is rare, accounting for 0.01% of all lung neoplasms. Though the pathogenesis of these tumors remains obscure, it is believed that primitive melanoblasts may migrate to the viscera during embryogenesis. New therapies have emerged for metastatic cutaneous melanoma that target specific kinases and protein receptors such as PD-1, CTLA-4, BRAF, and MEK. However, there is a paucity of data on the efficacy and safety of these treatments with non-cutaneous metastatic melanoma. This case illustrates the diagnostic and therapeutic challenges associated with this rare pathological entity.

#185  Stage ii endometrial carcinoma with false positive para-aortic lymphadenopathy: the importance of pretest probability

JA Cooper*

A Garcia

LSU Health New Orleans, New Orleans, LA

Purpose

Case report to increase awareness of pretest probability in the presence of abnormal imaging findings

Methods

Review of Electronic Health Records and literature review

Results

This patient is a 61-year-old female with a history of intermittent vaginal spotting since her early 50’s. On physical exam, normal vaginal atrophy was observed, and bi-manual recto-vaginal exam showed normal-sized uterus without adnexal masses or nodularity. A Pap-smear with dilation and curettage revealed endometrial carcinoma, endometrioid type with squamous differentiation. Hysterectomy with bilateral salpingo-oophorectomy, bilateral pelvic sentinel biopsy, and bilateral pelvic lymphadenectomy was performed. Pathology showed a grade 1 endometrioid carcinoma with greater than 50% myometrial invasion. The cervical stroma contained a 2 mm focus of tumor, and all 18 pelvic lymph nodes were negative meriting a diagnosis of Stage II endometrial carcinoma. Vaginal cuff brachytherapy, a non-morbid treatment, was offered as adjuvant therapy.

Staging PET/CT scan showed a hypermetabolic left para-aortic lymph node at the renal level suspicious for active neoplastic disease. Based on this the recommendation for adjuvant treatment was changed to extended field radiation and systemic chemotherapy, a treatment associated with significantly more toxicity. However, it was discussed that this was an unusual presentation and further evaluation was required. A CT-guided biopsy of the left para-aortic lymph node was performed and showed no malignant cells. Patient received vaginal brachytherapy. PET/CT three months later showed persistent, unchanged metabolically active left para-aortic lymphadenopathy. A repeat biopsy showed no malignancy. The scan also showed persistent chronic nephrolithiasis and obstructive uropathy of the left kidney with severe parenchymal atrophy. Uterenoscopic stone extraction with holmium laser lithotripsy and stent placement was performed. Patient remains without evidence of cancer recurrence 30 months after surgery.

Conclusions

This case shows the importance of using the pre-test probability when interpreting test results. PET/CT scan is highly sensitive and specific for endometrial cancer. However, para-aortic lymph node metastasis without pelvic lymph node metastasis from endometrial carcinoma is very rare (low pre-test probability). This low pre-test probability raised the concern about the PET/CT findings. The patient was spared the toxicity of unnecessary chemotherapy and extended field radiation. It was concluded that the findings on PET scan were related to the inflammatory process in the kidney associated with the nephrolithiasis and chronic obstructive uropathy. This case shows the importance of taking into the account the pre-test probability when interpreting any test.

#186  A different take on cardio-oncology: metastatic angiosarcoma presenting as a primary cardiac tumor

C Cutrer*

JC Henegan

The University of Mississippi Medical Center, Jackson, MS

Introduction

Cardiac tumors are very rare, mostly presenting as distant metastases. Only about 0.5% present as primary cardiac tumors and of those, the majority are benign, typically atrial myxomas (1). Angiosarcomas can rarely present as primary cardiac tumors and tend to be very aggressive. We present a case of a patient who presented with new coronary artery disease complicated by recurrent pericarditis and ultimately found to have metastatic angiosarcoma originating from the right atrium.

Case Report

Our patient is a 57 year old African-American male with a past medical history of hypertension and poly substance abuse who initially presented in January 2021 with chest pain and found to have acute coronary syndrome. He was taken for cardiac catheterization and percutaneous intervention (PCI) was performed with stent placement in left anterior descending (LAD) artery. A few weeks after his PCI, patient was readmitted for pericarditis and also complained of fatigue and weight loss, so computerized tomography of the chest, abdomen and pelvis (CT CAP) was obtained to rule out malignancy and it did not show any evidence of disease. Over the next few months, our patient had recurrent chest pain and weight continued to decline as fatigue worsened. He finally represented to the emergency room in July 2021 with chest pain and CT CAP was again obtained, only this time showing a large cardiac tumor abutting the right atrium and ventricle and evidence of metastatic disease throughout his vertebra, lungs and liver. Patient was referred to Oncology and had a biopsy of a bone lesion done via interventional radiology (IR), which showed metastatic angiosarcoma. We discussed palliative chemotherapy with single agent paclitaxel and initially patient agreed but there were delays over the next few weeks. By the time patient was able to make it back to clinic for treatment in early September, he had lost considerable weight and was wheelchair or bed bound for the majority of the day due to fatigue, weakness and pain. After further discussion, he decided to pursue hospice at home with family.

Discussion

Primary cardiac tumors are rare but when present, can be very aggressive. Angiosarcomas account for about 30% of primary malignant cardiac tumors and are typically very aggressive with a median overall survival of 4–6 months when unresectable (2). Typical treatment is surgical when feasible but chemotherapy and radiation therapy are often used, as well. Chemotherapy typically consists of taxane or anthracycline-based regimens (3). A small single arm study demonstrated a 25% (4/16) response rate for angiosarcomas to the combination of nivolumab and ipilimumab after prior chemotherapy which may lead to evaluations of this immunotherapy combination in first line therapy (4). In summary, prompt diagnosis and multidisciplinary treatment planning is important when taking care of patients with primary cardiac tumors.

#187  Pseudothrombotic microangiopathy and pancytopenia as a rare presentation of vitamin b12 deficiency

M Darweesh*

F Ghanem

R Musa

D Nunley-Gorman

East Tennessee State University James H Quillen College of Medicine, Johnson City, TN

Case Report

A 62-year-old male presented to our hospital with a few days of worsening dyspnea, associated with numbness in the left lower extremity, dizziness and transient brief chest pain that was described as a sharp intermittent pain. He denied any fever, chills, nausea, vomiting, diarrhea, headache, or recent ill contacts. The patient also denied any family history of blood or bone morrow disease. He had been released from incarceration 2 days prior to the presentation.

Complete blood count revealed pancytopenia with hemoglobin of 6.8 g/dL, MCV of 112 fL, white blood cell count of 1.2 K/uL, and platelet count of 78 K/uL. The patient was transfused with packed red blood cells and then admitted to the inpatient medicine ward for further treatment and evaluation. Blood smear confirmed the pancytopenia with severe neutropenia, macrocytosis, and moderate thrombocytopenia. In addition, it showed erythrocytes with marked poikilocytosis including occasional schistocytes and teardrop cells.

His lab investigations were notable for B12 level below 150 pg/mL (Normal range 211–911), fibrinogen of 144 mg/dL , haptoglobin less than 10 mg/dL, LDH of 1013 U/L. Other lab studies including troponin, ferritin, TIBC, serum iron, vitamin B1, PT/INR, PTT, SPEP, COVID-19, EBV, CMV, HIV, Hepatitis A, B, and C were all unrevealing. Abdominal ultrasound was significant for splenomegaly. CT head and chest x-ray were unremarkable. After starting treatment with cobalamin therapy, the patient has shown improvement in terms of cell counts, resolution of hemolysis. He also reported significant improvement in tingling and dizziness. All this confirms the diagnosis.

Vitamin B12 deficiency manifestations can vary between asymptomatic, mild, and severe. In our case, the patient presented with pseudothrombotic microangiopathy and pancytopenia. Both are rare and serious manifestations of vitamin B12 deficiency. Physicians should be aware of cobalamin deficiency as one of the etiologies for pancytopenia and pseudothrombotic microangiopathy. Therefore, an early recognition and treatment is crucial.

#188  Catastrophic antiphospholipid syndrome collides with refractory secondary evans syndrome: a therapeutic dilemma

H Daugherty*

C Hitchcock

GD Gibson

C Milner

The University of Mississippi Medical Center, Jackson, MS

Case Report

Antiphospholipid syndrome (APS) is an autoimmune disorder characterized by both venous and arterial thromboses in the setting of persistent antiphospholipid autoantibodies. APS can be associated with mild thrombocytopenia but has rarely been seen in combination with Evans syndrome (autoimmune hemolytic anemia, immune thrombocytopenia and/or neutropenia). Here we present a case of the therapeutic dilemmas faced when these two syndromes combine.

A 36-year-old female with antiphospholipid syndrome, cirrhosis due to Budd-Chiari syndrome (BCS), and recurrent pulmonary emboli on anticoagulation presented with right upper quadrant abdominal pain and shortness of breath in addition to a reported six-month history of fatigue, fevers, night sweats, and unintentional weight loss. The patient was pancytopenic and coagulopathic with elevated but stable liver enzymes of a mixed injury pattern. Imaging confirmed known hepatic venous thrombosis with progressive hepatic infarctions. The patient was not a candidate for IR intervention, and liver transplant evaluation was initiated. A mixed autoimmune hemolytic anemia (AIHA) was confirmed by blood smear demonstrating spherocytes without schistocytes in combination with both warm and cold antibodies on Coombs’ testing. A bone marrow biopsy later revealed hypercellularity with increased megakaryocytes consistent with immune-mediated thrombocytopenia (ITP). AIHA and ITP in the setting of APS are congruous with secondary Evans syndrome. The patient initially received mg/kg prednisone without response. She was then transitioned to IV Ig for six doses, romiplostim weekly for three weeks, rituximab weekly, and lastly, plasma exchange. Despite progression of therapy and frequent transfusions, platelets remained unresponsive with worsening hemolytic anemia. The clinical course was further complicated by significant bleeding involving diffuse alveolar hemorrhage resulting in acute hypoxic respiratory failure requiring mechanical ventilation and recurrent gastrointestinal bleeding. The patient developed widespread venous thromboses with our inability to anti-coagulate, resulting in multi-organ failure including cardiogenic shock and acute renal failure consistent with catastrophic antiphospholipid syndrome (CAPS). Ultimately, the patient suffered diffuse dural venous thromboses, cerebral herniation, and was subsequently palliatively extubated.

A small subset of APS patients will develop CAPS manifested by intravascular thrombosis resulting in multiorgan failure with high mortality. Treatment focuses on limiting thrombosis and suppressing the cytokine cascade. This case demonstrates the formidable processes of an immune system gone awry despite aggressive treatment with well-validated therapeutic options and the dilemma of anticoagulation with active bleeding. Lastly, we are uncertain if more expeditious plasma exchange or the addition of cyclophosphamide or eculizumab could have made an impact on this patient’s care.

#189  A case of acquired hemophilia a due to factor viii inhibitor

C Donath*

A Siddiqui

A Arnold

Florida State University, Tallahassee, FL

Introduction

Acquired Hemophilia A (AHA) is a rare and typically incidentally found by either bleeding complications after a GI procedure, postpartum or spontaneous bleeding into the skin and soft tissues.

Case Report

72y/o female with a history of coronary artery disease s/p percutaneous coronary intervention with drug eluting stent (DES) two months prior, hyperthyroidism and rheumatoid arthritis, was admitted for acute blood loss anemia. She was on aspirin and clopidogrel given the recent DES. She has a 1-week history of fatigue, bloody diarrhea and epistaxis. She was hemodynamically stable. Lab values showed WBC 10.2 k/mm3, hemoglobin 5.5 mg/dL, hematocrit 18%, platelet 113 k/mm3, MCV 100 fl, reticulocyte 14%, smear showed slight macrocytosis, B12 1021 pg/mL, folate 36.6 ng/mL, ferritin 66.4 ng/mL, transferrin saturation 17%, LDH 450 u/L, haptoglobin 225 mg/dL, PT 14 seconds, INR 1.0, PTT 85 seconds. Two units of packed red blood cells were transfused urgently. Post transfusion hemoglobin was 8.1 mL/dL. Gastroenterology was consulted for suspected upper gastrointestinal bleed. Esophagogastroduodenoscopy showed a Dieulafoy lesion in the gastric body and greater than 20 bleeding AVMs in the duodenum/jejunum. These lesions were ablated successfully. A few hours after the procedure, the patient complained of severe abdominal pain. An emergent CT abdomen w/o contrast showed a new duodenal intramural hematoma. General Surgery did not recommend surgical intervention given very poor surgical canidate. Aspirin and clopidogrel was stopped. Meanwhile, the mixing study result came back showing an abnormal mixing pattern not corrected by incubation. Factor VIII was found to be <1% and Bethesda Titer assay was >30. This study consistent with Acquired Hemophilia A secondary to Factor VIII inhibitor. Hematology/Oncology was consulted and the patient was started on high dose prednisone, recombinant FVIII and weekly rituximab infusions for immunosuppression.

Discussion

AHA is a rare entity of bleeding disorders that is often a missed diagnosis. 50% of diagnosed cases are idiopathic with spontaneous bleeding, with the rest being due to occult malignancy, autoimmune disease, infections or post-partum bleeding. Severe bleeding can occur in up to 70% of affected patients with fatality as high as 5–10%. Unlike Congenital Hemophilia, hemarthrosis is not a part of the syndrome. Rather, spontaneous bleeding into skin and soft tissues, intramural hematomas and mucus membranes is seen. Abnormal mixing studies should lead to investigation of which bleeding factor is being consumed/inhibited, commonly factor VIII or IX by investigating the factor’s activity. Bethesda Titer assay will be useful for levels of the inhibitor itself.

Conclusion

Isolated aPTT should prompt clinicians to order mixing studies to evaluate etiology of the prolonged coagulation cascade. AHA can lead to devastating bleeding sequelae, therefore prompt recognition and treatment of AHA can be lifesaving.

#190  Therapeutic challenges in treating a newly diagnosed aplastic anemia in a patient with coexistent covid-19 infection

Z Elharabi*

E Elgwairi

M Abohelwa

G Del Rio-Pertuz

K Parmar

D Pawar

A Abdalla

K Nugent

Texas Tech University Health Sciences Center, Lubbock, TX

Introduction

Aplastic anemia is a syndrome of bone marrow failure characterized bone marrow hypoplasia. Immunosuppressive therapy is one modality of its management. We report a case in which use of this modality was hindered by lack of data showing the effects of its use during the novel COVID-19 infection.

Case presentation

A 20-year-old man with a newly diagnosed pancytopenia presented with fever, cough, headaches, and exertional dyspnea. When his vital signs were obtained, he was afebrile but his blood pressure, heart rate and oxygen saturation were within normal range. Physical exam was unremarkable. Laboratory tests showed that the white blood cell count was 1.42 K/µL, hemoglobin level was 9.7 g/dl, and platelet count was 13 K/µL. He was tested for COVID-19 infection and was found to be positive. A peripheral blood smear showed pancytopenia. A bone marrow biopsy showed hypocellular marrow with trilineage hypoplasia. Flow cytometry showed no significant trilineage abnormalities. Vitamin 12 and folate levels were within normal range. Testing for antinuclear antibodies, rheumatoid factor, HIV, hepatitis C, and hepatitis B were negative. Ultrasound of the abdomen showed no enlargement of the spleen. The PNH FLAER test was done twice and was inconclusive possibly due to hemolysis or severe pancytopenia. The patient was diagnosed with aplastic anemia, but the cause was unclear. Anti-thymocyte globulin, cyclosporine, and steroids were considered for treating the aplastic anemia, but there was concern about their unknown effect on his active COVID-19 infection. Immunosuppressive therapy was decided to be held until he was cured from his COVID-19 infection, and he was discharged after his blood cell indices improved.

Discussion

Being an infection caused by a novel virus, COVID 19 can cause a therapeutic dilemma when no data are available about the effects of certain therapies on the infection. In our patient, immunosuppressive therapy was needed to treat the aplastic anemia but there was no published literature on the effect of this treatment on the course of the infection. This should become less of an issue with time as data surrounding COVID-19 infection and its effects on other diseases and treatment modalities grow.

#191  Pulmonary nodules in a patient with breast cancer; metastasis or co-existing disease?

N Eshak*

K Parmar

M Abdelnabi

K Nugent

Texas Tech University System, Lubbock, TX

Case Report

A 54 y/o patient presented to the Oncology clinic for recently identified breast cancer. She underwent an excisional biopsy of the right breast mass, which revealed invasive ductal carcinoma; this was followed by lumpectomy with sentinel lymph node biopsy that was negative for malignancy.

While doing a CT scan for her radiotherapy planning a 6 mm pulmonary pleural-based nodule was incidentally discovered. The patient completed her radiotherapy sessions and was started on anastrozole while completing work-up for pulmonary nodule.

A chest CT scan was done showed multiple pulmonary nodules, at least six, all <1 cm in size (figure 1A).

She underwent a PET scan (figure 1B) which revealed lymphadenopathy throughout the chest, including right supraclavicular and hilar lymph nodes, as well as periportal lymphadenopathy. There was no abnormal metabolic activity in pulmonary nodules, however, the sensitivity of PET scan to detect metabolic activity in nodules <8 mm is limited, overall the picture was suggestive of metastatic disease.

The decision was made to obtain a biopsy prior to initiating any further treatment.

The patient underwent VATs with resection of 2 pulmonary nodules and a right supraclavicular lymph node biopsy. Both revealed non-caseating granulomas, with negative stains for fungi and acid-fast bacilli, and were negative for malignancy.

She was referred to both infectious diseases and rheumatology, tests for TB, Histoplasma, ANA, RF, ANCA, were all negative, ACE level was mildly elevated. A diagnosis of sarcoidosis was made, and the patient was started on inhaled steroids for management of cough, no systemic therapy was initiated as the patient was otherwise asymptomatic. A follow-up CT scan showed regression of pulmonary nodules.

Conclusion

Pulmonary nodules and lymphadenopathy in a patient with breast cancer should raise the suspicion of metastasis. Occasionally the finding of non-caseating granuloma is found in these lesions. Sarcoidosis is a systemic granulomatous disease characterized by the development of non-caseating granulomas mostly in the lungs and hilar lymph nodes but can also involve skin, eyes, and other organ systems. The relationship between sarcoidosis or sarcoid-like reaction and breast cancer has been reported mainly as case series and case reports.

#192  Arterial thrombosis as the initial presentation of breast cancer

N Eshak*

M Abdelnabi

J Benjanuwattra

K Nugent

Texas Tech University System, Lubbock, TX

Case Report

A 45-year-old female patient presented to the clinic complaining of severe bilateral worsening leg pain for several weeks that increased with movement and improved with rest. She has a history of hypothyroidism treated with levothyroxine. Physical examination was significant for bilateral weak peripheral pulsations and cold lower extremities. Initial laboratory workup was normal. Peripheral angiogram revealed complete occlusion of the left common iliac artery and the right common femoral artery.

She underwent bilateral femoral artery exploration, open mechanical thrombectomy, stenting of the left common iliac artery, and angioplasty of the left common femoral and common/external iliac arteries (figure 1). Pathological examination of the removed tissue of the arteries confirmed to be thrombi. Hematology was consulted for work-up of a hypercoagulable state. She had no personal or family history of previous clots or miscarriage. Hypercoagulable work-up came back negative, but she was started on long-term anticoagulation. On follow-up in the clinic, she complained of a breast lump, mammogram with U/S followed by biopsy revealed bilateral invasive ductal carcinoma. She refused any surgical or medical interventions.

Patients with malignancy are at higher risk for thrombosis. Venous thromboembolism is a frequent complication in these patients and usually occurs after the diagnosis of cancer is confirmed. Thrombosis as the initial presentation of malignancy is uncommon with arterial thrombosis being more so, especially in a patient without atherosclerosis or cardiovascular risk factors.

#193  Post-obstructive acute kidney injury due to retroperitoneal fibrosis: an unusual presentation of esophageal signet ring cell carcinoma

M Gartner*

N Tidwell

Dwight David Eisenhower Army Medical Center, Fort Gordon, GA

Introduction

Signet ring cell carcinoma (SRCC) is an aggressive and rare form of mucin-producing adenocarcinoma with an estimated incidence rate of 0.04–0.11 cases per 100,000 individuals per year. SRCC most commonly arises from the gastrointestinal tract and has a higher prevalence among men and smokers. The most common sites of metastasis remain liver, lung, and distant lymph nodes. The low disease incidence and aggressive nature present a diagnostic challenge as early signs and symptoms can be subtle. This case highlights an unusual presentation of SRCC: acute kidney injury (AKI) due to retroperitoneal fibrosis (RPF).

Case Report

A 67-year-old male presented to the emergency department with complaints of abdominal pain and reduced urine output. Initial evaluation was notable for AKI with elevated blood pressure readings. The initial concern was for obstructive ureterolithiasis prompting evaluation via computed tomography (CT) urogram. The study was notable for bilateral hydronephrosis, bilateral proximal hydroureter, stricturing of the mid to distal ureters, and retroperitoneal lymphadenopathy and fibrosis. Cystoscopy with retrograde cystoureterogram confirmed ureteral stricturing. Bilateral nephrostomy tubes were placed with resolution of his AKI. Differential diagnosis at this time included both malignant and autoimmune RPF. Immunoglobulin subtyping (with IgG and IgG4) and antinuclear antibody (ANA) screening were unremarkable. Positron emission tomography/computed tomography (PET/CT) was notable for PET avidity throughout his retroperitoneum. There was also avidity in the distal esophagus favored to represent inflammatory changes. CT-guided biopsy of the avid retroperitoneal lymph nodes was notable for adenocarcinoma with signet ring cell features with positive pankeratin, CK7, CK20, and CDX-2 and negative for TTF-1, PAX-8, GATA-3 and PSA. Follow-up esophagogastroduodenoscopy (EGD) was notable for findings consistent with reflux esophagitis were noted in the distal esophagus. Biopsy of the inflamed tissue was also notable for adenocarcinoma with signet ring cell features.

Discussion

Early manifestations of esophageal SRCC, like other esophageal carcinomas, are often subtle. Symptoms such as regurgitation, dysphagia, hoarseness, weight loss, and iron deficiency from occult blood loss usually denote locally invasive disease. Later findings are most often dependent on location of metastasis, which is usually liver, lung, and lymph nodes. This patient was diagnosed with esophageal SRCC only after being found to have findings of RPF due to metastasis to retroperitoneal lymph nodes on cross sectional imaging. Early diagnosis of SRCC remains critical as prognosis of metastatic disease is grim with 5-year survival of 1.5% and median survival of 7.9 months. On review of literature, there have only been two reported cases of SRCC presenting as retroperitoneal fibrosis highlighting this case’s importance and contribution to the medical community.

#194  Primary diffuse large B-cell lymphoma of the cecum

L Gawey1*

E Ellent2

A Garcia1

1LSU Health New Orleans, New Orleans, LA

2West Jefferson Medical Center, Marrero, LA

Purpose of Study

Case report of primary diffuse large B-cell lymphoma (DLBCL) of the cecum in patient with underlying ischemic heart disease being treated with R-CHOP chemotherapy

Methods Used

Review of Electronic Health Records and literature review

Summary of Results

A 73-year-old male with underlying ischemic heart disease and history of renal cell carcinoma presented with persistent productive cough and shortness of breath. Chest CT found new noncalcified nodules in both lungs, and further PET scan imaging revealed circumferential cecal colonic wall thickening with hypermetabolic activity, in addition to the bilateral pulmonary nodules. His last colonoscopy had been 3 years prior with only finding of benign polyps. CT-guided biopsy of a pulmonary nodule showed neoplastic cells positive for CD45, CD20, PAX5, CD10 and BCL6, and negative for SOX11; the Ki-67 index was 75–80%. The patient underwent a colonoscopy which found an infiltrative, ulcerated and fungating 5 cm mass with stigmata of recent bleeding of malignant appearance in the cecum, causing a partial obstruction. Cold forceps biopsies were performed and neoplastic cells were positive for CD20, CD10, BCL6, and MYC (50%), and negative for SOX11, CD23, BCL2, and EBER; Ki-67 index was > 90%. Fluorescence in-situ hybridization (FISH) studies for BCL2, BCL6, and MYC were negative for rearrangement, which were performed to rule-out double-hit lymphoma. The patient was diagnosed with high-grade DLBCL with germinal center phenotype of colonic origin and metastasis to the lungs. The patient began treatment with R-CHOP-21 chemotherapy, and after the first two rounds of treatment, PET CT revealed significantly decreased size and metabolic activity of cecal mass with max SUV in the cecum measuring 8.7, which was previously 26.2 pre-treatment. He has tolerated the R-CHOP relatively well with only one episode of angina-type symptoms.

The GI tract is the predominant site of secondary extra-nodal non-Hodgkin lymphoma (NHL). Primary lymphomas of the GI tract are rare, accounting for only 1–4% of malignancies arising in the stomach, small intestine, or colon. Primary colorectal lymphoma is even more rare, accounting for only 0.3% of large intestinal malignancies and 3% of GI lymphomas. The cardiotoxic effects of doxorubicin must be accounted for in patients with underlying cardiac disease when administering R-CHOP. There is limited data available from small trials that can be used to guide both prevention and management of cardiotoxicity. Additionally, there are no randomized trials for chemotherapy treatment of DLBCL that includes patients of underlying cardiac disease.

Conclusions

This case highlights the treatment considerations for primary colorectal DLBCL in an older patient with underlying ischemic heart disease. Due to the rarity and severity of primary colorectal lymphoma, there is scarce literature regarding the outcome of chemotherapy treatment options, especially in patients with underlying risk factors.

#195  The importance of recognizing growing teratoma syndrome (gts): a rare complication of non-seminamatous germ cell tumors (nsgct)

W Gibson*

K Thomas

R Chowdry

LSU Health New Orleans, New Orleans, LA

Introduction

Growing teratoma syndrome (GTS) is a rare complication of non-seminomatous germ cell tumors (NSGCT) seen in testicular and ovarian cancers. According to the diagnostic criteria first described in 1982 GTS is defined by the following criteria: normalization of elevated serum alpha fetal protein (AFP) and human chorionic gonadotropin (HCG) levels, tumor growth during or after appropriate chemotherapy for NSGCT, and the exclusive presence of mature teratoma in the resected specimen. The incidence of GTS has been reported to be 1.9 – 7.6% in testicular cases and around 12% in ovarian cases (1,2).

Case

A 32-year-old man with a past medical history of schizophrenia and polysubstance abuse was evaluated for a right testicular nodule. The ensuing workup showed a complex testicular mass concerning for malignancy, numerous pulmonary nodules in bilateral lung fields concerning for metastatic disease, and lymphadenopathy in the left supraclavicular region, mediastinum, and retroperitoneum. Initial alpha fetal protein was elevated at 43 ng/ml, beta-HCG, and LDH were within normal limits. The patient underwent a right radical orchiectomy. The pathology revealed mixed germ cell tumor in 60% of the specimen and the remaining 40% was showed teratoma. The patient received an initial cancer staging of Stage IIIC (PT1aNXM1b). The patient underwent three cycles of bleomycin, etoposide, and cisplatin. Repeat imaging showed worsening pulmonary nodules as well as retroperitoneal and supraclavicular lymphadenopathy. Due to disease progression, the patient then received two cycles of cisplatin, etoposide, and ifosfamide. Repeat CT imaging revealed further worsening retroperitoneal lymphadenopathy with evidence of compression on the inferior vena cava. AFP and HCG levels remained within normal limits throughout treatment. Biopsy of the left neck mass showed benign teratoma. Upon meeting the patient and reviewing the case, GTS was highly suspected and further chemotherapy was not recommended at this time. Instead, surgical management was pursued.

