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2020 Southern Regional Meeting

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Cardiovascular club I

11:00 AM

Thursday, February 13, 2020

1 Cardiac fibrosis and heart failure in mice carrying genetic ablation of natriuretic peptide receptor-A: role of TGF-beta1 pathway

H Chen

R Samivel

U Subramanian

KN Pandey*

Tulane University Health Sciences Center, New Orleans, LA

Purpose of study

Mice carrying targeted-ablation of the natriuretic peptide receptor-A (NPRA) gene (Npr1) exhibit cardiac hypertrophy with increased collagen deposition and fibrosis. The goal of this study was to determine the underlying mechanisms that regulate the development of cardiac fibrosis and heart failure in Npr1 gene-knockout mice.

Methods used

The Npr1 null mutant (Npr1-/-, 0-copy), heterozygous (Npr1±, 1-copy), and wild-type (Npr1+/+, 2-copy) mice were orally administered with transforming growth factor-β1 (TGF-β1) receptor antagonist, GW788388 (2 mg/kg/day) by gavage for 28 days. Together, systolic blood pressure (SBP), heart weight-to-body weight (HW/BW) ratio, left ventricular end-diastolic dimension (LVEDD), left ventricular end-systolic dimension (LVEDS), and percent fractional shortening (FS) were analyzed. The heart was isolated and used for the analysis of fibrotic markers using quantitative reverse transcription-polymerase chain reaction (qRT-PCR) and Western blot.

Summary of results

The HW/BW ratio, LVEDD, and LVEDS were significantly increased in Npr1-/- and Npr1± mice than wild-type Npr1+/+ mice. At the same time the fractional shortening was greatly reduced in Npr1-/- and Npr1± mice compared with Npr1+/+ mice. The Npr1-/- null mutant (0-copy) mice showed 6-fold induction of cardiac fibrosis as compared with wild-type (2-copy) control mice. Moreover, the expression of fibrotic markers such as connective tissue growth factor (CTGF), α-smooth muscle actin (α-SMA), TGF-β1, TGF-βRI, TGF-βRII, and SMAD proteins were significantly increased in Npr1-/- and Npr1± mice hearts compared with age-matched wild-type control animals. The Npr1 gene-knockout (0-copy) mice also suffered with congestive heart failure at early adult age as compared with Npr1 wild-type (2-copy) mice. The treatment with TGF-β1 receptor antagonist, GW788388, significantly prevented the cardiac fibrosis and potentially down-regulated the expression of fibrotic markers and SMAD proteins in Npr1-/- and Npr1± mice.

Conclusions

The results of the present study indicate that, the induction and development of cardiac fibrosis and heart failure in Npr1 gene-disrupted (0-copy) and haplotype (1-copy) mice is regulated through the TGF-β1-mediated SMAD-dependent pathway.

2 Insulin-like growth factor-1 upregulates junction proteins, while it downregulates adhesion proteins in vascular endothelial cells: potential mechanisms for anti-atherogenic effects

Y Higashi1*

S Danchuk1

Z Li2

T Yoshida1

S Sukhanov1

P Delafontaine1

1Tulane University School of Medicine, New Orleans, LA

2University of Missouri School of Medicine, Columbia, MO

Purpose of study

Insulin-like Growth Factor-1 (IGF-1) is an anti-atherogenic growth factor, having anti-inflammatory effects on macrophages and pro-survival and profibrotic effects on vascular smooth muscle cells. However, its effects on vascular endothelial cells are not fully described. We assessed IGF-1 effects on expression levels of intercellular junction proteins and cell adhesion proteins in aortic endothelial cells.

Methods used

Human aortic endothelial cells (hAoECs) are exposed to 0–100 ng/mL IGF-1, anti-IGF-1 receptor (IGF1R) neutralizing antibody, or Picropodophyllin (PPP; IGF1R inhibitor), and protein expression levels were determined by Western blot. Small molecule permeability was assessed on monolayer of hAoECs by transwell permeability assay.

Summary of results

IGF-1 elevated expression levels of junction proteins, namely CD31, VE-cadherin, Occludin, Claudin-5, and Jam-A, while IGF1R inhibition by neutralizing antibody or PPP decreased their expression levels. Intriguingly, IGF-1 decreased expression levels of adhesion proteins, ICAM-1 and VCAM-1. We next tested barrier function of monolayer of hAoECs. Transwell permeability assay showed that IGF1R inhibition by PPP elevated permeation of dextran-FITC by 4.8±0.4-fold (P<0.01), consistent with the downregulation of junction proteins. Meanwhile, IGF-1 decreased adhesion of THP-1 monocytes on monolayer of hAoECs by 34±5% (P<0.05), while IGF1R-inhibition by PPP increased monocyte adhesion by 3.1±0.2-fold (P<0.01). In aortas of endothelial IGF1R-deficiency mice (Cdh5-Cre/Igf1rfl/fl mice) we found Occludin, Claudin-5 and Jam-C are downregulated, which is consistent with the observation in hAoECs. Finally, the Cdh5-Cre/Igf1rfl/fl mice on Apoe-deficient background produced more atherosclerosis than control mice by 33±8% (P<0.05) on a high-fat diet, suggesting anti-atherogenic effects of IGF-1 in the endothelium.

Conclusions

Our results suggest that IGF-1 supports endothelial barrier function by positively regulating junction proteins, while it suppresses leukocyte adhesion by negatively regulating adhesion proteins in endothelial cells, thereby exerting anti-atherogenic effects.

3 Spontaneous superimposed preeclampsia is associated with impaired gut microbiome remodeling

JA Ishimwe*

A Akinleye

A Johnson

MR Garrett

JM Sasser

University of Mississippi Medical Center, Jackson, MS

Purpose of study

Preeclampsia (PE), a hypertensive disorder of pregnancy, is a leading cause of maternal deaths. It affects 2–8% of all pregnancies, and its pathophysiology is not fully understood. Recent studies in preeclamptic patients have suggested a role for the gut microbiome in the disease. We have characterized the Dahl salt-sensitive (Dahl S) rat as a spontaneous rodent model of superimposed PE. Here, our purpose was to test the hypothesis that preexisting chronic hypertension impairs maternal gut microbiome remodeling and contributes to the development and/or progression of superimposed PE.

Methods used

Female Sprague Dawley (SD) and Dahl S rats were maintained in conventional caging in the same room and on the same diet (Teklad 7034, 0.3% NaCl). Half of the rats of each strain were mated to have pregnant and virgin groups (n=7–9/group). Fecal samples were collected at baseline (BL), gestation day 20 (GD20) and one-week postpartum (PP) to assess gut microbiome via 16S rRNA gene sequencing. Alpha diversity (Shannon index), beta diversity (Bray-Curtis index), and linear discriminant analysis effect size (LEfSe) were determined from the sequenced data to assess microbial differences. At BL, Dahl S rats had higher alpha diversity (P<0.0001) and distinctly clustered beta diversity (p<0.001) compared to SD rats.

Summary of results

Dahl S had higher proteobacteria abundance (665,980 vs 51,240, p<0.05), a marker of dysbiosis. In response to pregnancy, we observed significant differences among groups in both alpha and beta diversity during late pregnancy. Also, proteobacteria abundance in the SD rats rose from 0.56% (BL) to 13.46% (GD20) before subsiding to 7.36% (PP) suggesting that changes may be a normal adaptation to pregnancy. The Dahl S, however, showed no change in proteobacteria: 5.29% (BL) to 6.35% (GD20) and 7.8% (PP). In addition, the SD displayed elevations in pathogenic bacteria genera. The Helicobacter genus was higher in SD pregnant than SD virgin (580,110 vs 3,045, p<0.05) but similar between Dahl S groups (p=0.87). The Bacteroides and treponema genera exhibited a similar pattern.

Conclusions

In this study, we have presented preclinical evidence for pregnancy-specific alterations in the gut microbiota, and we suggest that these may be impaired in PE.

4 Regional differences in epidemiology and outcomes of heart failure admissions across the united states

A Goel*

H Paydak

JL Mehta

University of Arkansas for Medical Sciences (UAMS), Little Rock, AR

Purpose of study

Periodic surveillance of geographical variations in cardiovascular health is important to achieve the goal of reducing regional disparities in healthcare delivery. We aimed to study differences in epidemiology and outcomes of heart failure admissions by geographic regions in the United States.

Methods used

The National Inpatient Sample (NIS) database for the year 2016 was queried. Adult patients admitted with a principal diagnosis of heart failure were identified using validated ICD-10 codes. Comparisons were made between four regions - Northeast, Midwest, South and West. Statistical analysis was performed using STATA.

Summary of results

A total of 807,764 heart failure hospitalizations were identified. Of these, 153,233 were in the Northeast; 184,090 in the Midwest; 331,506 in the South; and 138,935 in the West. There was a small difference in the mortality rates (highest in West at 3%, and lowest in South at 2.66%, p=0.03) and length of stay (longest in the Northeast, and shortest in the West, p<0.001) between regions. A significant difference was observed in the total hospital charges per hospitalization (nearly $65,000 in the West, and only $37,000 in the Midwest, p<0.001).

Abstract 4 Table 1

Baseline characteristics and outcomes of heart failure admissions divided by geographic region Baseline characteristics and outcomes of heart failure admissions divided by geographic region

Conclusions

Our study demonstrates the existence of regional differences in the costs and outcomes of healthcare delivery to heart failure patients. Further research is needed to explore the reasons for these differences.

5 Inhibition of angiotensin II type 1 receptor in skeletal muscle stem (satellite) cells prevents angiotensin II-induced skeletal muscle wasting

T Yoshida*

P Delafontaine

Tulane University, New Orleans, MO

Purpose of study

Patients with advanced congestive heart failure (CHF) or chronic kidney disease (CKD) often have increased angiotensin II (Ang II) levels and cachexia. We previously demonstrated that Ang II infusion in rodents causes skeletal muscle wasting, likely contributing to cachexia in CHF and CKD. We also demonstrated that Ang II inhibits proliferation of skeletal muscle stem (satellite) cells (SCs) and reduces muscle regenerative capacity. We hypothesize that Ang II type 1 receptor (AT1R) signaling in SCs plays a role in developing Ang II-induced muscle wasting.

Methods used

We generated tamoxifen-inducible, SC-specific AT1R-null mice (SC-AT1R-/-) by crossing Pax7CreER and AT1R-floxed mice. By introducing Cre-reporter gene in these mice, we labeled SCs and their progenies with EYFP. These mice were infused with Ang II (1.5 μg/kg/min), and muscle regeneration and wasting were analyzed by qRT-PCR, western blotting and immunohistochemistry.

Summary of results

In hindlimb cardiotoxin injury model, Ang II reduced the number of regenerating myofibers (71.9% decrease, p<0.001) and the expression of SC proliferation/differentiation markers MyoD and myogenin (56.5% and 62.5% decrease, respectively, p<0.001). In contrast, SC-AT1R-/- mice were protected against these Ang II-mediated reductions in muscle regeneration. In vitro, Ang II inhibited primary cultured SC proliferation, whereas AT1R-null SCs were not affected. Importantly, SC-AT1R-/- mice restored skeletal muscle mass in high Ang II condition, likely due to the increased muscle regenerative capacity. SC lineage tracing and gene expression analyses revealed that, in the presence of high Ang II, AT1R-deficient SCs generated higher number of newly formed/repaired myofibers (12.4±2.7% of total myofibers in SC-AT1R-/- and ND in control, p<0.001), resulting in the restoration of muscle mass and cross-sectional area in SC-AT1R-/- mice.

Conclusions

These data indicate that inhibition of AT1R signaling in SCs could have a therapeutic potential to treat muscle wasting in chronic diseases with high Ang II, such as CHF and CKD.

6 Smooth muscle specific glyceraldehyde-3’-phosphate dehydrogenase reduces atherosclerosis and promotes the stable plaque phenotype

S Sukhanov1*

X Hou2

P Snarski1

T Yoshida1

P Delafontaine1

1Tulane University, New Orleans, LA

2University of Missouri at Columbia, Columbia, MO

Purpose of study

Glyceraldehyde-3’-phosphate dehydrogenase (GAPDH) is a glycolytic enzyme with a multiple glycolysis-unrelated functions. GAPDH protected smooth muscle cells (SMC) against apoptosis via upregulation of Ape1 endonuclease, the major DNA repair enzyme. GAPDH levels were reduced in atherosclerotic plaque SMC. We hypothesized that SMC-specific GAPDH overexpression will reduce atherosclerosis.

Methods used

We generated Apoe-null mice with SM22α promoter-driven GAPDH overexpression (SM-GAPDH mice). Atherosclerotic burden was quantified using aortic valve H&E-stained cross-sections, plaque composition by IHC with antibody for SMC, and macrophage markers; collagen by Trichrome, apoptosis by TUNEL, vascular reactivity by organ chamber assay, oxidative DNA damage by AP site ELISA, protein expression by Westerns (WB).

Summary of results

GAPDH levels were increased >2-fold (WB, P<0.05) in aortas from SM-GAPDH vs. Apoe-null controls. SMC GAPDH did not change aortic pulse wave velocity (SM-GAPDH, 1.1±0.1 m/s, control, 1.2±0.3 m/s) (ultrasound imaging) as well as aortic ring responses to PE, Ach or SNP. High-cholesterol fed SM-GAPDH had reduced atherosclerotic burden (28±3% decrease, P<0.05), elevated plaque SMC (IHC, a-SM actin, 3.1-fold increase, P<0.01; IHC, calponin, 3.8-fold increase, P<0.05), increased plaque collagen (2.1-fold increase, P<0.05), reduced plaque SMC apoptosis (3.2-fold decrease, P<0.05) without changes in macrophages (IHC, Mac3). Aortas isolated from SM-GAPDH have increased Ape1 endonuclease (WB, 31±5% increase, P<0.05), reduced cleaved PARP (WB, 74±12% decrease, P<0.001) and decreased oxidative DNA damage (2.5-fold decrease, P<0.05; WB, pSer139 histone H2AX, 33±3% decrease, P<0.05) compared to controls. Plaques in SM-GAPDH had thicker SMC-rich fibrous plaque cap (3.5-fold increase, P<0.05) and decreased area of necrotic core (1.8-fold reduction, P<0.05) suggesting enhanced plaque stability.

Conclusions

SMC’ GAPDH reduces atherosclerotic burden, DNA damage and cell apoptosis in atherosclerotic mice. Our data taken together with in vitro findings indicate that Ape1 upregulation mediates GAPDH effect on DNA and apoptosis. Stimulation of GAPDH/Ape1 axis is a novel potential anti-atherosclerotic therapy.

7 Two modes of entanglement between quantum state and the components of the intracellular renin angiotensin system

PI Altieri1,2*

W De Mello1

HL Banchs1,2

N Escobales1

1University of Puerto Rico Medical Sciences Campus, San Juan

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

Purpose of study

Superconductivity (S.C.) is a concept of pushing materials to the extremes to get maximum results of these processes.

Methods used

Applying this concept, experiments were done intracellularly in isolated heart cell pairs measuring intracellular electric conduction across junction gaps (G.I.) induced by Enalapril (E.) and Angiotensin II (Ang II). E. (25 ug/ml) was injected rapidly up to a dose of 1 ug/ml in 4 minutes. Ang II was injected intracellularly at 1 ug/min.

Summary of results

A reduction of G.I. with Ang II was 55% without a plateau. With E., an increase in G.I. (106%) was measured until a plateau was reached. We think the reason for the plateau seen with E. was a reduction of entanglement, due to a photon effect from an intracellularly electron cloud.

Conclusions

All these entanglement processes were during a superconductivity state. The end of the entanglement process was due to a photon effect; probably this didn’t occur with Ang II. The effect of E. is an effective way to produce coupling of the heart cells and a reduction of coupling by Ang II. This is a very important electrical effect in congestive heart failure with improvement of left ventricular function (L.V.F.) by E. and by a reduction of coupling by Ang II, reducing L.V.F. Further studies are needed to clarify these concepts.

8 Coxsackie B infection, would you think the kidneys?!: A rare case of coxsackie B-induced myopericarditis leading to severe acute heart failure and complicated by end-stage renal disease

S Metzler*

M Elmassry

TE Whisenant

J Kelley

B Mantilla

P Paz

K Nugent

Texas Tech, Lubbock, TX

Introduction

Coxsackie B virus is involved in 25–40% cases of acute myocarditis. Renal dysfunction due to Coxsackie is extremely rare and the mechanism is unclear. We present a case of Coxsackie B-induced myopericarditis with concomitant severe renal failure.

Case presentation

A 26-year-old female with history anemia who presented with worsening fatigue, weakness, SOB, pleuritic chest pain, cough, epistaxis, and hemoptysis, tingling in her arms and reduced UOP for 3 weeks. Labs showed BUN/sCr of 85/11.2 with FENa of 9.98 indicative of intrinsic renal process. UA positive for blood and protein, CK 432 and pro-BNP >70k. CXR showed pulmonary edema and cardiomegaly. US showed small kidneys consistent with chronic kidney disease. TTE showed LVEF 20–24% with global hypokinesis. The patient was started on hemodialysis and treated for CHF. Infectious disease work up was positive for Coxsackie-B antibody 2,5, and 6. Rheumatology work up was negative. Renal biopsy showed interstitial fibrosis and tubular atrophy consistent with extensive chronic kidney damage, with negative immunofluorescent study excluding autoimmune reaction. Myocardial biopsy showed active lymphocytic myocarditis. Renal failure never resolved and the patient was kept on intermittent hemodialysis. However, her LVEF recovered to 50–54% in about 2 months.

Discussion

This patient presented with myopericarditis leading to acute heart failure with EF 20%. This was likely due to Coxsackie B infection given the positive serology and biopsy results. Rhabdomyolysis-induced renal failure is unlikely in this case as CK was only 432. The cause of renal failure was unclear. We believe it may have been due to Coxsackie B infection. Cardio-renal syndrome was unlikely as FENa was 9.98 and renal function never recovered even after the quick recovery of the heart failure. Unlike our patient, reported cases with renal dysfunction due to Coxsackie had higher chances of recovery.

Conclusion

We recommend a high suspicion for Coxsackie B virus infection in young adults presenting with acute heart and kidney failure. More case studies are required to better understand the correlation between Coxsackie and renal failure.

Adult clinical symposium

12:00 PM

Thursday, February 13, 2020

9 A case of cholera acquisition within the united states

GB Maniam*

EN Nguyen

S Milton

Texas Tech University Health Science Center, Amarillo, TX

Introduction

Cholera has been woven into human history through numerous pandemics, with the most recent ongoing since 1961. Global rates of cholera continue to decline, but outbreaks continue to pose diagnostic challenges for clinicians, which delays initiation of treatment and prolongs the disease course. Despite millions of infections and thousands of deaths worldwide each year, cholera remains rare in the United States, with the few cases each year usually being the result of pathogen acquisition while the patient traveled abroad. This paper presents a unique case of cholera acquired in the United States, which emphasizes the necessary vigilance of symptom recognition, in the context of appropriate clinical investigation, in ensuring the patient had a full recovery.

Case presentation

A 58-year old female presented with gradual onset RLQ abdominal pain with associated diarrhea, fever, nausea, and dysuria; travel history is notable for a recent trip to Hawaii. Patient was admitted to the hospital due to concern of pyelonephritis, appendicitis, and adrenal crisis – but workup for these etiologies was unremarkable after a few days of inpatient hospitalization. A stool antigen test returned positive for Vibrio cholerae test, while negative for every other tested stool pathogen; the stool panel was repeated due to the rarity of cholera acquired in the United States, but confirmed the results. Treatment was initiated with doxycycline, ciprofloxacin, and metronidazole. After a week of inpatient hospitalization, the patient endorsed a complete resolution of her pain and diarrhea, and was recommended for discharge.

Discussion

Recognition and treatment of cholera in the United States is exceedingly rare, yet effective diagnosis with early initiation of treatment is known to reduce mortality and shorten disease course. While other more common diagnoses must definitely be excluded first, it is important for cholera to be kept on the differential for patients presenting with treatment refractory, watery diarrhea causing hypotension. This case of a patient with a recent travel history to Hawaii and infection with cholera underscores the importance of investigative medicine and clinical expertise in optimizing patient care, even when presented with rare illnesses.

10 Fatal primary cutaneous anaplastic large T-cell lymphoma presenting as sweet syndrome

I Ivyanskiy1*

J Yeary2

E Melese1

T Naguib1

1Texas Tech Univ HSC Amarillo, Amarillo, TX

2Amarillo VA Health Care System, Amarillo, TX

Case report

Primary cutaneous anaplastic large T-cell lymphoma (PC-ALCL) is a rare type of T-cell lymphoma that involves skin only without evidence of systemic disease. It has a favorable prognosis. We describe a case of a fatal PC-ALCL that masqueraded as sweet syndrome.

A 69-year-old woman presented with a painful rash on her forehead and temples for 2 months. She had extremely tender nodules on the frontal scalp and smaller lesions extending over the parietal scalp. She had a background of diabetes mellitus type 2, hypertension, and end-stage renal disease (ESRD) on peritoneal dialysis.

Initial biopsy showed a neutrophilic infiltrate with leukocytoclasia suggestive of sweet syndrome versus infectious etiology. Due to lack of clinical response a second biopsy was performed that showed an atypical lymphoid proliferation suggestive of T cell lymphoma. Whole body CT did not show other organ involvement. Bone marrow biopsy was normal as well. The diagnosis accordingly was PC-ALCL.

Due to End stage renal disease (ESRD) and extensive head involvement, she was not eligible for systemic chemotherapy, surgery, or radiotherapy. Topical treatment with bexarotene gel, and oral bexarotene did not control the rapid spread within a few weeks with secondary ulcerations. The patient opted for no treatment and stopped dialysis. She was admitted to hospice and died within a few days.

PC-ALCL is diagnosed as a solitary or grouped nodules growing over few weeks to months with further ulceration. Pathology reveals dermal infiltrate that appears initially like sweet syndrome but it has CD30 positive cell infiltration.

Differential diagnosis is broad including other lymphomas and reactive lymphoid hyperplasia. Treatment options are numerous and discussed here. Generally, PC-ALCL has a good survival rate of about 90 percent over 10 years.

Our case represents an extremely aggressive case of PC-ALCL with rapid progression and a fatal outcome. We recommend repeating skin biopsy to revisit the diagnosis whenever sweet syndrome fails to respond to steroids.

11 Hansen’s disease (leprosy) in the texas panhandle: a case series

EN Nguyen*

GB Maniam

S Milton

Texas Tech University Health Sciences Center, Amarillo, TX

Introduction

Hansen’s Disease, also known as leprosy, is caused by Mycobacterium leprae, a slow-growing acid-fast bacillus that causes significant disease of the skin, peripheral nerves, mucosa of the upper respiratory tract, and the eyes. The long-term complications include type 1 reversal reactions, type 2 erythema nodosum leprosum (ENL), neuropathy, and blindness. Globally, leprosy continues to be a leading infectious cause of disability, with the majority of new cases from India, Brazil, and Southeast Asia. While leprosy is rare in the United States, we report 4 cases of lepromatous leprosy that have been treated in West Texas in the past 5 years. These cases demonstrate the diversity of presentation, severity of complications, and variation in treatment and management of leprosy.

Case presentation

Case 1: A 39-year-old Liberian refugee presented with 8-month history of diffuse nodular skin rash and lagophthalmos of the left eye. Diagnosis was confirmed by biopsy. His ocular disease progressed to complete loss of vision.

Case 2: A 20-year-old Burmese refugee with a history of previously treated leprosy presented with painful nodular rash and fever, consistent with type 2 ENL, that improved with steroid therapy. She developed Addison’s disease and steroid-induced glaucoma of the left eye.

Case 3: A 41-year-old Mexican immigrant with a history of previously treated leprosy presented with erythematous nodular rash and fever. He received thalidomide and prednisone for type 2 ENL reaction.

Case 4: A 57-year-old Mexican immigrant with a history of previously treated leprosy presented with diffuse burning pain for 6 months. He was treated with gabapentin for neuropathic pain and prednisone for type 2 ENL. He received a total right hip replacement due to avascular necrosis of the right femoral head.

Discussion

This case series highlights the importance of recognizing leprosy and differentiating its recurrence from type 2 ENL reactions. Management with steroid therapy should involve appropriate tapering and monitoring for adverse effects. Leprosy patients should also be monitored for chronic neuropathic pain and ocular disease, which can lead to blindness. Early diagnosis and judicious treatment of leprosy and its complications are crucial in preventing permanent disability.

12 Human herpesvirus 6 (HHV-6) meningitis in an adult patient

GB Maniam

H Wilkerson*

S Milton

Texas Tech University Health Sciences Center, Amarillo, TX

Introduction

By efficiently diagnosing HHV-6 meningitis, proper treatment protocols are able to be initiated earlier in order to reduce morbidity and mortality. Given the rarity of HHV-6 meningitis in immunocompetent adults, there are currently no established standard treatment guidelines; previous cases reported in the literature, as well as this case itself, suggest that either IV ganciclovir or IV foscarnet are the most reasonable first-line treatment options.

Case presentation

A 62-year-old female presented to the hospital with status epilepticus and altered mental status, including visual hallucinations. An EEG showed significant abnormalities suggesting temporal lobe seizure activity, which resolved with administration of antiepileptics, but was followed by a postictal coma. An MRI was negative for any new cerebral infarcts, while cardiac monitoring was within normal limits. A lumbar puncture revealed no white blood cells, but was PCR tested for numerous viral agents and was only positive for only HHV-6. Patient was therefore started on IV ganciclovir, and then gradually emerged from her coma. After fourteen days of inpatient ganciclovir, the patient had improved significantly and was recommended for discharge. Final assessment was HHV-6 meningitis, status epilepticus secondary to viral meningitis, postictal coma secondary to viral meningitis, and metabolic encephalopathy secondary to seizure disorder.

Discussion

Many cases of viral meningitis are idiopathic, in that clinicians are often unable to identify a particular viral etiology. However, increased utilization of PCR has enabled physicians to better recognize rare causes of viral meningitis – such as human herpesvirus 6 (HHV-6) meningitis in immunocompetent adult patients – and will likely continue to improve the rate of causative agent identification in seemingly idiopathic viral meningitis. In this specific case, the test was performed twice and found to be positive both times for HHV-6. In cases of viral meningitis in which the causative agent is unclear, it is important to keep rare causes such as HHV-6 on the differential. Evidence from prior case reports, as well as this case itself, seem to suggest that IV ganciclovir or IV foscarnet are the most reasonable treatment options for HHV-6 meningitis.

13 The rocky road to malignancy in an elderly patient

NL Rabelo-Pagan*

LF Alvarez Perez

E Valdes

MJ Rodriguez

AT Ortiz

A Febles

University of Puerto Rico, San Juan, PR

Case report

Tonsils serve as a defense line against oropharyngeal infections. Tonsillitis is an inflammation of the palatine tonsils, self-limited and associated with viruses. Bacterial etiology are <10%. When inflammation is refractoryfurther workup is warranted. Lymphoid malignancy is common but tonsillar malignancy is rare.

A 68 y/o woman with HTN, DM2, and hypothyroidism who presented with 3 mo of intermittent odynophagia and dysphagia. Treated bacterial tonsillitis without improvement. Patient developed acute respiratory distress that required endotracheal intubation. Neck CT revealed enlarged palatine tonsils with lymphadenopathy. The patient was started on broad-spectrum IV antibiotics for suspected peritonsillar abscess. In the OR prophylactic tracheostomy and oropharynx evaluation were perfomed. Right tonsil tissue was pale, friable, with multiple areas of necrosis. Right tonsillectomy was performed and tissue samples were sent to pathology. Results consistent with necrotic tissue.

The patient was admitted to ICU for persistent respiratory compromise which required ventilatory support. Initially, she remained afebrile but with increasing leukocytosis. She continued on broad spectrum antibiotics for presumptive diagnosis of sepsis secondary to bacterial tonsillitis. After weaned from mechanical ventilation, she developed intermittent fever, chills, and hypoactivity. All infectious, rheumatologic, inflammatory, and metabolic workup was negative.

She continued with fevers

Reevaluation of extracted tonsil tissue was sent to the NIH pathology laboratory. Histologic resulted was Tonsilar Non-Hodgkin B Cell Lymphoma (NHL).

Lymphomas commonly involve lymph nodes

However, NHL may have between 24–48% extranodal sites of invasion, less than 10% invade the tonsils.

This case depicts the importance of proper clinical evaluation and follow up in elderly patients presenting with odynophagia, dysphagia, and tonsillar enlargement. Tonsillar B cell lymphoma is uncommon, it can present in the elderly population and be mistaken for infectious tonsillitis. The diagnosis possibility increases clinical awareness for identification and therapy.

14 In case your doctor is unaware of pica symptoms, excess ice consumption can ruin your brain function

GD Bedanie*

AG Tikue

T Thongtan

E Elgwairi

K Nugent

Texas Tech University Health Science Center, Lubbock, TX

Introduction

Pica is described as an unusual condition in which patients crave and chew substances that have no nutritive values, such as ice, clay, soil or paper. Pagophagia (craving & chewing ice) is often associated with iron deficiency with or without anemia. Iron absorption can also be reduced in the presence of non-nutritive substances. It is commonly seen in pregnant women and pre-adolescent age group. We report a patient admitted to medical intensive care unit (MICU) due to hyponatremia and seizures found to have iron deficiency associated pagophagia as underlying cause.

Case summary

A 54-year-old female transferred to our MICU from outside hospital due to altered mental status, seizures, and hyponatremia. On presentation to outside hospital, she was weak and had a grand mal seizure; her sodium was as low as 120 mEq/L. She was placed on normal saline and transferred to our medical center for a higher level of care. Upon further evaluation, she stated she was recently craving and chewing too much ice. Her sodium was gradually corrected. Lab test showed hemoglobin 9.2 gm/dl and MCV 75fL. Iron deficiency with pica was considered, and iron panel was done. Her iron level was 9 mcg/dl, ferritin 4.8ng/ml, and transferrin saturation 2%. CT of head and EEG were normal. Patient was given parenteral iron infusion for 3 days. Craving for ice improved significantly. Patient was stable and had no more seizures. She was discharged with oral iron supplementation.

Conclusion

Although pica has been noted since 6th centuries AD, it remains a mysterious and fascinating occurrence. Pica is strongly associated with iron deficiency and believed to be a symptom of deficiency rather than its cause. Studies showed that many physicians are unaware of the pica symptoms, and it can be missed unless specifically addressed. Thus, pica can have significant health risks, including electrolyte abnormalities and metabolic disorders like our patient. It is recommended to evaluate patient with pica for iron deficiency and it causes. Iron therapy can cure the pagophagia earlier than hemoglobin recovery.

15 False negative adenosine deaminase in tuberculous pleural effusion

K Saraczewski*

G Loh

Eisenhower Army Medical Center, Grovetown, GA

Case report

Pleural tuberculosis is the most common extra-pulmonary manifestation of Mycobacterium tuberculosis infection. Diagnosis of a tuberculous pleural effusion is challenging as patients commonly have negative acid fast cultures from the pleural fluid as well as sputum. Adenosine deaminase levels from pleural fluid have been widely used as a biomarker for infection with high sensitivity. Values less than 15–40 U/L are generally felt to exclude tuberculous pleural effusions.

A 26 year-old male from Panama presented after discovery of a positive Tuberculin Skin Test upon enlisting with the United States Army. He was found to have a unilateral left sided pleural effusion without any parenchymal disease. Thoracentesis performed identified a lymphocyte-predominant, exudative effusion with low glucose levels narrowing the most likely diagnoses to mycobacterial infection, malignancy, or autoimmune etiology. Quantiferon gold testing was negative. Pleural cultures were negative for acid fast organisms on 2 separate occasions and polymerase chain reaction (PCR) testing of pleural fluid was negative for Mycobacterium tuberculosis DNA. Adenosine deaminase level was 8 U/L. He subsequently underwent pleural biopsy which showed multiple necrotizing granulomas. Given these biopsy findings, Mycobacterium tuberculosis being highly endemic in Panama, and positive PPD, the patient was started on appropriate therapy. Although patient had negative acid fast smears on 5 separate occasions, tuberculosis was eventually identified on a single acid fast sputum cultures confirming the diagnosis after therapy had been started.

This case illustrates the challenges in diagnosing tuberculous pleural effusion. Due to very poor sensitivity of pleural fluid cultures, many advocate for use of adenosine deaminase as a sensitive test to identify tuberculous pleural effusions. Using lower cutoffs for adenosine deaminase less than 15 U/L, multiple studies identify a negative predictive value of 100%. This patient demonstrates that even low cutoff values for adenosine deaminase still fail to adequately exclude a tuberculous pleural effusion. Recognition of false negatives is critical for appropriate diagnosis and management of this challenging clinical entity.

16 XX pelvis in an XY body

N Rus1*

MF Habib2

A Kamat1

J Kimbugwe1

T Naguib1

1Texas Tech Univ HSC Amarillo, Amarillo, TX

2Amarillo Cardiovascular Center, Amarillo, TX

Case report

Testicular cancer is the most common neoplasia in young men (90%) with ages ranging from 15–44 years of age. Germ cell-derived tumors are the most predominant. Screening examination is insufficiently used for early detection. Testicular cancer can cause developmental, endocrine and reproductive problems like precocious puberty, gynecomastia, or infertility. Pelvis feminization has never been reported with testicular cancers.

We report a case of an 18-year-old male with right-sided flank, found to have a right testicular mass on CT scan, with multiple retroperitoneal masses with compression of right ureter with secondary hydronephrosis, displacement of inferior vena cava and abdominal aorta, as well lungs and liver lesions. CT scan also showed a circular shaped-wide inlet pelvis with an obtuse angled pubis and an outwards-flared pelvic bone, consistent with gynecoid pelvis. Labs were remarkable for pancytopenia and elevation of the tumor markers alpha-fetoprotein, beta HCG and LDH. Liver biopsy confirmed metastatic yolk sac testicular tumor. The patient received chemotherapy followed by a partial orchiectomy and was discharged home with oncology and urology follow-ups.

Our patient’s right testicular tumor was missed due to his hesitation to report a testicular mass as well as the lack of the physician’s regular genital examination. In addition, we also report that pelvic feminization in our patient was directly linked to the testicular tumor. This radiological finding has never been reported before.

We believe that improving patient and physician education on genital exam as well as pelvis feminization along with precocious puberty and gynecomastia are valuable clinical tools to help detect testicular cancer early in order to reduce its mortality and morbidity. However, a retrospective radiological review of patients with testicular cancer is needed to establish a true association between testicular cancer and pelvic feminization.

17 A hop and a skip to quadriplegia

N Rus1*

MF Habib2

J Kimbugwe1

AA Kamat1

R Chandra1

1Texas Tech Univ HSC Amarillo, Amarillo, TX

2Amarillo Cardiovascular Center, Amarillo, TX

Case report

Spinal epidural abscess (SEA) is increasing in incidence, with a median onset age of 50 years and a maximum prevalence in males of 50–70 years of age. Early clinical recognition is often challenging with potential dramatic outcomes if not detected early. The classic diagnostic triad of fever, spine pain and neurological deficit is present only in 13% of patients, thus increasing the risk of a delayed diagnosis. Skip non-contiguous lesions represent 9% of SEA, with thoraco-lumbar region being the most common affected area.

We report a case of a 40-year-old female presenting with lower back pain and a history of methamphetamine abuse. Initial non contrast CT scan of the spine showed chronic degenerative changes and lumbar disk herniation. Neurological exam on admission was unremarkable except for altered mental status, attributed to drug overuse. Cultures showed MSSA bacteremia. During her hospital course patient rapidly developed quadriplegia, abdominal distension, and decreased rectal tone. MRI of spine with contrast showed skipped non-contiguous epidural abscesses in cervical and lumbar spine. Patient was treated with antibiotics and underwent staged drainage of epidural abscesses.

Skip noncontiguous SEA are rare

Early detection is challenging. To properly screen emergency department patients for SEA recognition of risk factors like IV drug use, alcoholism, diabetes, HIV infection, trauma, tattooing, acupuncture, contiguous bony or soft tissue infection may be more sensitive than the classic diagnostic triad of fever, back pain and neurologic deficit. One study concluded that physical signs may be very subtle, patients may be normothermic and have normal white count. Urgent surgery is more likely to get performed for patients with neurological deficit, while patients treated conservatively go undiagnosed and develop irreversible neurological deficit later. Having a high clinical suspicion of SEA in patients with drug use and early detection with an MRI-contrast of the whole spine to detect skip-lesions would result in early initiation of treatment, and in turn would reduce the morbidity and neurological deficits. Along with antibiotic treatment, early surgical decompression within 24–36 hours is essential for ensuring a deficit free recovery.

18 Spontaneous rupture of iliopsoas tendon in a morbidly obese woman

A Tanbir1*

A Bharadwaj1

R Murray2

R Bharadwaj1

1Texas Tech HSC-Amarillo, Amarillo, TX

2Northwest Texas Hospital, Amarillo, TX

Case report

Spontaneous iliopsoas tendon rupture is a rare cause of hip pain and overall prevalence is estimated to be less than 1%. Most common predisposing factors are older age, prolonged steroid use and chronic illness. This is a rare case report of spontaneous ilopsoas tendon rupture in a middle aged morbidly obese patient with multiple comorbid conditions.

Fifty three year old morbidly obese female (BMI-45) presented with atraumatic right hip pain for 2 weeks. Patient also had multiple comorbid conditions including DM, ESRD on hemodialysis and depression. Initial X-ray examination was negative. Later on MRI examination of the hip revealed rupture of iliopsoas tendon (figure: 1&2). Her pain and functional status improved with conservative management with minimal residual weakness at right hip flexors.

Abstract 18 Figure 1

MRI image showing absence of iliopsoas tenden at its distal attachment site on the lesser trochanter and surrounding edema MRI image showing absence of iliopsoas tenden at its distal attachment site on the lesser trochanter and surrounding edema

Abstract 18 Figure 2

MRI image demonstrating retracted iliopsoas tendon MRI image demonstrating retracted iliopsoas tendon

Discussion

Iliopsoas tendon rupture should be considered as an unusual cause of hip pain especially at risk population such as morbid obesity, older age, and patients with prolonged use of steroid or quinolones. Prompt diagnosis may lead to early recovery and better functional status.

Neonatal case reports

12:00 PM

Thursday, February 13, 2020

19 Hypothyroidism after percutanous patent ductus arteriosus device closure in an extremely preterm infant: possible role of iodinated intravenous contrast

M Piatek*

E Abu Jawdeh

M Hanna

D Schneider

J Smith

University of Kentucky, Lexington, KY

Background

Iodine-induced hypothyroidism has been described in preterm infants exposed to antiseptic agents and other iodine containing contrasts. Iodine induced hypothyroidism in preterm infants may be transient; however cases of persistent hypothyroidism requiring treatment until early childhood have been reported. Untreated hypothyroidism, even for a short period, can have detrimental effects on brain development and neurodevelopmental outcomes; especially in the preterm patient population. We are presenting a case of intravenous contrast induced hypothyroidism in an extremely preterm infant who underwent percutaneous device closure of a hemodynamically significant patent ductus arteriosus (PDA).

Case presentation

Our patient is an extremely preterm infant born at 27 weeks gestation with birth weight of 715 grams. Catheter closure of hemodynamically significant PDA was performed on day of life 24 (weight at procedure date was 900 grams). During the procedure, patient received standard intravenous contrast (iodixanol, contains 320 mg iodine/ml). The dose of intravenous contrast received is within accepted previously reported ranges. Prior to the procedure the patient had normal thyroid function on multiple state newborn screens. After the procedure the patient was found to have elevated Thyroid Stimulating Hormone (TSH) of 48.66 uIU/ml and low free T4 (thyroxine) of 0.5 ng/dL. Confirmatory repeat thyroid studies were obtained and patient was subsequently started on levothyroxine.

Discussion

To the best of our knowledge, this is the first report of contrast-induced hypothyroidism in extremely preterm infant undergoing percutaneous PDA device closure. As the use of percutaneous PDA device closure is more frequently utilized in extremely preterm infants, in comparison to surgical ligation, guidelines for screening thyroid function tests pre and post contrast requiring procedures may be warranted.

20 Acquired diaphragmatic hernia in a neonate after group B streptococcal sepsis

A Klinger*

JB Philips

University of Alabama at Birmingham, Vestavia Hills, AL

Introduction

Right-sided congenital diaphragmatic hernia (CDH) may have a delayed presentation. Initial symptoms appear similar to pneumonia with respiratory failure and may delay diagnosis. Association with early onset Group B streptococcal (GBS) sepsis and delayed onset of right CDH have been reported previously. This case describes a neonate with GBS sepsis and delayed right CDH and attempts to raise awareness of necessary precautions and features.

Case description

A male infant with birthweight of 3.465 kg was delivered at 39 weeks and 2 days to a GBS positive mother who was not treated prior to a precipitous vaginal delivery. The infant required escalation of respiratory support with intubation and mechanical ventilation after birth. He was started on Ampicillin and Gentamicin and initial blood cultures were positive for GBS. Initial chest X-rays (CXR) showed no infiltrates and normal lung patterns. His course was complicated by a 5-day course of inhaled nitric oxide for pulmonary hypertension. After treatment and resolution of pulmonary hypertension and sepsis, he failed multiple extubation trials. CXRs and a chest CT at 3 weeks showed right lung volume loss and atelectasis. Flexible bronchoscopy at 4 weeks showed mild bronchomalacia but no other abnormalities. A repeat chest CT and CXR, performed while the infant was off positive-pressure, revealed a right diaphragmatic hernia. At one month, the infant underwent thoroscopy and open repair, which revealed a type-C diaphragmatic hernia without a sac and liver adherent to the lung.

Discussion

Approximately 40 cases of late diagnosis of a right CDH occurring after GBS infection in neonates have been reported in literature. In this case, the initial CXR obtained soon after birth revealed no evidence of CDH that was found at 4 weeks. One hypothesis for late diagnosis proposes that GBS sepsis with pneumonia could cause necrosis and rupture of the diaphragm. However, there was no mention of necrotic features during surgical repair in this case. Another hypothesis posits mechanical ventilation increases intra-thoracic pressure and delays any evidence of the herniation. In this case, the hernia was only diagnosed after removal of positive pressure ventilation.

21 Elevated alkaline phosphatase levels associated with isolated spontaneous intestinal perforations in premature infants

RC Lynch1*

EM Bergner2

L Gollins3

AB Hair1

1Baylor College of Medicine, Houston, TX

2University of Oklahoma Health Sciences Center, Oklahoma City, OK

3Texas Children’s Hospital, Houston, TX

Purpose of study

Spontaneous intestinal perforation (SIP) is a common entity in the NICU leading to increased morbidity and mortality. At our institution, we noted elevated alkaline phosphatase (ALP) levels in premature infants with SIP. We aimed to investigate this relationship.

Methods used

Infants followed by the Texas Children’s Hospital NICU Intestinal Rehabilitation team from 2014–2017 were reviewed and cases selected based on diagnosis. Demographic data, clinical characteristics, feeding history, alkaline phosphatase levels, surgical intervention, and patient outcomes were recorded.

Summary of results

Seven premature infants with a diagnosis of isolated SIP were identified (See table 1 for clinical summaries). Mean ALP was 2170±2.8 IU/L prior to the date of diagnosis (n=2) and 1504.1±806.6 IU/L at the time of diagnosis. The values then normalized to 280.4±64.1 post-intervention (figure 1).

Abstract 21 Table 1

Clinical presentation of seven infants with SIP Clinical presentation of seven infants with SIP

Abstract 21 Figure 1

Alkaline phosphatase levels surrounding SIP Alkaline phosphatase levels surrounding SIP

Conclusions

Infants with isolated SIP appear to have significantly elevated ALP around diagnosis that rapidly normalizes after intervention. Further delineation of the use of ALP as a marker of intestinal inflammation or hypoperfusion injury could assist with early diagnosis and appropriate surgical management.

22 Rhizopus in a preterm infant: novel use of posaconazole

R Fatemizadeh1*

E Rodman1,2

B Shivanna1

D Dinu1

1Baylor College of Medicine, Houston, TX

2Texas Children’s Hospital, Houston, TX

Background

Rhizopus species cause rapidly progressive and life-threatening infections. Treatment involves aggressive debridement and combination systemic antifungal therapy. Posaconazole is a triazole with activity against Rhizopus, but data on its use and pharmacokinetic profile in preterm infants is scarce.

Case

The male patient was born at 24 4/7 weeks and 585 g to a 32-year-old mother with severe pre-eclampsia and carcinoma of unclear etiology (carboplatin/paclitaxel given 5 days before delivery).

On DOL 14, an abrasion on the back near an adhesive probe was noted that evolved into a crusted eschar by DOL 16. Skin biopsy cultures resulted with ‘rare mold’ on DOL 17. The patient was started on IV amphotericin B deoxycholate and micafungin based on case reports of synergy in Mucor species. Lumbar puncture was deferred given the location of the lesion, but blood cultures were negative.

On DOL 20, the biopsy culture resulted with Rhizopus species, so debridement of the lesion was performed on DOL 22. The pathology showed angioinvasion, so coverage with amphotericin B and posaconazole was selected as optimal therapy for disseminated disease.

Enteral posaconazole suspension at 6 mg/kg every 8 hours was initiated on DOL 34 when enteral feeds were tolerated. A trough obtained at steady state after 7 days returned at 0.5 mcg/mL (goal >0.7 mcg/mL), so the dose was increased to 7 mg/kg every 8 hours. A steady state level on the new dosing achieved a therapeutic trough of 0.9 mcg/mL. At this time, liver enzymes became elevated, so micafungin was discontinued with return of liver function to baseline. Posaconazole was otherwise well tolerated.

The patient completed 7 weeks of amphotericin B with 4 weeks of micafungin followed by 3 weeks of posaconazole. The patient has not had any recurrence, and the back excision is healing well. Abdominal ultrasound, ophthalmologic exam, and brain MRI showed no evidence of infection.

Conclusion

This is the first report of posaconazole use in a preterm neonate with Rhizopus. This extremely premature infant was successfully treated with excision and combination therapy of amphotericin B and micafungin followed by enteral posaconazole. Posaconazole was well tolerated and may be considered for therapy in future neonatal Rhizopus infections.

23 The echoes of the wolff- uncommon and irregular arrhythmias in a case of neonatal wolff parkinson white

IE Sierra Lobo*

A Shah

SP Behere

Oklahoma University Health Sciences Center, Oklahoma City, OK

Case report

The patient is a 10 mo male with multiple VSDs. He presented at 2 months with congestive cardiac failure, ECG showed Wolff Parkinson White (WPW) pattern. He had pulmonary artery banding and post operatively developed regular broad complex tachycardia as well as regular narrow complex tachycardia, unresponsive to adenosine. He was managed with amiodarone for the next 4 months. Two morphologies of delta wave were seen variably at different times (figure 1). After his VSD repair at 9 months, he developed narrow complex tachycardia requiring cardioversion. He was discharged on propranolol.

During follow up he presented with irregular broad complex rhythm- likely antidromic echo beats. The arrhythmia persisted despite high dose of propranolol, and flecainide was added.

He subsequently had sinus rhythm with intermittent preexcitation and no evidence of reentrant echo beats.

Discussion

This was a rare case of narrow complex tachycardia (orthodromic reentrant tachycardia - ORT) and broad complex tachycardia (antidromic reentrant tachycardia - ART) in WPW. ART occurs with conduction of the sinus impulse via the accessory pathway in an antegrade fashion, and then retrograde via the normal AV node creating a reentrant circuit. ART is rarer than ORT.

The subsequent irregular grouped beating was also unusual. Single and double reentrant echo beats suggested the need for further rhythm control.

The presence of two different preexcitation patterns at baseline, the lack of response to adenosine, and the antidromic reentrant echo beats suggest presence of multiple accessory pathways.

Since flecainide (sodium channel blocker) is more selective for myocardium, it can slow accessory pathway conduction especially adjunctive to a beta blocker. The patient had a good response to flecainide.

24 Management of a PIK3CA-related overgrowth spectrum disorder in a preterm infant

A Moen1*

A Piazza1,2

C Ocampo3

1Emory University, Atlanta, GA

2Children’s Healthcare of Atlanta, Atlanta, GA

3Emory University School of Medicine, Atlanta, GA

Case report

Congenital overgrowth syndromes constitute a variety of diagnoses with many potential genetic mutations but often with similar clinical presentations. One category of malformations related to anomalies within lymphatic and low flow vasculature, along with the presence of overgrowth, is due to PIK3CA gene mutations, termed PIK3CA-Related Overgrowth Spectrum (PROS) disorders. This gene undergoes a gain of function mutation, leading to activation of the PIK3-AKT and mTOR pathway, followed by upregulation of cell proliferation. Sclerotherapy, mTOR inhibitors, and surgical debulking are treatment options. We present a case of a preterm infant with an extensive PROS disorder to review management and outcomes in this rare diagnosis.

This is a 31 6/7 week female infant born via C/S with prenatal diagnosis of suspected lymphatic malformation on fetal MRI. Multiple anomalies appreciated at delivery, most notably a massive cystic lesion encompassing the entire left arm and chest, and macrodactyly. Full-body MRI and chest MRA/MRV described extensive multi-cystic lesions consistent with lymphatic malformation with a possible venous component, phenotypically consistent with a PROS disorder. She underwent doxycycline sclerotherapy and drainage of cystic lesions and was treated with sirolimus. An overgrowth genetic panel encompassing PIK3CA, PTEN and AKT1 genes was negative. She had a normal microarray. The malformation progressively became smaller and firmer.

There are multiple overgrowth syndromes associated with PIK3CA gene mutations. Genetic testing to detect mutations in the PIK3CA gene may confirm the diagnosis, but often yields false-negative results given that most presentations are somatic mosaics and not detectable. A recent systematic review evaluating doxycycline sclerotherapy treatment outcomes in pediatric lymphatic malformations showed an 84% success rate with complete resolution. Sirolimus has also successfully treated complex lymphatic malformations in neonates. Surgical debulking is only recommended in extreme cases given risk for significant intraoperative bleeding. While patients may need frequent or lifelong management of cutaneous overgrowth, overall outcomes are related to co-morbidities.

25 Isolated right superior vena cava drainage into the left atrium: an uncommon cause of persistent hypoxia in a neonate

TD Smith1*

R Gulati1,2

M Siow2

L Batten1,2

1University of South Alabama Children’s and Women’s, Mobile, AL

2University of South Alabama, Mobile, AL

Case report

A 33 week gestation female was transferred to our institution for respiratory distress and given surfactant via endotracheal tube, but remained hypoxic with SpO2 70–80% on room air. A chest x-ray (CXR) showed right upper lobe atelectasis but a normal mediastinal silhouette. An echo on DOL 1 showed a patent foramen ovale (PFO) shunting left to right, mild tricuspid regurgitation, and a small patent ductus arteriosus also shunting left to right.

Atelectasis resolved but patient remained hypoxic without tachypnea. A limited echo on DOL 10 showed no evidence of pulmonary hypertension, so a cardiology consult was requested. After being unable to identify the right superior vena cava (RSVC) drainage on review of the initial echo, a contrast echo via a vein in the right hand demonstrated contrast entering the left atrium (LA) and left ventricle (LV); it only entered the right atrium (RA) via a PFO. Infant was weaned off oxygen, tolerated full feeds, and held SpO2 >80%. Infant was discharged at 3 weeks with a plan for surgery after 1 year.

Discussion

The hemodynamic effect of this anomaly is approximately one-third of systemic venous return causing left-sided volume overload with resulting hypoxia and the possibility of brain abscesses and strokes. The first case of a RSVC draining into LA was reported in 1956 in a 10 year old female who presented with cyanosis and dyspnea with normal CXR and left ventricular hypertrophy on ECG.

Through our review, we found only 42 cases of RSVC to LA without other cardiac anomalies. Of these, 31 (74%) were diagnosed prior to 19 years of age with 8 (19%) prior to 1 month of age. 25 patients were female and 16 were male. 5 patients suffered a brain abscess. 3 patients had only idiopathic hypoxia. Additionally, 8 patients had cyanosis without other complaints. Other patients had dyspnea, cardiomegaly, and polycythemia. A contrast echo was used in 21 patients for initial diagnosis. This case emphasizes the importance of recognizing this anomaly as an infrequent cause of persistent unexplained hypoxia in the neonatal period.

26 Clinical course of an infant with atelosteogenesis type one

L Weaver*

A Al-Beshri

JB Philips III

University of Alabama, Birmingham, AL

Introduction

Atelosteogenesis type 1 (AO1) is a skeletal dysplasia notable for incomplete ossification of the bones and resultant organ pathogenesis. It is one of a number of skeletal dysplasia characterized by a pathogenic variant of the FLNB gene. The gene codes for Filamin B an essential protein for cytoskeletal structuring and endocardial ossification. The phenotype ranges from mild to perinatally lethal. The disorder is very rare and its prevalence is unknown. Most mutations occur de novo but can be inherited from mildly affected or asymptomatic parent due to somatic mosaicism. We report on a male infant with AO1 who survived the neonatal period.

Presentation

Mother presented at 29 weeks gestation age (wga) with ultrasonographic evidence of polyhydramnios and micromelia. Mother was lost to follow up with representation for preterm vaginal delivery at 35.2wga. The infant had rhizomelia without stippling, tapering of distal humerus, absent fibula, shortened ulna, coronal clefting, platyspondyly, deficient ossification of the tarsal and carpal bones, and dislocation of proximal radii. He required escalation of respiratory support initiated at birth and ultimately transitioned to tracheostomy tube and ventilator dependence. He had a mixed apnea and pulmonary hypoplasia. Parents withdrew care at 1 year of age. Diagnosis of a de novo heterozygous pathogenic FLNB gene variant was made via exome slice sequencing of infant and parents. Despite this information, it was a challenging diagnosis given the clinical overlap of FLNB-related disorders, however, based on patient’s phenotype, the diagnose of atypical AO1.

Discussion

With a potential diagnosis of skeletal dysplasia it is important to distinct perinatally if mutation is lethal or not, however, despite using various clinical and radiographic criteria, this remains challenging. The number of skeletal dysplasias is growing rapidly thanks to the advancement in genetic testing technology, and over 450 different types currently exist. A detailed description of this patient should contribute to medical literature to aid in ongoing differentiation of the dysplasia types. Early prenatal diagnosis helps to guide genetic and palliative discussions.

27 An atypical presentation of waardenburg-shah syndrome with an incidental EDNRB mutation

J Butler1,2*

E Bassig1

1Phoebe Putney Memorial Hospital, Albany, GA

2Medical College of Georgia, Augusta, GA

Case report

Waardenburg syndrome is a group of four separate congenital neurocristopathies characterized by sensorineural deafness, characteristic pigmentation defects of the skin, hair, and iris, dystopia canthorum, and other defects of neural crest cell-derived tissues. Each of the four types of Waardenburg syndrome have their own diagnostic criteria with a few commonly mutated genes shared within each type. We report a case of a preterm newborn female presenting with swallowing difficulties, hypothyroidism, and failure to thrive who was subsequently discovered to have a mutation in the EDNRB gene which is commonly associated with Waardenburg syndrome type 4. This type, also called Waardenburg-Shah syndrome, presents with the same distinctive features of classic Waardenburg syndrome with the addition of gastrointestinal malfunction – most notably Hirschsprung’s disease. She later developed atypical facial abnormalities but lacked the characteristic hypopigmentation defects such as a white forelock or brilliant blue eyes. There was also the notable absence of any sensorineural hearing loss. She did, however, develop chronic constipation that begs further evaluation for possible intestinal motility defects. As of this writing, she is followed by the neurodevelopmental clinic in anticipation of expected neurodevelopmental delay. Ultimately, the patient’s presentation reinforces the phenotypic heterogeneity and variable expressivity associated with Waardenburg Syndrome. The goal of this report is to issue a challenge for the need for evaluation of diagnostic criteria, expansion of the genetic etiologies, and increased prognostic prediction and ultimate prevention in those with Waardenburg syndrome. This patient is an indication that well-established syndromes may have an increased prevalence than previously established due to patients with subclinical manifestations.

28 Acetazolamide, is it a reason for neonatal metabolic acidosis?- A case report

M Mansour1*

T Le2

S Suchomski1

1University of Florida- College of Medicine Jacksonville, Jacksonville, FL

2Naval Hospital, Jacksonville, FL

Case report

A term female was born to a mother who had idiopathic intracranial hypertension on 2,000 mg Acetazolamide twice daily. Routine cord blood gas showed pH of 7.22 and BE of -11.4. A repeat CBG had a PH of 7.18 and BE of -11.0 denoting metabolic acidosis. Complete lab work-up showed high anion gap metabolic acidosis. The infant had a benign physical exam at birth.

Work up for metabolic acidosis was negative for neonatal sepsis and AKI. Blood sugar and ammonia were normal excluding inborn errors of metabolism. Blood culture was negative. The infant was started on IV fluids including sodium acetate, potassium acetate, and calcium gluconate to correct metabolic acidosis. Over 48 hours, acidosis improved. The infant was feeding formula without any difficulties. We determined that this electrolyte imbalance was due to the intrauterine exposure if acetazolamide since sepsis and metabolic syndromes were excluded. The infant was discharged home at 48 hours of age. Well baby visits documented normal physical exam and development.

Discussion

In our case, the patient appeared well on clinical exam but laboratory workup showed high anion gap metabolic acidosis with mild hypocalcemia. A diagnosis of intrauterine exposure to Acetazolamide induced metabolic acidosis was made.

According to the US Food and Drug Administration, acetazolamide is classified as a class C drug. This indicates that animal reproduction studies have shown an adverse effect of acetazolamide on the fetus, but there are no well-controlled studies in humans. Animal studies have demonstrated that acetazolamide given to pregnant rats caused limb defects. (1) There are no enough sample size human studies done on intrauterine exposure of Acetazolamide. Few Case reports describe Renal tubular acidosis type 2 like picture (2), hypocalcemia, hypomagnesemia (3), limb defects (4), Teeth agenesis (3,5), and teratoma (6).

Conclusion

As a class C drug, acetazolamide should be prescribed only if the potential benefit justifies the potential risk to the fetus. This case draws attention to the neonatal effects of intrauterine acetazolamide exposure. More research and case reports are needed to investigate the long-term sequela from exposure.

29 Hepatic abscess with ascitis in premature infant

A Akhtar*

M Herbert

A Patra

University of Kentucky, Lexington, KY

Case report

Hepatic abscess (HA) related to malpositioned umbilical catheter is a rare but increasingly recognized cause of morbidity and mortality in premature neonates. We report an unusual case of hepatic abscess associated with ascites that presented remotely from umbilical catheterization in a growth restricted premature female infant born at 28 weeks with birth weight 450 g. Infant had unremarkable initial neonatal course requiring noninvasive continuous positive airway pressure (CPAP). Optimally placed umbilical venous catheter (UVC) was removed on day of life (DOL) 4 and replaced by peripherally inserted central catheter in the lower limb. Infant developed thrombocytopenia followed by clinical septic shock (blood culture negative) on DOL 14. Heterogeneous lesion in the liver measuring 1.6 × 1.4 × 1.4 cm with a hypoechoic periphery and circumferential blood flow consistent with HA and patent portal vein noted on ultrasound. Infant developed ascites requiring therapeutic paracentesis which may be secondary to lymphatic obstruction from mass effect of HA or inflammation related. Serum ascites albumin gradient was low indicating portal hypertension as unlikely cause of ascites. HA treated conservatively with 28 days of broad spectrum intravenous antibiotics including 1 week of fungal coverage. HA size was monitored weekly by ultrasound during treatment. Ultrasound at 46 weeks corrected age showed complete resolution of HA.

Abstract 29 Figure 1

Heterogeneous nonvascular hepatic lesion measuring 1.6 × 1.4 × 1.4 cm Heterogeneous nonvascular hepatic lesion measuring 1.6 × 1.4 × 1.4 cm

Abstract 29 Table 1

Peritoneal fluid results at DOL12 Peritoneal fluid results at DOL12

30 Sirolimus for kaposiform hemangioendothelioma in a neonate

TB Cabrera*

AL Speer

MR Greives

DA Goff

NM Menon

EW Reynolds

University of Texas Health Science Center @ Houston, Houston, TX

Case report

Kaposiform Hemangioendothelioma (KHE) is a vascular tumor often complicated by Kasabach-Merritt Phenomenon (KMP). We present a baby who developed complications during treatment of KHE.