Discussion

This case highlights the importance of having a high level of suspicion for GTS in any patient with NSGCT with tumor size progression and with normalization of tumor markers after systemic chemotherapy. GTS is generally resistant to standard chemotherapy regimens. Surgical resection of residual tumor burden is the primary treatment

#196  A classic case of a rare hematologic disorder

GD Gibson*

C Bigelow

The University of Mississippi Medical Center, Jackson, MS

Case Report

IgM multiple myeloma (MM) is an exceptionally rare hematological disorder comprising less than 0.5% of MM cases. Diagnosis of IgM MM can often be a challenge in differentiating from the more prevalent disorder of Waldenstrom macroglobulinemia (WM), in which both have an IgM monoclonal gammopathy. The importance of distinguishing between these two disorders is imperative since treatment and prognosis differ appreciably.

A 48 year-old female presented to the emergency department with progressive low back pain. She previously had a traumatic fall resulting in a T6 fracture four months prior while sledding that required no surgical intervention. She wore a back brace without any reported issues until 5 weeks prior to being evaluated in the emergency room. She had no previous past medical history. Physical examination was significant for thoracic-lumbar tenderness to palpation. Magnetic resonance imaging (MRI) of her spine showed multiple osseous lesions and pathologic fracture of T6 with epidural extension of neoplasm but no cord compression. No hepatosplenomegaly reported on imaging. Labs were significant for anemia, hypercalcemia, and acute kidney injury. Subsequent labs revealed a kappa/lambda free light chain ratio elevated at 518 and serum protein electrophoresis showed a monoclonal protein spike of 2.8 g with an immunofixation showing IgM kappa protein. A bone marrow (BM) biopsy showed 68% kappa restricted plasma cells. Cytogenetics revealed a t(11;14) consistent with a CCDN1/IGH gene fusion and was negative for a MYD88 L265 mutation. She was diagnosed with MM. Treatment with lenalidomide, bortezomib, and dexamethasone was initiated. Patient responded to treatment with improvement in renal function and is undergoing evaluation for an autologous stem cell transplant.

Establishing a diagnosis of IgM MM can be challenging given rarity of the disease. IgM MM presents as any other type of MM, and the diagnosis of IgM MM requires the presence of an IgM monoclonal gammopathy with 10% or more of clonal plasma cells on BM biopsy, along with at least 1 myeloma defining event. Myeloma events are defined by the acronyms CRAB (hypercalcemia, renal insufficiency, anemia, and bone disease) and SLiM (sixty% plasmacytosis on BM, light chain ratio >100 or <0.01, and 1 lesion on MRI). All CRAB and SLiM criteria were present in this case confirming the diagnosis of MM. WM is an IgM secreting lymphoblastic lymphoma that presents with signs and symptoms of anemia, lymphadenopathy, hepatosplenomegaly, neuropathy, and hyperviscosity. The presence of t(11;14) found in our patient which leads to cyclin D1 dysregulation is common in IgM MM, but absent in WM. Somatic mutation MYD88 L265 is observed in patients with WM. This case demonstrates the importance of a thorough diagnostic approach including physical exam, labs, pathology, imaging and cytogenetics in patients presenting with IgM monoclonal gammopathy to correctly identify patients with IgM MM vs WM.

#197  Analysis of the correlation of time to treatment failure with overall survival versus correlation of progression-free survival with overall survival

GD Gibson*

JC Henegan

The University of Mississippi Medical Center, Jackson, MS

Purpose of Study

In cancer drug trials, improvement in overall survival (OS) is a meaningful measure of drug efficacy to patients. Measuring OS requires large patient numbers and prolonged follow up. To address this limitation, studies have utilized endpoints such as progression free survival (PFS) and time to treatment failure (TTF) as surrogates of an OS benefit. PFS is defined as time from randomization to objective tumor progression or death and is felt to correlate more with OS as it includes death. TTF is defined as the time from randomization to treatment discontinuation for any reason and has been suggested as a practical endpoint using real-world evidence, although it is not commonly used in clinical trials. Hazard ratio (HR) is also frequently used to estimate the treatment effect for time-to-event end points such as OS, PFS, and TTF in randomized clinical trials. We conducted a study to examine the correlation between HR for TTF and HR for OS versus the correlation between the HR for PFS and the HR for OS in a publicly available database.

Methods Used

A search of studies in clinicaltrials.gov, last performed on October 7, 2021, was done to identify all trials reporting HR of OS, PFS and TTF – trials not reporting any of these measures or trials reporting values other than the HR for each of these measures were excluded. Trials with an upper bound of the 95% confidence interval of overall survival that was > 10 were excluded due to the uncertainty associated with these results. Trials reporting TTF, PFS, and OS for multiple arms were included with each unique set of comparisons considered an individual study. The correlation of OS with TTF, OS with PFS, and TTF with PFS was calculated in Microsoft Excel. The correlation between OS with TTF was compared to the correlation between OS with PFS by the test of the difference between two dependent correlations with one variable in common.

Summary of Results

A search of clinicaltrials.gov identified 147 studies that reported information on OS, TTF, and PFS. After excluding studies not meeting the above criteria, 39 sets of pairs of OS-TTF, OS-PFS, and TTF-PFS were able to be analyzed. The correlations between TTF-OS, PFS-OS, and TTF-PFS were:

TTF-OS: 0.63

PFS-OS: 0.43

TTF-PFS: 0.79

The correlation of TTF-OS is statistically significantly greater than the correlation between PFS-OS (two sided p-value: 0.02).

Conclusions

This analysis of publicly available results of clinical trials found statistically significant difference between the correlation of the HR for TTF with OS versus the correlation of the HR for PFS with OS, with the correlation for TTF with OS being the stronger correlation. Limitations of this work include the heterogeneity of the studies and the small sample size. Further work is needed to validate this finding in an independent dataset. If validated, it would suggest that monitoring TTF would serve as a stronger correlate of OS than PFS does.

#198  Acute respiratory distress syndrome after exposure to carfilzomib (kyprolis)

P Goel*

K Qureshi

A Ananthula

R Chowdry

LSU Health New Orleans, New Orleans, LA

Case Report

Introduction

Plasma cell leukemia is very rare and an aggressive form of leukemia with a poor prognosis. Interim analysis of a phase II trial (EMN12/HOVON 129) using carfilzomib, lenalidomide, and dexamethasone (KRd) in patients with PCL ≤65 years showed a very good partial response or greater response in 80% with 33% achieving at least a complete response. Carfilzomib (Kyprolis TM) is a proteosome inhibitor and is associated with ARDS and acute respiratory failure in 2% of the cases per FDA package insert. We present a case report of acute respiratory distress syndrome presumed to be potentiated 2/2 to carfilzomib infusion.

Case

A 58-year-old male with a history of hypertension, recent COVID-19 infection and new diagnosis of untreated Plasma Cell Leukemia presented to our hospital with worsening chest pain, fatigue and dyspnea. Vitals on admission were notable for BP 158/88, HR 101, Tmax 99F and sating 100% on room air. Peripheral blood exam showed WBC: 27.7 x109/L, Hb: 8 gm/dl, platelet: 121000, corrected calcium:13.3 mg/dl, creatinine: 1.16 mg/dl, total protein:11 g/dl, uric acid: 8.2 mg/dl, B-2 micro globulin: 5.8 mg/L, Mspike: 5.6 g/dl; IgA lambda type. CT Chest abdomen pelvis revealed diffuse lytic bone lesions. Due to inability to obtain bone marrow biopsy from limited resources after Hurricane Ida and aggressive nature of the cancer, treatment was initiated based off a previous flow cytometry from the peripheral blood which showed 55% plasma cells. Patient started on chemotherapy with Cyclophosphamide, Carfilzomib, and dexamethasone with plans to change to Revlimid from cycle 2. He was also started on fluid hydration and Zometa for hypercalcemia. Patient also received aggressive blood pressure control with metoprolol, amlodipine and IV labetalol as needed. After 2nd dose of Kyprolis, he developed acute hypoxic respiratory distress and was initiated on Bipap. Chest Xray was concerning for fluid overload and/or evolving pneumonia. He was supported with diuretics and broad-spectrum antibiotics; however, he eventually was intubated. He was also started on high dose steroids. Repeat CT chest was negative for thrombosis, but showed extensive bilateral pleural -parenchymal opacities. He had a bronchoalveolar lavage with no obvious infection. Over the next 2 days, patient showed improvement and eventually self-extubated. After his recovery, we continued chemotherapy with Kyprolis and he has tolerated it without issues.

Discussion

The etiology of ARDS is likely multifactorial, however Kyprolis may have played a major role in his decompensation mainly due to the timing and known side effects of the medication. Based on a study from 2018, only 5 case reports of Kyprolis-associated non-infectious progressive lung injury were found at that time. Clinicians should be mindful of Kyprolis induced lung injury and emphasize the need for tight blood pressure control and careful administration of intravenous fluids to decrease the possibility of lung injury.

#199  Holiday hemoglobinuria: paroxysmal cold hemoglobinuria in cooler months in a patient with treated syphilis

J Graham*

S Elkins

University of Mississippi Medical Center, Jackson, MS

Case Report

Our patient is a 61-year-old woman who came to our university hospital’s attention in January 2018 with an acute presentation of abdominal and back pain, nausea, and vomiting. She was discovered to have significant kidney disease and nephrotic range proteinuria. Work-up yielded a positive RPR, which was supported by a reactive Treponema pallidum particle agglutination study and by her report of having been treated for syphilis with penicillin in the distant past.

She returned to the hospital in January 2019 with hypoglycemia and acute kidney injury following cocaine consumption. Her hemoglobin was found to be 5 g/dL. The antibody screen before transfusions was positive. It was noted that ‘the agglutination pattern may reflect an additional emerging antibody or a nonspecific reaction. Antibody specificity may become clear upon further testing.’

Hemoglobin dropped from 8.3 g/dL to 5.5 in two days several days into the admission, shortly after a urine drug screen was positive for cocaine. Hemolysis labs were notable for LDH of 1168 U/L and haptoglobin of < 10 mg/dL. Hematology was consulted, and prednisone 1 mg/kg/day was started for a suspected warm autoimmune hemolytic anemia. Hemolysis had significantly lessened one week into prednisone. She was lost to hematology follow-up.

During another admission in January 2021 (for retropharyngeal abscess), a type and screen was performed, which showed another positive antibody detection screen and tube testing consistent with a cold autoantibody. She presented again in September 2021 after a fall at a nail salon. Her hemoglobin was 5 g/dL. She was transfused and discharged. Hemolysis labs were not checked, but a urinalysis showed a small amount of blood in the urine.

She presented again five days later after another fall at a nail salon. She experienced a syncopal episode in the emergency department and significant pain and nausea and vomiting afterward. Her hemoglobin had dropped mildly since her recent discharge. Urinalysis was notable for a large amount of blood. Hemolysis labs were notable for LDH of 352 U/L, haptoglobin of < 10 mg/dL, indirect bilirubin of 1.2 mg/dL, and 4.8% reticulocytes (138 x 10^9/L). Antibody detection screen was again positive, with DAT IgG 2+, DAT C3 negative. Tube testing was ‘negative at immediate spin, 37, and AHG with negative autocontrols, but panpositive at 4 C with a positive autocontrol (3+), consistent with the patient‘s history a cold autoantibody.’

Hematology was consulted and began prednisone 1 mg/kg/day for suspected paroxysmal cold hemoglobinuria secondary to Donath-Landsteiner antibody stemming from her treated syphilis. Confirmatory testing was sent.

This case illustrates the importance of following the antibody screen and direct antiglobulin test in patients with recurrent anemia, particularly those with underlying conditions associated with autoimmune hemolysis. The character of the autoantibody may become clearer with time.

#200  Disseminated fungal infection and marijuana use in a pediatric cancer patient

M Grassi1,2*

D Citla-Sridhar1,2

KK Mason1,2

JM Mack1,2

1University of Arkansas for Medical Sciences, Little Rock, AR

2Arkansas Children s Hospital, Little Rock, AR

Case Report

Several case studies have reported invasive fungal diseases in people who use marijuana and fungal contamination of cannabis. We report a case of an immunocompromised pediatric patient that was a known marijuana user, who developed disseminated fungal infection during the course of his treatment, and succumbed to death.

Patient was a 16 year old male with T cell lymphoma, while on chemotherapy phase with escalating doses of methotrexate, who developed febrile neutropenia. He presented in septic shock and was diagnosed with a pan-resistant fungal infection (Scedosporium prolificans) with multiple sites of fungal dissemination in lungs, sinuses, skin, brain and spleen. He was treated with Voriconazole, Amphotericin, Micafungin and a new drug in development – Fosmanogepix for 4 months. With worsening disseminated fungal disease, patient was discharged to hospice, and died at home. Of note, patient was a known user of marijuana (vaping) prior to diagnosis and continued to use marijuana during treatment. He also had a medical marijuana card.

Although the frequency and duration of his marijuana use is unclear, it raises suspicion if this exposure could have resulted in colonization of his lungs with fungal organisms. Immunocompromised status with fungal colonization, likely lead to disseminated fungal infection. Studies have also shown that marijuana may have a similar effect as tobacco, and cause structural and immunological lung damage confers increased susceptibility to infection. A recent study with a commercial database showed that people who use cannabis were more likely to have fungal infections, than people who did not use cannabis.

While medical marijuana is approved in several states in the United States, especially for patients receiving chemotherapy, it is important to discuss potential health implications and risk of fungal infections with patients.

#201  Cranial and sinus plasmacytoma- a rare entity

C Kamireddy*

O Mohammadi

P Vahhabaghai

K Chakraborty

S Singal

East Tennessee State University, Johnson City, TN

Case Report

Plasmacytomas are malignant proliferation of plasma cells which can be seen with different plasma cell dyscrasias. We present a rare entity of plasmacytoma presenting as cranial and sinonasal tumor. A 60 year old gentleman with diagnosis of IgG kappa multiple myeloma three years ago. Initial bone marrow evaluation showed 70% plasma cells with no high risk cytogenetic features. He received standard of care treatment with Bortezomib, Lenalidomide and dexamethasone for a total of 12 cycles. He refused to undergo hematopoietic stem cell transplant, subsequently relapsed in one and half year. Treatment was changed to Daratumumab, Pomalidomide and dexamethasone. Patient relapsed after 18 months of treatment, at which point he was initiated on third line therapy with Ixazomib, Lenalidomide and Dexamethasone. After 4 cycles into treatment he developed symptoms of headaches and diplopia. Clinical exam was consistent with left eye constricted pupil and left lateral rectal palsy. Magnetic resonance imaging of the brain revealed an infiltrative lesion at the skill base involving the bilateral cavernous sinuses and left sphenoid sinus opacification. Paraproteinemia work up showed increase in serum Immunoglobulin G from 1284 mg/dl to 6337 mg/dl, kappa free light chain from 91.47 mg/dl to 1815 mg/dl, with a kappa/lambda ratio of 637.11 increased from 19.54. Bone marrow biopsy confirmed relapsed multiple myeloma with 90% involvement by plasma cells. The skull base lesion was highly suggestive of plasmacytoma, given the systemic evidence of myeloma relapse. Biopsy of the left sphenoid sinus mass confirmed involvement by plasmacytoma with IgG kappa monotype and immunohistochemistry positive for CD138, CD56 and kappa and negative for MUMI1, CD20 and CK AE1,3. He received palliative radiation treatments to the skull base to achieve rapid tumor response. Patient also suffered recurrent epistaxis, requiring embolization of the nasal artery. Sinus endoscopic surgery with maxillary antrostomy was attempted as well. Unfortunately even with aggressive radiation and surgical efforts, patient continued to have significant epistaxis and decided to transition to comfort care.

Extra medullary plasmacytoma of head and neck is rare, comprising 3% of all plasma cell tumors. They constitute 1% of head and neck cancers 4% of all non-epithelial tumors of nasal tract. Direct compression or involvement of cranial nerves causes cranial nerve palsies, symptoms of raised intracranial pressure and epistaxis. Differential diagnosis of the base of the skull tumor includes nasopharyngeal carcinoma, meningioma, lymphoma, pituitary adenoma and metastatic carcinoma. Biopsy showing plasma cells with monoclonal staining pattern involving the heavy chain and light chain on immunohistochemical studies help differentiate plasmacytoma from the other tumors. Radiotherapy constitutes the mainstay of treatment, surgery is limited to biopsy and control of local symptoms.

#202  False positive heparin induced thrombocytopenia in patient with anti-phospholipid antibody syndrome

C Kamireddy*

J Kim

T Pham

D Jaishankar

K Chakraborty

East Tennessee State University, Johnson City, TN

Case Report

Heparin-induced thrombocytopenia (HIT) is a catastrophic complication of heparin therapy, caused by antibodies against platelet factor 4 (PF4)-heparin complex. Patients with antiphospholipid syndrome (APS) have been rarely reported with positive PF4-heparin complex antibodies.

A 20-year-old female with no medical comorbidities, presented with fever, dyspnea and confusion. She developed respiratory failure requiring mechanical ventilation. Labs were significant for platelet count of 47k/uL, hemoglobin of 6.3 g/dL, white blood cell count of 9k/uL, and serum creatinine of 1.47 mg/dl. Coagulation panel showed a prothrombin time of 16.1, International Normalized Ratio of 1.5, and partial thromboplastin time of 53. Chest imaging showed bilateral infiltrates. Bilateral cerebral infarcts and bilateral renal infarcts were also found, suggestive of thromboembolic arterial events. Echocardiogram showed severe mitral regurgitation with thickening and calcification of the mitral leaflets. Given the neurological symptoms, acute kidney injury and thrombocytopenia, thrombotic thrombocytopenic purpura (TTP) was high on the differential. Peripheral smear showed no evidence of schistocytes, ADAMTS13 level was normal. Hemolysis and nutritional deficiencies were not evident. Patient had no known prior heparin exposure, but HIT antibody was tested positive. Prophylactic heparin was discontinued. Argatroban infusion was initiated, but serotonin release assay resulted negative. Suspicion for autoimmune disease was high given the patient‘s age and acute presentation with multiorgan involvement. Her ANA and anti-double stranded DNA titers were elevated at 1:640 and 1:2560, respectively. Complement levels C3 and C4 were low. Lupus anticoagulant was detected, and anticardiolipin antibodies IgG, IgM were positive. Patient met the criteria for catastrophic antiphospholipid antibody syndrome (CAPS). High dose steroids, cyclophosphamide and plasma exchange were started. She underwent mitral valve replacement for mitral regurgitation and had improvement in her overall clinical status with appropriate management of APS.

Both HIT and APS are immune-mediated thrombotic conditions. Reports have noted patients with APS/systemic lupus erythematosus (SLE) can have positive PF4 antibodies, even without prior heparin exposure. It is believed vascular inflammation from such autoimmune conditions may trigger an autoantibody response to PF4 released from platelets, forming an antigenic complex with endogenous heparinoids, then causing false positive HIT immunoassays. This can be distinguished by testing the functional assays. Manifestations of HIT and APS may both overlap with thrombocytopenia and arterial or venous thrombosis. Therefore, cognizance of possible false positive HIT antibodies in patients with underlying APS/SLE is important. Caution should be executed in interpreting the tests as it can lead to misdiagnosis, overtreatment or undertreatment and risk of catastrophic outcomes.

#203  Diarrhea secondary to ileal neuroendocrine tumor with no liver metastasis

H Yousuf1*

M Khan1

M Amin1

J Rahesh1

R Anand1

R Hazam1

F Khan2

A Zakir3

S Pathapati1

1Texas Tech University Health Sciences Center, Amarillo, TX

2Khyber Medical University, Peshawar, Pakistan

3King Edward Medical University, Lahore, Pakistan

Introduction

Well-differentiated neuroendocrine tumors (NETs) are relatively rare tumors that most commonly originate in the gastrointestinal tract, lung, and rarely in the genitourinary tract. We present a similar case with ileal NET, presenting with chronic diarrhea as the sole symptom without liver metastasis.

Case

A 65-year-old Caucasian male with a past medical history of hypertension and alcohol abuse presented with 2 months of watery diarrhea. Initially, it was accompanied by fever, chills, and nausea. He also reported generalized weakness and recurrent falls due to syncope. He self-medicated with Ivermectin. He reported a 20 pounds weight loss over 2 months as well as worsening oliguria. However, three days prior to admission he noticed fresh blood in his stool, prompting him to seek medical attention. On admission, he denied abdominal pain, flushing, dyspnea, or chest pain. On examination, he had mild diffuse abdominal tenderness. He had never had a colonoscopy or endoscopy at this point. On admission, his labs were significant for S.Creatinine of 18 mg/dL, a BUN of 161 mg/dL, and HCO3 of 6 mmol/L. His kidney function improved with fluid resuscitation. Stool culture and Clostridioides difficile toxins were negative.

His CT abdomen with contrast was remarkable for ’misty mesentery’ suggestive of panniculitis. Colonoscopy showed a large 3 cm submucosal polypoid nodular mass at a 10 cm distance from the ileocecal junction. Biopsy reported a well-differentiated neuroendocrine tumor (carcinoid), spanning at least 2 mm involving the lamina propria and submucosa. Liver ultrasound showed only hepatic steatosis with no lesions. 24-hour urine HIAA was 12.5 mg, chromogranin-A 1048 ng/ml, and serum serotonin level of 883 ng/ml. TTE showed no valvular abnormalities. A small bowel mass was resected along with a right hemicolectomy his diarrhea improved.

Discussion

Small bowel neuroendocrine tumors are found incidentally as they are mostly asymptomatic. These tumors are usually underdiagnosed especially in patients who do not receive screening colonoscopy, like in our patient. Often these tumors metastasize to the lymph nodes and liver and are diagnosed at a late stage. 24-Hour urine excretion of 5-HIAA has high sensitivity and specificity for carcinoid syndrome. However, in our patient, these levels were normal but elevated serotonin and chromogranin markers indicated further investigation. Symptoms are mostly associated with metastasis but our patient had characteristic chronic diarrhea with no metastasis. The recommended treatment for tumors that have not metastasized is a resection of the bowel mesentery, which resolved our patient’s illness.

Conclusion

This case focuses on chronic diarrhea being the sole presenting symptom in ileal carcinoid tumors without objective evidence of metastasis.

#204  Metastatic high grade poorly differentiated large cell neuroendocrine carcinoma of the rectum, a rare entity

H Khazrik*

C Kamireddy

R Brudnik

K Chakraborthy

S Singal

East Tennessee State University, Johnson City, TN

Case Report

Rectal neuroendocrine neoplasm (R-NEN) is a rare entity which accounts for less than 1 per 100,000 population in United States of America. Gastroenteropancreatic (GEP) neuroendocrine tumor (NETs) are classified based on grade and differentiation. Well-Differentiated Neuroendocrine Tumors include grade 1 and grade 2, and Poorly Differentiated Carcinomas (PD-NEC) enclose only grade 3 neoplasms with small cell and large cell subtypes. A subtype of the high-grade NET with well differentiated histology was recognized in 2019 WHO classification of NEN of digestive system. High-grade (G3) NET characterized by a high mitotic rate and/or high Ki-67 index (more than 20).

We present a case of metastatic poorly differentiated high grade rectal large cell NEC. 59 years old gentleman presented to hospital with abdominal pain, rectal bleeding, and weight loss. Imaging demonstrated a 6 cm mass in the rectosigmoid junction with subtotal obstruction and 2.3 cm para-aortic adenopathy. Colonoscopy noted subtotal obstruction with large rectal mass 8 cm from the anal verge. CEA was within normal range. Biopsy of both rectal mass and para-aortic lymph node was consistent with large-cell neuroendocrine carcinoma positive for CD56, synaptophysin, CDX2 with a Ki-67 > 95%. Numerous mitoses noted in lymph node. He underwent palliative loop sigmoid colostomy followed by palliative cytotoxic chemotherapy with Carboplatin and Etoposide. Atezolizumab immunotherapy was added with cycle four. Positron emission tomography scan after 4 cycles of chemotherapy revealed stable disease. Both the small or large cell poorly differentiated subtypes of high-grade neuroendocrine carcinomas (NEC) are aggressive tumor with high propensity for distant metastasis and an ominous prognosis. There is a general lack of data from prospective trials to guide treatment. These tumors show similarities in morphology and biologic behavior to small cell carcinoma of lung (SCLC); thus, treatment paradigms have paralleled those established for SCLC. Platinum-based cytotoxic treatment with etoposide or irinotecan represents the backbone of treatment for advanced/metastatic/unresectable disease. Further option of chemotherapy after platinum failure, include, fluoropyrimidine, irinotecan, temozolomide and oxaliplatin-based regimens. The efficacy of second line or later lines of chemotherapy is limited, with short median survival estimated between 5–10 months.

#205  Primary cutaneous diffuse large B-cell lymphoma leg type

H Khazrik*

C Kamireddy

R Brudnik

K Chakraborthy

D Jaishankar

East Tennessee State University, Johnson City, TN

Case Report

Primary cutaneous B-cell lymphoma (PCBCL) is a non-Hodgkin lymphoma, involving the skin in the absence of extracutaneous disease at diagnosis. The three main subtypes include primary cutaneous follicular lymphoma (PCFL), primary cutaneous marginal zone lymphoma (PCMZL) and primary cutaneous diffuse large B-cell lymphoma leg-type (PCDLBCL leg-type). While the first two subtypes are more common and an indolent disease, the PCDLBCL leg-type is an aggressive disease with poor prognosis. PCDLBCL leg-type is typically seen in elderly female patients, affecting the lower legs but 10–15% of cases arise at other sites. Pathologic diagnosis is based on morphology and immunohistochemical features of skin biopsy regardless of anatomic location.

A 62-year-old female on chronic steroids for autoimmune vasculitis, developed sacral plaques and ulcers associated with bilateral cutaneous leg nodules, vision changes and weight loss. Punch biopsy of the sacral wound revealed large-sized lymphoid cells positive for CD20, BCL2, BCL6, MUM-1 and Pax-5 with high Ki-67 (90–95%) and negative for CD10 consistent with PCDLBCL leg-type. Double/triple hit lymphoma was ruled out. PET-CT scan demonstrated a 15.2 x 10.6 cm centrally necrotic sacral, soft tissue mass, FDG avid, with no distant disease, lymphadenopathy or splenomegaly. Biopsy of lower extremity nodules, vitreous body and bone marrow were negative for lymphomatous involvement. MRI brain revealed a 6 mm mass in the left frontal region. CSF cytology was negative. Brain biopsy showed reactive gliosis and rare isolated large B-cells positive for BCL2, BCL6 and Ki67 consistent with minimal involvement with lymphoma. Patient initiated dose-adjusted R-EPOCH (rituximab, Etoposide, prednisone, vincristine, cyclophosphamide, doxorubicin) to be followed by short course cranial radiation treatment (XRT) between cycle 1 and 2. She had remarkable response in the sacral tumor size after cycle 1 but rapid CNS progression complicated with hospitalization, neutropenic fever, internal jugular vein thrombosis and large retroperitoneal hematoma while on anticoagulation. She received two sessions of XRT to the brain before she decided to adopt hospice due to deterioration of performance status.

PCDLBCL leg-type has a gene expression profile similar to activated B-cell subtype of diffuse large B-cell lymphoma (DLBCL). The frequency of MYC rearrangement and MYD 88 mutation is higher in PCDLBCL – leg type. The treatment approach is with rituximab plus anthracycline-based combination chemotherapy followed by involved-field XRT if needed. PCFL and PCMZL can be treated with single modality XRT or single agent Rituximab. Unlike PCFL and PCMZL, PCDLBCL leg-type has a high frequency of eventual extracutaneous relapse leading to a lower rate 5-year disease specific survival of PCDLBCL leg-type with 50% vs 95%,98% in PCFCL, PCMZL, respectively. We present here a case of PCDLBCL Leg-type in an unusual anatomical location with rapid CNS progression.

#206  Post transplant lymphoproliferative disorder complete response with single agent rituximab treatment

D Kim*

O Mohammadi

J Kim

P Vahhabaghai

S Singal

D Jaishankar

East Tennessee State University James H Quillen College of Medicine, Johnson City, TN

Case Report

Post-transplant lymphoproliferative disorders (PTLD) is a fatal lymphoproliferative disorder associated with immunosuppression that arises in 5–10% of patients after solid organ or hematopoietic stem cell transplant. We present a case of PTLD that achieved complete response with single agent rituximab.

A 60-year-old male with juvenile type I IDDM, renal failure, severe diabetic retinopathy (legal blindness), prior renal and pancreatic transplant on maintenance immunosuppressive therapy with tacrolimus and mycophenolate presented to the hospital with acute onset abdominal pain. Imaging demonstrated high-grade small bowel obstruction, and he underwent exploratory laparotomy with enterectomy and R2 resection (gross residual disease). Labs revealed mild LDH elevation with negative EBV and CMV PCR status. Surgical pathology showed tumor cells positive for CD45, CD20, PAX5, CD10, BCL6 and negative for CD3, CD117, CK, cam 5.2, synaptophysin, CD56, CD5, CD23, MUM1, BCL2, cyclin D1, SOX11, CD34 and TdT. Ki-67 staining was 80%. MYC rearrangement was negative. TP53 mutation was positive. Final pathology reported diagnosis of monomorphic post-transplant lymphoproliferative disorder with diffuse large B-cell lymphoma. Subsequently, his tacrolimus was dose-reduced, and mycophenolate was discontinued. In addition to reduction of immunosuppressives (RI), chemoimmunotherapy was recommended to the patient, but he declined chemotherapy due to his functional status and comorbidities. He was started on rituximab weekly for 4 cycles with PET-CT monitoring for treatment response. At the completion of 4 cycles of weekly rituximab, PET-CT scan was obtained, which did not reveal any significant tumor activity. Consolidation rituximab every 3 weeks for a total of 4 cycles as per guidelines was instituted. Repeat PET scans continued to show complete response without evidence of new lesions or lymphadenopathy. Deauville score was 1 at the 10-month mark since the initiation of rituximab.

PTLD covers a wide spectrum of malignancies ranging from polyclonal early lesions to monomorphic lymphoma, as presented in this case. Available treatments include immunosuppressive reduction, chemoimmunotherapy, antiviral therapy, cytotoxic T-cell therapy, surgery or radiation. Single agent rituximab therapy has shown minimal toxicity with good efficacy with approximately 60% complete or partial response (ORR). CHOP given sequentially or concurrently with the rituximab improves overall response rate (ORR) to 90%.

Despite complete response with single agent rituximab, close surveillance is required in our patient. Inability to discontinue immunosuppressive agents completely, and exclusion of chemotherapy such as CHOP treatment portends higher relapse risk. Our case highlights a patient on immunosuppressive agents for more than 20 years after solid organ transplant developing monomorphic PTLD that was successfully treated with single agent rituximab.

#207  Retroperitoneal fibrosis in a case of metastatic breast cancer – drug or disease?

J Kim*

C Kamireddy

S Oad

S Singal

K Chakraborty

East Tennessee State University, Johnson City, TN

Introduction

Retroperitoneal fibrosis (RPF) in the setting of metastatic breast cancer is a rare pathological phenomenon. Here is a case of a patient presenting with acute kidney injury (AKI), subsequently finding ureteral obstruction.

Case

A 33-year old female reported a palpable right breast lump. Mammography and ultrasound found to have a 5.8 cm mass with biopsy confirming estrogen and progesterone receptor negative, HER2 negative intraductal carcinoma of the breast. Three right axillary lymph nodes were positive for cancer. Positron emission tomography (PET) found left-sided lymphadenopathy with biopsy proving oligometastatic breast cancer. Treatment with curative intent was pursued after multidisciplinary discussion. Neoadjuvant therapy with protein-bound paclitaxel and atezolizumab was started. Stable disease was noted on imaging, and neoadjuvant treatment changed to doxorubicin and cyclophosphamide. Then, she underwent right mastectomy, axillary lymph node dissection, and adjuvant radiotherapy. Unfortunately after completion, metastasis to the bones and contralateral breast was found. Palliative sacituzumab govitecan, a recently approved antibody-drug conjugate agent, was started, completing three cycles. Surveillance labs showed creatinine of 6.4 mg/dL, hyperphosphatemia and hyperuricemia, initially concerning for tumor lysis syndrome. Moderate bilateral hydronephrosis was found, and ureteral stents were placed. Retrograde pyelogram noted proximal ureter kinking for which etiology was unclear. Post obstructive AKI recurred, and bilateral nephrostomy tubes were placed. A month later, repeat retrograde pyelogram showed left ureter narrowing, then mid ureter dilation and narrowing again proximally. Repeat PET showed no evidence of retroperitoneal disease. Exploratory laparoscopy revealed diffuse RPF. Urology recommended higher caliber ureteral stents for subsequent follow-up.

Discussion

Structures within the retroperitoneal space include the kidneys, ureters, duodenum, ascending/descending colon, parts of the pancreas, the aorta, inferior vena cava, and iliopsoas muscle. RPF can lead to compression of these structures. Causes of fibrosis can include infection, autoimmune disease, medications, trauma, abdominal surgeries, radiotherapy, and malignancy. Malignancy contributes to 8–11% of cases. Associated cancers may include lymphomas, sarcomas, metastatic breast, gastrointestinal, renal, prostate, and lung as well as carcinoid tumors. PET scan can be used for assessment, although was unrevealing in this case. There is limited data to suggest sacituzumab contributes to RPF, but immunotherapy has been reported in a few cases, also received by this patient.

Conclusion

Retroperitoneal fibrosis is a rare entity. Management entails treatment of the underlying disease, and prognosis secondary to malignancy is poor.

#208  Mixed phenotype acute leukemia: when two lineages meet

D Kim*

J Kim

A Sinha

D Jaishankar

K Chakraborty

East Tennessee State University James H Quillen College of Medicine, Johnson City, TN

Case Report

Mixed phenotype acute leukemia (MPAL) is a rare disease entity that comprises less than 1% of all acute leukemias. It has characteristics of both acute lymphoblastic leukemia (ALL) and acute myeloid leukemia (AML). MPAL with features of T-ALL are particularly uncommon. We present a case of T/myeloid mixed-phenotype acute leukemia not otherwise specified (MPAL NOS).

A 70-year-old male presented to the hospital with complaints of dizziness and weakness of 2 months duration. He reported significant weight loss and easy bruising on his limbs. He was pancytopenic (WBC 3.3 K/uL, Hgb 5.7 g/dL, plt 5 K/uL) at the time of the presentation. Systemic CT imaging showed diffuse lymphadenopathy. FNA of an inguinal lymph node revealed neoplastic cells with immunohistochemistry expression of CD3, CD5, TdT, suggestive of T-ALL. Subsequent bone marrow biopsy/aspiration showed 50% blasts and 30% monocytes with atypia. Morphology was consistent with AML with monocytic features. Immunophenotyping revealed predominant myeloid differentiation with expression of MPO, CD117, CD33, HLA-DR, CD15, and partial expression of CD34 and CD13. A smaller population, approximately 10–15%, expressed T-cell lineage markers – cytoplasmic CD3, CD5, and CD7, and partially expressed CD34. T-cell gene rearrangements and JAK3 mutations noted in various T-cell neoplasms were reported. BCR-ABL1 translocation t(9;22) and KMT2A rearrangement were not detected. Abnormal male karyotype with rearrangements of chromosome 8q and 20p were suggestive of a neoplastic state.

Based on 2016 revised WHO diagnostic criteria, this patient’s leukemia met criteria for T/myeloid MPAL NOS. Outcomes in MPAL is uniformly poor compared to typical AML or ALL. Factors associated with adverse outcome include age, WBC count, Philadelphia chromosome status (Ph), extramedullary involvement, and immunophenotype (worse outcome in T-myeloid). To date, there is no optimal treatment strategy based on randomized prospective clinical trial data. For Ph(-) MPAL, a general consensus is to treat with an ALL-like regimen followed by allogeneic stem cell transplant. The patient was supported with appropriate blood product transfusion and was evaluated for intensive chemotherapy and possible hematopoietic stem cell transplant. A regimen of cyclophosphamide, vincristine, doxorubicin, and dexamethasone alternating with methotrexate and cytarabine (HyperCVAD) was proposed. He opted for best supportive care after several discussions taking his age, co-morbidities and overall prognosis of MPAL into account.

MPAL is a rare and aggressive disease entity. Treatments are generally extrapolated from typical ALL and AML treatment strategies. It is important to determine Ph(+/-) status. Preliminary data has suggested that MPAL with t(9;22) is favorably responsive to TKI. Unfortunately, older patients typically have more aggressive disease biology and suboptimal functional status limiting intensive chemotherapy or allogeneic transplant options.

#209  Composite lymphoma comprising peripheral t-cell lymphoma not otherwise specified and b-cell follicular lymphoma

S Kolagatla*

S Chaudhary

N Moka

Appalachian Regional Healthcare, Lexington, KY

Case Report

Composite lymphoma is defined as coexistence of 2 or more morphologically and phenotypically distinct lymphomas in the same anatomical site. Composite lymphoma may include combinations of Hodgkin lymphoma (HL) and B- or T- cell Non- Hodgkin lymphoma (NHL); B- cell NHL and T- cell NHL; two distinct B- cell or T- cell NHLs. The incidence of composite lymphomas varies from 1% to 5%. The exact pathogenesis of composite lymphoma is unknown. It is challenging to establish the diagnosis and chemotherapeutic protocols for composite lymphomas. The treatment is usually directed towards the higher-grade component. Most of the cases show worse outcome with the median survival of 12 months. Here we report a case of peripheral T cell not otherwise specified (PTCL-NOS) and follicular B cell composite lymphoma (FL).

A 66-year-old female, smoker initially presented with generalized lymphadenopathy, B symptoms, hypercalcemia and exudative right-sided pleural effusion. CBC positive for moderate normocytic normochromic anemia and mild thrombocytopenia. Generalized metabolically active lymphadenopathy on PET scan (figure 1A).

Left axillary lymph node showed effacement of the architecture due to an atypical follicular proliferation composed of follicles varying in size, positive for CD20, CD79, OCT2, BCL2, BCL6 and MUM1.FL Grade-3 (figure 1B) was favored.

Polymorphic population in the background was positive for CD3, CD5, CD2, CD7 (with partial loss), CD30 and CD43 (figure 1C). Epstein Barr virus (EBV) B- cells were expanded. Based on morphology and immunohistochemistry, diagnoses of PTCL-NOS with high proliferative index was favored in the atypical T- cell population.

Recurrent hypercalcemia treated with pamidronate while awaiting diagnosis. Since PTCL-NOS is aggressive of the two lymphomas she is currently receiving brentuximab vedotin, cyclophosphamide, doxorubicin, and prednisone (BV+CHP). Given paucity of evidence to use rituximab and brentuximab vedotin concurrently. We plan to use rituximab after completion of BV+CHP followed by autologous stem cell transplant evaluation.

Abstract #209 Figure 1

A) PET scan generalized PET avid lymphadenopathy; B) Atypical follicular proliferation composed of follicles varying in size; C) Polymorphic population in the background was positive for CD3

#210  Emicizumab in patients with acquired hemophilia a (aha)

R Ksayer*

K Zakharia

C Leissinger

M Janbain

Tulane University, New Orleans, LA

Case Report

Acquired hemophilia A(AHA) is a bleeding disorder resulting from the development of neutralizing autoantibodies (inhibitors) against FVIII in individuals with previously normal hemostasis. Subsequent to the decrease in FVIII activity, life threatening bleeds may occur. The median age at diagnosis is 64–78 years old. 89% of the patients are diagnosed after bleeding events.

Management of AHA includes eradication of the inhibitor with immunosuppressive treatment(IST) and control/prevention of bleeding.

Acute bleeding is currently managed using FVIII bypassing agents: recombinant FVIIa(rFVIIa), activated prothrombin complex concentrate, recombinant porcine FVIII(rpFVIII).Their use is associated with high risk of thrombosis in patients with underlying comorbidities and they are not good as prophylactic agents to prevent bleeds.

Emicizumab, approved for use in congenital hemophilia A as a FVIII mimetic that bridges activated factor IX and factor X, can be proposed for prophylaxis in AHA. We hypothesize that Emicizumab use in AHA can reduce the number of bleeds after first diagnosis of AHA and the need for bypassing agents. It promotes outpatient treatment and improves overall outcome in AHA.

We are reporting 2 cases of AHA successfully treated with Emicizumab.

Case#1 is a 78 y/o lady with Rheumatoid Arthritis presenting with subcutaneous hematoma on left arm and hematuria. On admission hemoglobin(Hgb) was 4.4, FVIII 5%, FVIII Inhibitor 106BU. She was started on prednisone, rpFVIII, Rituximab and red blood cells(RBC)transfusions to keep Hgb >7(3 units used). Her FVIII inhibitor was 40 after a month(mo) on treatment and FVIII recovery was low despite increased doses of rpFVIII, suggesting a cross-reacting anti-porcine activity of her inhibitor. Emicizumab was started(6 mg/kg/week for 2 doses, 3 mg/kg/week for 2 doses then 1.5 mg/kg weekly until normal FVIII level and FVIII inhibitor is not detected). Bleeding stopped after starting Emicizumab, FVIII and FVIII inhibitor levels were 78%, <0.6 after 4 mo respectively.

Case#2 is a 73 y/o man with metastatic prostate cancer admitted with leg hematoma and melena. On admission Hgb was 8.5, FVIII <1% FVIII inhibitor >500BU. He was started on prednisone, rFVIIa then rpFVIII, Rituximab, and RBC transfusions to keep Hgb >8(13 units over 2 weeks).1 week after discharge,he came back with muscle bleed Hgb 7.4, FVIII 31,FVIII inhibitor 362. FVIII recovery was inappropriate with rpFVIII. Emicizumab was started. 6 weeks later, FVIII levels recovered and the inhibitor was not detected.

In conclusion,the use of Emicizumab in AHA patients achieved marked improvement in preventing bleeding and ameliorated the overall outcome. No thrombosis were reported in both cases, nor re hospitalisation for bleeding events after starting Emicizumab. Larger studies are needed to support the benefit of initiation of such therapy at earlier stages in patients presenting with AHA.

#211  Lymphoma: a great masquerader

SM Lanka*

D Manogna

NS Saba

Tulane University School of Medicine, New Orleans, LA

Case Report

We describe the case of a 64 year-old man who presented with severe rigors, fevers, chills, night sweats, and associated unintentional weight loss. Initial computed tomography (CT) scan of the chest showed multiple lung nodules and enlarged mediastinal and hilar lymph nodes. CT of the abdomen and pelvis showed splenomegaly and multiple enlarged retroperitoneal, pelvic, and bilateral inguinal lymph nodes. Subsequent left upper lobe lung biopsy, right cervical lymph node fine needle aspirate, and left inguinal lymph node excision revealed increased eosinophils with extensive non-caseating granulomas. Cytology showed atypical cells but was indefinite for neoplasia or a B/T cell lymphoproliferative disorder. Prednisone, methotrexate, and infliximab were initiated for presumed sarcoidosis, and he was discharged from the hospital.

However, three months later, he re-presented with persistent rigors, fevers, chills, and weight loss. His symptoms did not improve with sarcoidosis treatment. CT showed persistent pleural-parenchymal opacities and thoracic lymphadenopathy with increased size of bilateral axillary lymph nodes. Cervical lymph node excision and bone marrow biopsy were performed and returned diagnostic for angioimmunoblastic T-cell lymphoma (AITL). Perivascular aggregates of atypical intermediate to large sized lymphoid cells were positive for CD3, CD4, CD5, CD10 and BCL6. The CD23 stain revealed disruption of follicular dendritic cell meshwork encasing high endothelial venules of the vascular proliferation. The patient was initiated on brentuximab, cyclophosphamide, doxorubicin, prednisone (A-CHP) regimen for chemotherapy. Repeat CT chest after cycle 1 of chemotherapy showed interval improvement in diffuse lymphadenopathy, indicating response to treatment.

AITL is a subtype of mature peripheral T-cell lymphomas derived from dysregulation of CD4+ T-follicular helper cells. It generally presents with constitutional symptoms, generalized lymphadenopathy, and hepatosplenomegaly, with a median age of diagnosis of 60 to 65 years. It can be associated with autoimmune features like hemolytic anemia, rheumatoid arthritis, and thyroiditis. The overall prognosis is poor, with a 5-year overall survival of 33 percent and a 5-year failure-free survival rate of 18 percent.

Our patient presented with presumed granulomatous disease consistent with sarcoidosis, had worsening of symptoms on immunosuppressive therapy, and was subsequently diagnosed with AITL. There are many similarities in imaging and clinical manifestations between the early stages of AITL and pulmonary sarcoidosis. AITL is often found with laboratory and autoimmune findings that can mask diagnosis, potentially delaying initiation of treatment. AITL should be suspected in patients not improving on therapy for an infectious or autoimmune process, with early consideration for re-biopsy. This can aid in early diagnosis and treatment of this aggressive disease, resulting in improved survival rate.

#212  Warm hemolytic anemia and hodgkin lymphoma

S Leon Paredes*

O Sanchez

University of South Alabama, Mobile, AL

Case Report

16-year-old female presented with dyspnea, light-headedness and fatigue for 2 days. Her past medical history was significant for anemia due to menorrhagia 5 months prior to admission. On physical exam she was found pale, tachypneic, tachycardic with normal pulse oximetry, wide pulse pressure and hepatomegaly. Blood work showed hemoglobin 3.6 g/dL, hematocrit 12.6%, MCV 115 fL, MCH 33 pg, RDW 29.3 fL with reticulocytes 47%, Ferritin 416 ng/mL, iron 98 mcg/dL and TIBC 382 mcg/dL, white blood count 22,200 mcL and total bilirubin 2.5 mg/dL, direct Coombs positive and warm antibodies positive.

Patient was diagnosed with high output cardiac failure due to autoimmune hemolytic anemia (AIHA) with warm antibodies. She was started on methylprednisolone and norethindrone.

Chest X-ray showed right upper lobe and left paravertebral masses, along with widened mediastinum. CT chest demonstrated enlarged lymph nodes with bilateral lung involvement and encasement of the trachea, consistent with lymphoma. Abdomen/pelvis CT revealed hepatomegaly, multiple splenic masses and multilevel nodal involvement. Supraclavicular lymph node biopsy performed demonstrated nodules of small lymphocytes admixed with areas of fibrosis and Reed-Sternberg-like cells but no diagnostic Reed-Sternberg cells. The atypical cells were strongly positive for CD15 and CD30, and weakly positive for PAX-5. Findings that are suggestive of Nodular Sclerosis Classic Hodgkin Lymphoma.

Anemia was treated with transfusions and hemolysis with dexamethasone until chemotherapy initiation with brentuximab, vedotin, doxorubicin, dexrazoxane, vinblastine and dacarbazine. After cycle 2 repeat CT chest showed positive response to treatment, Deauville score 3.

Autoimmune cytopenias (AICP), are associated with lymphoproliferative disorders particularly chronic lymphocytic leukemia and non-Hodgkin lymphoma. The etiology of AICPs is unknown, there are different theories such as they are triggered by paraneoplastic cytokine release or production of autoantibodies by the tumor. Warm antibodies are the typical IgG variate present and leads to RBC loss by splenic removal of sensitized cells.

Hodgkin’s lymphoma (HL) is rarely associated with autoimmune cytopenia, if at all present, it is usually at the time of diagnosis or relapse. There is a prevalence of 0.5–4.2%. The first-line treatment of AIHA is generally corticosteroids although, it seems to be less effective in the setting of HL. In some cases, treating the primary disease with chemotherapy leads to resolution of AIHA.

This case highlights the importance of further work up in pediatric patients with autoimmune hemolytic anemia (AIHA) with warm antibodies, considering than in about half of the patients AIHA is secondary to another pathology.

It also displays the significance of developing a thorough differential diagnosis even on frequent pathologies like anemia avoiding early closure.

#213  Healing power of mind body hypnosis

R Mahadevan*

S Gupte

L Linquest

C Nathan

LSU Health Shreveport, Shreveport, LA

Purpose of Study

Clinical hypnosis has been defined as a mind-body therapy that involves a deeply relaxed state, individualized mental imagery, and therapeutic suggestion. Head and neck cancer patients suffer from significant pain and distress ranging from xerostomia, anxiety, depression, surgery disfigurement, and post-radiation fibrosis resulting in several burdensome sequelae including dysphagia and decreased quality of life. Hypnosis is one of the most frequently cited forms of nonpharmacologic cognitive pain control therapy now growing in use in cancer care. Our objective is to demonstrate how clinical mind-body hypnosis can improve dysphagia in head and neck cancer survivors.

Methods Used

This will be a randomly double-blind controlled study. The aim is to understand the effect of once-daily hypnosis therapy on dysphagia severity over an eight-week treatment period. Furthermore, this study seeks to understand the necessary dosage of hypnosis therapy to influence various prevalent symptoms including mood, pain, self-image, and dysphagia in head and neck cancer survivors. One group of patients will be given the dysphagia audio to listen to daily for eight weeks while the other group will rank their top four symptoms from most to least bothersome and listen to two weeks of hypnosis audios for each symptom, totaling eight weeks. In both patient groups, pre-surveys will be given before hypnosis therapy and once weekly surveys will be administered throughout the hypnosis therapy. Final post surveys will be given at week 12 at the end of the study. As an objective measure, all participants will have a FEES (fiberoptic evolution of swallowing) study before and after the intervention. The subjective surveys administered throughout the study include Pre and Post MDADI questionnaire and PROMIS questionnaires on mood, sleep, pain, and quality of life.

Summary of Results

This study is currently in process.

Conclusions

This study is currently in process.

#214  A promising alternative: neoadjuvant endocrine therapy in estrogen and progesterone receptor-positive breast cancer

H Malik*

C Pham

A Garcia

LSU Health New Orleans, New Orleans, LA

Case Report

Neoadjuvant therapy (NT) is a treatment administered to breast cancer (BC) patients prior to surgery with either locally advanced breast cancer (LABC) or to facilitate breast-conserving surgery. In the US, neoadjuvant chemotherapy (NCT) is the most commonly used type of NT. However, an alternative is neoadjuvant endocrine therapy (NET). It has a lower toxicity profile and is suggested to be as effective as NCT. We will present 5 cases to discuss this underutilized approach.

The median age was 61 years old (range 46–77), and four patients were postmenopausal. Three patients had invasive ductal carcinoma, and two had lobular carcinoma. All patients had tumors that were strongly estrogen receptor-positive (ER+) and HER2 negative. Four had low Ki67. The 21 gene recurrence score (RS) was measured in three, and all were low. The median duration of therapy was four months (range 2–10). Three patients experienced a partial tumor response, two patients had no response. Four patients are alive, and one patient who didn’t respond developed metastatic disease and expired four years after diagnosis.

NCT is the most commonly used NT in treating BC. However, most BCs are ER+, and this subtype has been reported to respond poorly to chemotherapy. There is evidence proposing that NET may be more appropriate than NCT in certain BC subtypes. A randomized trial suggested a higher response to NET than NCT in ER+ BC. Additionally, other factors may help identify patients more likely to benefit from NET. Decreased Ki67, a marker for cell proliferation, suggests NET response, and low tumor gene profiling RS may identify patients unlikely to respond to chemotherapy.

Currently, NET is used in elderly or frail patients with ER+ BC because they cannot tolerate NCT. The use of biomarkers and tumor gene profiling may allow for the expansion to additional patient populations appropriate for NET. Furthermore, they may be spared the toxicity of NCT. Ongoing trials are evaluating this.

Abstract #214 Table 1

Patient treatment summary

#215  Hemolytic anemia secondary to methemoglobinemia after bupivacaine use

O Mohammadi*

H Khazrik

M Raafey

S Singal

K Chakraborty

East Tennessee State University, Johnson City, TN

Case Report

In methemoglobinemia, ferrous ions in hemoglobin convert to ferric ions and are unable to carry the oxygen to the tissues. Common causative agents are topical anesthetics like bupivacaine or lidocaine, antibiotics, rasburicase, nitrates, and chemical substances. Hypoxia and hemolysis are two adverse outcomes of methemoglobinemia. We present a scenario of methemoglobinemia-induced hemolysis.

This patient is a forty two-year-old male with a past medical history of type 1 diabetes and end-stage renal disease who got admitted due to osteomyelitis and underwent bone excision and debridement. Shortly after surgery, the patient started having shortness of breath and fatigue. Oxygen saturation dropped to 74% and hemoglobin trended down to 5.2 g/dl. Baseline hemoglobin was around 11 g/dl. The patient had been exposed to bupivacaine during surgery. Laboratory findings showed lactate dehydrogenase (LDH) of 650 U/L and total bilirubin of 2.9 mg/dl. Coombs’ test showed positive IgG and C3. Methemoglobin level peaked at 2.4%.

Glucose-6-phosphate dehydrogenase(G6PD) deficiency, myeloma panel, hepatitis, human immunodeficiency virus, paroxysmal nocturnal hemoglobinuria flow cytometry, hemoglobin electrophoresis, and cold agglutinin were unremarkable. Peripheral blood smear showed normocytic anemia with mild red cell clumping and spherocytosis.

The patient denied any active bleeding and underwent endoscopy which showed a clean based duodenal ulcer.

Laboratory findings were consistent with hemolysis.He responded poorly to blood transfusion, intravenous immunoglobulin showed short-term improvement and then started on prednisone 1 mg/kg which stabilized his hemoglobin, methemoglobin, LDH, and bilirubin.

Bupivacaine is a known cause of methemoglobinemia. Typical presentations of methemoglobinemia are cyanosis with low oxygen saturation, but normal arterial oxygen saturation and usually seen when methemoglobin level is more than 15%. Prompt diagnosis of methemoglobinemia is important to avoid life-threatening complications. Our patient presented with shortness of breath, hypoxia and anemia shortly after exposure to bupivacaine.

Methemoglobinemia-induced hemolysis is more common in patients with G6PD deficiency which was negative in the above patient. It should be noted that hemolysis can be seen even without G6PD deficiency.

Treatment cornerstone is to stop the offending agent. Methylene blue is indicated in methemoglobinemia when the patient is symptomatic and the methemoglobin level is more than 20%. Also, methylene blue can cause further hemolysis if a patient has G6PD deficiency and should be avoided in this population. Methylene blue was not indicated in our case due to lower levels of methylene blue.

#216  Hemophagocytic lymphohistiocytosis sans triggers

O Mohammadi*

D Kim

B Youssef

S Singal

D Jaishankar

East Tennessee State University, Johnson City, TN

Case Report

Hemophagocytic Lymphohistiocytosis (HLH) is an hyperinflammatory state due to hyperactivation of macrophages and T-cells which rarely affects adults. It can be familial or sporadic. Triggers are infections, auto-immune diseases, malignancies, and immune checkpoint inhibitors. HLH diagnostic criteria are fever, splenomegaly, bicytopenia, hypertriglyceridemia, hemophagocytosis, low/absent NK-cell-activity, elevated ferritin, and high-soluble interleukin-2-receptor (IL-2R). Five out of eight criteria are required for diagnosis.