Case

AR required delivery at 34 weeks for IUGR, dilated vena cava and reverse end-diastolic flow. He had a vascular tumor on the RLE. Coagulation labs showed KMP. He required many transfusions over 2 weeks and developed Grade 4 IVH. MRI found vascular channels in the mass without fascia involvement. Biopsy confirmed KHE. Sirolimus was started (0.8 mg/m2BID). He developed anasarca and acidosis requiring mechanical ventilation. DOL 6 echo showed EF=11%. It improved with fluid restriction and medications by DOL13. Sirolimus levels peaked at 48ng/mL (target=10–15ng/mL) requiring interruption of therapy. A lower dose was started on DOL 24. Levels were within range for the rest of treatment.

Discussion

KHE can be associated with KMP. KHE is distinct from arterio-venous malformation. Historically, treatment was surgical excision. Current therapy is vincristine/steroids requiring central IV access. Sirolimus is used to treat vascular tumors with IV or PO options.

Our case highlights issues arising while treating neonates with KHE. Physicians should have a high index of suspicion for KMP in patients with KHE. Check platelet and coagulation studies early. Patients with KMP may require large volumes of blood products. Monitor strict I/Os. Watch for heart failure. The usual dose of sirolimus may be too large for neonates. Levels should be monitored until a steady-state in the target range is achieved.

Conclusion

Patients with KHE can have complications from both the disease and treatment. Attention to platelets, coagulation studies, volume status, heart function and drug levels are required for successful treatment.

31 What’s the defect?

CK Glendye*

N Dankhara

S Ramarao

R Moore

M Famuyide

University of Mississippi Medical Center, Jackson, MS

Case report

We report a case of a preterm female born via vaginal delivery. At delivery, the neonate was noted to have a large right sided abdominal mass. Examination showed a large right sided para umbilical mass covered with discolored skin. The mass was minimally reducible and soft with palpable loops of bowel. Initial abdominal X-ray demonstrated a large herniation of the bowel to the right of midline with subsequent imaging showing dilatation of bowel loops concerning for bowel obstruction. Abdominal US confirmed herniation of multiple loops of bowel through a large anterior abdominal wall defect to the right of the umbilicus. Pediatric surgery was consulted for evaluation. Surgical exploration of hernia showed meconium staining and in utero perforation with obstruction in the mid jejunum, 1 cm of jejunum was resected and the hernia was repaired. Intestinal biopsy taken from the ileum demonstrated normal pathology. Post-op course was complicated by feeding intolerance and continued gaseous distention and paucity of stool. Barium enema showed a small caliber colon consistent with disuse.This defect is likely an isolated defect due to vascular disruption and similar to gastroschisis. Abdominal wall defects occur in approximately 4 in 10,000 live births. Although rare, case reports of para-umbilical wall hernias exist and should be considered in patients with abdominal wall defects that don’t fit the standard classification for gastroschisis or omphalocele.

Pediatric clinical symposium

12:00 PM

Thursday, February 13, 2020

32 Late-onset necrotizing enterocolitis in a healthy, full-term neonate

HS Rose*

A Monroe

O Titus

I Kane

Medical University of South Carolina, Charleston, SC

Case report

A 24-day-old term, male infant born via uncomplicated C-section to a G7P7 mother with gestational diabetes presented to the emergency department for evaluation of emesis. The infant had new-onset non-bloody, non-bilious emesis for one day as well as non-bloody loose stools. The infant was ill-appearing with a pulse 190, blood pressure 55/25, respiratory rate 70, and saturation 94% on room air. The infant’s abdomen was notably distended, tender, and tense. The patient was placed on oxygen and resuscitated with 40 ml/kg of normal saline. Empiric antibiotics (pipercillin/tazobactam) were started to cover for common causes of neonatal and intraabdominal sepsis. Labs were remarkable for Hgb 7.7 gms/dL, Hct 22.7%, WBC 5.97 k/cumm, and platelets 83 k/cumm. CMP was remarkable for K 6.1 mmol/L, anion gap 17 mmol/L, BUN 25 mg/dL, and Cr 0.8 mg/dL. A venous gas showed pH 7.31, PCO2 35, PO2 31, Bicarbonate 18, and lactate 6.3 mmol/L. Blood and urine cultures were drawn, but CSF studies were deferred given the patient’s clinical instability. Respiratory and gastrointestinal viral PCR assays were both obtained and were ultimately negative. An abdominal radiograph showed diffuse gaseous distention of the bowel without evidence of obstruction or pneumatosis. Pediatric surgery was consulted and performed a digital rectal exam which produced a large volume loose stool that was hemoccult positive. A nasogastric tube was placed for bowel decompression and intravenous fluid resuscitation was continued. The child was transferred to the PICU where he was intubated and started on pressor support. The most likely diagnosis at that point was felt to be malrotation with volvulus but an upper GI study was non-diagnostic. Given the patient’s rapid decompensation with continued concerns for volvulus, surgery performed an emergent exploratory laparotomy which revealed global ischemia of the small bowel consistent with diffuse necrotizing enterocolitis (NEC). Over the next 48 hours the child returned to the OR for two additional laparotomies without improvement in bowel perfusion. The infant transitioned to comfort care and died on hospital day 6. This case highlights the unique presentation of NEC in an otherwise healthy term neonate and the challenge of managing undifferentiated abdominal pathology in the neonatal period.

33 Timing is everything: a suspected case of trimethoprim-sulfamethoxazole induced sepsis

EM Karolczuk*

KN McIntyre

SM Thompson

DR Wells

Le Bonheur Children’s Hospital, Memphis, TN

Case report

A previously healthy 11-year-old female from Mississippi presented from an outside hospital with hypotension, fever, and leukocytosis. She had a 2-week history of worsening flank pain that started in the area of a suspected insect bite. The day of admission she presented to her PCP for worsening erythema and pain, and was prescribed Trimethoprim-Sulfamethoxazole (TMP-SMX). 3 hours after taking her first dose, she developed vomiting and fever. At an outside hospital she was found to be hypotensive and febrile with leukocytosis, with an elevated lactate and CRP, and was transferred to our PICU. She remained hypotensive needing fluid resuscitation and pressors, and was intubated for hemodynamic instability. She improved clinically and was discharged 5 days later with a diagnosis of culture negative sepsis vs loxoscelism on doxycycline for tick-borne illness coverage and clindamycin for concern of superadded bacterial infection of the insect bite. Subsequently, the wound grew MRSA (sensitive to doxycycline) but resistant to clindamycin, and her PCP switched her to TMP-SMX. Shortly after the first dose of TMP-SMX (4 hours), she was found to be febrile and severely hypotensive and was readmitted to the PICU. The wound on her back initially thought to be the source of infection was non-tender and significantly decreased in size. Due to the temporal relationship of TMP-SMX with fever and drastic hypotension, we suspect that her clinical presentation was related to acute drug toxicity which may mimic culture negative sepsis. Although there have been 2 reported cases of TMP-SMX induced sepsis-like syndrome in adults, to our knowledge this is the first report of this clinical presentation in a child.

34 Renal failure following an acute gastrointestinal illness: a case of P-ANCA vasculitis

C Ali*

N Mathews

University of Oklahoma Health Sciences Center, Oklahoma City, OK

Case report

Our patient is a previously healthy 11 year old Hispanic female who presented with fatigue, anorexia, generalized weakness, and oliguria. Two weeks prior, she acquired an acute gastrointestinal illness with fever and diarrhea while vacationing in Mexico. She was prescribed Amoxicillin and NSAIDs. Physical exam was significant for edema. Her initial labs were suggestive of acute kidney injury secondary to glomerulonephritis: WBC 11.8, Hgb 6.6, Plt 486, BUN 137, Creatinine 10.9. Hemolytic Uremic Syndrome was ruled as a less likely etiology. Treatment was initiated with high dose corticosteroids and hemodialysis while awaiting renal biopsy. P-ANCA titer was positive. Anti-proteinase 3 and myeloperoxidase were negative. Renal biopsy findings were consistent with ANCA vasculitis with crescentic glomerulonephritis and chronicity. Due to persistent cough, CT chest was performed and significant for pulmonary hemorrhage. Solumedrol pulse, plasma exchange, and Rituximab were initiated. After her second Rituximab dose, she had worsening cough and hypoxia. She developed hemoptysis after readmission. She was transferred to our PICU for acute hypoxic respiratory failure and hemorrhagic shock requiring massive transfusion protocol requiring progression to veno-venous extracorporeal membrane oxygenation. A week later, she was extubated, stabilized on room air, and discharged in fair condition after a few days. She remains on hemodialysis.

ANCA-associated vasculitides (AAV) have multi-organ involvement with necrotizing small-vessel vasculitis. These diseases can be severe and life-threatening if not appropriately managed. Prompt recognition and early treatment are important. The diagnosis of AAV is strongly suggested by a positive ANCA test; however, biopsy of the affected organ is the most definitive method to establish a diagnosis. Despite treatment, AAV carries considerable disease-related morbidity and mortality mainly due to progressive renal failure or aggressive respiratory involvement. Our case is unique as pediatric AAV are rare, and her rapid progression highlights the importance of thorough workup and monitoring.

35 An unusual overdose causing methemoglobinemia and acute kidney injury in a 14 year old girl

CG Lares Romero1*

A Lisenbe2

B Dillard1

1University of Mississippi, Ridgeland, MS

2University of Mississippi Medical Center, Jackson, MS

Case report

Acute poisoning remains an important reason for admission in the pediatric emergency department. Often, determining the causative agent to guide the patient’s care can be very challenging. We present a 14 years old female who came to the pediatric emergency due to diffuse abdominal pain and non-bilious emesis along with red urine 12 hours after taking a large amount of an unkwon medication at home as a suicide attempt. On arrival she had low oxygen saturations (88%) and dusky/cyanotic nail beds with an otherwise reassuring physical exam. Despite oxygen therapy, oxygen saturation remained low. Since the lungs were clear and her cardiac exam/perfusion were normal, the sat probe was moved to the ear lobe where oxygen saturation was normal. Workup revealed elevated methemoglobin (28.2%), lactate (2.5), creatinine (1.64), indirect bilirubin (1.81) and LDH (750). Urine drug screen, Acetaminophen, Aspirin and alcohol levels were all negative. Chest radiography, electrocardiogram and renal ultrasound were normal as well. After a very thorough investigation (including a home search for empty bottles) it was discovered that the patient had taken a large amount of Clarithromycin and Pyridium. The patient’s clinical findings along with her Methemoglobinemia and acute kidney injury, lead the team to identify Pyridium as the causative agent. The patient was subsequently treated with Methylene blue, Acetylcysteine and aggressive fluid rehydration. Her creatinine peaked at 2.54 but normalized after interventions. She was ultimately medically cleared and transferred to a Psychiatric facility for treatment of her depression and multiple suicidal attempts.

A variety of case reports describe the adverse effects caused by Pyridium. Acute kidney injury, Methemoglobinemia and hemolysis are common presentations. Almost all of these cases involve adults with pre-existing renal disease using therapeutic doses. Pyridium overdose is very rare. Our goal is to increase awareness of the potential toxic effects of this over the counter medication, as well as, to highlight the importance of conducting a thorough investigation towards finding the causative agents in pediatric drug intoxications.

36 Group B streptococcus: a culprit in more than just infants

A Meraj*

A Wahba

A Balachandran

J Hsu

University Of Texas Health Science Center Houston, Houston, TX

Case report

Introduction: Group B Streptococcus (GBS), S. agalactiae, is a bacterium often screened for in pregnant women and associated with neonatal infections. However, GBS disease is also rising among nonpregnant adults, especially among immunocompromised patients. The mean age of nonpregnant adults with invasive GBS disease is 60 years, and the associated mortality rate is 25%. It can present as skin and soft tissue infection, osteomyelitis, pneumonia, urosepsis, and meningitis. There is very limited data on GBS disease occurring in the pediatric population past the infancy stage.

Case presentation

A 16 yr old African American male with history of fibroma of the right knee presented with 3 week history of right foot and knee pain that started after a febrile illness. Pain was severe and limited his ability to walk. Due to persistent right knee swelling, MRI of the knee was done. Lytic lesions were noted on imaging, so bone biopsy and knee aspiration were subsequently performed. He underwent debridement and irrigation of right knee in the operating room and then started on intravenous (IV) antibiotics. Joint fluid culture grew Group B Streptococcus. Bone biopsy showed acute on chronic osteomyelitis with infiltration of histiocytes and plasma cells. He was discharged on 4 weeks of IV ceftriaxone followed by 4 weeks of amoxicillin.

Conclusion

While the incidence of GBS infections is rising, the percentage of those in non-pregnant adults who present with joint infections is 5.4%, with few cases reported in children greater than 2 years of age. GBS osteomyelitis most often occurs by contiguous spread or direct inoculation. Patients at increased risk for GBS include those with underlying medical conditions, immunocompromised states 10%, diabetes 41%, cardiovascular disease 36%, and underlying malignancy 17%. There is risk for recurrent infection in 4.3% of survivors. Given that it is an unusual pathogen for the adolescent presenting with osteomyelitis, pediatricians should rule out any underlying comorbidity that may have increased their patient’s chance of developing the disease. Since our patient had presence of lytic lesions with histiocytes on bone biopsy, he was referred to oncology for further workup of Langerhans histiocytosis.

37 Tricyclic antidepressant induced brugada pattern

CJ Sang*

YR Lau

University of Alabama at Birmingham, Birmingham, AL

Case report

16-year-old male presents with encephalopathy after suicide attempt. Toxicology labs notable for amphetamines, opiates, THC, and TCAs. Initial ECG was normal. Repeat ECG two hours after admission showed Brugada Type 1 pattern in V2 as did repeat ECG at eight hours (though in V3). Patient had a history of two syncopal episodes over the prior two months, with a history most consistent with postural orthostatic tachycardia syndrome (often a diagnosis of exclusion). Genetic testing showed variation of uncertain significance in the TRPM4 gene. An echocardiogram was normal. Given diagnostic uncertainty, an EP study was performed with no inducible arrhythmia. He was therefore classified as having Brugada pattern rather than Brugada Syndrome.

Discussion

Sudden cardiac arrest (SCA) is an uncommon phenomenon in patients with a structurally normal heart, and the extensive differential diagnosis includes rare diseases, such as Brugada Syndrome (BrS). A hallmark of BrS is an ECG pattern of pseudo-right bundle branch block with ST-elevations in V1-V3. Diagnosis is challenging when a patient has an ECG with Brugada pattern Type 1 without significant clinical history, as this alone is not sufficient to support the diagnosis of BrS. The most recent 2013 HRS/EHRA/APHRS consensus statement requires either clinical symptoms with Type 1 Brugada ECG pattern, or the ECG pattern with additional clinical findings including 1st degree AV block with left axis deviation, atrial fibrillation, ventricular refractory period <200 ms with HV>60 ms. EP testing is not routinely indicated in patients with Brugada pattern EKG without associated clinical features. However, in the presence of equivocal symptoms such as a history of syncope, an EP study can help determine if the patient meets criteria for BrS and if ICD placement is advisable. In our patient, the EP findings would not support the diagnosis of BrS. TCA’s represent a known cause of inducing Brugada pattern on ECG due to their interaction with sodium currents. It is important to recognize the appropriate ECG findings and diagnostic criteria necessary to diagnose BrS.

38 A lapse in differential: wrong place, wrong time

AN Medine1*

N Marak1

A Byrd1,2

MH Roy1,2

1Our Lady of the Lake Children’s Hospital, Baton Rouge, LA

2Our Lady of the Lake Pediatric Residency Program, Baton Rouge, LA

Case report

5 year old male presented to the emergency department due to intermittent cramping abdominal pain for 1 week. Associated symptoms included watery stools and non-bloody, non-bilious emesis. His abdomen was diffusely tender to deep palpation with no guarding or rebound tenderness. Work-up included a normal abdominal x-ray. Parents gave a history of recent constipation and withholding behavior.

Oral polyethylene glycol regimen was started and patient received an enema. The enema lead to partial rectal prolapse that spontaneously reduced. CT showed colo-colonic intussusception involving the left colon without obstruction or identifiable mass. Barium enema reduced the intussusception and revealed a filling defect at the splenic flexure. Pain returned and ultrasound confirmed recurrent intussusception. Colonoscopy, EGD, and laparoscopy did not reveal a lead point. Exploratory laparotomy discovered a ganglioneuroma, a rare slow growing, benign tumor.

80–90% of intussusception cases present in children under two years old, and 90% of cases are ileo-colonic. Intussusception is largely idiopathic, with pathologic lead points present in only 25% of cases. Our patient was at higher risk for delayed diagnosis due to atypical age range, with colo-colonic intussusception, caused by a lead point not visible on CT scan.

Abstract 38 Figure 1

Colo-colonic intussusception involving the left colon Colo-colonic intussusception involving the left colon

39 All that scales is not ringworm

C Marbrey*

E Aitken

MN Frascogna

University of Mississippi Medical Center, Jackson, MS

Case report

Tinea capitis is a common pediatric condition characterized by hair breakage, lymphadenopathy (LAD) and scaly scalp lesions. Even routine treatment requires weeks of antifungal therapy. We present a case involving a 17-month-old healthy male who initially presented to his pediatrician with what seemed to be a stubborn case of tinea capitis, which despite several months of treatment failed to resolve. He was transferred to the pediatric emergency department (PED) for severe, worsening scalp lesions and LAD, with new onset fever and anemia.

Seven months prior to presentation, the patient was started on a prolonged course of Griseofulvin for presumed tinea capitis. Over the next 6 months he developed left post auricular and large right preauricular swelling, worsening scalp lesions and was ultimately hospitalized for intravenous (IV) antifungal, antibiotic and steroid therapy. His scalp had good response to IV therapy, however shortly after discharge the lesions returned. A few days prior to arrival in the PED, he developed fever to 105 and increased LAD. He was taken to an outside hospital where laboratory tests showed an elevated white blood cell count (15), anemia (8.5), normal platelets (445) and was transferred.

In the PED, he was afebrile and vitals were stable. He had thick scaly plaques throughout his scalp, swelling with tender fluctuance to the left post auricular area and swelling with overlying bruising and petechiae to the right preauricular area. He also had bilateral otitis media. Ultrasonography was concerning for bilateral, complex head/neck abscesses. Further investigation with computed tomography revealed aggressive, osseous destruction of the bilateral temporal bones with invasion into the right zygoma and LAD, concerning for Langerhans Cell Histiocytosis (LCH). Lung nodules were also noted. The diagnosis of LCH was confirmed with biopsy of the head/scalp lesions and he was started on chemotherapy.

Treatment remains ongoing with resolution of the lung nodules and overall good response to therapy. LCH is an incredibly rare childhood illness with a poorer prognosis in children under 2 years of age. This case highlights the varying presentations of this rare illness, as well as our own anchoring biases that can lead to delays in diagnoses and potentially dangerous therapies.

40 Pneumonia with effusion? Guess again!

T Le1*

A Dixon1

A Wright2

R Warrier2

1Tulane University, New Orleans, LA

2Ochsner Medical Center, Jefferson, LA

Introduction

Pleural effusions are uncommon in the pediatric population. Fifty to seventy percent of pleural effusions are caused by infectious etiologies: pneumonia, lung abscesses, or bronchiectasis. The incidence of pleural effusions associated with pneumonia has been increasing, with a current estimated incidence of 3.7 per 100,000 children.

Case summary

This case describes a 19-month-old African American female with a history of recent influenza infection who developed respiratory distress. She was admitted to the pediatric intensive care unit due to hypoxic respiratory failure. Initial labs showed evidence of acute liver failure with significant elevations of her liver enzymes and an elevated INR at 2.3. A chest x-ray (CXR) demonstrated a right upper lobe pneumonia and a right-sided pleural effusion. A chest tube was placed and revealed sanguineous drainage. The patient was presumed to have a pleural effusion and acute liver failure secondary to sepsis as her blood cultures were positive for GAS. However, upon review of her initial chest x-ray, she had a displaced fracture of the posterolateral right eighth rib. Both the pleural effusion and the acute liver failure were determined to potentially be caused by blunt trauma. This was concerning for non-accidental trauma (NAT), so a skeletal survey and ophthalmology exam were completed and were both normal. The patient’s respiratory status and liver function improved, and she was discharged to home after DCFS involvement.

Discussion

NAT can present in various ways. Examples of initial physical exam findings include bruises in various stages of healing, skeletal fractures in non-ambulatory children, and immersion burns. Risk factors for NAT can be grouped into three categories: parental, child, and social. This patient had multiple social risk factors for NAT. It is important to have a high index of suspicion for NAT in children with risk factors. Not every pleural effusion is caused by the most common etiology.

Case reports in cardiovascular medicine

2:00 PM

Thursday, February 13, 2020

41 Acute pericarditis complicated by atrial flutter and acute stroke: a management dilemma

D Daniels1*

JD Pollard2

1University of Mississippi Medical Center, Brandon, MS

2University of Mississippi Medical Center, Jackson, MS

Case report

No clear guidelines have been established in the management of Atrial Fibrillation/Flutter in the setting of Acute Pericarditis. These arrhythmias are generally thought to be transient and resolve with treatment of the underlying disease. A 47 year old African American male with history of hypertension, substance abuse and a questionable history of stroke presented to the Emergency Department with chest pain and elevated blood pressure. A CTA did not show dissection or PE, but did show a pericardial effusion. He had an electrocardiogram that showed inferior ST elevation; however coronary angiography was without significant CAD. The patient had elevated inflammatory markers and was thought to have acute pericarditis. The patient developed palpitations and was found to have symptomatic paroxysmal atrial flutter. Anticoagulation for new onset atrial flutter associated with pericarditis, was discussed. The theoretical risk of hemorrhage into the pericardium was weighed against the risk of a cardio-embolic stroke. As the patient had a CHADsVASC of 1 or 3 depending on the history of stroke, neurology was consulted for evaluation and recommended MRI, which showed an acute lacunar infarct consistent with a cardio-embolic origin. Patient was therefore started on Apixaban for further stroke prevention. This case highlights that even though atrial arrhythmias occur in pericarditis, one study suggest an incidence of 4.3%,1 they are usually considered transient and related to inflammation; however the risk of stroke is also present. Moreover, there are no specific guidelines for management of atrial fibrillation in acute pericarditis, and therefore the theoretical risk of hemorrhage into the pericardium and benefits (stroke prevention) should be carefully considered and instituted if thought reasonably safe.

Reference

  1. Imazio M, et al. Heart 2015;101:1463–1467. doi:10.1136/heartjnl-2014-307398

42 A rare presentation of eosinophilic thrombotic myocarditis

VF Civelli1,2

A Heidari1

J Kim3

P Sharma4*

S Singh2,5

N Doctor2

R Sharma2

1UCLA Kern Medical Center, Bakersfield, CA

2Central Cardiology Medical Center, Bakersfield, CA

3Touro University California, Vallejo, CA

4Golden State Hospitalists, Bakersfield, CA

5Cedars-Sinai, Los Angeles, CA

Purpose of study

A 62-year-old very healthy gentleman with a history of seasonal allergies presented to the hospital with stroke symptoms. Initial complete blood count showed an eosinophilic count of 44 thousand. The patient’s transesophageal echocardiogram showed a left atrial appendage with thrombus. For initial concern of cardiac embolic phenomenon, anticoagulation therapy was started but a subarachnoid hemorrhage soon developed. His course complicated quickly to include renal failure. The patient was transferred to a tertiary center where he was dialyzed and improved significantly. Despite improvements and steroid treatment, he remained in critical condition.

Methods used

Patient chart and literature review of ‘Eosinophilic Myocarditis with Thrombosis’.1 2

Summary of results

His cardiac magnetic resonance imaging (MRI) and biopsy both suggested confirmatory diagnosis of eosinophilic myocarditis with left ventricular ejection fraction of 35% with acute congestive heart failure symptoms. He received a dose of cyclophosphamide and heart failure medications. Eosinophilic myocarditis with thrombotic phenomena is a very rare condition and presents as a stroke phenomenon and often found to have a left atrial appendage clot. There are 3 stages of eosinophilic myocarditis progression: inflammatory, thrombotic and fibrotic.

Conclusions

The patient is doing very well on cyclophosphamide to date.

References

  1. Keogh KA, Specks U. Churg-Strauss syndrome. Semin Respir Crit Care Med2006; 27:148. Available at: https://www.vasculitisfoundation.org/education/forms/eosinophilic-granulomatosis-with-polyangiitis-churg-strauss-syndrome/. Accessed on Sept 2019.

  2. Ramakrishna G, Connolly HM, Tazelaar HD, et al. Churg-Strauss syndrome complicated by eosinophilic endomyocarditis. Mayo Clin Proc 2000;75:631–635.

43 Pulmonary embolism mimicking as acute myocardial infarction; searching for a non-ischemic cause of troponin elevation

RD Silva-Cantillo*

J Escabi-Mendoza

VA Caribbean Healthcare, San Juan

Case report

Most patients with type-1 acute myocardial infarction (AMI) have evidence of obstructive coronary artery disease (CAD) on coronary angiography. However, in the absence of obstructive CAD, the remaining patients can be separately termed as myocardial infarction (MI) with non-obstructive CAD (MINOCA) after excluding other ischemic and non-ischemic etiologies. We present this case as an example of the appropriate diagnostic work-up.

A 66-year-old man with history of hypertension and active smoker, presented to the emergency department with complaints of ongoing chest pain and shortness of breath (SOB) that resolved after treatment with nitrates and morphine. His physical examination was unremarkable. Arrival electrocardiogram (ECG) revealed inferior ST segment depressions, and serial cardiac troponins were positive for AMI. He was admitted to the coronary care unit with a diagnostic impression of non-ST segment elevation MI. A coronary angiography was performed within 24-hours with evidence of non-obstructive CAD. In view of echocardiographic evidence of mild right ventricle dilatation and initial complaints of SOB, D-dimers were requested for assessment of pulmonary embolism (PE). Despite a Wells’ score of 0, the reported D-dimer were markedly elevated. Prior to a diagnostic chest CT angiography (CCTA) the patient became clinically unstable with respiratory failure and hemodynamic compromise requiring emergent endotracheal intubation and vasopressors. His ECG showed new onset atrial fibrillation and right bundle branch block. After stabilization, CCTA confirmed the presence of a large saddle PE. He was treated with alteplase and full anticoagulation, and despite a prolonged hospital stay, he was finally discharged on chronic anticoagulation.

This case illustrates an atypical presentation of PE mimicking as AMI, highlighting the importance of a thorough evaluation prior to assuming a diagnosis of MINOCA which should be kept as a diagnosis of exclusion. Expert opinion recommends a stepwise diagnostic approach that should start by promptly excluding alternative causes of non-ischemic myocardial injury for pertinent time sensitive diagnosis and care.

44 Stemi opens eyes to right atrial metastatic renal cell carcinoma

A Hallak*

F Mubeen

K Shepherd

Texas Tech University, Amarillo, TX

Introduction

Renal cell carcinoma (RCC) represents about 3% of all cancers in the United States. While the incidence of RCC has been increasing, there has been a steady decline in the size of tumors and a rise in the 5-year survival, which may be due to increased utilization of improved imaging and curative surgery.

RCC is a highly vascular tumor which tends to metastasize early. Up to 10% of patients with RCC have tumor thrombus involving the renal vein and inferior vena cava (IVC) and up to 1% of patients have tumor thrombus extending into the right atrium. Hematogenic spread of the tumor to the heart is much more common than lymphatic spread and associated with a better prognosis.

Case

A 62-year-old female presented to an outlying facility with paresthesia of the left arm, lightheadedness, and nausea, and was found to have ST elevations in the inferior leads with sinus bradycardia and elevated troponin of 0.184 ng/mL. She was administered alteplase, placed on heparin and nitroglycerin drips, and transferred to our facility. Echocardiography showed normal left ventricular function and a right atrial inferior wall mass. She underwent coronary angiography, which showed significant 3-vessel disease with significant left main disease and was referred for coronary bypass grafting, during which the mass was excised and sent for pathology and found to be clear cell renal carcinoma. CT imaging revealed a 5 cm right kidney lower pole lesion with no enhancement, filling defects within the intrahepatic IVC and right inferior renal vein extending into the IVC, and no pulmonary or intracranial metastasis.

Discussion

The typical symptoms of RCC include flank pain, a palpable mass and hematuria, however with increased utilization of imaging this has become more the exception rather than the rule. This is the only documented case of a patient diagnosed with RCC incidentally during MI, as cardiac metastasis is asymptomatic until coronary compression or outflow tract obstruction.

The median survival period of patients with metastatic RCC is 6 to 12 months, and the 5-year survival rate is only 9%. In the non-emergent setting, it may be warranted to investigate a new mass which may be associated with a poor prognosis prior to undergoing any invasive operations.

45 Cardiac tamponade from metastatic appendiceal adenocarcinoma: first reported case

MB Omar*

K Sanders

M Oye

A Richardson

University of Florida College of Medicine, Jacksonville, FL

Case presentation

A 69 year old female presented with worsening dyspnea for a month. She had a history of moderately differentiated non-mucinous appendiceal adenocarcinoma, diagnosed 10 months prior on pathology from an emergent appendectomy for acute appendicitis. On this presentation, echocardiography revealed a large pericardial effusion with tamponade physiology (figure 1A). Emergent pericardial window was successfully performed. Pericardial fluid cytology and tissue histology with immunostaining was consistent with metastatic adenocarcinoma (figure 1B). Investigations for an alternative primary were unremarkable including mammography and computerized tomography imaging of the chest, abdomen and pelvis. The patient was referred to oncology for further management.

Discussion

Appendiceal adenocarcinoma accounts for less than 1% of all gastrointestinal neoplasms. There are rare reports of metastases to the liver, lung, thyroid and genitourinary system. However, cardiac involvement has never been reported. The rarity of metastatic appendiceal cancer portends a paucity of high quality evidence behind management strategies. Whilst select cases may be suitable for solely appendectomy, most experts recommend right hemicolectomy to reduce the risk of poorer outcomes, as in our case. There are promising data supporting systemic chemotherapy, usually combination fluouricil and platinum based regimens, that have shown improved overall survivability even in patients with metastatic disease.

Learning points

Incidentally diagnosed appendiceal cancer on pathology should prompt case specific consideration for aggressive management given the risk of late distant metastasis.

Abstract 45 Figure 1

A) Pericardial effusion with diastolic collapse of the right ventricle (arrowhead) and B) Pericardial histology with adenocarcinoma cells (arrow) A) Pericardial effusion with diastolic collapse of the right ventricle (arrowhead) and B) Pericardial histology with adenocarcinoma cells (arrow)

46 Phlegmasia cerula dolens in a patient with systemic lupus erythromatosis_ a case report

H Mallah*

J Makram

A Motes

J Sekhon

M Elmassry

A Yepes-Hurtado

Texas Tech University Health sciences Center, Lubbock, TX

Introduction

Phlegmasia cerulea dolens (PCD) is a type of deep venous thrombosis (DVT), that results from acute massive venous thrombosis obstructing the venous drainage.

Systemic lupus erythematosus (SLE) is a chronic inflammatory disease, that can affect any organ. Thromboembolic disease is a common manifestation and mostly associated with antiphospholipid antibodies.

We report a rare case of PCD, complicated by septic thrombophlebitis in a patient with SLE, but not antiphospholipid syndrome.

Case report

A 27-year-old female patient, known to have SLE, was transferred to our facility for suspected necrotizing fasciitis, with progressive swollen, painful, and discolored right lower extremity of 3 weeks duration. She was found to have large venous thrombosis of the right popliteal and posterior tibial veins. She was diagnosed with PCD and underwent tissue plasminogen activator catheter-directed thrombolysis, and mechanical thrombectomy, followed by a heparin infusion, with improvement in lower limb pain, and mobility. Antiphospholipid antibodies were negative.

She also had Methicillin-resistant staphylococcus aureus bacteremia and septic shock. Chest CT scan PE protocol showed bilateral cavitary lung nodules, consistent with septic embolization. TTE and TEE were negative for endocarditis, and the only suspected source of infection was the septic thrombophlebitis. She was appropriately managed with Vancomycin.

Discussion

PCD is a rare complication of DVT, that is most frequently associated with malignant neoplasms and severe heart failure. However, it has been associated with other conditions, including primary antiphospholipid syndrome; femoral vein catheterization; abdominal aortic aneurysm; hypercoagulability situations. Only a few cases found in the literature of patients with SLE complicated by PCD.

In the case reported, the inflammatory activity of the underlying disease was most likely contributed to the occurrence of DVT, which evolved to PCD.

PCD is a medical emergency, has high morbidity and mortality rates, requiring limb amputation in several cases. Therefore, it is imperative to identify these patients and consider more aggressive management, usually thrombolysis and/or thrombectomy.

47 Hyperkalemic cardiac suppression

CH Harris1*

R Drutel1

M Modica1

LS Engel1

V Falco1,2

1LSU Health Sciences Center, New Orleans, LA

2Crescent City Physicians, New Orleans, LA

Introduction

The cardiac effects of hyperkalemia are a manifestation of electrophysiological changes. Hyperkalemia causes alterations in the resting membrane potential secondary to differences in potassium concentration across the cell membrane. Initially, hyperkalemia can present as subtle ECG changes. However, if left unresolved, conduction abnormalities and fatal cardiac arrhythmia are possible.

Case

A 64 year old man presented with one day of chest pain and generalized fatigue. Initial ECG was significant for third degree A-V block. A temporary pacemaker was placed emergently and a left heart catheterization revealed severe 3-vessel disease. The patient subsequently underwent coronary artery bypass graft (CABG) days later. Postoperatively, the patient’s urinary output declined, and he developed acute encephalopathy. Prior to the change in mental status, the patient’s ECGs consistently showed atrial-sensed ventricular pacing to intrinsic rates as high as 110 beats per minute (bpm). However, an ECG obtained during the episode of encephalopathy showed new atrial pacing in addition to ventricular pacing and a slowed heart rate to the pacemaker’s setting of 80 bpm. Labs resulting after the EKG was captured revealed acute renal failure with potassium elevated to 7.3 mEQ/L. The ECG comparisons before and after the onset of hyperkalemia were significant for a new conduction delay indicative of hyperkalemia.

Discussion

The presence of hyperkalemia poses the threat of fatal cardiac arrhythmias. Therefore, when present, it is an electrolyte abnormality that must be quickly addressed and corrected to prevent such life threatening sequelae. Although ECGs do not reliably indicate hyperkalemia in every situation, in cases of marked hyperkalemia, changes found on ECGs may be the first warning. Here, we report a case of hyperkalemic cardiac suppression manifesting as a conduction delay. This case demonstrates that ECGs are a useful tool for early recognition and treatment of hyperkalemia even in the absence of labs.

48 Are implantable cardioverter defibrillators appropriate among patients with chronic kidney disease?

JN Kiage1*

Z Latif1

MA Craig1

NE Mansour2

R Khouzam1,2

1University of Tennessee Health Science Center, Memphis, TN

2Methodist University Hospital, Memphis, TN

Purpose of study

To investigate whether the use of implantable cardioverter defibrillators (ICDs) among patients with chronic kidney disease (CKD) or end stage renal disease (ESRD) is appropriate for primary or secondary prevention of sudden cardiac death (SCD).

Methods used

We conducted a systematic review of studies that have investigated the use of ICDs among patients with CKD or ESRD in PubMed. The following MeSH terms were used: (‘ICD’ OR ‘Implantable cardioverter defibrillator’ OR ‘defibrillator’) AND (‘CKD’ OR ‘Chronic kidney disease’ OR ‘Kidney failure’ OR ‘ESRD’ OR ‘End stage renal disease’ OR ‘Renal Failure’) AND (‘appropriate’). We then compared the rates of anti-tachycardia pacing (ATP) or shock and mortality among patients with versus those without CKD or ESRD.

Summary of results

We identified 57 studies out of which we selected 18 studies that had data on the use of ICDs among CKD/ESRD patients and ATP/shock and/or mortality. Most of these studies were either prospective or retrospective cohort in design. Patients with CKD/ESRD tended to be older and had more comorbidities. Patients with CKD/ESRD were more likely to get ATP or shock and they had higher cardiac and/or all-cause mortality compared to patients without CKD/ESRD. Moreover, studies showed that these associations had an inverse dose-response effect with worse outcomes with decreasing kidney function. Our search revealed only one small randomized controlled trial (n=188) that investigated the use of ICDs in ESRD among patients with left ventricular ejection fraction ≥35% and showed no mortality benefit.

Conclusions

Our review findings show that the use of ICDs in CKD/ESRD is associated with increased ATP/shocks and mortality suggesting that their routine use in this patient population may be associated with more adverse outcomes than benefits. Large and better designed studies to further investigate the usefulness of ICDs in CKD and ESRD are warranted.

Moving your clinical case presentation into a published manuscript

2:45 PM

Thursday, February 13, 2020

49 Testicular pain: it’s the heart of it all

P Snyder*

A Watson

EB Crawford

C Tan

UAB, Birmingham, AL

Case report

A 12-year-old male presents to the emergency department with four days of abdominal pain and vomiting. A few days prior, he was diagnosed with dehydration after reassuring abdominal x-ray and basic screening labs. In the interim, his symptoms worsened including progressive exhaustion with activity. On exam, he was tachycardic to 112, tachypneic to 40, and hypotensive to 91/64 mmHg. His abdomen was diffusely tender to palpation with guarding, and he endorsed right-sided testicular pain without swelling, skin color changes, or abnormal testicular lie. Initial workup included a testicular ultrasound negative for testicular torsion followed by a contrasted abdominal and pelvic CT scan notable for small bowel to small bowel intussusception and hepatomegaly. Upon further review, imaging also identified incidental cardiomegaly. Labs noted significantly elevated liver transaminases with synthetic dysfunction, acute kidney failure with associated electrolyte disturbances, and a brain natriuretic peptide >3,000. Echocardiogram revealed grossly dilated cardiomegaly with an ejection fraction of 23%, significantly enlarged left atrium, and severe mitral regurgitation. Patient was admitted to the CVICU for further management of dilated cardiomyopathy (DCM) with associated decompensated heart failure. He then required a prolonged course of veno-arterial extracorporeal membrane oxygenation for persistently low cardiac output in addition to continuous renal replacement therapy for renal failure. Once decannulated, he required both right and left ventricular assist devices to bridge him until a successful heart transplantation.

Discussion

The etiology of congestive heart failure in pediatrics varies by age group, and DCM tends to be the most common cause. Clinical features include abdominal pain, anorexia, respiratory distress, poor weight gain, and hepatomegaly. In DCM, abdominal complaints are the most common presenting symptoms across all pediatric age groups, and occur more frequently than respiratory symptoms in adolescents. With isolated GI complaints, heart failure is often recognized late and picked up incidentally on imaging.

Conclusion

Unlike adults, children can present with a myriad of nonspecific symptoms when in heart failure. Recognition of these symptoms is critical in timely diagnosis and treatment.

50 Mysteries of cardiac tamponade

N Rus1*

MF Habib2

RI Hazam1

T Naguib1

1Texas Tech Univ HSC Amarillo, Amarillo, TX

2Amarillo Cardiovascular Center, Amarillo, TX

Case report

Cardiac tamponade (CT) is a potentially fatal medical emergency caused by the accumulation of fluid in the pericardial space.

We report a case of low-pressure CT of unknown etiology, where PR segment depressions in 12-lead EKG could help with early recognition of this condition.

44-year-old female referred from outside facility with chest pain and dyspnea. Initially, she received tPA for a possible ST-segment elevation myocardial infarction. However, upon arrival to ED, her EKG showed PR depressions in inferior leads along with troponin elevation. Left heart catheterization was normal, as was the work up for pulmonary embolism. Echo showed underfilling of the left ventricle, small right ventricle with collapse of right atrium, small pericardial effusion and respiratory variations on mitral valve inflow. While being worked up, she went into PEA cardiac arrest. Emergent pericardiocentesis was performed with 140cc of serosanguineous fluid aspirated, but patient could not be revived.

Among various types of CT, low-pressure tamponade is often seen in patients with severe volume depletion. These patients often have abnormal findings on ECHO, such as chamber compression, IVC dilation and respiratory variations in flow and volumes. However, they lack the classic physical presentations of elevated JVD or pulsus paradoxus. In an acute setting, 150cc of pericardial fluid is enough to cause severe hemodynamic instability. Presence of PR depression on EKG was found to be 86% specific with 95% negative predictive value. Early diagnosis and treatment are keys to reducing mortality. European Society of Cardiology (ESC) Working Group on Myocardial and Pericardial Diseases developed a scoring system, which is used to stratify patients who need immediate intervention. CT is a fatal condition without prompt intervention. Early detection of low-pressure CT is challenging due to lack of classic clinical signs. Careful examination of other indirect evidence like PR depressions on EKG may aid with early recognition of this fatal condition. We conclude that combining above-mentioned EKG findings with ECS Working group scoring system helps with early recognition and prevention of potentially fatal outcome.

Adolescent medicine and pediatrics

Joint plenary poster session and reception

4:30 PM

Thursday, February 13, 2020
51 Not just another virus: leukopenia and transaminitis in an 18-month-old male

F Ajmal1*

M Goldstein1

F Levent1

F Laham2

S Carroll1

1AdventHealth Orlando, Orlando, FL

2Orlando Health Arnold Palmer Hospital, Orlando, FL

Case report

Human Immunodeficiency virus (HIV) is a relatively uncommon diagnosis in the United States since 1985 as a result of highly effective screening assays and protocols. Since the mid-1990s the number of reported pediatric Acquired Immunodeficiency Syndrome (AIDS) cases has significantly decreased because of prevention of mother-to-child transmission of HIV, and implementation of antenatal HIV testing. Early diagnosis and treatment are essential, as the prognosis for survival is poor in untreated infants. Untreated infants may develop AIDS-defining conditions during the first 6 months of life, including pneumocystis pneumonia, progressive neurologic disease and severe wasting; all poor prognostic markers.

Case presentation

We present a case of a previously healthy 18-month-old male who presented for evaluation of leukopenia and worsening transaminitis, with recurrent vesicular rash. He underwent extensive evaluation, all unremarkable, however nasal polymerase chain reaction swab was positive for rhinovirus/enterovirus. HIV screen was positive with DNA qualitative PCR resulting HIV 1 Ab positive. Further evaluation revealed a high viral load of 988,009 copies/ml, and low CD4 count of 188cell/uL and CD4 helper cells 8.2%, consistent with a diagnosis of AIDS. Additional work up of the vesicular rash included a positive serum varicella zoster PCR, and he was initiated on Acyclovir. He improved clinically and was discharged home to continue treatment with Zidovudine, Lamivudine and Raltegravir, with Acyclovir, Pentamidine and Azithromycin prophylaxis. Maternal intrapartum screening seven months prior to delivery was reported as negative, however after diagnosis parental evaluation was performed which yielded a positive HIV screen in both parents. The transmission is thought to be from breastfeeding.

Conclusion

Our goal is to emphasize the importance of HIV testing in the setting of unexplained transaminitis and leukopenia, with a reminder that an AIDS defining illness such as varicella zoster may be the initial presentation.

52 Relationship of depression with blood pressure and pulse rate

JJ Burns1

R Amin2*

J Bruyere1

E Eloselly1

A Kurady1

S Khurana1

1University of Florida, Pensacola, FL

2University of West Florida, Pensacola, FL

Purpose of study

The purpose of this study is to determine if there is a relationship between depression on blood pressure and pulse for adolescents over 13 years attending an Adolescent Medicine clinic.

Methods used

A database was created of adolescents diagnosed with a depressive disorder. A control group without depressive diagnosis was used for comparison. Outcomes were systolic and diastolic BP percentiles and pulse rates.

A two-way type III ANOVA using transformed variables was used to analyze systolic and diastolic BP percentiles and pulse rates as dependent variables and sex (male/female) and depression status (depressed/non-depressed) entered as fixed factors.

Summary of results

On two-way type III ANOVA using transformed variables, there was a significant main effect of depression with higher systolic blood pressure percentiles in depressed patients vs. non-depressed (57.4 vs. 47.8; F (DF 1)=4.48, p=0.036) but not with sex of patient. There was no sex by depressed interaction. There was no main effect of depression on diastolic blood pressure but higher diastolic blood pressure percentiles were found in females vs. males (64.5 vs. 54.1; F (DF1)=4.18, p=0.043) with no sex by depressed interaction. Finally, there was a significant interaction effect of depression and sex on Pulse Rate so that higher Pulse Rates were found in depression only among males but not females. (males depressed 86.7 vs. males non-depresswed 72.1; F (DF 1)=8.61, p=0.003) (figure 1).

Conclusions

In summary, systolic BP was higher in depressed patients, diastolic BP was higher in female patients and pulse rate was higher for depressed males vs. non-depressed males but this was not found for depressed females vs. non-depressed females.

Abstract 52 Figure 1

Interaction plot for pulse: depression and sex Interaction plot for pulse: depression and sex

53 A case report of mercury poisoning presenting with neurologic manifestations, desquamating rash, tachycardia, and hypertension

KL Angelle*

J Yeager

C Styres

SB Palombo

M Williams

LSUHSC-New Orleans, New Orleans, LA

Purpose of study

Mercury poisoning can present with multisystem involvement. Without an obvious history of exposure it can be challenging to diagnose and even harder to treat.

Methods used

Chart review.

Summary of results

A previously healthy 13-year-old male who was exposed to elemental mercury found in a home garage developed multiple sequelae over the course of several weeks. His symptoms included weakness, anorexia, neuropathy, acrodynia, tachycardia, hypertension, and new onset seizures. Heavy metal screening labs were finally obtained seven weeks after exposure, which were significant for a blood mercury level of 9 ug/L and a 24-hour urine mercury level >72 ug/d. He also had an elevated 24-hour urine normetanephrine level (725 ug/d), which explained his pheochromocytoma-like manifestations. Our patient was started on chelation therapy with DSMA Succimer. Despite initiation of chelation and additional symptomatic supportive care, he had a fluctuating clinical course with an ongoing long-term recovery.

Conclusions

This case represents the complex presentation of mercury poisoning in children. We hope that promoting screening for heavy metal exposure leads to earlier recognition and treatment, which can potentially mitigate toxic effects. At this time, long-term sequelae are not fully understood.

54 Pediatricians’ knowledge of digital media use in the home: the forward families study

J Roberts1

J Brown2*

S Renaud3

N Arthur3

N Malhotra3

C Turley3

1Medical University of South Carolina, Charleston, SC

2Spartanburg Regional Hospital System, Spartanburg, SC

3University of South Carolina, Columbia, SC

Purpose of study

Despite the American Academy of Pediatrics’ (AAP) policy statement on the use of digital media, children exceed the amount of recommended screen time. The AAP recommends that families establish a family media use plan but studies about implementation have not been done. The purpose of this study is to evaluate pediatricians’ level of knowledge of these guidelines and understand future educational needs for families.

Methods used

A 13-item survey was developed and distributed to practicing pediatricians. Questions were developed by the author team based on policy statement content. Questions were designed to explore their knowledge base about maximum screen time by age group, attitudes about digital media use, current counseling practices for families including specific recommendations about electronics use and if they recommended a family media use plan. The survey was developed through an iterative process, piloted by a small group of practicing pediatricians for content and clarity and revised accordingly. Surveys were then distributed via an email link or on paper to pediatricians in our statewide research network as well as pediatricians attending the South Carolina AAP meeting. Frequency distributions were calculated.

Summary of results

While nearly all (93%) providers recognized that children between 6 and 12 years of age should have 2 hours or less screen time per day, just over half (57%) were aware of the recommended amount of screen time for 3–5 year olds. Providers identified sleep problems (74%), obesity (71%) and impaired social skill development (44%) as top negative effects from screen time. Only 13% recommend that families create a family media use plan. Most providers (84%) are interested in a tool box of materials to guide family counseling, with sleep habits (77%), Internet safety (72%), exposure to age appropriate content (67%), as topics generating the most enthusiasm.

Conclusions

Initial analysis demonstrates that despite a high frequency of digital media counseling, most pediatricians do not recommend a family digital media use plan. Pediatricians were very interested in digital media use educational materials for their practice.

55 Confluent and reticulated papillomatosis presenting a diagnostic challenge

JJ Burns*

University of Florida, Pensacola, FL

Case report

Patient is a 13-year-old white male presenting with a rash on chest and neck for 6 months not responding to multiple applications of selenium sulfide lotion and Clotrimazole cream. Of note there was never a microscopic analysis or culture for fungal elements performed.

In addition to the rash, the patient has been diagnosed with obesity, essential hypertension and gynecomastia with negative workup.

On examination, there was a reticulated rash consisting of hyperpigmented, patches over the chest, neck and arms. The patches were mostly brown in color with some pink areas with increased density over the neck. Otherwise findings included obesity, hypertension and gynecomastia.

Microscopic examination of skin scrapings failed to reveal any fungal elements; fungal culture was negative.

Course

A diagnosis of of Confluent and Reticulated Papillomatosis was made based on clinical appearance of the rash and negative fungal studies. A course of Azithromycin 500 mg 3 x per week for 3 weeks was prescribed with complete resolutions. He subsequently had a recurrence succesfully treated with Minocycline 50 mg BID for 42 days.

Discussion

Clinical criteria for CARP includes scaly brown macules and patches, involvement of the upper trunk and neck, negative fungal staining of scales and no response to antifungal treatment with an excellent response to minocycline. There are many theories of etiology including the bacteria Dietzia Papillomatosis a gram positive actinomycete. It is found more commonly in obese patients and patients with polycystic ovaries with a recurrence rate of 15% and is often confused with tinea versicolor.

Abstract 55 Figure 1

Initial presentation of rash not responding to antifungal treatment Initial presentation of rash not responding to antifungal treatment

56 17 year old with serious liver disease presenting with mildly elevated liver enzymes

JJ Burns*

S Durganna

University of Florida, Pensacola, FL

Case report

A 17-year-old morbidly obese female presented for evaluation of abdominal pain in the epigastric, and right and left upper quadrant area, with 7/10 intensity that was worse with eating fatty foods accompanied with some dyspepsia. She also had nausea/vomiting, 3–4 times a week and recurrent weekly fevers of 101–102 F for years. She denied history of constipation, diarrhea, hematochezia, dysphagia, perianal disease, polyuria/polydipsia or dysuria. She has past medical history of anxiety, depression and migraine headaches. She was on Omeprazole 20 mg daily, Phenergan PRN nausea, Sertraline 100 mg daily and Topamax 25 mg daily.

On review of systems, she had fatigue, fever, shortness of breath, nosebleeds, headache, and dizziness, no snoring, and she had regular menstrual periods.

Social history was positive for depression and anxiety; she had suicidal thoughts in the past but none at time of presentation.

On physical exam, vital signs were remarkable for a weight of 291 pounds, BMI of 45.7 (>99th percentile). Blood pressure was 144/79 mmHg. She was not in acute distress, her lungs were clear to auscultation, heart sounds normal, abdomen was soft with no organomegaly. Fundi were normal and she had a normal neurological exam.

Liver enzymes were elevated with AST of 60 with repeat 3 months later of 70, ALT was 40 with repeat 3 months later of 53. CBC was normal. Her ESR 38, HbA1C was elevated at 6.4. Lipid profile revealed elevated LDL of 153 and triglycerides 187, with a low HDL. Tissue transglutaminase antibody, hepatitis serology, alpha 1- antitrypsin, ceruloplasmin, TSH, Vitamin B12, Folate, smooth muscle antibody and immunoglobulin levels were all normal.

An abdominal ultrasound showed an enlarged liver and spleen with findings suspicious for fatty liver. Further workup included an upper GI endoscopy which showed esophageal varices indicative of portal hypertension. A liver biopsy was later obtained which showed cirrhosis of the liver due to Nonalcohlic Steatohepatitis (NASH).

Nonalcoholic fatty liver disease (NAFLD) is found in 10 percent of children, with 23 percent of those with NAFLD having NASH. This patient presenting with early cirrhosis and portal hypertension, illustrates presentations can occur early in life with only mild elevations in liver enzymes.

57 Current state of point-of care ultrasound in pediatric emergency medicine: survey of pediatric emergency medicine fellowship directors

T Coco1*

NP Shah1

M Fifolt1

L Patel2

J Chao3

I Kim4

1University of Alabama at Birmingham, Birmingham, AL

2University of Missouri- Kansas City, Kansas City, MO

3Suny Downstate Medical Center, Brooklyn, NY

4University of Louisville, Louisville, KY

Purpose of study

Point-of-care ultrasound (POCUS) in pediatric emergency medicine (PEM) has been in use for more than 10 years. However, there is lack of recent data about POCUS in PEM fellowship programs. We assessed the current state of POCUS in PEM including POCUS use, methods of training, and perceived barriers to a structured POCUS program.

Methods used

A survey link was emailed to 80 PEM fellowship directors (FD) and 4 reminders were sent from October 2018 to January 2019. The survey included questions about POCUS use in their program, POCUS training, credentialing and perceived barriers to a structured program. Incomplete responders were included in the analysis. We used descriptive statistics to calculate frequencies and proportions.

Summary of results

Of 80 FD, 43 (53.8%) responded to the survey and 40 (50%) were complete. All responders reported that an ultrasound machine is available in the Pediatric Emergency Department (PED). Only 34% of FD reported performing POCUS in the past 6 months. All FD reported that PEM Fellows receive some form of POCUS training of which 25% reported that fellows receive POCUS training from PEM physicians or general EM physicians and 26/39 (66%) of programs provide dedicated shifts for POCUS training. 25/40 (62.5%) reported having an US credentialing process; of those, 15/24 (62.5%) reported using criteria based on the 2016 American College of Physicians guidelines. 26/39 (66%) reported that lack of infrastructure and training was the most common barrier to a structured POCUS program at their institution.

Conclusions

The type of POCUS training and the credentialing process varies widely among fellowship programs. This suggests the need for a standard curriculum for POCUS education in PEM fellowship training as well as recommendations for a specific credentialing process.

58 Differences in levels of inflammatory cytokines and psychological assessment of obese and healthy adolescents: impacts of sex

S Hefley*

A Hernandez

T Vasylyeva

Texas Tech University Health Sciences Center, Amarillo, TX

Purpose of study

Obesity in adolescents is increasing at an alarming rate, as the CDC estimates that 1 in 5 adolescents is obese. The gut microbiome has been shown to contribute to obesity. Importantly, dysbiosis of the gut microbiome can lead to an increase in inflammatory cytokines, leading to the development of chronic diseases. In this study, we collected blood and urine for assessment of levels of the inflammatory cytokines, markers of endothelial dysfunction, and stool for gut microbiome. Furthermore, we evaluated mental health status of our participants. The goal of the study was to assess sex impacts on those parameters.

Methods used

Twenty-two adolescents (10 males, 12 females) ages 8–18 (BMI >25) were recruited into the study. Blood, urine, and stool was collected from obese adolescents, while urine and stool were collected from participants with BMI <25. We obtained IRB approval and informed consent from the parents and participants. Blood and urine samples were analyzed for TNF-a, IL-6, and ET-1 by ELISA. Lipid profile and HbA1c of obese adolescents was analyzed by Quest Diagnostics. A self-assessment PSC-Y questionnaire was administered to all enrollees to evaluate mental health. Stool analysis includes V3–V4 regions of bacterial 16S rRNA genes amplified by PCR with a bacterial universal primer set containing Illumina adaptors.

Summary of results

Interestingly, obese males were more likely to have high blood pressure and larger hip-to-waist ratios compared to females while lipid profile evaluation revealed differences between male and female obese adolescents, as we noted that males had increased triglycerides and HDL compared to females. Levels of TNF-a and ET-1 in obese adolescents were increased compared to healthy controls. Obese female adolescents were more likely to exhibit symptoms associated with depression and anxiety, while obese males were more likely to exhibit externalization and attention problems. Gut microbiome analysis in currently underway.

Conclusions

This data suggests that adolescent obesity increases inflammatory cytokines and that sex impacts not only physiological parameters in response to elevated weight, but also psychological perceptions.

59 Descriptive analysis of pediatric drownings seen in emergency department

B Hinten*

AR Wheeler

BO Foxworthy

A Ricci

A Webb

NP Shah

K Monroe

MH Nichols

University of Alabama at Birmingham, New Market, AL

Purpose of study

Drowning is the #1 cause of injury death in children aged 0–4 years. The objective of our study was to describe the pediatric drowning cases seen in the emergency department (ED).

Methods used

We reviewed medical records of all patients presenting to the Pediatric ED between January 2016 through June 2019 with the diagnosis of drowning identified using ICD-9 and ICD-10 codes. Demographics (gender, age, race), Emergency Severity Index (ESI) level (ESI is stratified from level 1 to level 5 with 1 most severe), drowning body of water (pool, pond, hot tub/bath tub, septic tank, creek, river, lake), whether cardiopulmonary resuscitation (CPR) was performed and outcomes including disposition and length of hospital stay were reviewed. We performed descriptive analysis including frequencies and proportions, mean (± SD) and median (interquartile range).

Summary of results

110 patients were identified with a mean age of 4.6 (± 1.2) years. 66 (60%) were male, 70 (63.6%) were Caucasian, 37 (33.6%) Black, and 3 (2.7%) Other. In 83 (75.5%) cases, pool was the drowning body of water. About 60% of drowning cases were seen during the summer months (May –June). Median ESI level was 3 (2.75 to 4). CPR was performed by medical professionals in 48 (43.6%) cases and CPR was given by non-professionals in 43 (39.1%) cases. 58 (52.7%) were discharged home and 50 (45.5%) were admitted to the hospital including 8 cases admitted to the Intensive Care Unit. One patient expired. One patient left the ED without being evaluated. Mean length of stay was 23 (± 9.7) hours.

Conclusions

Despite efforts to prevent drowning, the number of pediatric drowning events is significant leading to increased hospital burden. Drowning prevention education, especially with regard to pools and pre-school aged children, should be a focus of our injury prevention efforts.

60 Moraxella catarrhalis infectious arthritis as an inaugural event in the diagnosis of systemic lupus erythematosus

SP Holt1*

S Parkhurst2

1University of Tennessee Health Science Center, Memphis, TN

2UTHSC, Memphis, TN

Case report

Systemic lupus erythematosus can manifest in nearly any organ, but it almost always involves the musculoskeletal system with symmetrical, migratory, and polyarticular arthritis. Contrastingly, Infectious arthritis is a rare presentation of lupus and can create a diagnostic hurdle to prompt recognition and treatment.

Case presentation

A 15-year-old female presented with 2 days of right elbow pain and swelling and fatigue and presyncope. Workup included a CBC significant for pancytopenia and elevated inflammatory markers. Ultrasound of the right elbow revealed a complex joint effusion containing debris and subsequent arthrocentesis showed a neutrophilic–predominant white blood cell count of only 18,500/mm3. As this was well-below the diagnostic criteria of infectious arthritis, antibiotics were not initially started until the patient became febrile without another source of infection. Her synovial fluid grew Moraxella catarrhalis, which in conjunction with her pancytopenia prompted an autoimmune workup. Based off the SLICC criteria (positive ANA and dsDNA, low complements, hemolytic anemia, and leukopenia), she was diagnosed with mild-moderate systemic lupus erythematosus. Once treated for septic arthritis, she was then started on pulse-dose steroids and hydroxychloroquine.

Conclusions

While infectious arthritis has been described in patients with SLE, it typically manifests in patients with pre-existing joint disease with a significant delay following the initial diagnosis. Additionally, the most commonly cited organisms are S. aureus and Salmonella species rather than a typically commensal respiratory organism, Moraxella catarrhalis, as this pathogen’s ability to cause invasive infection is exceedingly rare. To our knowledge, there is only one other reported case of Moraxella infectious arthritis in a lupus patient, but this patient was previously diagnosed with lupus and was on immunomodulators for advanced lupus nephritis. This demonstrates that lupus should be considered in demographically appropriate patients presenting with infectious arthritis as we present a rare case of infectious arthritis secondary to M. catarrhalis as the inagural event in the diagnosis of SLE.

61 Improving sexually transmitted infection screening rate in inpatient pediatric patients

M Hood*

MM Michalopulos

M Bowden

University of Tennessee Health Science Center, Memphis, TN

Purpose of study

Sexually transmitted infections (STIs) are a group of infectious diseases that can cause acute illness and life-long health effects. Nearly half of all diagnosed cases occur in adolescents and young adults, ages 15–24. The American Academy of Pediatrics and Centers for Disease Control provide guidelines for routine STI screening for all sexually active adolescents. Despite these recommendations, screening rates in adolescents admitted to our hospital are low. The purpose of this study is to use quality improvement methodology to improve screening for HIV, gonorrhea, chlamydia, and syphilis in adolescent patients in an inpatient setting.