A 54-year-old female was noted to have leukopenia during a routine visit with her family physician. Follow up labs revealed worsening leukopenia, anemia and a normal platelet count. She received Amoxicillin/Clavulanic acid for a presumed upper respiratory infection and developed nausea, diarrhea and decreased appetite. She was referred to Hematology Oncology for leukopenia. During workup she developed fatigue, night sweats and high fevers. Workup revealed WBC 2400 mcL, microcytic anemia, transaminitis with lactate dehydrogenase of 1725 U/L and ferritin of >15000 ng/mL . Peripheral blood smear showed leukopenia without immature cells or blasts and mild microcytic erythrocytes. Further tests detected CXCL-9 of 125050 pg/mL, D-dimer of >5000 ng/mL and interleukin-2- receptor of 20604 pg/mL. EBV, CMV, HSV, HHV-6, parvovirus, bartonella, leishmaniasis, bacteria and COVID-19 were negative. Computed tomography of the chest, abdomen and pelvis did not reveal lymphadenopathy. Brain imaging showed no abnormalities. Cerebrospinal fluid cytology was unremarkable. Bone marrow biopsy (BMBX) showed prominent histiocytic phagocytosis of erythroid precursors and platelets. HLH-94 treatment protocol including weekly steroid and etoposide initiated. Patient’s fever, night sweats and leukopenia resolved during hospitalization, with subsequent down trending of ferritin to 103 ng/ml, CXCL-2 to 2663 pg/mL and interleukin-2-receptor to 2,265 pg/mL. Repeat BMBX revealed significant improvement.

HLH is a rare life-threatening diagnosis. This patient with nonspecific symptoms was diagnosed with HLH (fever, bicytopenia, elevated ferritin, high-soluble IL-2R and hemophagocytic lymphohistiocytosis on BMBX). Several HLH gene mutations were tested including PRF1, UNC13D, STXBP2, although none was mutated. No infectious, rheumatologic or oncologic triggers were detected. Early diagnosis and treatment are critical. Without treatment, survival is measured in months due to multiorgan failure. This syndrome rarely presents in the absence of triggers which may cause delay in diagnosis and successful treatment. 5-year overall survival with HLH 94 protocol is 54% as opposed to 0% prior to the advent of this protocol. Etoposide and steroids are the mainstay of HLH-94. Cyclosporine can be added in the maintenance phase and hematopoietic stem cell transplant is reserved for familial or relapsed HLH.

#217  Treatment of burkitt leukemia in adolescents: an unmet need

K Parmar*

A Jones

D Pawar

K Nugent

K Bharathidasan

J Rios

S Rehman

Texas Tech University Health Sciences Center, Lubbock, TX

Introduction

Non-Hodgkin’s lymphoma accounts for approximately 7% of cancers in patients under 20 years.

Case presentation

A 21-year-old male presented with diffuse abdominal pain and weight loss. Abdominal exam showed a soft, nontender abdomen with absent bowel sounds and a hard mass inferior to the umbilicus. CT scan showed a 12 cm x 7 cm lobulated soft tissue mass in the upper abdomen posterior the stomach and surrounding the mesenteric vessels. A biopsy of the abdominal mass showed markers consistent with Burkitt lymphoma. MYC gene rearrangement was detected. LDH was 1501 u/l. MRI-Brain showed extra-axial material over cerebral convexities. Due to patient being an adolescent and evidence of better outcomes with pediatric regimens, he was started on COG ANHL1131 regimen. Pre-phase with cyclophosphamide, vincristine and prednisone was begun. Patient developed tumor lysis syndrome (TLS) with phosphorus 15.5 mg/dL despite being on prophylaxis. Patient required continuous renal replacement therapy. Ommaya reservoir was placed for intrathecal chemotherapy days 2,4 and 6. He tolerated this well with tumor lysis syndrome resolving. Repeat CT of the abdomen on hospital day 25 showed resolution of the mass found at presentation. On hospital day 31 the induction phase was begun with COPADM2- rituximab, vincristine, prednisone, high dose methotrexate, folinic acid, cyclophosphamide, and doxorubicin. On hospital day 50 round 2 of induction phase was started. The patient tolerated this course well and was discharged on hospital day 57. Repeat CT Abdomen showed disappearance of the mass.

Abstract #217 Figure 1

A-CT Abdomen with contrast showing approximately 12 cm tra nsverse x 7 cm large area of lobulated soft tissue mass in upper abdomen posterior to the stomach and surrouniirg the mesenteric vessels. This multilobulated soft tissue mass encases majority of the mesenteric vessels and completely obscures the pancreas. B-complete resolution of the mass on repeat CT scan

Discussion

Non-Hodgkin lymphomas (NHLs) occurring in children and adolescents and young adults (AYA) are characterized by various age-related differences in tumor biology and survival. For the most part, there is remarkable divide in how pediatric patients (under the age of 18 years) with lymphoma are treated vs their young adult counterparts, and molecular data are lacking, especially in pediatric and AYA series. Due to the paucity of clinical trials in this age group, the impact of these different strategies on outcomes is not well known and needs to be studied.

#218  Vancomycin induced thrombocytopenia-a possible drug reaction

K Parmar*

A Gutal

D Pawar

G Del Rio-Pertuz

K Nugent

Texas Tech University Health Sciences Center, Lubbock, TX

Introduction

Vancomycin is considered relatively safe, but hematologic side effects have been reported infrequently.

Case Report

A 77-year-old man presented with generalized weakness. His medical conditions included peripheral arterial disease, diabetes mellitus, hypertension, and hyperlipidemia. Five weeks before, patient underwent trans metatarsal amputation of left foot due to osteomyelitis and was discharged on vancomycin and ceftriaxone for six weeks. His other medications were lisinopril, januvia, aspirin, atorvastatin, clopidogrel, ceftriaxone and Tylenol. Physical examination was unremarkable. His labs were significant for a platelet count of 3K/uL and hypokalemia. The platelets were elevated on previous admission. Coagulation panel showed an elevated prothrombin time and international normalized ratio(INR). He received enoxaparin last admission. HIT antibody was negative. Peripheral blood smear showed no schistocytes. Vancomycin was held. He received 2 bags of platelets and two doses of intravenous immunoglobulin. His anti-platelet antibody and vancomycin dependent antibodies were negative. Three days later, his platelets went up to 130X103/uL, and the patient was discharged.

Discussion

Drug induced thrombocytopenia (DIT) is usually associated with bleeding however, our patient was asymptomatic. The thrombocytopenia was seen 33 days after drug initiation. Usually, DIT is known to present 5–10 days after exposure. Antibody testing was negative which could be due to prior immunoglobulin administration or a nonimmune mechanism which is rarely reported.

#219  A very unlikely transformation

R Patel*

GD Gibson

C Milner

The University of Mississippi Medical Center, Jackson, MS

Introduction

Chronic Lymphocytic Leukemia (CLL) and Mantle Cell Lymphoma (MCL) are two lymphoid neoplasms characterized by the proliferation and accumulation of mature small CD5+ B cells that may involve bone marrow, blood, lymphoid tissues, and extranodal sites.1 CLL accounts for about 25–30% of all lymphoid neoplasms; while MCL accounts for only about 6% of all B-cell lymphomas.2Richter syndrome (RS) is defined as the transformation of CLL into an aggressive lymphoma, most commonly diffuse large B-cell lymphoma. RS occurs in approximately 2%-10% of CLL patients during their disease.4 Here we report a case of a patient with a highly unusual RS with CLL transformation to MCL.

Case presentation

A 76 year old female with history of CLL presented with progressive fatigue and abdominal discomfort. Physical exam was significant for abdominal distension and splenomegaly. Computed Tomography (CT) imaging revealed massive splenomegaly and diffuse adenopathy in chest/abdomen/pelvis. Patient with a history of multiple treatments for CLL including Bruton tyrosine kinase (BTK) inhibitors but has not been able to tolerate therapy due to side effects with medications. Initial workup revealed a white blood cell count (WBC) >600,000 and lactate dehydrogenase (LDH) >2500. Peripheral flow cytometry analysis showed CD23 negative, CD200 negative, and FMC7 positive. Fluorescence in situ hybridization analysis with extra signal of CCND1 in 86% of cells. Cytogenetics showed a deletion of 13q14. Overall findings consistent with MCL. Patient treated with Obinutuzumab and dose reduced CHOP (cyclophosphamide, doxorubicin, vincristine, and prednisone). Patient did not tolerate Obinutuzumab due to facial edema, however CHOP therapy was continued. She received a dose of rasburicase and required brief dialysis due to concern for tumor lysis syndrome. Following chemotherapy, patient had overall symptomatic improvement. WBC and LDH down trended to 395k and 1649 respectively at day of discharge. At follow up, patient reported improvement in abdominal discomfort, however patient’s daily activity remained predominantly bed bound. Discussed continued therapy with another cycle of CHOP vs Acalabrutinib/Venetoclax, or hospice. After further family discussions, patient and family collectively decided to pursue hospice care.

Discussion

MCL accounts for approximately 5–10% of non-Hodgkin’s lymphoma and is less likely to occur from RS of CLL. MCL has an aggressive clinical course with poor response to conventional chemotherapy and a median overall survival of 3–4 years. Deletion of 13q14 is significantly associated with a poorer outcome. MCL in the setting of Richter syndrome is a poor prognosis and it is important to have collaborative discussions with patients to make decisions on treatment options and goals of care.

#220  A case of hlh in a patient with rcc and mds

D Pawar*

K Parmar

A Deb

S Duangkham

Z Elharabi

S Rehman

Texas Tech University Health Sciences Center, Lubbock, TX

Introduction

Hemophagocytic lymphohistiocytosis (HLH) is a life-threatening syndrome of abnormal immune activation causing cytokine storm leading to systemic inflammation and multiple organ failure. It is rare in the adult population and initial presentation can be sub-acute that can progress rapidly. Early treatment improves survival, but diagnosis is challenging and requires a high index of suspicion.

Case report

A 69-year-old male presented with 2 days of hematuria, nausea/vomiting, poor oral intake and a syncopal episode due to dehydration that was resolved with intravenous fluids. He had a one-month history of generalized malaise, fatigue, subjective fever, night sweats, and 7lb weight loss. His past medical conditions were significant for psoriatic arthritis, hypothyroidism, and diffuse large B cell lymphoma that was treated 7y ago. His family history was significant for father with psoriasis, mother with breast cancer, and lupus and breast cancer in his sister.

Initial labs showed pancytopenia with WBC 2000, platelets 45000, hemoglobin 10.3, mild transaminitis, elevated total bilirubin of 1.8, and acute kidney injury with Cr 1.8. CT abdomen revealed cholelithiasis, 19 cm splenomegaly, and a 4x3 cm right renal mass which was biopsied and found to be grade 1 clear cell renal cell carcinoma (RCC). Two days after presentation, his pancytopenia had worsened with WBC of 830 (ANC 547), platelets 39, hemoglobin 9, and patient developed neutropenic fever of 102.9F. Workup was negative for infectious etiology. Peripheral blood smear was unrevealing. Bone marrow biopsy showed hypercellular marrow with 0% blasts, consistent with low-risk myelodysplastic syndrome (MDS). His transaminitis, bilirubinemia, renal function continued to worsen with AST in 800s, ALT in 250s, mixed bilirubinemia of 8.4, BUN 110, Cr 4.8, anion gap acidosis of 20, respectively. Five days after presentation, DIC panel was significant for coagulopathy with severe hypofibrinogenemia of 80 and D-Dimer 15688. Ferritin level had increased to 14102 from 5344 at presentation. Fasting triglycerides were elevated at 284. Clinical picture was suspicious for HLH and further HLH workup revealed his HScore to be 269, indicating 99% probability of HLH. He was started on HLH-94 protocol based treatment with improvement of lab parameters, but patient elected to pursue comfort care after 2 cycles of treatment due to severe treatment related toxicity.

Discussion

HLH has been associated with predisposing genetic defects, infections, malignancies, and rheumatologic conditions. Our patient did not show signs of recurrence of lymphoma, however both MDS and RCC have been associated with HLH and our patient had history of rheumatologic disorders. Diagnosis involves fulfilling 5 out of 8 diagnostic criteria; our patient matched 6. H-Score > 250 indicates 99% probability of HLH. Untreated patients have survival of only months and treatment based on HLH-94 protocol can improve survival.

#221  A case of dvt and pe and cardiac arrest in a patient with factor v leiden mutation and covid

D Pawar*

K Parmar

G Del Rio-Pertuz

C Morataya

Z Elharabi

M Abohelwa

K Nugent

Texas Tech University Health Sciences Center, Lubbock, TX

Introduction

Heterozygous Factor V Leiden (FVL) mutation is the most common inherited thrombophilia, most common in people of Northern European descent and in some Middle Eastern population. It increases risk of developing a deep venous thrombosis (DVT) by 5- to 7-fold, and is considered a weak risk factor, and most people never develop blood clots.

Case report

19-year-old woman presented with left lower quadrant abdominal pain, left groin pain, chest tightness and shortness of breath that had started 1 week prior to presentation. Patient had tested positive for COVID-19 six months but had only minor symptoms and recovered without needing treatment. She had completed 2nd dose of Moderna Covid vaccine two weeks ago and had been using oral contraceptive pills (OCP) for two years. Family history was significant for a paternal uncle with history of blood clots. Physical exam revealed swelling and erythema in left lower extremity up to groin area. Doppler ultrasound showed an acute DVT in the left external iliac vein, common femoral vein, deep femoral vein, superficial femoral vein, and proximal popliteal vein. Spiral computed tomography imaging of chest showed pulmonary emboli (PE) in the segmental branches of the pulmonary arteries with mild dilation of the right ventricle. Cardiac echogram (TTE) showed intact right ventricular function. Patient underwent mechanical aspiration thrombectomy with extirpation of DVT, but the procedure was complicated by PEA arrest requiring CPR for about 50–60 mins. During episode, bedside TTE showed evidence of right ventricular dilation and RV strain concerning for a massive PE. She was cannulated for V-A ECMO then underwent therapeutic hypothermia, and was successfully decannulated after six days. Patient eventually recovered and was neurologically intact. She was discharged home on warfarin and aspirin. Hypercoagulable work-up was remarkable for heterozygous FVL mutation R506Q. Both her father and mother were found to have heterozygous FVL mutation and her sister was found to have homozygous FVL mutation.

Discussion

People with FVL have additional risk factors that contribute to the development of DVT, and FVL alone does not increase the risk of developing arterial thrombosis, heart attacks and strokes. DVTs in heterozygous FVL population are considered provoked DVTs requiring anti-coagulation for a definite period, like that in general population. Curiously, patients with severe COVID-19 infection requiring ventilator support were found to have factor V levels high above the normal reference range and were found to have elevated risk for blood clots. Use of OCPs, and perhaps a recent COVID infection, although mostly asymptomatic, might have contributed to hypercoagulability in our patient. FVL mutation is not considered a contraindication to having COVID vaccination and patients with hereditary clotting disorders are recommended to have the vaccine.

#222  Acute myepolathy in an acute myelogenous leukemia

A Pivovarova*

S Ahuja

C Milner

The University of Mississippi Medical Center, Jackson, MS

Case Report

A 23 year old Choctaw female with history of acute myeloid leukemia (AML) in first clinical remission presented with painful neuropathy and bilateral ascending paralysis. Two months prior, patient was diagnosed with AML and treated with induction therapy with cytarabine and daunorubicin, along with one dose of prophylactic intrathecal methotrexate due to high white blood count at presentation. About a month prior to presentation, she received Cycle 1 of consolidation with HiDAC. She tolerated consolidation therapy with HiDAC well and was discharged home. Three weeks following consolidation, she presented with acute onset of pain and progressive arm and leg weakness. ANA reflex, acetylcholine receptor antibody, Mu SK antibody and CSF paraneoplastic panel were negative. MRI of the spine, and brachial plexus unremarkable. MRI of the brain with small incidental lesion likely related to PRES in a setting of low sodium and plasma exchange, not suggestive of AML involvement. EMG revealed predominantly motor and some sensory polyneuropathy. Cerebrospinal fluid studies significant for elevated protein and cytology without malignancy. Studies for GBM, GM1/2, GDMG, GR1B were negative. Due to concern of potential chemotherapy induced neurologic event, patient underwent plasma exchange for 5 sessions and was on a prolonged prednisone taper. Unfortunately, she failed to respond to these measures. Sural nerve biopsy was obtained which favored cytarabine toxicity.Repeat MRI brain revealed findings consistent with PRES sequela vs metabolic/toxic insults and encephalitis. Patient had a prolonged hospital stay after developing demyelinating polyneuropathy which led to her becoming ventilator dependent requiring tracheostomy placement. Due to her acuity of illness and lack of neurologic improvement, she was not given further chemotherapy. She subsequent developed a rapidly progressing relapsed AML with worsening leukocytosis, anemia and thrombocytopenia. Her clinical status continued to decline. She was developed a multiorgan failure requiring maximum pressors and cardioversion, and subsequently family elected to withdraw further life sustaining care. HiDAC regularly used as induction and consolidation therapy in AML patients. Although central nervous toxicity of HiDAC has been frequently reported and is well recognized, there are only a few publishes cases of polyneuropathy following its administration (Openshaw et al 1996). Previous study by Openshaw et al. determined a 1% prevalence rate of demyelinating polyneuropathy among 194 courses of HiDAC (Openshaw et al. 1996). Although different mechanisms of HiDAC toxicity and resulting polyneuropathy have been proposed, it still remains largely unknown.

#223  From good to worst: gaining an extra chromosome in burkitt-type acute lymphoblastic leukemia

A Rojas Figueroa*

Hospital Municipio de San Juan, San Juan, Puerto Rico

Case Report

Burkitt Lymphoma and Burkitt leukemia are classified as different manifestations of the same disease. A combination of several diagnostic methods including morphological, cytogenetics, chromosomal analysis, and immunophenotyping are necessary to diagnose B-Cell Acute Lymphoblastic Leukemia (B-ALL) with certainty. Cytogenetic abnormalities frequently seen in both manifestations include the translocations t(8;22)(q24;q11), and least frequently, the translocation t(2;8)(p12;q24) involving the immunoglobulin kappa gene locus on 2p12.

We present a rare case of a 51-year-old male patient without prior medical history who arrived at the urgency room after various episodes of epistaxis of two days of evolution as well as a petechial rash on the upper left arm and axilla. Peripheral blood examination showed hemoglobin of 10.4 g/dL, hematocrit 29.90 and platelets 23 x 10^8/L, white blood cells 12.8 x 10^7, with a lymphocytic predominance, atypical lymphocytes, and immature cells. Increased LDH at 2700 IU/L, hypercalcemia of 15.2 mg/dL, and increased alkaline phosphatase at 531 IU/L, and total bilirubin 1.64 mg/dL. HIV test and Hepatitis panel were negative. Abdominal Ultrasound was remarkable for hepatosplenomegaly. No evidence of abnormal lymphadenopathy in the chest, abdomen, or pelvis. Bone marrow biopsy was compatible for B-ALL with increased TdT-positive lymphoblast as expected in ALL. Analysis of the flow cytometric data showed a population of B-lymphoblasts (CD10+/CD19+/CD20-/CD10+/CD38+/HLA-DR+). Although the majority of the B-lymphoblast were negative for surface light chain, a minority demonstrated a tendency towards lambda light chain restriction. Chromosome analysis showed an abnormal karyotype with an apparently balanced t(8:9) (q24.;p13) involving MYC at 8q24.2 and an unknown gene on 9p13, together with the gain of chromosome X, 13, 20, and CRLF2 compatible with B-ALL with MYC rearrangement (formerly Burkitt leukemia). MYC translocations are known to be associated with an unfavorable prognosis in B-ALL.

The t (8:9) is a rare yet recurrent abnormality in B-ALL. Chromosomal aberrations in addition to t(8:14) are often present in B-ALL, most commonly affecting chromosomes 1, 6, 7,13, 17, and 22. To the best of our knowledge, B-ALL with this unknown gene on 9p13, together with the gain of chromosome X, 13, and 20 have not previously been reported. Over 60% of patients diagnosed with B-ALL and additional aberrations will have a high risk for relapse or mortality. Literature review regarding treatment alternatives for patients with these cytogenetic changes in the adult population is scarce. Our patient was started on HyperCVAD chemotherapy as induction and will be evaluated by a bone marrow transplant center.

#224  An underdiagnosed hemolytic anemia in alcoholic liver disease: zieve syndrome

D Román-Colón1*

J Feliciano-Idelfonso1

O Rodriguez-Amador1

LO Gerena Montano2

L Vazquez-Zubillaga2

O Cantres-Fonseca2

1VA Caribbean Healthcare System Mental Health Services, San Juan, Puerto Rico

2VA Caribbean Healthcare System, San Juan, Puerto Rico

Case Report

Anemia in alcoholics is a common finding which presents a broad differential diagnosis. An adequate classification requires a high index of suspicion. One of the causes is Zieve syndrome (ZS), a rarely reported disease with an unclear pathogenesis that results from alcohol abuse and consists of a triad: hemolytic anemia, jaundice and transient hyperlipidemia. This condition is frequently under-recognized and under-reported possibly due to physician unawareness and lack of pathognomonic signs or symptoms. We present a case in which a detailed evaluation for hemolytic anemia led to identification of a possible commonly missed diagnosis

A 58-year-old male with medical history of chronic liver disease secondary to alcoholism presented to the ER due to a one-week onset of left arm and leg ecchymosis secondary to a fall. Vital signs were remarkable for sinus tachycardia and orthostatism. Physical examination revealed an oriented man, a benign abdomen, scleral icterus, a large ecchymosis in his left mid arm and left lower extremity. Distal peripheral pulses and sensation remained preserved. Imaging without evidence of fractures or dislocation and abdominal CT with contrast was remarkable for chronic hepatocellular disease and a left iliacus muscle hematoma without signs of active bleeding. Laboratories revealed hemoglobin at 7.8 g/dL from 12 g/dL baseline, no leukocytosis, chronic thrombocytopenia, elevated total bilirubin with indirect predominance and multiple electrolytic deficiencies in the setting of malnutrition. Peripheral smear revealed anisocytosis with macrocytosis, burr cells and some target cells with associated elevated LDH, elevated reticulocyte count, decreased haptoglobin and negative direct antiglobulin test. Lipid profile and vitamin E were normal. HIV and hepatitis non-reactive. Patient required a total of 4 units pRBCs without signs of bleeding suspicious of non-immune hemolytic anemia. Patient’s hospitalization course then became complicated with alcohol withdrawal, septic shock secondary to aspiration pneumonia and hepatic encephalopathy requiring endotracheal intubation. Given poor prognosis, family decided for comfort measures.

When considering ZS the presence of acute-onset, extrinsic, non-immune hemolytic anemia helps distinguish the conditions. It is important to be aware of atypical presentations such as a normal lipid panel and vitamin E levels, which have been reported in literature and do not rule out the condition. Hyperlipidemia may be transient and can decrease before anemia presents. In these patients, anemia typically resolve with abstinence of alcohol intake. For this reason, it is important that clinicians confronted with hemolysis in a patient with alcoholic liver disease be aware of ZS as it will allow earlier diagnosis, prevent invasive intervention, and allow anticipation of supportive therapy with blood transfusions and nutritional supplementation.

#225  Two unexpected causes of eosinophilia

J Schaub*

S Elkins

University of Mississippi Medical Center, Clinton, MS

Introduction

Eosinophilia is a common finding in general medicine. Therefore, it is crucial to be aware of the multiple etiologies which can range from allergy/asthma, connective tissue disease, chronic infection/inflammation, medications, and uncommon hematological disorders such as myeloproliferative disorders. Here we present a patient case with chronic eosinophilia who surprisingly had two causative factors.

Case

A 43-year-old African American male with chronic eosinophilia was referred to hematology clinic for our evaluation after seeing a local oncology practice for eosinophilia that was suspected to be allergy related. In addition to an extensive history and physical exam, IgE, BCR/ABL, ANA, ANCA were collected for the workup and were normal/negative. Strongyloides IgG antibody was obtained and came back positive. Referral to Infectious disease was made regarding the concern for Strongyloidiasis and he was started on empiric treatment with Ivermectin. A CBC was repeated 3 months later revealed no improvement in eosinophilia. During this time, a previously collected CHIC2 (4Q12) deletion returned abnormal. This resulted in the diagnosis of Myeloid/Lymphoid neoplasm with PDGFR-A mutation. He then started on low dose Imatinib at 100 mg once daily with subsequent normalization of his complete blood count with differential one month later.

Discussion

Strongyloidiasis, involving the helminth Strongyloides stercoralis, is endemic in rural areas of southeastern United States. Acute infection may have urticaria or irritation at the site of skin penetration, whereas the chronic stage of strongyloidiasis is frequently asymptomatic. Treatment for patients with uncomplicated strongyloidiasis is with the anti-parasitic ivermectin. PDGFRA-related neoplasms are due to mutations on the long arm of chromosome 4 (4q12) leading to the fusion of FIP1L1 and PDGFRA. The most common presenting signs and symptoms are weakness, fatigue, cardiopulmonary symptoms, myalgias, rash, and fever, and with eventual progression to endomyocardial fibrosis with restrictive cardiomyopathy. Diagnosis is made by FISH for the CHIC2 probe and treatment utilizes low dose imatinib targeting PDGFR.

Conclusion

This case highlights the importance of maintaining a broad differential when undertaking an eosinophilia evaluation in a patient, whether from the general medicine or subspecialist standpoint. There are many causes for eosinophilia, two of which were discovered in this patient case as strongyloides infection and the hematological disorder of Myeloid/Lymphoid neoplasm with PDGFR-A mutation. Although both are uncommonly found, it is crucial to consider these two etiologies in the general workup of eosinophilia.

#226  A case of warm autoimmune hemolytic anemia

N Sherwani*

B Roubique

VR Jaber

T DiMasi

LS Engel

S Walvekar

LSU Health New Orleans, New Orleans, LA

Introduction

Warm autoimmune hemolytic anemia (AIHA) is a rare clinical disease which usually arises during or after concomitant clinical pathologies. Autoantibodies are formed against the red blood cell membrane, destroying them and causing extravascular hemolysis.

Case

A 68-year-old woman with medical history of anemia requiring transfusions, CAD s/p stents in 2007 and 2021, type 2 diabetes mellitus, hypertension, and COVID-19 infection nine months ago presented with chest pain and shortness of breath on exertion for two months. She described the pain as central, non-radiating chest tightness associated with dyspnea on exertion, which resolved with a few minutes of rest. She originally attributed this chest pain to her recent cardiac stent. Three weeks prior , She was treated for anemia (hemoglobin 5.4 gm/dL) with four units of packed red blood cells. Her hemoglobin increased to 7.9 gm/dL after transfusion with temporary improvement of her symptoms until this presentation. Her admit vitals were BP 154/65, HR 99, RR 20, O2 99% on room air, T 97.9°F. Physical exam was notable for generalized jaundice and scleral icterus. Laboratory results included hemoglobin of 6.5 gm/dL, MCV 106 fL, reticulocyte count 17.3%, peripheral blood smear with polychromatophils, total bilirubin 6.5 mg/dL, lactate dehydrogenase 321 U/L, and haptoglobin <30 mg/dL. Her EKG and troponin were normal. She was found to have hepatosplenomegaly on abdominal ultrasound. Further workup showed a direct antiglobulin test was positive with anti-IgG and complement C3 antibodies. This result confirmed the diagnosis of warm autoimmune hemolytic anemia. She received one unit of packed red blood cells with a subsequent hemoglobin of 6.1 gm/dL. She was then started on rituximab and prednisone with an increase in her hemoglobin to 6.9 gm/dL prior to discharge. The patient was discharged on high dose prednisone, scheduled for further rituximab infusions and given close follow-up with hematology and PCP. Atovaquone was added for pneumocystis jirovecii pneumonia prophylaxis during rituximab and prednisone treatment.

Discussion

Warm autoimmune hemolytic anemia is the most common type of AIHA, and its prevalence is approximately 170 per million. It can present with symptoms of chest pain, shortness of breath, and dyspnea on exertion which may at first seem to be cardiac in nature. However, further investigation with laboratory workup can reveal underlying hematologic abnormalities which can present similarly with more severe cases of AIHA. Approximately 50–60 percent of warm AIHA are associated with underlying conditions including EBV, HIV, HCV, lymphoproliferative disorders, and immunodeficiency states. It is important to consider AIHA in anemic patients with immunocompromised conditions. Cases have also been reported of new onset AIHA in association with COVID-19 infection, although there is no evidence yet of AIHA occurring several months after resolving COVID-19 infection.

#227  Compartment syndrome: an unsuspected consequence of hypercoagulable state in a patient with severe COVID-19 illness

S Siddiqui1*

K Creed1

N Randhawa2

S Shahbandar1

V Test1

1Texas Tech University Health Sciences Center, Lubbock, TX

2Franciscan Saint James Health Olympia Fields Campus, Olympia Fields, IL

Introduction

Coronavirus 19 (COVID-19) is a viral illness that is caused by SARS-CoV-2. It has a surface spike protein that binds to human angiotensin-converting enzyme 2 receptors expressed in the kidneys, lung, and vascular endothelium. Here we present a case of a 73-year-old critically ill male with COVID pneumonia and acute respiratory distress syndrome (ARDS), who developed compartment syndrome and rhabdomyolysis as a consequence of extensive right lower extremity arterial thrombosis related to a COVID induced hypercoagulable state.

Case

A 73-year-old COVID positive male with past medical history of coronary artery disease status-post triple coronary artery bypass 10 years ago and type 2 diabetes mellitus presented to the emergency department with progressively worsening dyspnea for one week. His initial oxygen saturation on room air measured 85%, so he was placed on 3 liters per minute supplementation via nasal cannula. CXR showed bilateral diffuse alveolar infiltrates and he was admitted for observation. He developed worsening respiratory failure five days into hospitalization, placed on maximum supplementation via high flow nasal cannula (HFNC), and transferred to the medical ICU. Ultimately, he was intubated and mechanically ventilated for the remainder of his hospitalization due to severe ARDS. After three days in the ICU, his right lower extremity was cold, without palpable nor detectable pulses via bedside Doppler from the femoral to pedal landmarks. Formal ultrasound Doppler that morning confirmed arterial clot extending from the right external iliac to posterior tibial arteries. The patient received embolectomy, stenting, and therapeutic heparin. Within 24 hours, though his creatinine kinase was normal, he developed significantly elevated myoglobin, lactate and worsening acidosis. The patient had a fasciotomy to the right lower extremity at bedside. The next day, he was anuric, with severe acidosis, hyperkalemia, and hypotension, requiring continuous renal replacement therapy (CRRT) and vasopressor support.

Discussion

Compartment syndrome is characterized by increased pressure within fascial compartments, leading to circulatory compromise, cellular necrosis, and rhabdomyolysis. In this case, the COVID-19 viral effect on coagulation led to extensive arterial thrombosis, complicated by compartment syndrome and renal failure necessitating CRRT. While the exact pathophysiology of the hypercoagulable state in COVID-19 illness is debated, we have observed its manifestations ranging from deep venous thrombosis (DVT), pulmonary embolism (PE), to stroke.

Conclusion

COVID-19 is known to be a virulent, multifactorial, intelligent virus with myriad end-organ and vascular consequences. When attending to the most critically ill patients with COVID-19, it is wise to consider all forms of vascular thromboembolism.

#228  Transplant associated-thrombotic microangiopathy (TA-TMA) and posterior reversible encephalopathy syndrome (PRES) in a patient with sickle cell disease post bone marrow transplant

CM Sullivan*

Z LeBlanc

LSU Health New Orleans, New Orleans, LA

Case Report

A 15-year-old male with history of severe sickle cell disease, five months post 10/10 HLA matched sibling donor bone marrow transplant, complicated by graft versus host disease (GVHD) of the skin and GI tract, hypertension, and steroid induced diabetes mellitus, presented in status epilepticus. The patient had presented one month previous with a pericardial effusion, proteinuria, and Coombs positive hemolytic anemia. He was admitted (transplant day +125) with severe headache and hypertension concerning for transplant associated thrombotic microangiopathy (TA-TMA) with supratherapeutic tacrolimus levels. Initial TMA work up was negative with normal terminal complement complex activity (sC5b- 9). He was treated with Rituximab and IVIG with resolution of his pericardial effusion and Coombs+ hemolytic anemia and was discharged. Subsequent sC5b-9 activity was elevated in conjunction with hypertension requiring >1 antihypertensive agent, thrombocytopenia, microangiopathic (Coombs negative) hemolytic anemia, and schistocytes on blood smear, consistent with diagnosis of TMA. Treatment with eculizumab, a monoclonal antibody binding to terminal compliment complex C5, was initiated. Following his 3rd eculizumab treatment he presented with hypertensive emergency in status epilepticus and was confirmed to have PRES based on MRI. Multiple anti-epileptic medications (AEDs) were required to achieve cessation of seizure activity including propofol infusion for burst suppression. Patient slowly improved with continued eculizumab infusions and strict anti-hypertensive management as evidenced by compliment blockade and cessation of epileptic activity on EEG. Following a prolonged PICU stay he was transitioned first to the BMT inpatient service and subsequently required inpatient rehabilitation for several weeks. Currently, 6 months following these events, his neurologic status has returned to baseline, has no evidence of GVHD, and no longer requires eculizumab infusions for TMA.

Discussion

TA-TMA with multi-organ dysfunction is a life threatening potential complication of bone marrow transplant caused by compliment over-activation. Multiple triggers for TMA have been identified including calcineurin inhibitors, GVHD, and viral infections. Prospective studies have demonstrated importance of monitoring for compliment over activation in high-risk patients to allow early diagnosis and treatment in improving patient outcomes. Treatment of TMA requires brief but intensive courses of eculizumab.

Conclusion

This case outlines the importance of early identification and treatment of TMA in patient outcomes and the delicate balance of controlling compliment activation and maintaining effective prophylaxis and treatment of GVHD.

#229  COVID-19 pfizer-biontech vaccine induced VTE

Y Tawfeeq1*

OH Al-Jobory1

A Hallak2

M Saadaldin1

1Texas Tech University Health Sciences Center, Amarillo, TX

2Ochsner Health, New Orleans, LA

Case Report

Between December 2019 and May 2021, there were around 200 million cases of COVID-19, with more than 3.5 million deaths all over the world. In the United States alone, there were more than thirty million cases, with around six hundred thousand deaths attributed to COVID-19. Incidents of hypercoagulability after receiving different types of COVID-19 vaccine have been reported. The incidence of deep vein thrombosis (DVT) is about 1 in 1000, and about 50% of these patients with DVT develop pulmonary embolism (PE). The incidence of DVT affecting the upper extremity exceedingly rare with an approximate incidence of 1 in 10,000. While patients receiving anticoagulation are still at risk of DVT, data on apixaban reflects a 98% protection from recurrent thrombosis.

Hypercoagulability including DVT and PE is always a rising concern in patients with COVID-19 pneumonia. We are reporting a case of a hypercoagulability state in 73-year-old lady after receiving first and second dose of Pfizer vaccine; despite being on apixaban. she has a past medical history of COPD on 2 L home oxygen. She presented with acute hypoxic respiratory failure few days after receiving the first dose of COVID-19 Pfizer vaccine. Imaging revealed right interlobar pulmonary embolism and right superficial femoral vein thrombosis without any provoking factors. She improved clinically and was discharged on apixaban. Few months later she came in with right upper extremity DVT, 7 days after receiving a second dose of Pfizer vaccine. Transesophageal Echo revealed a round mass in the left atrial appendage, which was likely a thrombus, she was discharged on warfarin.

Incidence venous thromboembolism is about 1 in 1000 individuals in the United States. Several factors can increase the hypercoagulable state. SARS-COV-2 is hypothesized to increase the risk of thromboembolism by infecting cell expressing surface receptors of ACE-2 by binding the SAR-COV-2 spike protein and activating cell pyroproptosis which activates neighboring cells inflammatory response and then activate coagulation pathway. BioNTECH mRNA vaccine induces immune response by engulfing S protein mRNA into the cell to produce spike protein and induce antibody production against SARS-COV-2 spike protein. At this time, this is the Third reported Vaccine related VTE after reporting a Pfizer BioNTech vaccine induced DVT on January 2021 After ruling out other causes of VTE in this case as well as the time between receiving the vaccine and the onset of symptoms, vaccine-induced thrombosis is the most likely cause for our patient’s thrombosis, including venous thrombosis, pulmonary embolism, and left atrial appendage thrombosis. The mechanism remains unknown but may possibly be due to enhanced immune response to the vaccine.

In patients at increased risk of thrombosis, BioNTech mRNA vaccination may induce Intravascular Coagulation, venous thromboembolism, possibly due to enhanced immune response to spike protein production.

#230  An unorthodox case of may thurner syndrome: a treatment dilemma

E Thekkedath*

R Sharma

J Farah

P Reddy

University of Florida Health at Jacksonville, Jacksonville, FL

Introduction

May Thurner Syndrome, also known as iliac vein compression syndrome, is a rarely diagnosed condition that is classically described as compression of the left common iliac vein by the overlying right common iliac artery resulting in subsequent deep venous thrombosis (DVT) of the ileofemoral veins. It most commonly affects women between the ages of 25–50 years old with a reported prevalence of approximately 20%. Here we present an atypical presentation of May Thurner syndrome due to extrinsic malignant mass compression with formation of a large left lower extremity DVT.

Case presentation

A 67 year old male with a large sigmoid colon adenocarcinoma presented to the ED with complaints of syncope and left lower extremity swelling and pain of several days duration. He was admitted to the intensive care unit for hypovolemic shock in the setting of acute gastrointestinal bleeding and was stabilized after transfusion of blood products. Computed tomography (CT) scan of the chest, abdomen, and pelvis with contrast demonstrated a pulmonary embolism and a large 6 cm mass in the sigmoid colon with compression of the left common iliac vein (Image). Doppler ultrasound of the left lower extremity confirmed the presence of a large acute occlusive deep venous thrombosis extending from the left common femoral vein to the left popliteal vein. Subsequent endoscopy and colonoscopy showed mucosal infiltration of the mass with chronic bleeding. Interventional radiology was consulted for the possibility of stent placement in the left common iliac vein, but due to the patient‘s inability to tolerate anticoagulation in the setting of chronic bleeding he was not deemed a good candidate. After discussing the severity and extent of the patient‘s cancer burden and prognosis he was agreeable to hospice care and discharged.

Abstract #230 Figure 1

Left: Coronal cut demonstrating clot; Right: Axial cut demonstrating tumor compression

Discussion

May Thurner Syndrome is an underdiagnosed vascular disorder resulting from compression of the left common iliac vein. Our case presents an atypical presentation of this pathology with a difficult treatment dilemma. The current literature suggests that systemic anticoagulation alone is insufficient for management. The propensity for clots is generated by venous stasis distal to the anatomic compression. Stent placement in the area of compression is warranted with subsequent anticoagulation for at least 6 months to minimize risk of stent thrombosis. Unfortunately in this case, the patient was at a very high bleed risk secondary to his malignancy, and could not tolerate anticoagulation for any length of time. He was also not deemed a surgical candidate due to the significant tumor burden, metastases, and infiltration of surrounding structures.

#231  Bone pain, bleeding, organomegaly – the case of the wrinkled tissue paper

C Tilly1,2*

JM Mack1,2

1University of Arkansas for Medical Sciences, Little Rock, AR

2Arkansas Children s Hospital, Little Rock, AR

Case Report

We describe a case of an 8-year-old patient with bone pain, easy bruising, fatigue, splenomegaly concerning for malignancy – leukemia, lymphoma, solid tumor with metastatic spread – but found to have Gaucher disease.

An 8-year-old female with a history of right lower extremity fracture presented with a one-year history of intermittent right and left lower extremity pain and swelling associated with antalgic gait, fever, loss of appetite, easy bruising, fatigue, and frequent nosebleeds. Physical exam was notable for periorbital hyperpigmentation, a 2/6 systolic murmur best heard at the left lower sternal border, splenomegaly about 4 cm below the costal margin, and swelling with tenderness noted at the medial aspect of the distal left thigh and knee.

A complete blood cell count was consistent with the following: WBC 6.89 K/uL, hemoglobin level 9.0 g/dL, hematocrit 28.9%, platelet 60 K/uL, MCV 75.9 fL, MCHC 31.1 g/dL, red blood cell distribution width 17.5%, reticulocyte count 1.3%. Erythrocyte sedimentation rate >105.0 MM/HR, c-reactive protein 103.0 mg/L, LDH 185 U/L, and uric acid 5.4 mg/dL. Basic metabolic panel, calcium, and phosphorus were within normal limits. Prothrombin time 11.9 seconds (9.8–13.3 seconds), partial thromboplastin time 34.8 seconds (23.0–29.7 seconds), D-Dimer 1.10 mg/L (0.17–0.59 mg/L), and fibrinogen 567.3 mg/dL (150–400 mg/dL).

MRI of the lower extremities was diffusely abnormal with heterogenous marrow replacement concerning for an infiltrative process. CT of the chest, abdomen, and pelvis revealed marked hepatosplenomegaly. Bone marrow aspirate was normocellular with increased histocytes showing abundant, pale blue-gray cytoplasm with wrinkled paper-like fibrillar pattern. Flow cytometry was negative for an abnormal population of blasts. Genetics and orthopedic surgery were consulted. Angiotensin converting enzyme was elevated at 161.9 IU/L; chitotriosidase was elevated at 23073.3 nmol/hr/ml; and tartrate resistant acid phosphatase was elevated at 57.64 IU/L. Glucosylsphinosine was elevated at 243 ng/mL, and beta-glucosidase was decreased at 0.176 nml/mL blood/hr. Laboratory results in the setting of hepatosplenomegaly, pancytopenia, and bone involvement are consistent with Gaucher disease.

The patient received Cerezyme 60 units/kg every 2 weeks for Gaucher disease. She was followed by Hematology-Oncology due to pancytopenia with the need for blood transfusions during hospitalization. Due to significant bone pain, the patient was prescribed acetaminophen, naproxen, and oxycodone with close follow-up in chronic pain clinic.

Bone pain, pancytopenia, and hepatosplenomegaly are consistently associated with malignancy; however, it is important to include genetic abnormalities, like Gaucher disease, in the differential diagnosis. There are three distinct types of this rare inherited genetic disorder. Because the most common form can occur at any age, prompt diagnosis is crucial to prevent long term effects of Gaucher disease.

#232  Questioning the use of absolute neutrophil count to diagnose spontaneous bacterial peritonitis in patients with malignant ascites

P Zito

S Mahato

A Chapple

D Tran*

W Beversdorf

E Rinker

A Garcia

LSU Health New Orleans, New Orleans, LA

Purpose of Study

Spontaneous Bacterial Peritonitis (SBP) is defined as an infection in the ascitic fluid (AF) without evidence of an intra-abdominal infection. It occurs almost exclusively in patients with cirrhosis and it is seen in up to 30% of hospitalized patients. It is reported to be rare in patients with malignant ascites (MA) occurring in less than 5% of patients. The gold standard to establish the diagnosis of SBP is a positive AF bacterial culture. However, studies in patients with ascites secondary to cirrhosis and portal hypertension demonstrated that an absolute neutrophil count (ANC) ≥250 cells/mm3 highly correlated with positive cultures and became the diagnostic test used in clinical practice. There is very limited data evaluating the ANC count in patients with MA. We hypothesized that ANC alone many not be enough to diagnose SBP in patients with MA. The purpose of this study is to describe the characteristics of AF in patients with MA, with particular emphasis on ANC and compare them with the AF in patients with other conditions (cirrhosis, SBP, liver metastasis).

Methods Used

We identified all patients with cytology proven malignant ascites treated at University Medical Center New Orleans New Orleans between an 8-year period as well as patients with ascites due to other conditions. Patients were identified through tumor registry, pathology databases, and medical and surgical oncology clinics. Fisher exact tests were used to compare categorical variables across ascites groups and Wilcoxon rank-sum tests were used to compare continuous categorical variables. Linear regression was used to adjust for potential confounders in assessing the relationship between type of ascites and the logarithm of ascites ANC.

Summary of Results

We identified 50 patients that met inclusion criteria (35 with malignant and 15 with benign ascites and no SBP). The primary outcome of interest was whether ascites ANC differed between ascites type. We found that the ascites ANC of malignant ascites was significantly higher than benign patients (average 2187.46 vs 132.11, p-value=0.001). 34% of patients with MA had ANC > 250. Multivariable linear regression model predicting log of ascites ANC value shows that the only significant predictor of ascites ANC is ascites type (Estimate = -2.17, 95% CI = -3.69,-0.65, p-value <0.001) indicating that the log of ascites ANC value decreases if a patient has benign ascites compared to malignant ascites. MA also had higher albumin, LDH and RBCs.

Conclusions

Our study confirms that malignant ascites is characterized by a high ANC compared to ascites in cirrhosis. Using ANC alone may not be enough to diagnose SBP in patients with malignant ascites. Limitations to this study include small sample size and retrospective analysis. Our patients with MA and high ANC could indeed have SBP. If so, a very large proportion of our patients with MA had SBP, which contradicts the literature. In future analysis, we will compare the characteristics of MA with that of SBP.

#233  Sinonasal melanoma – a dramatic diagnostic turn of events

J Kim

H khazrik

P Vahhabaghai*

S Singal

K Chakraborty

East Tennessee State University, Johnson City, TN

Case Report

Sinonasal melanomas are a rare entity in comparison to cutaneous melanomas. Here we share a case with this diagnosis.

An 82-year old male presented with persistent tooth pain, sinus headache, and right nasal drainage. Evaluation by otorhinolaryngology detected a right sinus mass. Biopsies were inconclusive and then sent to an outside tertiary center. He developed severe pain, apraxia/ophthalmoplegia, fixed pupillary dilation, and vision loss of the right eye. Magnetic resonance imaging of the face and sinuses showed a large mass eroding through the middle and superior turbinates, extending past midline, into the right orbit, maxillary sphenoid, and through the cribriform plate. There was dural enhancement, suggesting perineural spread without overt brain parenchyma invasion. The tumor was deemed unresectable. Positron emission tomography scan noted a station IIB left hypermetabolic cervical lymph node and a non-hypermetabolic right lower lobe lung nodule. The pathologic diagnosis was an exhaustive effort, requiring reviews of three pathologists. Ultimately, malignant melanoma with rhabdoid features was confirmed. Immunohistochemistry (IHC) revealed SOX10 focally positive, a marker seen in melanoma but negative for other melanoma markers such as MART1 and S100. BRAF mutation was negative. A tentative stage III versus Stage IV status was confirmed after multidisciplinary discussion. Palliative radiation to the right sinus and left neck was administered followed by palliative immunotherapy with pembrolizumab. The patient was able to complete one cycle before declining. Several weeks later, he succumbed to his disease.

As 90% of head and neck cancers are of squamous cell histology, this case shares a rare instance of sinonasal melanoma without classic IHC markers. Greater than 90% of melanomas are cutaneous, about 5% uveal in origin, and 1% are mucosal melanomas. Among the rare mucosal melanomas, head and neck (55%), anorectal (24%), and vulvovaginal (18%) are the commoner anatomic sites of origin than the urinary tract, small bowel, and gallbladder. For melanomas arising in the head and neck, 70% develop in the sinuses and nasal cavities. Forty percent of mucosal melanomas are amelanotic, whereas amelanosis is noted in less than 10% of cutaneous melanoma. Etiology, risk factors, and pathogenesis are largely unclear. These tumors can be difficult to diagnose and often IHC is vital. Unlike cutaneous melanoma, data on treatment specifically for sinonasal/mucosal melanoma is limited. Surgery and radiotherapy are used when feasible. Systemic therapy may include immunotherapy and targeted therapy as in cutaneous melanomas.

The prognosis of sinonasal melanoma is grim with a mean 5-year survival is estimated to be 0–30%. Early diagnosis is challenging but may assist curative approaches. Although a rare pathology, sinonasal melanoma can be a consideration in the differential diagnosis of persistent upper respiratory symptoms even if seemingly innocuous.

#234  Dermatomyositis, the great predictor

C Vaughn*

P Say

LS Engel

S Guillory

LSU Health New Orleans, New Orleans, LA

Case Report

A 59-year-old male with newly diagnosed dermatomyositis four months prior to admission presented with a complaint of left neck swelling that he noticed the morning prior to presentation. The swelling was non-tender and did not affect his swallowing or breathing. Other complaints at this time included skin rash and proximal muscle weakness that the patient stated were improving since he was started on prednisone by his dermatologist. His vital signs were normal, and his physical examination was significant for left sided supraclavicular lymphadenopathy that was non-tender, firm, and mobile. Skin examination was significant for heliotrope rash, gottron papules on both hands, and a rash on the extensor surfaces of both upper extremities. CT imaging of the neck revealed lymphadenopathy of the left supraclavicular and infraclavicular regions. Follow-up CT chest and CT abdomen/pelvis revealed a proximal gastric mass with extensive regional adenopathy. EGD demonstrated a large, fungating, ulcerated, and partially circumferential mass extending from the gastroesophageal junction to the posterior wall of the stomach. These findings were highly suspicious for malignancy. Biopsies of the mass were taken and the pathology is currently pending.

Discussion

Dermatomyositis is an idiopathic inflammatory myopathy that can develop as a paraneoplastic process. In patients with dermatomyositis there is a six-fold higher risk of malignancy compared with the general population. Therefore, following the diagnosis of dermatomyositis, patients, should at minimum, undergo age-appropriate cancer screening. Our patient with recently diagnosed dermatomyositis presented with new lymphadenopathy concerning for malignancy and further work-up revealed that the malignancy was likely gastric in origin. This patient had received age-appropriate cancer screening including colonoscopy yet went undiagnosed. The cancers linked to dermatomyositis may go undiagnosed by only following age-appropriate screening guidelines as these cancers may arise from sites not routinely screened. Therefore, blind screening may be appropriate for patients with newly diagnosed dermatomyositis, who do not have any suspicious clinical findings.

#235  Outcomes of central nervous system lymphoma treated with non-thiotepa based consolidation chemotherapy and autologous stem cell transplant

A Vegel1*

H Safah1

F Socola1

N Ziglar2

J Rink2

S Entwisle2

NS Saba1

1Tulane University School of Medicine, New Orleans, LA

2Tulane Medical Center, New Orleans, LA

Purpose of Study

To investigate and report outcomes for patients with either primary or secondary CNS lymphoma who received a non-thiotepa based consolidation chemotherapy, specifically Busulfan, Cyclophosphamide, and Etoposide (BuCyE), with autologous stem cell transplantation (ASCT). Although recent studies have suggested superior outcomes with thiotepa base regimens, there continues to be difficulty obtaining thiotepa for some patients due to recurrent national shortage, insurance coverage and cost within our patient population. In addition, the doses of BuCyE used at Tulane are higher (Bu:3.2 mg/kg on Day –8 through Day –5, E:30 mg/kg on Day –4, Cy:50 mg/kg on Day –3 and Day –2) than those reported in the literature when compared to thiotepa based regimens (Bu: 3.2 mg/kg on Day –7 to Day-5, E:200 mg/m2 once daily on Day –5 and Day –4 OR 200 mg/m2 twice daily on Day –5 and Day –4, Cy:50 mg/kg on Day –3 and Day –2). With this in mind, we set out to identify and report the outcomes of patients treated within our facility with BuCyE consolidation therapy and ASCT for CNS lymphoma.

Methods Used

We conducted a retrospective case series in which we identified patients who were treated for CNS lymphoma with BuCyE consolidation chemotherapy and ASCT from the year 2005 through 2021 at Tulane University. We obtained clinical information via retrospective chart review.

Summary of Results

We identified a total of three patients, 1 patient with primary CNS lymphoma and 2 patients with secondary CNS lymphoma who were treated with BuCyE consolidation and ASCT within our facility since 2005. To date, all patients remain alive, with follow-up times as follows: 7 years for the patient with primary CNS lymphoma; 7 years for the patient with a secondary CNS lymphoma who achieved complete remission and was discharged from our institution to his local oncologist for routine follow up; 3 years for the second patient with secondary CNS lymphoma who continues to follow up while maintaining clinical remission. In contrast with the literature, our improved outcomes could be linked to the higher doses of BuCyE used which could result in improved CNS penetration and anti-lymphoma effect.

Conclusions

Although a small cohort, our three patient case series suggests that the BuCyE consolidation regimen that our facility protocol follows may extend survival times as compared to initially reported (Median OS reported: 4.9 years). We believe that our extended survival times in these three patients may be due to increased dosing as compared to prior literature. With these findings, we suggest that future investigations, possibly with higher dosing of Busulfan and Etoposide, may further clarify the optimal regimen as compared to thiotepa based regimens for patients with CNS lymphoma.

#236  To err is human: a case of poems syndrome

C Wynn*

T Hilal

University of Mississippi, Jackson, MS

Introduction

POEMS syndrome (polyneuropathy, organomegaly, endocrinopathy, monoclonal protein, and skin changes) is a rare plasma cell neoplasm that is often difficult to diagnose. Here we present a case of POEMS syndrome with significant morbidity due to delayed diagnosis.

Case Description

A 53 year-old male presented to his primary care physician with new-onset peripheral edema and numbness and tingling in his extremities. Chest x-ray and echocardiogram were within normal limits, and he was prescribed increasing doses of furosemide. Neuropathic symptoms were initially treated with gabapentin. Several months later he began having shoulder pain. MRI showed focal areas in the right proximal humerus concerning for multiple myeloma. Serum protein electrophoresis showed a small IgA lambda and IgG kappa biclonal gammopathy at 0.5 g/dL. Kappa and lambda light chains were both mildly elevated (kappa 3.1 mg/dL; lambda 2.9 mg/dL). Free light chain ratio was normal at 1.1. On laboratory evaluation he was noted to have thrombocytosis but no anemia, renal insufficiency, or hypercalcemia. CT scans of the chest, abdomen, and pelvis showed multiple sclerotic bone lesions in the axial and appendicular skeleton. PET scan also showed widespread axial and appendicular osseous lesions. Two bone biopsies were nondiagnostic.

During this time his neuropathy progressively worsened to the point where he was having difficulty walking, and he developed bilateral contractures and clawing of his hands. He was unable to work due to his symptoms. MRI spine showed only his known sclerotic bone lesions without evidence of cord compression. Electromyogram showed ongoing very severe sensory motor large fiber primarily axonal neuropathy with demyelinating features. Bone marrow biopsy showed 5–10% polyclonal plasma cells with only a very small kappa (0.1%) and lambda (<0.1%) biclonal population by flow cytometry. Vascular endothelial growth factor (VEGF) level was elevated at 200 pg/mL (upper limit of normal, 96.2 pg/mL). He was diagnosed with POEMS syndrome and is currently undergoing treatment with lenalidomide and dexamethasone.

Discussion

This case illustrates the significant morbidity patients with POEMS syndrome can suffer due to delayed diagnosis. The presence of a monoclonal (M) protein and peripheral neuropathy are required for diagnosis, but the magnitude of the M-protein is usually small (around 1 g/dL). The minor features of the disease are easily overlooked, such as peripheral edema and thrombocytosis as in our patient. Peripheral motor neuropathy typically dominates the clinical picture, and it is not uncommon for patients to be wheelchair-bound at diagnosis. Almost all patients will have osteosclerotic bone lesions. Treatment with lenalidomide-based regimens is first-line and symptoms will improve. Younger, fit patients may be considered for autologous stem cell transplant. Improved knowledge and awareness of this syndrome will aid in faster diagnosis.