Methods used

Eligible patients included all adolescents ages 14–18 admitted to our hospital over 12 months. Following baseline data collection, we introduced three interventions; rates of sexual history documentation and STI screening were recorded after each one. Interventions included providing resident education on the importance of STI screening, introducing prompts for sexual history taking in the History and Physical form, and providing residents with a ‘badge buddy’ as a sexual history taking template.

Summary of results

Prior to the intervention, 50% of patients ages 14–18 were asked questions regarding their sexual histories; 30% of patients were tested for STIs. After three interventions, sexual history taking improved to 70%, and STI screening rate improved to 53%. This represents an improvement in the rate of STI screening by 23%.

Conclusions

Simple interventions targeted at improving sexual history taking and knowledge of STI testing recommendations led to a significant increase in STI screening in an inpatient adolescent population.

62 Assessment of infant sleep habits in memphis, tennessee: unsafe practices continue

EM Karolczuk*

SR Arnold

A Marshall

J Yaun

Le Bonheur Children’s Hospital, Memphis, TN

Purpose of study

Despite safe sleep education provided to caregivers, the overall infant mortality rate in the US remains comparable to those of developing countries. In 2015, the infant mortality rate in Tennessee was higher than the national average, at 7/1000 live births, with Shelby County having the second highest infant mortality rate in the state at 8.2/1000 live births. Nearly 92% of these infant deaths occurred in the first six months of the infant’s life, and while cause of death in many children cannot be explained, most of the sleep-related deaths occurred while the infant was sleeping in an unsafe sleep environment.

Methods used

Infant sleep practices among caregivers with infants less than 6 months of age were assessed through a self-reported questionnaire administered in an academic general pediatrics clinic setting. 13 multiple choice and 4 true/false questions were used to obtain data on topics such as demographics, sleep location and position, access to safe sleep location, and bed sharing frequency.

Summary of results

Surveys were collected and analyzed from 91 respondents, of which 84% were African American and 51% had completed a high school education or less. 93% of caregivers reported owning a crib used solely for their infant. While 39% of caregivers reported never placing their child in a sleeping area other than a crib/bassinet at night, the remaining 61% admitted to at least sometimes placing their child elsewhere. Among these, 31% stated their children slept outside the crib less than 20% of the time. 41% of caregivers reported their infant sleeps with another child or an adult in a bed/chair at night at least some of the time. The majority (88%) of caregivers place their infants on their backs to sleep.

Conclusions

Though the majority of caregivers surveyed own a crib for their infant and place the infant on their back to sleep, many still report unsafe sleep practices such as co-sleeping and not always utilizing their dedicated safe sleep location. The larger issue in the surveyed population was not access to safe sleep location, but unsafe sleeping practices despite adequate materials. Physicians should ensure adequate parent education regarding risks of unsafe sleep practices and its relation to high infant mortality rates, and stress safe infant sleep practices for every sleep.

63 Evaluation of a model for adolescent sexual health education

J Robinson1*

R Pasternak1,2

1LSUHSC School of Medicine, New Orleans, LA

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

Purpose of study

New Orleans Adolescent Reproductive Health Project (NOARHP) teaches evidence-based sexual education to high school students in Orleans and Jefferson Parishes. Evidence suggests reproductive health education reduces teen pregnancy rates and STI/HIV transmission. The Centers for Disease Control and Prevention (CDC) ranked Louisiana sixth in the nation for teen birth rate and second for STI and HIV diagnoses. Our project focuses on developing a sustainable education model for students that contributes to reducing these rates.

Methods used

Our curriculum was evaluated by an adapted CDC Health Education Curriculum Analysis Tool: Module Sexual Health guidelines. Students were administered a 47-question test before and after the 9-lesson instructional intervention to determine mastery in Healthy Behavior Outcomes (HBO). Using a pretest-posttest design, we compared learning across four charter schools and determined the effects of curricular modifications. Additionally, pretest results were compared across schools to determine any significant difference of baseline knowledge before intervention. Improvement in test scores was also stratified based on HBO group, and improvement in each HBO category for each school was determined.

Summary of results

Average pretest grades showed significant difference in prior knowledge of sexual health between most schools. G.W. Carver (Spring 2014) scored 20.6%, Sci Academy 37.2%, G.W. Carver (Spring 2015) 26.6%, Kipp Renaissance 42.5%, and Lusher Charter 65.7%. Only the pairs G.W. Carver (Spring 2014)/G.W. Carver (Spring 2015) and Sci Academy/Kipp Renaissance did not show significant difference in pretest scores (p 0.382, p 0.502 respectively). The differences between pretest and posttest performance among the schools were: G.W. Carver (Spring 2014) 6.9% p 0.177, Sci Academy 18.1% p 0.001, G.W. Carver (Spring 2015) 0.001, KIPP Renaissance 7.1% p 0.013, Lusher Charter 11.2% p 0.001. Learning was also stratified based on HBO group. Increases in scores were observed in almost all questions across schools.

Conclusions

Improved knowledge on reproductive health were observed in all participating schools. NOARHP continues to refine the existing curriculum and educate adolescents with the goal to improve adolescent sexual health and normalize a culture of disease prevention.

64 More than weak bones: a case of vitamin-D dependent rickets with 1-alpha hydroxylase deficiency

B Saadat*

M Kleinman

UTHSC Memphis, Memphis, TN

Case report

A 7-month-old male who presented with respiratory distress and difficulty swallowing. He quickly underwent direct laryngoscopy, bronchoscopy, and frenectomy; however, he ultimately required endotracheal intubation. Initial chest x-ray showed diffuse osteopenia and multiple rib fractures suggestive of underlying rickets. Labs at admission: serum cal 7.1 mg/dL, ical0.95 mmol/L, PTH 1465pg/mL, alkaline phosphatase 4086units/L, 25OHD of 36.7 ng/mL, and 1,25(OH)2D of <10pg/mL. Pt was started on calcium, phosphorous, and calcitriol. Genetic testing was significant for CYP27B1 mutation. A diagnosis of vitamin D-dependent type 1 rickets secondary to 1-alpha hydroxylase deficiency was made.

Vitamin D-dependent rickets type 1A (VDDR 1A) is a rare autosomal recessive disorder associated with mutations in CYP27B1 gene which encodes 1-alpha hydroxylase. This enzyme converts 25-OHD to 1,25(OH)2D. Pts are healthy at birth and develop symptoms around 6 months of age. It is characterized by hypotonia and growth retardation. On imaging, individuals display enlarged metaphases, long bone bowing, and other signs of diffuse osteopenia As in our patient, lab findings include hypocalcemia, low 1,25(OH)2D, elevated PTH, and increased to normal serum 25OHD. Treatment of VDDR 1A is calcitriol—better known as 1,25(OH)2D. With adequate treatment, children are seen to have restoration of muscle tone, improvement of radiographic findings, and achievement of milestones.

Abstract 64 Figure 1

Diffuse osteopenia with bilateral rib fractures Diffuse osteopenia with bilateral rib fractures

65 Using 24-hour ambulatory blood pressure monitoring to optimize the treatment of pediatric hypertension

K Singhapakdi*

D Preud’homme

University of South Alabama, Mobile, AL

Purpose of study

Pediatric hypertension is a strong predictor of future end-organ damage, making it critical to optimize treatment. One-time blood pressures are routinely obtained at office visits; however these are subject to inaccuracies such as white coat hypertension. 24-Hour Ambulatory Blood Pressure Monitoring (ABPM) circumvents this but is not routinely performed in the pediatric population. This study describes our institution’s experience using ABPM to optimize our management of pediatric hypertension.

Methods used

This is a retrospective chart review of patients with treated HTN who received 24-hour ambulatory blood pressure monitoring (ABPM) due to persistent elevated blood pressures.The ABPM data included awake and asleep blood pressures, MAP and blood pressure load. We then reviewed the subsequent management of their medications.

Summary of results

Our study included 31 ABPM performed on patients with HTN. 70% were obese with an average BMI of 30.9 kg/m2. The mean 24-hour systolic blood pressure for the obese patients was 120 mmHg (118 mmHg in the healthy patients). In the obese patients 68% of the blood pressures were abnormal (22% in the healthy patients). 40% of the obese patients had an abnormal echocardiogram (25% in the healthy patients). The mean blood pressure load was 37.7% in the obese patients (20.4% in the healthy patients).

Of the patients already on blood pressure medication, 17 had abnormal ABPMs. They all subsequently had their medications increased by cardiology. We therefore conclude that ABPM can help optimize the treatment of pediatric hypertension. ABPM may be especially valuable in obese patients due to their risk of cardiac remodeling.

Conclusions

Isolated blood pressure measurements at clinic visits are not sufficient to diagnose or optimize the treatment of pediatric hypertension. 24-hour blood pressure monitoring is a somewhat expensive test and may not always be covered by insurance; however there is no doubt that in these patients this test led to a significant improvement of the management of their hypertension. We therefore conclude that ABPM can help optimize the treatment of pediatric hypertension and may be especially valuable in obese patients due to their risk of cardiac remodeling.

66 Right supraclavicular lymphadaenitis caused by kikuchi-fujimoto disease

G Toor1*

K Mather1

A Karikari1

G Kirkpatrick2

1University of Oklahoma, Tulsa, OK

2Saint Francis Hospital, Tulsa, OK

Case report

Kikuchi-Fujimoto disease is a rare and self-limiting condition with unknown etiology that is most commonly seen in young Asian women. It usually presents with low grade fever that varies from one week to one month, cervical lymphadenopathy between 2–7 cm, and other non-specific symptoms including rash, arthralgias, fatigue, night sweats, and weight loss. Histopathology of the involved lymph node typically differentiates it from more serious, life-threatening conditions that mimic similar clinical features, such as lymphoma.

Case Description

We present the case of a previously healthy 15-year-old, African-American male, with a two week history of fatigue, low grade fever, and cervical lymphadenopathy with a prominent right supraclavicular lymph node. Lab work was significant for leukopenia, anemia of chronic disease, and mildly elevated ESR and LDH. Monospot testing was initially negative; however, EBV titers (both IgM and IgG) were found to be elevated. CT scan of the neck was consistent with bilateral cervical chain lymphadenopathy with a right supraclavicular lymph node measuring 2.5 × 1.6 cm. Peripheral smear was negative for blasts and immunotyping by flow cytometry was negative for lymphoid population. Supraclavicular lymph node excisional biopsy showed focal areas containing lympho-histiocytic infiltrate and immunochemistry stain with CD68 of the lymph node was positive for histiocytes, consistent with proliferative phase of Kikuchi-Fujimoto lymphadenitis.

Discussion

Here is a very rare case of Kikuchi-Fujimoto disease preceded by EBV in a young African-American male. Etiology is unknown but preceding viral etiology has been proposed, such as EBV, HHV-6, HIV, Parvovirus, or Parainfluenza, or even bacterial etiology with Yersinia enterocolitica or Toxoplasma. Although it is a self-limiting condition, patients should be followed within one to four months for recurrence and increased risk of developing systemic lupus erythematosus. This intriguing case of an uncommon cause of right supraclavicular lymphadenitis prompts investigation and recognition by physicians and pathologists for commonly misdiagnosing it as lymphoma and initiating treatment with cytotoxic agents.

67 Eating disorder screening in an urban pediatric clinic

MM Vickers1,2*

C Rikard1,2

E McCoy1,2

M Bowden1,2

1UTHSC, Memphis, TN

2LeBonheur Children’s Hospital, Memphis, TN

Purpose of study

Eating disorders are a heterogeneous group of psychiatric illnesses that occur in 1–7% of adolescents. They are associated with many medical comorbidities and have the highest likelihood of death among all psychiatric disorders. Despite these risks, they are under-diagnosed in adolescents both at the national level and in our local clinic setting. The purpose of this study is to establish the feasibility of screening in our clinic, estimate our population’s prevalence, and identify high-risk groups for further studies.

Methods used

Patients ages 10–21 years presenting to our general pediatric or gastroenterology clinics were screened for eating disorders using the SCOFF questionnaire, a validated 5-question survey targeting food-related behaviors and body image. After identifying patients with positive screens, we performed a retrospective review of secondary characteristics, such as BMI percentile, gender, age, and chief complaint for office visit. Each patient with a positive screen was notified and referred to adolescent clinic.

Summary of results

Of the 452 patients surveyed, 43 (9.5%) had positive screens with an average age of 13.7 years and average BMI of 28.4 (BMI percentile of 83.8). 16 of the positive screens were male (37%). The most common chief complaint was well child visit; there were 4 positive surveys with the chief complaint of abdominal pain. Of the positive screens, the mean number of ‘yes’ answers was 2.3, most commonly ‘do you worry you have lost control over how much you eat.’ Less predictably, a third of positive screens were from males and half were from obese patients.

Conclusions

The SCOFF questionnaire was successfully implemented into both clinics, and the percentage of positive screens surpassed the national average of eating disorders. This study proves feasibility and usefulness of SCOFF screening in our clinics and indicates the likelihood of under-diagnosis of eating disorders in our patient population. Further, it prompts providers to recognize that these patients may not have a low BMI, be female, or have food-related behaviors such as restriction but rather binging. Additional investigation is needed into long-term follow up in adolescent clinic and eventual diagnosis.

Adult Case Reports/Aging/Geriatrics

Joint plenary poster session and reception

4:30 PM

Thursday, February 13, 2020
68 De novo pulmonary embolism in a nursing home resident after discontinuing aspirin: a case report

F Abu-Farsakh*

M Chavez

EA Aguilar

L Ali

TM Reske

R Pattabhi

LSU Health Sciences Center in New Orleans, New Orleans, LA

Case report

An 86 year old Caucasian female, resident in a nursing home, with history of Parkinson disease, dementia related to Parkinson’s disease, and age-related osteoporosis, has decreased mobility and dependent edema in her lower limbs. She started to complain of blood oozing from her lower limbs. She discontinued Aspirin after taking it for many years. Ten days later, while working with the physiotherapist, the patient developed sudden-onset shortness of breath, her O2 saturation was 82% on room air, BP 158/99, HR: 92, RR: 30. Her chest and heart examination was unremarkable, except for the tachypnea.

Laboratory workup showed hemoglobin level 10.9 g/dL, platelet count 210/mcL, the metabolic profile was unremarkable, however, BNP level was 878. CT chest showed sub massive pulmonary embolism in right main pulmonary artery. Her EKG also showed right axis deviation and incomplete right bundle branch block, and the echocardiogram showed systolic flattening of interventricular septum consistent with right ventricular pressure overload, and severe right ventricular enlargement with mild to moderate tricuspid regurgitation and PA pressure of 75 mm Hg. Doppler ultrasound of bilateral lower limbs showed no evidence of DVT in lower extremities. She was given rivaroxaban and discharged on 4 L/min oxygen via nasal cannula.

In elderly population, bed rest is the most frequent risk factor for DVT and PE (15 – 67%). DVT is detected in only 15–50% of patients with PE, which means that older adults are at increased risk of developing de novo PE even in the absence of DVT. Despite the paucity of data regarding the use of aspirin as a prophylaxis for VTE and PE in high-risk patients, it is worthy to study the benefits and risks of using aspirin, low dose vs. high dose, in elderly population as prevention for thromboembolism.

69 All hip pain is arthritis...or is it?

M Barnhart*

M Kleinman

University of Tennessee, Memphis, TN

Case report

Complaints of hip pain seem ubiquitous. Causes include trauma, arthritis, gout, bursitis, synovitis and osteonecrosis. This case presents an unusual diagnosis for hip pain. An 80 yo M with a history of hypertension, IBS-D, and mild dementia presented to his PCP with hip pain. The pain was in the right anterior, upper thigh. It worsened after initiating a new walking regimen. It was worse at night and with leg rotation. He denied any swelling, knee pain, gait changes, weakness, or parasthesias. Exam was unremarkable: no edema, negative straight leg, strength 5/5, full range of motion and sensation intact in bilateral lower extremities, patellar reflexes 2+, negative Babinski. He was initially managed with physical therapy, and gabapentin. Due to persistent pain, we referred him to Orthopedics. He received a steroid injection without relief. EMG studies and x-ray were normal. Hip MRI showed a large mass (11 × 13 cm) in the right retroperitoneum adjoining the psoas muscle and displacing the kidney, concerning for a neoplastic process. CT chest/abdomen showed no evidence of distant metastasis. He had neo-adjuvant radiation and surgical resection of the tumor and right nephrectomy. Pathology showed well-differentiated liposarcoma. Repeat CT 5 months later showed no recurrence. This patient presented with hip pain due to lumbosacral nerve compression from a large retroperitoneal liposarcoma. These malignant tumors typically have vague presentations: abdominal/flank pain, early satiety, and lower extremity swelling. Local compression causes musculoskeletal, neurologic, and obstructive GU symptoms, however, the majority are asymptomatic and found incidentally.

Abstract 69 Figure 1

Retroperitoneal liposarcoma Retroperitoneal liposarcoma

70 Mesenchymal stem cell therapy for rotator cuff injury: a meta-analysis of animal and human studies

DC Carlisle*

N Morton-Gonzaba

A Moreira

C Emukah

K Chorath

University of Texas Health and Science Center at San Antonio, San Antonio, TX

Purpose of study

To systematically review and investigate the impact of mesenchymal stem cell (MSC) therapy on function and repair of rotator cuff injury (RCI).

Methods used

Data sources: Five databases through November 2019.

Study selection: Human and animal studies examining MSC treatment for RCI. Three authors independently screened articles, titles, and abstracts. The protocol was registered with CAMARADES and adheres to the SYRCLE guidelines.

Primary outcomes: Preclinical: biomechanical metrics (ultimate load at failure, stiffness), Clinical: re-tear rates and shoulder imaging.

Secondary outcomes: histologic analysis, gene/protein expression of inflammatory/fibrotic/regenerative factors.

Statistics: Continuous data was pooled and expressed as standardized mean difference (SMD) with 95% confidence interval (CI).

Summary of results

858 abstracts were screened.

Preclinical: 19 animal studies were included; three-fourths of the MSCs were derived from adipose/bone-marrow tissue. All studies administered a single dose of MSCs at the time of surgical tendon detachment. Dose ranged from 500k to 300M cells. MSC treatment improved ultimate load failure [SMD 0.43, 95%CI (0.24, 0.62), p<0.01] and trended towards improved stiffness [SMD 0.20, 95%CI (0.42, -0.01), p=0.06].

Subgroup analysis favored autologous administration of adipose-derived MSCs; post surgical biomechanic assessment within two weeks; >10 million cell dose; and the rat animal RCI model. In general, MSC’s abated genes/proteins involved with inflammation, fibrosis, and improved wound healing.

Human studies: Six studies were included in the review comprising 319 patients. MSC tissue sources included adipose, bone marrow, and umbilical cord blood. All studies administered a one-time local dose of MSCs at the time of surgical repair. Overall, MSC therapy was safe, decreased rotator cuff re-tears, and in some studies decreased pain and improved functional outcome.

Conclusions

Use of MSCs for rotator cuff injury suggests a promising role in biomechanical metrics and wound healing. Despite these findings, decreasing heterogeneity, risk of bias, and optimizing MSC characteristics may encourage translational success.

71 High-grade B-cell lymphoma presenting as chronic osteomyelitis

HR Cintrón-Colón*

WD Marrero

V Fonseca-Ferrer

JG Rodriguez Velez

C Rivera-Franceschini

M Miranda

San Juan City Hospital, San Juan

Case report

A 64 y/o male patient w/medical history of chronic left leg ulcer presented to the emergency department complaining of further progression in ulcer size noticed more drastically during the past few months. He refers increase on lesion fetidness, size, and bleeding, but denied any constitutional symptoms or complaints. Physical examination was remarkable for hepatosplenomegaly, tachycardia, fever, left lower extremity ulcer comprising both fibular and tibial sides completely, and left ankle chronic lymphedema. Laboratory work-up was remarkable for normo-normo anemia with a hemoglobin of 6.5, and an ESR of 20. No further disturbances were noticed. Blood products were immediately transfused without any complications. Chest X-ray on admission day was found within normal limits; left lower extremity X-ray showed soft tissue swelling with thick periosteal reaction. Surgeon was consulted and provided debridement and enterostomal care of area. However, before proceeding with surgery, MRI of the leg was conducted to rule out osteomyelitis. Official report showed multiple lytic bone lesions highly suggestive of malignancy. Obtained Bone biopsy showed changes consistent with chronic osteomyelitis. A few days later, patient started presenting with shortness of breath and new chest x-ray was obtained, demonstrating large bilateral pleural effusion and a small speculated lesion abutting from pleura. Thoracentesis was performed and exudate without neoplastic cells was obtained. Biopsy from speculated lesion was also obtained and immunohistochemistry was positive for c-myc, bcl-6, CD45, CD20, CD79a, and Ki-67, consistent with High-Grade B-Cell Lymphoma.

Chronic osteomyelitis unresponsive to appropriate management should raise concern for an alternate diagnosis as the main etiology for presentation. It is important for physicians to be aware of the possible association between chronic inflammation and HGBCL since prompt identification will lead to early initiation of appropriate medical management, thus, resulting in better prognosis.

72 It’s not my immunity! a case of syphilitic membranous glomerulonephritis

EM Clark1,2*

P Hirachan2,3

1LSU Family Medicine Residency – Alexandria, Alexandria, LA

2Rapides Regional Medical Center, Alexandria, LA

3Cenla Kidney Specialists, Alexandria, LA

Case report

A 22-year-old white female with a past medical history of celiac artery stenosis, renal calculi, herpes, and methamphetamine abuse presented with three days of worsening abdominal and flank pain with associated nausea and vomiting.

Physical examination was unremarkable except for mild CVA tenderness. Lab results revealed a drug screen positive for methamphetamines. Urinalysis showed >500 mg/dL protein. Serum creatinine peaked at 4. An abdominal ultrasound revealed enlarged bilateral echogenic kidneys. Immunologic studies were unremarkable. A kidney biopsy revealed membranous glomerulonephritis with negative PLA2R suggesting secondary etiology. Infectious studies showed RPR titers at 1:128. FTA/BAS was confirmatory for syphilis.

She received three doses of steroids before kidney biopsy for possible glomerular pathology. Due to the patient’s known penicillin allergy and history of noncompliance, the patient underwent penicillin desensitization and treated for secondary syphilis. Renal function continued to improve.

In the handful of reported patients with this particular presentation, all were immunolgically suppresed. This should be a differential for patients despite immunologic status.

Abstract 72 Figure 1

Renal biopsy of patient. Subepithelial deposits visualized, evidence of membranous glomerulonephritis Renal biopsy of patient. Subepithelial deposits visualized, evidence of membranous glomerulonephritis

73 A curious case of anemia

MS Cross1,2*

K Vyas1,2

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

2Central Arkansas Veterans Healthcare System, Little Rock, AR

Case report

Anemia is commonly encountered in the outpatient setting with a prevalence of roughly 15 million cases in the United States. Though internists frequently encounter iron deficiency anemia, we must remain vigilant to accurately diagnose more complex cases.

This is the case of a 33-year-old Hispanic man who presented to the ED from his primary care clinic for further evaluation of anemia. He reported daily nausea, vomiting, non-bloody diarrhea, and fatigue for the past month. His trial of Bismuth subsalicylate did not provide relief. He related a 35lb unintentional weight loss over the same time. Additionally, he reported non-productive cough and intermittent 5-minute episodes of aching, non-radiating, peri-prandial chest pain present for one month. The patient was diagnosed with iron deficiency anemia 2 weeks prior and had been started on ferrous sulfate. He was also diagnosed with H. pylori and started on triple therapy. On exam, T 99.9F BP 110/68 HR 106 RR 25 SpO2 100% on room air. The patient appeared thin, but well with the exception of pale conjunctivae. Initial laboratory data included leukocyte count 1.65 (ANC 0.8), hemoglobin 6.8, and platelet count 120,000. Iron studies demonstrated Fe saturation 61%. HIV and HCV screen were both positive. Chest radiograph revealed mediastinal lymphadenopathy. Subsequent chest CT confirmed mediastinal lymphadenopathy with increased micronodularity throughout the lungs. Beta-D-glucan was normal. Histoplasma antigen in urine was positive. Serum reticulocyte count was depressed, so bone marrow biopsy was performed. Pathology revealed marked erythroid hypoplasia, atypical erythrocytes and atypical lymphocytes. Immunostaining was negative for Histoplasmosis and TB but was positive for Parvovirus B19 and serum qPCR was markedly elevated >1.38E+10. Norovirus was also detected in his stool. The patient was newly-diagnosed with HCV, HIV, disseminated histoplasmosis, parvovirus B19 with aplastic crisis, and norovirus gastroenteritis. He was treated with liposomal amphotericin and itraconazole for histoplasmosis, received IVIG for parvovirus, started on anti-retroviral therapy for HIV, and managed symptomatically for norovirus.

74 A case of oculomotor nerve palsy in an uncontrolled diabetic

D Pawar*

I Matthews

B Rogers

N Khandelwal

K Nugent

Texas Tech University Health Sciences Center, Lubbock, TX

Case report

Up to 1% of diabetic patients may experience cranial nerve mononeuropathy, characterized by abrupt onset, pain, and spontaneous recovery in 3–12 months. Cranial nerves III, IV, VI, and VII are most commonly affected. However, because the extraocular muscles are often only partially paralyzed, the clinical presentation is often diverse and identification of affected cranial nerve (CN) is a challenge [1]. The differential diagnosis includes giant cell arteritis (GCA) and posterior communicating artery (PCA) aneurysm, which must be ruled out. Treatment consists of strict glucose control and medication adherence. A thorough history, along with complete neurological and ophthalmological assessments is critical to diagnosis. An isolated pupil sparing CN III, IV, or VI palsy, in a patient age >50 years, erythrocyte sedimentation rate (ESR) <30 and normal c-reactive protein (CRP) with pre-existing vascular risk factors favors a microvascular cranial nerve neuropathy.

Case presentation

A 63-year-old female with a history of type 2 diabetes, migraine, hypertension, and dyslipidemia who presented with left-sided ptosis, diplopia headache, and facial pain. Vital signs were within normal limits. Physical exam was significant for near-complete ptosis of the left eye, slight abduction with markedly reduced supraduction, adduction, and no infraduction. Her blood glucose was significantly elevated at 485 and hemoglobin A1C was 11.6. Lipid panel showed total cholesterol 381, LDL 136, and triglycerides 904. ESR and CRP were within normal limits. Neuroimaging studies including CT, CTA, MRI, and MRA were unremarkable. She was treated with butalbital/acetaminophen/caffeine and Verapamil however, pain remained refractory. Based on her symptoms and diagnostic test, she was diagnosed with CN III palsy secondary to ischemic vascular disease from uncontrolled diabetes. She was started on a long and short-acting insulin regimen as well as an oral sulfonylurea and counseled on medication adherence.

Conclusion

Cranial nerve neuropathies are rare complications of type 2 diabetes, secondary to microvascular ischemia. Careful history taking, neuroimaging, or biopsy is essential to diagnosis. Spontaneous recovery is seen within months with strict glucose control.

75 Multiple cavities post influenza not responding to treatment. What can it be?

N Rus1*

MF Habib2

AA Kamat1

RI Hazam1

N Elzubeir1

T Naguib1

1Texas Tech Univ HSC Amarillo, Amarillo, TX

2Amarillo Cardiovascular Center, Amarillo, TX

Case report

Influenza increases mortality and morbidity through complications such as acute lung injury and secondary bacterial pneumonia. We report a case of multiple cavitary lung lesions with Influenza A not responding to usual treatment.

A 58-year-old homeless female with chronic obstructive pulmonary disease presented with upper respiratory tract infection symptoms. Computed Tomography (CT) showed extensive bilateral consolidative changes. She required noninvasive ventilation for oxygenation. She was treated with vancomycin, piperacillin/tazobactam and oseltamivir for influenza A positive lung consolidation. After initial improvement, she developed respiratory distress and fever. Repeat CT scan showed extensive bilateral necrotizing lung lesions. Bronchoscopy revealed bilateral purulence. A diagnosis of community acquired methicillin resistant staphylococcus aureus (CA-MRSA) necrotizing pneumonia in the setting of Influenza A was made, based on sensitivity to clindamycin and trimethoprim/sulfamethoxazole (TMP/sulfa) of the organism isolated from both blood and respiratory secretions. Antibiotics were changed to linezolid. The overall condition improved, and she was discharged to a long-term acute care facility.

Necrotizing pneumonia due to CA-MRSA is rare and thought to be related to the production of Panton-Valentine Leucocidin (PVL) toxins that cause destruction of immune cells leading to tissue damage. The presence of type IV staphylococcal cassette chromosome in CA-MRSA allows it to remain susceptible to TMP/sulfa and clindamycin. Studies showed vancomycin monotherapy to have higher failure rate. Both linezolid and clindamycin inhibit protein synthesis leading to less production of PVL, reducing mortality in CA-MRSA pneumonia. Our patient also initially deteriorated on vancomycin but improved with linezolid.

CA-MRSA necrotizing pneumonia is a rare but fatal disease in combination with influenza. It is important to keep a high suspicion for CA-MRSA pneumonia in influenza cases with rapid decline in their respiratory status. Hence, we suggest empiric treatment with linezolid instead of vancomycin in patients with suspected PVL toxin producing CA-MRSA.

76 Bladder perforation with foley catheter

F Smith*

D Vangipuram

K Nugent

Texas Tech University Health Sciences Center, Lubbock, TX

Case report

Foley urinary catheters are commonly used in hospitals across the country; important complications include infection. Foleys have also been rarely reported to cause bladder rupture. Bladder rupture is typically associated with trauma or a complication from surgery or endoscopy. Only 1.6% of blunt abdominal traumas result in bladder injury, and spontaneous injury is even more rare. Risk factors for spontaneous injury include carcinoma, chronic cystitis, chronic catheterization, and outflow obstruction. We present here a case of Foley catheter-induced bladder rupture.

Summary of results

A 67-year-old Hispanic male nursing home resident with history of HTN, HLD, CAD, and stroke who is dependent on indwelling Foley for incomplete bladder emptying presented to emergency room with 2-day history of decreased responsiveness noticed by the caretakers at his nursing home. His initial workup suggested sepsis. Previous Foley was improperly placed, and possibly inflated in the urethra. Computed tomography scan on admission showed a Foley deeply inserted and pushing against the dome of the bladder. Urology was consulted and said no intervention was necessary unless the Foley stopped working. The patient soon decompensated and required central line placement for vasopressor support and intubation. The patient’s hemoglobin trended downward, so a repeat CT scan was ordered to assess for bleeding. The CT scan indicated the Foley catheter tip and bulb were extralumenal and superior to the bladder. Urology was again consulted and suspected small perforation in the dome of the bladder caused by the Foley. The Foley was repositioned and attached to intermittent wall suction. For the rest of his hospital stay, the patient was treated for sepsis with Escherichia coliand Proteus mirabilis. He steadily improved and did not require surgery.

Conclusion

Foley catheters have an often-overlooked risk of bladder perforation. Bladder rupture has high morbidity and mortality; so prompt diagnosis and management are imperative. Large bladder ruptures require surgical correction. Successful conservative, nonsurgical management of small intraperitoneal bladder ruptures have been reported previously and was demonstrated in our case.

77 Giant bladder stone: a very rare finding in clinical practice

A Tikue*

G Bedanie

M Zitun

A Amatullah

E Islam

Internal Medicine, Texas Tech University Health Sciences Center, Lubbock, TX

Case report

A giant bladder stone is a rare urinary tract stone not commonly seen in clinical practice. It is defined as a stone weighing more than 100 grams and measuring more than 4 cm along its longest dimension.

We are reporting a case of a giant bladder stone (measuring 8 cm × 6 cm × 6 cm) associated with bilateral hydronephrosis, renal insufficiency, left renal staghorn stone, and septic shock.

Case summary

A 57-year-old male without significant past medical history was transferred to our hospital and admitted to the MICU with the diagnosis of urosepsis and septic shock. He presented with fever, chills, dysuria, and left flank pain.

Upon evaluation, he was pale and acutely sick looking. His blood pressure was 99/55 mmHg, heart rate 102 beats/minute and temperature of 38 C.

Laboratory testing revealed WBC count 27,000 cells/mm3, serum creatinine 2.8 mg/dl. Urinalysis was positive for nitrates, it showed WBC count of >182/HPF and RBC 32/HPF. Urine and blood cultures grew methicillin-sensitive Staphylococcus aureus.

CT renal stone protocol revealed bladder stone 8 cm × 6 cm × 6 cm, bilateral hydronephrosis, and a left kidney staghorn stone filling the left renal collecting system. Transthoracic echocardiogram showed aortic valve vegetation and abscess.

He was started on IV fluids, vasopressors and broad-spectrum IV antibiotics for septic shock. Bilateral decompressive nephrostomy tubes were placed, and cardiothoracic surgery planned intervention after controlling the sepsis. But patient remained critically ill and died of uncontrolled sepsis and persistent shock.

Conclusion

Giant bladder stones have virtually disappeared from modern literature due to increased awareness of conditions leading to urinary tract stone formation.

This report aims to showcase that this rare clinical presentation can develop in the absence of clear predisposing factors related to bladder outlet obstruction, and in an area where patients have access to medical facilities.

Accurate diagnosis along with relieving any urinary obstruction, eliminating the infection, and meticulous surgical technique are essential to proper treatment.

78 The history, exam, and diagnostic tools, it’s not just one or the other

K Wilson*

H Tran

C Gregg

P Lowery

S Brown

E Peirce

LS Engel

R McCarron

LSU Health Sciences Center, New Orleans, LA

Case report

A 64 year old man with a history of hypertension, anxiety, treated hepatitis C, and a previous cervical spine fusion was evaluated in the emergency room for chest pain that began the morning of his presentation. He experienced a sudden onset of substernal crushing chest pain that radiated to his left arm when he stood up. He endorsed feeling diaphoretic, nauseous, and his colleague described his appearance as pale. Rest did not improve the symptoms, walking did not worsen the pain, and after 20 minutes the symptoms completely resolved without intervention. On evaluation, his vital signs, physical exam and lab findings were unremarkable. BNP was 49, initial troponin was <0.006, and his EKG showed normal sinus rhythm with no T wave or ST changes. TTE was showed normal EF with no wall motion abnormality or diastolic dysfunction. Treadmill stress test was inconclusive as he was unable to participate for greater than 2 minutes due to shortness of breath and his inability to achieve the targeted heart rate. Cardiology was consulted due to his significant risk factors and performed a left heart catheterization that demonstrated 40% occlusion of his proximal LAD, 70–80% occlusion of his mid LAD, 70–80% occlusion of his left circumflex, and 80% occlusion of his RAD. No stents were placed at that time, and he was referred to Cardiothoracic Surgery. He opted to not undergo surgical intervention and elected to have drug eluting stents placed. He has remained asymptomatic since his presentation.

Discussion

In a medical world that continues to evolve with ease of access to laboratory studies and imaging modalities, it is still important for any physician to obtain an adequate history. Despite a serial of normal EKGs and non-elevated troponins, our patient’s clinical scenario was a ‘classic’ story for acute coronary syndrome. Without an adequate history and exam, treatment based off of laboratory findings could have resulted in our patient being discharged without diagnosis of his three vessel coronary artery disease.

Allergy/Immunology/Rheumatology/Inflammation

Joint plenary poster session and reception

4:30 PM

Thursday, February 13, 2020
79 A case of isolated oculomotor palsy in systemic lupus erythematosus

G Maniam

R Dean*

I Ivyanskiy

N Davey-Ranasinghe

R Chandra

M Saadaldin

M Souliman

T Naguib

Texas Tech Univ HSC Amarillo, Amarillo, TX

Case report

Systemic lupus erythematosus (SLE) is a systemic autoimmune disease of unknown etiology, though genetic and environmental factors are widely believed to play a role in the pathogenesis. When it affects the central nervous system (CNS) as neuropsychiatric systemic lupus erythematosus (NPSLE), it typically presents as headaches, mood disorders, cognitive impairment, and even seizures. Isolated cranial nerve palsies are rare. Recognition and management of such cases can present a diagnostic challenge to clinicians. We present a case of isolated CN III palsy in a patient with previously quiescent SLE.

Case presentation

A 47-year-old woman with lupus in remission presented with diplopia and ptosis of two days duration Only other symptoms were headache and fatigue. She had previously refused therapy with hydroxychloroquine due to concerns of retinopathy as an adverse effect. Her disease was thought to be in remission due to mild symptoms earlier. CTA and MRA were negative for any significant abnormalities. ANA was positive with low titer dsDNA, and low C4 with +1 proteinuria suggestive of SLE. Treatment with pulse steroids followed by oral steroids taper was utilized. A noticeable improvement followed. However after five days no further improvement took place. She was then discharged on hydroxychloroquine therapy and oral steroids, with outpatient rheumatology care.

Discussion

Diagnosis of neuropsychiatric manifestations in SLE requires clinical suspicion upon the development of CNS symptoms in the context of existing SLE. Upon exclusion of other etiologies, the neurological work-up with CSF analysis, neuroimaging via CT/MRI, and serum studies can aid in the diagnosis of NPSLE. Previous studies suggest treatment of NPSLE CN III palsy with pulse corticosteroid therapy. Our patient had clinical clear improvement upon administration of high dose pulse methylprednisolone. This case emphasizes the importance of clinical recognition in NPSLE, even when faced with exceedingly rare CNS manifestations of the disease such as isolated CN III palsy.

80 Social media and a case of anaphylaxis

D Matrana

E Smith

G Myers

LS Engel*

S Kamboj

LSU Health Sciences Center, New Orleans, LA

Case report

Alpha Gal Syndrome (Tick Induced Meat Allergy) is a rare cause of delayed anaphylaxis that develops weeks to months after a tick bite following ingestion of mammalian meats.

Case

A 28-year-old woman without significant past medical history presented to the Allergy/Immunology clinic with concerns of having alpha gal syndrome. Three months prior she was bitten by a tick while hiking in Northwest Georgia. She reports about four hours after ingestion of a pepperoni pizza, she woke up experiencing wheezing, shortness of breath, and hives all over her body. She took Benadryl and by the morning her symptoms had completely resolved. A few weeks later, she ate a cheeseburger, and approximately four hours later, developed similar symptom. Coincidentally, she heard a podcast about meat allergy and the symptoms described matched her symptoms, prompting her to seek medical evaluation. As her history and presentation were consistent with Alpha Gal syndrome, a specific IgE level to Alpha-Gal was ordered and found to be elevated at 46.3 IU/mL. A diagnosis of Alpha Gal syndrome was confirmed, and she was instructed to avoid beef, pork, lamb and other mammalian meats.

Discussion

The diagnosis of alpha gal was initially discovered in 2006. Approximately three months after a Lone Star tick bite, there is a immunoglobulin class switch that is thought to occur in the skin that leads to the development of an IgE antibody to alpha-1,3-galactose, a carbohydrate found on mammalian meats. Through the use of public awareness regarding tick bites, various news outlets and social media platforms have increased awareness of this rare disease. When produced correctly and factually accurate, social media can be a powerful tool to help raise awareness to rare diseases.

81 Os styloideum resection complicated by severe contact dermatitis to dermabond

B Googe*

C Cushing

University of Mississippi Medical Center, Jackson, MS

Case report

Os styloideum is an accessory wrist bone at the base of the third metacarpal that can cause chronic pain from development of osteoarthritis and tendon irritation.

A 29-year-old surgical resident and golfer presented to clinic for a firm dorsal hand mass at the base of the third metacarpal. The mass had been present for 18 months and slowly enlarged causing chronic pain worsened by tendon excursion across the mass during motion and was unimproved by conservative management with splinting and steroid injections. CT revealed 1 cm os styloideum. The patient was taken to OR for mass resection. Closure was performed with buried Monocryl followed by Dermabond and a volar resting splint. On POD#6 the splint was removed, and the patient was found to have a well-demarcated, severe dermatitis with erythema and bulla formation overlying the incision site extending across the dorsal surface of the hand. The skin sloughed along much of the surface revealing full thickness injury over the incision and deep partial thickness injury to the ulnar aspect of the hand. Remaining glue was removed and the patient was treated with methylprednisolone dose pack and local wound care with xeroform dressing changes. A secondary closure was performed on POD#25.

The presented case demonstrates two unusual diagnoses: a rare cause of hand mass and the severe contact dermatitis to Dermabond. Resection is treatment for a symptomatic os, but caution should be used when applying Dermabond under a splint. Removal of glue and steroids should be considered for severe contact allergy.

Abstract 81 Figure 1

Os styloideum (arrow) at base of 3rd metacarpal Os styloideum (arrow) at base of 3rd metacarpal

82 Recurrent thromboembolism in antiphospholipid syndrome patient despite anticoagulation with direct- acting oral anticoagulats

RI Hazam1*

A Kamat1

C Ashangari1

J Kimbugwe1

N Elzubeir1

O Bazzaz2

N Rus1

1Texas Tech University Health Science Center, Amarillo, TX

2Thomas E. Creek VA Medical Center, Amarillo, TX

Introduction

Available evidence suggests DOACs like Apixaban may be less effective than warfarin in the secondary prevention of thrombotic events in high-risk antiphospholipid syndrome patients.

Case presentation

A 69-year-old male with a past medical history of mild interstitial lung disease, Antiphospholipid syndrome (APLS), cerebrovascular accident and chronic kidney disease stage III-IV. Patient presented with dyspnea, pleuritic chest pain for 3 days, has no cough or sputum production or fever. Vital signs, oxygen saturation of 86% in room air other vital sign were within normal. Physical exam and Labs were unremarkable. Chest x-ray showed slight worsening of left lower lobe fibrotic changes. Patient was diagnosed with acute hypoxic respiratory failure secondary to community acquired pneumonia. he required a venti Mask. Patient was diagnosed with APLS in 1980s. Since then he was maintained on warfarin for secondary prevention. Three months prior to this admission, he was switched to apixaban. with lack of clinical findings of pneumonia and Despite being on apixaban suspicion for pulmonary embolism (PE) was raised. V/Q scan showed high probability for PE. Apixaban and Antibiotics were discontinued and the Patient was started on warfarin. Patient improved clinically, and his oxygen requirement decreased down to 2 liter of nasal cannula.

Discussion

The available evidence suggests that DOACs are less effective than warfarin for thrombosis prevention in patient with APLS, particularly among patients who are considered high risk; patient tested positive for all three antiphospholipid antibodies and those with a history of arterial thrombosis like our patient.

In our case the PE diagnosis was made with V/Q because of elevated creatinine. Although a normal chest radiograph is usually required prior to V/Q scanning scan interpretation, our patient had a high clinical probability and paired with a high-probability scan we had a 96 percent chance of the patient to have a PE.

In patient with APLS on DOAC, the clinician should not rule out PE as clinical suspicion raised.

83 Recurrent urticaria and angioedema in a patient with graves’ disease, association or co-incidence?

MB Khalid*

O Yahya

R Peravali

R Usman

A Kumar

University of Tennessee Health Sciences Center, Memphis, TN

Case report

A previously healthy 42-year old female presented with abrupt onset lower lip swelling and intensely pruritic rash. She also reported symptoms of heat intolerance, eight pounds weight loss, and palpitations for one month. Vital signs were within normal limits. Physical examination was notable for urticarial rash involving back and both forearms, fine hand tremors, and lower lip swelling without evidence of macroglossia or airway compromise. She did not manifest any signs of ophthalmopathy. She reported no recent medications use, insect bites, or family history of urticaria or angioedema. Initial workup revealed low TSH: <0.01 mU/L, elevated free T4: 4.48 ng/dL, and elevated thyroid-stimulating immunoglobulin (TSI): 2.6% (Normal <1.3%). Radioactive iodine scan showed symmetrically increased uptake throughout the gland. She was given a working diagnosis of hyperthyroidism secondary to Graves’ disease. Additional workup was unremarkable, which included complement levels, C1 esterase inhibitor level and function, immunoglobulins, and an autoimmune panel including antinuclear antibody. She was treated with antihistamines and steroids with resolution of rash in less than 24 hours and resolution of angioedema in three days. Propylthiouracil and propranolol were initiated for the treatment of hyperthyroidism. The patient continued to have intermittent episodes of urticaria without recurrence of angioedema. Subsequent radioiodine ablation led to normalization of thyroid function and gradual resolution of urticarial episodes, with continued absence of symptoms at 6- and 12-month follow-ups.

Discussion

CSU is defined by the presence of hives with or without angioedema lasting for more than 6 weeks. A high prevalence of CSU in association with hypothyroidism has long been reported. We report a unique case that manifested with co-existing symptomatic hyperthyroidism, where symptoms including urticaria responded to radioiodine therapy. Current evidence suggests possible cross-reactivity of autoantibodies with complement pathway activation which triggers mast cells and basophils, leading to urticaria and/or angioedema. Further research is needed to explore the association between autoimmune thyroid disorders and CSU.

84 Lupus nephritis in a young male presenting with arthritis

W Kogler*

C Canha

AS Stack

C Isache

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

Case report

A 28 year old male with no medical history presented to the hospital with knee pain and swelling that had progressed over the last week with associated fever and chills. His physical exam was significant for joint swelling and erythema. He was febrile at 103.3. Labs were significant for a leukocytosis of 28, serum creatinine of 1.8, and hematuria on urinalysis. Arthrocentesis revealed a WBC of 13 with absence of crystals. Infectious work up was negative. ANA screen was positive with a homogenous pattern and a titer of 1:320. Anti-DS DNA antibody level was over 300. Renal biopsy showed endothelial tubuloreticular inclusions in the glomerular tuft with full house immune deposits along with small segment of fibrinoid necrosis in the tufts suspicious for class V disease. The patient was started on steroids and hydroxycholoroquine.

Systemic Lupus erythematosus (SLE) is a systemic autoimmune disease resulting in production of nuclear antibodies resulting in end-organ damage; most commonly affecting the kidneys, skin, heart, and joints. Lupus nephritis (LN) is present in 30–75% of cases. It is most common in women of child-bearing age but in very rare cases (4–22%) it can affect males. Because of how rare SLE is in the male population it is poorly understood. LN is a common manifestation in men, usually presenting as a nephrotic syndrome with histology consistent with class IV disease. To diagnosis LN you must first establish a diagnosis of SLE with kidney dysfunction. Kidney biopsy is diagnostic and can differentiate the histopathological class of LN, which is important to determine management and prognosis. Treatment involves high dose steroids during flares along with ant-malaria drugs and cyclophospomide; however different histopathological classes are treated differently. Prognosis can be affected by delay in treatment, which makes a high index of suspicion vital especially in males as it can be overlooked.

LN causes significant morbidity and mortality in SLE patients. Timely diagnosis and treatment is important in order to help delay progression. Given the rarity of SLE in males, this diagnosis can often be delayed leading to worse outcomes. A high index of suspicion is vital, especially in males so that accurate and timely diagnoses can be made.

85 Can sarcoidosis be acute? Lofgren’s syndrome in a farm worker

A Kunnumpurath*

S Singh

White River Health System, Batesville, AR

Case report

Saroidosis is a multi system granulomatous disease with an unknown etiology. Lofgren’s syndrome is an acute sarcoid arthritis, which presents as acute symmetric polyarthritis, bilateral hilar lymphadenopathy with or without erythema nodosum.

Case

41 y/o Caucasean male, farm worker was referred by his primary care physician for acute onset joint pain involving bilateral ankles, left knee and left ankle swelling which did not respond to NSAID’s. He also had a rash on his legs, which was diagnosed as cellulitis, treated with multiple course of antibiotics without much improvement. In the rheumatology clinic, he reported symmetric polyarthritis involving bilateral ankles and erythematous rash involving bilateral knees. His review of systems was positive for new onset cough, mild wheezing and myalgia. On exam - he had multiple tender subcutaneous nodules on shin and back of right leg consistent with erythema nodosum along with swelling and tenderness in left wrist and bilateral ankle’s. Laboratory work up including CBC and CMP were normal. He had a high positive ANA titer, ESR – 74 mm/hr and S.ACE elevated at 88U/L but negative RF, Anti CCP, Anti Ds DNA, Anti SSA and SSB antibodies, Anti Scl 70 antibodies. Chest radiograph showed bilateral hilar and paratracheal lymphadenopathy. Infectious work up was negative for CMV and EBV. Patient was started on Prednisone 60 mg daily to which he responded with symptom resolution. His ESR the following month improved to 40 mm/hr and S.ACE trended downwards. He was treated with tapering dose of prednisone, with complete resolution of his symptoms.

Discussion

Lofgren’s syndrome is diagnosed based on the clinical triad of symmetrical polyarthritis, bilateral hilar lymphadenopathy and erythema nodosum. It presents with acute onset symmetric oligoarthritis which can be confused with pseudodgout, gout, rheumatoid arthritis. The erythema nodosum can be confused with cellulitis as in our patient. High degree of suspicion in a young patient presenting with symmetrical polyarthritis and nodular rash helps make right diagnosiss.The prognosis is good as the disease is generally self-limiting and responds well to NSAID’s and steroids.

86 Hypovolemic shock as presentation of microscopic polyangiitis

T Pearson*

JJ Maguire

J Treece

East Tennessee State University, Johnson City, TN

Case report

Microscopic polyangiitis (MPA) is a rare condition characterized by nonspecific symptoms of fatigue, fever, or loss of appetite and additional symptoms related to areas of involvement. The rarity of the disease and its variable presentation can delay its diagnosis in the acute setting, potentially leading to delay in effective treatment and poor outcomes. This is a 68-year-old man, who presented to the hospital after being found poorly responsive at home. He was sedated and intubated in the field and brought to the ER. He was found to be hypotensive. History revealed abdominal aortic aneurysm, but no known history of GI bleed or use of blood-thinners. Physical exam revealed rhonchi throughout both lung fields, a soft, nondistended abdomen with normoactive bowel sounds, and cool extremities with weak palpable pulses. CBC was suggestive of anemia. BUN and creatinine were elevated indicating kidney injury. CT abdomen showed no acute pathology and an intact abdominal aortic aneurysm. CT chest revealed left lower lobe atelectasis and bilateral pulmonary edema. CT head was consistent with watershed infarction. Numerous blood transfusions were given. However, slow decline in hemoglobin continued to occur. Bronchoscopy was performed, revealing extensive mucus plugging and bloody plugs. He was eventually weaned off sedation but remained largely unresponsive, occasionally opening his eyes to stimuli. On hospital day 7, necrotic lesions were noted on the patient’s ear and fingers. These findings prompted serologic studies for vasculitis that resulted in positive p-ANCA and myeloperoxidase antibodies, consistent with MPA. He was started on pulse dose IV steroids resulting in modest improvement in renal parameters and hemoglobin, but no discernible change in neurological status. The patient was transitioned to comfort care and palliative extubation was performed. Four days later, the patient expired. This case illustrates the potential for hypovolemic shock as a consequence of undiagnosed vasculitis. Although this patient may not have recovered even with early immunosuppressive treatment, vasculitis should be considered as a differential diagnosis in patients presenting in hypovolemic shock, especially if history, exam and noninvasive testing are unrevealing.

87 It’s just a rash they said ! acute skin manifestations in a patient with vasculitis

K Sawalha*

AT Kunnumpurath

R Kamoga

White River Health System, Batesville, AR

Case report

Cryoglobulinemia is thought to be a rare condition with the most common clinical manifestations in >90% of patients being skin lesions, specifically orthostatic purpura and ulcers.

Case

76-year-old Caucasian male with a history of Cryoglobulinemic vasculitis,Raynaud’s phenomenon and pulmonary embolism on warfarin, presented with acute onset of rash in bilateral lower extremities. He had a one-week history of worsening bilateral lower extremity rash which initially looked like ‘a bad sunburn’. The rash eventually turned into clear blisters progressing into painful open sores that were oozing serosanguinous fluid.He was recently started on trimethoprim -sulfamethoxazole due to concern for cellulitis by his primary case physician. Our differential at this point included trimethoprim- sulfamethoxazole associated rash, warfarin induced skin necrosis, vasculitis, pyoderma gangrenosum and infection. Significant laboratory results included leukopenia with WCC 2.0 K/ul with ANC >1500/µL, INR 3.9, CRP 5.7 mg/dl, ESR 95 mm/hr, low C4 level at 4 mg/dl, normal C3 level at 95 mg/dl, Rheumatoid factor 557.1 IU/ml; positive ANA IgG and negative cryoglobulin, ANCA and hepatitis C. Venous and arterial doppler ultrasound were negative for arterial or venous thrombosis bilaterally. Patient was started on high dose steroids for acute vasculitis. Punch biopsy showed acute small vessel vasculitis with fibrin thrombin. Patient’s rash improved significantly and he was discharged on oral steroids.

Conclusion

Cryoglobulinemic vasculitis refers to a systemic inflammatory syndrome that generally involves small-to-medium vessel vasculitis due to cryoglobulin-containing immune complexes. Cutaneous manifestations develop in nearly all patients with cryoglobulin syndromes and may precede extra cutaneous manifestations by decades. Typically, the lesions consist of erythematous macules and purpuric papules of the lower extremities (90–95%) as infarctions, hemorrhagic crusts, and ulcers. Acute skin manifestations in vasculitis are difficult to diagnose as a clinician and going through the differential based on history and clinical presentation is crucial in making the right diagnosis. Often times, tissue diagnosis is required to make the right diagnosis.

88 Stemi in setting of undifferentiated vs. mixed connective tissue disease

V Silver*

K Oakden

H Tran

C Saraceni

LS Engel

M Modica

LSU Health Sciences Center, New Orleans, LA

Case report

Introduction: Mixed Connective Tissue Disease is a generalized connective tissue disorder characterized by the presence of high titer anti-U1 ribonucleoprotein (RNP) antibodies in combination with clinical features commonly seen in systemic lupus erythematosus (SLE), systemic sclerosis, and polymyositis. There is a distinctive overlap in features of the above diagnoses, so commonly in early course it is often referred to as Undifferentiated Connective Tissue Disease. Cardiac involvement of MCTD accounts for approximately 20% mortality.

Case

A 46 year old woman with a history of undifferentiated vs. mixed connective tissue disease and chronic left lower extremity lymphedema presented with one day of chest pain that occurred while driving. Of note, she was physically active and jogs 2 miles a day. The chest pain radiated down her left arm and was associated diaphoresis. She was found to be hypertensive 220/110. Her initial ECG demonstrated sinus bradycardia. She became symptomatic again with chest pain and lightheadedness during the admission interview. Repeat EKG revealed new ST elevation in precordial leads in septal distribution. STEMI protocol was activated and patient was taken emergently for cardiac catheterization, which revealed 98% mid LAD occlusion to which one DES was deployed, as well as 50–70% circumflex lesion.

Echocardiogram demonstrated a normal ejection fraction, apical and septal wall abnormalities and left ventricular wall motion abnormalities. Troponin peaked at 27.46 then down-trended. She was initiated on DAPT (aspirin and ticagrelor), an ACE inhibitor and beta blocker therapy as well as statin therapy.

Discussion

Patients with connective tissue diseases are at risk for mortality from cardiac involvement of their disease process. Although pericarditis is the most common manifestation of cardiac involvement of MCTD and in lupus, accelerated atherosclerosis has also been recognized. The greatest increase in relative risk of accelerated atherosclerotic coronary disease in patients with SLE paradoxically occurs in young women with otherwise low risk of coronary heart disease.

89 It is not always lupus: a rare case of simultaneous occurane of iga nephropathy and P-ANCA crescentic glomerulonephritis in a pulmonary sarcoid patient with anti-double stranded DNA antibodies seropositivity

M Elmassry1

M Zitun1*

S El Nawaa1

J Makram1

AF Al Mahmoud2

K Nugent3

1Texas Tech University Health Sciences Center, Lubbock, TX

2King Faisal University, Jeddah, Saudi Arabia

3TTUHSC, Lubbock, TX

Case report

Sarcoidosis involves multiple tissues and organs with noncaseating granulomatous reaction. Positive (Anti-DsDNA) antibodies is an extremely rare finding in sarcoidosis as it is more specific of lupus. P-ANCA positivity is also a very rare finding in IgA nephropathy.

Case presentation

A 42-year-old male with celiac disease and sarcoidosis on steroids and gluten-free diet who presented with leg rash and abdominal pain shortly after steroid taper. He was hemodynamically stable. CBC unremarkable. CMP showed AKI. UA showed RBC casts and proteinuria. CXR showed bilateral hilar LAD and ground glass opacities. ANA and Anti-DsDNA antibodies were positive. C2, C3 levels were normal. Anti-smith Abs negative. MPO-P-ANCA antibodies positive. Kidney biopsy showed IgA crescentic GN. Skin biopsy showed leukocytoclastic vasculitis. TB and SLE were ruled out. He was started on cyclophosphamide and steroids with improvement of renal functions and then was discharged on azathioprine.

Discussion

IgA nephropathy (IgAN) is the most common GN. Typically presents with macroscopic hematuria shortly after an URTI. Patients with celiac disease suffer a 3-fold increased risk of future IgAN. Treatment include corticosteroid, mycophenolate, rituximab.

Sarcoidosis is a multisystem disorder characterized by an immune response to antigens with the production of various antibodies as RF and ANA. The prevalence and significance of anti-dsDNA antibodies in sarcoid patients is unknown but is very rare. GN is a very rare entity in sarcoidosis.

Our patient had positive Anti-DsDNA in sarcoidosis and simultaneous IgA nephropathy with P-ANCA vasculitis induced crescentic glomerulonephritis which is very rare.

Conclusion

We believe that ANCA postivity in AKI due to IgA nephropathy can indicate a superimposed IgA crescentic glomerulonephritis or an associated small vessel vasculitis and this confers a worse prognosis. Also, we have to exclude other rheumatological diseases with a positive anti-DsDNA and avoid blaming it on lupus.

90 Possible reasons for elevation of HDL after both variants of bariatric surgerie

PI Altieri*

A Suárez

HL Banchs

N Escobales

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

Purpose of study

To analyze the possible reasons for the alteration of the immunologic system, by producing many metabolic changes, including elevation of HDL and changes in other lipids after bariatric surgeries (R-N-Y and Sleeve).

Methods used

Analysis of 136 patients (P.) who underwent two variants of bariatric surgeries. 102 R-N-Y and 34 Sleeves (S.). P. were analyzed with emphasis in changes of HDL in both surgeries.

Summary of results

After the analysis of 136 P., 102 R-N-Y and 34 S., the HDL increased 11.37% in R-N-Y and 23% in S. The changes in FBS and other lipids will be discussed. We think the changes in HDL are due to:

  1. Swelling of intra–abdominal lymph nodes, producing immunological dysfunction.

  2. Changes in intestinal microbiota, producing metabolic and genetic abnormalities.

  3. Intra–abdominal inflammation due to partially digested food and surgery reflected as changes in immunological state and C reactive protein.

  4. Intra–abdominal trauma due to the surgery, inducing immunology abnormalities reflected as an elevation in HDL (S. > R–N–Y) and other parameters.

Conclusions

We prefer S. over R-N-Y. These bariatric P. has greater benefits in losing weight, but complications in R-N-Y group were greater. Follow up in the 2 groups have the same changes in the metabolic state, reflected as an increase in C-reactive protein of 20% and especially elevation of HDL which is anti-inflammatory.

91 Plasma angiopoietin-2 and angiopoietin-2/1 ratio are elevated in children with sepsis and are associated with clinical markers of organ injury

A Ashtekar*

RP Richter

L Zheng

J Pittet

J Richter

University of Alabama at Birmingham, Birmingham, AL

Purpose of study

We sought to characterize plasma angiopoietin-1 (Angpt-1) and Angpt-2 levels and Angpt-2/1 ratios in children admitted to the pediatric intensive care unit (PICU) for sepsis and to correlate these biomarkers with clinical measures of organ injury.

Methods used

We enrolled 34 children with sepsis and 34 healthy controls in a prospective observational study between July 2018 and September 2019. Blood samples were collected from children with sepsis 0, 24, 48, and 72 hours after PICU admission. Plasma Angpt levels were measured using ELISA.

Summary of results

Over the 72 hours after PICU admission, Angpt-1 levels fell below the median Angpt-1 value for controls, and Angpt-2 levels rose above the median Angpt-2 control value, translating in elevated Angpt-2/1 ratios at all time points compared to controls. Angpt-2 levels and Angpt-2/1 ratios at 24-, 48-, and 72-h were significantly higher in sicker children with sepsis as defined by elevated admission Paediatric Index of Mortality (PIM) 3 scores. Children who remained on vasopressors three days after PICU admission (a clinical measure of aberrant vasoreactivity) also had significantly elevated 24-, 48-, and 72-h Angpt-2 levels and Angpt-2/1 ratios compared to children not requiring vasopressors. Angpt-2 levels and Angpt-2 ratios at 72-h correlated with mechanical ventilation duration (an indicator of acute lung injury) even after adjustment for illness severity using PIM 3 scores. Twenty-four- and 48-h Angpt-2 levels predicted the development of acute kidney injury after adjustment for illness severity.

Conclusions

These findings contribute to our knowledge of angiopoietin dysregulation in pediatric sepsis, indicating that significant elevations in plasma Angpt-2 and Angpt-2/1 ratios during the first 72 hours after PICU admission are associated with clinical measures of lung and kidney injury and dysregulated vascular tone.

92 Insidious and rare: a case of acute fibrinous and organizing pneumonia

R Kemnitz1

S Jin1*

M Barr1,2

1Tulane University, New Orleans, LA

2Southern Louisiana Veterans Health Care System, New Orleans, LA

Case report

1. Review underlying causes of AFOP including inflammatory and infectious etiologies, 2. Recognize common symptoms of AFOP to improve timeliness of clinical intervention.

68 yo M with a history of Myelodysplastic Syndrome (MDS) admitted from Infectious Disease clinic for fever of unknown origin for 6 months and 3 weeks of non-productive cough worse at night and fevers to 102 F. In work-up of fever, he was found to have undifferentiated aortitis, surgical repair 5 months prior. Completed 6 weeks of IV antibiotics post-repair, transitioned to oral antibiotics for persistent fevers. No recent bone marrow transplant or immunosuppression for MDS.

On exam, febrile to 101.4 F, pulse 115, 90% on RA. Bibasilar coarse breath sounds, exam otherwise unremarkable. Labs with elevated inflammatory markers, stable chronic anemia. No leukocytosis. Blood, urine, and sputum studies negative for infective pathology. CT Chest with multifocal consolidative lung lesions with bibasilar ground glass appearance.

Patient started on broad spectrum antibiotics on admission, subsequent bronchoscopy with biopsy consistent with AFOP, at which time he was transitioned to prednisone and discharged to a skilled nursing facility. At 2 week follow-up, symptoms had resolved and repeat imaging showed improvement of lung lesions.

Discussion

AFOP is exceedingly rare, with only 29 unique cases reported between 2015–2016. Definitive diagnosis is based on histopathology of intra-alveolar fibrin balls in patchy distribution, usually bibasilar. Underlying etiologies include infection, immunologic, rheumatologic, or hematologic disease, and malignancy, but is most often idiopathic. Two clinical courses include acute fulminant disease, leading to respiratory failure and death within average 29 days, and a subacute course, with good prognosis and response to treatment. Mainstay of treatment involves antibiotics and corticosteroids, alone or in combination, based on patient presentation. Most patients received some form of anti-infective agents prior to initiation of steroids. Despite prior cases with good response to corticosteroids, the dose and duration has not yet been standardized.

93 A mess of mesh – an unusual case of inguinal lymphadenopathy

H Kenninger*

P Caldwell

S Heer

Tulane University, New Orleans, LA

Case report

1)Develop an approach to working up lymphadenopathy, 2)Identify foreign body implantation as a cause of benign lymphadenopathy.

Case

A 40-year-old man presented with a six-hour history of bilateral swollen inguinal regions, subjective fevers, and penile edema. He had a history of recurrent un-witnessed episodes of inguinal lymphadenopathy with associated fevers over the last 15 months. Episodes persisted for a few hours to days with spontaneous resolution. Surgical history was notable for right and left inguinal hernia repair with ‘plug’ mesh.

Temperature was 98.7F, heart rate 84bpm, blood pressure 106/71 mmHg, respiratory rate 20 breaths/min, oxygen saturation 99% on RA. He was well appearing and comfortable. Inguinal region was mildly erythematous and swollen with tenderness to palpation bilaterally. No palpable inguinal lymph nodes. Penis was circumcised with mild edema and erythema.

WBC was 15.8 K/microliter with 80.9% neutrophils and 11.6% lymphocytes on differential, RPR nonreactive, Chlamydia Trachomatis and Neisseria Gonorrhea PCR negative. Prior workup revealed HIV non-reactive, histoplasma antigen negative, ANA negative, fungus panel non-reactive.

CT abdomen/pelvis without contrast revealed small lymph nodes in the left inguinal region (<8 mm, cluster of 4–5). Lower Extremity ultrasound completed prior to admission revealed a prominent lymph node in the right groin measuring 3.2 × 0.8 × 2.6 cm.

General surgery was consulted for excisional biopsy of the lymph node. Upon further reivew, the patient likely had inflammation of his inguinal region secondary to mesh placement from prior inguinal hernia surgeries. The excisional biopsy was canceled and the patient was discharged with surgery follow-up.

Discussion

Lymphadenopathy is commonly encountered by Internal Medicine physicians and requires a systematic approach to develop an appropriate differential diagnosis. The differential will cover three broad categories: infectious, malignant, and benign non-infectious lymphadenopathy. Benign, non-infectious causes of lymphadenopathy are discussed less frequently. Granulomatous inflammation, reactive lymphoid hyperplasia, and foreign bodies are a few possible etiologies of benign, non-infectious lymphadenopathy. A foreign body reaction, in this case implanted mesh, is an under recognized cause of lymphadenopathy.

94 Juvenile dermatomyositis presenting as severe malnutrition

M Nuss*

C Montcalm

N Evans

A Byrd

Our Lady of the Lake Children’s Hospital, Baton Rouge, LA

Case report

11-year-old female presented to her pediatrician with a three month history of unintentional weight loss and constipation. Patient was noted to have generalized weakness, dry skin, and thinning hair. Labs showed elevated LDH and leukopenia with normal ESR, CRP, ANA, and rheumatoid factor. At two week follow-up, weight had decreased from the 16th to the 1st percentile despite increased caloric intake. Patient was admitted for further work-up. Initial labs revealed normocytic anemia, hypoalbuminemia, and elevated ferritin, ESR, and LDH. She continued to have intermittent fevers and generalized weakness and on further exam, was noted to have papular lesions on her knuckles, calcinoses, and proximal muscle weakness. Further work-up revealed elevated CPK and diffuse myositis on MRI of the lower extremities. The diagnosis of Juvenile Dermatomyositis (JDM) was later confirmed by muscle biopsy.

JDM is a rare, inflammatory myopathy affecting striated muscle, subcutaneous tissue, skin, and nails. It can result in a heterogenous symptomatic presentation that may mimic other disease processes including non-inflammatory and metabolic myopathies, viral myositis, and other rheumatologic disorders such as SLE, scleroderma, and mixed connective tissue disease. Though weakness and fatigue are the most common „presenting symptoms in children, physicians should recognize disease-specific findings including photosensitive ‘shawl sign’, heliotropic rash, and Gottron’s papules. Limb-girdle muscle involvement is classic, however children may complain of „generalized weakness.

JDM requires a high index of suspicion given its clinical variability and vague symptomatic presentation. Diagnosis requires four of the following: symmetric, proximal muscle weakness, characteristic skin findings, elevated muscle-derived enzymes, denervation or myopathy on EMG, and inflammatory changes on MRI.4 Early recognition and treatment with high-dose steroids and disease-modifying agents has been proven to improve survival and long-term outcomes.5 Therefore, it is important for physicians to consider JDM on their differential for any child with weakness, fatigue, and elevated muscle-derived enzymes.

95 Disseminated sarcoidosis with isolated extrapulmonary manifestation

A Sajjad*

H Mazek

TTUHSC, Lubbock, TX

Case report

Sarcoidosis is a multisystem inflammatory disease characterized by non-caseating granulomas. It predominantly affects the lungs and intrathoracic lymph nodes but any organ system can get affected. Isolated extrapulmonary sarcoidosis is very rare accounting for less than 10% of cases reported. In this case report we highlight a case of vertebral, neurologic, cutaneous, and liver involvement of sarcoidosis without any pulmonary manifestation.

Case presentation

This is a 72 year old female with a past medical history of type II diabetes who was admitted to the hospital for a fracture of the right neck of the femur secondary to ground-level fall. Trauma work up initially showed lytic bone lesions at skull, several small mediastinal lymphadenopathy, poorly circumscribed lucencies in the lumbar and thoracic spines, and multiple liver lesions. Patient denied shortness of breath, cough, fever, chills, chest pain, orthopnea, abdominal pain, dysuria, diarrhea, or weight loss. She complained of bilateral peripheral neuropathy that had been going on for the past year. Work up was performed to rule out differential diagnoses including infection, inflammation, multiple myeloma, malignancy, lymphoma, leukemia. Liver and bone marrow biopsy performed showed noncaseating granulomas. Patient also complained of a scalp rash that had started two months ago and was diagnosed and treated as psoriasis without any improvement. Vertex scalp biopsy suspected sarcoidosis granuloma. Patient was diagnosed with disseminated sarcoidosis as diagnosis of exclusion secondary to her mediastinal lymphadenopathy, and biopsy findings of noncaseating granulomas in the bone marrow, scalp, and liver. Patient was started on methotrexate and plaquenil.

Discussion

Extrapulmonary involvement of sarcoidosis without any pulmonary manifestation is very rare and make it a diagnostic challenge. Clinical and radiologic presentation of sarcoidosis mimics many different disease conditions including malignancy and infection. Sarcoidosis is a diagnosis of exclusion; thus it is very important to keep a broad differential diagnosis during the diagnostic process, especially taking into consideration malignancy as a differential diagnosis. This case is being reported to help expanding knowledge on isolated extrapulmonary presentation of sarcoidosis and its diagnosis.

Cardiovascular

Joint plenary poster session and reception

4:30 PM

Thursday, February 13, 2020
96 The fish oil dilemma- does it increase recurrence risk of ventricular arrythmia?

AA Asfaw*

MK Teshome

B Cave

RN Khouzam

UTHSC, Memphis, TN

Purpose of study

Review the current knowledge on the effect of PUFA on patients with history of ventricular arrythmia(VA).

Methods used

We reviewed relevant publications on PUFA and Cardiovascular mortality

Summary of results

The cardioprotective effect polyunsaturated omega fatty acids (PUFA) has been of interest starting from the early 1930’s. Multiple studies have been conducted on the effect of fish (high PUFA) consumption on cardiovascular mortality, including sudden death, with inconsistent results. Meta-analysis of observational studies by Whelton et al have shown that fish oil consumption was associated with reduced risk of both fatal and total coronary heart disease(CHD) (RR=0.83; 95% CI0.76 to 0.90) and (RR 0.86; 95% CI 0.81 to 0.92). Metanalysis of cohort studies by He K et al have shown that each 20 gm/day increase in fish intake is associated with a 7% lower risk of CHD mortality. In contrast a randomized controlled factorial trial by Burr ML et al has shown that there was an excess sudden cardiac death among those who were supplied with fish oil. The GISSI-Prevenzione trial evaluated PUFA in patients within 3mo of myocardial infarction(MI). PUFA treatment resulted in a modest, but statistically significant, reduction in the risk of primary outcome, death, nonfatal MI and stroke. Although the study was not specifically designed to analyze sudden death, much of the benefit was driven by this outcome. Further study was completed by Raitt et al to confirm PUFA’s role in the prevention of sudden cardiac death. In patients with an implantable cardioverter defibrillator (ICD) and recent episode of sustained ventricular tachycardia ventricular fibrillation were randomly assigned to PUFA or placebo. Over a median follow-up of 718 days, in patients with a recent episode of sustained VA and an ICD, PUFA did not reduce the risk of recurrence and may be proarrhythmic in some patients. Furthermore in a subsequent meta-analysis of >500 patients, PUFA supplementation in patients with ICD did not reduce ICD discharge or the risk of recurrence.

Conclusions

The controversy of benefit or harm with PUFA supplementation may lie in the underlying cause (ischemia versus scar-related ventricular tachycardia) or whether treatment is for primary or secondary prevention of ventricular arrhythmias in which further study is needed.

97 The relationship between tricuspid regurgitant jet velocity and QTC prolongation in sickle cell disease

P Dauphin*

JD Pollard

T Tanawuttiwat

J Maher

University of Mississippi Medical Center, Jackson, MS

Purpose of study

Sudden cardiac death is a leading cause of mortality among patients with sickle cell disease (SCD). QTc prolongation is frequently observed in the SCD population and is associated with ventricular arrhythmias, a common etiology of sudden cardiac death. Additionally, the degree of pulmonary hypertension, and elevation of the tricuspid regurgitant jet velocity (TRV), a measure of right ventricular systolic pressure, have been shown to be risk markers for early mortality in patients with SCD. However, the relationship between TRV and QTc prolongation has not been fully elucidated.

Methods used

We are conducting a study of QTc-modifying genetic and secondary risk factors in SCD, targeting enrollment of 500 (250 adults, 250 children) patients with SCD, at baseline status, from sickle cell clinics in the University of Mississippi Medical Center. A transthoracic echocardiogram and resting 12-lead ECG are being collected, along with laboratory and demographic information. In this interim analysis, a total of 150 participants (107 adults, 43 children) were included. We investigated the relationship between elevated TRV (defined by TRV ≥ 2.5 m/s), the QTc interval, and its subcomponents: QRS and JTc interval (milliseconds).

Summary of results

Elevated TRV was not significantly associated with increased QTc interval in all participants (432.56 vs 427.84, p=0.671) and in adults (432.26 vs 429.51, p=0.967). Similarly, there is no significant difference in the JTc interval between those with and without elevated TRV in all participants (339.79 vs 340.43, p=0.559) or in adults (340.04 vs 341.09, p=0.616). However, the elevated TRV was significantly associated with increased QRS duration in all participants (92.77 vs 87.42, p<0.001) and in adults (92.21 vs 88.42, p=0.020).

Conclusions

While elevated TRV is a risk marker for early mortality in SCD, we do not find evidence of repolarization abnormalities in patients with elevated TRV, given the lack of a significant association of TRV with QTc or JTc prolongation. However, the significant association between elevated TRV and QRS duration raises the possibility that an intraventricular conduction abnormality could be contributing.

98 Drug-coated balloon angioplasty verses standard balloon angioplasty for in-stent restenosis revascularization in peripheral artery disease

V Kohli1*

R Chopra2

L Namburu1

A Griwan1

T Paul1

1East Tennessee State University, Johnson City, TN

2James J Peters VA Medical Center, Bronx, NY

Purpose of study

Drug coated balloon angioplasty (DCBA) is preferable for de novo lesions of PAD because they are superior to the standard balloon angioplasty (SBA). One of the complications that are seen commonly in patients undergoing angioplasty is in-stent restenosis. Our aim is to compare the DCBA with SBA in the revascularization of in-stent restenosis.

Methods used

PubMed, Cochrane and Google Scholar were comprehensively searched to obtain randomized controlled trials until August 2019. The outcomes included mortality and total lesion revascularisation (TLR). The effect size of each study was computed and compared using the random-effect, inverse variance method.

Summary of results

Five studies were included with 380 patients who underwent angioplasty. They were divided into two groups with group 1 (undergoing DCBA) including 193 and group 2 (undergoing SBA) including 187 patients. No significant increase in mortality was present between the two groups with OR of 1.60 [0.47, 5.47], p=0.45. There was a significant reduction in the TLR in the group who underwent DCBA versus who underwent SBA with OR of 0.29 [0.12, 0,73], p=0.009.

Conclusions

DCBA is superior to SBA in reducing future total lesion revascularization rate at 6 months but there was no difference in the rate of mortality between the two groups.

99 A heart stopping dive: a classic presentation of congenital long QT syndrome

JO Kolawole*

T Tanawuttiwat

T Suzuki

University of Mississippi Medical Center, Jackson, MS

Case report

Long QT syndrome (LQTS) is a group of channelopathies characterized by delayed repolarization of the ventricular myocardium, QT prolongation and increased risk for sudden cardiac death, in the setting of structually normal heart and healthy individuals. Several studies have shown associations between specific LQTS genotypes and particular triggers or events. Diving and swimming-related events are associated with Type 1 LQTS.

Case description

28 year-old previously heathy Caucasian male was admitted to our hospital for a witnessed cardiac arrest. He was at a summer recreational camp with a group of students when he dove into a lake. He was reported to resurface for a few seconds and then noticed to sink and not resurface. Lifeguards were able to pull him out and noticed he was unresponsive without a pulse. An automated external defibrillator showed polymorphic ventricular tachycardia during cardiopulmonary resuscitation with return of spontaneous circulation upon defibrillation.

He had no previous history of syncope, seizures or sudden cardiac arrest or no family history of sudden death or cardiomyopathy. He takes no medications. Laboratory investigation on presentation reviewed mild acute kidney injury with unremarkable serum potassium, magnesium, urine drug screen, alcohol levels and thyroid function tests. Serial electrocardiograms obtained on presentation and during admission showed prolonged QT interval (QTc 524 ms) in the absence of QT prolonging agents and normal electrolytes. Echocardiogram was normal with no structural abnormalities.

He was successfully extubated with resolution of his pneumonitis and acute kidney injury within 24 hours. He was evaluated by the cardiac electrophysiology team and started on propanolol as medical therapy for LQTS. He was safely discharged with a life vest with subsequent placement of an implantable cardioverter-defibrillator.

Conclusion

Diagnosis of LQTS can be challenging and relies on clinical assessment with a detailed history and electrocardiogram finding, supplemented with genetic testing. Near-drowning episodes with sudden cardiac arrest in the context of prolonged QT intervals, especially in previously healthy swimmers, should raise the suspicion for Type 1 LQTS.

100 Can anthropometric measures of obesity predict liver surface nodularity in a diverse nafld population?

S Miller*

E Varney

CM Howard

University of Mississippi Medical Center, Jackson, MS

Purpose of study

To assess the ability of anthropometric measures and body composition to predict liver surface nodularity in a diverse NAFLD population.

Methods used

For this retrospective observation study, adult patients with various degrees of non-alcoholic fatty liver disease (NAFLD) and non-enhanced CT images of the abdomen and pelvis obtained (N=367) to assess how specific anthropometric measures of obesity correspond to liver surface nodularity and NAFLD clinical index in patients diagnosed with NAFLD. Abdominal adipose volumes (superficial and visceral) and muscle volumes (abdominal wall, psoas and paraspinal) were quantitatively measured on CT images of the abdomen using a multi-layer segmentation technique. Abdominal diameters (SAD) were measured and two readers independently assessed liver surface nodularity (LSN) scores of the 367 patients. LSN scores were obtained using a previously validated quantitative technique. LSN scores were analyzed and correlated with SAD, NAFLD clinical index, body weight, regional fat and muscle volumes and liver and spleen attenuation using a regression model and the coefficients of determination were calculated. Intraclass correlation coefficient (ICC) with 95% confidence intervals (95%CI) and coefficient of variation (CV) were used to assess inter-observer agreement among the two readers assessing LSN scores.

Summary of results

In patients with NAFLD, SAD showed direct correlation with weight, LSN score, and NAFLD index and an inverse correlation to spleen and liver attenuation. SAD correlated best with patient weight (R2=0.64, p<0.001) and LSN score (R2=0.38, p<0.001). We also showed significant correlations between adipose volumes and both anthropometrics and LSN scores (p<0.001). Correlations between SAD and liver attenuation/spleen attenuation/NAFLD were present but were of minimal clinical significance (R2=0.07/0.11/0.05, respectively, p<0.001) without the support of other clinical data.

Conclusions

Anthropometric measures and regional fat volumes showed direct correlations with weight, LSN score, and NAFLD index. This study has data indicating that SAD and body composition show significant correlation with weight and LSN score within a high risk, diverse NAFLD population.

101 Incidence of acute myocardial infarction and hurricane katrina: twelve years after the storm

A Nakhle*

BP Deere

M Razavi

S Srivastav

H Rawal

D Harrison

AK Irimpen

Tulane University School of Medicine, New Orleans, LA

Purpose of study

We aimed to evaluate the incidence of acute myocardial infarction (AMI) in New Orleans during the twelve years since Hurricane Katrina.

Methods used

This was a single-center, retrospective study performed at Tulane University Health Sciences Center of patients admitted for AMI during two years before Hurricane Katrina and twelve years after Hurricane Katrina. The pre-Katrina and post-Katrina cohorts were compared according to pre-specified demographic and clinical data.

Summary of results

In the 12-year post-Katrina period, there were 3,067 admissions for AMI out of a total census of 90,494 (3.4%) compared to 150 admissions out of a census of 21,079 (0.7%) in the 2-year, pre-Katrina group (p<0.0001). The post-Katrina group had a higher prevalence of known coronary artery disease (CAD) (46.1% vs. 30.7%, p<0.0001), diabetes mellitus (40.5% vs. 28.7%, p=0.002), hypertension (80.1% vs. 74.0%, p=0.0281), hyperlipidemia (57.0% vs. 45.0%, p=0.0006), smoking (53.6% vs. 39.3%, p=0.0005), drug abuse (18.0% vs. 6.7%, p=0.0003), and psychiatric disease (15.9% vs. 6.7%, p=0.0002). The post-Katrina group was more often prescribed aspirin (50.2% vs. 31.3%, p<0.0001), beta-blocker (47.8% vs. 34.0%, p=0.0017), ACE inhibitor or ARB (52.4% vs. 36.0%, p=0.0002), and statin (52.7% vs. 28.0%, p<0.0001) but with higher medication non-adherence (16.4% vs. 7.3%, p<0.0001). The post-Katrina patients were also more likely to be unemployed (18% vs. 2.0%, p<0.0001), uninsured (11.0% vs. 6.0%, p=0.05), and non-married (62.0% vs. 54.7%, p<0.0001). Rates of STEMI were lower in the post-Katrina group (29.0% vs 42.0%, p=0.0013). There was no significant difference between the two groups in terms of sex or prior coronary artery bypass grafting.

Conclusions

There was a nearly 5-fold increase in the incidence of AMI twelve years following Hurricane Katrina. Prevalent psychosocial, behavioral, and traditional CAD risk factors were significantly higher among the post-Katrina group. These findings add to the growing body of literature demonstrating adverse cardiovascular outcomes after a natural disaster. Further research is needed to elucidate underlying mechanisms to help mitigate future cardiac morbidity.

102 Can computed fractional flow reserve coronary CT angiography (FFRCT) offer an accurate noninvasive comparison to invasive coronary angiography (ICA)? ‘The non-invasive cath.’ A review

J Raja*

A Vanlandingham

B Thompson

HK Marella

I Pour-Ghaz

M Maturana

RN Khouzam

UTHSC, Memphis, TN

Purpose of study

This abstract aims to review the accuracy of Computed Fractional Flow Reserve in the diagnosis of hemodynamically significant coronary artery stenosis and ruling out non-significant coronary artery stenosis when compared to FFR obtained during invasive coronary angiography.

Methods used

We conducted a Medline search using various combinations of ‘Computed Fractional Flow Reserve,’ ‘Invasive coronary angiography’ ‘noninvasive,’ ‘significant stenosis’ and ‘coronary artery disease’ to identify randomized trials published before October 1, 2019, for inclusion in this review. The following section reviews data from pivotal trials to determine a noninvasive strategy in appropriate patients to accurately detect functionally significant stenosis.

Summary of results

FFRCT is a novel noninvasive modality which localizes significant stenosis by means of crystal fluid dynamics eliminating the need for vasodilators. The specificity of FFRCT as analyzed by DISCOVER-FLOW, DeFACTO, NXT trials were 93%, 90%, and 86% respectively. Decrease in PCI was 5% in DISCORD trial and 61% in PLATFORM trial. The ADVANCE registry showed significantly lower events of CV death or MI with a negative FFRCT (>80) when compared with abnormal FFRCT. Furthermore, when FFRCT was studied as a first-line diagnostic modality in stable angina, it showed effectiveness in differentiating patients who require no invasive intervention in low risk (FFR >0.80) compared to high risk (FFR</=0.80).

Conclusions

FFRCT is a novel modality for analyzing significant stenosis in CAD non-invasively. The high sensitivity of this modality could make it a good rule out tool to avoid unnecessary intervention in physiologically insignificant lesions. Limitations of this modality include low specificity and double exposure to contrast if the test is positive warranting further intervention. Further query into this matter is warranted.

Abstract 102 Table 1

Per patient sensitivity, specificity and accuracy of FFRCT Per patient sensitivity, specificity and accuracy of FFRCT

103 Risk factors and mortality in dialysis patients with abdominal aortic aneurysm

D Xia1*

D Linder1

J Waller1

W Bollag1

AA Mohammed1

S Padala1

S Nahman1,2

M Kheda1

SL Baer1,2

V Taskar1

N Weintraub1

B Siddiqui1

1Augusta University, Duluth, GA

2Charlie Norwood VA Medical Center, Augusta, GA

Purpose of study

In the general population, abdominal aortic aneurysm (AAA) is synonymous with vascular disease and associated with increased mortality. Once diagnosed, AAA can be followed noninvasively or corrected surgically. Vascular disease is common in dialysis patients, but there is limited information on the incidence and outcomes for AAA in this population. To address this question, we queried the United States Renal Data System (USRDS) for risk factors associated with diagnosis of AAA, survival of patients diagnosed with AAA, and overall risk factors for mortality.

Methods used

Incident dialysis patients from 2005–2014 from the USRDS were queried. ICD-9 and ICD-10 codes were used to define a diagnosis of AAA and identify clinical co-morbidities. Cox proportional hazards (CPH) modeling was used to determine the adjusted hazard ratio (aHR) and 95% confidence intervals (CI) for death.

Summary of results

From a total cohort of 868,799, we identified 22,121 subjects with a diagnosis of AAA. When compared to patients without the diagnosis, AAA patients were older and had higher percentages of white race, male gender, tobacco use, Charleston comorbidity index (CCI), and hypertension as end stage renal disease (ESRD) etiology, but lower percentages of diabetes as ESRD etiology. A bivariate CPH model of survival time of the AAA group showed that AAA patients had significantly increased mortality compared to patients without a AAA diagnosis (HR=1.29, p-value<0.0001). However, in the final CPH model, patients with an AAA diagnosis had a decreased risk of mortality (aHR=0.85, 95% CI 0.844–0.860), after controlling for age, CCI, and other demographic and comorbid variables.

Conclusions

ESRD patients with a diagnosis of AAA are more likely to be older, white, male, smokers with hypertension as the cause of ESRD. Patients with AAA are less likely to have diabetes as an etiology of ESRD. AAA is associated with a decreased risk of death, which suggests that AAA in the ESRD population by itself may not increase mortality, but the comorbid factors that come with it do.

104 A rare case of fractured inferior vena cava filter strut migrating to the right ventricle

M Elmassry

S El Nawaa

P Tantrachoti

M Zitun*

J Abdelmalek

P Sethi

K Nugent

TTUHSC, Lubbock, TX

Case report

Inferior Vena Cava (IVC) Filter placement is indicated in patients with absolute contraindication to anticoagulation. IVC filter fracture with fragment embolization is a very rare complication and is usually missed due to lack of symptoms. We present a case of IVC filter fracture and strut migration.

Case presentation

A 43-year-old female with history of severe MVA 20 years ago which was complicated with prolonged immobilization. She suffered recurrent DVTs despite anticoagulation eventually requiring Greenfield IVC filter. She presented to our hospital with right flank pain, and as a part of her work up, a CT scan of the abdomen and lower chest showed high-density linear structure in the anterior wall of the right ventricle. This likely represented a fractured IVC filter strut as the IVC filter was off axis and missing one strut (figure 1,2). The rest of history and physical exam were unremarkable. Cardiothoracic surgery recommended leaving the IVC filter and strut in place and opted for surveillance by regular follow-ups and CT scans. Patient was sent home on novel oral anticoagulant.

Discussion

The most common complication of IVC filter placement is filter site thrombosis. Filter fracture with strut migration is a rather rare complication. Most of the time the patient is asymptomatic. Surgical intervention is not usually recommended and may be associated with more complications. Management is mainly surveillance with imaging.

Conclusion

Forty percent of IVC filter placements are placed without validated indications. Physicians should be aware of serious adverse effects as in our case before considering this procedure.

Abstract 104 Figure 1

Showing sturt in right ventricular wall Showing sturt in right ventricular wall

Abstract 104 Figure 2

IVC filter with missed strut IVC filter with missed strut

105 Can fish oil kill you?

AA Asfaw*

RN Khouzam

B Cave

MK Teshome

M Barnhart

J Counts

UTHSC, Memphis, TN

Case report

A 74-year-old male patient with past medical history of symptomatic Ventricular Tachycardia (VT) post Intra Cardiac Cardioverter Defibrillator (ICD) placement in June 2019. He was admitted after he had multiple shocks from his ICD. He had no chest pain, dizziness or syncope. His home medications included Metoprolol succinate 100 mg daily, enalapril 10 mg and Poly Unsaturated Fatty Acid (Fish Oil) 1200 mg daily. A left heart catheterization at the initial diagnosis of monomorphic tachycardia revealed normal coronaries. Transthoracic Echocardiogram showed normal LV systolic function at the time of diagnosis with mild aortic valve regurgitation. During the current admission, ICD interrogation showed that he had received 46 appropriate shocks for VT. A 12 lead EKG showed a wide complex tachycardia with LBBB morphology. Subsequently, he underwent electrophysiology study and ablation of VT focus identified in the right ventricle apical lateral wall.

106 What should physicians know about takayasu arteritis and risks with the use of vasopressors?

GD Bedanie1*

F Abaleka2

M Rahman1

M Ansari1

J Saverimuttu2

M Zaw2

S Galiboglu2

1Texas Tech University Health Science Center, Lubbock, TX

2Richmond University Medical Center, Staten Island, NY

Case report

Takayasu arteritis(TA) is a rare systemic inflammatory arteritis affecting the large vessels, predominantly the aorta and its branches. It is commonly seen in women age <40 years. Etiology is not clearly known, but it may be related to autoimmune inflammatory damage to arterial wall. Progression of the disease following vasopressor administration has not been reported, but it is recommended to avoid vasopressors. We report case of undiagnosed and asymptomatic TA who developed gangrene of extremities after vasopressor administration for septic shock.

Case summary

A 31-year-old female was admitted with left foot amputation site infection and progressive limb ischemia. One year ago, her feet and hand were amputated due to acute limb ischemia following administration of vasopressor for septic shock. Patient was re-admitted with left foot stump darkening and discharge. Upon evaluation, she was alert and comfortable. Blood pressure measurement showed discrepancy in both arms, 94/60(right arm) and 70/38 mmHg(left arm), no tachycardia or fever. Left brachial pulse was non palpable, and right radial pulse was feeble. Bilaterally amputated feet and upper extremities amputated at the trans-metacarpal and wrist level. Given the above clinical signs, TA was suspected. CT angiogram revealed focal stenosis of the left innominate artery, at the origin of the left vertebral artery and the left subclavian artery, suggestive of TA.

Conclusion

TA can be associated with substantial morbidity and life-threatening complication. There are no clear approach in patient with TA and septic shock who requires vasopressors. There are few case reports of patients who developed anesthesia related hypotension in whom administration IV fluid and Trendelenburg position are recommended. It was also stated vasopressors are best avoided in patient with TA. Vasopressors can further compromise blood flow and increase risk of acute limb ischemia like in our patient.

107 A challenging case of recurrent prosthetic valve endocarditis in adult congenital heart disease with multiple sternotomies

MR Brunner1*

PS Jagadish1

D Layman1

C Kaiser2

E Rodriguez2

MD Koplon1

1University of Tennessee Health Science Center, Memphis, TN

2St. Thomas, Nashville, TN

Case report

Prosthetic valve endocarditis (PVE) can lead to abscess formation and septic emboli. Antibiotics are often insufficient. About 50% of cases require cardiac surgery.

Case

A 42-year-old female with congenital heart disease and recurrent PVE presented with fever after receiving antibiotics by central line for colitis. She had 8 previous sternotomies for childhood repair of aortic stenosis, ventricular septal defect, bi-ventricular outflow tract obstruction, and 3 aortic valve and root replacements (AVR), including a 2018 transcatheter AVR.

Severe sepsis from methicillin-sensitive S. aureus bacteremia was diagnosed. Vancomycin and piperacillin/tazobactam therapy failed. Infectious Disease began gentamicin, oxacillin, and rifampin but stopped gentamicin due to tubular toxicity.

A new neurological deficit warranted brain magnetic resonance imaging that confirmed multiple septic embolic infarcts. Computed tomography confirmed TAVR valve vegetations and prosthetic aortic root abscess (figure 1). A 30-day perioperative mortality rate of 53% precluded in-house repair. She was transferred for aortic root and ascending aorta replacement with uneventful recovery and resolution of bacteremia.

Discussion

Despite high mortality, the patient’s PVE, aortic root abscess, and persistent bacteremia precluded TAVR in TAVR, making surgery the only curative option. It was successful in this case.

Abstract 107 Figure 1

Computed tomography confirmed TAVR valve vegetations and prosthetic aortic root abscess Computed tomography confirmed TAVR valve vegetations and prosthetic aortic root abscess

108 All clots no plop: an atypical presentation of atrial myxoma

WS Crews*

RC Cutrer

A Clark

University of Mississippi Medical Center, Jackson, MS

Case report

We present a 53-year-old male with a history of bipolar disorder, hypertension, previous stroke and sciatica who presents with complaints of ‘frozen toes’. The patient explained that he had been experiencing numbness and discoloration of the right 1st, 2nd, and 5th toes. He stated that he noticed these changes over the past couple of weeks however he now began having sensations to which he continued to describe as ‘frozen’ associated with numbness and mild pinprick sensations. He admitted to using ten cigarettes daily but denied any current/previous IV drug use or history of bacteremia or endocarditis. He was noted to have had a full mouth extraction six years ago. The patient had no other complaints. On review of systems, he denied any chest pain, shortness of breath, claudication, swelling, weight changes, cough, fevers, chills or recent trauma. On exam he was noted to have dry mucous membranes, clubbing of the fingers bilaterally and mottled appearing skin of the right great toe with small non-blanching purpura on the 2nd and 5th digits. He also endorsed decreased sensation with palpation, but no pain was elicited. The initial labs revealed elevated hemoglobin and hematocrit of 18/55. Additional workup was unremarkable. The patient was started on heparin infusion with additional imaging obtained including a CTA runoff and TTE in along with a surgery consult. The patient’s echocardiogram showed a large friable mass measuring 2.4 × 1.9 inches at the junction of the mitral annulus and interatrial septum protruding into the mitral valve. Adult cardiac surgery was consulted, and an additional CTA chest and Cardiac MRI were obtained. CTA was remarkable for a left upper lung nodule with a spiculated appearance concerning for malignancy. The patient was taken for an open excision of intracardiac mass to decrease the risk of additional organ damage from seeding clots before additional malignancy workup was considered. Pathology was consistent with a large atrial myxoma with gelatinous covering and adherent clots. The patient had an uncomplicated recovery and has since continued to the Pulmonology department. Additional lung and lymph node biopsies have since been obtained that are consistent with Adenocarcinoma. The patient is currently undergoing chemotherapy and radiation treatment.

109 Recurrent arterial occlusions: a case of severe peripartum cardiomyopathy

P Patel1

KG Hesterberg1*

I Ifedili1

F Aslam1

RN Khouzam1,2

1UTHSC, Memphis, TN

2Methodist LeBonheur Health Care System, Memphis, TN

Case report

A 32 year-old female with diabetes mellitus, recurrent miscarriages, and PPCM diagnosed at 32 weeks, caesarian section complicated by wound dehiscence presented with abdominal pain and bilateral lower extremity edema. Imaging was significant for bilateral pulmonary emboli along with splenic, renal and intestinal infarctions. Transthoracic echocardiogram showed a LV ejection fraction of 15% with a LV thrombus; she was started on intravenous anticoagulation. Soon after she complained of left leg pain; distal pulse was absent. Imaging showed a left femoral artery occlusion and she was taken to the OR emergently. Later she was noted to have absent pulses in the right lower extremity and needed additional surgical interventions for recurrent arterial thrombi. Hematology was consulted due to concern for catastrophic APL.

In PPCM, there is a higher risk for thromboembolic events due to a low ejection fraction and stasis of blood, in addition to the hypercoagulable state associated with pregnancy. Guidelines suggest considering anticoagulation in this situation, however, it is unclear which patients benefit most. Thromboembolism is the most severe complications of PPCM, affecting up to 7% of cases. This is particularly of concern in patients with suspected or diagnosed hypercoagulable states. APL is an autoimmune condition associated with pregnancy loss or vascular thrombosis in the presence of positive antiphospholipid antibodies. The manifestations vary and APL can present with diffuse vascular thrombosis leading to rapid multiorgan failure.

Guidelines recommend considering anticoagulation in patients with PPCM. In APL, anticoagulation is considered the standard of care. In the case presented, anticoagulation may have been appropriate. Due to her catastrophic presentation, several strategies were considered including changing anticoagulation and plasmapharesis illustrating the management challenges of PPCM in the setting of a suspected hypercoagulable state. This case highlights that when faced with recurrent arterial thromboembolisms in PPCM, especially in high-risk hypercoagulable patients, the best management remains unclear.

110 Mid-ventricular variant takotsubto following sudden cardiac arrest

W Kogler*

MB Omar

M Chahin

C Canha

P Reddy

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

Case report

A 60 year old male with past medical history of HTN arrived to the hospital after a witnessed v-fib arrest. ACLS was initiated with successful resuscitation. The history was given by his wife who reported that the patient had diarrhea for a few days prior, denied any reported chest pain. An EKG showed a QTC of 580. Labs showed a K of 2.7 and Mg of 0.8. Left heart catheterization revealed normal coronaries, however ventriculogram showed hyperdynamic contraction of the apex and base, with akinesis and ballooning of the mid-ventricle. Electrolytes were replaced and his QTC improved. Repeat imaging 3 months later showed resolution.

Takotsubo Cardiomyopathy (TC) is a reversible stress induced cardiomyopathy caused by excess adrenergic activity resulting in myocardial stunning. Causes include emotional stress, illness, trauma, and cardiac arrest. TC can mimic myocardial infarction by presenting with chest pain, ST segment changes, cardiac enzyme elevations and symptoms of heart failure. Imaging usually reveals apical ballooning with hyperdynamic contraction of the bases. There are rare variants such as mid-ventricular TC which involves hyperdynamic contraction of the apex and base with akinesis of the mid-section. Acute coronary syndrome must be ruled out for a diagnosis of TC, especially important with mid-ventricular TC as this presentation can be confused with wall motion abnormalities caused by an MI. Treatment is similar to heart failure therapy with most patients making a full recovery.

TC is a well described phenomenon, however multiple variants have been emerging recently. Typically the apex is more at risk for TC as it has been postulated that this region is more sensitive to adrenergic stimulation. However with the emergence of other variants, it is imperative that they become recognized as they can be mistaken for wall motion abnormalities during a myocardial infarction.

111 Venous and arterial thromboembolic events in a patient with anti-adalimumab antibodies

J Makram*

HM Mallah

D Vangipuram

J Sekhon

M Elmassry

K Nugent

Texas Tech University Health Sciences Center, Lubbock, Texas, USA, Lubbock, TX

Case report

Adalimumab is a human monoclonal TNF-α antibody that is widely used to treat ulcerative colitis (UC) given its favorable side effect profile compared with other systemic treatments. Its long term efficacy and safety profile remain unknown. We report a case of venous and arterial thromboembolic events occurring in a UC patient during adalimumab treatment.

Case description

A 59-year-old man with UC was recently started on adalimumab 6 weeks before admission. He presented with gradually increasing pain in the right leg and shortness of breath on exertion. He was tachypneic, hypoxic, and had right leg swelling. Computed tomography pulmonary embolism (PE) protocol was positive for bilateral PE. Ultrasound (US) Doppler revealed acute occlusion of the right distal superficial femoral and popliteal veins. He was started on heparin drip and transitioned to rivaroxaban upon discharge. He presented 3 days later to our facility with sudden sharp right leg pain while working on his farm. Distal pulses were absent in the right leg, and US arterial Doppler showed acute occlusion of the right popliteal artery. He was restarted on heparin drip and underwent emergent peripheral angioplasty of the proximal right posterior tibial artery and aspiration thrombectomy of the right popliteal artery. Given his extensive thrombosis, we consulted the hematology team to rule out an underlying hypercoagulable state. He was negative for antiphospholipid antibodies; however, he had elevated anti-adalimumab antibody levels (29.7 AU/ml)

Discussion

Only 4.5% of all the spontaneously notified adverse reactions of the TNF-α blockers are arterial and venous thromboembolic events. According to Korswagen et al, the occurrence of venous and arterial thromboembolic events during adalimumab treatment was higher in patients with anti-adalimumab antibodies than in those without anti-adalimumab antibodies. This may be secondary to the formation of immune complexes that can activate platelets via the Fc γ receptor and complement system, inducing a prothrombotic state.

Primary care physicians should be aware of this possible association despite its rarity, pending further evaluation by controlled studies.

112 Systemic gas identified on transthoracic echocardiogram – an ominous sign of intestinal ischemia

K Murdock*

JD Pollard

University of Mississippi Medical Center, Jackson, MS

Case report

A 56 year-old male with a known history of alcoholism, hepatitis C, hepatic cirrhosis complicated by esophageal varices and spontaneous bacterial peritonitis (SBP) presented with abdominal pain. Diffuse abdominal pain was reported, associated with nausea and vomiting. On exam, abdomen was distended and diffusely tender to palpation. An abdominal x-ray ordered on admission was concerning for a small bowel obstruction. A paracentesis was performed which ruled out SBP. The patient developed shortness of breath while in hospital. A chest X-ray revealed a right sided pleural effusion and multi-focal areas of consolidation concerning for pulmonary edema or multi-focal pneumonia. A trans-thoracic echocardiogram revealed normal left ventricular function; however, there were continuous small gas formations noted to be originating from the inferior vena cava (IVC). The gas was visualized circulating and exiting into the systemic circulation, leading to suspicion for a patent foramen ovale (PFO) or a pulmonary arterio-venous malformation (AVM). Based on the echocardiogram findings, a computed tomography of the abdomen (CT) was ordered to evaluate for bowel ischemia. The CT scan revealed pneumatosis of the small bowel wall and concern for bowel ischemia. A repeat echocardiogram after resolution of his abdominal pain demonstrated no further gas formation.

Conclusion

This represents a rare case of intestinal ischemia discovered after identification of systemic gas on echocardiogram. Portal venous gas is a well-documented sign of intestinal ischemia1. It can also occur with a faulty intravenous line, post-surgical manipulation of abdominal organs, colonoscopy and is idiopathic in 15% of cases3. Gas entering the IVC through porto-systemic anastomosis has been previously reported2, and we suspect similar mechanism in this case. The gas then likely entered systemic circulation via a PFO or pulmonary AVM. Continuous gas formation crossing into the systemic circulation via a right to left shunt is a rare occurrence, and even more rare to be initially identified by echocardiogram. The gas in itself is not a measure of the severity of illness but rather a diagnostic clue. When gas is visualized on echocardiogram, it is important to evaluate for potentially fatal intra-abdominal events.

113 The octopus trap: an atypical presentation of broken heart syndrome

A Nieves Ortiz*

V Fonseca-Ferrer

A Rivera-Gonzalez

JG Rodriguez Velez

K Hernandez Moya

I Rivera

L Piñeiro

San Juan City Hospital, Canovanas, PR

Case report

Transient apical ballooning syndrome also known as Takotsubo cardiomyopathy, is a very difficult recognized transient left ventricular dysfunction, a non ischemic cardiomyopathy in which there is a sudden temporary weakening of the myocardium. The clinical presentation resembles an acute coronary syndrome, with chest pain, ischemic type ST-segment changes and cardiac enzyme elevation. Prognosis is good, with full recovery of cardiac function within 2–4 weeks.

We present a 70 year old woman with a history of colon and bladder cancer who presented to the Hospital complaining of abdominal pain localized at the epigastria area with associated nausea and vomits. Physical examination was remarkable for dry mucosa and diffuses abdominal pain to deep palpation. Laboratory findings were normal except for hematuria and proteinuria. The chest X ray show elevated right hemidiaphragm with subpulmonic effusion. The abdominal sonogram reveals Bilateral Renal Cyst and hepatic steatosis. Abdominal CT scan showed right lung base atelectasis and consolidation, bilateral renal cysts, small hiatal hernia and calcified coronary atherosclerosis. She was admitted with diagnosis of intractable abdominal pain to rule out small bowel occlusion. We perform a small bowel series which shows up nearly total mid jejunal obstruction. Medical management consisted of hydration, NPO, and surgery services consulted for an exploratory laparotomy for lysis of the occlusion. The next day after surgery patient presented with dyspnea and elevated cardiac enzymes: troponin and CK MB. EKG showed ST segment elevation of V1 to V6 and T wave inversion in the same lead. She was transfer to CCU for critical care management.

This case illustrates Transient apical ballooning syndrome as a complication type of primary acquired cardiomyopathy occurring commonly after a recent stressful event and characterized by transient myocardial systolic dysfunction that is mainly confined to the apical region of the left ventricle. Treatment is supportive but coronary angiography is necessary to establish the diagnosis. The purpose of this case presentation is to alert the medical community on a rare and uncommon cardiac syndrome which mimics a deadly disease.

114 Heart disease in a young female

OB Obafemi*

WF Campbell

JD Pollard

University of Mississippi Medical Center, Jackson, MS

Case report

34-year-old female with no past medical history presented to the emergency room with two day history of left, lateral chest pain. Patient’s EKG appeared dynamic with inferolateral t-wave inversions and troponins were elevated. Patient initiated on aspirin, heparin infusion and had coronary angiography that showed moderate to severe stenosis of the mid to distal left anterior descending (LAD). No percutaneous coronary intervention (PCI) was performed for LAD as concern for Type 2 Spontaneous Coronary Artery Dissection (SCAD). Transthoracic echocardiogram (TTE) showed anterior wall motion abnormalities, ejection fraction (EF) of 35–40% and a small apical thrombus with soft thrombi in left ventricle (LV) apex. Cardiac MRI showed near full-thickness delayed enhancement of the mid to apical septum, apical anterior and inferior LV myocardium and LV apex most compatible with myocardial infarction in the LAD distribution. Myocarditis could not be completely excluded. Patient managed medically for SCAD and LV dysfunction with Plavix, Lipitor, Lisinopril, and Metoprolol. Discussed the benefits and risks of anticoagulation with patient for LV thrombus and proceed with anticoagulation after consulting OB Gyn, who gave assistance with contraception. Follow up TTE evident for EF of 35% and no LV thrombus was visualized. A Multigated Acquisition Scan (MUGA) showed a calculated EF of 47%, an implantable cardioverter-defibrillator was not indicated. Patient had symptomatic anemia and Warfarin was discontinued. Patient continued on aspirin, Plavix, medical therapy for LV dysfunction and referred for cardiac rehabilitation.

Heart disease is the leading cause of death for women in the United States, killing 299,578 women in 2017 or approximately 1 in every 5 female deaths1. SCAD is a rare cause of acute myocardial infarction and is the cause of acute coronary syndrome (ACS) in 0.1 to 4 percent of cases2. SCAD is an important cause of ACS in young women without traditional cardiovascular risk factors. It is rare, underdiagnosed and must have coronary angiography to differentiate from other causes. SCAD presents with ST-segment-elevation MI in up to 50% of cases, however coronary angiogram must be performed when SCAD is suspected even without EKG changes. Short term and long term complications are associated with SCAD.

115 Caseous mitral annular calcification presenting as a stroke

MB Omar*

AE Martinez

A Niazi

J Ruiz-Morales

University of Florida College of Medicine, Jacksonville, FL

Case report

A 70-year-old female presented with left sided hemiparesis. Magnetic resonance imaging showed an acute right parietal ischemic infarct and multifocal punctate infarcts of varying chronicity. There were no paroxsysmal arrhythmias or carotid disease. Echocardiography revealed severe mitral annular calcification with caseous central necrosis (figure 1) and mild mitral regurgitation. Mitral valve replacement was pursued.

Discussion

Mitral annular calcification (MAC) is commonly an inconsequential finding. Au contraire, caseous MAC is a very rare entity occurring in only 0.63% of patients with MAC. Typical echocardiographic findings of a smooth, rounded periannular, echo-dense mass with central echolucency and no acoustic shadowing may be diagnostic. Differentials may include abscess, calcified myxoma or hydatid cyst. Its rarity portends an uncharted clinical course. Purportedly a dynamic process of liquefactive necrosis, scant data suggests it may be benign and reversible. Yet, there is a conceivably increased risk of embolic phenomena or valve dysfunction given its dynamic nature. Rare case reports on caseous MAC favor cavity obliteration with valve replacement over valve preservation techniques to reduce the risk of clinical sequelae.

Abstract 115 Figure 1

a) and b) Transesophageal echocardiogram showing the left atrium (LA), left atrial appendage (LAA) and left ventricle (LV) separated by the mitral valve apparatus with caseous mitral annular calcification (arrow) a) and b) Transesophageal echocardiogram showing the left atrium (LA), left atrial appendage (LAA) and left ventricle (LV) separated by the mitral valve apparatus with caseous mitral annular calcification (arrow)

116 Cardiac arrest and left ventricular function: ‘popsicle heart’ and allowing enough time for the heart to melt

I Pour-Ghaz*

B Cave

S Lavie

J Raja

A Vanlandingham

UN Ibebuogu

R Askari

RN Khouzam

UTHSC, Memphis, TN

Case report

The annual incidence of sudden cardiac arrest (SCA) is estimated to be about 350,000 in the United States. The impact of insertion of implantable cardiac defibrillator (ICD) after SCA in reduced left ventricular ejection fraction (LVEF) patients is not well established. but is associated with higher mortality (1). It has also been demonstrated that prolonged targeted temperature management at 33 degrees Celsius for 48 hours may improve the recovery of LVEF (2). Here, we present a case of ‘popsicle heart’ and propose that timing of ICD implantation should be carefully considered in these patients.

Case presentation

A 26-year-old Caucasian female history of alcohol dependence presented with witnessed cardiac arrest post self-detox from alcohol at home and was admitted for post-arrest management. The patient had ventricular fibrillation and was defibrillated twice with return of spontaneous circulation just prior to arrival. She was placed under hypothermia protocol for a total of 72h. Transthoracic echocardiogram was done on 1st day and 5th day of hospitalization. First echocardiogram showed a visually estimated LVEF was 25–30% with most of the left ventricular segments being akinetic. Second echocardiogram showed a visually estimated LVEF of 40–45%. The patient made dramatic improvements with therapy and was discharged home.

Conclusion

As our case has demonstrated, prolonged hypothermia protocol and allotment of enough time for cardiac function recovery resulted in a dramatic recovery in LVEF and did not necessitate placement of ICD. Thus, in such cases of ‘Popsicle Heart’ having patients placed under hypothermia protocol for at least 48h and repeating echocardiogram in timely interval can help avoid costly and unnecessary procedures such as ICD placement.

References

  1. Aman Gupta, et al. Change in myocardial function after resuscitated sudden cardiac arrest and its impact on long-term mortality and defibrillator implantation. Indian Pacing and Electrophysiology Journal 2019;19:150–4.

  2. Anders Morten Grejsa, et al. Effect of prolonged targeted temperature management on left ventricular myocardial function after out-of-hospital cardiac arrest − A randomised, controlled trial. Resuscitation 2017;115:23–31.

117 Guidewire directed transesophageal echocardiography in the setting of zenker’s diverticulum

A Sabharwal*

K Patel

ER Fox

SJ Tang

University of Mississippi Medical Center, Jackson, MS

Case report

Esophageal diverticula are difficult cases for transesophageal echocardiography (TEE) even for experienced operators due to risk of perforation. Accordingly, they are listed among the absolute contraindications by the American Society of Echocardiography. Here we describe a case in which an endoscopic retrograde cholangiopancreatography guide wire is used to direct a TEE probe into the esophageal introitus adjacent to a large Zenker’s diverticulum (ZD).

A 75-year-old male with a past medical history of mitral valve prolapse, severe mitral regurgitation and ZD was evaluated for MitraClip. He was unable to complete evaluation due to difficulty passing the TEE probe through the oropharynx. The patient underwent esophagogastroduodenoscopy (EGD) with diverticulectomy of 4–5 cm in depth diverticulum and dissection of a prominent septum. Surveillance EGD one month later revealed a prominent ZD with a small esophageal introitus at the hypopharynx. With multidisciplinary planning, a guide wire catheter was affixed to the TEE probe. A guide wire was then passed through the esophagus and into the stomach with gastroscopic guidance. The TEE probe was then advanced into the esophagus using the wire as a rail. Entry of the TEE probe into the esophagus was assisted and confirmed with endoscopic visualization. TEE was then performed per protocol without complications to complete evaluation for mitral valve repair. This case demonstrates a safer approach for addressing esophageal diverticula.

Abstract 117 Figure 1

Guide wire entering esophagus superior to large diverticulum Guide wire entering esophagus superior to large diverticulum

118 Takotsubo cardiomyopathy and left atrial myxoma: does an association exists?

A Sajjad*

H Mazek

TTUHSC, Lubbock, TX

Case report

Transient left ventricular apical ballooning syndrome (Takotsubo cardiomyopathy) is characterized by transient LV dysfunction, EKG changes that can mimic acute myocardial infarction, and minimal release of myocardial enzymes in the absence of obstructive coronary artery disease. It is typically preceded by exposure to emotional or physical stressors, although in some cases, precipitant stressors have not been identified.

Case presentation

This is a 72-year-old active female with a past medical history of hypothyroidism who presented with acute onset of nausea, vomiting, and diarrhea for one day. She received 2L of intravenous fluid but then developed acute shortness of breath. Patient was in moderate distress and tachypneic but denied chest pain, palpitation, leg swelling, and abdominal pain. Chest exam revealed bilateral crackles. Initial labs were remarkable for Troponin 0.22 and BNP 898 with normal creatinine levels. EKG showed no ST elevations or depressions. Chest x-ray showed mild interstitial edema with small left effusion. Patient was treated for acute pulmonary edema with diuretic and started on acute coronary syndrome protocol. Cardiac catheterization showed normal coronary arteries with severe depression of systolic function and hypokinesia of the mid ventricle and apex with hypercontractile basilar segments consistent with Takotsubo cardiomyopathy. Transthoracic echocardiography (TTE) revealed an ejection fraction of 35–39%, severe hypokinesis of the apical segment, restrictive diastolic dysfunction and mobile echogenic mass consistent with left atrial myxoma. Patient symptoms improved, however TTE repeated 6 days later did not show any improvement in the LV systolic function. Patient underwent surgery, myxoma was removed and confirmed with pathology. Her TTE repeated 4 months later showed significant improvement of LVEF 65–69% with no regional wall motion abnormalities.

Discussion

Takotsubo cardiomyopathy was initially recognized in Japan in 1990 with the first report emerging from the United States in 1998. Cardiac myxomas, although rare, are the most common primary benign tumors of the heart. In our case report, the patient was diagnosed with atrial myxoma which could be one of participating factors for stress induced cardiomyopathy. Reviewed literature showed only 2 cases reports with similar presentation.

119 A special case of uncorrected tetralogy of fallot in pregnancy

H Shi*

C Caplan

D Brady

R Blewett

S Tunovic

Tulane University, New Orleans, LA

Case report

Adult congenital Tetralogy of Fallot (ToF) patients in the United States often receive surgical repair in infancy or childhood. Pregnant women with corrected ToF can suffer right sided heart failure, pulmonary regurgitation as well as prematurity, abortion, and small for gestation age deliveries. However, although very rare, uncorrected ToF women that become pregnant have morbidity rates up to 60–70% with poor fetal prognosis.

Case presentation

A 20 year-old nulligravida female with a history of uncorrected ventricular septal defect (VSD), presented for delivery at 34 weeks of pregnancy. A transthoracic echo (TTE) showed ejection fraction (EF) 55%, severe right ventricular hypertension with estimated systolic RV pressure (RVSP) 153 mmHg. Cardiac MRI (figure 1 below) showed right-sided aortic arch with bidirectional VSD. Decision for a high-risk Cesarean section delivery was made; she was extubated uneventfully after. 3 months after discharge, she underwent a VSD patch closure, right ventricular (RV) muscle bundle division, and RV outflow tract patch for her ToF. Repeat TTE showed significantly improved RVSP to 40 mmHg. The baby was born with prematurity and a small secundum atrial septal defect.

Discussion

Very limited (two studies with <10 patients each) analyses of pregnant women with uncorrected ToF exist. Careful and active postpartum monitoring and a multidisciplinary effort at our institution was essential to a favorable outcome.

Conclusion

Here we describe a successful delivery of a child in an uncorrected ToF pregnant mother. This case serves as a reminder of the profound physiologic changes in cyanotic congenital heart disease.

Abstract 119 Figure 1

from clockwise: 2-chamber short axis, 2-chamber coronal view showing over-riding aorta, and 3-chamber axial view showing hypertrophied right ventricle (RV) and abnormal connection to left ventricle (LV) from clockwise: 2-chamber short axis, 2-chamber coronal view showing over-riding aorta, and 3-chamber axial view showing hypertrophied right ventricle (RV) and abnormal connection to left ventricle (LV)

Endocrinology and metabolism

Joint plenary poster session and reception

4:30 PM

Thursday, February 13, 2020
120 Severe hypercalcemia in a patient treated with lenalidomide

A Eapen*

E Nyenwe

University of Tennessee Health Science Center, Memphis, TN

Case report

Lenalidomide has been used in the treatment of hematological malignancies. We here present a case of severe hypercalcemia in a patient with myelodysplastic syndrome treated with lenalidomide. A 57-year-old Caucasian male with myelodysplastic syndrome presented to the hospital after sustaining a fall in his bathtub. He reported extreme body weakness, poor appetite, polyuria, polydipsia, constipation. He had started lenalidomide four days prior to presentation. He had been treated with azacitidine for approximately two months prior to starting lenalidomide.

On presentation, found to have severe hypercalcemia with corrected calcium of 15.7 mg/dL. He was treated with intravenous hydration, pamidronate and calcitonin. He responded to treatment with improvement in serum calcium level. Other workup revealed PTH 6.7 pg/mL, 25-hydroxyvitamin D 25.5 ng/mL, 1,25-dihydroxyvitamin D <8 pg/mL.

Discussion

There have been a few case reports implicating lenalidomide in the etiology of hypercalcemia in patients with hematological malignancies. In two of the cases, the patients were treated with lenalidomide and rituximab. In the third case, the patient was treated solely with lenalidomide. Serum calcium ranged from 11.78 to 16.95 mg/dL; the patients were treated with IV fluids and steroids in one case and IV fluids, pamidronate, calcitonin and steroids in other instances. These described patients had normal serum vitamin D and PTH levels. One case was noted to have slightly elevated PTHrp, in whom hypercalcemia recurred when challenged with lenalidomide. Hypercalcemia is a rare complication of advanced B-cell malignancies due to lymphokine and cytokine mediated bone disruption; increased production of 1,25-dihydroxyvitamin D by lymphocytes may also lead to hypercalcemia. Other postulated mechanisms include effect of cytokines such as IL-6, TNFα, MIP-1α, which can influence osteoclastogenesis.

Conclusion

The incidence of hypercalcemia with lenalidomide indicates that serum chemistries of patients being treated with this agent should be monitored closely, especially, in the first weeks of therapy.

121 Malignant and treatment-resistant hypertension in a patient with bilateral adrenal hyperplasia

K Ganesan*

AS Sallar

D James

University of Tennessee Health Science Center, Memphis, TN

Background

Treatment resistant hypertension (TRH) may affect about 15% of patients with hypertension; it is mainly diagnosed in obese, elderly patients. Early diagnosis of the mechanism of TRH may prevent or delay the onset of complications. While primary hyperaldosteronism appears to be a major factor in causing TRH, other adrenal hormones need to be considered in young patients.

Case report

45-year-old hospitalized man was referred to endocrinology for evaluation of TRH. His past medical history is significant for TRH of five years duration, ischemic cardiomyopathy, congestive heart failure (Ejection Fraction 45–50%) and chronic kidney disease. Physical examination was unremarkable except for a blood pressure (BP) of 193/124 mmHg while on Lasix, isosorbide mononitrate, hydralazine, carvedilol and clonidine. Chart review showed occasional serum potassium levels just below the normal range over the last year. Computed tomography of the abdomen without contrast showed bilateral enlarged nodular adrenal glands that had increased over the last 3 years with left adrenal gland of 6.3 cm and right adrenal gland of size 5.6 cm. Initial work up showed normal levels of free plasma metanephrine, normetanephrine, aldosterone renin ratio, 17-hydroxyprogesterone and undetectable random adrenocorticotropic hormone with random cortisol of 29 mcg/dl. Subsequent evaluation revealed elevated deoxycorticosterone (3030 ng/dL), 11-deoxycorticosterone (42 ng/dL) and 18-hydroxycorticosterone (640 ng/dL). His BP subsequently became well controlled after the addition of Spironolactone and was referred to general surgery for adrenalectomy. Two months later, he appeared mildly Cushingoid and was diagnosed with new onset diabetes mellitus with hemoglobin A1c of 11.1%.