#237  Don’t look at my belly, look at my heart – pericardial effusion from an occult malignancy

S Yohannan*

S Alhariri

A Deoker

Texas Tech University Health Sciences Center El Paso, El Paso, TX

Introduction

Malignant pericardial effusion is a serious manifestation of some advanced malignancy. Of this, lung and breast cancer along with leukemia and lymphoma are the most common etiologies of malignant pericardial effusion. Even though most patients have a known malignant tumor before any evidence of pericardial involvement, sometimes malignant pericardial effusion can be the initial presentation. Therefore, malignancy must be excluded in every case of an acute pericardial disease, especially with cardiac tamponade at presentation.

Case Presentation

A 52-year-old male with a history of hyperthyroidism, diabetes mellitus, and hypertension presented with progressively worsening shortness of breath of 2 days duration and fatigue for the past 4 months. He quit smoking 20 years ago. While initial vitals seemed normal, pulsus paradoxus of 10–12 mmHg was noted on physical examination. This was checked due to distant heart sounds, jugular venous distention, and low voltage QRS on EKG. He also had reduced right lower lung breath sounds. His initial labs showed neutrophilic leukocytosis, otherwise no anemia, thrombocytopenia, electrolyte, kidney or liver abnormalities. Chest X Ray revealed moderate loculated right sided pleural effusion and cardiomegaly. Echocardiogram demonstrated a large circumferential pericardial effusion with evidence of cardiac tamponade and he underwent urgent pericardiocentesis, with removal of 1.5L of red, turbid pericardial fluid. Cytological analysis of the pericardial fluid showed metastatic adenocarcinoma of an unknown primary source. CEA, PSA, LDH were within normal limits. CT Abdomen was significant for chronic cholecystitis otherwise no masses. CTA chest was unrevealing of visible masses but rather multiple, enlarged, bilateral mediastinal and hilar lymphadenopathy. Colonoscopy and endoscopy with biopsies were negative for malignancy. In search of the primary malignancy, endobronchial ultrasound-guided biopsy was performed and the cells obtained stained positive for cytokeratin 7, TTF1, NapsinA, and BerEP4, consistent with metastatic adenocarcinoma from primary lung cancer. Similarly, a diagnostic thoracentesis was performed and cytology was consistent with malignant pleural effusion from metastatic adenocarcinoma. All throughout, oncology was consulted and the decision was made to start the patient on palliative chemotherapy.

Discussion

This patient with no known malignancy presented with clinical and echocardiographic signs of tamponade. Pericardial cytology was positive for adenocarcinoma from an unknown source. Interestingly, CT scans were unrevealing of masses but rather hilar lymphadenopathy, and it was only through the endobronchial biopsy that we were able to identify the primary source as lung cancer. Hence the etiology of pericardial effusions should always be investigated as malignancy can be a devastating cause of pericardial effusion.

#238  Vitamin b12 deficiency can be severe enough to mimic ttp

H Yousuf*

OH Al-Jobory

MA Tanbir

Y Al-Hilli

M Khan

F Al-Jubory

S Wright

Texas Tech University Health Sciences Center School of Medicine, Amarillo, TX

Case Report

Microangiopathic hemolytic anemia (MAHA) is one of the presentations of TTP. If not diagnosed early, TTP can have severe complications including ischemic colitis, progression to end-organ failure, and death. However few other conditions can also present as TTP, we report a case of a patient who presented with macrocytic anemia, thrombocytopenia, high lactate dehydrogenase, and bilirubin, schistocytes on peripheral blood smear mimicking TTP but negative ADAMTS13 and low vitamin B12 levels.

64-year-old male patient, with a history of untreated chronic vitamin B12 deficiency, hypertension, hypothyroidism, presented to the ER with shortness of breath, lightheadedness progressively worsening from the past 3 weeks. On initial presentation, the patient was alert and oriented, afebrile with stable vitals. The physical exam was unremarkable except for mild scleral icterus. Lab work was significant for severe anemia with hemoglobin of 5.8 g/dL, MCV:136.7 fL, platelet: 42L K/cumm. The patient received two units of blood on presentation. Further lab studies were significant for WBC of 4.4L K/cumm, HCT 17.0 L%, lactate dehydrogenase level: 2049 IU/L, haptoglobin <8L mg/dL, total bilirubin: 2.5 mg/dL, vitamin B12 level: 67 pg/mL (180 – 914), serum folate level: 11.62 ng/mL (5.2 – >20), reticulocyte count: 3.64H% (0.7 – 2.9). Chemistry profile, direct antiglobulin test, fecal occult blood test, ANA comprehensive panel, hepatitis profile was negative and coagulation profile, thyroid profile, methylmalonic acid levels were all within normal limits. Initially at presentation ADAMTS13 activity was pending, the patient received steroid treatment along with urgent plasmapheresis. Repeat smear on the third day of admission showed 2–3+ schistocytes. Eventually, ADAMTS13 activity came back within normal limits. Intrinsic factor antibody came positive, further strengthening vitamin B12 deficiency diagnosis. Clinical improvement was seen when the patient was provided with intramuscular vitamin B12 therapy with 1000 mcg daily for 1 week followed by 1000 mcg weekly for four weeks. Repeat vitamin B12 levels after 1 month of initial therapy were normal including his MCV and platelet count. The patient was advised to take his vitamin B12 monthly injections and was closely followed up by a hematologist.

Vitamin B12 deficiency is not commonly seen with hemolytic anemia, upon literature review few cases have been reported where vitamin B12 deficiency can mimic hemolytic anemia but thrombotic microangiopathy with the presence of schistocytes on the peripheral smear is rarely seen. With this case, we highlight an unusual presentation of severe vitamin B12 deficiency. If suspicion for TTP is high, prompt and accurate diagnosis of TTP is necessary in order to start emergent plasmapheresis, but at the same time, physicians should also recognize that severe vitamin B-12 deficiency can also present similarly, in which case treatment would simply be vitamin B12 supplementation.

#239  Transient carcinoembryonic antigen elevation in response to chemotherapy for stage II colon cancer

X Zheng1*

A Garcia1,2

1LSU Health New Orleans, New Orleans, LA

2University Medical Center New Orleans, New Orleans, LA

Purpose of Study

Transient carcinoembryonic antigen elevation in response to chemotherapy for stage II colon cancer

Methods Used

Review of Electronic Health Records and literature review

Summary of Results

A 54-year-old female with a history of normocytic normochromic anemia presented with a one-inch colonic mass in the hepatic flexure. The patient underwent a biopsy of the mass lesion, revealing invasive, moderately-differentiated MSI-stable adenocarcinoma. Preoperative Carcinoembryonic Antigen (CEA) was 24. CT scans showed no distant metastases. A right hemicolectomy was performed showing a moderately differentiated adenocarcinoma invasive through muscularis propria into pericolorectal soft tissue. All 15 lymph nodes were negative for metastatic cancer. Post-operative CEA was 3.9. She was started on adjuvant chemotherapy with XELOX (capecitabine plus oxaliplatin). CEA increased progressively during adjuvant chemotherapy to a peak of 9.3 at the end of treatment; however, CT scans of the chest, abdomen, and pelvis and PET/CT at the end of chemotherapy found no evidence of metastatic disease. 2 months after chemotherapy completion, CEA levels decreased to normal. Two years after surgery, the patient remains alive and well, with normal CEA levels and no evidence of recurrence.

Rising CEA following treatment in colorectal cancer is typically considered an indicator of tumor progression. CEA biomarker testing is utilized during a chemotherapy course to assess the effectiveness of treatment; however, chemotherapy may induce transient increases in CEA despite providing clinical benefits. An initial rise in CEA after chemotherapy initiation should not be presumed to indicate tumor progression or ineffective treatment. Repeat tests and imaging should be done to distinguish transient chemotherapy-induced CEA elevation from tumor progression. CEA can transiently increase in up to 30% of patients receiving adjuvant chemotherapy.

Conclusion

This case highlights an important caveat in the use of CEA biomarker testing as an indicator of chemotherapy response and colorectal tumor progression. CEA elevation after initiation of chemotherapy may represent a transient surge versus indicator of tumor progression, requiring additional consideration of clinical context and testing to differentiate.

Infectious diseases, HIV, and AIDS

Joint plenary poster session and reception

4:30 PM

Thursday, February 10, 2022
#240  Triple-valve infective endocarditis mssa (+) in a young IVDU

HR Cintrón-Colón*

C Rivera-Franceschini

WD Marrero

Hospital Municipio de San Juan, San Juan, Puerto Rico

Case Report

Infective Endocarditis (IE) is one of the major causes of increased mortality among intravenous drug users (IVDU). It typically involves one valve, with Staph Aureus being the major pathogen affecting the tricuspid-valve among IVDU. Involvement of more than one valve its very atypical. Only 14 cases of triple-valve endocarditis have been reported on the literature, and Methicillin-Sensitive Staphylococcus Aureus was found to be the main pathogen in only 2 of them. We present a very rare case of MSSA-Triple-Valve endocarditis in a young IVDU male. To our knowledge, this is the second case reported among IVDU.

Case of a 30 y/o male IVDU with no previous medical history that came to the emergency department complaining of extreme fatigue of 1 month of evolution. He referred a 1-week history of unquantified fevers, chills, night sweats, tachycardia, palpitations, headaches, myalgias, arthralgias, chest pain, fatigue, generalized weakness, shortness of breath and productive cough. However, one day before arriving to hospital, symptoms worsened and made him look for medical aid. Physical exam was remarkable for hypoactivity, high-fever, tachycardia, hypotension, dry oral mucous, bilateral diffuse crackles and ronchies, 3/6 holosystolic murmur at both sides of sternum, bilateral lower extremity pitting edema, presence of multiple needle track marks, multiple lower extremity purulent ulcers surrounded by erythema and edema, and lower extremity weakness (3/5). Labs were suggestive of an acute infectious process, myocardial demand ischemia, and moderate dehydration. Chest CT was remarkable for cardiomegaly, presence of multiple septic embolies, and small pleural effusions on both lung fields. EKG showed sinus tachycardia and left ventricular hypertrophy. Transthoracic Echo demonstrated evidence of mild anterior pericardial effusion, and presence of large vegetations at aortic, tricuspid, and mitral valves. Blood Cultures came back positive for Methicillin-Sensitive Staphylococcus Aureus (MSSA) in three bottles. Organism was treated with nafcillin IV. Later on, case was complicated by the development of bilateral spontaneous hydropneumothoraxes and patient succumb before valve replacement was achieved.

Even though extremely rare, MSSA-Triple-valve endocarditis possess a high-mortality rate if not promptly treated. Therefore, despite its sensitivity, MSSA-endocarditis should be treated aggressively from the start to avoid development of further complications.

#241  Rickettsial meningitis

M Darweesh1*

F Ghanem1

F Al-Ghzawi2

R Elmezayen1

1East Tennessee State University James H Quillen College of Medicine, Johnson City, TN

2The Hashemite University Faculty of Medicine, Zarqa, Jordan

Case Report

Rickettsial infections are caused by Rickettsia species of obligately intracellular gram-negative bacteria found in ticks and can cause Rocky Mountain spotted fever (RMSF). RMSF is the most common rickettsial infection in the United States. The incidence rate of RMSF ranges by state. Most cases of RMSF occur in the spring and early summer when outdoor activity is most frequent.

A 73-year-old male, with a history of rheumatoid arthritis and on weekly Methotrexate, presented with complaints of fever for 5 days associated with a severe headache. His temperature was up to 101.2F. He denied any rash, cough, SOB, nausea, vomiting, diarrhea, abdominal pain or urinary symptoms. Physical examination was unremarkable.

Initial blood work was significant for WBC count of 17 k/mcL, Hgb of 13.5 g/dL and platelet count of 140 k/mcL. Chest X-ray was negative for focal pneumonia. His MRI of the brain was negative for paraspinal abscesses and other intracranial abnormalities. He underwent lumbar puncture and the CSF analysis revealed WBC of 1376, RBC 0.0, PMN 94%, Protein 217 and Glucose 23. Cryptococcus Ag, gram stain, cultures, meningitis panel by PCR, mycology culture and AFP culture were all nonrevealing. RMSF IgM was positive. The patient received a course of intravenous ceftriaxone and doxycycline for two weeks with complete recovery.

The typical presentation of Rickettsial infection is fever, headache and skin rash. However, the absence of skin rash should not exclude the diagnosis. Definitive diagnosis relies mainly on serology that can be limited in the initial phase, emphasizing the need for high clinical suspicion. Rickettsial infection may present as meningitis and should be included in the differential diagnosis.

#242  Nocardiosis with dissemination to the skin and the central nervous system in a patient with end stage renal disease and diabetes mellitus

Z Elharabi*

G Del Rio-Pertuz

M Abohelwa

E Elgwairi

K Parmar

D Pawar

A Abdalla

K Nugent

Texas Tech University Health Sciences Center, Lubbock, TX

Case Report

Nocardiosis is a disease caused by a weakly acid-fact gram positive bacillus that has branching morphology. The bacteria can be found in the environment in soil. It is usually an opportunistic infection. However, in immunocompromised hosts it can cause disseminated disease. We report a case of nocardiosis caused by Nocardia brasiliensis with dissemination to the central nervous system (CNS) and skin.

An 80-year-old man presented to the ER with shortness of breath, fever, chills, diarrhea, bilateral leg swelling and bilateral leg lesions of three days duration. His wife also noticed that his mental status was altered. A physical exam revealed multiple bilateral erythematous nodules in his lower extremities. Some of the nodules had central pustules, and others had central eschars. His blood pressure was 107/44, and he was afebrile. His oxygen saturation was low, and he was started on oxygen via nasal cannula. The rest of the physical examination was unremarkable. His medical history was significant for end-stage renal disease, diabetes mellitus, and heart failure. Social history was significant for gardening. The patient was allergic to multiple antibiotics. Laboratory tests showed mild leukocytosis with neutrophilia, hyponatremia, elevated BUN, elevated creatinine, hypoalbuminemia and elevated proBNP. The skin lesions were initially treated as cellulitis with vancomycin and aztreonam. However, when pus from the skin lesions was cultured, it grew N. brasiliensis. Subsequently, vancomycin and aztreonam were discontinued and linezolid was started. Linezolid was later changed to trimethoprim-sulfamethoxazole. A computerized tomography scan of the chest showed moderate bilateral pleural effusions, left upper lobe opacities, and mediastinal lymphadenopathy. Magnetic resonance imaging of the brain showed pus within the lateral ventricles as well as within the posterior aspect of the posterior fossa going with ventriculitis. His mental status improved with antibiotic therapy, but it did not return to his baseline. His skin lesions formed central eschars when healing. Due to the multiple comorbidities and his functional status, the hospice route was chosen, and the patient was discharged to home hospice care.

Nocardiosis is disease that can mimic other diseases. It can be an acute, subacute, or chronic infection. It can also be limited to the lungs, skin, or can disseminate in immunocompromised hosts. Nocardia brasiliensis is the most common cause. The patient had two chronic diseases, diabetes mellitus and end-stage renal disease, that could have decreased his immunity. Bilateral involvement of his lower extremities makes a cutaneous infection as the source of entry unlikely. It is speculated that the patient had pulmonary involvement with dissemination to his skin and central nervous system (CNS). His altered mental status is likely due to the CNS involvement. Due to the variable clinical picture, nocardiosis is difficult to diagnose unless suspected.

#243  Catastrophic covid-19 in a pregnant woman

J Ekane*

R Chakra

S Tran

A Chao

Augusta University Medical College of Georgia, Augusta, GA

Case Report

We report here a case of a 26-year-old woman at week 29 of a pregnancy who was transferred to our hospital for OBGYN care after on the same day she presented to an outside facility for one day of vaginal bleeding. At the transferring facility she was noted to be hypertensive with concerns for pre-eclampsia, acute kidney injury and non-reactive nonstress test. She had not had any pre-natal care or screenings done. Upon arrival she was normotensive but became obtunded and emergently taken to the OR. She was found to have a 50% placental abruption with uterine atony, hemorrhaging, and unfortunately fetal demise. She tested positive for SARS-CoV-2 on screening though she had no initial respiratory symptoms. Following extubation, she was noted to have very labored breathing, continued disorientation, and repeatedly stated that she was blind. She was subsequently re-intubated both to protect her airway and due to her work of breathing. Chest imaging showed bilateral patchy opacifications of her lungs and she was initiated on treatments for COVID19 pneumonia. She was lymphopenic at this time with an absolute lymphocyte count of 800 cells/mm. She had not been vaccinated against SARS-CoV-2.

Over her hospitalization, she underwent extensive workup. For her complaints of vision loss she underwent ophthalmologic exam which did not find uveitis or other changes consistent with syphilis but rather for ischemic central retinal vein occlusions. She had persistent hypoxic respiratory failure and ultimately necessitated tracheostomy due to prolonged dependence of mechanical ventilation support. Approximately 1 month after her hospitalization, she developed a new left lower lung opacification as well as scattered tree-in-bud nodular findings on chest CT imaging. On bacterial and culture workup she grew methicillin-susceptible Staphylococcus aureus as well as Aspergillus species (identification still pending). She was treated with a short course of cefazolin for bacterial pneumonia and was started on a 3-month course of isavuconazonium sulfate for probable COVID-19-associated pulmonary aspergillosis (CAPA). After a two-month long hospitalization, she had gradual clinical improvement and was transferred to a skilled nursing facility for long-term care.

In this case, the devastating impact of COVID-19 disease in a young, unvaccinated, and pregnant woman is clearly seen, as are multiple sequelae. She unfortunately lost her pregnancy and developed severe visual impairments and several opportunistic respiratory infections. Her placental abruption, ischemic retinal vein occlusions and pulmonary aspergillosis were all felt to be directly attributable to her COVID19 disease. The case presented here serves as a cautionary tale that even the young are at risk for severe COVID-19 disease. Healthcare professionals should continue to advocate for screening and vaccination for these high-risk individuals.

#244  A deceptive case of tb

F Kandah*

S Nuthulaganti

N Chowdhury

J Verdecia

University of Florida Health Science Center Jacksonville, Jacksonville, FL

Case Report

We present a case of a newly diagnosed HIV patient presenting with pulmonary symptoms and an impressive opacity on chest x-ray with acid fast bacilli (AFB) sample negative on two separate bronchoalveolar lavage (BAL) samples and eventual positive mycobacterium PCR confirming pulmonary tuberculosis.

A 60 year old male with a history of polysubstance abuse presents to the emergency room with shortness of breath. In ED, the patient was found to be lethargic and hypoxic with oxygen saturations of 84% on room air. Patient was hemodynamically stable, febrile, tachycardic, and tachypneic. Chest x ray was significant for large focal consolidation in the right upper lobe. Computed tomography (CT) chest was done to rule out pulmonary embolism (PE), which was negative for PE but showed enlarged mediastinal lymphadenopathy as well as the large opacity in the right lobe of the lung. Given risk factors, a HIV test and quantiferon were ordered and both returned positive. CD4 count was found to be 190. A BAL was done to rule out active tuberculosis, which was nondiagnostic with negative cultures, pneumocystis pneumonia (PCP) smear and AFB stain. With the CT chest revealing lymphadenopathy, there was suspicion for malignancy. It was decided to perform an EBUS with lymph node biopsy. Repeat studies were again significant for negative AFB stain. However, mycobacterium PCR was also done, which returned positive, confirming active tuberculosis. The patient was started on RIPE therapy and discharged in stable condition.

The detection of pulmonary tuberculosis (TB) can be challenging at times. Imaging, clinical signs and symptoms, and the patient‘s background can provide clues that point towards TB, but the official diagnosis relies on several various methods. AFB sputum staining is usually quickly done and if positive, can effectively diagnose and guide therapy for patients. However, it can often be unreliable in detecting TB, with about half being smear negative, especially in the HIV population. In these patients, basing the diagnosis of smear negative TB off of clinical symptoms and judgements is even more challenging as these patients have a wider differential including opportunistic bacterial, fungal and viral pneumonias. In our case, MTB PCR was able to accurately detect pulmonary TB, while other methods failed. In immunocompromised patients, conventional techniques in diagnosing TB may not be reliable and more thorough approaches are warranted.

Abstract #244 Figure 1

Chest x-ray (left) demonstrating large right upper lobe opacity; CT Chest (right) with impressive right lung opacity

#245  Aids-related kaposi sarcoma in well-controlled HIV

M Lieux*

D Beyer

B Van Court

C Van Dreumel

R Foret

SM Ford

LS Engel

S Sanne

LSU Health New Orleans, New Orleans, LA

Introduction

Kaposi Sarcoma is an angio-proliferative malignancy characterized by spindle-cell morphology commonly associated with human herpes virus-8. In the era of anti-retroviral therapy, the incidence of Kaposi Sarcoma in HIV-infected patients has diminished significantly. We present a case of Kaposi sarcoma in a patient with well-controlled HIV.

Case

A 43-year-old man with past medical history of HIV on anti-retroviral therapy (last CD4 count 1024 and undetectable viral load several months prior to presentation) and rheumatoid arthritis on hydroxychloroquine presented with severe right knee pain with recurrent swelling. He reported fever, chills, right groin swelling and inability to move or bear weight on his right knee. Initial vital signs were significant for tachycardia and low-grade temperature. Physical exam was significant for limited passive range of motion of the right knee, which was warm, erythematous, tender to palpation and associated with right-sided inguinal lymphadenopathy. No cutaneous lesions were present. Arthrocentesis demonstrated septic arthritis, and broad-spectrum antibiotics were initiated. Right inguinal lymph node biopsy revealed spindle-cell neoplasm expressing HHV8 and CD34, consistent with Kaposi Sarcoma. After discharge, he was started on liposomal doxorubicin for treatment of Kaposi Sarcoma.

Discussion

Kaposi Sarcoma, an AIDS-defining illness, is a spindle-cell malignancy commonly associated with HHV-8 and severe immunodeficiency. Although this patient’s HIV was well controlled with anti-retroviral therapy, he subsequently developed Kaposi sarcoma without cutaneous involvement. Kaposi Sarcoma is staged based on tumor extent, immune status and systemic illness severity. Treatment modalities include continued antiretroviral therapy, intralesional or systemic chemotherapy and radiation.

#246  Hepatitis c treatment with sofosbuvir/velpatasvir inducing uveitis in multiple sclerosis

N Mittal*

M Abohelwa

Z Elharabi

S Shahbandar

G Del Rio-Pertuz

A Elkheshen

K Nugent

Texas Tech University System, Lubbock, TX

Introduction

Sofosbuvir/velpatasvir, known as Epclusa in markets, is one of the new treatments for hepatitis C with 90% efficacy on genotype 1. Sofosbuvir acts by inhibiting a nucleotide analog that inhibits HCV NS5B polymerase as a chain terminator when incorporated into HCV RNA; velpatasvir works by blocking the NS5A protein.

Case presentation

We present a 39-year-old woman with a history of untreated hepatitis C and multiple sclerosis. The patient was started on sofosbuvir/velpatasvir for her active hepatitis C genotype 1 infection. Three months later, she arrived at the ED with a reported loss of vision in her left eye. Ophthalmology team evaluation indicated left eye uveitis with increased intraocular pressure, and she was prescribed cyclo-phenolate eyedrops.

Discussion

This case showcases a unique association of uveitis with sofosbuvir/velpatasvir in the setting of ongoing multiple sclerosis. Although multiple sclerosis by itself can induce uveitis, there are reports about uveitis occurrence after sofosbuvir/velpatasvir. What supports our assumption is that our patient developed the eye symptoms after starting the sofosbuvir/velpatasvir; however, another theory could propose immuno-stimulation from the hepatitis C treatment can lead to a relapse of the inflammatory reaction leading to uveitis which is also strongly supportive in literature.

Conclusion

Infectious disease specialists or gastroenterologists should be vigilant about the ophthalmic side effects of sofosbuvir/velpatasvir with urgent referral to ophthalmology. In our case, reactivation of multiple sclerosis might be a possibility as well.

#247  Acute bacterial meningitis secondary to streptococcus salivarius infection with no clear iatrogenic cause

B Quraishi*

EM Alhussain

T Vo

M Anees

Texas Tech University Health Sciences Center, Amarillo, TX

Case Report

Streptococcus salivarus is a native member of the human oral cavity and is not a typical pathogen associated with infection. In fact, it is used as a probiotic to suppress colonization and infection with more pathogenic Streptococcus species. However, it is increasingly being reported as a cause of life-threatening central nervous system (CNS) infections. A great majority of cases are iatrogenic in origin, subsequent to procedures such as spinal anesthesia and neurological surgery. Other causes include trauma-induced cerebral spinal fluid (CSF) leak and even translocation from the gastrointestinal tract. Prognosis is favorable with prompt antibiotic treatment, usually a beta-lactam antibiotic. We present a case of acute bacterial meningitis secondary to S. salivarius infection. This is a relatively rare presentation of infection with no clear iatrogenic cause.

Case

A 82-year-old female with a past medical history of hypertension, osteoporosis, and gastroesophageal reflux disease (GERD) presented to the emergency department (ED) of an outside facility intubated and sedated with midazolam after she was reported to emergency medical services (EMS) for altered mental status and combative behavior. She was given intranasal and humeral intraosseous doses of midazolam for her agitation, however, due to respiratory depression she required intubation in the field. She had been experiencing a headache for 2 days, a cough for 2 weeks and altered mental status for 4 hours leading up to her initial presentation. In the ED, the patient was mechanically ventilated on minimal settings and hemodynamically stable. Initial labs showed elevated white blood cell (WBC) count of 18 × 103/uL. CT scan of the head was negative for any acute processes. Lumbar puncture was performed and findings included an opening pressure of 21 cm H2O (10–20 cm H2O), purulent fluid, WBC count of 7.3 x 103/uL (0–5/uL) with 95% polynuclear cells, glucose of 2 mg/dL (normal range: >60% serum glucose), and protein of 721 mg/dL (<45 g/dL). Gram-stain of CSF fluid showed many WBCs and no organisms. Consistent with acute bacterial meningitis, the patient received vancomycin, piperacillin-tazobactam, and ampicillin, and was subsequently transferred for admission to the ICU of our facility.

At our initial evaluation, the patient was intubated and sedated. On the second day of admission. 36 hours after CSF was cultured at the outside facility, strep salivarius was grown which was sensitive to ceftriaxone. The patient continued to show improvement in mentation and ultimately received a 10-day course of ceftriaxone and piperacillin-tazobactam before being discharged with mentation at baseline, a normal WBC count, afebrile, and no other clinical or laboratory signs of infection.

Conclusion

S. salivarius meningitis usually associated with iatrogenic or traumatic events, but in rare cases it can be spontaneous

#248  Weil’s disease: a severe presentation of a variable illness

D Román-Colón*

CA Cortes

VA Caribbean Healthcare System Mental Health Services, San Juan, Puerto Rico

Case Report

Leptospirosis is an acute zoonotic infection with a worldwide distribution that often is under-reported. It usually affects young males due to their working or recreational activities and during the rainy season. Humans usually are incidentally infected after exposure to contaminated water, soil or animal urine. The clinical course of this disease is variable as it involves a broad spectrum of clinical manifestations. These may range from a mild influenza-like illness to a severe, potentially fatal form of multiorgan involvement called Weil’s disease that can include high fever, significant jaundice, renal failure, hepatic necrosis, pulmonary involvement, cardiovascular collapse, neurologic changes or hemorrhagic diathesis. We present a case of an elderly farmer with a severe presentation.