Conclusion

Workup for TRH, especially in younger patients, should not be stopped after ruling out the ‘usual suspects’ since malignant hypertension resulting in end organ dysfunction can develop if not appropriately treated. In our patient, TRH was due to elevated 18-hydroxycorticosterone (precursor of aldosterone) secretion from bilateral adrenal hyperplasia. It appears that the adrenals were also producing excess cortisol, which most likely contributed to the development of his new onset diabetes.

122 Uncontrolled diabetes mellitus in a patient with lipohypertrophy

K Ganesan*

J Ross

E Nyenwe

University of Tennessee Health Science Center, Memphis, TN

Introduction

We present a case of uncontrolled diabetes mellitus in a patient with pronounced lipohypertrophy (LH) due to repeated insulin injection at a single site.

Case report

59 years old woman with history of hypertension, hyperlipidemia, morbid obesity, and chronic kidney disease was seen in the endocrinology clinic for uncontrolled diabetes mellitus. She was diagnosed with diabetes mellitus 26 years ago and at the time of evaluation, she was using insulin detemir 100 units twice a day and insulin lispro 100 units with meals. Her blood glucose levels ranged from 64 to 300 mg/dL. Her last hemoglobin A1c was 13.7%. On exam, she had LH with lichenification over the lower aspect of the right upper quadrant of her abdomen, the only site she reported injecting insulin for many years. She was counseled on varying the site of insulin injection. To reduce the volume of insulin administered, her insulin was changed to regular insulin U-500.

Discussion

LH is excess fat deposition at sites of insulin injection, since repeated administration of insulin in the same site concentrates the lipogenic effect of insulin. Insulin absorption is reduced in areas of LH, increasing the risk of hyperglycemia. In addition, hypoglycemia may occur when a patient arbitrarily injects insulin into healthy tissue, exposing the body to a higher insulin load. Periodic self-examination at home and detailed inspection of injection sites is necessary during office visits, as patients may be unaware of gradual skin changes. Patient may use an injection site up to one week before rotating. If LH develops at an injection site, that site should be avoided until the skin becomes normal. After LH is diagnosed, insulin dosage may need to be reduced once patients begin to use new, healthy injection sites. If a site of LH does not return to normal after conservative management, liposuction is a surgical option.

Conclusion

LH is a complication of insulin administered with poor technique, and it contributes to poor glycemic control and risk of hypoglycemic events. While it is important to examine injection sites and counsel on best practice at all patient visits, LH should be considered and addressed if a patient’s blood glucose is labile or resistant to insulin treatment.

123 An atypical case of acromegaly debuting as sudden DKA

K Hernandez Moya*

I Rivera

A Nieves-Ortiz

NI Vergne

JM Garcia Puebla

San Juan City Hospital, Guaynabo, PR

Case report

Acromegaly is a rare clinical syndrome resulting from excessive growth hormone secretion with an annual incidence of 6–8 cases per 1M individuals with a mean age between 40–45 y/o. Pituitary adenomas are one of the principal reasons of anterior pituitary somatotroph cells overgrowth and account for approximately one third of all hormone-secreting pituitary adenomas with an incidence of 3–4 cases per 1M individuals. The onset of acromegaly is insidious and at the moment of diagnosis, approximately 75% of patients have macroadenomas, after extensive workup and incidental brain imaging. One of the most common preceding diagnosis of acromegaly is the development of diabetes mellitus and presentation with DKA de novo could be a determining factor in disease progression due to higher GH levels correlating with an increased prevalence of insulin resistance.

A 28y/o G5P3A2 female without PMHx presented to ER with abdominal pain, malaise, dysarthria, headache and gait difficulty of 3 days of evolution. Upon initial evaluation, patient was found with blood glucose of 317 mg/dL, high anion gap metabolic acidosis and ketones suggestive of DKA de novo. Head CT performed showed an incidental parasellar/suprasellar/temporal hyperdense mass of 2.5 × 2.6 cm with optic chiasm compression. Upon further questioning, she referred progressive amenorrhea of 3 years, bitemporal hemianopsia, galactorrhea, marked facial feature changes, frontal bossing, weight gain, and acanthosis nigricans for 1 year. Pituitary adenoma workup revealed low prolactin levels (1.38), markedly increased growth hormone (501) and IGF-1 (893) suggesting acromegaly, most likely secondary to a functioning macroadenoma. She was initially treated with cabergoline, diabetes was managed and was referred to Neurosurgery service for further evaluation and tumor removal.

Based on current literature, the incidence of acromegaly is low, more specifically when presenting with new onset DKA and marked insulin resistance as secondary manifestation of functioning pituitary macroadenomas. Medical awareness should be promoted to assess for consideration of signs, symptoms, workup, management and treatment to minimize further health complications and physical burdens acromegaly and pituitary adenomas could pose for affected patients.

124 Immune reconstitution inflammatory syndrome manifesting as hyperthyroidism in an HIV patient

DW Jones*

S Cross

S Dagogo-Jack

University of Tennessee Health Science Center, Memphis, TN

Introduction

Immune reconstitution inflammatory syndrome (IRIS) involves the paradoxical development of infectious signs and symptoms or autoimmune conditions after starting antiretroviral therapy (ART) in patients with HIV. We present a case of hyperthyroidism associated with HIV and ART, explained by IRIS.

Case

A 32 year-old Ethiopian male, diagnosed with HIV in 2016, began treatment with ART which included dolutegravir/abacavir/lamivudine shortly after diagnosis. In April 2016, HIV viral load was 372,650 copies/mL and CD4 count was 224 cells/µL. He stopped therapy in June 2017 and established care with our clinic in June 2018. At that time, viral load was 78,900 copies/mL and CD4 count was 609 cells/µL. He resumed his original ART regimen in July 2018. By December 2018, viral load was undetectable and CD4 count was 694 cells/µL.

One month after resuming ART, the patient reported fatigue, palpitations and dizziness. On exam, heart rate was 124 bpm. Serum TSH was 0.00 µIU/ml (nl: 0.34–5.60) and FT4 was 8.22 ng/dl (nl: 0.58–1.64). Propranolol was started and he was referred to endocrinology. During endocrinology evaluation, he denied family history of thyroid or autoimmune disorders. He was clinically and biochemically hyperthyroid, without exophthalmos or palpable goiter. The 24-hr radioiodine study showed a diffuse uptake of 72.8% (nl: 10–30%). TPO and TSI antibodies were ordered; results are pending. Methimazole was added and follow-up evaluation showed clinical improvement and decreasing FT4.

Discussion

This 32-year-old man without family history of endocrine or autoimmune disorders developed hyperthyroidism within one month of resuming ART. Male sex, lack of family history and the temporal relationship between resumption of ART and developing hyperthyroidism suggest an underlying mechanism of IRIS rather than classic Graves’ disease. Our patient had a rapid, drastic decline in viral load after resuming ART (a known independent risk factor for developing IRIS). This case demonstrates the need for increased awareness of thyroid dysfunction as a manifestation of IRIS in HIV patients receiving ART.

125 How does metformin produce its anti-cancer effects?

S Kode1*

A Amro2

D James1

A Vacheron1

S Solomon1,3

1University of Tennessee, Memphis, TN

2Meharry Medical School, Nashville, TN

3VA Medical Center, Memphis, TN

Purpose of study

For the past 7 years, we have been able to demonstrate significant anti-cancer properties for the biguanide drug, Metformin, which is used extensively in treatment of Diabetes Mellitus 2 (DM2). Previous retrospective chart review data from patients have demonstrated significant improvement in outcomes, including reducing the risk of developing certain cancers, reducing the progression of metastases of a primary tumor, and also increasing survival time in patients suffering from prostate, colon, lung, thyroid, esophageal, pancreatic cancer and lymphoma. Aim of our study is to investigate the mechanisms underlying the anti-cancer effects of metformin.

Methods used

We propose to explore mechanisms of the anti-cancer effects of Metformin through our own past publications and review of other scientific literature.

Summary of results

Possible mechanisms for Metformin’s anti-cancer effects are the following: 1) Metformin decreases circulating insulin levels by increasing the sensitivity of cells to insulin. Decreased insulin and Insulin-like Growth Factor levels, both of which are growth factors that stimulate cell growth and inhibit cell apoptosis, leads to reduced stimulation of growth of cancer cells; 2) Metformin works through AMP kinase, which phosphorylates raptor, inhibiting mTOR and preventing it’s activation (inhibits cell growth by this and other mechanisms, including possibly through rewiring of it’s signaling); 3)Metformin activated AMP-kinase inhibits the Warburg effect (anaerobic glycolysis) and suppresses tumor growth; 4) Metformin induces apoptosis and encourages cell cycle arrest through it’s effects on Cyclin D ;5) Metformin blocks action of Hexokinase II, disrupts metabolism of glucose, and facilitates anti-neoplastic activity through inhibition of cancer cell metabolism; 6) Metformin indirectly inhibits mTOR which leads in multiple ways, to inhibition of cancer cell growth; 7) other.

Conclusions

Understanding the antineoplastic activity of Metformin through both mTOR and other key pathways, may help in the development of adjunct cancer treatments in both diabetic and non-diabetic patients.

126 Pheochromocytoma presenting as a stroke

K Sanders*

MB Omar

R Patel

UF Health COM Jacksonville, Jacksonville, FL

Case report

A 53-year-old man with a history of depression, multiple suicide attempts, and premature ischemic strokes who presented with sudden-onset left-sided weakness and aphasia. Patient was tachycardic, tachypneic and hypertensive. Although afebrile, he displayed diaphoresis, rigidity, and myoclonus with hyperactive reflexes on physical exam. CT head was negative for acute ischemic stroke. Given a history of prior suicide attempts and multiple serotonin reuptake inhibitors on medication list, clinical picture was more concerning for serotonin syndrome and was admitted for cyproheptadine treatment. However, his tachycardia, hypertension, and diaphoresis persisted. MRI brain was negative for meningitis but notable for acute ischemic cerebral infarct. Of note, prior extensive workup for evaluation of premature stroke was negative. CT chest revealed an incidental finding of bilateral adrenal gland thickening. Urine and plasma metanephrines were significantly elevated. MRI of the abdomen was limited due to motion artifact but was notable for bilateral adrenal hyperplasia without enhancement or cystic changes. Given these findings, treatment with a selective alpha blocker was started with improvement of his symptoms. Upon discharge, an outpatient MIBG scan was recommended to evaluate for presence of pheochromocytoma versus paraganglioma.

Pheochromocytoma is a rare yet important cause of secondary hypertension. Devastating cardiovascular complications can arise if the condition goes unrecognized and untreated. The episodic nature of this tumor makes it difficult to recognize and diagnose. The classic triad of paroxysmal headaches, palpitations, and diaphoresis presents in less than half of patients. Rarer still are the cerebrovascular manifestations of pheochromocytoma. Although this patient left before an MIBG scan could be obtained to confirm pheochromocytoma, his history of multiple premature strokes, labs, and imaging findings strongly suggest pheochromocytoma. We highlight this case to draw attention to the possibility of pheochromocytoma in patients presenting with hypertension and neurologic deficits, especially in young patients lacking the traditional risk factors, as early diagnosis and treatment can prevent life-threatening complications and reduce morbidity and mortality.

127 Hypertriglyceridiemia induced pancreatitis … but how high is the triglyceride?

K Sawalha*

AT Kunnumpurath

R Kamoga

White River Health System, Batesville, AR

Introduction

Hypertriglyceridemia induced acute pancreatitis is well reported etiology in 1–14% of cases of pancreatitis of at least 1000 mg/dL 1. The management of HTG-induced acute pancreatitis is usually supportive care. Insulin or aphaeresis may be given to help lower hypertriglyceridemia.

Case

A 28 year old Caucasian female with a past medical history of insulin dependant type 2 diabetes mellitus, dyslipidemia, obesity and recurrent pancreatitis presented with epigastric, bilateral upper abdominal pain, nausea and vomiting that started in the morning after eating her breakfast. She denied any history of smoking, gallbladder stones, alcohol intake or any new medication. On admission she was found to have WBC 12.2 K/ul, triglyceride level of 11602 mg/dl,amylase 308 U/L, lipase 5517 U/L correlating with acute severe pancreatitis.

Abdomen-pelvic CT showed findings consistent with pancreatitis with peripancreatic fluid with no evidence of pseudocyst or walled off necrosis.She was started on insulin drip in the following which her triglyceride decreased to 4783 mg/dl on the second day with insulin alone.Her gallbladder ultrasound and HIDA scan was negative for stones or acute but showed chronic cholecystitis.Insulin drip was stopped when her triglyceride level was less 1000 mg/dl. Her abdominal pain resolved and she was discharged on insulin, statin, fenofibrate, niacin.

Conclusion

Hypertriglyceridemia is defined by fasting serum triglyceride level of >150 mg/dL and is classified as very severe when level ≥2000 mg/dL. The risk of developing acute pancreatitis is approximately 10 to 20 percent with triglycerides >2000 mg/dL as the breakdown of triglycerides into toxic free fatty acids by pancreatic lipases is the cause of lipotoxicity during acute pancreatitis. The severity of acute pancreatitis in patients with hypertriglyceridemia is dependent on both the inflammatory response caused by pancreatitis itself, plus the injury caused by lipotoxicity from triglyceride hydrolysis. The unique feature of our case can be emphasized with the quick and effective response to insulin therapy alone. Additionally, the cost-effectiveness of plasmapheresis remains uncertain. We recommend for patients with Diabetes Mellitus, Hyperlipemia and recurrent pancreatitis to be on insulin pump to prevent further attacks.

128 Thyroid storm: secondary to infection versus iodinated contrast

V Silver*

H Oddo Moise

H Tran

C Saraceni

LS Engel

M Modica

LSU Health Sciences Center, New Orleans, LA

Introduction

Thyroid storm is a rare, life-threatening condition characterized by severe clinical manifestations of thyrotoxicosis. Patients typically present with exaggerated symptoms of hyperthyroidism, and are at increased risk for cardiovascular collapse.

Case

A 38 year old woman with a history of hyperthyroidism, presented with a 2 day history of left sided flank pain, subjective fevers, and nausea and vomiting. The patient’s work up revealed a 5 mm left ureterovesical junction stone with associated moderate hydronephrosis, perinephric fluid and fat stranding per CT scan of abdomen and pelvis. Prior to hospital discharge the patient was found to be tachycardic, dyspneic, and febrile. The concern was that the patient was septic secondary to pyelonephritis and possibly was in thyroid storm. The physical exam was significant for a large, boggy goiter. Initial labs revealed a leukocytosis of 16.9 cells/mm3, TSH 0.14 UIU/ml with Free T4 5.33, and a lactic acidosis of 3.8 mmol/L. The patient was admitted to the ICU and was started on broad-spectrum antibiotics for pyelonephritis, methimazole with iodine drops and propranolol for suspected thyroid storm. Further studies revealed a diffusely enlarged, heterogeneous thyroid gland with three echogenic nodules on the left and a small nodule on the right. A left ureteral pigtail stent was placed by Urology. An echocardiogram did not demonstrate high output heart failure in relation to her tachyarrhythmia from thyrotoxicosis. She was eventually discharged home to complete a course of antibiotics for pyelonephritis. She was maintained on methimazole for hyperthyroidism and follow up with endocrine and ENT for management of her goiter including thyroid biopsy.

Discussion

Thyroid storm is a rare complication of hyperthyroidism, usually precipitated by surgery, infection, or contrast load. Physicians should carefully monitor and screen patients for thyroid disease if considering a contrasted study in the setting of infection, especially in patients who are non-compliant or have not started treatment for thyroid disease.

129 Pancreatogenic diabetes mellitus, an uncommon complication of pancreatitis

H Tran*

W Baumgartner

S Vignes

J Van Dyke

LS Engel

L Nunez

LSU Health Sciences Center, New Orleans, LA

Case report

Pancreatogenic Diabetes, classified by the American Diabetes Association (ADA) and World Health Organization (WHO) as Type 3c diabetes Mellitus (T3cDM), is a secondary form of diabetes. Chronic pancreatitis is associated with nearly 80% of patients diagnosed with T3cDM

Case

A 45 year old woman with a history of hypertension, constipation, and hypercalcemia presented with intermittent cramping abdominal pain, nausea and emesis for 2 weeks. She was started on hydrochlorothiazide for hypertension 5 days prior. Physical exam demonstrated epigastric and LLQ abdominal pain. Her initial labs included a Glucose of 148 mg/dL, Calcium of 15.8 mg/dL, Phosphate of 0.9 mg/dL, Lipase of 548 U/L, and PTH 282.2 pg/mL. CT imaging showed peripancreatic inflammatory stranding and fluid in the upper abdomen consistent with pancreatitis. She recieved IVF, pain control, and calcitonin. The following day, her glucose was 314 mg/dL, her hemoglobin A1c was found to be 5.3%. Zoledronic acid was administered, as her hypercalcemia did not improve. Endocrinology suggested that her pancreatitis was due to hypercalcemia from a parathyroid adenoma that worsened after taking hydrochlorothiazide. She initially improved, but 3 days into her hospitalization her abdominal pain acutely worsened and she required basal insulin. Repeat imaging revealed acute necrotizing pancreatitis. ENT performed a parathyroidectomy. Her clinical symptoms improved, and the basal insulin was no longer required. However, at her follow up endocrinology appointment, her glucose was noted to be >500 and she required basal and prandial insulin therapy. At her 3- and 6- month appointments, she still required basal and prandial insulin and c-peptide was 0.8, consistent with pancreatogenic diabetes mellitus.

Discussion

Here we present a case that illustrates the acute and chronic complications of even a single episode of pancreatitis. Previous meta-analysis showed that 15% of patients developed diabetes within 12 months after their first episode of pancreatitis. Insulin therapy is the preferred treatment for T3cDM due to exocrine pancreatic insufficiency. Our case highlights the importance of adequate follow-up for hyperglycemia in patients with pancreatitis.

130 Relapsing diabetes ketoacidosis during step down from intesive care unit

Y Zakai*

S Dagogo-Jack

University of Tennessee Health Science Center, Memphis, TN

Introduction

Diabetic ketoacidosis (DKA) is a life-threatening complication of diabetes. Treatment of DKA is resource intensive and expensive, costing $5.1 billion annually. Recurrent DKA accounts for ~ 20% of DKA admissions. Here we present a patient with recurrent/relapsing DKA during transition from ICU to the medical floor.

Case presentation

A 61-year-old previously healthy man presented with 3-day of generalized weakness and nausea. Review of systems was positive for polyuria and polydipsia. His brother has type 2 diabetes (T2D). On examination, he was afebrile, pulse was 166/min, BP 146/104 mmHg. Oropharynx was dry, and sensations were intact. Labs: Serum glucose was 689 mg/dl, sodium 126 mmol/L, potassium 4.8 mmol/L, chloride 78 mmol/L, bicarbonate 8 mmol/L, creatinine 4 mg/dl, anion gap (AG) >20, hematocrit 57.6%, HbA1c 10.4%, c-peptide 1.5ng/ml. Urine: ketones++. A diagnosis of new-onset diabetes presenting with DKA was made.

Management

Patient was admitted to ICU and insulin and saline were infused per protocol. Because the AG closed promptly and bicarbonate improved to 20 mmol/L, insulin drip was stopped in the ICU and patient was transferred to medical floor. Evaluation on the medical floor 4 hours later showed bicarbonate of 14 mmol/L and AG >20. Due to deterioration, patient returned to the ICU for management of recurrent DKA. Following resuscitation in ICU, patient was returned to the medical floor and was successfully discharged on basal-bolus insulin.

Discussion

Recurrent DKA due to abrupt cessation of IV insulin prolonged this patient’s hospitalization. The practice of overlapping IV insulin with SQ insulin for >30 min prevents dissipation of insulin action during resolution of DKA. Half-life of IV insulin is 3 min, so SQ insulin must be given before cessation of the insulin drip to prevent the relapse. Omission of this practice, as occurred in this patient, unfortunately caused relapse in DKA and prolonged hospitalization. Education of ICU staff on this practice is warranted to prevent healthcare cost: the cost of a DKA hospitalization was $26,566 in 2014.

Gastroenterology

Joint plenary poster session and reception

4:30 PM

Thursday, February 13, 2020
131 A case report of a solid pseudopapillary neoplasm with unusual demographic and location features

A Alhussain*

A Shalaby

D Grunes

University of Mississippi Medical Center, Jackson, MS, USA Minor Outlying Islands

Case report

Solid pseudopapillary neoplasm (SPN) is an uncommon pancreatic neoplasm typically seen in young women as a pancreatic body or tail mass. We present a case of a 19 year-old African-American male who presented with abdominal pain, weight loss and jaundice. Computed tomography scan showed a large mass in the pancreatic head and patient underwent Whipple procedure. Gross examination showed a well-circumscribed mass in the pancreatic head with central hemorrhage and necrosis (figure 1A). Histologic sections revealed a largely necrotic tumor with solid nests of viable cells with characteristic pseudopapillae (figure 1B). Neoplastic cells had amphophilic cytoplasm with uniform nuclei showing occasional grooves and focal aggregations of hyaline globules (figure 1C). Tumor cells were positive for B-catenin (figure 1D), CD10, alpha-1 antitrypsin and synaptophysin and negative for E-cadherin and chromogranin. SPN should be differentiated from other solid cellular pancreatic tumors such as pancreatic neuroendocrine tumor, acinar cell carcinoma and pancreatoblastoma using clinical features along with immunohistochemical stains. SPN is considered a neoplasm with low-malignant potential and excellent overall prognosis. However, local recurrences and metastases have been reported in 10–15% of cases. Our patient had no evidence of metastasis and continues to do well one year following surgery. In the literature, SPN tends to be more aggressive in older male patients. However, the behavior of SPN in the head of the pancreas of a young African-American male is unclear.

Abstract 131 Figure 1

Gross examination showed a well-circumscribed mass in the pancreatic head with central hemorrhage and necrosis Gross examination showed a well-circumscribed mass in the pancreatic head with central hemorrhage and necrosis

132 Black cohosh induced cholestatic liver injury

HS Brar*

UMMC, Flowood, MS

Case report

Black cohosh, a herbal product used to treat menopausal symptoms is associated with hepatotoxicity. The spectrum varies from acute hepatitis to FHF.

A 50 year old AAF presented with a several month history of severe jaundice, fatigue, weight loss and pruritis. Work up at outside hospital was inconclusive and was started on Ursodiol. Her known medical problems are HTN, anemia, COPD and asthma. Her medications included FeSo4, Albuterol, Symbiocort, Norethindrone, Coreg and Pepcid. Herbal medicines included Black cohosh for 3 months for hot flashes. Family history is positive for Lupus and PSC. She denied tobacco, alcohol or illicit drug use. There was no history of recent travel, transfusions, tattoos or sick contacts. Physical exam was positive for alopecia, icterus, RUQ tenderness and no ascites. Laboratory studies revealed Total bilirubin 26.20 with direct 17.14, AST 37, ALT 26, ALP 207, GGT 21. PT/INR 11.9/1.02. H/H 9.9/28.7. MCV 71.9. Albumin 3.6. Serologies for : Hepatitis A,B and C, CMV, EBV, ANA, AMA Ab, anti-sm Ab, anti-LKM Ab were negative. Serum ceruloplasmin and Alpha-1 antitrypsin levels were elevated.

CT Abdomen showed multiple liver hemangiomas. ERCP demonstrated the CBD and common hepatic ducts were approximately 5 mm,smooth and without stricture. The biliary duct system was completely normal without ductal irregularity. EUS demonstrated no sign of significant pathology in the main pancreatic duct and CBD.

Liver core biopsy showed cholestasis with mild lobular and portal inflammation with mixed inflammatory infiltrate with equicoval features of biliary injury with no interface activity. There were no changes diagnostic of PBC. Intra-hepatic band cholestasis with bile within dilated bile canaliculi was seen. Findings suggested drug-induced liver injury which was confirmed at Mayo Clinic. Patient was started on Budesonide. Bilirubin decreased to 13.

Black cohosh(Actaea racemosa) is used for postmenopausal symptoms. It may contain Formononetin, an estrogenic isoflavone. There have been few reported cases of hepatotoxicity associated with it’s use, some of which resulted in liver transplantation. This case presented had no known hepatic history, no potential hepatotoxic usage and a negative workup for any pathology. Biopsy confirmed Drug-induced liver injury. It hereby emphasizes the importance of recognizing herbal supplements like black cohosh as a cause of liver failure.

133 It’s not working out: a rare case of whey-protein-induced liver injury

L Buchanan*

S Iqbal

P Hosseini-Carroll

University of Mississippi Medical Center, Jackson, MS

Case report

Drug-induced liver injury (DILI) is a common cause of liver injury and failure. It has an incidence of 14–19 per 100,000 cases and is responsible for 5% of hospital admissions for jaundice. Medications such as Tylenol or antibiotics are commonly associated with DILI, but injury from dietary and herbal supplements is increasing. From 2004 to 2014 there was an estimated rise from 8% to 20% of supplement-induced liver injury. Here we document a case of severe DILI caused by ingestion of whey protein powder workout supplement, a poorly described cause of DILI.

Our patient is a 44-year-old male with no medical history who has been incarcerated since 2010. He presented to an outside hospital after two weeks of worsening fatigue and jaundice. He had decreased appetite, nausea, vomiting and a 40lb weight loss over the last month. On admission to our facility, ALT and AST were mildly elevated to 53 and 64, respectively. His alkaline phosphatase was 312 and total bilirubin was 26.86 with a direct bilirubin of 19.01. Viral, autoimmune, and other causes of liver injury were ruled out. ERCP revealed no stricture, obstruction or mass. Liver biopsy was performed with subsequent pathology suggesting DILI. He denied any medication use but admitted to taking whey protein (Universal Super Whey) supplementation daily for several months. About a month prior to symptom onset, he had increased his usage, consuming a minimum of 5 scoops, up to 3 times a day. Supportive care was provided and over the next two weeks, he improved and was discharged.

DILI is the most common cause of acute liver failure in the Western world. While often associated with medications, the incidence of DILI from dietary and herbal supplements has been rising. It can lead to more severe and prolonged cases of liver injury, as shown here. Additionally, liver injury secondary to supplements often presents with a cholestatic pattern, as also seen in this case. While no available ingredients for Universal Super Whey are on the NIH liver toxicology list, there have been a few documented cases of whey-protein induced liver injury. When faced with DILI from an unknown source, protein supplementation, including whey-protein, should be considered.

134 Two cases of false positive hepatitis B surface antigen with positive hepatitis B E-antigen

NJ Shah1,2

MJ Burton2*

E Paine1,2

1University of Mississippi Medical Center, Madison, MS

2G.V. (Sonny) Montgomery VA Medical Center, Jackson, MS

Case report

Hepatitis B surface antigen (HBsAg), is a HBV surface protein particle that indicates current HBV infection. Hepatitis B e Ag (HBeAg) indicates active HBV replication. HBsAg, is a sensitive test, and weakly positive HBsAg test needs to be further confirmed using a neutralization test to confirm infection. Below we have briefly described two cases of unusual hepatitis B serology.

Case 1: 78-year-old Caucasian male, who initially, on history he had provided positive risk factors for hepatitis B infection. He denied any recent vaccinations. The transaminases were within normal limits. The hepatitis B surface antigen (HBsAg) was positive. However, the neutralization test was not confirmatory on two occasions. He had hepatitis B e Ag (HBeAg) positive as well. He was negative for Hepatitis B surface antibody (HBs Ab), hepatitis B core total antibody (AntiHBc-T), hepatitis B core IgM (AntiHBc- IgM) and HBV e antibody (anti-HBe). The hepatitis B viral DNA was also negative. The patient was negative for hepatitis C and HIV. The results were similar 6 months later.

Case 2: 65-year-old Caucasian male, with psoriatic arthritis, was screened for hepatitis B as part of his management for psoriatic arthritis and was found to have a reactive HBsAg. He had no defined risk factors for HBV. His initial HBsAg was negative 7 years back. His hepatitis B serological profile was similar to the first patient.

Discussion and review of literature

Heterophile antibodies could explain why the confirmatory test for HBsAg, after neutralization, was negative. Our patient had not received the HBV vaccination. Also, it does not explain why the HBeAg would be positive. HBsAg ‘escape’ mutants are generally considered when the HBeAg and/or the HBV DNA is positive, in chronic HBV infection, which was not our case.

Conclusion

Present HBV guidelines do not define our case. We were unable to identify similar case reports. We continue to monitor our two patients, for if we decide to start them on hemodialysis (our first patient) or initiate immunosuppressive agents (for our second patient), the challenges of HBV reactivation are real.

135 A case of purtscher’s retinopathy secondary to acute alcohol induced pancreatitis

R McPeters

T Thorne

TV Joshi

M Bilalis

LS Engel*

J Amoss

S Landreneau

LSU Health Sciences Center, New Orleans, LA

Case report

Purtscher’s retinopathy is a rare condition first described following intracranial trauma. This occlusive microvasculopathy is characterized by sudden visual loss with multiple areas of retinal whitening in the posterior pole of the eye. Similar findings have been seen in atraumatic patients termed Purtscher-like retinopathy (PuR). PuR has been described with acute pancreatitis and connective tissue diseases. PuR, associated with systemic disease, develops through the activation of the complement cascade which leads to leukoembolization of the retinal precapillary arterioles and cause the clinical appearance of Purtscher’s retinopathy.

Case

A 28 year-old woman with a history of alcohol abuse and multiple of admissions for acute pancreatitis presented with chief complaint of emesis and abdominal pain. Physical exam, initial blood tests and abdominal ultrasound were consistent with acute pancreatitis. The patient received appropriate fluid resuscitation and adequate pain control. During her hospital stay, the patient awoke from a nap, noted blurry vision, and saw ‘little dots floating around,’ which almost completely resolved in the evening. The following morning, she had worsening blurry vision and floaters. Exam by Ophthalmology revealed cotton wool spots, macular edema and neurosensory detachment of the macula bilaterally secondary to PUR. Visual field testing showed loss of central field bilaterally with preserved peripheral vision. Visual acuity was 20/200 bilaterally with no improvement with pinhole. The patient received intraocular steroid injections with weekly follow up. Examination at 8 weeks demonstrated improvement of visual acuity to 20/40 bilaterally.

Discussion

The development of PuR is independent on the severity of pancreatitis and presents with a wide range of manifestations. The diagnosis is made clinically with sudden loss of visual acuity associated with optic nerve edema and visual field loss, along with retinal findings such as cotton-wool spots, retinal hemorrhage, artery attenuation, venous dilation, and Purtscher flecken. The treatment for the ocular complications of PuR have not been proven and prognosis depends on the areas of the retina that are affected.

136 New insights into dumping syndrome: more wine please!

JD Herlihy1*

C Phan2

R McCallum1

1Texas Tech El Paso, El Paso, TX

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

Case report

Dumping syndrome (DS) occurs after meals due to rapid gastric emptying of voluminous hyperosmolar contents resulting in early and late symptoms including tachycardia, abdominal pain, nausea, vomiting, bloating, reactive hypoglycemia, syncope, and diarrhea. The main etiologies of DS are vagal nerve injury related to diabetes mellitus or previous gastric or esophageal surgeries, and an ‘idiopathic’ subgroup.

We report a 65 year old female with DS following Nissen fundoplication presenting with abdominal pain, bloating, nausea, vomiting, and diarrhea immediately after meals. A 4 hour gastric emptying study (GES) utilizing the standardized isotope labeled egg beater meal (250kcal) showed 71% emptied at one hour, meeting criteria for DS of >65% emptied by 1 hr, accompanied by the expected spectrum of DS symptoms. She was treated with diet modification, dicyclomine, glycopyrronium bromide, and octreotide injections for four years with only moderate success and was also complaining of side effects from treatment. The patient noticed that when she drank an 8oz glass of red or white wine, specifically beginning before eating and continuing throughout the meal, her DS symptoms were eliminated. We subsequently repeated the GES using the same nuclear medicine methodology with the patient drinking a glass of wine in her usual fashion and this resulted in a completely normal pattern of gastric emptying, specifically only 23% emptied at 1 hr, with no accompanying symptoms (see detailed results of both gastric emptying studies in table 1).

Literature has identified that ethanol inhibits the release of several neurotransmitters in the GI tract, including acetycholine, thus reducing smooth muscle contractions and inhibiting the tonic ‘pressure pump’ mechanism that drives chyme into the small intestine. This is one hypothesis that could explain the efficacy of a glass of wine with meals in our patient with DS. We conclude that this observation does open the door for further research and potentially new therapy for dumping syndrome.

Abstract 136 Table 1

Gastric emptying studies Gastric emptying studies

137 Unexpected cause of altered mental status

SE Huse*

T Cuoco

Medical University of South Carolina, Charleston, SC

Case report

A 63 year old man with a past medical history of multiple myeloma in remission and pancytopenia was brought to the emergency department by his family for evaluation of strange behavior with fever of 102. His wife noticed he seemed intermittently confused and lethargic with uncharacteristic behaviors. The patient reported feeling diaphoretic, but denied chills, sore throat, shortness of breath, abdominal pain, diarrhea, neck stiffness, or vision changes. Notably he is known to frequently work outdoors and had removed 2 ticks from his body over the past week. He denied any new rashes. Initial vital signs included a temperature (102.2), BP 111/68, pulse of 76, and respiratory rate of 15. On exam he was well nourished with a benign HEENT, cardiovascular, respiratory and abdominal and neurologic exam. The patient was admitted to medicine and labs were sent to rule out tick vector illness (Lyme, anaplasmosis, erhlichia). He was started on empiric doxycycline in addition to broad-spectrum vancomycin and Zosyn. The following day, his encephalopathy cleared, however he developed 6–8 loose bowel movements with continued impressive fevers (103). The stool GI PCR identified salmonella, and this was treated with levofloxacin. The patient quickly defervesced and had cessation of diarrhea. The tick vector workup was returned negative, and was discharged with on a 14- day course of levofloxacin.

Non-typhoidal salmonella is a leading cause of gastroenteritis worldwide, and in the US is responsible for over a million illnesses, 23,000 hospitalizations and 450 deaths annually. Salmonella confers over 2,000 serotypes which dictate much of the course of illness. Serotypes typhi and paratyphi are responsible for typhoid fever/enteric fever whereas most other serotypes lead to gastroenteritis. This case highlights the importance of a broad differential diagnosis that is flexible in the light of new information. Additionally, early infectious etiologies may present with vague subjective complaints. Generally, patients can recover with only supportive care, however, immunocompromised or severe cases should be treated with fluoroquinolones as first choice antibiotics. Follow up screening is not recommended as convalescent excretion of salmonella occurs in up to 5 weeks post infection and the incidence of chronic carriers is low (0.2 to 0.06%).

138 Where in the world is H. pylori?

C Joseph*

N Zeky

E McDonough

N Sims

Our Lady of the Lake Children’s Hospital, Baton Rouge, LA

Case report

Helicobacter Pylori (H. Pylori) is the most common bacterial infection worldwide, with higher prevalence in developing nations. In the United States, serologic evidence of H. Pylori infection in children is extremely rare, and steadily increases with age.

A 5 year-old adopted female from India with history of Beta Thalassemia Major (BTM) was admitted for 1 day history of hematemesis. Prior to presentation, she had 2 episodes of non-bloody emesis. On the day of presentation, she had one episode of coffee ground emesis which evolved to become bright red. In the Emergency Department, she was tachycardic but well-appearing. Her hemoglobin was 12.9 gm/dL following a transfusion the day prior for BTM. Her baseline 1hemoglobin is 10.3 gm/dL. Her physical exam was unremarkable other than tachycardia with no abdominal tenderness. She was admitted to the hospital where she was found to have further hematemesis and plan for esophagogastroduodenoscopy in the morning after fluid resuscitation and transfusion. Upon arrival to the floor, patient had additional episodes of hematemesis, became increasingly tachycardic and light-headed. She was then transferred to the Pediatric Intensive Care Unit. Repeat hemoglobin was 7.4 gm/dL. Patient was started on pantoprazole, octreotide drip, packed red blood cell transfusion and was taken to operating room. She was found to have two duodenal ulcers with gastric pyloric changes consistent with H.Pylori gastritis. Clips were applied and biopsies were obtained. Patient was stable following procedure and started on triple therapy of Clarithromycin, Amoxicillin for two weeks, and Omeprazole for six weeks. At follow up appointment, average hemoglobin measured at 9.3 gm/dL.

Given the extremely low prevalence and incidence in pediatric populations within industrialized nations, H. Pylori infection and subsequent complications remain low on clinical differentials. Nevertheless, this case demonstrates the importance of considering social history in patients, especially patients with international adoptive history. Comprehensive approach and special international consideration will allow for earlier screening, identification, and treatment of pathology that would otherwise lead to serious complications.

139 Esophageal phlebectasias: a difficult diagnosis to swallow

TV Joshi*

S Vellanki

J Hutchings

Louisiana State University, New Orleans, LA

Case report

In an attempt to further investigate a three-year complaint of dysphagia as well as evaluate a three-month history of diarrhea, we performed an endoscopic evaluation which revealed a unique case of esophageal phlebectasias. Phlebectasias are non-neoplastic, non-tortuous, fusiform dilatations of veins, and their pathogenesis although not fully understood, is hypothesized to be caused by the thinning of the muscular layer of venous walls. Phlebectasias differ from venous aneurysms as they are not secondarily acquired, and they can also be located in various organ systems. Early diagnosis of phlebectasias effects both its management as well as the prevention of its complications such as rupture and bleeding.

A 59-year-old Caucasian male with history of HTN, GERD, HLD and hypothyroidism presented to clinic with a 3 month history of loose, watery, non-bloody diarrhea that occurs 3 times per day. He reported a long history of GERD improved with Omeprazole and a 3-year history of substernal dysphagia to solids without regurgitation, which improved with fluid wash. Vitals were normal and his physical examination was unremarkable. Endoscopic evaluation of his dysphagia revealed numerous medium sized venous outpouchings with a localized distribution found within the lower two-thirds of the esophagus. Endoscopic ultrasound confirmed venous lakes throughout the esophagus. Manometry has been scheduled to to rule out any other causes of dysphagia.

Most cases of phlebectasias found in the GI tract are asymptomatic and incidentally discovered during either GI procedures or surgeries. Guidelines on treatment have yet to reach a consensus, and most literature recommends close surveillance and conservative therapies over surgical management. Surgical management is needed for those lesions that rapidly evolve leading to risk of rupture and subsequent hemodynamic instability, or in lesions in which the venous dilation can lend itself to forming an intramural thrombus. Phlebectasias are one of multiple vascular anomalies which can exist in the GI tract. Due to the scarcity of published reports, there are currently no associations of dysphagia as a complication of esophageal phlebectasias. Nevertheless, this case demonstrates the seriousness of early recognition to prevent potentially fatal complications.

140 Essence of banding on screening endoscopy to prevent first time variceal hemorrhage

S Khan*

K Vedala

A Kunnumpurath

A Kumar

K Khasawneh

White River Medical Center, Batesville, AR

Case report

In 2017 nearly 4.5 million adults had chronic liver disease and cirrhosis in US. Acute variceal bleeding is one of the major causes of death in patients with cirrhosis. Variceal bleeds account for 70% of upper GI bleeds (UGIB) and mortality reaches 15–20% in first time variceal hemorrhages (VH). Timely intervention with EVL (Endoscopic Variceal Ligation), on screening endoscopy, can help prevent VH and death.

A 67 y/o Caucasian Male with no known prior history presented for melena and ascites. Total Bilirubin of 2.30, Alk Phos 367, AST 96, ALT 47, positive for Hep C, Hgb of 11.9, Hct of 34.1, Plt 231, PT 137 and INR 1.1. CT of the abdomen had revealed large volume ascites, cirrhosis, and mass like areas. The patient subsequently had MRI performed that revealed multiple large masses throughout both lobes. Paracentesis was performed with 5.8L drained. Subsequently patient had EGD and Colonoscopy performed that revealed Grade IV non-bleeding varices in the middle and distal third of the esophagus. No acute intervention was performed. Through the night patient decompensated and care was escalated to ICU where patient became unresponsive and required immediate intubation. Stat Hgb/Hct showed a drop 6.4/20.6. Through the course of the day patient began to actively bleed through oral and rectal orifices and mass transfusion protocol needed to be initiated along with multiple pressor support in order to maintain hemodynamic stability. Surgery team was consulted that eventually needed to insert a Blakemore tube on bedside EGD. Patient was transferred to outside hospital for higher level of care.

Acute variceal hemorrhage can be fatal if not addressed during screening endoscopy. Per AASLD guidelines, recommendations are to have EVL for medium or large sized varices for primary prevention of first time VH. In the case of this patient, Grade IV varices were noted on screening endoscopy in the distal lower and middle third of the esophagus without any intervention. Given that resources are limited and specialty services are not always available in rural hospitals, physicians should have a lower threshold to intervene with EVL on screening EGDs in order to help prevent fatal variceal hemorrhages.

141 Hyperammonemic encephalopathy following 5-fluorouracial therapy: an unusual complication

HK Marella1*

R Peravali1

A Jain1

K Joglekar2

S Nair2

U Agbim2

R Verma2

1University of Tennessee Health Science Center, Memphis, TN

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

Case report

A 66-year-old male with a past medical history of colon cancer status post right hemicolectomy and orthotopic liver transplantation secondary to hepatitis C and alcoholic cirrhosis was found to have recurrent colorectal metastases to the liver and lung. Adjuvant chemotherapy consisting of FOLFOX (5-FU, Oxaliplatin, Folinic acid) and Bevacizumab was initiated. Two days after receiving the third dose of FOLFOX, the patient presented with altered mental status, agitation, abdominal pain, nausea, and vomiting. According to the patient’s wife, symptoms began abruptly three hours prior to arrival to the emergency room. On examination, the patient was afebrile with normal respirations. Patient was normotensive with a blood pressure of 129/85 mmhg and slightly tachycardic at a rate of 105 beats/min. On neurologic exam, patient was disoriented to person, place and time.

Abdominal series and Computed Tomography(CT) head showed no abnormalities. Liver function tests (ALT/AST, ALP, PT/INR, Albumin etc.) were all within normal limits. However, blood ammonia level was significantly elevated at 434 umol/L (normal=11–32 umol/L). Patient was diagnosed with grade III hepatic encephalopathy. The patient was started on Rifaximin 500 mg BID, lactulose 200 g retention enema, and lactulose (30 g/45 ml) via nasogastric tube. Patients 5-FU infusion was stopped immediately and IV hydration was started. Within 24 hours, the patient’s encephalopathy resolved. Hematology/oncology decreased the dose of FOLFOX by 50%.

The biological basis for 5-FU induced encephalopathy is not completely understood, but multiple pathways have been proposed. One possible mechanism contributing to the neurotoxicity is accumulation of toxic by-products of 5-FU catabolism. Fluorocitrate, the final metabolite of 5-FU, has been shown to inhibit aconitase, a Krebs cycle enzyme. Inhibition of the Krebs cycle by 5-FU metabolites can impair the urea cycle, resulting in accumulation of ammonia and lactic acidosis. It is important for physicians to be aware of hyperammonemic encephalopathy as a possible adverse effect of 5-FU therapy.

142 Duodenal intramural hematoma causing acute pancreatitis and partial obstruction due to elective diagnostic endoscopy in a child

C Murray1*

AM Ponnambalam2

1University of South Alabama, Mobile, AL

2University of South Alabama Childrens and Womens Hospital, Mobile, AL

Case report

Esophagogastroduodenoscopy (EGD) with biopsy is a relatively safe and commonly used diagnostic procedure in children. Though limited publications are available in literature, duodenal hematoma (DH) causing obstructive pancreatitis may be a significant cause of morbidity and or mortality.

Case description

8 year old female, otherwise healthy without underlying disorders, admitted with severe epigastric pain and vomiting following EGD for abdominal pain. Symptoms started on the same day after EGD. Her lipase was 11,000 (normal <199)) with a normal coagulation profile. CT of abdomen showed intramural DH with partial obstruction and diffuse pancreatitis. She was conservatively managed with NPO, NGT aspiration for gastric decompression, pain control, NGT feeds and parenteral nutrition. DH was monitored with serial US. Despite fever during hospitalization her MRI did not show evidence of necrotizing pancreatitis. She stayed for a total of 11 days.

Discussion

The true incidence of DH is not known, however, it was 1 in 1922 endoscopies in a retrospective study. There are very few case reports of DH with pancreatitis in children without underlying disorders. DH has been reported in children with coagulation disorders or after bone marrow transplantation. The cause of the hematoma is not known although postulated to be due to the shearing force of the scope or biopsies in a relatively fixed retroperitoneal vascular structure. Acute pancreatitis is rare and due to obstruction of the ampulla. The condition is managed conservatively, although rarely aspiration of the hematoma is required to relieve duodenal obstruction or biliopancreatic compression. CT or US is the preferred imaging modality. Although rare, mortality has been described due to necrotizing pancreatitis.

Conclusions

Routine endoscopy is not always a benign procedure, hence appropriate case selection is needed. In children with underlying conditions, bleeding disorders should be ruled out and corrected before biopsy. It is suggested to take biopsies in the third part of duodenum to avoid the ampulla and also not the extend the forceps beyond 2–3 cm from the endoscope.

143 Knot in the stomach: metastatic DLBCL disguised as an ulcer

S Prakash1*

K Banwait1

AS Banwait2

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

2University of Texas at Austin, Austin, TX

Case report

Early primary gastrointestinal non-Hodgkin’s lymphomas can be difficult to diagnose and can be mistaken for diseases such as Crohn’s disease or intestinal tuberculosis. We present a 73-year-old patient who had large duodenal bulb ulcer on endoscopy that resulted in metastatic DLBCL.

A 73-year-old female with history of osteoporosis, Vitamin B12 deficiency, and leukopenia presented to her primary care physician complaining of feeling knot in upper abdomen for 1 month. She felt the knot was getting progressively bigger with mild abdominal discomfort. She had also complained of early satiety, abdominal bloating, and weight loss of 3–4 pounds in past few months. She denied diarrhea or blood in the stools. Physical exam was significant for minimal distension with prominent palpable pass in right upper abdomen with tenderness to palpation and no lymphadenopathy. Labs showed WBC 4.0, Hb 11.6, Plt 417, Alkaline Phosphatase 235, Alb 3.3. CT scan of abdomen pelvis showed circumferential mass like thickening along gastric antrum, mesenteric implants, and periaortic lymphadenopathy concerning for gastric carcinoma or lymphoma. She was referred to oncologist and gastroenterologist for evaluation with upper endoscopy. EGD showed large duodenal bulb ulcer with necrotic base and small ulcer in antrum. Duodenal biopsy showed newly diagnosed stage IV Diffuse Large B-Cell Lymphoma that also involved gastric, gallbladder, and pancreas. She completed 3 cycles of R-CHOP chemotherapy and was started on high-dose methotrexate for CNS prophylaxis. She had Whipple procedure done after leak of gastrografin and noted to have perforated duodenum.

Diagnosis of primary GI lymphoma is difficult as it can be nonspecific and often benign gross appearance on endoscopy or colonoscopy. The duodenum represents roughly 6–8% of sites involving small intestine lymphoma. DLBCL of the intestine treated with surgery plus CHOP or R-CHOP chemotherapy has very good prognosis. Early diagnosis and treatment is crucial due to the aggressive nature of this malignancy.

144 Metronidazole-induced encephalopathy

Y Reddy*

V Patel

S Duncan

C Jackson

University of Tennessee Health Science Center, Memphis, TN

Case report

Metronidazole is an antibiotic commonly used to treat anaerobic infections. It is used in the management of brain abscesses due to its ability to penetrate the blood brain barrier. While it is a safe drug, it can cause serious adverse effects including peripheral neuropathy, dysarthria, cerebella ataxia and encephalopathy especially at doses exceeding 1 g/day for at least 30 days or a total dosage of 50 g. We report a case a case of metronidazole induced encephalopathy (MIE) is a patient treated for brain abscess.

Methods used/Case report

A 67-year-old male with a past medical history of hypertension, chronic obstructive pulmonary disease, hereditary hemorrhagic telangiectasias and a recent brain abscess that was drained by neurosurgery who presented to the hospital with a few weeks duration of increasing weakness and difficulties with balance. His weakness progressed into difficulty with speech and inability to sit up in bed due to extreme dizziness. He described a sensation of vertigo and light headedness. He also reported difficulty in coordinating his movements. Notably, the patient had been prescribed a two-month course of metronidazole and ceftriaxone post his neurosurgery.

On physical examination, patient’s vital signs were within normal limits. His motor strength was 5/5 in upper and lower extremities bilaterally. Sensation was intact throughout. He had severe dysmetria with finger to nose and knee to shin testing bilaterally. His gait was wide based and severely ataxic.

Summary of results/Findings

Magnetic resonance imaging (MRI) showed prominent bilateral symmetrical regions of increased intensity in the cerebellar dentate nuclei, the dorsal pons, and the medullary olives. Work up of other cause of encephalopathy were negative. His history, physical exam, and MRI findings were consistent with a diagnosis of MIE, a rare toxic encephalopathy. Metronidazole was promptly discontinued, and the patient reported improvement in his symptoms two weeks later and resolution of MRI changes.

Conclusions

The progression of MIE is related to the persistent drug effect in the blood or brain. Our case is unique as simply stopping the offending agent helped reverse the patient’s symptoms. Methylprednisone was not required.

145 Is necrotizing enterocolitis totalis different? Describing a rare disease

A Rose1*

I Zaniletti2

MT Santore3

I Adams1

A Piazza1

1Emory University, Decatur, GA

2Children’s Hospital Association, Lenexa, KS

3Emory University, Atlanta, GA

Purpose of study

The aims of this study are to describe a national cohort of infants with necrotizing enterocolitis totalis (tNEC) and to identify unique tNEC risk factors when compared to non-totalis, surgical NEC (sNEC).

Methods used

Infants undergoing NEC surgery were identified from 34 hospitals over 6 years through the Children’s Hospitals Neonatal Database (CHND). Those with isolated spontaneous intestinal perforation, intestinal anomalies, and major congenital anomalies were excluded. Those with NEC surgery beyond 5 days from admission were excluded. tNEC was defined at surgery as such a degree of bowel involvement that the disease was considered lethal. Demographic, admission, and peri-operative characteristics were compared between sNEC and tNEC infants.

Summary of results

Of 1059 infants who met inclusion criteria, 161 had tNEC. Maternal characteristics, gestational age, birth weight and delivery room interventions did not differ between groups. sNEC infants were more likely to have a patent ductus arteriosus medically treated prior to referral (26% vs 19%, p=0.048) and more likely to have grade 3 to 4 intraventricular hemorrhage (11% vs 5%, p=0.029). Intestinal perforation was more common at referral in sNEC (13% vs 6%, p=0.005) and at time of surgery (67% vs 39%, p≤0.001). 13% of the full cohort had a positive blood culture at diagnosis with similar organism profiles between the two groups. tNEC infants had more severe signs of illness including need for invasive ventilation, use of neuromuscular blockade, use of vasoactive agents and lower pH at surgery (p<0.001 for all). The number of tNEC cases varied by center. Hospital mortality was 26.5% for sNEC and 96.9% for tNEC (p≤0.001).

Conclusions

tNEC is a devastating disease with high mortality as demonstrated in this national cohort. While they have more signs of severe illness compared to sNEC, tNEC infants are less likely to have intestinal perforation. Center variation exists and may be related to differences in practice and subjectivity of disease definition.

146 When the treatment is coca-cola®

P Schroff1*

A Aery1

K Purvis2

G Esoimeme1

1UAB Montgomery Internal Medicine, Montgomery, AL

2UAB School of Medicine, Birmingham, AL

Case report

Bezoars, undigested concretions of ingested material, can cause intestinal obstruction, ileus, gastric ulceration, and even perforation. Symptoms are non-specific such as nausea, vomiting and early satiety, therefore a high index of suspicion is needed for diagnosis.

Case Description

A 28-year old lady with Type 1 diabetes mellitus, without history of gastroparesis presented with nausea and coffee ground emesis for 3 days. She had crampy lower abdominal pain, but no diarrhea or constipation. Endoscopy done a month ago was normal. All labs were normal except HbA1c of 10.1%. Acute abdominal series was unremarkable. Due to hematemesis on presentation, an endoscopy was done which revealed a large phytobezoar in the gastric cavity. Patient was advised to drink 2 L of Coca-Cola® per day for 3 days to be followed with a repeat endoscopy. This proved challenging as the patient continued to vomit and required promethazine. Endoscopy was repeated after 7 days which revealed no signs of a phytobezoar. Mucosal biopsy showed mild chronic gastritis. She was discharged with appointment for outpatient gastric emptying study and advised to take pantoprazole, avoid high fiber diet, and eat small, frequent meals.

Discussion

Phytobezoars commonly present with nausea, vomiting and early satiety, symptoms that are not an immediate indication for endoscopy. They often present a diagnostic challenge, especially given an endoscopic incidence of less than 0.5%. Barium studies and CT can help with diagnosis, but endoscopy is the gold standard. Phytobezoars consist of cellulose and lignin. Fruits high in tannins, like persimmons, can facilitate polymerization when exposed to gastric acid. Predisposing conditions include gastroparesis (which was the reason in our patient), previous gastrectomy, poor mastication, and ingestion of large amounts of fiber. Coca-Cola® is successful in dissolving 90% phytobezoars, its acidic pH and mucolytic effect of NaHCO3 are thought to be responsible. Coca-Cola® lavage or Coke Zero® is equally effective if hyperglycemia is a concern. Cellulase and papain containing meat tenderizers can be used but are less effective. Unsuccessful chemical dissolution requires endoscopic removal or surgery in rare instances.

147 Complete liver torsion presenting as abdominal distention and constipation

C Toohey*

S Palle

University of Oklahoma, Oklahoma City, OK

Case report

Liver torsion is a rare condition, and most reported cases have a predisposing factor for torsion. This case is complete liver torsion in a patient with no known factors predisposing to torsion.

A 17-year-old female with history of spinocerebellar ataxia type 8, epilepsy, chronic constipation, and gastrostomy-tube dependence presented with abdominal pain and worsening distention. The patient is nonverbal due to her neurodegenerative disease, but her mother reported that she had been crying out in pain and having abdominal cramping or pain for a few months. She previously had explosive diarrhea once weekly without any normal stools, associated with gas and abdominal distention. After multiple formula changes, Neocate Jr. improved stools to soft and occurring every 3–4 days, but the pain and distention continued. Computed Tomography abdomen/pelvis was done in an Emergency Department for abdominal pain which prompted further imaging workup and admission.

MRI on admission demonstrated full liver torsion from right to left with compression of hepatic vein outflow, gastric compression, and the liver rotated along a fixed inferior vena cava which was causing some vascular compromise and possible mesenteric ischemia. In addition, there was severe bowel distention and gallbladder sludge.

The patient was taken to the operating room by a liver transplant surgeon for liver torsion reduction, hepatopexy, and cholecystectomy. Her recovery was complicated only by increased pain and distention with no stool or gas passed until post-operative day 3. With nutritional and pain management support, she recovered well and was discharged with minimal abdominal distention not requiring narcotic pain management.

Discussion

The liver is expected to be found fixed in the right upper quadrant, maintaining its position with hepatic suspensory ligaments (coronary and triangular ligaments), connective tissue, pressure from surrounding organs, and abdominal musculature. Hypermobility of the liver could be due to congenital or acquired anomalies in the hepatic suspensory ligaments, lack of inferior vena cava tethering, or persistence of the ventral mesentery containing the hepatic veins. Hepatic torsion should be considered in a case of repeated bowel obstructions or persistent difficulties in colonic motility.

148 Metastatic primary gastric diffuse large B cell lymphoma in a patient with multiple comorbidities

H Tran*

J Gaines

M Bulathsinghala

LS Engel

L Nunez

LSU Health Sciences Center, New Orleans, LA

Case report

A 65 year old woman with chronic combined diastolic and systolic heart failure, CAD s/p CABG and multiple coronary stents, CVA with residual L sided hemiparesis, peripheral artery disease with bilateral common iliac stents, Hypertension, type 2 diabetes, hyperlipidemia, obstructive sleep apnea presented to the Emergency Department with a 2 month history of intermittent epigastric abdominal pain associated with decreased oral intake, nausea, non-bloody non-bilious vomiting, intermittent watery diarrhea, weight loss, and generalized weakness. Upon presentation, vital signs were normal and physical exam was unremarkable outside of her stable residual CVA deficits which was left sided facial asymmetry and 3/5 strength in her left extremities. Notable laboratory studies showed a troponin of 0.30 ng/mL and a creatinine of 2.0 mg/dL. Her CXR and CT Angiography of the Abdomen were non-diagnostic. Lexiscan was negative for any new ischemic changes. The patient had 2 episodes of hematemesis requiring PRBC transfusion. EGD revealed many nonbleeding cratered gastric ulcers. Biopsies were taken and pathology was consistent with diffuse large b cell lymphoma. Bone marrow aspiration and biopsy was performed with dry tap and poor sample, but no evidence of lymphoma. Throughout her admission, she continued to have recurrent episodes of hematemesis which required further PRBC transfusions. Gastric XRT was initiated while inpatient to control her hematemesis. The PET scan obtained after discharge showed lesions concerning for metastasis to the left tonsillar region, left posterior paracervical lymph node, and spleen. She is to continue XRT followed by chemotherapy.

Discussion

Our patient with multiple medical comorbidities presenting with elevated cardiac biomarkers highlights the difficulties in identifying gastric cancers. Due to their non-specific symptomatology, clinicians must identify gastric cancer alarm symptoms and consider Primary gastric lymphoma (PGL) in their differential diagnoses to expedite early diagnosis and treatment. The clinical course, prognoses, and treatment of PGLs are dependent on their histopathological subtype and staging at the time of diagnosis.

149 Noninvasive tests for staging chronic liver disease: are they all equal?

E Varney*

K Zand

B Becker

D Gordy

M Roda

CM Howard

University of Mississippi Medical Center, Jackson, MS

Purpose of study

To prospectively compare the diagnostic performance of different noninvasive tests for staging hepatic fibrosis in patients with chronic liver disease.

Methods used

This is a single-center, IRB-approved, HIPAA-compliant, prospective pilot study. Adults with chronic liver disease presenting to Interventional Radiology for random liver biopsy are enrolled prior to biopsy. Ultrasound shear-wave elastography (USE) of the liver is performed using a Philips scanner with standard acquisition parameters to measure liver stiffness (USE). MR elastography of the liver is performed using a 1.5 T Siemens scanner with standard acquisition parameters to measure liver stiffness (MRE). All patients undergo CT-guided liver biopsy and obtained CT images of the liver are used to measure liver surface nodularity (LSN score) using a previously described semi-automated method. Serum labs within 30 days of liver biopsy are used to calculate the FIB-4 score, a serum biomarker of hepatic fibrosis. The concordance of FIB-4 score, LSN score, USE, and MRE with histologic Metavir staging of hepatic fibrosis are assessed using Harrell’s C statistics. Odds Ratios (OR) from ordinal logistic models are reported.

Summary of results

The preliminary data includes fifteen adults (11 female; age range 34–72, mean 55). Mean interval between biopsy and ultrasound/MR is 0 days. The histologic range of hepatic fibrosis includes livers with no fibrosis (F0): 7, mild-moderate (F1-2): 3, and advanced fibrosis-cirrhosis (F3-4): 5. Mean LSN score is 2.7 (2.0 – 4.1). Mean FIB-4 score is 1.7 (0.5 – 4.4). Mean USE and MRE liver stiffness are 8.8 kPa (3.9 – 17.9) and 5.4 kPa (2.4 – 15.7) respectively.

The C-stat concordance for FIB-4, LSN, USE, and MRE are 0.84, 0.87, 0.77, and 0.85 respectively. Odds of moving up one fibrosis stage are 3.84 (p=0.013), 69.8 (p=0.005), 1.01 (p=0.267), and 1.53 (p=0.019), respectively, per one unit increase.

Conclusions

Liver surface nodularity score has non-inferior diagnostic performance compared to FIB-4 score, USE, or MRE in staging hepatic fibrosis in patients with chronic liver disease.

Health care research, quality improvement & patient safety

Joint plenary poster session and reception

4:30 PM

Thursday, February 13, 2020
150 Predictors of department of children and family services reporting among children presenting to the emergency department with fractures

M Collins*

L Kemp

A Byrd

P McMahon

Our Lady of the Lake Regional Medical Center, Baton Rouge, LA

Purpose of study

Approximately ¼ of the estimated 676,000 children in the US who were victims of abuse and neglect in 2016 were less than 3 years of age. Fractures are a common manifestation of child abuse. To our knowledge, no research study to date has examined the factors associated with medical staff filing Department of Child and Family Services (DCFS) reports of suspected abuse among pediatric patients seen in the Emergency Department (ED) with fractures. Therefore, we aim to study children less than 3 years of age who presented to the ED with at least one fracture in order to describe types of presenting fractures, and to identify factors associated with DCFS filing.

Methods used

A retrospective chart review was performed on all patients less than 3 years of age with a diagnosis of fracture seen in the ED from January 1, 2017-December 31, 2018. Data collected include demographics (age, sex, race, and insurance type) and information regarding the patients’ fracture(s), history, work-up, and outcome. Patients with known metabolic or other bone disease were excluded as were subsequent emergency room visits for the same fracture.

Summary of results

302 children presented with fractures; 56 (18.5%) were reported to DCFS. There were 103 skull, 87 upper extremity, 77 lower extremity and 35 trunk or chest fractures. Younger age, African American race, skull fractures, hospital admissions, and absence of a witness resulted in increased odds of DFCS reports in simple logistic regression analyses. These factors were entered simultaneously into a multiple regression model to understand which predictors had the best explanatory power. Younger age (OR=0.905, 95% CI:0.866 – 0.946, P<0.001), hospital admission (OR=2.99, 95% CI: 1.434–6.217, P=0.003) and absence of a witness (OR=0.161, 95% CI: 0.076-.338, P<0.001) were significant predictors of DCFS report filing. Race and fracture type were no longer significant predictors in the multiple regression model.

Conclusions

Using a retrospective chart review, we found that there was an increased odds of physicians filing DCFS reports when children <3 years of age presented to the ED with fractures if the child was younger, admitted to the hospital, and had no witness to the situation resulting in injury.

151 The association between insurance type and patient satisfaction scores

C Cooper*

K Little

C de Riese

Texas Tech University Health Sciences Center, Lubbock, TX

Purpose of study

Patient satisfaction is becoming an increasingly important factor affecting reimbursement of healthcare providers. For example, Medicare uses the results of their HCAHPS survey as a part of their provider compensation policy. Studies have shown that there are many factors that affect a patient’s perception of any given encounter, including wait times, previous medical knowledge and socio-economic status among others, but these studies never analyzed how each factor specifically affected survey scores, either positively or negatively. Given the limited exploration into the drivers of patient satisfaction, the present study aims to evaluate the role of third party payor status in satisfaction scores.

Methods used

Self-reported satisfaction surveys were collected from the Press Ganey System at an academic center and classified into five groups according to patient insurance type: Medicare, Medicaid, other government, commercial, and self pay. The differences in mean survey scores were then compared between groups. One-way ANOVA and Tukey HSD tests were used for statistical analysis.

Summary of results

The mean patient satisfaction scores were highest for those with Medicare, followed by those utilizing other government insurance, commercial insurance, self-pay, and Medicaid, in that order. These differences were statistically significant (p≤0.001) for all insurance groups except between commercial insurance and self-pay (p=0.12).

Conclusions

Our data shows that patient satisfaction scores are affected by third party payor type. This is in line with current literature indicating that patient satisfaction is multifaceted in nature. As patient experience has become an increasingly important driver of health care reimbursement, it is critical to consider the complexity of this subject. The results are also particularly poignant as it relates to patient populations with varying insurance coverage that different institutions serve. Further studies are warranted to explore the various factors comprising patient satisfaction, recognizing that survey scores are impacted by more than provider performance alone.

152 Creating a tool to evaluate interpretive services on a hospital system level

N Soulages Arrese1

S DeLeon1*

A White2

S Chen2

M Cooper1

1Oklahoma University Health Science Center, Oklahoma City, OK

2The University of Oklahoma Health Sciences Center, Oklahoma City, OK

Purpose of study

Barriers to using professional interpretive services (IS) in the hospital environment, such as time limitations and availability, have been described. These may lead to poor use of IS and result in limited communication with limited English proficient (LEP) families. Effective evaluation of attitudes towards IS and perceived barriers to use may allow hospital administration to improve services, resulting in improved care for LEP patients. To our knowledge, there is no standardized tool to evaluate IS on a hospital system level from the point of view of staff and providers. Our goal is to create an efficient and reliable method to evaluate knowledge, attitudes, self-reported utilization of and perceived barriers to usage of IS with minimal respondent burden.

Methods used

A 42 question survey was created and an invitation to participate was distributed by email to faculty physicians, residents, and nursing staff at The Children’s Hospital at OU (University of Oklahoma) Medical Center. This was a voluntary, de-identified, web-based survey. Exploratory factor analysis was used to explore the construct and underlying factor structure of the questionnaire. Screen plot and Cronbach’s alpha were used to determine factor retention, followed by factor loading for each item in the selected factors.

Summary of results

281 individuals completed the survey (response rate 26%). As a result of the factor analysis, seven conceptual factors were retained as meaningful based upon a Cronbach alpha >0.7. Factors were reviewed by subject matter experts and named to reflect content: Satisfaction, Utilization, Inclusivity, Dismissiveness, Defeatist, Knowledge, and Susceptibility to Barriers. Multiple items were included in each factor based upon their scoring coefficient. Items with a scoring coefficient >0.1 or <0.1 were retained. A condensed survey will be constructed.

Conclusions

From an initial 42 question survey, a revised survey will be developed. This survey will be piloted for validity in January 2020. Results from this survey will be compared to the original. This will hopefully represent the first validated tool healthcare systems can use to evaluate the effectiveness of their IS.

153 Barriers to accurate length measurement in the NICU

JL Fish*

S Yow

S Frost

A Stegall

M Famuyide

P Alur

University of Mississippi Medical Center, Jackson, MS

Purpose of study

Linear growth in extremely low birth weight infants (ELBW) is associated with the neurodevelopmental outcome. Hence, accurate length measurement is vital in the care of this population. Measurement of length via length board (LB) is the most accurate and considered as the gold standard. Aim: To determine the barriers to the use of length boards as the preferred method of measuring the length in ELBW infants in our University of Mississippi Medical Center (UMMC) NICU.

Methods used

The UMMC NICU transitioned the standard of measuring the length of infants from paper measuring tape to LB in October 2018. Length measurements remained highly variable after the transition, and the use of the LB was inconsistent. A survey was developed to assess the use of length boards and perceived barriers to its’ use. The questionnaire included: what is the current method of measuring infants they use, how difficult it is to use the length boards, how easy it is to find another staff member to help measure, what is the most significant barrier to the use of LB and the perception of accuracy of measurements by the nurses. A Likert scale of 1–5 was used, with 5 being the most difficult. At UMMC, infants are measured weekly on Sunday nights. The night staff participated in the survey on two consecutive Sunday evenings to poll the different shifts of nurses who measure infants. No identifiable information was obtained. An iPad was used to log answers and upload to RedCap for assessment.

Summary of results

67-night shift nurses were polled. 90% of staff reported using a tape measure to obtain lengths. Only 1.5% of those surveyed believed the measurements to be accurate. Only 25.9% of nurses felt the length boards were easy to use. 53% reported it was difficult to find another staff member to help use the LB. 56.1% indicated the accessibility of the boards as the biggest barrier, where 25.8% felt that it was time it takes to use.

Conclusions

The majority still used paper tape to obtain length. 98.5% considered length measurements to be inaccurate. A majority expressed some difficulty in using the LB. Time and accessibility were the most significant barriers to the use of LBs. Identifying, understanding, and reassessing staff perceptions and concerns will enable us to tailor educational plans and interventions for our unit to improve LB usage.

154 The code conundrum: improving code communication at a county hospital

PS Jagadish*

B Hansen

L Spradley

J Hogan

C Dorko

University of Tennessee Health Science Center, Memphis, TN

Purpose of study

Without appropriate communication and response to codes, patient morbidity and mortality is adversely affected. This resident-based quality improvement (QI) project analyzed problems underlying miscommunication of codes to medicine floor teams at a county hospital.

Methods used

Using the Plan-Do-Study-Act (PDSA) QI model, we assembled a team to identify causes for code miscommunication and implement relevant changes at a county hospital. The team met with QI leaders, reviewed protocol, and joined the Resuscitation Committee that examines code procedures. We surveyed residents about experiences with codes, from awareness of calls to concerns about response to them. For one month, codes to which floor teams responded were noted and compared to hospital logs. Recommendations were then presented to the Resuscitation Committee.

Summary of results

On initial review, we found that code pages to medicine teams were being sent to incorrect pagers. We corrected this issue prior to survey and field data collection due to immediate implications for patients safety.

Of the 62 survey respondents, 100% were familiar with code activation. The most prevalent concern was lack of communication of codes, at 82%.

Over one month, six codes occurred. Medicine teams responded to 50%: 2/6 were called overhead or to pagers and 1/6 involved direct nursing communication to the floor team. All codes that were not communicated to floor teams occurred in an intensive care unit and were addressed directly by critical care providers. Notably, medicine teams were informed of one code of which the hospital had no record. Data analysis identified a flaw in alert procedures after resident teams were the first physicians present at only 17% (1/6) of codes; there was a technical issue resulting in a delay of up to 5 minutes between code activation, overhead notification, and arrival of the code page to specified personnel’s pagers.

Conclusions

Despite editing pager numbers in policy, which addressed a major communication deficit, fewer than 50% of codes were correctly called both overhead and to pagers. Data presented to the Resuscitation Committee yielded system improvements over 1.5 years with continued data collection.

155 Can delivery location influnece neonatal abstinence syndrome in opioid exposed pregnancy?

M Jameson1*

D Shah1

N Noordin1

B Bailey2

1ETSU Quillen College of Medicine, Johnson City, TN

2University of Colorado School of Medicine, Aurora, CO

Purpose of study

Rates of opioid use disorder have grown substantially over the last decade and subsequently there has also been a large increase in the number of infants born withdrawing from opioids and diagnosed with Neonatal abstinence syndrome (NAS). There continues to be disparities in healthcare between individuals living in rural versus urban populations. The primary aim of this study was to determine the association between NAS diagnosis amongst opioid exposed infants born at rural versus urban delivery centers.

Methods used

We examined data from 6 different delivering hospitals in Northeast Tennessee and Southwest Virginia. A total of 18,728 charts were collected for opioid exposed and non-opioid exposed neonates born over a 5-year period from July 1, 2011-June 30, 2016. The sample was limited to newborns who had prenatal opioid exposure, resulting in 1585 newborns, 1421 who were born at urban hospitals, and 164 born at a rural hospital.

Summary of results

When comparing the two delivery locations, newborns born at the rural hospital were significantly more likely to develop NAS (44.5%) than were newborns born at the urban hospital (21.1%) (χ2=44.74, p<0.001). Chi-square and t-tests demonstrated that there were significant differences between maternal age, marital status, smoking during pregnancy, and breastfeeding initiation. A logistic regression analysis showed, after controlling for background and other prenatal exposure differences, newborns born at the rural hospital were nearly three times as likely to develop NAS.

Conclusions

Rural hospital delivery is more likely to result in NAS in opioid exposed pregnancy. The increased rate of NAS could be the result of lack of resources for non-pharmacological management, along with nursing/physician comfort, and other factors this study did not assess. Improving prenatal care and community resources, monitoring of substance use during pregnancy along with education of providers and nurses may help in decreasing NAS incidence in rural settings.

Abstract 155 Table 1
156 Improving birth certificate accuracy in alabama: a quality improvement initiative

M Moore1*

B Brugh2

R Blackmon3

A Todd1

M Wingate1

S Mazzoni1

SJ Gentle1

1University of Alabama at Birmingham, Birmingham, AL

2Alabama Department of Public Health, Montgomery, AL

3Alabama Hospital Association, Montgomery, AL

Purpose of study

Accurate vital statistics data are critical for monitoring population health and strategizing public health interventions. Previous analyses of state-wide birth data have identified several factors that may reduce birth certificate accuracy including systematic errors, insufficient interrater reliability assessments, and limited data review by clinicians. The aim of this initiative was to increase the proportion of hospitals in Alabama reporting accurate monthly birth certificate data from 67% to 80% by October 2019.

Methods used

This was a statewide collaborative effort by the Alabama Perinatal Quality Collaborative. Process measures included eleven variables monitored across ten patient charts per month per hospital. Three months of retrospective, baseline accuracy data were collected prior to project initiation from which actionable drivers and change ideas were identified at individual hospitals (figure 1). Accuracy determination, defined as ≥ 95% accuracy of the variables analyzed, was performed by health care specialists at each hospital by comparing birth certificate variables from vital statistics with data obtained from original hospital source materials. Overall monthly accuracy rates and individual variable accuracy rates were reported to hospitals at bimonthly webinars. Data were analyzed using statistical process control measures.

Summary of results

Twenty-five hospitals entered data throughout the course of the initiative, accounting for 850 chart analyses and 9,350 variable assessments. At baseline, 67% of hospitals reported birth certificate accuracy rates ≥ 95%, which increased to 90% in March 2019 and was sustained for the remainder of the initiative (figures 2 and 3). The least accurately reported variables included birth weight and antenatal corticosteroid exposure.

Conclusions

Statewide, multidisciplinary quality improvement efforts increased birth certificate accuracy. This improved accuracy is vital to accurate public health surveillance and monitoring of trends in infant outcomes.

Abstract 156 Figure 1

Driver diagram demonstrating primary and secondary drivers of increasing birth certificate accuracy with hospital specific change ideas Driver diagram demonstrating primary and secondary drivers of increasing birth certificate accuracy with hospital specific change ideas

Abstract 156 Figure 2

Panel A. Percentage of hospitals reporting ≥ 95% accuracy by month. Data analyzed isomg statisfical process control (p-chart) with special cause Variation noted during March of 2019. Panel B. Birth certificate reports by month across the 25 hospitals in the initiative Panel A. Percentage of hospitals reporting ≥ 95% accuracy by month. Data analyzed isomg statisfical process control (p-chart) with special cause Variation noted during March of 2019. Panel B. Birth certificate reports by month across the 25 hospitals in the initiative

157 Patient dismissal for vaccine delay and refusal in oklahoma pediatricians

C Nguyen*

M Cooper

C Garbe

PM Darden

M Pogemiller

OUHSC, Oklahoma City, OK

Purpose of study

Vaccine delay and refusal is increasing. One response to this problem is that physicians have decided not to provide care for (decline) or dismiss families who wish to refuse or delay vaccines. O’Leary, et al, in 215 reported that, nationally, 21% of Pediatricians dismiss families for vaccine refusal. The objectives are to assess: (1) the frequency of declining new families or dismissing current patients who request to delay or refuse vaccines, (2) which specific vaccine prompt physicians to decline/dismiss patients.

Methods used

An online REDCap survey of active (excluding retirees and students) members of the Oklahoma Chapter of the American Academy of Pediatrics assessed provider demographics, attitudes, practices including individual vaccines related to vaccine delay and dismissal. Analyses used Chi square tests.

Summary of results

After 5 emailed survey links there were 113/263 (43%) responses to the survey; 100 provide immunizations to their patients. In the last 2 weeks most (57%) have encountered vaccine refusal and most (53%) report refusal is increasing. A substantial minority state that their willingness to provide care to families who delay or refuse has decreased (41%). Forty eight percent (48/99) reported declining to accept new patients for refusing/delaying vaccines, and 36% (34/94) reported dismissing current patients for refusing/delaying vaccines. Pediatricians with more years in practice (>10 vs 10 or less) were less likely to decline (15% vs 44%, p=0.003) and dismiss (8% vs 33%, p=0.01) patients for refusing/delaying one or some, but not all vaccines. Rural pediatricians were less likely than urban to decline (12% vs 30%, P=NS) or dismiss patients (0% vs 22%, p=0.03) who refuse one or some vaccines but not all vaccines. Pediatricians were unlikely to decline or dismiss patients if they refused/delayed the influenza, HPV, or MenB vaccines.

Conclusions

Dismissing/declining patients for vaccine refusal is more common among Oklahoma pediatricians than nationally reported. Patterns differ by practice setting, years in practice, and vaccine refused. The practice of dismissing or declining patients who refuse vaccines should be further assessed and tracked over time.

158 Identifying risks and preventing injuries in the emergency department

I Omairi*

A Webb

E Jorge

MH Nichols

K Monroe

University of Alabama, Birmingham, AL

Purpose of study

Injuries are the number one cause of death in children. Studies show education with safety equipment increases likelihood of parents instituting safety suggestions. This project surveyed parents about specific injury prevention behaviors and provided education and equipment in a pediatric Emergency Department (ED) setting.

Methods used

Families were approached and surveyed regarding injury risks in the home. Additional questions included knowledge of CPR, relationship to patient, number and age of children under 18 in the home. Educational handouts were reviewed, and participants were provided with medication lock box, trigger lock, toilet lock and pool watcher tags as indicated by need from survey questions. Process measures of number of children potentially affected and number of products given were measured.

Summary of results

A total of 110 parents were approached with 100 participating. Among the participants, 87% were mothers. The participants had an average of 2.57 number of children in the home. Ages of children in the home ranged from months to 17 years old with an average of 7.5 years of age. Participants reported having medication appropriately stored in the home in a locked area in 10%; Other common areas of medication storage include dresser, counter, table, nightstand (93%); in the refrigerator (24%); in a purse/bag (7%).

Firearms were without proper storage (in the home, unlocked and loaded) in 6% of participants.

Participants reported prior CPR classes in 74%. 99% provided a follow up phone number for the post survey

The researcher spent a total of 40 hours in the ED and was able to impact 257 children’s lives. Only 10% of individuals refused to participate. Reasons behind refusal were mainly due to anxiousness and empathic concern for their children’s situation. Other refusal reasons included disinterest and/or irrelevant timing. A total of 64 trigger locks, 89 med lock boxes and 16 toilet locks and 70 drowning prevention tags were distributed. 60% of participants answered follow up phone calls with 48% saying they were utilizing the products provided. Reasons given for not using the products were lack of time, disliked the design, or felt their home environment was safe.

Conclusions

The ED setting is an excellent location for injury prevention. Families often report unsafe storage of both medications and firearms.

159 Improving dental health practices in early childhood

O May

SB Phillips*

K Burgess

University of Alabama, Northport, AL

Purpose of study

Early childhood caries (dental caries occurring in children 0–71 months of age) are the most prevalent chronic disease in childhood. Tooth decay not only causes harm to a child’s general health, it can negatively impact a child’s social and intellectual development. Numerous preventive factors have been identified, specifically adequate fluoride exposure after tooth eruption and establishment of a dental home in the second year of life.

This study utilized a provider and parent survey as well as a chart review to determine how providers discuss dental health with their pediatric patients and how PCPs document dental care within the electronic health record (EHR). This data will be used to improve discussion and documentation of dental health.

Methods used

Information regarding children’s nutritional intake and dental care is to be assessed at annual check-ups. This information includes water source in the home, beverage intake, and dental concerns. Providers are to record this information when documenting the visit. The EHR has structured data fields for recording this information.

This project asked PCPs at a university based medical center to reflect on well child visits with young children. The PCPs were asked several questions regarding their documentation and perception of dental health practices. A chart review was performed which collected data from structure data fields regarding dental health assessements during well child visits and dental preoperative visits. Parents also completed a survey about their dental health beliefs.

Summary of results

The project showed most parents in our practice do not give their child fluoridated water, do not believe their child needs fluoride, and had not taken their child to the dentist. Most PCPs report asking about dental health. However, most of them are not documenting it in a structured data field. Sixty six percent did not document date of last dental exam. Half did not document if there was fluoride in the patient‘s water.

Conclusions

It appears there is a lack of education regarding dental health in both parents and providers. PCPs are not effectively communicating the importance of childhood dental health and are not routinely documenting dental health practices. Educational initiatives for both providers and parents are needed to improve the overall dental health of our pediatric patients.

160 Screen a little, stay a lot: a retrospective analysis of mrsa screening of inpatients

A Royer*

University of Mississippi Medical Center, Pearl, MS

Purpose of study

To analyze how ‘blanket’ MRSA screening and subsequent Contact Isolation Precautions (CIP) influence measurable outcomes of inpatient hospitalizations.

Methods used

Data was obtained from the University of Mississippi Medical Center (UMMC) health system database for persons treated inpatient from 2013–2019. These patients were filtered to meet set parameters including location of care limited to main UMMC hospital and Critical Care Tower, primary service as General Medicine, isolation status limited to MRSA nares positive CIP only, and diagnosis (excluding cellulitis or MRSA bacteremia) in order to assess LOS compared to the second cohort (MRSA nares positive encounter after CIP guideline change at UMMC) of which data was gathered from July 1, 2018 – May 31, 2019. After filtering for confounding variables, cohort 1 had 496 patients and cohort 2 had 246 patients. Statistical significance was determined using the Z-test method.

Summary of results

Data obtained yielded 38286 encounters screened with MRSA nasal swab screening test between Jan 1, 2013, and May 31, 2019, of which only 6923 were placed in isolation, and 1984 were specifically placed on isolation with only MRSA nare positivity. Mean LOS (in days) for Cohort 1 was 12.6 and for Cohort 2 was 8.99 with a known variance of 115.13 and 69.17 respectively. The two groups were determined to be significantly different with P-value<0.01.

Conclusions

MRSA nares screening and subsequent CIP was shown to be associated with a significant increase in LOS. Placing patients on CIP for MRSA nares positivity may lead to unintentional harm. Further studies are needed to analyze other possible undesirable/unintentional effects related to direct patient care, cost, and effects on healthcare providers and learners to encourage healthcare providers will be more judicious with MRSA screening and subsequent CIP.

Abstract 160 Table 1
161 Barriers to retention in hepatitis C care continuum among homeless people of new orleans

R Santiago*

J Wisniewski

Tulane University, New Orleans, LA

Purpose of study

Improvement in care for persons infected with hepatitis C virus (HCV) can reduce HCV-related morbidity and mortality. Although screening for HCV is readily available, barriers exist which prevent assessment and treatment in individuals potentially infected with HCV, especially in indigent and transient populations. The National Viral Hepatitis Action Plan (2017–2020) aims to decrease viral hepatitis health disparities by partnering community-based organizations, health-care providers, and patients.

We implemented HCV testing and linkage-to-care program between local homeless shelters and health centers in New Orleans, LA. This study has two primary aims: 1) to evaluate points of success and failure in connecting HCV positive homeless patients identified through testing services at six homeless to care following a preliminary positive rapid HCV test result, and 2) to describe the main barriers cited by patients who drop out at each step in the care continuum.

Methods used

A retrospective longitudinal analysis of adult homeless individuals accessing shelter at six homeless shelters in New Orleans, LA was conducted. Every patient who came through a testing site received a survey collecting information on demographics, barriers, and recent utilization of health services. A retrospective chart review of hospital medical records was used to track patient linkage to care and their progress through the HCV care continuum.

Summary of results

A total of 1719 unique patients were identified from August 2016 through August 2019 which included 36% self-identified as African American/Black, 55% identified as White and 8% identified as mixed-race or other. A total of 24% of individuals reported no insurance coverage while 66% of patients reported having insurance of which 83% had Medicare/Medicaid.

Overall, 85 patients reported they experienced no barriers to healthcare. Self-reported barriers included finances/insurance (44%), transportation (22%), personal drug use (18%), personal alcohol use (9%), and a distrust of healthcare providers or the system (7%). Other barriers included long wait times, distance, and recent incarceration.

Conclusions

This study will instruct testing and referral practices at other homeless, free, and charitable clinics in the city while providing insight to the attrition rates of homeless individuals in the HCV care continuum.

162 FIB-4 scores in primary care patients with metabolic syndrome and abnormal liver tests

A Schreiner*

P Mauldin

J Zhang

JD Marsden

W Moran

D Rockey

Medical University of South Carolina, Charleston, SC

Purpose of study

Non-alcoholic fatty liver disease (NAFLD) is under-diagnosed in primary care. We evaluated FIB-4 scores (non-invasive fibrosis assessment tool) in primary care patients with metabolic syndrome (MetS), abnormal liver tests (LFTs), and no other liver diagnosis, then analyzed the proportion of patients with FIB-4s suggestive of significant liver disease and a diagnosis of NAFLD.

Methods used

This retrospective study of primary care data from 2007 to 2018 analyzed a sample of patients with MetS variables, including: BMI>30 kg/m2, A1c>6.5%, triglycerides>150 mg/dL, HDL<50 mg/dL for women (<40 for men), and blood pressure>130/85 mm Hg. Abnormal LFTs included elevations in bilirubin, transaminases, or alkaline phosphatase. FIB-4 scores for each LFT panel and the mean FIB-4 per patient were calculated. The mean of mean FIB-4s per patient sample, the proportion of patients with means exceeding thresholds of 1.3 (advanced fibrosis) and 2.67 (cirrhosis), and the proportion of patients with ICD-9/10 codes for NAFLD were calculated.

Summary of results

Overall, 13,171 patients met inclusion criteria. The mean FIB-4 score for the entire sample was 1.73, with 49.2% of patients exceeding 1.30, and 12.1% exceeding 2.67. Only 2.7% of patients had an ICD-9/10 code for NAFLD (table 1).

Conclusions

Many primary care patients with MetS and abnormal LFTs have FIB-4 scores suggestive of liver disease but lack formal diagnoses of NAFLD.

Abstract 162 Table 1

Patient-Mean FIB-4 scores by MetS components, and number of components Patient-Mean FIB-4 scores by MetS components, and number of components

163 Pediatric ingestions with gummy formulated medications: a retrospective study

EB Crawford1

T Coco1

NP Shah1*

L Gaines2

A Slattery2

1University of Alabama at Birmingham, Birmingham, AL

2Children’s of Alabama, Birmingham, AL

Purpose of study

Gummy formulations are widely available with 65% marketed for children. Currently, there are no studies describing children ingesting gummy formulated medications. The aim of this study was to quantify and identify the type of ingestions due to gummy formulated medications, describe demographics, associated symptoms and adverse outcomes.

Methods used

Retrospective study in children aged 0–19 exposed to gummy formulated medications from 2015–2017 as identified by calls made to the Regional Poison Control Center (RPCC). A list of potentially toxic gummy formulated medications was compiled and reviewed by a medical and clinical toxicologist. The list included multivitamins, vitamins C, D3, and B6, prenatal vitamins, calcium, co-enzyme Q 10, niacin, melatonin, immune complex, iron, caffeine, cannabidiol, GNC and Hydroxycut weight loss products. For the purpose of analysis, list was classified into vitamins, melatonin, minerals and supplements and other. Data collected included: medication name, number of tablets, age, sex, symptoms described, emergency department (ED) visit, hospitalization, and unintentional or intentional ingestion.

Summary of results

Of the 129,394 calls, 1,143 (0.9%) involved gummy formulated medications. 45 cases were excluded due to incomplete data and 1,098 included in the analysis. Median age was 3 years, 57.7% were males and 77 (7%) were symptomatic. 74% exposures involved vitamins and 24% melatonin. Presence of symptoms and ED visits were statistically significantly higher in those ingesting melatonin (69%, 59% respectively) than those ingesting vitamins (31%, 42% respectively), p<0.001. The predominant symptoms reported were drowsiness, GI upset, and hyperactivity. One patient was hospitalized who ingested multiple medications.

Conclusions

Gummy formulated medications comprised 1% of the total pediatric calls to the RPCC. Although, the occurrence of symptoms is rare, these medications especially those containing melatonin should be safely stored. As more medications are being manufactured in this formulation, it will become more paramount that appropriate childproofing is routinely utilized.

164 Pitfall in assissing the decision making capacity of a patient with schizophrenia

A Tanbir1*

L bacon1

A Bharadwaj2

R Bharadwaj1

1Texas Tech HSC-Amarillo, Amarillo, TX

2Amarillo Highschool, Amarillo, TX

Case report

Patients with psychiatric illness may retain ability to make informed decisions about medical care but this decision-making capacity is often overlooked due to ongoing psychiatric symptoms and complicated medical illness. It is a provider’s ethical responsibility to respect the right of a person to decide about their bodies but also to protect those with diminished capacity for decision making. Assessment of the capacity needs to be ongoing (since capacity can be fluctuating), context-specific and made with careful deliberation.

We reflect on a decision-making process surrounding the choice to offer a PEG tube to a 58-year-old, patient with schizophrenia, vocal cord atrophy and resultant silent aspiration and sepsis. Patient recovered well from acute illness/sepsis but remained malnourished and high risk for aspiration due to vocal cord atrophy. Patient developed active psychotic symptoms and became suspicious towards any long-term medical plans including PEG tube insertion/rehab placement. This led to an assumtion that he is unable to make medical decisions for himself and that resulted in prolonged hospitalization. Without a PEG tube, starvation and pneumonia seemed likely probabilities. Our inpatient psych facility refused patients without PEG tube. These ongoing medical issues made us reconsider and reevaluate the desicion making capacity of the patient. With careful consideration of his psychotic symptoms and repeated explanations, we were surprised to find that he had very good reasoning and was able to make an informed decision even in presence of psychotic symptoms. After informed consent, patient received PEG tube and quickly started recovering.

Published literature describes many common pitfalls encountered while assessing decision making capacity, including active psychotic symptoms. It is important to appreciate that capacity does not follow an ‘all-or-none rule’ and can change over time, and therefore careful reassessment is important. When juggling physical, mental and ethical considerations, we should start with the principle that a psychiatric illness, no matter how debilitating, does not diminish the value of that individual as a human being and his ability to make informed decisions.

165 Chronicling calories: the case of improving the missed-documentation of malnutrition

Y Vengalasetti1*

J Hom2

M Joseph3

L Shieh2

1University of Central Florida College of Medicine, Orlando, FL

2Stanford Hospital, Palo Alto, CA

3Stanford University Hospital, Stanford, CA

Purpose of study

Creating an intervention that improves nutritional assessment and captures malnutrition-based diagnoses in an inpatient setting.

Methods used

Study done at the inpatient medicine setting of a tertiary academic medical center. A survey was conducted to understand the comfort and confidence of physicians in diagnosing malnutrition. A macros was set up within a physician’s note that autopopulates from dietician’s note (group note). We combine the results of this group note with that of the physician survey. The measures the physician’s comfort in diagnosing malnutrition and utilizing the dietician in making the diagnosis. We use a test of proportion to compare and contrast the query rate before and after the intervention.

Summary of results

Our group note improves malnutrition documentation. Malnutrition was frequently the number one queried at high average of 18–20 times over the past few years. There has been a 55% drop in the number of malnutrition queries for August (8) compared to the numbers from last three previous months: July (18), June (18), May (17). More than 92% of those surveyed (60% of the new interns) reported using the new progress note template (group note). More than 86% of responders indicated average or below average comfort in distinguishing between the various malnutrition categories.

Conclusions

Physicians can partner with dieticians to make the malnutrition diagnosis. The wide-spread adoption of the group note can streamline our documentation process. Our intervention is replicable within other divisions and can become a scalable solution improving malnutrition diagnosis at other hospitals.

Abstract 165 Figure 1

Malnutrition trend Malnutrition trend

Hematology and oncology

Joint plenary poster session and reception

4:30 PM

Thursday, February 13, 2020
166 Obstructive uropathy: would you think multiple myeloma?!

J Abdelmalek*

M Elmassry

S El Nawaa

M Zitun

K Nugent

Texas Tech University Health Sciences Center, Lubbock, TX

Background

Multiple Myeloma is known to cause intrinsic kidney injury due to paraproteinemia and associated intratubular cast formation, direct tubular toxicity and deposition of light and heavy chains.This has been described in literature as multiple myeloma kidney.

Case presentation

This a 56-year-old male patient with recent history of relapsed multiple myyeloma. He presented to our hospital with acute hypercalcemia and acute on chronic kidney injury. His serum creatinine has deteriorated acutely from 3 to 6.5 in a period of one month. He was initially treated with aggressive IV fluids with improvement in hypercalcemia but no significant improvement in his kidney functions was noted.

Renal untrasound showed bilateral severe hydroureteronephrosis more on the right. CT confirmed hydronephrosis and interestingly showed diffuse bladder outlet thickening and normal prostate size.

Foley’s catheter was placed and he underwent induction hemodialysis. At follow-up after one month, CT showed reduction in bladder thickening with chemotherapy, and his kidney function returned to baseline.

Discussion

This case of multiple myeloma had a rare presentation with post renal acute kidney injury on top of his chronic multiple myeloma kidney disease. Paraproteinemia infiltration of the bladder has affected both the bladder outlet and mostly the right vesico-ureteral junction as well. Patient had initial improvement with Foley’s catheter placement but definitive return to baseline occured after he resumed chemotherapy for his relapsed disease.

Conclusion

High level of suspicion and judicious use of imaging is needed for correct dignosis and treatment of even uncommon causes of kidney disease asociated with multiple myeloma.

167 Giant hepatic tumor with a gentle predisposition

A Abonofal*

A Mansurov

H Mhadgut

S Oad

D Jaishankar

East Tennessee State University, Johnson City, TN

Case report

Solitary fibrous tumors (SFTs) are a form of mesenchymal fibroblastic tumors that usually present in the thorax and less often intracranially. SFTs in the liver are an uncommon and rarely described entity. We present a case of a massive SFT in the liver treated with partial hepatectomy. A seventy-one-year-old female patient was incidentally found to have a liver mass when she underwent a CT chest due to recurrent pneumonia. Liver function tests, LDH and tumor markers were within normal range. Further evaluation with PET-CT revealed a large (12 × 12 cm) heterogenous right hepatic lobe mass with no associated metabolic activity. Liver biopsy demonstrated a lesion composed of spindle to ovoid cells without overt features of atypia or pleomorphism. Rare mitotic figures were seen. No necrosis was noted. Immunohistochemistry (IHC) was positive for CD34 (diffuse) and STAT6 (diffuse nuclear) and negative to CK AE1/AE3, MSA, desmin, S100 and CD117 stains consistent with a diagnosis of a solitary fibrous tumor. Patient underwent complete surgical resection (major partial hepatectomy) of the tumor with negative marginal status after initial hepatobiliary surgical opinion deemed her unresectable. Gross examination of the mass revealed a 14.5 cm mass lesion. Histologic sections demonstrated a circumscribed mass of uniform spindle cells with fascicle formation. Additional IHC corroborated the diagnosis of SFT. The patient’s age at presentation, the histological findings, anatomic location and tumor size put her at a low-intermediate risk for metastatic disease according to risk stratification modules. Radiotherapy has no role while limited efficacy with targeted agents has been reported in the metastatic setting. Our case demonstrates that while SFTs arising in the liver are a rare and a difficult to diagnose entity, it is important to be aware of its tumor biology and apply the appropriate modality of treatment. Surgical excision, careful follow up and risk assessment for recurrence and metastasis are essential modalities to dealing with this type of tumor especially given that SFTs have a 10–25% chance of recurrence after resection.

168 Never let the sun set on an empyema- a rare and lethal presentation of a pleural effusion

S Ahuja*

A Meyer

University of Mississippi Medical Center, Madison, MS

Case report

A 38y/o M with no PMH presented to the emergency department with a 2-month history of severe right-sided chest pain, shortness of breath and progressive weight loss. In the ED was found to have a right-sided pleural effusion and underwent a thoracentesis with drainage of 350 ccs of an exudate, fluid studies and cytology were non-diagnostic. He soon had a reaccumulation of this pleural effusion and subsequently underwent a VATS procedure with placement of a chest tube. Pleural fluid studies were negative for infection or malignancy. He eventually underwent an open thoracotomy with decortication, pathology revealed a poorly differentiated pleural based malignancy, specimens were sent out to a larger referral center where they were found to be consistent with a diagnosis of a yolk sac tumor. Testicular ultrasound was negative. A non-contrast CT head was negative for suspicious pathology. AFP was 660.4, beta hCG undetectable at <1. MRI Brain showed obliteration of the sphenoid sinuses in association with expansion of the clival and skull base marrow signal but no focal lesions suggesting CNS spread. He was referred to the Genitourinary Oncology Division at the University of Mississippi in August. Although no obvious masses were seen on MRI, non-specific changes around the suprasellar region were concerning therefore, an LP was performed, and CSF AFP was, in fact, noted to be elevated at 6.1 ng/ml while beta HCG was 0.9 ng/ml. He was admitted to our hospital and completed one cycle of VIP chemotherapy. (Etoposide, Ifosfamide, Cisplatin). He became oxygen-dependent, repeat CT Chest showed extensive pleural thickening and near-complete consolidation of the right lung however no further surgical intervention could be pursued. Patient was eventually discharged home on supplemental oxygen with a plan for the next cycle of VIP in three weeks. Unfortunately, he expired at home a few days later.

Discussion

Germ cell tumors are classified as extragonadal if there is no evidence of a primary tumor in the testes. They typically arise in midline location. In adults, the most common sites are anterior mediastinum, retroperitoneum, and pineal and suprasellar regions. Radical resection is often carried out after four cycles of etoposide, ifosfamide, and cisplatin (VIP) chemotherapy.

169 High grade myxofibrosarcoma metastatic to the lung, report of a case with cytohistologic correlation

A Alhussain*

Y Al Hmada

V Manucha

I Akhtar

University of Mississippi Medical Center, Jackson, MS, USA Minor Outlying Islands

Case report

Myxofibrosarcoma (MFS) is a soft tissue sarcoma that typically presents superficially in the lower extremities of adults in their 6th–8th decades, with slight male predominance. Although MFS is one of the most common sarcomas, the cytomorphologic features have been rarely described. We present a case of a 47 year-old male with a history of left thigh mass who presented with multiple lung nodules on imaging. Fine-needle aspiration (FNA) of the lung nodules revealed hypercellular spindle mesenchymal cells with moderate cytologic atypia and scant myxoid stroma (figures 1A&1B). A previous biopsy from the thigh mass was compared to the concurrent lung biopsy and features appeared identical. Both biopsies showed spindle cells with eosinophilic cytoplasm, hyperchromatic pleomorphic nuclei and rare mitotic figures in a background of myxoid matrix and curvilinear vessels consistent with MFS (figures 1C&1D). The patient is receiving neoadjuvant radiation therapy to be followed by surgical excision of the thigh mass and chemotherapy. There are no specific immunostains to diagnose MFS. Cytomorphology and comparison with original slides whenever available is the best way to diagnose metastatic lesions. MFS should be differentiated from other myxoid tumors such as myxoma, nodular fasciitis, myxoid liposarcoma, and low-grade fibromyxoid sarcoma. Metastasis and mortality rate are primarily associated with histologic grade with an overall 5-year survival of 50–70%. Our case highlights the importance of recognizing the different cytomorphological patterns and correctly categorizing soft tissue tumors on FNAs.

170 TIC TAC TMA! a case of atypical hemolytic uremic syndrome

I Alwine*

University of Mississippi Medical Center, Ridgeland, MS

Case report

Our case is a thirty one year old white male with a significant medical history of two failed living donor renal transplants. The patient is currently status post a deceased donor renal transplant in 2011 and was admitted for acute kidney injury (AKI) with concern for antibody mediated graft rejection. However, significant labs noted an acute thrombocytopenia, low haptoglobin, elevated lactate dehydrogenase, and many schistocytes on peripheral blood smear. Hematology was consulted with concern for thrombotic microangiopathic hemolytic anemia. The patient was started on plasma exchange with mild improvements. However, ADAMTS 13 returned normal and thrombotic thrombocytopenic purpura (TTP) was ruled out. Yet genetic evidence of atypical hemolytic uremic syndrome returned positive and with the treatment of eculizumab the patient had resolution of thrombocytopenia and hemolysis. In addition the patients AKI resolved.

Atypical Hemolytic uremic syndrome (aHUS) is one of the disease processes that belong to thrombotic microangiopathies (TMAs). The common features for TMAs are microangiopathic hemolysis, acute thrombocytopenia, and thrombi in small vessels that lead to end organ damage. The most frequently encountered TMAs clinically are HUS associated with Shiga toxin–producing Escherichia coliinfection and TTP, followed by aHUS. Atypical Hemolytic Syndrome is either genetic or acquired and leads to complement mediated microangiopathic hemolytic anemia. Treatment of this disorder with eculizumab inhibits complement activation by specifically binding to the complement protein C5 with high affinity, thereby inhibiting its cleavage to C5a and C5b and preventing the generation of the terminal complement complex C5b-9. Atypical Hemolytic Uremic Syndrome may prove to be the very reason why this patient had multiple failed transplants in the past.

171 Stop! in the name of blood

J Ashcraft*

E Alva

University of Alabama Birmingham, Birmingham, AL

Case report

Familial erythrocytosis (FE) is a rare disorder that causes an increase in the erythrocyte component of the blood and can lead to hyperviscosity syndromes. In the congenital category there are primarily 2 groups: those caused by mutations in the erythropoietin receptor (EPOR) and those caused by a defect in the production and regulation pathway of erythropoietin. A literature review revealed that there are 28 known variants in the EPOR known to cause FE. Here we present a novel EPOR mutation that has been linked to elevated hemoglobin and FE. Our patient was found to have an elevated hemoglobin at 9 months of age on routine screening. On presentation to our institution his hemoglobin was 18.6 and hematocrit was 51.1. Interestingly, the patient was also found to have a strong family history of ‘rich blood’. Paternal uncle, paternal grandmother of child, and paternal great grandfather of child all reportedly had blood abnormalities; paternal grandfather required phlebotomy as treatment. Given family history and elevated hemoglobin, patient had genetic testing and was found to have an EPOR mutation on the hereditary erythrocytosis panel from Mayo Medical laboratories. This patient was found to have a variant, c.1307T>A, that has not previously been reported in the literature. This variant results in a missense mutation causing a premature stop codon in the regulatory domain of the protein. Though a novel mutation, it is found in close proximity to other known pathogenic variants and is thought to support a diagnosis of FE type 1. Type 1 is considered to be an autosomal dominant disorder; which would be consistent with this patient family’s inheritance pattern. Patient has been followed over time and found to have a baseline hemoglobin of 17.5. He has not experienced any reported symptoms to date, but has been found to have elevated systolic blood pressures along with intermittent hematuria. Renal imaging revealed bilateral echogenic kidneys which are thought to be due to increased renal blood flow congestion secondary to polycythemia. Given that there are no sustained clinical symptoms or abnormalities at this time, patient has not begun any forms of treatment but will continue to be followed closely. We believe this novel mutation is pathogenic and explain this patient‘s findings consistent with Type 1 Familial Erythrocytosis.

172 Endoscopic radiofrequency ablation as palliative treatment of cholangiocarcinoma

A Ataei*

G Reiss

A Garcia

Louisiana State University Health Science Center, New Orleans, LA

Case report

Purpose

Case report to increase awareness on the use of Endoscopic Radiofrequency Ablation (ERFA).

Methods

Review of Electronic Health Records and literature review.

Results

An 87-year-old man presented with jaundice. Work up included a CT, Endoscopic Ultrasound and ERCP which respectively showed intra & extrahepatic bile duct (BD) dilatation, a mass invading into the liver and a lobulated mass in the common BD. Biopsy showed an adenocarcinoma. A biliary stent was placed with resolution of his symptoms. Liquid biopsy did not identify targetable mutations. Due to his advanced age and extent of disease, he was not considered a surgical candidate. Patient and family were concerned about the toxicity of chemotherapy and/or radiation. ERFA for palliation and to prevent stent occlusion was offered and successfully performed. His disease progressed and he expired 15 months after diagnosis. He remained mostly asymptomatic with good quality of life and avoided the toxicities and risks of surgery, chemotherapy and radiation.

Cholangiocarcinomas (CCAs) are cancers that originate from the BD and are generally classified as intrahepatic, perihilar, or extrahepatic. They present with jaundice, malabsorption, abdominal pain, and/or cholangitis. Untreated patients with CCA die within 3–4 months, often from cholangitis and abscesses. Therefore, an important treatment goal is to reduce biliary obstruction and cholangitis, which is typically achieved with biliary stenting. Standard treatment for perihilar CCAs involves BD, lymph node and hepatic resection. Chemotherapy and/or chemoradiation are recommended as adjuvant treatment and as therapy for unresectable tumors. Unfortunately, the prognosis of perihilar CCA remains poor with median survival of approximately 13 months.

Conclusions

This case report serves to increase awareness of ERFA as a therapeutic option in patients with CCA. Studies have reported the safety and feasibility of ERFA with promising results for palliation of malignant obstructions, preventing stent occlusion, ablating ingrowth of blocked metal stents, prolonging stent patency and improving survival compared to stent placement alone. Information on additional cases will be presented.

173 Metastatic melanoma interferring with limb movement

B Attarha*

AE Martinez

J House

University of Florida Jacksonville, Jacksonville, FL

Case report

A 60 year old female with a past medical history of metastatic melanoma presented to the ED with the chief complaint of fatigue and decreased ability to perform ADLs. Patient was predominantly complaining of inability to move her right leg due to intense pain with ambulation. Gross physical exam revealed multiple visible ulcerating melanotic lesions in the right inguinal and right upper thigh area, the largest of which was around 4–5 cm in diameter (figure 1). Patients melanoma had been first diagnosed in March 2018 (staged pT4B) where she had went excision of nodular melanoma from the plantar aspect of her right foot along with 10 cycles of chemotherapy with Ipilimumab and Nivolumab. A venous ultrasound of both lower extremities was done which revealed subcutaneous edema with numerous enlarged bilateral inguinal and lower extremity lymph nodes. This patients stage IV melanoma presentation was severe enough that the ulcerating melanotic lesions were interfering in movement of the right lower extremity. Oncology consult was ordered and given the severity of patient metastasis, patient was offered treatment of carboplatin/paclitaxel or palliative care with hospice. While metastatic melanoma is not an uncommon occurrence, the size and severity of this patients ulcerative lesions are unusual in that it interfered with normal limb movement, contributing to further functional decline in this patient.

Abstract 173 Figure 1

Cutanenous melanoma along right inner thigh Cutanenous melanoma along right inner thigh

174 Think twice; don’t go with the flow

G Bader*

C Milner

University of Mississippi Medical Center, Ridgeland, MS

Introduction

Common causes of thrombocytopenia in solid tumors are myelosuppression due to chemotherapy, destruction due to disseminated intravascular coagulation and bone marrow infiltration. However, some cytotoxic drugs can cause immune-mediated thrombocytopenia. Differentiating the 2 mechanisms is crucial since it impacts management.

Case report

A 32 y old F presented with vaginal bleeding. Pelvic exam revealed prolapsing cervical mass. Biopsy was consistent with invasive squamous cell carcinoma. CT Scan showed 7.4 × 7.1 × 5.1 cm cervical mass with no metastases. She completed chemoradiation with Cisplatin and had complete response. Eight months later, she relapsed with lung metastases and was started on Paclitaxel/Cisplatin/Avastin. She had expected drop in hemoglobin (Hb) and platelet (PLT) count 2 weeks after 1st cycle. However, with the 2nd, PLT count dropped from 214000/microliter on day 1 to 2000/microliter on day 4 with no associated drop in Hb. She didn’t develop schistocytosis or coagulopathy. HIT was ruled out. Immature PLT fraction was elevated at 17%. Prednisone 1 mg/Kg/day was started and PLT count rapidly improved and normalized within 2 weeks. She received Dexamethasone with subsequent cycles with relative stabilization of PLT count.

Discussion

Although thrombocytopenia and worsening anemia after 1st cycle were probably due to myelosuppression, drop in PLT count after 2nd cycle was more consistent with immune thrombocytopenia based on rapid decline in PLT count, elevated immature PLT fraction, response to steroids and absence of other causes of PLT consumption. Immune-mediated PLT destruction has been reported in solid tumors. However, in this case, we think it was drug-induced since it was transient and the timing was related to the chemotherapy administration. Well documented culprits of drug-induced immune thrombocytopenia are Oxaliplatin and Irinotecan. Very limited cases of Cisplatin ± Paclitaxel induced immune thrombocytopenia have been reported.

Conclusion

Autoimmune thrombocytopenia should be considered in patients with solid tumors, especially when early or prolonged thrombocytopenia follows chemotherapy. Differentiating immune-mediated thrombocytopenia from myelosuppression is critical because it affects management.

175 Autoimmune complications post autologous hematopoietic stem cell transplant: case study of a pediatric patient with stage III neuroblastoma

A Bauchat*

G Marrero-Rivera

Z LeBlanc

LSUHSC Children’s Hospital of New Orleans, New Orleans, LA

Case report

Autoimmune disorders have been described following autologous and allogeneic hematopoietic stem cell transplantation (HSCT). After HSCT, the innate immune system recovers rapidly whereas the adaptive immune system may take years to fully reconstitute. During this reconstitution of the immune system, patients are at risk for developing graft versus host disease, experiencing graft versus tumor effects, or more recently documented developing autoimmune disorders. Autoimmune disorders have become more reported in up to 5 percent of patients post-transplant.

This report describes an 18-year-old female who developed multiple autoimmune findings following autologous hematopoietic stem cell transplantation for Stage III Neuroblastoma. Her initial transplant admission was complicated with severe mucositis, Adenovirus colitis, and severe veno-occlusive disease (VOD) requiring Defibrotide. She was readmitted on transplant day +70 for hematemesis and melena. She developed profuse diarrhea requiring Octreotide, TPN with additional fluids to compensate for insensible losses, and additional supportive care measures. During this time, she developed pancytopenia, indirect hyperbilirubinemia, pleural effusions and atypical pneumonia. Testing ruled out VOD, neuroblastoma relapse, and TMA. Between days +70 to +90, she developed the following autoimmune manifestations: autoimmune hemolytic anemia, prolonged aPTT related to an acquired factor inhibitor, positive ANA, thyroglobulin antibodies, autoimmune thrombocytopenia, and autoimmune colitis. IVIG provided brief improvement in her thrombocytopenia, but this was not sustained. She was subsequently started on Mycophenolate Mofetil and Methylprednisolone with improvement in her constellation of symptoms allowing for the weaning of supportive care measures. She is now six months post-transplant with complete resolution of her symptoms and normalization of all antibodies. She has completed the remainder of her treatment plan, is currently off all immunosuppression for four months, and is cancer free. It is important for clinicians caring for patients post autologous transplant to consider autoimmunity as a post-transplant complication.

176 Adrenocortical carcinoma: an uncommon pediatric diagnosis with a common chief complaint

A Zulfer1

C Benjamin1*

G Kirkpatrick2

1University of Oklahoma School of Community Medicine, Tulsa, OK

2St. Jude and St. Francis Children’s Hospital, Tulsa, OK

Case report

Adrenocortical carcinoma is a rare cause of childhood cancer. In the pediatric population, a majority of these tumors are functioning, resulting in symptoms directly related to the overproduction of sex hormones, cortisol, and/or aldosterone. Intra-abdominal tumors frequently present as abdominal distension and constipation, which may present a diagnostic challenge for pediatricians.

Case presentation

A 2 year old male with history of constipation presented with abdominal distension, constipation, and emesis. On physical exam, he was found to have a palpable left-sided abdominal mass. A genitourinary exam was significant for phallus enlargement with stage II pubic hair. Labs demonstrated microcytic anemia and elevated lactic acid dehydrogenase. An ultrasound revealed bilateral renal masses, while CT of the abdomen revealed bilateral retroperitoneal masses. Testosterone level was elevated, without elevated corticosterone or aldosterone, suggesting possible adrenocortical carcinoma. PET scan demonstrated no distant metastasis or lymph node involvement. The patient underwent tumor biopsy with pathology report confirming the diagnosis of adrenocortical carcinoma. The patient was started on a Children’s Oncology Group study for the treatment of adrenocortical tumors, utilizing surgery with lymph node dissection and multi-agent chemotherapy.

Discussion

Identifying the signs and symptoms consistent with hormone over-production can be crucial in diagnosing adrenocortical carcinoma. In this case, the evidence of abdominal mass and virilization on examination allowed for appropriate diagnostic testing. Labs demonstrated an elevated testosterone level, consistent with virilization on exam, which allowed the diagnosis to be made. It is essential for medical providers to obtain a careful history and perform a detailed physical exam, including a genitourinary exam, even if the presenting complaint is as common as constipation.

177 Headache heralds diagnosis of chronic lymphocytic leukemia

S Bhama*

University of Mississippi Medical Center, Jackson, MS

Case report

Chronic lymphocytic leukemia (CLL) is the most common leukemia in older adults in the western world. Central nervous system(CNS) involvement at initial presentation is exceedingly rare. Risk factors for CNS involvement are unknown, and portend a poor prognosis.

A 54 year old male was diagnosed with disseminated zoster in May 2018. Physical exam revealed submental, axillary and inguinal adenopathy without any hepatosplenomegaly. His white blood cell count was noted to be 18,400 with 78% lymphocytes. Imaging showed diffuse lymphadenopathy and a 5.8 cm left lower lobe mass-like consolidation. He was referred to hematology outpatient, but was lost to follow-up.

He re-presented with the worst headache of his life lasting 2 weeks in November 2018. White blood cell count was 120,000 with 75% lymphocytes, normal hemoglobin and platelets. CT head was normal. Flow cytometry of peripheral blood showed a CD5+ CD23+ CD38+, cyclin D1 negative and ZAP70 negative B cell lymphoma consistent with CLL. Flow cytometry of the CSF was positive for CLL. Bone marrow cytogenetic analysis showed t(14;19)(q32;q13.3) and a trisomy 12. He did not have a 17P deletion.

He was treated with 1 cycle of FCR (fludarabine, cyclophosphamide, rituximab) and then transitioned to ibrutinib/rituximab for 6 cycles upon discharge and is now on ibrutinib monotherapy. He has had complete resolution of CNS symptoms, lymphadenopathy and lymphocytosis.

Symptomatic central nervous system invasion in undiagnosed CLL is rare and associated with decreased survival. t(14;19)(q32;q13.3) is a translocation which involves the immunoglobulin heavy chain locus on chromosome 14 and the BCL3 gene on chromosome 19. This rare cytogenetic abnormality is identified in B-cell neoplasms, most often classified as CLL. It is unknown whether there is an association between t(14;19)(q32;q13.3), trisomy 12 and CNS involvement. Neurological symptoms are variable and etiology must be distinguished from unrelated conditions. No standardized treatment protocols for CNS involvement are reported in the literature, but patient can have durable responses if treatment is initiated early. Further studies are warranted to determine whether there is an association between the t(14;19)(q32;q13.3) and CNS disease because it may alert clinicians to initiate prompt treatment.

178 A case of Disseminated Intravascular Coagulation (DIC) following ARDS

BL Boudreaux*

H Oddo Moise

LS Engel

R McCarron

LSU Health Sciences Center, New Orleans, LA

Case report

A forty-one year old woman with a history of lupus presented with complaints of fever to 101.5 degrees, productive cough, headache, joint pain, and shortness of breath for two days and was diagnosed with community acquired pneumonia. She was initially stable, but then one morning she became progressively more tachypneic and had an oxygen requirement of 15L. Repeat chest x-ray and blood gas indicated she had developed ARDS (acute respiratory distress syndrome) and she was moved the ICU and intubated. Bronchoscopy was consistent with infection, though cultures never grew an organism. She developed a lupus flare as well as an AKI requiring renal replacement therapy during her time in the ICU. She was extubated on day 7 of her hospital course. By day 8 she began to have oozing from her trialysis line. She initially responded to DDAVP for uremic platelet dysfunction, but the next day it began again. She did not respond to DDAVP again, so a panel of DIC (disseminated intravascular coagulation) labs were obtained. Her labs were consistent with DIC. She responded to 2 doses of cryoprecipitate, bleeding ceased and her repeat labs normalized. She was discharged from the hospital on day 14.

Discussion

DIC is the widespread coagulation leading to vascular clot deposition with impaired synthesis of coagulation proteins and protease inhibitors, as well as exhaustion of platelets and coagulation factors. DIC is characterized by system thrombin generation and widespread clotting and bleeding that is usually associated with sepsis, trauma, malignancy, and childbirth. Therapy involves replacing factors and treating the underlying cause. In some studies, ARDS was related to the development of DIC. Treatment of the DIC lead to the resolution of ARDS and extubation of the patients. In this case, the combination of sepsis, ARDS, development of renal failure, and an active lupus flare were all factors that lead to the development of DIC.

179 A case of spontaneous tumor lysis syndrome in solid tumor metastases

LA Bryant*

Eisenhower Army Medical Center, Grovetown, OH

Case report

Tumor lysis syndrome is a potentially fatal oncologic emergency in which tumor cells lyse releasing large amounts of potassium, phosphate and nucleic acids into circulation. Spontaneous tumor lysis syndrome is a rare condition but has been described in the literature typically with non-Hodgkin’s lymphoma and acute leukemia. It is also rare to see tumor lysis syndrome from non-hematologic solid tumors; however this has been described in patients who have undergone treatment. We present an unusual case of a patient who had both spontaneous tumor lysis syndrome as well as tumor lysis syndrome of a solid tumor.

An 88 year old male with a past medical history of metastatic castrate-resistant prostate cancer with innumerable metastases to axial and appendicular skeleton, who was last treated 3 months prior with chemotherapy. He presented to the emergency department after oncology called him in due to lab abnormalities. Labs in the emergency department showed potassium of 7.0 mmol/L, corrected calcium of 6.6 mg/dL (ionized calcium of 0.76), phosphate of 4.7 mg/dL, serum creatinine of 3.6 mg/dL increased from baseline of 1.6 mg/dL, uric acid level of 16.6 mg/dL and alkaline phosphatase of 657 I/U. The patient was treated over the next week with Rasburicase, intravenous crystalloid for renal failure and correction of electrolyte abnormalities.

Discussion

We present a rare case of a patient who met Cairo-Bishop criteria for tumor lysis syndrome that was spontaneous and of a solid tumor. There are 28 cases of spontaneous tumor lysis syndrome from the first reported case in 1977 to 2017 per literature review. There is scant reporting for spontaneous tumor lysis syndrome in prostate cancer metastases; however, there are two documented cases of treatment naïve spontaneous tumor lysis syndrome of prostate cancer metastases. This both highlights the rarity of this case and shows that clinicians should entertain the diagnosis of tumor lysis syndrome when encountering these types of lab abnormalities despite this condition being most commonly seen in hematologic malignancies. This case also highlights the likelihood that this condition is underdiagnosed in patients with large tumor burden. This is evident by our patient who only came to the hospital because of lab abnormalities found during a routine oncology visit.

180 Human immunodeficiency virus aiding in development of thrombotic thrombocytopenic purpura

B Case*

S Elkins

University of Mississippi Medical Center, Jackson, MS

Case report

A 39-year old female with no significant medical history presented to our ER after she was found having seizure-like activity. She had no history of seizures in the past. She was taken to an outside hospital, where CT Brain was negative for acute findings. Upon arrival to our hospital, she was obtunded. Per the family, patient had no recent complaints of bleeding or excessive bruising. She was in her normal state of health one day prior to the development of these symptoms. Notable labs included white count of 14,000, hemoglobin of 7.2, and platelet count of 10,000. CMP was notable for creatinine of 5.6, total protein of 10.3, and albumin of 3.0. Bilirubin and electrolytes were normal. LDH was found to be elevated at 1750, uric acid was 13.3, and haptoglobin was <10. Peripheral blood smear confirmed schistocytes. There was concern for TTP, so ADAMTS13 was obtained, and she received emergent plasma exhange with FFP. ADAMTS13 activity later came back at <5%. Given her elevated globulin gap, she was tested for HIV and hepatitis. HIV testing came back positive with a CD4 count of 32 and a viral load of 864,000. Patient‘s mental status improved significantly after 2 sessions of plasma exchange. Creatinine improved to 1.5 prior to discharge, and platelets normalized. Haptoglobin and LDH improved as well. ADAMTS13 activity improved to >100%. She continues to follow in clinic one year later and is doing very well on no treatment for TTP.

Although TTP is a rare diagnosis, one must have a high clinical suspicion in a patient who manifests several of the five characteristics--mental status changes, acute renal insufficiency, microangiopathic hemolytic anemia, thrombocytopenia, and fever. It is important to remember that a patient might not have all five criteria. There are several case reports of TTP in the setting of newly diagnosed or untreated HIV, usually with CD4 <200. HIV increases the risk of TTP 14–50 fold when compared to non-HIV infected individuals. ADAMTS13 levels in these patients can be highly variable.