A 72-year-old male with medical history of arterial hypertension that presented due to a three-day onset of fever, malaise and lower extremity pain with associated chills, headache, anorexia and diarrhea. He denied chest pain, palpitations, shortness of breath or cough. Upon further questioning he worked as a farmer and had exposure to livestock and rodents. Vital sign remarkable for fever, tachycardia and hypotension despite IV fluid rehydration. Physical examination with dry oral mucosa, conjunctival suffusion, jaundice and bibasilar crackles. Laboratories with stable hemoglobin and no leukocytosis, but acute severe thrombocytopenia and bandemia. Chemistry with stage 3 non-oliguric acute kidney injury along with borderline-low sodium and potassium levels. A marked elevation of creatine kinase, lactic acid, bilirubin with direct predominance, and alkaline phosphatase were noted. Patient was started on Ceftriaxone 2 gm IV daily due to suspected leptospirosis and vasopressors. His clinical course was further complicated with de novo atrial fibrillation and a short course of renal replacement therapy. Leptospirosis PCR was later detected, and patient completed 7 days of Ceftriaxone with resolution of thrombocytopenia, bandemia, leukocytosis, hyperbilirubinemia, transaminitis and adequate spontaneous urine output. He was then transferred to PM&R inpatient rehabilitation and was discharged fully functional.

It is important to consider leptospirosis in the differential diagnosis of acute febrile illness. This case highlights the importance of a detailed history and physical examination with consideration of social aspects such as occupational exposures. Clinical diagnosis of the disease and serologic verification of infection are fundamental along with rapid initiation of antibiotics and other symptomatic therapy. It is important to be aware of the possible development of Jarisch Herxheimer reaction in these patients. Although our patient had a severe presentation of a highly variable disease, a high index of suspicion enabled rapid identification along with correct medical management and an interdisciplinary approach resulting in a complete and functional recovery.

#249  Purple urine bag syndrome due to enterococcus faecalis

B Roubique*

EC Bourgeois

G Sapora

L Gawey

C Pesson

M Morvant

LS Engel

R McCarron

LSU Health New Orleans, New Orleans, LA

Case Report

A 21-year-old man with recent history of gunshot wound to the abdomen complicated with numerous intraabdominal injuries and infections requiring percutaneous nephrostomy tube placement, presented to the hospital about 3 months later with a 3-day history of green nephrostomy drainage. He was complaining of fever, chills, headache, and non-bloody emesis. On hospital day two, his urine, urine bag, and nephrostomy tubing were dark purple. He was started on broad spectrum IV antibiotics for sepsis secondary to pyelonephritis and underwent nephrostomy tube exchange. Urinalysis from the purple urine bag contained urinary sediment with greater than 100 WBC/high-power-field and positivity for leukocyte esterase. Urine cultures yielded 50,000 CFU/mL of Stenotrophomonas maltophilia and greater than 100,000 CFU/mL Enterococcus faecalis. The patient was switched to a 14 day course of levofloxacin and amoxicillin post nephrostomy tube replacement. The patient’s urine was no longer purple in color.

Discussion

Purple urine bag syndrome (PUBS) is a rare manifestation of a urinary tract infection (UTI) that is typically seen in women and chronically debilitated patients with long-term indwelling urinary catheters. There have also been reported occurrences of PUBS in patients undergoing hemodialysis and patients with nephrostomy tubes. The purple color of the urine is due to breakdown products formed by bacterial enzymes in the urine. The common bacteria capable of producing these enzymes include Providencia spp, Klebsiella, and Proteus. However, many more bacterial species have been reported in association with PUBS, including Escherichia coli, Enterococcus spp, Morganella morganii, and Pseudomonas aeruginosa. PUBS resolves by treating the underlying UTI, as well as ensuring proper care and sanitary practices for upkeep of urinary catheters. While this condition is benign, it can be distressing for patients who are unaware of this phenomenon. Physicians should recognize that purple urine bag syndrome may signal underlying recurrent UTIs due to improper urinary catheter care.

#250  Saved from a surgeon’s blade: hypercalcemia due to fungal infection in a 60-year-old patient with history of hiv

MA Tanbir*

M Al-bayati

R Chandra

H Yousuf

Texas Tech University Health Sciences Center, Amarillo, TX

Background

Hypercalcemia is a common clinical problem, most commonly due to primary hyperparathyroidism and malignancy (~90% of reported cases). Less than 10% of cases are made up of granulomatous diseases such as sarcoidosis, fungal infections, or tuberculosis. Severe hypercalcemia is defined as >14 mg/dl and such patients commonly present with confusion, stupor or even coma. We present a case of recurrent hypercalcemia in an HIV patient caused by disseminated coccidioidomycosis that was initially suspected to be due to lymphoma.

Case presentation

Patient is a 60-year-old Caucasian male with a history of HIV and recurrent hypercalcemia who was referred to the emergency room after he was found to have severe hypercalcemia on routine lab work. Patient had symptoms of polyuria, polydipsia and constipation but was alert and oriented x3 on presentation. He was started on IV fluids and given a dose of zoledronic acid on admission. His calcium levels normalized over a period of 3 days. The primary differentials for his hypercalcemia were malignancy versus an infectious process. History suggesting a paraneoplastic process included noncompliance with ART for 5 years- increasing likelihood of lymphoma, especially considering his mediastinal lymphadenopathy, numerous splenic lesions and 45 lbs weight loss in 4 months. He was empirically treated for a fungal infection ~3 months prior and had a decrease in mediastinal lymphadenopathy, suggesting an underlying infectious etiology. Additionally, Fungitell value had decreased from >500 to 299 over this time. His largest lymph node (11 mm), however, was at a hard-to-reach region. CT surgery had recommended endobronchial ultrasound done by pulmonology, but oncology assessed that a fine-needle aspiration would not be a useful study to diagnose lymphoma. We obtained an IR guided splenic biopsy and fungal studies from the specimen was positive for coccidioidomycosis.

Discussion

Patient was referred for a splenectomy after his initial presentation as he was presumed to have lymphoma. Since obtaining a biopsy was challenging, a splenectomy was considered as it would serve both a diagnostic and a therapeutic purpose. Surgery should always be reserved as a final option and meticulous evaluation of reasonable differentials should also always be exhausted first. A splenic biopsy was initially considered too risky. Joint discussion with the oncologist and interventional radiologist, however, lead to the assessment that there were suitable lesions at an accessible spot, with an acceptable risk of bleeding. This collaborative management decision led to a positive outcome for this patient who was spared a life of living without a spleen and has since started appropriate treatment for coccidiomycosis.

#251  Atypical presentation of a neuroinvasive west nile fever

Y Tawfeeq*

L Bellamkonda

OH Al-Jobory

A Odimegwe

T Naguib

Texas Tech University Health Sciences Center, Amarillo, TX

Background

West Nile Virus is the most common cause of arboviral diseases and is endemic in the US. It can cause clinical presentation ranging from asymptomatic infection to neuroinvasive disease. Factors like old age, hematologic malignancies, and organ transplantation result in more severe disease.

Case presentation

An 85-year-old male farmer with coronary artery disease, congestive heart failure (CHF) and diabetes presented with sore throat, cough and shortness of breath. He was treated for CHF exacerbation and discharged but returned with fatigue, headache, Nausea, and vomiting. Imaging of the head and chest, inflammatory markers, COVID-19 PCR,and metabolic panel were all negative, . Then he became febrile, lethargic, and had altered mentation. He exhibited asymmetric weakness, bilateral positive Babinski and neck rigidity. Intravenous ceftriaxone, vancomycin, bactrim, and acyclovir were all initiated for meningoencephalitis of unclear etiology. CSF analysis showed 51 WBC, 0 RBC, 49% granulocytes, 55 glucose and 74 proteins. He was transferred to ICU with a glasgow coma score of 6–7 but was on a ‘DO NOT INTUBATE’ status. He developed acute anuric kidney injury which prompted emergent hemodialysis and vasopressor support had to be initiated. Family elected for palliative care, and he passed away shortly after. A day later, West Nile serology was reported positive as IGM in CSF. Also, both IgG and IgM were positive in serum

Discussion

This case demonstrates a rare, severe presentation of West Nile infection. Most persons infected with West Nile (WNV) virus are asymptomatic; symptoms are seen in only about 20 to 40 percent of infected patients [1]

Serologic surveys and extrapolations from blood donor screening data indicate that neuroinvasive disease following infection is infrequent, with estimates ranging from 1 in 140 to 1 in 256 infections resulting in meningitis or encephalitis[2]

The patient’s risk factors for severe infection include elderly age and male sex. Age in particular is the most important risk factor for neurologic progression of disease.[3]

The diagnosis can be suspected in patients who have altered mental status, signs of meningitis, unexplained fever, and focal neurologic deficit, especially when it presents in late summer months and has no obvious etiology. The CSF West Nile IgM antibody detection via ELISA is diagnostic as was in our case. The treatment is mainly supportive. The preventive measures including mosquito control, personal protective devices are of substantial importance. Vaccines are not available. [4 5 6]

Conclusion

The high index of suspicion is necessary to diagnose WNV neuroinvasive diseases especially in patients with atypical presentation. Seasonal clues must be considered.

#252  Soft tissue mucormycosis in an immunocompetent patient

S Tran*

P Coffey

Augusta University Medical College of Georgia, Augusta, GA

Case Report

A 28 year-old woman without significant medical history presented to the hospital as a level 1 trauma secondary to a motor vehicle accident in which she was ejected from the vehicle. She was serially taken to the operating room for exploratory laparotomies for management of multiple intraabdominal injuries including bladder rupture, liver laceration, and pelvic fracture. She also required multiple soft tissue debridements of the abdominal wall and right buttock and was also noted to have an extensive closed soft tissue degloving injury of the flank. The patient remained intermittently febrile up to 39 °C with progressive leukocytosis up to 21,300 cells/mm3. On subsequent debridement of the flank wound, visible fluffy, white growth consistent with mold was visualized. She was empirically started on micafungin but was transitioned to isavuconazonium sulfate in the presence of a rapidly progressive transaminitis and acute kidney injury. Despite aggressive debridement, visible mold growth continued to be seen with each successive debridement, and liposomal amphotericin was added despite the potential additive nephrotoxicity and hepatotoxicity. Further operative evaluation deemed additional surgical debridement to be futile. Multi-system organ failure progressed, the patient’s family made the decision to not pursue aggressive care, and the patient passed away soon thereafter due to asystolic cardiopulmonary arrest. After death, her tissue fungal cultures returned with growth of Mucor species and pathology staining demonstrated invasive fungal organisms.

Mucormycetes are commonly environmental organisms and aggressive soft tissue infections have been reported in the immunocompetent, especially with this patient’s severe soft tissue injury and environmental exposure due to being ejected from her vehicle. Aggressive surgical debridement is paramount in management of this disease. For antifungals, while amphotericin B is classically considered first-line therapy, there is mounting evidence stating that isavuconazonium sulfate is non-inferior. Other studies have also demonstrated some efficacy using various combination therapies. Unfortunately, mortality remains incredibly high with this disease process despite optimal therapy. As such, this case illustrates the need to maintain high clinical suspicion to facilitate early identification and aggressive medical and surgical management of invasive mold infections.

#253  West nile virus infection with resulting meningoencephalitis in a middle-aged female: a case study

V Vemulapalli*

A Falco

T Naguib

Texas Tech University Health Sciences Center School of Medicine, Amarillo, TX

Learning Objective

Recognize the signs and symptoms of West Nile Virus Meningoencephalitis.

Case presentation

A 42-year-old female with no known chronic medical illnesses presented with 5 days of fever, confusion, and generalized body aches and weakness. She became more confused and agitated in the 2 days before she presented to the ED and she complained of headache, nausea, and vomiting. Patient had been confused, wandering around, and had involuntary jerking movements affecting her upper and lower extremities. She denied chest pain, shortness of breath, cough, urinary symptoms, or diarrhea. Patient tested negative on a Covid-19 PCR test, returned to ED with confusion, and was started on empiric antibiotics with ceftriaxone and vancomycin, acyclovir for meningitis, and clonazepam and Keppra for myoclonus. Fever and leukocytosis indicated sepsis and sepsis protocol was initiated. Patient was intubated 3 days after admission to control her condition. Urinalysis was negative for significant infection. CT of head showed no acute findings, and CT of chest/abdomen/pelvis showed no evidence of pneumonia or inflammatory processes. Meningitis PCR screen came back negative. Lumbar puncture showed increased white blood cells in CSF of 65 with 43% neutrophils, 40% lymphocytes, and increased total protein. Gram stain and CSF culture were both negative. HSV PCR came back negative so acyclovir was discontinued. CSF analysis for West Nile Virus IgG was positive although the WNV IgG was negative in the blood. Subsequently both IgM samples of the CSF and blood resulted as positive. The CSF pleocytosis and elevated protein supported the diagnosis of recent West Nile Virus infection with resulting meningoencephalitis. Antibiotics were discontinued. Patient was extubated after 4 days and physical therapy, speech therapy, and tube feeding were started. The patient was discharged in stable condition with a plan for home physical therapy.

Discussion

Since the West Nile Virus was first reported in the U.S. in 1999, it has become the most common cause of meningoencephalitis in the U.S. WNV can have varied presentations, ranging from asymptomatic to severe neurologic deficits (flaccid paralysis, seizures, or altered mental status) due to encephalitis or meningitis. The mortality rate for neuroinvasive WNV is approximately 9%, with that rate increasing in elderly populations. In addition, the neurological illnesses caused by WNV often result in long-term sequelae, further highlighting the need for early diagnosis and management. While current management for WNV are primarily supportive, novel treatments such as interferon therapy, ribavirin, and intravenous immunoglobulin (IVIG) are being explored and show potential.

Conclusion

We conclude that although neuroinvasive WNV is rare, occurring in less than 1% of infected individuals, it should be suspected due to the severity of morbidity and mortality especially during mosquito seasons.

#254  Pulmonary infection by a novel nocardia species in a patient with advanced aids

T Yau*

S Kelleher

V Burke

Louisiana State University, New Orleans, LA

Case Report

The differential diagnosis of respiratory infection in pateints with human immunodeficiency virus (HIV) is noticeably broad. We present a case of necrotizing pneumonia secondary to pulmonary nocardiosis from a novel Nocardia species in a patient with advanced HIV.

A 36 year old male with untreated HIV (CD4 count of 14 and HIV viral load of 21,596) was admitted for several months of weight loss, night sweats, fatigue, and productive cough. On physical examination, he was significantly cachectic with a body mass index of 12. On pulmonary examination, the patient had crackles in the right upper lung field. He also had oral candidiasis and diffusely diminished strength. CT chest revealed a right upper lobe necrotizing cavitary lesion with adjacent tree in bud nodularity. MRI brain revealed diffuse cerebral and cerebellar atrophy, but no focal abnormality. Sputum cultures were collected. Patient was placed on airborne precautions and treated with empiric vancomycin, cefepime, and metronidazole. He underwent bronchoscopy for bronchioalveolar lavage (BAL) and tissue biopsy. On hospital day 9, sputum AFB cultures revealed gram positive branching filamentous bacteria, which was later identified as Nocardia niwae. BAL and lung tissue cultures also yielded the same bacterium. Empiric antibiotics were discontinued and directed therapy with IV imipenem and trimethoprim-sulfamethoxazole (TMP-SMX) was initiated. TMP-SMX was subsequently replaced with oral linezolid due to concern for acute drug-induced liver injury indicated on inpatient laboratory studies. The patient completed a 6-week course of IV imipenem and linezolid with clinical improvement and resolution of liver injury. He was transitioned to oral TMP-SMX and minocycline. Combination antiretroviral therapy (cART) was initiated as well.

Nocardia species are ubiquitous in the environment and cause pulmonary infection in those with chronic lung disease or opportunistic infection in immunocompromised host. Pulmonary nocardiosis often clinically resembles tuberculosis or atypical mycobacterial infection. Nocardia niwae, identified in our case, is a novel species associated with isolated pulmonary disease first described from taxonomic analysis of isolated human pulmonary sources in 2011. Clinicians should have high suspicion of nocardiosis in patients presenting with pulmonary infection in the setting of immunocompromise.

#255  Cryptococcal meningitis complicated by covid-19

G Zarkua*

J Ekane

R Chakra

A Chao

S Tran

Augusta University Medical College of Georgia, Augusta, GA

Case Report

A 45-year-old man with a history of end-stage renal disease s/p kidney transplant 14 months prior presented with severe headaches, neck pain, nausea, and vomiting for the past week. He takes tacrolimus, mycophenolate mofetil, and prednisone. Exam was notable for fever of 38.1°C, photophobia, and neck pain induced with forward flexion. Non-contrasted CT head found no intracranial processes. Lumbar puncture demonstrated an opening pressure of 45 cm H20 with CSF showing 108 WBCs with 67% neutrophils, normal glucose, and protein elevated to 112 mg/dL. Due to our high suspicion for cryptococcal meningitis, he was started on induction therapy with amphotericin B and flucytosine. CSF and serum cryptococcal antigens later returned positive at 1:320 and 1:2560, respectively. CSF culture also grew Cryptococcus neoformans/gattii complex. He underwent serial lumbar punctures and completed 14 days of induction therapy. He was transitioned to fluconazole consolidation after CSF cultures cleared and opening pressures on lumbar puncture had normalized.

After induction, he acutely developed a severe leukopenia to 100 cells/mm3 along with profuse diarrhea. Over the next 1–2 days, he had progressive cough and dyspnea followed by hypotension, tachycardia, and hypoxemia, at which point he was diagnosed with SARS-CoV-2. He had completed his SARS-CoV-2 vaccinations 4 months prior to hospitalization. He was started on broad spectrum antibiotics and dexamethasone, placed on high-flow oxygen, and transferred to the intensive care unit. He was diagnosed with Klebsiella pneumoniae bacteremia. He developed progressive multi-organ failure and suffered a cardiac arrest. After discussion with family, the patient was transitioned to comfort care and passed away.

Patients on immunosuppressive therapy are high risk for severe outcomes from both opportunistic infections and common infections that may affect the immunocompetent. It was critical to maintain a broad differential on this patient’s presentation, as while cryptococcal meningitis is classically a disease of advanced HIV/AIDS, it may also occur in patients with alternative causes of immunosuppression. These patients often have other features that complicate therapy, such as an inability to reduce immunosuppression to control the disease, or drug interactions between antifungals and their immunosuppressive medications.

This patient also suffered other complications from his chronic immunosuppression; a poor response to his initial SARS-CoV-2 vaccination and predisposition to more severe COVID-19 disease. Both leukopenia and diarrhea are common findings in COVID-19, which provoked the Klebsiella pneumoniae bacteremia.

This unfortunate case demonstrates the need to always remain vigilant for both opportunistic and routine infections in an immunocompromised patient, especially in the setting of an ongoing viral pandemic.

Inflammation

Joint plenary poster session and reception

4:30 PM

Thursday, February 10, 2022
#256  Partial response to tofacitinib in a patient with extensive calcinosis: navigating the challenges of calcinosis cutis

M Hassan*

S Alhariri

J Gutierrez

S Sairam

Texas Tech University Health Sciences Center El Paso, El Paso, TX

Introduction

Dystrophic calcinosis cutis is a skin condition associated with autoimmune connective tissue diseases, including dermatomyositis and systemic sclerosis [1]. It is a difficult to treat condition with recent interest in Rituximab and Tofacitinib as treatment options (seen in several case reports) [2,3]. We herein report our experience with Tofacitinib in a 48-year-old female diagnosed with dermatomyositis and who developed treatment-resistant, progressive, ulcerative calcinosis cutis with recurrent abscesses since 2007. The authors aim to highlight the challenges in management of such a case.

Case description

A 48-year-old Hispanic female was diagnosed with dermatomyositis in 2004 following an episode of proximal muscle weakness and diffuse skin rash. At that time, CK level was noted to be 16303 IU/L, aldolase 179.3 U/L and ANA titer 1:5120 in a homogenous pattern. Muscle biopsy and electromyography confirmed severe active dermatomyositis. Autoantibody workup including anti-dsDNA, anti-Scl-70, anti-SSA, anti-RNP, anti-Jo-1 were all negative. Patient was on varying doses of prednisone, with immunosuppressants methotrexate, cyclosporine and azathioprine at different time periods between 2004 and 2018. In 2007, imaging revealed soft tissue calcification in the proximal thigh, which was later confirmed to be dystrophic calcification of the dermis and subcutaneous fat via punch biopsy. In 2021, she presented with worsening calcinosis while off immunosuppressants for 3 years with normal CPK and stable clinical weakness. Several ulcerative lesions extruding calcium were present evoking pain upon lying flat or sitting. In view of significant morbidity, Tofacitinib was started in April 2021. Prednisone and colchicine were added 2 months later as no improvement was seen. While she now reports improvement in pain and muscle strength, the calcinotic discharge persists.

Discussion

A careful review of virtually accessible literature highlights the lack of effective treatment for calcinosis cutis. Data concerning the efficacy of suggested treatment modalities is significantly insubstantial with only positive response reported in case reports. Widespread calcinosis cutis with ulcers, as seen in our patient, deters surgical management. Besides mild pain alleviation, no significant reduction in calcinosis was observed in response to Tofacitinib 4 months after initiation. Ultimately, the authors aim to shed light on the challenges that accompany dystrophic calcinosis cutis including ambiguous pathogenesis, lack of a blueprint for disease management, infection and pain control, and other psychosocial factors adversely impacting quality of life.

#257  Inflammatory myopathy, a diagnostic dilemma

K Parmar*

N Eshak

Z Elharabi

K Nugent

Texas Tech University Health Sciences Center, Lubbock, TX

Introduction

Acquired immune and inflammatory myopathies (IIMs) are subdivided into dermatomyositis, polymyositis(PM), inclusion body myositis (IBM) and immune-mediated necrotizing myopathy (IMNM),

Case presentation

A 24-year-old female presented with sudden onset weakness of proximal muscles initially around hips and later involved shoulder and arms. It was associated with generalized soreness. On physical examination there was weakness in bilateral lower extremity(LE) with 2/5 strength and upper extremity with 4/5 strength. She had knee tenderness, limited range of motion of hip and tenderness with palpation of trapezius muscles bilaterally. She had erythematous maculopapular rash on face and chest inconsistent with rash described for dermatomyositis. Laboratory testing revealed highly elevated creatine kinase (CK) >1,000 IU/L, mildly elevated ALT, AST and aldolase. Serological testing revealed a positive ANA, rest of antibody testing were negative including , anti-dsDNA, myositis specific antibodies, EBV IgM, and anti MDA-5 ab. MRI of the LE showed diffuse myositis (figure 1). Electromyography suggested diffuse myopathy associated with denervating potentials, compatible with a necrotizing myopathy. Patient was started on prednisone for 3 days during which she had improvement in pain and weakness but was held for muscle biopsy. Post biopsy patient was started on methyl-prednisone 125 mg BID and mycophenolate mofetil 50 mg BID. Patient’s pain improved but there was no change in muscle strength. Muscle biopsy showed no inflammatory changes and single necrotic fiber which was considered minimal nonspecific finding. Patient was CK again began to rise with >7000 and 2 rituximab infusion were given. CK down trended and patient was discharged. On follow up in 1 month patient still had muscle tenderness and developed difficulty swallowing however motor strength had improved.

Discussion

This case presented with some criteria for polymyositis as defined by Bohan et al however the muscle biopsy was negative. PM has prevalence rates of approximately 1 per 100,000. Another differential diagnosis was IMNM, a type of autoimmune myopathy characterized by weakness, myofiber necrosis with minimal inflammatory infiltrate on biopsy. Treatment remains immunosuppressive therapy.

Neonatology case report

Joint plenary poster session and reception

4:30 PM

Thursday, February 10, 2022
#258  Ecthyma gangrenosum in an extremely preterm infant

J Elleston*

SR Komic

R Mosley

Augusta University Medical College of Georgia, Augusta, GA

Case Report

Albeit rare, Ecthyma gangrenosum (EG) is a recognized cutaneous manifestation known to be most associated with pseudomonal sepsis in the immunocompromised patient. This condition is noted to generally begin with the appearance of painless, erythematous macules that quickly progress to pustules or bullae and eventually develop into gangrenous ulcers with black-grey eschar. This case report presents a 6-day-old, 23-week premature neonate with numerous EG lesions in the setting of culture-confirmed Serratia marcescens bacteremia, her post-mortem exam additionally notable for disseminated hyalin mold infection most consistent with Aspergillosis. Our case is unique in that our patient’s EG lesions were found to derive from a less-commonly associated organism, especially for a young neonate.

#259  Occult bacteremia in neonate with staphylococcus aureus conjunctivitis

L Gagnon*

H Knight

R Mangat

LSU Health New Orleans, New Orleans, LA

Purpose of Study

Conjunctivitis in the neonatal period has several potential etiologies, of which many can result in serious morbidity or mortality. Most commonly, etiology is suspected based upon the temporality and severity of presenting symptoms. In the outpatient pediatric setting, the diagnosis of conjunctivitis is made clinically, and a self-limiting course is expected. Treatment is therefore equivocal and guided by clinical judgment, rather than discrete data. In this case, subtle findings in a neonate’s history and physical exam guided further workup resulting in the detection of occult bacteremia. This report serves to highlight the importance of a high clinical index of suspicion for systemic involvement in diagnoses evaluated in the outpatient setting.

Methods Used

Case Report

Summary of Results

A 13-day-old female was admitted for treatment and workup of possible gonococcal or HSV conjunctivitis after the presentation of sudden onset, rapidly progressive, bilateral, purulent conjunctivitis. Less than 24-hours prior, the patient had been healthy and without concerns at a well-baby checkup. That night, the mother reported development of left eye swelling and erythema which advanced to bilateral eye involvement with significant purulent drainage. At the time of presentation, subtle findings of new-onset poor feeding and listlessness were also detected, increasing suspicion for systemic disease involvement. Gonococcal conjunctivitis typically only presents within five days of life, but because prompt treatment is required to prevent permanent sequelae and potential blindness, rule out was pursued. Additionally, maternal risk factors were revealed as HSV infection during pregnancy, prolonged rupture of membranes, and possible chorioamnionitis. Herpes simplex virus is a less common etiology of conjunctivitis, but if systemic can be life-threatening. Upon admission, topical, oral, and intravenous antibiotic treatments were begun, and blood cultures were collected. Within 24-hours a near resolution of symptoms was achieved, and cultures revealed S. aureus conjunctivitis with concurrent S. aureus bacteremia. The neonate was managed inpatient for 10 days to complete the appropriate antibiotic course.

Conclusions

The decision to pursue additional workup and treat with intravenous antibiotics was based on the maternal history of HSV infection and the rapid onset nature of severe ocular symptom development with poor feeding and decreased activity on examination. The detection of S. aureus bacteremia in a healthy, term newborn without a prolonged newborn hospital stay is rare. This case illustrates that signs of bacteremia may be subtle in the neonatal period, emphasizing the importance of investigation regardless of risk factors. Furthermore, it reiterates the importance of correlating physical exam findings with pertinent history to reveal a systemic condition in conjunctivitis.

#260  Escherichia coli- infected cephalohematoma: a case report

A Gurung*

F Eyal

K Dolma

University of South Alabama Health System, Mobile, AL

Introduction

Cephalohematoma is one of the common birth injuries usually associated with instrumental delivery. Most cephalohematoma spontaneously resolves on its own over the course of a few weeks. However, a small percentage of the cases can be complicated by either infection or calcification. We report a case of Escherichia coli (E. coli) infected cephalohematoma in a 5-week-old infant that was managed with surgical intervention.

Case Report

A 37+1 weeks large for gestational age female was born to a 23-year-old diabetic mother via vaginal delivery. The mother received 3 doses of penicillin G prior to delivery for group b streptococcus (GBS). Delivery was complicated by shoulder dystocia, clavicular fracture, and left-sided parietal cephalohematoma. Apgar scores were 2 and 7 at 1 and 5 minutes. Cord gas revealed respiratory acidosis. The infant was admitted to the neonatal intensive care unit due to hypoglycemia and presumed sepsis. She was initially treated with IV ampicillin and gentamicin. The initial blood culture was negative. At 5 weeks of life, the infant was noted to have increased irritability with decreased oral feeding. Physical exam revealed an increased large, fluctuant, and non-erythematous swelling occupying the left parietal scalp. Laboratory evaluation revealed an elevated c-reactive protein of 2.3 and white cell count of 20380. IV vancomycin and gentamicin were empirically started. Blood, urine, and cerebrospinal fluid (CSF) cultures showed no growth. Magnetic resonance imaging of the brain revealed parietal cephalohematoma with no findings suggestive of intracranial extension or osteomyelitis. Neurosurgery was consulted due to the increasing size of cephalohematoma. Incision and drainage were performed, and a large amount of pus was drained. A Penrose drain was placed initially for continuous drainage which was later removed after 6 days. Wound culture grew pan sensitive E. Coli. IV cefepime was initiated and continued for 6 days. The infant improved clinically on antibiotics after the procedure and was discharged home on 4 more days of amoxicillin.