181 Blastic variant of mantle cell lymphoma: a difficult diagnosis

D Ramos1

HR Cintrón-Colón2*

C Rivera-Franceschini2

W Cáceres-Perkins1

1VA, San Juan, PR

2San Juan City Hospital, Guaynabo

Case report

Mantle Cell Lymphoma is a mature B-cell neoplasm usually composed of monomorphic small to medium-sized lymphoid cells with irregular nuclear contours. It accounts for 3–10% of non-Hodgkin lymphomas and has been traditionally considered a very aggressive and incurable lymphoma. Most patients initially present with stage III or stage IV disease with lymphadenopathy, hepatosplenomegaly and bone marrow involvement. Patients presenting with the blastic variant are associated with a poorer prognosis.

A 73-year-old male with no comorbid conditions presented to the emergency department with 1-week-history of cough, shortness of breath and general fatigue. Few days prior he was treated with antibiotics and expectorant elixir without improvement of symptoms. He was found with tachycardia and hypertension. Physical exam was remarkable for a systolic ejection murmur and mild jaundice. No hepatosplenomegaly or lymphadenopathy was present. Laboratory demonstrated leukocytosis, thrombocytopenia and microcytic anemia requiring emergent blood transfusion. Bone marrow biopsy demonstrated blastoid cells with prominent nucleoli, many smudge cells without schistocytes or Auer rods, suggestive for a leukemic phase. Immunohistochemistry was positive for cyclin D1 within neoplastic cells. Peripheral blood fluorescent in situ hybridization revealed translocation t(11;14) consistent with mantle cell lymphoma, blastoid variant. Mantle Cell International Prognosis Index score was 9, indicating poor prognosis. He was started on Hyper-CVAD chemotherapy and rituximab, in addition to filgrastim and prophylactic antibiotherapy for neutropenia. As of today, the patient has completed 4 cycles of Hyper-CVAD chemotherapy without complications, showing sustained remission despite refusing bone marrow transplantation.

Blastic variant of mantle cell lymphoma may have a leukemic phase presentation challenging the initial diagnosis and prompt management. Additional entities such as acute lymphoblastic leukemia and diffuse large B-cell lymphoma with leukemic phase can often be mimicked. A high clinical suspicion and awareness of the prognosis of mantle cell lymphoma is required for early diagnosis in patients presenting peripheral smear suggestive of leukemia.

182 A question stem in real life: a case of dengue fever presenting with breakbone fever and thrombocytopenia

C Cutrer*

C Bigelow

University of Mississippi Medical Center, Jackson, MS

Case report

Dengue fever (or dengue hemorrhagic fever) is a febrile illness that is endemic to certain areas in Central and South America, Africa and Southeast Asia. It is transmitted by Aedes mosquito bite and causes a very severe febrile illness that can ultimately lead to hemorrhagic shock. We present a case of Dengue fever in a patient with recent mosquito exposure in Cuba.

Patient is a 49 year old Latino male who presented to Emergency Department (ED) with 1 week history of back pain and headaches, fever, abdominal pain and nausea. He reported that symptoms began shortly after returning home from week-long visit to Cuba where he was bitten by multiple mosquitos. He also noted that his back pain and headaches were only present while he was febrile. At time of presentation, patient’s exam was notable for conjunctival injection of right eye, abdominal tenderness to palpation, maculopapular rash in bilateral lower extremities and petechiae across chest and upper extremities. Labs remarkable for leukopenia (2300), thrombocytopenia (34,000) and erythrocytosis (17/52). Also noted to have mild transaminitis with ALT and AST of 100 and 52, respectively. Patient was admitted to General Medicine service and Hematology was consulted for leukopenia and thrombocytopenia. Infectious disease also consulted given recent mosquito exposure. Ultimately, patient had bone marrow biopsy which showed hypocellularity and viral serologies which confirmed presence of Dengue virus. He was treated with intravenous fluids, antipyretics and other supportive care and symptoms resolved within a few days and he was discharged home.

Dengue fever is a rare but potentially life-threatening febrile illness that can present with several different hematologic phenomenon. The most common hematologic presentation is thrombocytopenia, leukopenia and erythrocytosis initially. This exact mechanism is unclear but it is proposed that the leukopenia and thrombocytopenia may be immune-mediated processes. Erythrocytosis is not always present but can be indicative of vascular permeability and may predispose patient to hemorrhagic complications.

183 Extranodal marginal zone B-cell lymphoma of the conjunctivae and orbits

R D’Cunha*

N D’Cunha

Texas Tech Health Sciences Center, Lubbock, TX

Introduction

Orbital lymphoma originates in the conjunctiva, lacrimal gland, soft tissues of the eyelid, or extraocular muscles and makes up about 1–2% of non-Hodgkin’s lymphoma. Extranodal marginal zone lymphoma of the conjunctivae and orbits presents unique challenges in diagnosis and treatment. We present a rare case of extranodal marginal zone lymphoma of the conjunctivae and orbits that was effectively treated.

Case presentation

A 63-year-old Hispanic female presented to her PCP with complaints of ‘knots’ in both her eyes. The patient denied any impairment of vision. Upon examination, she was found to have mechanical left superior eyelid ptosis and salmon pink, fleshy, vascular lesion visible in the right conjunctiva. A MRI of the orbit/face/neck showed bilateral enhancing masses within the preseptal fat, with right-sided postseptal and intracoronal extension. The right-sided mass measured approximately 0.9 × 2.6 × 1.2 cm, and the left-sided mass measured approximately 3.3 × 2.4 × 1.2 cm. The extraocular muscles bilaterally appeared to be extrinsic from these masses and optic nerves appeared to be spared bilaterally. A biopsy of the left orbital tumor showed stage II extranodal marginal zone lymphoma of the conjunctivae and orbits. Immunochemistry from the biopsy was positive for CD20, bcl-2, Ki-67(~10%) and negative for CD3, CD5, CD10, cyclin D. A PET CT showed the disease in the conjunctivae and posterior orbits bilaterally.

The patient was treated with definitive involved site radiotherapy followed by chemotherapy with Rituxamab, Cyclophosphamide, Vincristine and prednisone. The bilateral conjunctival lesions were treated utilizing an opposed laterals beam arrangement technique. The patient tolerated the treatment well but with expected grade 1 skin and conjunctival irritation. Three weeks after radiation the patient will be started on 6 cycles of chemotherapy.

Discussion

Radiation therapy is the treatment of choice as these tumors. Other treatment options include anti CD20 monoclonal antibodies and chemotherapy. A common etiological agent for these patients is chlamydia psittacosis and a few patients may respond to doxycycline. Chemotherapy is often reserved for relapse. However, because of the bilateral involvement of the orbits, in this case, it was decided to add chemotherapy to the treatment regime.

184 DIC in colon cancer: a presentation of metastasis to the bone

S Desikan*

R Desikan

White River Medical Center, Batesville, AR

Introduction

Disseminated Intravascular Coagulation (DIC) is the result of inappropriate activation of the coagulation cascade in conjunction with unregulated fibrinolysis. While DIC is common in hematologic malignancies; it is uncommon in solid tumors. Here we present a case in which DIC was the initial presentation of bone metastasis.

Case study

A 75yoF with history of colon cancer s/p hemicolectomy and one round of FOLFOX presented with hematuria. Her oncologist was trending her CEA with the most recent value was 148 (prior value 107). CT abdomen and pelvis was negative for metastasis. After 2 weeks of persistent hematuria and antibiotic treatment, she was admitted to the hospitalist service. Physical exam was significant for bilateral upper extremity bruising, lower extremity petechiae, and exquisite CVA tenderness. On laboratory evaluation, WBC was 11.5, PLT of 98, PT 23.6, INR 2.1, and PTT 45.1. She was started on Zosyn for a presumed UTI and Oncology was consulted. D-dimer and Fibrinogen were ordered and were >20 and 60 respectively. She was diagnosed with DIC. She received 2U FFP, 1U PRBC, and 1U Cryoprecepitate. As her CEA was increasing and imaging was negative, a bone marrow biopsy was performed. Results showed colon cancer metastasis involving 30% of the marrow space. She received one dose of FOLFOX as salvage therapy, which she tolerated well. At this time, prognosis was discussed and it was decided that hospice was the best course of action.

Discussion

While common in hematologic malignancies, DIC is rare in solid tumors. Current theories revolve around tF expression on vessels, metastasizing cells and microparticles. When present, DIC significantly worsens prognosis. Our patient presented for hematuria and had been treated with antibiotics for suspected UTI. Upon presentation, she was diagnosed with DIC and responded to treatment prior to choosing hospice.

Only a handful of cases have reported on DIC in colon cancer. A trend that was noted was the association of DIC with bone metastases in patients with mucinous histology. Our case exemplifies the importance of considering DIC in colon cancer as a potential harbinger of bone metastasis.

185 A rare case of langerhans cell histiocytosis presenting as a colon polyp

T Do*

S Kolagatla

B Sergent

N Moka

Appalachian Regional Health, Whitesburg, KY

Case report

Langerhans Cell Histiocytosis (LCH) is a rare benign tumor arising from Langerhans dendritic cell. The incidence of LCH in children is estimated as 5 to 15 cases per 1 million. In adults, LCH is very rare, and the incidence is not yet defined. LCH can present as unifocal, multifocal, or multisystem involving disease. The most common organs involved are bone and skin, contributing to over 85% of the cases. LCH of GI tract involvement is rare and is limited to a few case reports in the literature. Here we report such a case of LCH presenting as a colon polyp.

A 42-year-old Caucasian male presented to the GI clinic for additional workup for a long-standing history of watery diarrhea that was not associated with a particular food type; weight loss, bone pain, or skin rash. His past medical history is significant for stroke, hypertension, and anxiety. Social History is significant for cigarette smoking and alcohol consumption. No significant findings on the exam. He underwent colonoscopy for evaluation of chronic diarrhea and was found to have an isolated polyp in the ascending colon. Pathology of the polyp showed positive for S100, CD1a favoring the diagnosis of LCH. PET scan, CT of the head with contrast, and bone marrow biopsy demonstrated no evidence of involvement by LCH. Currently, the patient is under clinical surveillance without recurrence for the past six months.

LCH is a rare dendritic cell tumor. The most common organs of involvement are bone and skin. LCH of the GI tract is extremely rare and can present as a polyp, ulcer, or nodular lesions. Natural course and recurrence rates of LCH with isolated GI tract involvement are not fully evaluated. No standard consensus exists for treatment of LCH of GI tract. However, follow-up colonoscopy and imaging studies are often performed. Data is limited, but for patients with unifocal or multifocal single organ involvement (e.g., skin, lymph nodes, or bones), treatment options include Vinblastine and prednisone. In one French children’s cohort, fifty percent of patients harbored BRAF V600E mutation, and that was associated with resistance to first-line therapy.In adults, BRAF V600E frequency and impact or treatment and prognosis is yet to be defined.

186 Eribulin induced encephalopathy in a patient with advanced breast cancer

A Shredi1

E Elgwairi1*

A Abdalla2

S El Nawaa1

GD Bedanie1

M Elmassry1

M Zitun1

K Nugent1

1TTUHSC, Lubbock, TX

2Benghazi University, Benghazi, Libya

Introduction

Eribulin is an antineoplastic agent indicated in advanced breast cancer. It inhibits the growth phase of microtubules. We report a patient with advanced breast cancer who developed drug-induced central nervous system (CNS) toxicity following treatment with Eribulin and responded promptly to corticosteroid administration.

Case presentation

A 49-year-old woman with a history of advanced breast cancer (HER-2 negative, ER and PR positive) presented with a decreased level of consciousness and confusion. She underwent extensive treatment in the past, including partial lumpectomy, multiple chemotherapeutic regimens, hormonal therapy, and radiation therapy. Eribulin was initiated due to disease progression. She received the first cycle two weeks prior to her presentation. Physical examination was remarkable only for disorientation. Vital signs: were within normal limits. Laboratory tests, including electrolytes, were within normal limits. CT of head without contrast did not reveal any abnormalities. MRI of head showed bilateral symmetrical basal ganglia hyperintensity. Eribulin was stopped, and she was treated with dexamethasone 4 mg TID. She regained orientation in 12 hours and was discharged on a tapering dexamethasone regimen over 1 week.

Discussion

Eribulin common side effects include neutropenia and peripheral neuropathy. Transient CNS toxicity with diplopia was reported in few cases. However, encephalopathy has not been previously reported. Eribulin does not cross BBB, however radiotherapy may have facilitated its entrance to the brain causing toxicity. Another theory is Eribulin may cause autoimmune induced CNS injury. It is unclear why the basal ganglia were specifically affected. More studies are needed to help determine the underlying pathogenesis of CNS toxicity.

187 Managing dual antiplatelet therapy and disseminated intravascular coagulopathy: a case report

CH Harris

K McQueen

LS Engel*

A Garcia

LSU Health Sciences Center, New Orleans, LA

Case report

A 58-year-old woman presented with chest pain one week after placement of drug-eluting stent (DES) for ST-elevation acute coronary syndrome (STE-ACS). She was compliant with dual antiplatelet therapy (DAPT) regimen of aspirin and prasugrel. Evaluation included an EKG without ST-segment changes. CBC revealed an elevated WBC (35,200 cells/mL) with 87% immature mononuclear cells, leading to a diagnosis of acute myeloid leukemia (AML). Disseminated intravascular coagulopathy (DIC) was diagnosed based on increasing D-dimer and decreasing fibrinogen. DAPT was transitioned to aspirin monotherapy. The patient completed 7+3 induction chemotherapy with complete remission. On day 34 of hospitalization, the patient developed multiple deep vein thromboses and aspirin monotherapy was transitioned to clopidogrel plus apixaban. The patient was discharged on hospital day 41. She is currently receiving consolidation chemotherapy.

Discussion

DAPT is the standard of care to prevent stent thrombosis after coronary stenting. Managing antiplatelet therapy in the setting of elevated bleeding risk presents a challenging clinical scenario. AML consists of a group of hematopoietic malignancies involving bone marrow precursor cells. DIC is associated with AML and may present at time of diagnosis or with induction therapy. DIC is characterized by activation of coagulation cascades resulting in the consumption of platelets and coagulation factors, which increases bleeding risk.

Concurrent presentation of STE-ACS and AML is exceedingly rare with only nine cases previously reported. Aspirin monotherapy was initiated for this patient after considering the risk of stent thrombosis against the risk of hemorrhage. Although expert opinion recommends DAPT for one month for isolated thrombocytopenia, the risk of profound coagulopathy associated with DIC prohibited DAPT for our patient. Historical data of aspirin monotherapy shows a 3.4% risk of early stent thrombosis, significantly higher than the risk associated with DAPT. Although no longer considered standard of care, aspirin monotherapy may be a reasonable approach to prevent thrombosis of coronary artery stent in the presence of DIC.

188 30-year relapse of renal cell carcinoma presenting as recurrent and refractory epistaxis

Z Fatima*

H Rehman

A Mansurov

K Chakraborty

D Jaishankar

East Tennessee State University, Johnson City, TN

Case report

Renal Cell Carcinoma (RCC) tends to recur at unusual locations often with hemorrhagic metastasis. Sino-nasal metastasis is rare and can present as uncontrolled epistaxis or nasal obstruction. We report a case of massive uncontrolled epistaxis secondary to an isolated metastatic RCC in the nasal cavity requiring maxillary artery ligation. An 86-year-old female with a history of early stage renal cell carcinoma, status post nephrectomy 30 years ago, presented with recurrent, heavy, right nasal bleeding. She experienced intermittent mild epistaxis over the past 2 years, treated with conservative measures, except one episode requiring arterial embolization. Repeat ENT evaluation, nasal packing, rhinorocket tamponade and eventually maxillary artery ligatures lead to some degree of control. Biopsy from right inferior turbinate confirmed metastatic renal cell carcinoma. Imaging studies revealed no evidence of other systemic involvement other than sino-nasal metastasis. She completed palliative radiation therapy to prevent further episodes of bleeding. Given her age and comorbidities, she expressed interest in best supportive care to maintain her quality of life and was transitioned to palliative care. Epistaxis is one of the most common complaints confronted by the Emergency department. Massive epistaxis should always prompt clinicians to exclude more perilous pathologies, including malignancy. The primary tumor was resected in our patient 30 years ago, highlighting the fact that RCC can recur after many years and can have a varied clinical presentation. RCC can metastasize to nasal and paranasal sinuses either through the caval tract or through the vertebral plexus. Tumor related-secondary epistaxis typically does not respond to conservative management and usually requires resection or palliative radiation therapy. We present this case to illustrate the unusual late presentation of isolated metastatic RCC and stress a low threshold to investigate bleeding in cancer survivors. Early diagnosis can lead to effective treatment while decreasing morbidity and complications.

189 Uncovering a deadly disease: a rare case of synovial sarcoma

V Fonseca-Ferrer1*

WD Marrero1

DN Vera Santiago2

A Nieves-Ortiz1

HR Cintrón-Colón1

1San Juan City Hospital, Toa Alta

2Damas Hospital, Ponce, Puerto Rico

Case report

Synovial sarcomas are aggressive soft tissue malignant tumors that arise near the joints, usually the ankle, hip and knee. Other sites include the neck, blood vessels and nerves. Etiology is unknown, still many SS are characterized by chromosomal translocation that fuses the SYT gene from Chromosome 18 to either the SSX1 or the SXX2 gene. The most common symptoms experienced is a mass/swelling associated with tenderness and pain.

We present a 26y/o female G4P2A2 who came to the emergency department complaining of 4 months history of discomfort at the left gluteal and thigh area with associated pressure-like sensation. Patient with history of multiple visits to ER due to persistent feeling of increasing size, all imaging test were unremarkable. Denied any other associated symptoms; including anorexia, fever, SOB or weight changes. As patient continue with worsening symptoms, left lower extremity MRI requested. Vital signs unremarkable. Physical examination with no mark difference in size between the left and right thigh, no underling skin changes, FROM, no tenderness to palpation or weakness. Laboratories with stable cell count. Chemistry with preserve renal function. MRI of the left thigh showed Left gluteus Medius muscle with large well defined soft tissue mass measuring 14.5 cm AP x 10.5 cm transverse x 13.5 cm long. It starts superiorly almost at the level of iliac crest and extends to the level of the greater trochanter. Bone scan showed increased activity in the left acetabulum and greater trochanter of the femur. Left Thigh Biopsy demonstrated Synovial Sarcoma, Intermediate Grade; with presence of monophasic, spindle cell, positive for EMA and keratins AE1/AE3. She was referred to Sylvester Cancer Center, who recommended induction/neoadjuvant with Adriamycin, Ifosfamide and Dexrazoxane.

SS are rare tumors that are slow growing and commonly misdiagnosed due to vague symptoms and poor visualization on X-ray imaging. Our case illustrates an uncommon disease seen in 1 to 3 individuals in a million people each year. Prognosis depends on age, size, site and mitotic activity. Physician awareness of the disease as possible differential diagnosis will allow for early treatment and improve survival rate.

190 A case of hemophagocytic lymphohistiocytosis in the setting of classic hodgkin lymphoma, HIV, and EBV

W Gibson*

P Zito

LS Engel

LSU Health Sciences Center, New Orleans, LA

Introduction

Hemophagocytic Lymphohistiocytosis (HLH) is characterized by overactivation and proliferation of CD8+ T cells, histiocytes, and macrophages. This results in systemic inflammation and phagocytosis of various cell lines. Five out of eight criteria (recurrent fevers, cytopenia, splenomegaly, hypertriglyceridemia, hemophagocytosis in bone marrow or spleen or lymph nodes, elevated ferritin (>500), elevated soluble CD25 (>2,400 U/ml), and low or absent natural killer cell activity) are required for diagnosis.

Case

A twenty-seven-year-old man with a past medical history of untreated HIV presented to the Emergency Department with syncope. He also reports experiencing chills, fevers, diaphoresis, and weakness over the past month. Initial labs revealed a hemoglobin of 5.6 g and platelets 81. Subsequent labs revealed a ferritin 5,825.7, triglycerides 370 mg/dl, soluble IL-2 at 21,540 pg/ml, and HIV viral load 267,742. CT scan demonstrated mild enlargement of the spleen and several enlarge abdominal and pelvic lymph nodes. Recurrent fevers up to 103f were documented. The patient met six out of eight criteria for the diagnosis of HLH. A bone marrow biopsy revealed an atypical CD30 positive lymphoid infiltrate, suspicious for lymphoproliferative disorder. The CD30+ cells were large-sized, most were mononuclear and some were binuclear with large nuclei and conspicuous nucleoli. Atypical, large-sized lymphoid cells were positive for Epstein Barr virus RNA. Flow cytometry was significant for a mix of T, B and NK cells with T cells predominating. A left cervical lymph node biopsy revealed classic Hodgkin lymphoma, nodular sclerosis type.

Discussion

HLH can be acquired genetically (primary) or environmentally (secondary). Mutations in the genes PRF1, MUNC 13–4, and STX11 are associated with primary HLH. Secondary HLH is often triggered by autoimmune disorders or viral infections. The most common trigger for HLH is an infection with Epstein-Barr virus. HLH is often fatal making prompt diagnosis and treatment of great significance.

191 Human epidermal growth factor receptor-2 mutated metastatic lung adenocarcinoma treated with trastuzumab based antibody-drug conjugate

I Haddad*

A Mansurov

H Rehman

S Singal

D Jaishankar

ETSU, Johnson City, TN

Case report

Human epidermal growth factor receptor 2 (HER2/ERBB2) mutation is detected only in 1.5% of all cases of Non-Small Cell Lung Cancer (NSCLC). Despite being a rare event in NSCLC carcinogenesis, it has a significant therapeutic implication. Here we present a patient with ERBB2 mutation-positive lung adenocarcinoma who had a clinically meaningful response to Ado-Trastuzumab Emtansine (T-DM1). A 52 years old female with a history of sarcoidosis and previous tobacco dependency was found to have a left upper lobe nodule on follow up imaging for sarcoidosis. Biopsy showed moderately to poorly differentiated adenocarcinoma with lymphovascular space invasion in the background of non-necrotizing granulomatous inflammation. On staining, the atypical epithelial cells were positive for keratin AE1/AE3, TTF1, ERBB2, and 40% PD-L1 and negative for EGFR, ALK, KRAS, BRAF, MET, RET, and ROS-1 alterations with CD163 positive histiocytes concentrated in the area of the granulomatous inflammation. Given the history of sarcoidosis, upfront immunotherapy was avoided, and palliative carboplatin and pemetrexed chemotherapy initiated. The patient had a good partial response on interim scans and mixed responses on final scans only to develop early progression on maintenance Pemetrexed. She then proceeded on T-DM1 with stable disease on imaging after six cycles of therapy. Low toxicity, good tolerance to treatment and improvement in the quality of life noted. The patient eventually progressed on antibody-drug conjugate and moved to experimental therapy on a clinical trial. The clinical implication of ERBB2/HER2/Neu alterations is well established in breast and gastroesophageal malignancy, but its role in lung cancer is an area of active research. ERBB2 mutations are present in 1.5% of all NSCLC cases with higher prevalence among women, non-smokers, and adenocarcinoma patients. A phase 2 clinical trial with 18 patients has reported a response rate of 44% and a median progression-free survival of 5 months. We believe that our case serves as a good example of HER2/ERBB2/Neu activating mutation being a suitable target for therapy in NSCLC.

192 Incidental finding of para-testicular epidydimal rhabdomyoma in a 22-month old african-american male

B Jafar*

H Imran

P Marri

University of South Alabama, Mobile, AL

Case report

Rhabdomyoma is a rare benign lesion of striated muscle typically categorized as cardiac and extracardiac. They generally occur in the head and neck region but may be found at other sites. Genital rhabdomyoma is extremely rare and mostly observed in females. Few cases of para-testicular rhabdomyomas in adolescents and adults have been reported.

We report a 22 month old African American male with a sudden onset of bilateral tender testicular swelling.

Testicular ultrasound revealed thickening of the right scrotal soft tissues compared to left. The testicles were grossly symmetric and within normal limits in echogenicity and color flow. The right epididymis demonstrated heterogeneity and increased size as compared to the left which measured 1.5 × 0.6 × 0.6 cm and 0.4 × 0.3 × 0.5 cm respectively. Right groin exploration with excision of non-viable torsioned right scrotal mass with orchiopexy was performed.

Specimen measured 2.9 × 1.4 × 0.6 cm; microscopically revealed blood and clusters of large orbicular-ovate cells with small bland peripheral nuclei, discrete cellular borders and a full rounded, laminated cytoplasm with no atypia or malignant changes(Image2).

Staining for desmin was positive(Image1). Staining for S100, NSE, CD34, myogenin, CD68, vimentin, CK cam 5.2, CK AE1/AE3, CD68, p53 SMA, SMA, PASH with and without diastase were all negative. These findings were consistent with a final diagnosis of fetal type rhabdomyoma. Relevant oncologic workup including CT chest, abdomen and pelvis were negative.

This is one of the very few documented cases of pediatric paratesticular epidydimal rhabdomyoma in the literature. Although benign in nature, the exact prognosis is unknown due to its rarity. Available data suggests monitoring due to remote possibility of a recurrence.

Abstract 192 Figure 1

1. Desmin stain- left 2. H&E stain- rightAbstract 192 Figure 1 1. Desmin stain- left 2. H&E stain- right

193 The pulmonary complications of a paraneoplastic autoimmune vasculitis in a patient with myelodysplastic syndrome

PS Jagadish1*

AR Uhelski2

J Redfield1

N Thomson1

TS Strom1

A Weir1

O Ogbeide1

1University of Tennessee Health Science Center, Memphis, TN

2Johns Hopkins University, Baltimore, MD

Introduction

Paraneoplastic autoimmune phenomena may occur in up to 30% of patients with myelodysplastic syndrome (MDS). We present a case of a patient with MDS who developed a paraneoplastic autoimmune vasculitis that led to diffuse alveolar hemorrhage.

Case

A mid-50s male presented with acute chills, weakness, and night sweats. He lost 20 lbs over 2 months and had 2 weeks of fatigue, exertional dyspnea, and epistaxis. His temperature was 102.3°F and oxygen saturation was 84% on room air. He was tachycardic to the 90s. He appeared ill with bilateral pitting edema to knees. Labs showed a hemoglobin of 5.7 g/dL, hematocrit of 17.2 g/dL, and platelet count of 27 kµL. The peripheral blood smear showed 4% blasts and frequent dyspoietic granulocytes. Bone marrow biopsy (BMB) was performed to screen for acute leukemia and MDS, eventually confirming MDS with excess blasts and erythroid predominance.

During hospitalization, the patient developed acute hypoxemic respiratory failure confirmed by bronchoscopy as diffuse alveolar hemorrhage secondary to thrombocytopenia. High-dose corticosteroids (2 mg/kg prednisone) were started for suspected paraneoplastic autoimmune vasculitis, pending BMB results. The patient steadily improved, was extubated, and had reduced oxygen and transfusion requirements.

The patient developed recurrent diffuse alveolar hemorrhage after steroids were stopped for decitabine chemotherapy. He experienced cardiogenic shock and passed away after multiple episodes of cardiac arrest. The family declined autopsy.

Discussion

Diffuse alveolar hemorrhage is a rare but potentially deadly pulmonary complication of paraneoplastic autoimmune vasculitis in MDS. Patients who initially present with atypical autoimmune phenomena need screening for underlying MDS, which can change the promptness, extent, and duration of immunosuppressive therapy. Failure to promptly treat patients with corticosteroids can lead to serious complications and death.

194 Persistent fever and pancytopenia following epstein-barr virus infection- A ‘can’t miss’ diagnosis

A Kala*

E Davis

Louisiana State University Health Science Center, New Orleans, LA

Case report

A 14-year-old male presented with worsening low back pain and two weeks of intermittent high fever. Previous evaluation at outside facilities was significant for leukopenia and a positive monospot test. Just prior to admission, the patient was found to have worsening leukopenia, prompting referral to our emergency department. On presentation, he was tachypneic with splenomegaly, hypotension, and delayed capillary refill. Laboratory findings were significant for neutropenia and thrombocytopenia. The patient was admitted to the ICU for hypotension and was started on broad-spectrum antibiotics. Serum ferritin and lactate dehydrogenase were found to be elevated at that time. An extensive infectious workup and bone marrow biopsy were completed after hospitalist consultation with various subspecialists. Despite early suspicion for macrophage activation syndrome (MAS) or hemophagocytic lymphohistiocytosis (HLH), the diagnosis was not established until hospital day eight when the soluble interleukin – 2 receptor resulted as elevated. Just prior to his final diagnosis, however, the patient’s fevers improved. His neutrophil count began to trend upward. High-dose steroids were initiated, the patient continued to show clinical improvement, and he was discharged on hospital day 10. Despite his initial improvement, the patient’s fever returned two days after discharge. Initial work-up in the ED revealed a significant pancytopenia, and the patient was re-admitted to the hematology/oncology service. During this 27 day admission, etoposide and rituximab were initiated for management of EBV-associated HLH. This case highlights the importance of timely recognition of findings consistent with HLH. Due to the nonspecific constellation of symptoms seen, patients with this condition often undergo extensive, multidisciplinary evaluations prior to receiving a diagnosis. Prompt diagnosis with initiation of appropriate management is vital, as patients can deteriorate extremely rapidly. Increased awareness of the diagnostic criteria for HLH helps physicians to effectively identify and treat this rare and life-threatening syndrome.

195 The epitome of vitamin B 12 deficiency

AA Kamat*

MA Alsharif

J Kimbugwe

C Ashangari

N Rus

K Shepherd

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

Introduction

In the developed world where serologic testing is readily available, the classic presentation of vitamin B12(B12) deficiency is rare. In this case report we present a case of 64 year old male with a classic presentation of B12 deficiency.

Case report

A 64 year old male presented to the emergency room with tingling, numbness in hands and feet for 2 weeks, generalized weakness, unsteady gait and memory problems for 2 months. He had not seen a physician in 7 years. Vital signs were significant for respiratory rate 22/min. Notable findings were of icteric sclera, poor short-term memory, normal motor exam, reduced sensation to touch in a glove and stocking distribution, reduced proprioception and positive Romberg sign. Labs were pertinent for pancytopenia, MCV121fl, reticulocyte count3%, hypersegmented neutrophils on PBS, indirect hyperbilirubinemia, LDH>4000U/L, haptoglobin<10 mg/dL, B12<60pg/mL and folate19ng/mL. Intrinsic factor and anti-parietal cell antibodies were positive. On treatment with B12 replacement he showed hematologic and clinical improvement. The classic presentation of B12 deficiency including pancytopenia with macrocytic anemia, jaundice and neurologic abnormalities are rare in the developed world. Because of readily available and routinely obtained serologic testing, many deficiencies come to attention due to the incidental finding of anemia or macrocytosis. Our patient had not seen a physician in 7 years and this explains why he had all the classic features. Lab findings including pancytopenia, elevated LDH and indirect bilirubin, and low haptoglobin were due to ineffective erythropoiesis resulting in hemolysis. Unlike other forms of hemolytic anemia, the reticulocyte count was low. For individuals with typical laboratory findings, the only initial testing needed is a serum B12 level. The neurologic features include subacute combined degeneration due to demyelination of the posterior and lateral columns of the spinal cord and can be present in the absence of macrocytic anemia. Clinician should be aware of rare findings related to B12 deficiency. The presence of anemia also carries important prognostic significance. Anemia detected at baseline is inversely related to the severity of neurological improvement at the diagnosis.

196 Multiple myeloma with initial presentation of spinal shock

N Krishnan1*

M Chahin1

M Oye1

M Shaikh2

1University of Florida College of Medicine, Jacksonville, FL

2University of Florida College of Medicine Jacksonville, Jacksonville, Afghanistan

Case report

Multiple Myeloma (MM) comprises nearly 20% of all hematologic malignancies in the United States. The importance of recognizing MM cannot be overstated due to the mortality of the disease and the improved outcomes in the setting of early detection and treatment. We present the case of a 58-year-old male who presented to the emergency department with acute bilateral flank pain, urinary retention and worsening left sided weakness and progressive dysarthria. He has a past medical history of hypertension, hyperlipidemia, benign prostatic hyperplasia and stroke with residual LLE weakness and impaired cognition. This was his third presentation to the emergency room for similar complaints. This time, he had a new-onset functional deficit and lower extremity symptoms. He had a stroke scale of 5, but was outside the tPA window. A computed tomography (CT) scan of the head did not demonstrate any acute infarct. The patient underwent MRI of the spine, demonstrating a large lobular enhancing mass from t5-t7 and extension through the neural foramina and paraspinal musculature and pleura with mass effect from the level of t5-t7. IR biopsy of the enhancing mass showed a plasma cell neoplasm with immunostaining positive for CD138 and with lambda monoclonal light chain predominance. Due to concern for neurological emergency, neurosurgery was consulted, but did not intervene due to patient’s debility. A subsequent bone marrow biopsy showed only 6–8% plasma cell proliferation. Though this is not in the common range of MM, a metastatic bone survey showed several lytic lesions and the patient was diagnosed and offered treatment for MM, which he declined.

This patient’s care and medical treatment were significantly delayed due to lack of recognition due to the patient’s vague symptoms and poor history. The patient also did not have any significant renal disease, hypercalcemia or anemia throughout his several admissions that may have otherwise raised suspicion for Multiple Myeloma. This case is an important reminder for internists to be diligent in all parts of the physical examination, including the neurological exam, which can unmask medical emergencies requiring prompt intervention as in this patient.

197 Ovarian high-grade serous carcinoma with neuroendocrine component, a rare and aggressive tumor

V Kumar*

A Shalaby

V Manucha

University of Mississippi Medical Center, Jackson, MS

Case report

High-grade serous carcinoma with neuroendocrine component has been described in the endometrium, but very rarely in ovaries. We describe a case of a 60-year-old female who presented with abdominal pain, foul vaginal discharge and elevated CA-125. Computed tomography scan revealed two large pelvic masses with metastasis to the small bowel and mesenteric lymph nodes. Patient underwent bilateral salpingo-oophorectomy with omentectomy and bowel resection because of mass adhesions to the mesentery. Gross examination showed bilateral ovarian masses (left, 8.2 cm and right, 7.5 cm) with solid and cystic areas and extensive necrosis causing near-complete effacement of the ovarian parenchyma. Histologically, the adnexal masses consisted of two components; high-grade serous carcinoma and a discrete and focally intermingling neuroendocrine carcinoma (figure 1). The small bowel, omentum and mesenteric lymph nodes showed neuroendocrine carcinoma only (figure 2). The serous and neuroendocrine carcinomas were positive for PAX8 (figures 3&4), WT1, p53, p16 and ER (patchy) and negative for PR. Chromogranin was positive only in the neuroendocrine carcinoma component (figure 5) with a Ki-67 of >95%. Patient passed away 10 days following the surgery. Ovarian serous carcinomas with neuroendocrine components are rare and aggressive tumors that at sites of metastasis may only be comprised of neuroendocrine component, which may result in a delayed diagnosis or misdiagnosis. This case highlights that an ovarian epithelial tumor with neuroendocrine differentiation is important to consider when a neuroendocrine tumor in abdominal lymph nodes or bowel wall is detected in regional or temporal connection with an ovarian/adnexal mass.

198 The persistent inhibitor

E LeJeune*

C Milner

University of Mississippi, Flowood, MS

Case report

Acquired Factor VIII inhibitors, also referred to as acquired hemophilia A is a rare bleeding disorder that differs in several ways to its congenital hemophilia A counterpart. Aquired hemophilia A has no inheritance pattern, presents typically later in life, and is seldom found to present with hemarthroses, but rather soft tissue bleeding and purpura. Unfortunately, it is associated with high mortality.

This is a 79 year old female who presented to her hematologist from her PCP with new bruising. Upon evaluation she was found to have a normal CBC, platelet function test, PT, and INR. However, the patient had a prolonged PTT of 146.2 seconds that did not correct with a mixing study, an undetectable Factor VIII level, and a Factor VIII inhibitor level greater than 1000 bethesda. She was started on prednisone 1 mg/kg for her acquired Factor VIII inhibitor and repeat studies 2 weeks later revealed PTT of 121.2, a continued undetectable Factor VIII level, and a continued Factor VIII inhibitor level greater than 1000 bethesda. She later developed a left hand hematoma and was admitted to the hospital for FEIBA (factor eight inhibitor bypassing agent). While hospitalized she showed no further signs of bleeding and her hematoma remained stable in size. The patients prednisone was continued, and she was started on Rituxan 375 mg IV weekly and Cyclophosphamide 100 mg daily. Despite 4 weeks of this therapy, her Factor VIII level remained undetectable, her PTT prolonged, and her Factor VIII inhibitor level although initially decreased to 614.4 bethesda later increased to 901 bethesda. She currently continues to exhibit no active signs of bleeding and stability in her hand hematoma.

Given the extreme rarity of this disease of 1 to 4 million/year, studies and literature regarding treatment are limited primarily to case reports, small retrospective studies, and personal clinician experience. Approaches to inhibitor eradication typically require a combination of immunosuppressive agents, high dose intravenous immunoglobin (IVIG), and immune tolerance. Given this patients persistent elevated inhibitor level despite steroids and immunosuppressive agents we will need to pursue other agents such as calcineurin inhibitors, mycophenolate mofetil, and/or IVIG to achieve inhibitor eradication and restore the patients normal hemostasis.

199 An exceedingly rare case of bullous pemphigoid in the setting of multiple myeloma

J Lung*

J Ruiz

AE Martinez

B Attarha

JR Rivas

University of Florida, Jacksonville, FL

Case report

Multiple myeloma is a neoplastic process involving the proliferation of plasma cells. Very rarely, multiple myeloma has been found to have an association with pemphigus vulgaris. Exceedingly less likely is finding bullous pemphigoid in the setting of myeloma. We report a rare case of bullous pemphigoid in a patient with newly diagnosed myeloma.

A 55 year old African American female presented to the emergency department for sudden eruption of large bullae. She reported recent admission for chest pain one week prior to presentation. Chest radiograph showed multiple lytic lesions and metabolic panel with an elevated serum creatinine. Acute coronary syndrome was ruled out, but urine and serum immunofixation and protein electrophoresis suggested monoclonal gammopathy. Bone marrow biopsy of the vertebrae revealed approximately 70% involvement by abnormal plasma cells consistent with multiple myeloma. Several days later, blisters developed on her thighs. It was thought to be a drug rash at first, but worsening progression led her to pursue a second opinion. On presentation, she had multiple large bullae on her lower extremities. Skin biopsy showed subepidermal splitting with superficial perivascular lymphohistiocytic inflammation most consistent with bullous pemphigoid. Topical steroids as well as methotrexate and folic acid were initiated for bullous pemphigoid. Regarding the patient’s multiple myeloma, she was started on bortezomib infusions, oral dexamethasone weekly, as well as allopurinol due to high risk of tumor lysis syndrome.

Multiple myeloma is a neoplastic proliferation of plasma cells who’s signs and symptoms are related to the organs the plasma cells invade. Bone involvement may lead to pain, anemia, and hypercalcemia. Both bullous pemphigoid and pemphigus vulgaris are blistering diseases. Pemphigus vulgaris tends to involve mucous membranes, occurs in younger people, has easily ruptured blisters, and carries a poor prognosis. Bullous pemphigoid, however, has no mucosal involvement, occurs in older people, contains tense bullae, and has a favorable prognosis. Treatment depends on disease severity. Our patient is particularly interesting in that bullous pemphigoid is very rare in general and even more so in the setting of multiple myeloma.

200 A rare case of isolated hypothalamic-pituitary langerhans’ cell histiocytosis

J Makram*

H Mallah

A Wichmann

A Motes

M Elmassry

A Rivas-Mejia

K Nugent

Texas Tech University Health Sciences Center, Lubbock, Texas, Lubbock, TX

Introduction

Langerhans’ cell histiocytosis (LCH) is a rare disease with isolated neurological involvement in 5–10% of cases. We report a case of isolated hypothalamic-pituitary LHC in a woman presenting with altered mental status.

Case description

A 35-year-old woman presented with increasing confusion for several weeks with occasional headaches and blurred vision. Her mental status was altered; she was hypotensive, afebrile, and dehydrated. We intubated her for airway protection and started inotropes after her condition did not improve with intravenous fluid boluses. Her sodium level was 167 mmol/L. After admission to the intensive care unit, her urine output was 1000 mL/hr. A diabetes insipidus (DI) workup revealed a serum osmolality of 350 mOsm/kg and urine osmolality of 100 mOsm/kg; her urine output decreased after desmopressin administration. Further testing revealed panhypopituitarism.

Magnetic resonance imaging (MRI) of her brain revealed a mass in the optic hypothalamic region involving the optic chiasm and medial walls of the third ventricle with extension into the pituitary stalk. Tissue biopsy from a right craniotomy showed atypical cells in focal aggregates positive for CD68, CD1a, and Langerin consistent with LCH. She was managed with hydrocortisone, desmopressin, and levothyroxine. A skeletal survey was negative for lytic lesions, and we found no bone marrow involvement. She received 2 cycles of vincristine and cytarabine; her overall condition improved, and her sodium levels normalized.

Discussion

LCH is a rare multisystemic disease with uncertain pathogenesis characterized by clonal proliferation of bone marrow-derived immature dendritic cells and usually affects children. Adult-onset LCH occurs in approximately 2/million populations/year. LCH is often misdiagnosed given its rarity, multisystemic involvement, and various presentations. DI seems to be the most common, well-described manifestation of hypothalamic-pituitary involvement. Anterior pituitary dysfunction is reported in <20% of patients with LCH and is usually concurrent with DI. Physicians should suspect LCH in patients developing central DI regardless of anterior pituitary dysfunction and underlying intracranial mass on MRI.

201 Oligometastatic disease: an emerging concept in oncology

H Malik1*

A Garcia1

W Dawson2

J Ellis2

1LSU Health Science Center, Metairie, LA

2West Jefferson Medical Center, Marrero, LA

Purpose

OGM breast cancer case report and increase awareness on the use of locoregional therapies (LRT) in metastatic cancer.

Methods

Review of Electronic Health Records and medical literature

Results

A 46-year old presented with pain in her right axilla. She was diagnosed with triple negative breast cancer (TNBC) in 2008 treated with surgery (SRG), radiation (XRT) and chemotherapy (CTX). She recurred in the axilla in 2014 treated with SRG and CTX, in the chest wall in 2016 treated with CTX and XRT and with an unresectable chest wall mass in 2018. Due to prior XRT she was considered at high risk for tissue necrosis with additional XRT. She received chest wall cryoablation (CAB) x 2 with symptom resolution. Pain remained under control for 8 months.

Metastatic TNBC (mTNBC) is an incurable disease. CTX is the cornerstone of treatment with goals of improving survival and palliation. Median survival (MS) is 2 years. Second line CTX has modest activity with response <20% and time to tumor progression <6 months. XRT and SRG are reserved for symptomatic or non-responsive disease. OGM cancer is an emerging concept, in which some metastatic tumors remain locoregionally confined while others are widely disseminated. It was not until recently that this concept is taking more relevance. RNA analysis shows that oligo- and poly- metastases are distinct entities. OGM tumors may benefit from LRT which include stereotactic body radiation (SBRT), minimally invasive SRG or ablation. Ablation may be achieved by heat (radiofrequency, microwave and laser), or by the use of CAB which cools tissue to <−40°C producing tissue necrosis. CAB can be used in the treatment of bone lesions, renal masses, lung, prostate and breast tumors. Recent data supports the use of LRT in patients with OGM cancer. The SABR-COMET phase II randomized trial reported a MS of 28 months for systemic therapy alone vs 41 months with the addition of SBRT (Lancet 2019). A phase II/III trial is underway in OGM breast cancer.

Conclusions

This case is an example of OGM TNBC with survival >6 years. The use of local therapies probably contributed to her survival. This case also exemplifies the value of CAB in treating recurrent tumors, particularly when other techniques, such as SRG or XRT are not appropriate.

202 Nsclc targeted therapy: excellent response to every other day osimertinib

S Manthri*

P Sharma

S Singal

K Chakraborthy

ETSU, Johnson City, TN

Case report

Osimertinib is an oral, third-generation, irreversible epidermal growth factor receptor (EGFR) tyrosine kinase inhibitor approved as first-line treatment for EGFR mutant non-small cell lung cancer (NSCLC). In a double-blind, phase 3 trial osimertinib standard dose was 80 mg once daily until disease progression or unacceptable toxicity. If coadministration with a strong CYP3A4 inducer cannot be avoided, osimertinib dose of 160 mg once daily was recommended. We present a rare case with unique dosing schedule of every other day osimertinib 40 mg in metastatic NSCLC with excellent response. A 78-year-old male with a known diagnosis of early-stage lung adenocarcinoma was status post lobectomy followed by adjuvant 6 cycles of chemotherapy. Approximately two years later, the patient experienced local recurrence and was treated with concurrent chemoradiation. Surveillance scans three years later showed widespread progression and biopsy confirmed the metastatic disease. EGFR Exon 19: p. E746_A750 deletion was detected. The patient was started on erlotinib 150 mg daily; eventually, it was switched to osimertinib 80 mg once daily due to lack of tolerance and requiring multiple interruptions secondary to diarrhea, rash and hand-foot syndrome. Erythematous and desquamating rash along with diarrhea continued. Osimertinib was subsequently dose reduced to 40 mg once daily followed by 40 mg every other day. The pharmacogenomics test did not reveal patient was poor metabolizer leading to increased toxicity. Targeted therapy has changed the landscape and management of NSCLC. The results of the FLAURA trial show that in patients with previously untreated EGFR mutation-positive advanced NSCLC, osimertinib treatment resulted in significantly longer progression-free survival (18.9 months). Despite such promising outcomes, toxicities like skin rash and diarrhea could be dose-limiting. With the trend towards precision medicine with customization of healthcare, with medical decisions, treatments being tailored to the individual patient, we still need more understanding of pharmacokinetics/pharmacodynamics. Our patient with the current odd dosing schedule of osimertinib 40 mg every other day has better tolerability and continues to show excellent treatment response for approximately 10 months.

203 Multiple squamous cell skin cancers as a complication from long-term hydroxyurea therapy

S Manthri*

P Sharma

D Jaishankar

T Lord

ETSU, Johnson City, TN

Case report

Hydroxyurea is an antimetabolite that selectively inhibits ribonucleoside diphosphate reductase thereby halting the cell cycle at the G1/S phase. Patients with myeloproliferative neoplasms at high risk for venous or arterial thrombosis should be treated with cytoreductive therapy to reduce the risk of thrombosis. Hydroxyurea is the preferred cytoreductive agent for most patients. Squamous cell carcinoma is a rare side effect reported in less than 1% of patients on Hydroxyurea and is not widely known. A seventy-two-year-old male with JAK2 V617F mutation-positive essential thrombocytosis was treated with hydroxyurea for 6 years, which was effective in controlling his platelet count. After 6 years of therapy, the patient developed several skin lesions over the scalp, face, and extremities (sun-exposed areas). He was evaluated by dermatology and biopsies were consistent with multiple squamous cell skin cancers. He had recurrent squamous skin cancers requiring treatment/removal almost on a weekly basis. Hydroxyurea was slowly tapered and discontinued, and we noted an improvement in skin lesions that required weekly intervention to no further new lesions requiring removal. The patient was simultaneously started on Ruxolitinib, which selectively inhibits Janus Associated Kinases based on phase 2 data refractory to or intolerant of hydroxyurea. Following discontinuation of hydroxyurea, WBC increased from 9.4 K/uL to 36 K/uL and platelets increased from 439 to 1762 K/uL. Ruxolitinib was increased to 10 mg BID and WBC improved to 15.5 K/uL and platelet count of 836 K/uL. Cutaneous cancers associated with the use of hydroxyurea are often multiple and include both squamous cell carcinomas and basal cell carcinomas. They typically appear on sun-exposed surfaces. Despite being rare, these complications often have clinical consequences affecting a patient’s quality of life and requiring dermatologic intervention. This case highlights the need to be cognizant of this rare toxicity and advocate expedited dermatological evaluation if concerning symptoms arise. Patients may need skin surveillance for many years after the discontinuation of hydroxyurea therapy because of the apparent prolonged duration of the risk of skin cancers.

204 Erythema nodosum – a rare side effect of immunotherapy

S Manthri*

E Spradling

ETSU, Johnson City, TN

Case report

Erythema nodosum (EN) is a delayed-type hypersensitivity reaction that most often presents as erythematous, tender nodules on the shins. EN can occur secondarily to a wide variety of conditions. We present a rare case where EN developed while receiving nivolumab immunotherapy. A 38-year-old female with a history of invasive malignant melanoma involving her right foot plantar surface (pT3ApN0). She noticed another skin lesion in close proximity to her existing scar. It was biopsied and demonstrated acral lentiginous melanoma with a Breslow thickness of 0.4 mm, mitoses 0/mm2, and no ulceration. She subsequently underwent wide excision of her right great toe melanoma and split-thickness skin grafting. Biopsy results were negative for residual melanoma. While completing wound healing, she had recurrent acral lentiginous melanoma. PET/CT showed interval development of a right inguinal lymph node measuring 1.4 × 1.1 cm with a maximum SUV value of 2.2. Right inguinal core biopsy confirmed involvement with metastatic melanoma. Neoadjuvant immunotherapy with nivolumab for three months followed by right inguinal dissection and excision of the primary lesion was planned. After receiving the third cycle of nivolumab, she developed diarrhea and bilateral skin and soft tissue changes in her lower extremities. She was evaluated by dermatology and underwent a left shin biopsy that showed septal panniculitis most consistent with EN. To date, no reports of nivolumab causing EN have been reported in the literature. Common triggers for EN include infection, drugs, pregnancy, malignancy, and inflammatory conditions. However, many cases are idiopathic. EN usually resolves spontaneously within several weeks. When necessary, treatment can be given to reduce symptoms or hasten resolution. Our patient was not a candidate for first-line NSAID therapy, so second line prednisone was used with excellent response and resolution of all EN lesions. She was also referred to gastroenterology for a colonoscopy to rule out inflammatory bowel disease and immune-mediated colitis. There is a resurgence of interest in the rapidly evolving field of immunotherapy treatment of advanced solid tumors but the understanding of toxicity profiles is incomplete. Our patient was a cautionary tale and treating physicians should continue to monitor patients closely for rare side effects.

205 Early onset T cell leukemia in a 48- year-old man presenting with rapidly progressive liver disease

B Mantilla*

MN Vinan Vega

C Cinthya

E Mohamed

Texas Tech University Health Science Center, Lubbock, TX

Case report

T cell prolymphocytic leukemia (T-PLL) is a rare and highly aggressive proliferative disorder of lymphoid cells. It accounts for 2% of mature lymphocytic leukemias in adults. Males are predominantly affected with a median age of 61 years. Clinical presentation consists of a short period of B symptoms, hepatosplenomegaly and lymphocytosis. Lymphadenopathy is usually not clinically evident.

A 48-year-old Hispanic male with no medical history presents with 3 months of progressive fatigue accompanied by abdominal pain, diarrhea and fever. He also noted hematospermia, choluria and epistaxis. Physical exam was positive for scleral icterus, hepatosplenomegaly and a non-pruritic maculopapular rash on the chest. No lymphadenopathy was noted on physical exam. Initial studies showed lymphocytic leukocytosis (19.88), normocytic anemia (9.9) and thrombocytopenia (23). Laboratory findings also revealed transaminitis (ALT 112, AST 123), elevated ALP (507) and hyperbilirubinemia (5.1) with mild elevation in INR (1.54). Workup was consistent with anemia of chronic disease with normal haptoglobin and direct coombs test. Peripheral blood smear was significant for leukoerythroblastosis with 5% blasts. Imaging studies revealed hepatosplenomegaly and multiple small lymph nodes in retroperitoneum and mesentery. Hepatitis B, C and HIV screening were negative as well as acute infection with EBV, CMV, HSV-1 and HSV-2. Patient had a rapid decline in liver function within 10 days of hospitalization. Other causes of liver disease including hemochromatosis, alpha 1 antitrypsin deficiency, Wilson disease, autoimmune hepatitis and primary biliary cirrhosis were ruled out. Liver biopsy showed acute hepatitis with bridging necrosis, no lymphocytic infiltration was observed. Bone marrow biopsy results finally revealed the diagnosis of T cell prolymphocytic leukemia. Patient is currently receiving treatment with alemtuzumab.

The prognosis of T-PLL is poor with a median survival of 1–2 years with treatment. Current management is determined on best available evidence as no randomized controlled trials have been developed. More observational and experimental studies are required to understand this rare disease.

206 An incidental case of transient erythroblastopenia of childhood

A Mao1,2*

BS Gavan1

CW Turner1

1USA Children and Women’s Hospital, Mobile, AL

2USA College of Medicine, Mobile, AL

Introduction

Transient erythroblastopenia of childhood (TEC) is a transient red cell aplasia characterized by a temporary cessation of erythrocyte production in previously healthy children. It is the most common pure red cell aplasia in children. The etiology is unknown, although possible causes of TEC include viral illness, serum inhibitors against erythroid progenitor cells, and cell-mediated suppression of erythropoiesis. TEC is seen in children between the ages of 6 months to 6 years, with a predilection for males. Patients generally have an anemia with Hb between 6–8 g/dL and reticulocytopenia due to transient suppression of RBC production by bone marrow.

Case

Our patient is a healthy 12 month old African American male born to a 21 year old mother with a history of asthma. Patient was born at 39 weeks with no complications. Prenatal history was unremarkable. No reported family history of anemia, bleeding disorders, or hemoglobinopathies.

The patient presented to our clinic for his 12 month well child visit in which routine CBC and lead level were drawn. Besides a resolving viral illness reported 2 weeks prior to his clinic visit, his checkup was normal. Labs showed severe anemia with a Hb of 5.5, MCV of 76, and a reticulocyte count of 1.7. The patient was subsequently admitted to the hospital for further evaluation. Labs for Parvovirus B19, EBV, and CMV were drawn and were found to be negative, and blood smear showed normocytic normochromic anemia. Patient received PRBC transfusion at 15cc/kg during hospital admission. He remained clinically stable and was discharged to follow-up with Heme/Onc as an outpatient. Repeat CBC post-transfusion revealed Hb of 14.2. The constellation of lab results, history of recent viral illness, and quick resolution of symptoms led to the diagnosis of transient erythroblastopenia of childhood.

Discussion

TEC is a diagnosis of exclusion; therefore, it is important for the clinician to have a broad differential diagnosis of anemia. These include congenital hemolytic anemias such as hereditary spherocytosis, RBC enzyme defects, and hemoglobinopathies. Another important consideration is Diamond-Blackfan anemia, which presents with abnormal physical exam findings including microcephaly, growth failure, and triphalangeal thumbs.

207 Acute graft versus host disease in a patient with autologous hematopoietic stem cell transplant

WD Marrero*

V Fonseca-Ferrer

HR Cintrón-Colón

A Cruz

JG Rodriguez Velez

San Juan City Hospital, San Juan, PR

Case report

Acute graft-versus-host disease (GVHD) is an unpredictable severe inflammatory complication of allogeneic hematopoietic cell transplantation. Theoretically should not exist in autologous-hematopoietic stem-cell-transplant (auto-HSCT).

We report the case of a 61-year-old -female diagnosed with multiple myeloma (MM) in May 2017. Treatment was started with 6 courses of bortezomib-dexamethasone regimen. Bone Marrow biopsy on December 2017 revealed complete remission. After evaluation; patient was considered a candidate for auto-HSCT. Mobilization of stem cells was performed with filgastrim-perixaflor regimen followed by collection of cells with no complications. Conditioning chemotherapy was given with melphalan (200 mg/m2) for two days. The regimen was tolerated without complications, stem cell infusion was performed with a total dose of 2.82 × 106 CD34 negative cells/kg. During the post- transplant period low hemoglobin and platelets counts were managed with transfusions. Granulocyte colony stimulating factor was started on day+7 until successful engraftment on day+12. On day +14 patient developed gastrointestinal discomfort and watery diarrhea that got worse despite treatment with anti-motility and anti- microbial agents. Work up for infective causes of diarrhea, such as Clostridium difficile assay, PCR for Cytomegalovirus, Epstein-Barr virus, and stool cultures for bacteria, parasites and fungal pathogens were negative. Further evaluation with a sigmoidoscopy and biopsy revealed the presence of mucosa with regenerative changes, crypt architectural distortion, crypt abscess, and focal crypt loss and increase apoptosis. Findings consistent with acute GVHD.

Acute GVHD is a common and serious complication of allogeneic HSCT. The presence of acute GVHD in autologous HSCT is extremely rare. There is no proven explanation for the pathophysiology of auto-GVHD. Our case illustrates a rarely seen complication of auto-HSCT and a possible different etiology for the mechanism of GVHD. Also the importance of including it in the differential diagnosis of a patient with Auto-HSCT and no infective cause for diarrhea or any gastrointestinal manifestation.

208 5-fluorouracil induced cardiomyopathy in a pediatric patient: rare event

G Marrero-Rivera*

D Leblanc

Children’s Hospital of New Orleans-LSUHSC, New Orleans, LA

Case report

5-fluorouracil (5-FU) is a commonly used chemotherapy agent in adult solid tumor patients but is less frequently used in pediatrics. In adults, 5-FU is a well-recognized inducer of cardiotoxicity, particularly among patients with underlying cardiovascular disease. 5-FU mediated cardiotoxicity has rarely been reported in the pediatric population.

Objectives

We report a rare case of 5-FU induced cardiomyopathy in a pediatric patient with nasopharyngeal carcinoma (NPC), successfully managed with milrinone and uridine triacetate, underscoring the need for awareness and prompt recognition and medication administration for this potentially reversible condition.

Design/Method

A literature search was conducted for 5-FU induced cardiomyopathy in pediatric patients. We identified 2 previously reported cases published in 1995 and 2011, both in patients with similar diagnoses and chemotherapy regimens.

Results/Conclusion

A 16 year old female diagnosed with stage IVA NPC began treatment with a regimen of Cisplatin and 5- FU. Echocardiogram prior to chemotherapy demonstrated normal cardiac function. Planned initial chemotherapy regimen consisted of 3 cycles of Cisplatin (80 mg/m2) on day 1 and 5-FU (1,000 mg/m2/day continuous infusion) on days 1–4. On day 3 of cycle 1, she acutely developed hypotension and tachycardia. Electrocardiogram demonstrated sinus tachycardia and echocardiogram showed significantly compromised cardiac function, with LV ejection fraction (EF) of 33% (previously 68%). She was transferred to the pediatric ICU and milrinone drip was initiated. Chemotherapy was continued and the 5-FU infusion was completed. Cardiac function continued to decline. Due to the temporal relationship, 5-FU cardiotoxicity was considered as the etiology of the patient’s cardiomyopathy. At hour 60 past the completion of 5FU infusion, uridine triacetate therapy was initiated. Within 48 hours, LV EF had improved to mid-40%. By 96 hours, EF normalized and milrinone was weaned off. She completed the full regimen of uridine triacetate (10 grams q6 hours; total of 20 doses). Prompt recognition and appropriate management is essential to achieve favorable outcomes, and normalization of cardiac function is possible following an event.

209 Symptomatic anemia due to upper gastrointestinal bleed in the setting of ITP

AE Martinez*

B Attarha

J Lung

University of Florida Health Jacksonville, Jacksonville, FL

Case report

This is a 60-year-old African America male with past medical history of diabetes type 2, hyperlipidemia, and hypertension that came to the emergency room with shortness of breath, dizziness, and multiple syncopal events throughout the past four weeks or so. Patient admitted to having worsening symptoms with increased activity, he also noticed dark sticky stools for the past 2–3 weeks as well. In the emergency room the patient was found to be severely anemic with hemoglobin of 3 g/dL (normal range 14–18 g/dL) as well as thrombocytopenic with an undetectable number of platelets, thus he was admitted to internal medicine service for severe symptomatic anemia and thrombocytopenia. Upon physical exam patient had a fairly unremarkable exam except for a mildly tearful/anxious male, tachycardic, pale conjunctiva, fingernail clubbing, and a guaiac positive digital rectal exam. Patient had not been on any anticoagulation, hasn’t been compliant with any medication, has never had a colonoscopy and has no known family history of colon cancer or any other neoplasm. Patient admits to being a current every day smoker with 52 years at 1–1.5 packs per day, but denies drugs and alcohol use. In total the patient was given 6 units of packed red blood cells, 1 unit of fresh frozen plasma, and 2 units of platelets. These blood products helped to increase the hemoglobin to a safer level, but did not help the platelets move above 1 thou/cu mm. He was monitored closely; the gastrointestinal team was consulted for possible need for EGD/Colonoscopy due to suspected cause of the anemia being a bleed somewhere in the gastrointestinal tract. Hematology oncology was also consulted due to the near zero count of platelets, and common things being common a hemoglobin this low usually is not accompanied with an undetectable thrombocytopenia. Once hemolysis labs and iron panel were negative, this pathology most closely pointed to Immune Thrombocytopenic purpura (ITP). Hematology decided to treat as such with IVIG and decadron 40 mg daily for four days. Patient was monitored closely and with the stabilization of his hemoglobin and slight rise and up trend of his platelets the patient was safely discharged home.