Conclusion

Although the management for cephalohematoma is observation and monitoring, practitioners should suspect infection in case of expanding cephalohematoma size from baseline. Early diagnosis and treatment with IV antibiotics and surgical drainage can avoid potential complications.

#261  Zosyn induced severe thrombocytopenia in an extreemly premature neonate

KG Holder1*

A Giakas2

EN Sargent1

B Gutierrez1

O Adesanya1

1Texas Tech University System, Amarillo, TX

2University of South Carolina School of Medicine, Columbia, SC

Learning Objective

Identifying Zosyn-induced thrombocytopenia in a newborn.

Case presentation

An AGA female neonate born at 23.1 weeks was admitted to the neonatal intensive care unit due to extreme prematurity, suspected sepsis, and respiratory failure. Complications at the time of delivery included preterm spontaneous, prolonged rupture of membranes for 18 hour and 15 minutes, Group B Strep (GBS), and maternal sepsis secondary to chorioamnionitis. CBC and blood culture were obtained for the neonate and prophylactic ampicillin and gentamicin were started at birth.

On day of life (DOL) 5, blood cultures came back negative, but the infant became febrile and exhibited signs of clinical deterioration. At this time, the patient was started on 10 days of Zosyn and prophylactic fluconazole with repeat cultures pending. On DOL 6, after 2 doses of Zosyn, platelets dropped to 34,000. The decision was made to begin a 6 ml platelet transfusion bringing the platelet count to 98,000. By DOL 8, platelet counts began to trend downward. On DOL 13, CBC was reassuring except for persistent thrombocytopenia of 17,000. Another platelet transfusion was given. Zosyn continued for the full 10 days in anticipation of final cultures as the patient continued exhibiting signs suggestive of sepsis. On DOL 14, CSF analysis was suggestive of meningitis, viral panel was positive for HSV 6, and culture showed a KOH positive, rare, budding yeast. Repeat blood cultures tested positive for Staphylococcus and Candida. Pediatric Infectious Disease was consulted and recommended vancomycin to treat Staph sepsis and Amphotericin B to treat fungemia with fungal meningitis. Her infection is now well controlled, but her platelets continue to drop when she receives Zosyn. Zosyn has been continued as no other equally efficacious antibiotics are available. Thrombocytopenia is being successfully managed with platelet transfusion as necessary.

Discussion

Drug-induced thrombocytopenia is a common culprit of blood dyscrasias, but it often goes unrecognized due to the difficulty in identifying the culprit drug. Thrombocytopenia is a known reaction to several antibiotics although specific reports of Zosyn-induced thrombocytopenia in neonates are rare. Our patient’s close monitoring in the NICU required frequent blood draws and allowed us to directly correlate her thrombocytopenia with Zosyn dosing. The patient’s critical status made Zosyn the preferred treatment, and platelet transfusion was used as needed to mitigate secondary thrombocytopenia.

Conclusion

Although Zosyn-induced thrombocytopenia has been reported in a wide spectrum of patients, the effects of Zosyn on cytopenia induction in premature infants has yet to be described. This case outlines the importance of recognizing Zosyn-induced thrombocytopenia and in a premature infant and provides a successful example of treating the thrombocytopenia with platelet transfusion when patients are critically ill.

#262  A case of fetal methotrexate syndrome--a rare cause of multiple congenital anomalies

P Joslyn*

A Barkemeyer

R Gupta

B Barkemeyer

LSU Health New Orleans, New Orleans, LA

Case Report

Methotrexate (MTX) is a folate antagonist which interferes with normal DNA synthesis. MTX is used in the treatment of neoplastic and autoimmune disorders, as well as for the termination of intrauterine and ectopic pregnancies. Given its known teratogenic effects, it is rare for a fetus to be exposed to MTX. In rare instances of fetal exposure, the constellation of congenital anomalies that arises has been termed fetal methotrexate syndrome (FMS). FMS has been reported to affect nearly all organ systems, with the most common abnormalities including facial dysmorphisms, limb anomalies, cardiac lesions, cranial anomalies, CNS anomalies, and fetal growth restriction.

Case presentation

Here, we report a case of a term female infant born with anomalies consistent with FMS following an unsuccessful medical abortion in the first trimester with the combination of MTX and misoprostol. The mother also consumed alcohol between the 10 and 20 weeks of gestation. At birth, the patient was noted to have several anomalies. Both feet displayed oligodactyly with only great and lateral toes present. The patient had short thumbs with anonychia and single transverse palmer creases. Facial anomalies included mild retrognathia with mandibular hypoplasia, low-set ears, and epicanthal folds. The patient was growth restricted with a birthweight < 2% for gestational age. Renal, spinal, and cranial ultrasounds as well as an echocardiogram were all within normal limits. A chromosomal microarray was normal. The patient initially had difficulty with PO feeds; however, feeding ability improved throughout admission. She was discharged home to her mother on day of life 6 having surpassed her birthweight.

The patient is followed closely by a multidisciplinary team of specialists. New diagnoses since discharge from the NICU include gross motor delay; infantile hemangioma; unilateral ptosis; and bilateral occipital mastoid craniosynostosis with mild ventriculomegaly. Orthopedic surgeries will be considered in the future once functional impairment is better understood.

Abstract #262 Figure 1

Oligodactyly with only great and lateral toes present

#263  Not every hip clunk is a dislocated hip: a case of a femur fracture in a newborn

S Mende*

M Meder

V Threadgill

A Safarulla

The University of Oklahoma College of Medicine, Oklahoma City, OK

Case Report

We present a case of a newborn infant with a left hip clunk noted on routine hip exam following delivery.

The patient was delivered to an 18-year-old mother, who presented to labor and delivery for a scheduled external cephalic version for breech presentation at 38 weeks gestation. During the external cephalic version, fetal heart tracing declined to 70–90 bpm which resulted in an emergency cesarean section. Physical exam following delivery revealed a significant hip clunk with passive movement of the left leg. With the associated risk of breech presentation at delivery, this was attributed to possible developmental dysplasia of the hip (DDH) and an ultrasound of the hip was ordered. However, repeat physical exam at 24 hours of life showed new onset swelling of the middle one third of the left thigh with increased discomfort with palpation and movement of the left leg. Subsequent X-rays of lower extremities revealed an oblique, acute appearing fracture extending to mid-left femoral diaphysis with displacement of fracture fragments. Orthopedic surgery fitted the patient with a Pavlik harness to stabilize the bone, with outpatient follow-up in one week. Given the severity and timing, an osteogenesis imperfecta (OI) panel and skeletal survey were performed. Skeletal survey did not reveal any other acute or healing fractures. OI panel is pending at this time. Hip ultrasound revealed possible left hip DDH, but this could be due to the femur fracture. Follow up radiograph 2 weeks later revealed healing fracture of the mid left femur with stable alignment of fracture fragments with developing osseous callus formation at fracture site.

Discussion/Conclusion

This case highlights the need for a meticulous physical exam for all newborn infants including an examination of the hips. In the setting of a difficult or complicated breech delivery, there needs to be a high index of suspicion and clinical awareness to properly identify if a clunk found on passive movement of the hip is consistent with DDH or may indicate a different injury. Femur fractures are rare compared to other birth-related fractures, but they have occurred with breech vaginal and caesarian deliveries.

In conclusion, it is important that a thorough physical exam and condition of the patient is considered, and perform further investigations if warranted. The swelling of thigh and visible discomfort of patient with exam, triggered us to obtain appropriate imaging and consultation in a timely manner.

#264  Finnish type congenital nephrotic syndrome with nphs1 gene mutation

A Nuttli*

J Gallois

J Farge

K Simms

M Knecht

LSUHSC-New Orleans, Metairie, LA

Case Report

Congenital Nephrotic Syndrome (CNS) is a rare disorder characterized by nephrotic-range proteinuria, hypoalbuminemia, and edema presenting in utero or in the first 3 months of life. Patients with CNS are at risk for complications such as hemodynamic instability, recurrent infections, thrombosis, impaired growth, and kidney failure. CNS can present with a wide clinical spectrum ranging from asymptomatic to critical illness requiring intensive treatment for severe proteinuria, anasarca, and hemodynamic compromise.

We present the case of a late preterm male infant diagnosed with Congenital Nephrotic Syndrome. He was born at 35 weeks and 3 days of gestation via vaginal delivery to a 29-year-old G2P0201 mother. There was prenatal suspicion of CNS due to placentomegaly, oligohydramnios, and a full sister with fatal complications of CNS. After birth, he was monitored in the NICU for prematurity and workup for CNS. On day of life 2, he was transferred to a Level IV NICU for multidisciplinary management including pediatric nephrology. Genetic testing confirmed the diagnosis of Finnish Type Congenital Nephrotic Syndrome with an NPHS1 gene mutation.

Following several days of an initially uneventful hospital course, he went on to develop several known complications of CNS. Many of these complications occur due to the increased urinary protein losses that result from the underlying defect in podocytes characteristic of CNS. Infants with CNS are at increased risk for thrombosis due to an imbalance of procoagulant and anticoagulant factors that result from urinary leakage of important hemostatic proteins, such as antithrombin and protein S. On day of life 4 he was diagnosed with an occlusive iliac thrombus. This was initially treated with a continuous heparin infusion, then transitioned to intermittent enoxaparin with resolution of the thrombus. By day of life 10 he had developed worsening edema and proteinuria requiring furosemide and albumin infusions. He developed hypertension requiring treatment with an ACE-inhibitor, which also helped to decrease his proteinuria. He required prolonged antibiotic treatment for multiple infections with methicillin-resistant staphylococcus aureus bacteremia, consistent with the increased risk of infections in CNS due to urinary losses of immunoglobulins. Cornerstones of his management also included optimization of fluid, calorie, and protein intake. At 3 months of life, he remains in the NICU for ongoing management of the above complications.

In terms of future management, many infants with CNS often undergo bilateral nephrectomy and begin dialysis around 6–12 months of life. They subsequently receive kidney transplant months later, once weighing 10kg. Regular life-long follow up is required to monitor for potential acute as well as chronic complications associated with CNS and to minimize the need for hospitalization and optimize the quality of life for these children and their families.

#265  The conundrum of phenibut withdrawal management in a neonate

F Peña*

R Huq

I Roca

M Dominguez- Garcia

S Manzar

LSU Health Shreveport, Shreveport, LA

Case Report

Phenibut is a neuropsychotropic, GABAB agonist used as a dietary supplement, recreational drug, to increase attention and concentration, as a muscle relaxant and to decrease anxiety in social situations. It is easily obtained online but it is not an FDA approved drug in the US. It causes addiction in the adult population but its effects have not been described in newborns. We present the case of an infant with neonatal abstinence syndrome due to maternal use of phenibut.

#266  Prolonged survival in a liveborn male prenatally diagnosed non-mosaic (complete) trisomy 22

D Pineda*

K Schmidbauer

J Holcomb

Augusta University, Augusta, GA

Case Report

Trisomy 22 is a chromosomal disorder rarely associated with the survival of live-born infants secondary to severe, congenital anomalies. Most affected children die before one year of age. Typical malformations include microcephaly, congenital heart disease, and renal malformations, along with other atypical features. This case presents a complete, non-mosaic trisomy 22 male with prolonged survival to day of life 112 (four months of age). Trisomy 22 was confirmed via peripheral blood cytogenic studies showing complete, non-mosaic additional chromosome 22 in all metaphases. The patient had multiple organ malformations consistent with previously documented cases of trisomy 22, with the addition of intrahepatic cholestasis, congenital hypothyroidism, absent gallbladder, and early onset seizures. To date, only 29 liveborn cases have been reported (Kehinde et al, 2014) and none have reported liver and gallbladder disease in association with Trisomy 22.

#267  Methotrexate and the ischemia hypothesis in mobius syndrome: a report of two cases

R Rawhani*

P Maertens

University of South Alabama, Mobile, AL

Case Report

Introduction

Möbius syndrome (MS) is a rare congenital dysembryologic disorder resulting in palsy of multiple cranial nerves (CN), most commonly CN VI and VII. The etiology of MS remains unclear, but ischemia or teratogenicity have been hypothesized to be the cause.

Purpose of Study

To describe the first known report of methotrexate use leading to MS, providing further support for the ischemia hypothesis as the proposed pathophysiology.

Case 1: Patient is an African-American female infant born at 26 weeks’ gestation via Caesarean section for non-reassuring fetal status. Pregnancy was complicated by preeclampsia and premature prolonged rupture of membranes. She was small for gestational age and was found to have poor respiratory effort requiring positive pressure ventilation before being transferred to our Neonatal Intensive Care Unit (NICU) for further care.

Case 2: Patient is a Caucasian female born at 37+6 weeks’ gestation via spontaneous vaginal delivery. Apgar scores were 2, 4, and 5 at 1, 5, and 10 minutes respectively. She was small for gestational age and was found to have poor respiratory effort in the setting of microstomia and clenched jaw requiring intubation prior to admission to NICU for further management.

Both mothers had ingested methotrexate early in the pregnancy in an attempt to abort the pregnancy. Both patients were noted to have multiple limb and craniofacial anomalies including palsy of CN VI and VII. Chromosomal Microarray Analyses were within normal limits. Cranial Ultrasound findings were consistent with calcifications at the floor of the 4th ventricle, ventriculomegaly, and pontine hypoplasia. A diagnosis of MS was made based on physical exam and neurosonographic findings.

Discussion

Ischemia leading to calcification in the region of the CN nuclei has been hypothesized to cause CN palsies. Abortifacient exposure during the embryonic phase of development may increase the risk of developing MS. Ischemic events leading to such outcomes have been previously described with misoprostol, but not with methotrexate. Methotrexate has known teratogenic effects, but its use has not been previously described to be associated with MS as is described in this report.

Conclusion

Abortifacient exposure may lead to further vascular vulnerability in the region of CN VI and VII during embryologic development leading to calcification and development of MS.

#268  Cranial ultrasound: a bed side diagnostic modality for edwards syndrome

S Shaik*

P Maertens

R Gulati

University of South Alabama, Mobile, AL

Case Report

Case

A newborn female was born at 37 weeks 6 days gestational age, at our children’s hospital with prenatal ultrasound findings of intrauterine growth restriction, two-vessel cord, and fetal echo suggestive of tetralogy of Fallot (TOF). No prenatal genetic testing was performed as parents denied. After birth, infant developed transient tachypnea of newborn without requiring supplemental oxygen. Physical exam was consistent with syndromic appearance with a flat nasal bridge, low set ears and clenched fists. Postnatal echocardiography on day of life 1 confirmed TOF with a large patent ductus arteriosus with no pulmonary stenosis. On the day of life 3, the infant developed multiple cyanotic episodes associated with a blank stare. Repeat echocardiography reported no evidence of pulmonary stenosis or shunt reversal. Given the negative echocardiogram, neonatal seizures were suspected which were confirmed on electroencephalogram (EEG). The infant responded well to intravenous seizure medications with no further cyanotic episodes. Cranial ultrasound (CUS) (figure 1) showed a small vermis with a large subarachnoid space as well as decreased volume of corpus callosum, ventriculomegaly strongly suggestive of Edwards Syndrome, with similar findings on MRI. Subsequently, chromosomal karyotype confirmed our radiological diagnosis of Edwards syndrome with 47 XX +18 in all cells. The infant improved clinically and was discharged home on day of life 55.

Abstract #268 Figure 1

Sagittal ultrasound and T1 MRI: hypoplasia of vermis and corpus callosum

Discussion

Edwards syndrome has a well-known association with apnea and epilepsy with the prevalence of epilepsy either focal or generalized up to 65% with onset reported to be after the neonatal period. A previous review of 8 patients with Trisomy 18 with multifocal EEG abnormalities, earlier age at seizure onset and underlying structural brain malformation on CUS has been reported which is similar to our case. A review of 21 infants diagnosed with Edwards Syndrome at our institution from 1986 to 2000 had similar findings on postnatal cranial ultrasound with cerebellar hypoplasia in 20/21, corpus callosum hypoplasia in 17/21 and ventriculomegaly in 10/21 infants, who were later confirmed to have Edwards Syndrome. This case report emphasizes CUS to be an excellent bedside diagnostic modality for infants with Edwards Syndrome who have not been diagnosed prenatally.

#269  A case of early-onset sepsis with enterococcus faecalis in a neonate born to a COVID-positive mother

SP Williams1*

W Livingston2

A Safarulla2

1Edward Via College of Osteopathic Medicine – Auburn Campus, Auburn, AL

2South Georgia Medical Center, Valdosta, GA

Case Report

We present a case of early-onset sepsis with enterococcus faecalis in a neonate born to a covid-positive mother.

36 week spontaneous vaginal delivery to a mother with late entry prenatal care. She presented with prolonged rupture of membranes of approximately 24 hours. She tested positive for COVID-19 upon admission, but was asymptomatic. At delivery, baby was noted to have foul-smelling and meconium-stained amniotic fluid increasing the risk for infection. CBC and blood culture were sent. Empiric ampicillin and gentamicin were started. The baby was placed in isolation due to COVID exposure but was found negative after 24 and 48 hours of life. At 18 hours of life the blood culture was noted to be positive for Gram positive cocci in chains, preliminarily thought to be Streptococci. Ampicillin therapy was increased to every 8 hours to cover for meningitis, and a repeat blood culture was sent. Lumbar puncture performed to rule out meningitis, revealed 30 WBCs and identification of gram-positive organisms on microscopy. The organism was identified as Enterococcus faecalis and was ampicillin sensitive. Patient completed a 10-day course of gentamicin and a 21-day course of ampicillin. The repeat blood culture showed no growth at 120 hours, a repeat lumbar puncture confirmed the infection cleared. The patient remained clinically stable and eventually discharged home.

Early onset sepsis (EOS) with enterococcus faecalis in a neonate is extremely rare. The occurrence of EOS with meningitis due to this organism in the setting of being born to a COVID positive mother has not yet been described.

#270  A case of amniotic band syndrome and congenital adrenal hyperplasia

SP Williams1*

W Livingston2

A Safarulla2

1Edward Via College of Osteopathic Medicine – Auburn Campus, Auburn, AL

2South Georgia Medical Center, Valdosta, GA

Case Report

We present a case of amniotic band syndrome coexisting with congenital adrenal hyperplasia (CAH).

32 week preterm neonate delivered via spontaneous vaginal delivery. Unremarkable pregnancy with normal 20 week anatomy scan. Upon delivery patient was noted to have respiratory distress syndrome which was subsequently treated with CPAP without the need for surfactant. Further examination revealed amputation of the distal 2/3 of multiple digits on bilateral hands and feet. Constrictive rings as well as residual fibrous band tissue was visualized. The patient was also noted to have ambiguous genitalia consisting of clitoromegaly with posterior labial fusion. Abdominal ultrasound was performed to visualize sex organs which revealed a uterus. Karyotype sent to confirm the sex, revealed 46, XX. A head ultrasound and echocardiogram revealed normal anatomy. As part of the work up for CAH, 17-hydroxyprogesterone levels to assess the level of enzymatic deficiency resulted at 276 mg/dL which is normal. Serial metabolic profiles were obtained since patient was at risk of developing adrenal crisis. Patient to follow with endocrinology, who will investigate for non-classical forms of CAH, and plastic surgery as outpatient. The patient continued to gain weight, improved respiratory function, and was discharged home.

Amniotic band syndrome (ABS) comprises various congenital anomalies, which include disruption, deformation, and malformations of organs that were intended to develop normally. From our extensive literature review, a case of amniotic band syndrome concurrently presenting with features suspicious for CAH has not been previously described.

Abstract #270 Figure 1

Autoamputated digits of (A) R head and (B) L foot with residual fibrous bands. (C) Clitoromegaly with posterior labial fusion

Neurology and neurobiology

Joint plenary poster session and reception

4:30 PM

Thursday, February 10, 2022
#271  Myesthenic crisis after thymoma removal in a 60-year old male

MA Tanbir1*

L Bellamkonda1

R Philip2

KG Holder1

1Texas Tech University Health Sciences Center, Amarillo, TX

2Amarillo VA Health Care System, Amarillo, TX

Background

Myasthenia Gravis (MG) is an autoimmune condition characterized by progressive muscle weakness secondary to inhibitory autoantibodies at postsynaptic acetylcholine receptors of the neuromuscular junction (NMJ). MG is often seen as a paraneoplastic syndrome secondary to thymomas. Surgical removal of any anterior mediastinal mass when possible is currently accepted as standard of care and primary treatment of MG secondary to thymoma involves thymic removal. However, the literature is limited in evaluating efficacy of thymectomy as definitive treatment for secondary MG.

Case Report

We present a case of a 60-year-old male with a history of falls who presented with progressively worsening left lower extremity weakness and muscle cramping for 5 days. Head CT, brain MRI, and chest x-ray, showed no acute changes. Pan-CT scan was used to evaluate for occult malignancy and revealed an anterior mediastinal mass. Cardiothoracic surgery was consulted and patient was scheduled for outpatient surgery. His neurologic symptoms resolved without intervention and the patient was discharged home.

The resected mass measured 3.0 x 3.5 cm, and biopsy confirmed the diagnosis of medullary thymoma (WHO Type A) without evidence of malignancy. Three weeks after surgery, he returned to the hospital with complaints of bulbar/ocular symptoms: difficulty speaking, blurry vision, bilateral ptosis, balance problems, difficulty swallowing and difficulty breathing. Physical exam was only notable for bilateral ptosis and speaking with a lisp. His symptoms worsened throughout the day and improved with rest.

Patient’s symptoms completely resolved after starting pyridostigmine, which was titrated to 60 mg thrice daily, and he was discharged home. Acetylcholine receptor antibodies (AChR Ab) were drawn but pending at the time of discharge.

Discussion

In addition to having a very typical constellation of symptoms on presentation, rapid diagnosis of MG was supported by the ice pack test, which has a >90% sensitivity for MG. A negative ice-pack test does not exclude MG and AChR Ab testing is recommended due to its near high specificity for thymoma associated MG.

Initial management of MG involves use of acetylcholinesterase inhibitors such as pyridostigmine or neostigmine, which allow ACh to accumulate in the NMJ and overcome AChR Ab. In severe cases, intravenous immunoglobulin and steroids can be used.

It is important to note that the diagnosis of MG in this patient was unknown at the time of his surgery. If his diagnosis had been known, surgery would have been postponed until the patient was on optimal medical management as surgery can trigger a myasthenic crisis. As seen in this case, surgery triggered our patient’s MG as he returned three weeks after surgery with classic symptoms of MG. If patient’s symptoms recur on current medical therapy, he may also need radiation therapy.

#272  Lateral medullary infarction with hypoplastic vertebral artery

J Ciubuc*

M Anees

EM Alhussain

J Walker

Texas Tech University Health Sciences Center, Amarillo, TX

Case Report

A 42-year-old male with a past medical history of hypertension presents to the emergency room with a sudden onset of difficulty swallowing 9 hours constant prior to ER presentation. The patient reports being unable to swallow liquids or solids. He describes having a similar episode in the past in 1998 following physical neck trauma because of altercation which was resolved within few days. On presentation, the patient was afebrile with a blood pressure of 152/116 mm Hg, a heart rate of 81 beats per minute, a respiratory rate of 18 breaths per minute, with an oxygen saturation of 95% in the emergency room. Computed tomography angiogram (CTA) of the head with intravenous contrast revealed a diminutive right vertebral artery. No other intracranial abnormalities. magnetic resonance images (MRI) of the brain were performed with and without intravenous contrast. MRI revealed a 7 x 4 mm acute infarct of the right medulla with no associated mass effect or hemorrhage. CTA of the neck with intravenous contrast revealed a dominant left vertebral artery with a developmentally decreased right vertebral artery. The right vertebral artery revealed several areas of high-grade stenosis in the upper cervical levels above C4. Further evaluation of the patient revealed mild paresthesia on right half of his face, with loss of temperature sensation on the left side of the body. A Dobhoff nasogastric feeding tube was placed, and the patient worked with speech therapy on swallowing exercise. The patient was discharged to rehabilitation placement six days after admission. After 2 weeks patient still unable to swallow and PEG tube was placed.

Discussion

Variations of vertebral artery flow are common in the general population, with approximately 75% of individuals having left or right dominant flow. Atretic vertebral arteries are uncommon, making up 2–15% of vertebral artery variations. Despite the asymmetry of vertebral vascular flow.

The patient does not have a previous history of stroke and has a past medical history notable only for hypertension, which limits his stroke risk factors. Uniquely, the patient noted a similar dysphagia presentation in 1998 following physical trauma, but it is unsure if vertebral hypoplasia was a factor in that presentation.

While previous cases of young adults with WS and hypoplastic vertebral arteries have been reported, they were often associated with multiple stroke risk factors. The patient we present shows a rare relationship between vertebral artery hypoplasia and lateral medullary infarction in the presence of limited stroke risk factors. people with vertebral artery hypoplasia are usually asymptomatic but have a higher potential risk to develop lateral medullary infarction compared to people with normal vertebral artery, even in individuals with minimal stroke risk factors.

#273  Reversible posterior leukoencephalopathy (rpls) in the setting of axitinib use for renal cell carcinoma (rcc)

P Nimmagadda*

D Velu

A Alapati

F Ibrahim

UAB, Huntsville, AL

Case Report

RPLS is a clinical radiographic entity characterized by the abrupt onset of neurological symptoms with evident brain lesions. It still remains a conundrum as the lesions can involve any regions of brain not just posterior, and also, they are not always reversible. The known etiologies include hypertension, vasculopathies, vasoactive drugs and malignancy. Here, we report one such rare case presented as a drug adverse effect.

A 79-year-old well-functioning elderly Caucasian female with history of hypertension and recent diagnosis of metastatic RCC presented to us with acute onset encephalopathy. History revealed initiation of primary treatment with nivolumab and ipilimumab around 8 weeks ago and recent addition of Axitinib 10 days prior. On arrival, with a poor GCS of E2V1M2, she was intubated. Initial CT head ruled out acute emergencies like hemorrhagic stroke and cerebral edema but showed suspicious ischemic lesions. Further, MRI Brain exposed the relatively symmetric bilateral extensive cortical and subcortical increased signal on the DWI and T2/FLAIR involving frontal, parietal and occipital lobes with no reciprocal changes in ADC ruling out infarcts. With these above mentioned findings, the diagnosis of RPLS was deemed to be more likely and the treatment was started focusing on the inciting agents by controlling blood pressure and discontinuation of offending drug – Axitinib.

With the repeat MRI in 3 weeks, there were signs of resolution of few cortical lesions instilling hope for meaningful recovery. In midst of the course, we were also able to rule out possible differentials including Acute disseminated encephalomyelitis (ADEM), Multiple sclerosis (MS) and other infectious etiology with necessary CSF testing.

Our case highlights the incidence of RPLS with Axitinib use, a VEGF inhibitor used for the treatment of Renal cell carcinoma. The imaging findings were classic as mentioned above. The use of VEGF inhibitors was already linked to hypertensive crisis in literature which could be serving as the pathology behind the incidence of RPLS. Strict monitoring of blood pressure is thus advisable during the treatment with VEGF inhibitors/Axitinib.