210 Complete clinical and histological response to combination nivolumab and ipilimumab in a patient with metastatic renal cell carcinoma

Y Pandey

A Matin*

A Kunthur

University of Arkansas for Medical Sciences, Little Rock, AR

Introduction

About 25% of the patients with renal cell carcinoma (RCC) present at an advanced stage. The treatment approach of metastatic RCC depends on various prognostic risk factors. Currently, Immunotherapy with checkpoint inhibitors and targeted therapy with vascular endothelial growth factor inhibitors are the primary systemic modalities for the management of advanced RCC. We present a unique case of metastatic RCC with heavy disease burden and poor-risk features treated with combination checkpoint therapy resulting in a complete radiological and pathological response.

Case description

A 56-year-old-male presented with gross hematuria, left flank pain and weight loss. Initial laboratory workup showed hemoglobin of 10.7 g/dl, platelet of 3.5 × 109/L, neutrophil count of 8.7 × 109/L, creatinine of 1.2 mg/dl, lactate dehydrogenase (LDH) of 674 U/L and corrected calcium of 11.5 mg/dl. Computed tomography(CT) scan revealed large enhancing mass in the left kidney measuring 10.2 cm × 11.8 cm × 11 cm with extension into the left renal vein, renal pelvis, left ureter, bladder, multiple liver nodules, left adrenal nodule and multiple subcentimeter lung nodules. Ultrasound-guided needle biopsy of the liver confirmed clear cell RCC. The patient was categorized as stage IV RCC with the high-risk disease by IMDC prognostic model. The patient was started on combination nivolumab at 3 mg/kg and ipilimumab at 1 mg/kg at every three weeks for four cycles followed by nivolumab at 3 mg/kg every 4 weeks. Re-staging CT scan done after 36 weeks showed a significant reduction in left renal mass with complete resolution of liver metastasis, lung nodules, left adrenal mass and left renal vein involvement. The patient subsequently underwent left radical nephrectomy and pathology revealed complete tumor necrosis without evidence of viable RCC. The patient continues to be on maintenance nivolumab monotherapy without evidence of recurrence.

Conclusion

Currently, combination nivolumab and ipilimumab in combination is a preferred option for intermediate- and poor-risk patient with clear cell stage IV RCC. Further molecular studies are needed to better understand why some patients have a dramatic response,while some have only a partial response to immunotherapy.

211 Ruptured angiomyolipoma: an uncommon consequence for a common association

A McKernan1*

C Styres1

B Stevens1

A Rafique2

1LSU Health Sciences Center, New Orleans, LA

2Tulane University School of Medicine, New Orleans, LA

Case report

Renal angiomyolipomas are found in approximately 2% of the general patient population. There is an increased frequency in patients with tuberous sclerosis complex (TSC) where the frequency rises to 50–60%; however, most patients with TSC remain asymptomatic. A small subset will have spontaneous rupture, leading to potentially life-threatening hemorrhage. We present the case of a 16-year-old male with tuberous sclerosis who presented with a life-threatening hemorrhage and was found to have a large retroperitoneal angiomyolipoma.

A 16 year-old male with tuberous sclerosis complex presented to the emergency department with two days of supra-pubic pain with associated hematuria, dysuria and dizziness; two weeks prior, he had experienced blunt trauma to the left flank with intermittent pain. Initial urine studies showed macroscopic and microscopic hematuria as well as proteinuria, without white blood cells or infectious etiology. Lab work showed a microcytic anemia with elevated PTT and fibrinogen, but a normal PT/INR, factors 8, 9, 11 and 13 assays, platelet count, electrolytes and kidney function. CT scan demonstrated a large heterogeneous mass consistent with a large pseudoaneurysm with possible active extravasation. Patient was stabilized with intravenous fluids and pain control with temporary resolution of gross hematuria. On day two of admission, patient developed worsening dizziness and increasing amounts of gross hematuria with orthostasis. He received red blood cell transfusion and emergently underwent intervention radiology-guided embolization. Due to the vascularity of the lesion, biopsy was unable to be obtained but follow up MRI showed a left-sided bleeding angiomyolipoma with small bilateral renal lesions. The patient returned with a Foley catheter in place that was able to be removed as hematuria gradually improved prior to discharge with resolution of symptoms.

This case demonstrates an important tumor association of tuberous sclerosis complex presenting with a life-threatening hemorrhage. It stresses the importance of initiating potentially life-saving interventions, while also limiting the possible risks of additional hemorrhage for a definitive diagnostic biopsy.

212 Mucinous adenocarcinoma of lung mimicking interstitial lung disease

H Mhadgut*

H Rehman

D Jaishankar

East Tennessee State University, Johnson City, TN

Case report

A 72-year-old gentleman with multiple comorbidities presented to our hospital as a transfer with symptoms of progressive dyspnea. He developed productive cough and dyspnea over a 4-month span and was treated with IV antibiotics and steroids without relief. He reported night sweats but denied any fevers, chills, decreased appetite or weight loss. CXR and subsequent CT chest revealed bilateral consolidative reticulo-nodular opacities suggestive of interstitial lung disease. Progressive worsening of symptoms and radiographic imaging led to a bronchoscopy which was nonconclusive. Eventually video-assisted thoracoscopic surgery with wedge biopsy of right upper, middle and lower lobes revealed invasive adenocarcinoma with predominant mucinous pattern, positive for CK7, CK20 and CDX2. The tumor cells were negative for TTF-1, napsin 1 and SATB2. Staining pattern was concerning for a primary lung or pancreatic tumor. Labs reported normal LFT’s. CT abdomen and pelvis did not reveal any suspicious pancreatic lesions. CT head was negative for intracranial metastatic disease and Bone scan was normal. Recent EGD and a colonoscopy within the last year was reported as normal. In the setting of bilateral extensive lung lesions on CT chest, he was diagnosed with stage IV invasive mucinous adenocarcinoma of lung. Invasive mucinous adenocarcinoma (IMA) is a rare subtype of lung adenocarcinoma with an incidence of 0.2% among lung cancers. The mucin produced by these tumors is usually expressed as phlegm. Multilobular consolidation pattern with air bronchograms on CT chest is a characteristic radiographic picture. IMA lack TTF-1 expression, correlate with KRAS mutation and lack EGFR mutation. Tumor cells with intracytoplasmic mucin and lepidic growth pattern with microscopic skip lesions are a histologic hallmark. Absence of significant atypia can make the diagnosis challenging on biopsy. IMA may be associated with poor survival outcomes when compared with other subtypes of lung adenocarcinoma. This case highlights the importance of keeping IMA in our differential when evaluating patients with clinico-radiographic findings of interstitial lung disease.

213 Androgen deprivation therapy in androgen receptor positive salivary gland tumor A tissue agnostic approach?

H Mhadgut*

A Mansurov

K Chakraborty

D Jaishankar

East Tennessee State University, Johnson City, TN

Case report

A 65-year-old reported gradually enlarging right parotid mass over 2 years. Superficial parotidectomy with facial nerve dissection revealed high-grade poorly differentiated salivary ductal gland carcinoma measuring 2.8 cm with positive margins. Pathology was positive for androgen receptor and negative for HER2 Neu. He was staged as T2 NX M0. He completed adjuvant concurrent chemo radiation with carboplatin and paclitaxel. A subsequent PET-CT showed no evidence of loco-regional or distant metastatic disease. He is currently on androgen deprivation therapy(ADT) with Leuprolide with additional Bicalutamide as total androgen blockade as a one-year adjuvant therapy plan. Salivary duct carcinoma (SDC) is a rare cancer accounting for 0.3% of all cancers and 6% of head and neck cancers in the United States. Salivary gland malignancy is a heterogeneous histological group. Androgen receptor overexpression has recently been linked with several salivary gland malignancies and especially SDC (66%–98% per some case series). HER2 (Human epidermal growth factor receptor) overexpression and NTRK (Neurotrophic receptor kinase) mutation testing would complete work up for SDC. Surgery is the primary modality of treatment with radiotherapy as an adjunct modality. Systemic therapy is usually reserved for palliation in metastatic disease with modest benefit if at all with a variety of agents that have been attempted including platinum agents, cyclophosphamide, Adriamycin and vinca alkaloids. ADT and Androgen blockade has been tested in the metastatic setting with a 50% clinical benefit ratio (18% partial response and 32% stable disease) and improved overall survival data. While this adjuvant approach is being considered in clinical trials for high-risk patient, established guidelines are lacking. We present here a rare case of high risk SDC receiving adjuvant ADT. Our patient highlights a tissue agnostic approach to cancer therapy akin to Immunotherapy in MSI (micro satellite instability) positive cancers irrespective of the tissue of origin.

214 Sclerosing epithelioid fibrosarcoma: how genetics changes cancer

L Mitchell*

A Schuyler

A Wadhwa

UAB, Fultondale, AL

Case report

Case 1: A 5-year-old male was found to have a fracture of the right humerus through a unicameral bone cyst. After multiple fractures, a biopsy was performed and a diagnosis of osteosarcoma was made. Upon surgical resection, the tumor was found to have 20–30% necrosis after pre-surgical chemotherapy. Genetic sequencing was pursued and demonstrated EWSR1-CREB3L1 fusion, a characteristic finding in sclerosing epithelioid fibrosarcoma (SEF). The tumor was also negative for SATB2, an osteoblastic transcription factor, commonly found in osteosarcoma. With no standard therapy for SEF, the family opted not to pursue additional treatment since the patient was without active disease.

Case 2: A 4-year-old male presented with a right distal femur fracture through an expansile lesion. Further imaging showed a large left renal mass and metastatic disease to lungs and bones. Patient was unable to be diagnosed by pathology and treatment was started based on presumptive diagnosis of clear cell sarcoma of kidney. After one round of treatment, patient had evidence of disease progression. Genetic sequencing of the tumor revealed EWSR1-CREB3L1 fusion and led to diagnosis of SEF and a change in treatment. He underwent chemotherapy, radiation and surgical resection of tumor sites, including the primary tumor arising from the left kidney. Patient achieved partial response after initial treatment. At follow-up scans, new metastatic disease and enhancement of bony lesions were noted. He is currently undergoing evaluation for further treatment.

Discussion

Genetic sequencing in pediatric sarcomas is useful when a diagnosis is unable to be made on pathology. Genetic sequencing of pediatric sarcomas may lead to reclassification of histologic subgroups.

215 Renal extramedullary hematopoiesis mimicking lymphoma in appearance

S Mittal*

R D’Cunha

M Zitun

L Tijani

N DCunha

M Quirch

Texas Tech University Health Science Center, Lubbock, TX

Case report

Extramedullary hematopoiesis is an unfortunate complication of some myeloproliferative neoplasms. Patients with polycythemia vera and essential thrombocytosis (ET) can progress and develop a form of myelofibrosis. As the disease worsens, patients may experience worsening symptoms from anemia, thrombocytopenia, and splenomegaly. However, extramedullary hematopoiesis is rarely seen in sites beyond the liver and spleen. We present a rare case of renal extramedullary hematopoiesis presenting with urinary obstruction in an elderly male with prior ET.

Case presentation

A 72-year old Caucasian male with prior diagnosis of ET treated with Agrylin presented to the ED for generalized weakness, 20lbs weight loss from early satiety, and anasarca. Initial workup found anemia with acute renal failure, and CT scans of the abdomen indicated hydronephrosis. Further investigation with an MRI of the abdomen demonstrated hepatomegaly, splenomegaly, and bilateral renal masses responsible for the hydronephrosis and concerning for lymphoma by radiologic appearance.

Placement of bilateral nephrostomy tubes relieved the outlet obstruction, and biopsy of the masses was initially recommended for further evaluation. However, considering the patient’s diagnosis of ET and hepatosplenomegaly, it was decided to investigate the possibility of progression from ET to myelofibrosis.

Bone marrow biopsy confirmed severe myelofibrosis. Sulfur colloid scan of the abdomen indicated extramedullary hematopoiesis in the liver, spleen, and renal masses. Biopsy of the renal masses was not pursued, and the patient was placed on Ruxolitinib to treat symptomatic myelofibrosis.

Discussion

Extramedullary hematopoiesis in hematologic disorders can present in a variety of ways, such as early satiety in splenomegaly. However, renal manifestations of extramedullary hematopoiesis are not well discussed. Biopsy of renal masses would typically be pursued, however, the complications of biopsy are numerous, with severe morbidity and mortality resulting from bleeding. This patient with essential thrombocytosis with progression to myelofibrosis demonstrates the importance of consideration for extramedullary hematopoiesis in atypical locations for patients with myeloproliferative neoplasms.

216 Atypical sarcoidosis in an atypical patient demographic

T Modi*

R D’Cunha

M Quirch

S Rehman

Texas Tech Health Sciences Center, Lubbock, TX

Case report

The most common presentation of sarcoidosis involves bilateral hilar lymphadenopathy on CXR, with non-caseating granulomas on biopsy that stain negative for AFB/GMS in a young, African American adult who clinically presents with cough and dyspnea. Sarcoidosis, however, can also present with extrapulmonary manifestations. We present a rare case of osseous and hepatic sarcoidosis with skin manifestations in an elderly Caucasian patient.

Case presentation

A 74-year-old Caucasian female, who lives in Arizona, presented to the ED in Texas for a closed intertrochanteric fracture of the right femur after a ground-level fall. X-rays showed multiple lytic lesions in the skull, thoracic spine, and lumbar spine. Workup revealed lucent liver lesions, pelvic mass, and lymphadenopathy in the mediastinum, retroperitoneum, and pelvis.

Multiple myeloma was suspected, but bone marrow and liver biopsy were negative for malignancy. Two months prior to hospitalization, she developed a posterior scalp rash with hair loss, which was being treated as psoriasis. She also noted recent onset bilateral lower extremity neuropathy. She has otherwise been hemodynamically stable, afebrile, and without leukocytosis throughout hospitalization.

The patient tested negative for HIV, hepatitis panel, syphilis, TB, and ANA. LDH, and B12 levels were within normal limits. Coccidioides, Brucella IgG/M results are pending. AFB/GMS stains of biopsies were negative making fungal etiology unlikely. Procalcitonin was elevated at 0.34 ng/mL, ESR was elevated at 104 mm/hr., CRP was elevated at 3.5 mg/L and ACE levels were elevated at 57 nmol/mL/min. 25-hydroxyvitamin D level was low at 9 ng/mL. Calcium level was high-normal at 10.4 mg/dL. As all other workups were negative, sarcoidosis was suspected. Dermatologic pathology conducted showed non-caseating granulomas in her scalp skin. Liver biopsy and bone marrow biopsy conducted also revealed non-caseating granulomas.

Discussion

Sarcoidosis is a diagnosis of exclusion that must be kept in a differential, particularly when presented with atypical features in an atypical patient demographic. This patient with disseminated sarcoidosis is being treated with methotrexate and hydroxychloroquine. Disease progression will continue to be monitored clinically and radiographically.

217 A case of cutaneous ewing sarcoma

LD Murphy1*

G Orman1

J Gardner1

DP Douglass1,2

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

2Arkansas Children’s Hospital, Little Rock, AR

Case report

Ewing sarcoma is a rare solid tumor with a pediatric annual incidence of 250. Approximately 85% of cases arise from bone and 15% from soft tissue. Cutaneous Ewing sarcoma is exceedingly rare. There is a paucity of research and thus a lack of consensus on the treatment of this form of the disease.

Case presentation

A 17 year old female presented with a 2 cm palpable, soft, reddish-blue nodule on her anterior left thigh that had been growing slowly over four years. The lesion was originally diagnosed as a varicose vein. Initial ultrasound showed a solid, vascular mass. MRI revealed a 2.5 × 1.5 × 2.4 cm, well-circumscribed, cystic cutaneous/subcutaneous lesion thought to be compatible with a hemangioma. The mass was surgically excised and pathologic investigation yielded markers positive for CD99. RT-PCR was positive for EWSR1-FLI1 translocation, confirming the diagnosis of Ewing sarcoma. Initial margins were negative, but narrow, so the patient underwent a wider excision with satisfactory margins. Metastatic work-up was negative.

Discussion

Cutaneous Ewing sarcoma is rare and there is a lack of clinical consensus on appropriate treatment. Given a few reported cases of relapses and fatalities, some pediatric oncologists recommend a full course of 28 weeks of chemotherapy. Other oncologists feel that the indolent course and overall favorable outcomes justify a shortened 12 week course of chemotherapy. After a literature review and discussions with the patient, her family and other experts who have treated cutaneous Ewing sarcoma, it was decided to treat her with a shortened course of chemotherapy consisting of 12 weeks of induction therapy following COG AEWS 1221 protocol, but not the 16 weeks of consolidation. Sequencing of the patient’s tumor DNA is underway to look for a unique genetic fingerprint that might distinguish cutaneous Ewing sarcoma and possibly explain its more indolent course.

218 Waldenström macroglobulinemia: an unusual cause of severe immune thrombocytopenic purpura

M Oye*

N Krishnan

B Patel

P Reddy

UF Health, Jacksonville, FL

Case report

A 64-year-old male with no significant past medical history presented to the hospital with a two-month history of progressive sweats, weight loss, and malaise. Vital Signs on arrival revealed Blood Pressure 139/84 mm/Hg, Pulse 72 beats/min, Temperature 98.3F. Physical Examination was significant for extensive ecchymosis and petechiae on the lower extremities, with no signs of lymphadenopathy or splenomegaly. On arrival his CBC was significant for Hgb of 6.0, g/dL and platelet count of 7,000/uL. Hemolytic Anemia was ruled out, and Hemoglobin remained stable after one unit of packed red blood cells was transfused. Infectious workup was initiated and was negative for HIV and Hepatitis. Autoimmune workup for Systemic Lupus Erythematous was also negative. He was not taking any prescribed medications outpatient, and his urine drug screen was unremarkable. The patient was administered high-dose IV corticosteroids, Intravenous Immunoglobulin, and platelet transfusion however thrombocytopenia failed to resolve. Due to his poor response to treatment a bone marrow aspirate and biopsy was performed. Marrow was consistent with Lymphoplasmacytic Lymphoma/Waldenström macroglobulinemia, with adequate megakaryocytes. CT Chest, abdomen, and pelvis with IV contrast were negative for lymphadenopathy or extramedullary involvement. Decision was made to start treatment with Rituximab as this would treat both his low-grade lymphoma and his Immune Thrombocytopenia. With the initiation of Rituximab his platelets increased, and his constitutional symptoms resolved. Prolonged non-steroid dependent remission was induced, and on follow-up patient is doing well and back to performing his daily activities with no limitations.

Discussion

Waldenström macroglobulinemia (WM) is a rare lymphoproliferative disorder characterized by the presence of lymphoplasmacytic cells in the bone marrow and IgM monoclonal protein in the serum. Immune thrombocytopenic purpura is a rare manifestation of Waldenström macroglobulinemia and is seen in fewer than 5% of patients with WM. When ITP is diagnosed in the setting of a B-cell malignant disorder, treatment of the underlying disease is often more beneficial than standard regimens for idiopathic ITP.

219 Malignancy-associated sweet’s syndrome mimicking cellulitis

M Oye*

U Wardan

N Krishnan

W Aung

UF Health, Jacksonville, FL

Case report

A 56-year-old male with no past medical history presented with fever and painful skin lesions. One week prior he presented to an urgent care clinic with the same issue and was prescribed Clindamycin for suspected cellulitis. He finished the treatment without any improvement of symptoms. Vital signs on arrival revealed that he was febrile to 38.7°C with tachycardia at 102 bpm. Skin exam revealed a 6.5 cm × 2.5 cm raised, tender, erythematous, and edematous plaque on the left side of his neck, a 3.5 cm circular erythematous lesion on the left elbow, as well as an erythematous papule on his left leg. In setting of multiple skin lesions concerns for sepsis secondary to bacterial skin infection arose. He was started on IV Vancomycin and Cefepime. Infectious workup returned with unremarkable results. Lesions failed to improve despite being on broad spectrum antibiotics. Labs revealed leukocytosis, macrocytic anemia, and thrombocytopenia. CBC differential showed 21% immature mononuclear cells. Bone marrow biopsy revealed Acute Myeloid Leukemia. Skin biopsy of lesions demonstrated dense neutrophilic infiltrates in the dermis without vasculitis, suggestive of Sweet’s syndrome. He was promptly started on chemotherapy for leukemia. Corticosteroids were initiated for treatment of the skin lesions.

Discussion

Sweet’s syndrome, also known as acute febrile neutrophilic dermatosis, is characterized by fever, leukocytosis, characteristic skin lesions, and dense dermal infiltrate on histology. Skin lesions are painful, red, edematous papules or plaques usually present on the upper extremities, face, or neck. Etiologies of Sweet’s syndrome include malignancy, drug-induced or idiopathic. The characteristic lesions seen in Sweet’s syndrome can be the presenting sign in malignancy as seen in our patient. Skin biopsy is essential in making the diagnosis, and corticosteroids are used to treat the lesions.

Conclusion

Malignancy-associated Sweet’s syndrome is an uncommon clinical entity. It is imperative to have a high index of suspicion for Sweet’s syndrome in patients with unresolving cutaneous lesions, and to search for an underlying cause. This can lead to earlier diagnosis of malignancy and prompter initiation of chemotherapy.

220 The clinical conundrum of intravascular large cell lymphoma

Y Pandey*

D Middleton

A Baltz

A Roy

A Godbole

M Veeraputhiran

University of Arkansas for Medical Sciences, Little Rock, AR

Case report

(ILCL) is a rare type of large cell lymphoma characterized by the proliferation of lymphoma cells within the lumen of small blood vessels without an obvious extravascular tumor mass. It tends to have an aggressive course often with poor outcomes with a majority of cases recognized only at autopsy. We present two unique cases of ILCL.

Case 1: A 60-year-old male was admitted for a 4-month history of dry cough, malaise, intermittent fever, night sweats, and unintentional weight loss. The patient was febrile, ill-appearing and icteric on examination. Initial blood work-up showed anemia and elevated liver enzymes. The infectious work-up including bacterial and fungal cultures, viral panel, tick-borne illness serology, and HIV serology was negative. The bone marrow exam was consistent with ILCL. The patient received rituximab combined with gemcitabine and oxaliplatin. The hospital course was complicated by tumor lysis syndrome and sepsis. The patient‘s clinical course continued to deteriorate with hypotension and acute renal failure despite appropriate antibiotics. The patient pursued comfort care and passed away shortly in the hospital.

Case 2: A 71-year-old male was admitted for further evaluation of a 2-month history of worsening shortness of breath, dry cough, generalized fatigue, and weight loss. On initial evaluation, the patient was found to have hemolytic anemia, splenomegaly, and lactic acidosis without clear etiology. The patient underwent extensive infectious and serological workup which was all normal. Bone marrow biopsy showed hypercellularity. Various imaging modalities including Positron Emission Tomography-Computed Tomography (PET-CT) of the vertex to feet were performed which showed non-specific findings. The patient continued to deteriorate despite supportive treatment. The patient pursued comfort care and passed away shortly in the hospital. The autopsy showed intravascular lymphoma cells within small vascular spaces of most vital organs.

Conclusion

ILCL is an aggressive subtype of lymphoma which presents with atypical clinical features leading to delayed diagnosis and often resulting in poor outcomes. A high degree of clinical suspicion is the key to prompt diagnosis and improved outcomes.

221 Rare case of vision loss in a patient with waldenstrom macroglobulinemia

NB Patel*

C Bigelow

V Herrin

University of Mississippi Medical Center, Madison, MS

Case report

Waldenstrom Macroglobulinemia (WM) is a rare hematological disorder characterized by the presence of monoclonal IgM gammopathy in the blood and clonal lymphoplasmacytic cells in the bone marrow. Clinical presentation is linked to the IgM monoclonal protein the blood (hyper-viscosity, cryoglobulinemia, bleeding disorders, and autoimmune hemolytic anemia), marrow or tissue involvement (anemia, hepatosplenomegaly, or lymphadenopathy), or autoimmune disorders (neuropathy). We present a rare case of vision loss due to cancer associated retinopathy in patient with newly diagnosed WM.

This is a 78-year-old Asian male with history of Hypertension, Type II Diabetes Mellitus, and Chronic Obstructive Pulmonary Disease who presented with five-month history of vision changes. His symptoms initially started with floaters that progressed to visual loss in a few months. He was evaluated by multiple ophthalmologist without any definitive findings. He had associated symptoms of dyspnea on exertion, cough, epistaxis, weight loss, and night sweats. Patient had Computed Tomography imaging of chest, abdomen, and pelvis without suspicious adenopathy or hepatosplenomegaly. He was evaluated by ophthalmology at our institution and lab work at that time was significant for elevated IgM level at 2117 mg/dl and normal serum viscosity. Cancer Associated Retinopathy (CAR) Panel returned positive for anti-Aldolase, anti-Enolase, and anti- glyceraldehyde 3-phosphate dehydrogenase (GAPDH). Patient was evaluated by hematology and admitted for therapeutic plasma exchange. Patient vision improved after five treatments of plasma exchange and completion of weekly rituximab. Currently patient is on ibrutinib for treatment for his WM with stable disease and no further vision changes.

CAR is rare in patients with WM In CAR, retinal degeneration occurs in presence of auto-antibodies that cross react with normal retinal tissue. The vision loss can proceed the diagnosis of the underlying illness. Long term immunosuppression and treatment of the underlying disease in the mainstay of treatment for CAR. Many different immunosuppressive combinations (high dose steroids, intravenous immunoglobulins, plasma exchange) have been used with varying results alongside treating the underlying disease.

222 Identifying hereditary breast cancer (HBC): a case of LI-fraumeni syndrome (LFS)

C Pham*

A D’Angelo

A Garcia

LSU Health Sciences Center, New Orleans, LA

Case report

LFS case report and increase HBC awareness.

Methods

Review of Electronic Health Records and medical literature.

Results

A 28-year-old female presented with a left breast mass. Workup, including bilateral breast MRI, revealed masses in both breasts. Biopsies showed ductal carcinoma in situ in the left breast and triple-negative invasive ductal carcinoma in the right. Significant maternal family history (FH) revealed uterine cancer at 40 in an aunt and breast cancer (BC) at 63 in a first cousin once removed. The patient received genetic counseling and panel germline testing. A mutation was found in TP53 (c.1025G>C), confirming a diagnosis of LFS. She was treated with neoadjuvant chemotherapy and bilateral mastectomies with reconstruction.

Approximately 5–10% of BC is due to hereditary causes. Identification of patients with HBC is crucial as there are implications for the patient’s and family members’ management. Clinicians should be aware of the genetic referral/testing criteria, including the NCCN criteria. Suspicious personal/family histories include early-onset, triple-negative, bilateral or male BC, and ovarian, pancreatic and high-risk/metastatic prostate cancers.

Clinicians are aware of the role of BRCA1/2 mutations in patient care but may not be as familiar with other HBC syndromes. LFS is a rare autosomal dominant disorder that is caused by mutations in the tumor suppressor gene, TP53. The core cancers seen in LFS are sarcomas, brain tumors, adrenocortical carcinoma, leukemia, and extremely early-onset BC. The overall lifetime risk of cancer is over 90% in females and 75% in males. Cancer surveillance recommendations include whole-body MRI, colonoscopy and skin exam. Radiation therapy and X-ray diagnostic imaging should be avoided.

Conclusions

This case highlights the importance of HBC knowledge beyond BRCA1/2 and is particularly interesting as testing confirmed the TP53 mutation was inherited from the patient’s father, who is in his 60s with no personal or FH of cancer. This is unusual for an individual with LFS and highlights that family history alone may not identify all candidates with HBC. The patient’s daughter tested positive for the familial TP53 mutation. She is now undergoing surveillance protocols that would not have been available without the genetic diagnosis.

223 Acquired combined factor VIII & XI deficiency: an uncommon hemophylia presentation

J Rivas Rios*

J Lung

A Rivas

W Aung

M Shaikh

University of Florida, Jacksonville, FL

Case report

An 80 year old male with past medical history of diabetes mellitus, hypertension, atrial fibrillation, history of head trauma and dementia was brought to the emergency room with altered mental status after having a mechanical fall. He did not have prior history of major bleeding or abnormal coagulation tests in the past. On examination, he was found to have multiple body bruises with an expanding left pectoral hematoma that lead to a significant drop in hemoglobin requiring multiple transfusions. He underwent an angiography of the left shoulder with embolization of the lateral thoracic artery with continued bleeding from the catheter insertion site after the procedure. Further work up found a prolonged Partial Thromboplastin Time (PTT). A thromboelastographic study reported elevated R time and K time reflecting a difficulty to form the clot. Mixed studies did not correct PTT immediately and after incubation suggesting factor inhibitors. A Bethesda assay result above 60 units representing very high levels of a factor VIII inhibitor. Blood clotting factor activity levels showed a deficiency in Factor VIII and XI. He received factor VIIa, Aminocaproic acid, IVIG, and steroids. Further work up for malignancies and autoimmune disorder was negative. His bleeding stopped, hemoglobin stabilized and prolonged PPT improved. Rituximab and Cyclophosphamide were not indicated due to clinical improvement.

Discussion

Acquired factor deficiencies are a result of autoantibodies targeting factors involved in coagulation associate with active malignancies or autoimmune disorders, but seldom do they arise spontaneously. The reasons to produce these autoantibodies are not clear but may involve the presence of certain gene polymorphisms and/or autoreactive CD4+ T lymphocytes. Our case involves multiple factor deficiencies, presenting with severe blood loss in a patient previously having normal coagulation studies. It is possible that the patient had two inhibitors, or one inhibitor binding to both factor VIII and XI. Two other possibilities include excess factor consumption to stop excess bleeding and dilutional coagulopathy from multiple blood transfusions without fresh frozen plasma.

224 A sailor’s cure for petechiae

K Rogers*

ME Littrell

JM Mack

University of Arkansas for Medical Sciences, Little Rock, AR

Case report

To report a case of a 12-year-old female with acute lymphoblastic leukemia who presented with scattered petechiae on her extremities and found to have scurvy.

Methods used

Chart review

Summary

Patient is 12-year old female with acute lymphoblastic leukemia who presented to the emergency room with petechiae and foot pain. She had petechiae on her extremities and was previously asymptomatic. She had an injury to her right foot when a door fell on it two weeks prior but showed no evidence of fracture. She has fatigue, arthralgias, joint swelling, easy bruising, bleeding, osteopenia, and neuropathy. For the past year, she has had a poor diet consisting of junk foods.

On exam, she had severe petechiae on bilateral upper and lower extremities. She had swelling to her right ankle with decreased range of motion due to pain and hypersensitivity greater on the right foot than the left. Her hemoglobin was 9.1 and platelet count of 81,000. Both remained stable throughout her admission. Her PT was 12, PTT was 28.6, and fibrinogen was 286.7. Because of the petechiae that was out of proportion to her level of thrombocytopenia, a vitamin C level was obtained and was <5 umol/L. A previous bone density test showed low bone mineral density less than two standard deviations below the mean for age.

Vitamin C is a required in making collagen, an important component of connective tissues that are essential in providing structure and support to blood vessels. Because of patient’s poor nutrition, severe vitamin C deficiency, fatigue, neuropathy, arthralgias, osteopenia and depression, she was diagnosed with scurvy. The patient was treated with 200 mg of ascorbic acid daily for 2 weeks and then decreased to 100 mg daily for at least a month. Her petechiae resolved within a week and a repeat vitamin C level 2 weeks later was 32 umol/L.

Conclusion

There are several causes for the development of petechiae in addition to just thrombocytopenia. Vitamin C deficiency should be explored when petechiae but not significant thrombocytopenia is present. Given the rare occurrence of scurvy today in the US, it’s also important to be aware that it still does occur especially in pediatric populations that are at risk for poor diet or that have chronic malnutrition.

225 Undiagnosed acquired hemophilia a- culprit for recurrent gastrointestinal bleeding

A Roy*

A Ananthula

Y Pandey

A Goel

M Joseph

A Venkata

University of Arkansas for Medical Sciences, Little Rock, AR

Case report

Acquired Hemophilia A (AHA) is a rare bleeding disorder caused by autoantibodies against clotting factor VIII (FVIII) which predisposes to life-threatening hemorrhage. Unlike joint bleeds that characterize congenital hemophilia A, AHA manifests as spontaneous hematomas and extensive bruising, very rarely gastrointestinal bleeding (GIB), and intracranial hemorrhage may occur. We present a unique case of AHA which presented as recurrent GIB and was undiagnosed for a year.

Case

74-year-old female with Myasthenia Gravis, small bowel perforation requiring bowel resection and anastomosis admitted for melena with a hemoglobin of 5.8. She had a history of GIB from Mallory Weiss tear and esophagitis. She had multiple admissions for anemia which were treated with blood transfusions and endoscopies which revealed no bleeding sources. During the admission for melena, tagged red blood cell scan, CT angiogram (CTA), capsule endoscopy showed oozing from the bowel anastomotic area. Surgical exploration was deferred given her multiple comorbidities. Coagulation studies performed in the setting of repeated bleeding showed isolated activated partial thromboplastin time (APTT) prolongation which was not corrected by mixing study. Her FVIII activity was markedly reduced to <1% and Factor IX activity was normal. Fibrinogen, lupus anticoagulant, malignancy workup, hepatitis panel were normal. Bethesda titer showed elevated inhibitor levels at 91 Units. Steroids and rituximab were given and discharged with a plan of weekly rituximab for four weeks. She was readmitted with spontaneous chest wall hematoma. CTA showed large pectoral hematoma with active extravasation. Factor Eight Inhibitor Bypassing Agent (FEIBA), recombinant Factor VIIa and steroids were given to control active bleeding. Her clinical condition worsened, and she passed away. Intracranial hemorrhage was considered as a possible cause for her sudden death although an autopsy was not performed.

Conclusion

Coagulation studies should be considered when patients with autoimmune disease present with any bleeding. Prompt diagnosis and treatment are vital which includes controlling bleeding and eradication of inhibitors, as it can lead to fatal hemorrhages.

226 Is this working well for pediatrics? Diagnosis and treatment of thromboembolic disease in a pediatric patient with protein C deficiency

R Samuel1*

A Dixon1

K Paige1

MB Fletcher2

R Warrier2

1Tulane University School of Medicine, New Orleans, LA

2Ochsner Hospital for Children, Jefferson, LA

Case report

In the pediatric population, a pulmonary embolism is a medical emergency. Currently there are neither evidence based screening tools for diagnosis nor oral anticoagulants for treatment.

Our patient is an 18-year-old female with a complicated medical history including seizure disorder and protein C deficiency. She presented with acute shortness of breath and right hip pain without any recent trauma, surgery, or prolonged immobility.

She was tachycardic with otherwise stable vital signs. Laboratory values were within normal limits apart from a D-dimer elevated to 10.3. The Wells Score for DVT was 4 (high risk). A lower-extremity venous ultrasound showed occlusive thrombus involving the right common iliac, common femoral, femoral, and popliteal veins. The Wells Score for pulmonary embolism (PE) was 9 (high risk). Chest CT demonstrated multiple subsegmental pulmonary emboli. An echocardiogram demonstrated mild tricuspid valve insufficiency without right heart strain. The patient was started on a Lovenox drip and admitted to the Pediatric Intensive Care Unit. She underwent rheolytic aspiration thrombectomy and catheter placement for site-directed thrombolysis. She was started on a TPA drip for one day before another thrombectomy and venoplasty. Per pediatric hematology recommendations she was discharged on Rivaroxaban 15 mg twice a day for 21 days, then 20 mg daily for 3 months minimum.

The events of this case are notable for a genetic coagulopathy, DVT/PE development while taking aspirin, and the lack of guidelines in pediatric PE diagnosis and treatment. While the Wells criteria can be applied to pediatric cases, its low specificity and moderate sensitivity for the pediatric population should be taken into account. Per recommendations by the American Heart Association and American College of Chest Physicians, provoked PEs and DVTs should be treated with low molecular weight heparin. However, long term use is not advised. With prominent side effects of traditionally used medications more studies are needed to determine the long term efficacy of direct-acting oral anticoagulants in children, especially in the case of coagulopathy.

227 The natural killer

J Schaub*

V Herrin

University of Mississippi Medical Center, Jackson, MS

Case report

Peripheral T-cell lymphomas comprise a heterogenous group of uncommon non-Hodgkin Lymphomas in adults. While they are rarely encountered in clinical practice, here we present a unique case of Extranodal natural killer/T-cell lymphoma, nasal type (ENKL) in someone without significant risk factors for the disease. A 58-year-old white male presented to an ENT physician for chronic nasal congestion and difficulty breathing. Review of systems were otherwise unremarkable and underwent nasal endoscopy and biopsy that came back as ENKL, nasal type. He then underwent staging PET-CT and bone marrow biopsy/aspirate that were negative for extensive disease involvement of lymphoma. He then underwent chemotherapy treatment (SMILE) with subsequent radiation therapy to the area of the mass. ENKL is typically located in the nasopharynx, but the non-nasal type can be found in other extranodal locations such as the skin or gastrointestinal tract. It is typically associated with the Epstein-Barr Virus, as well as having a strong association with those of Asian and South American ancestry. Additionally, it tends to favor men in the 5th-6th decades of age. Interestingly, he does not have Asian ancestry, but did live in Japan for seven years as a child. Localized disease typically presents with obstructive nasal symptoms or epistaxis. Although much less common, advanced stage disease can present with bone marrow involvement or B-symptoms of fever and weight loss. Definitive diagnosis is obtained by pathology from a lymph node at the site of suspected involvement with flow cytometry consistent with Natural Killer cell origin that express CD2, cCD3 and CD56. A majority of patients with localized disease have improved significant response rates to a combined chemotherapy and radiation therapy approach, while extensive stage disease is treated with chemotherapy regimens only. Growing evidence is in support for the SMILE regimen (dexamethasone, methotrexate, ifosfamide, l-asparaginase, and etoposide) for treatment of this aggressive neoplasm with improved overall response rate. Extranodal natural killer/T cell lymphoma is a rare malignancy in the United States. However, due to its progressive course and harsh chemotherapy regimens, it is critical to consider this pathology early on in those with obstructing nasal tumors.

228 Rattlesnake envenomation causing recurrent coagulopathy

J Sekhon*

B Rogers

A Wichmann

D Vangipuram

K Arthur

S Olayiwola

Texas Tech Health Sciences Center, Lubbock, TX

Case report

Crotalinae (rattlesnakes, pygmy rattle snakes, moccasins) bite envenomation is a significant problem in the United States which can result in systemic coagulopathy. Prompt use of antivenom can correct resulting laboratory abnormalities, however despite antivenom use coagulopathy may recur, persist, or may even result in death after a latency period.

Case description

79 year old male with no significant past medical history presented as a transfer to the burn intensive care unit after a rattle snake bite to the left hand, complicated by severe nausea, vomiting, and diarrhea along with left hand erythema and swelling extending to mid forearm. On admission, he was found to have low fibrinogen levels and an elevated D-Dimer, was given bolus dose of CroFab with subsequent maintenance doses; this resulted in marked improvement within two days. However, over the subsequent three weeks, he continued to have refractory coagulopathy noted on outpatient follow up requiring readmissions and multiple additional doses of CroFab. The coagulopathy resolved after total 38 vials of CroFab and cryoprecipitate.

Discussion

Snakebite envenomation is not uncommon in the United States with an annual incidence of approximately 7,000. In the past duration of coagulopathy after snakebite has been considered short-lived and patients were discharged after initial correction of coagulopathy. However, envenomation may result in latent venom release from soft tissue depots that can last up to two weeks. This case demonstrates the importance of close hemodynamic monitoring and laboratory monitoring after snakebites and describes the importance of prompt and appropriate use of antivenom to neutralize latent venom release and correct residual coagulopathy.

229 Nasal cavity plasmacytoma in multiple myeloma

P Sharma*

S Manthri

D Jaishankar

D Krishnan

East Tennessee State University, Johnson City, TN

Case report

Multiple myeloma is a neoplastic proliferation of plasma cells producing monoclonal proteins. Symptoms and signs of multiple myeloma are usually due to infiltration of plasma cells into the bone or other organs, and can manifest as anemia, acute kidney injury, hypercalcemia and bone pain with osteolytic lesions. Extramedullary plasmacytomas (EMP) may present at the time of diagnosis, or will develop later in the disease indicating relapsed/refractory disease. The extramedullary plasmacytomas most often involve the upper aerodigestive tract – including oro-nasopharynx and paranasal sinuses

Case presentation

A 58 year old Caucasian male patient with known history of multiple myeloma diagnosed 6 months ago, with lambda-light chain restriction on bone marrow biopsy with complex cytogenetics and ISS - Stage III with high risk features and multiple osteolytic lesions presented with uncontrolled epistaxis for past several weeks. On examination, patient was found to have a large pale mass filling the left nasal cavity, tender to manipulation. CT sinuses showed soft tissue mass in the anterior aspect of the left nasal passageway, along with mucosal thickening of the left maxillary sinus and inferior aspect of left frontal sinus. Patient underwent left-sided image-guided endoscopic sinus surgery along with excision of left intra-nasal mass. Biopsy of the mass was positive for malignant sheets of markedly pleomorphic lymphoid cells with prominent nucleoli with lambda-light chain restriction consistent with plasmacytoma.

Conclusion

The incidence of EMP is 7 to 17% at the time of diagnosis and around 20% at the time of relapse1 Possible mechanisms of extramedullary spread include decreased adhesion molecule expression and downregulation of chemokine receptors. EMPs usually arise from local growth of soft tissue masses from focal bone involvement, sometimes it can also occur via hematogenous spread. It is unclear if presence of high risk cytogenetics is associated with increased incidence of EMPs. The incidence of EMPs in patients with MM is high and is associated with poor outcome in patients treated conventionally.

230 ‘I am a non-bleeder’ extreme ptt prolongation sans bleeding – a classic case of factor XII deficiency

P Sharma*

S Manthri

D Krishnan

D Jaishankar

East Tennessee State University, Johnson City, TN

Case report

Factor XII (Hageman factor) plays a key role in activation of intrinsic coagulation system gauged by the activated partial thromboplastin time (aPTT). Factor XII deficiency is often an autosomal recessive condition, but autosomal dominant inheritance is also reported. Literature reports a possible 2% prevalence in the community but is difficult to ascertain as patients are often asymptomatic. We present a rare case of an incidentally found severe Factor XII deficiency during pre-operative work-up. Afifty-seven-year-old Caucasian male presented with atypical chest pain and work up revealed mediastinal lymphadenopathy. Patient underwent bronchoscopy, endo-bronchial ultrasound and right carinal lymph node aspiration. Cytology was positive for necrosis with atypical cells. PET scan showed increased uptake in the sub-carinal and mid para-esophageal lymph nodes, low-grade uptake in right lower paratracheal and mesenteric lymphadenopathy. During pre-operative work-up for mediastinoscopy, patient was found to have a very prolonged partial thromboplastin time (PTT) of 112. Repeat testing was still high - PTT 189, with normal prothrombin time on both the occasions. Patient had normal platelet count and normal thrombin time. He had no history of bleeding diathesis and had no bleeding complications with multiple previous surgeries. Mixing study showed complete correction of PTT-32, consistent with a factor deficiency affecting the intrinsic (PTT) pathway. Factor level VIII, IX and XI were normal, however Factor XII activity was markedly low at 1%. Patient was diagnosed with factor XII deficiency which can cause abnormally prolonged PTT. As Factor XII deficiency carries no risk of clinically significant bleeding, patient was cleared for mediastinoscopy and underwent the procedure successfully with no complications. Factor XII deficiency is commonly diagnosed incidentally with abnormally prolonged aPTT. Patients usually do not exhibit a bleeding diathesis. Homozygous patients have non-detectable levels of factor XII, while heterozygous individuals have variable levels ranging from 20–60%. It could be associated with paradoxical fatal thromboembolic complications including myocardial infarction and pulmonary emboli.

231 Hemophagocytic lymphohistiocytosis in a patient presenting with gastrointestinal symptoms

AS Stack*

M Chahin

University of Florida Jacksonville, Jacksonville, FL

Case report

A 45-year-old Hispanic female with a history of uncontrolled diabetes mellitus presented with five days of bilateral lower abdominal pain with radiation to lower back. She also complained of fever, myalgia, and a history of joint pain and rash. Her aspartate aminotransferase and alanine aminotransferase were 274 IU/L and 161 IU/L, respectively. She was pancytopenic.She was started on broad spectrum antibiotics and admitted to the surgical intensive care unit due to concern for cholecystitis or hepatitis. Computed tomography of the abdomen and pelvis showed mild pericholecystic fluid and hepatosplenomegaly. Ferritin was markedly elevated to 45,765 ng/mL. Her peripheral blood smear was significant for large platelets and vacuoles with slight anisocytosis. She continued to be tachycardic and leukopenic. Infectious workup was negative and broad spectrum antibiotics were stopped. No monoclonal paraproteins were detected. EBV testing suggested convalescence or a previous infection. Her H-score, for diagnosing reactive hemophagocytic syndrome, was 293 points, with >99% probability for diagnosis. She was started on a long term taper of steroids.

Discussion

Hemophagocytic lymphohistiocytosis (HLH) is a progressive, life-threatening syndrome of exaggerated immune response resulting from hyperinflated and inefficient activation of macrophages and lymphocytes. Primary HLH generally appears in childhood and is typically caused by genetic defects causing T and NK cell dysfunction. Secondary HLH occurs in patients without known familial mutation, typically in the setting of lymphoma, autoimmune disease, or viral infection. Most cases of viral-associated HLH are due to Epstein-Barr, Cytomegalovirus, and herpes virus infection. Patients often present with a wide variety of clinical manifestations and may quickly deteriorate. Treatment involves managing the underlying cause and administering immunomodulatory and immunosuppressive therapy. As mortality is high in patients left untreated, prompt diagnosis and initiation of treatment are vital in increasing chances of survival in these patients. We report a case of HLH associated with Epstein-Barr virus in a patient presenting with gastrointestinal symptoms.

232 Extraosseous manifestation of multiple myeloma: an unusual presentation

PR Treutel*

V Herrin

C Milner

University of Mississippi Medical Center, Jackson, MS

Case report

We present a 61-year-old African-American female with a past medical history of pulmonary embolism and IgG Kappa multiple myeloma diagnosed nine years prior who was admitted to the hospital for evaluation of increased liver enzymes, bilirubin, and alkaline phosphatase. Her myeloma has followed an aggressive course, requiring multiple changes in chemotherapy and two autologous peripheral blood stem cell transplants. Two weeks prior to the current admission, she was hospitalized for altered mental status and was found to have a pseudomonas UTI. She was treated initially with cefepime, with increase in her bilirubin and liver enzymes. GI was consulted and initially felt this was secondary to a drug induced liver injury. Liver ultrasound at that time showed hepatic steatosis. Antibiotics were changed and her liver enzymes trended downward to near normal at the time of discharge. She presented to clinic for hospital follow up and was found to have marked increase of her liver tests with an obstructive pattern so she was readmitted. An ultrasound showed cholecystitis without cholelithiasis. Further evaluation with CT of the abdomen showed peribiliary enhancing soft tissue extending from the porta hepatis to the distal common bile duct with extensive common bile duct narrowing. ERCP was performed which also illustrated common bile duct narrowing; a stent was placed into the common bile duct to allow for bile flow. Biopsies of the soft tissue masses obtained during the ERCP showed plasma cell neoplasm, consistent with extraosseous myeloma. She was begun on VD-PACE (bortezomib, dexamethasone, platinum agent, doxorubicin, cyclophosphamide, etoposide) and had near resolution of the masses and normalization of her liver enzymes. Unfortunately, despite that improvement, she has developed CNS involvement requiring weekly intrathecal methotrexate. She remains hospitalized with a poor overall prognosis.

Extraosseous myeloma is not uncommon in cases of multiple myeloma, especially in cases of aggressive and refractory disease such as in this patient. Hepatic and common bile duct involvement is uncommon, though not impossible. It is important to consider extraosseous myeloma in the differential when evaluating hepatic dysfunction in patients with aggressive disease.

233 Bilateral wilms tumor in a patient with isolated hemihyperplasia

A Vasileva1*

A Cruz2

M Popescu2

1Ballad Health, Johnson City, TN

2East Tennessee State University, Johnson City, TN

Case report

Children with body asymmetry or other signs of tissue overgrowth including hemihyperplasia are at increased risk for developing embryonal tumors like Wilms tumor. Based on this association it is recommended that children with hemihypertrophy undergo screening with renal ultrasound every 3 months after birth till age of 7. Here we present a case of delayed diagnosis of Wilms Tumor in a patient with hemihyperplasia syndrome.

A 2 years old girl with no significant past medical history was evaluated by pediatrician for chief complaint of asymmetry of lower extremities. She was referred to orthopedic clinic, but no other intervention was recommended. Months later, the patient was seen in the Emergency department for fever and abdominal pain. US of the abdomen revealed bilateral kidney masses. Considering that bilateral renal tumors in pediatric population usually represent Wilms tumor, the patient received neoadjuvant vincristine, actinomycin, doxorubicin followed by bilateral partial nephrectomy. Final pathological diagnosis was stage 2 Wilms tumor on the right, with diffuse anaplasia and indeterminate stage Wilms tumor of the left kidney, favorable histology. Therefore, therapy was augmented to vincristine, doxorubicin, etoposide and cyclophosphamide, and right flank irradiation. Patient is currently disease free 3 years off therapy.

Most Wilms tumors occur in otherwise well children, but a small proportion occur in children with genetic syndromes associated with increased risk. Though hemihyperplasia can be an isolated finding, it is typical for different growth disorders. In our patient the presence of hemihypertrophy and bilateral Wilms tumors prompted genetic testing for underlying Beckwith–Wiedemann syndrome (BWS). Extensive genetic testing did not identify an inherited growth disorder, but 15–20% of individuals with classic BWS would have normal test results.

Our case serves as a great example of importance in early surveillance of children with hemihyperplasia for underlying embryonal tumors. Early detection leads to decreased treatment-related morbidity and possibly mortality.

234 Breast cancer therapy in africa is severly limited

RM Walker1*

M Morrison1

J’ Walker2,3,4

1Oklahoma Baptist University, Shawnee, OK

2Texas Tech, Amarillo, TX

3ELWA Hospital, Monrovia, Liberia

4Accolade Hospice, Amarillo, TX

Case report

Provision of health care in resource-poor environment can present unique challenges. In June 2019 in Monrovia, Liberia, West Africa, a 40 yo F presented with 4 month ho breast cancer with mets, hypoxia, bilateral pleural effusions and SOB.

Four months ago at DX, consideration was given to curative resection but effusion on CXR ruled out curative surgery. She presented w right breast mass and evidence of mets, but surgery and biopsy were not carried out due to poor prognosis. The hospital has provided chemotherapy to 3 children in the past 2 years but lacks reliable access to chemotherapy and training for adult nurses.

We attempted to obtain two drugs, but both were unavailable in Liberia. The patient asked to go to Ghana, 1000 miles away, but had no transportation. The patient continued to suffer from SOB as we found access to ‘palliative care’ unavailable as well. She was lost to follow up.

Discussion

Liberia lacks access to care in many areas, including chemotherapy and palliative care. GDP is only $460 US per capita, 4% of the world average. We were unable to find suitable chemotherapeutic meds. Furthermore, nursing staff outside of the pediatric ward did not feel comfortable giving chemotherapy. Training for nursing staff is not currently available. We also had concerns regarding our ability to monitor the patient for adverse effects from chemotherapy. Access to palliative care such as narcotic analgesic medications and home care is not available. Many people also believe that discussing death promotes death, and discussions about hospice and comfort in dying are believed to increase the likelihood of adverse outcomes.

235 A rare case of anaplastic large cell lymphoma presenting as obstructive uropathy

M Elmassry1

S Wongsaengsak1*

S El Nawaa1

AF Al Mahmoud2

M Mortagy3

K Nugent4

1Texas Tech University Health Sciences Center, Lubbock, TX

2King Faisal Specialist Hospital, Jeddah, Saudi Arabia

3New Giza University, Giza, Egypt

4TTUHSC, Lubbock, TX

Case report

Anaplastic large cell lymphoma (ALCL) constitutes about 2% of all Non-Hodgkin lymphomas (NHL). We are reporting a case of ALCL in an elderly female with no B-symptoms and no BM or extranodal involvement. NHL is a great mimicker of retroperitoneal fibrosis and can cause obstructive uropathy.

Case presentation

A 79-year-old female with no significant PMH who presented with acute bilateral flank pain. Associated with distended abdomen, frequency, urgency, and hematuria. She was hemodynamically stable. Examination showed bilateral costovertebral angle tenderness and bilateral inguinal LAD. CBC showed normocytic normochromic anemia. CMP showed AKI. LDH was elevated. UA showed hematuria. CT abdomen showed bilateral hydronephrosis with enlarged retroperitoneal bilateral pelvic and inguinal LNs. Bilateral nephrostomy tubes were inserted and switched later to ureteric stenting. Inguinal LN excisional biopsy revealed anaplastic Large T-cell NHL which was BCL-2 positive as well. Serum protein electrophoresis showed IgA-Kappa Monoclonal gammopathy. BM biopsy showed Normocellular marrow with no BM involvement by lymphoma. Renal function normalized after stent insertion and chemotherapy was started.

Discussion

Anaplastic large cell lymphoma is a very rare disease. ALCL is a bimodal disease with a median age of onset of 34 years. ALCL has a male predominance. B symptoms occur in 60% of patients. Skin is a common extranodal site sparing the BM. Our patient was an old female with neither B symptoms nor skin involvement labeling it as a very rare presentation. Patients with ALCL are candidates for upfront BM transplant after chemotherapy. However, the patient’s age and comorbidities opposed this approach. Treatment options include miniCHOP, CVP, or replacing vincristine with brentuximab and other palliative protocols. CVP was started with fair response.

Conclusion

ALCL is a rare and aggressive type of NHL that can present as obstructive uropathy which is a sign of extensive disease. It has a poor prognosis and treatment is usually challenging.

236 A stormy diagnosis of hemophagocytic lymphohistiocytosis and hodgkin lymphoma secondary to ebstein-barr virus

M Elmassry1

S Wongsaengsak1*

S El Nawaa1

AF Al Mahmoud2

M Mortagy3

K Nugent4

1Texas Tech University Health Sciences Center, Lubbock, TX

2King Faisal Specialist Hospital, Jeddah, Saudi Arabia

3New Giza University, Giza, Egypt

4TTUHSC, Lubbock, TX

Case report

Hemophagocytic Lymphohistiocytosis (HLH) is a defect in NK and cytotoxic T-cell function, resulting in excessive cytokine production and accumulation of T cells and macrophages leading to multisystem damage. We present a case of HLH with HL in the setting of EBV infection.

Case presentation

An 18-year-old man with CVID and recurrent pneumonia who presented with fever of unknown origin (FUO), night sweats and weight loss for 3 months. He was hemodynamically stable with a temperature of 102.5 F. Physical exam showed marfanoid features, differential clubbing, organomegaly with no LAD.

CBC showed pancytopenia,CMP showed transaminitis,Serum ferritin was high. EBV viral load was very high (>1,000,000 copies). Hepatitis and HIV tests were negative. All cultures were negative. Serum IL-2 (CD25) was high. CT of the abdomen and pelvis showed hepatosplenomegaly and no LAD. BM biopsy confirmed Hodgkin lymphoma. The patient was started on Dexamethasone for the HLH and (ABVD+R) regimen. He responded well with complete resolution of his symptoms.

Discussion

HLH is diagnosed with 5 out of 8 criteria: fever, splenomegaly, pancytopenia, hypertriglyceridemia, hemophagocytosis (in BM, spleen or LN biopsies), low or absent NK cell activity, high serum ferritin and high IL-2 (CD25). Our patient met 6 of these criteria and hence diagnosed with HLH.

HLH can be genetic or acquired secondary to infections (e.g, EBV), cancers (e.g, lymphomas), immune disorders (e.g., SLE) and transplant recipients. Our patient developed HL secondary to EBV which is the leading infectious cause of HLH. Mortality is very high without specific therapy which includes treatment of the cause plus a combination of chemotherapy and immunosuppressive therapy or BM transplant in resistant cases.

Conclusion

We should rule out lymphoma and HLH in patients with FUO and pancytopenia. Prompt diagnosis of HLH in the setting of Lymphoma and early treatment will regulate the impaired cytolytic activity of T-cells and NK cells with the ultimate resolution of HLH and a better outcome.

237 Successful treatment of accelerated small lymphocytic leukemia with single agent ibrutinib

J Xie1*

Y Hajja1

Y Mouawad2

A Baghian1

B Berbari1

JJ Schmieg1

H Safah1

NS Saba1

1Tulane Medical Center, New Orleans, LA

2Tulane University, New Orleans, LA

Case report

Chronic lymphocytic leukemia (CLL)/small lymphocytic lymphoma (SLL) is the most common adult leukemia in the USA, characterized by accumulation of clonal CD5+ B-lymphocytes in blood, bone marrow and lymphatic systems. Histological architecture shows a pseudofollicular pattern (i.e. proliferation centers) comprised of paraimmunoblasts, which are larger cells with prominent central nucleoli and more cytoplasm. A histological subtype of CLL/SLL that consists of paraimmunoblastic progression and expanded proliferation centers has been reported. This ‘accelerated’ chronic lymphocytic leukemia (A-CLL/SLL) has an aggressive clinical behavior, along with a worse prognosis. Due to the rarity of A-CLL/SLL (<1% of all cases), which is distinct from Richter’s syndrome, the optimal management remains ill-defined.

We report three cases of A-CLL/SLL from our institution. All cases were males in the 60–70s age range who presented with enlarging adenopathy. Excisional lymph node biopsy confirmed a diagnosis of A-CLL/SLL. They were treated with anthracycline, purine analogue, and alkylating agent-based chemo-immunotherapy, which resulted in a brief period of remission. However, the adenopathy re-appeared after several months, and ibrutinib was then given. All achieved rapid, deep and durable responses following treatment with single agent ibrutinib, as evidenced by resolution of symptoms and improving adenopathy. To date, treatment of A-CLL/SLL with ibrutinib has not been reported to our knowledge.

Conclusion

Based on available survival data, A-CLL/SLL represents an aggressive histologic variant of CLL/SLL that manifests with rapidly enlarging adenopathy. Unlike Richter’s syndrome, A-CLL/SLL responds poorly with chemo-immunotherapy. However, patients may achieve long term remission with ibrutinib monotherapy and this should be considered first-line treatment as a bridge to more definitive therapies, such as stem cell transplant.

Infectious diseases/HIV/AIDS

Joint plenary poster session and reception

4:30 PM

Thursday, February 13, 2020
238 Seizure in a kidney transplant patient: would you think post transplant lymphoproliferative disorder?!

J Abdelmalek*

M Elmassry

S El Nawaa

M Zitun

J Makram

HM Mallah

K Nugent

Texas Tech University Health Sciences Center, Lubbock, TX

Case report

Solid organ transplant patients require lifelong immunosuppression to prevent organ rejection. Complex immunosuppressive regimens render the patients susceptible to infections and malignancy.

Case presentation

A 50-year-old man with previous two renal transplants in 1995 and 2003 presented to our hospital after having an episode of seizure-like activity. His immunosuppressive regimen consisted of mycophenolate mofetil, prednisone, and sirolimus. Brain MRI showed multiple enhancing brain lesions in both frontal lobes and temporal lobes and the right caudate nucleus.

Brain biopsy from his right frontal lobe lesion confirmed polymorphous post-transplant lymphoproliferative disorder (PTLD), Epstein-Barr virus positive.

Patient did not have any other seizure activity during his hospital stay. His mycophenolate dose was reduced, and he was treated with rituximab and brain irradiation. He is currently stable with no further seizure episodes or focal neurological symptoms reported.

Discussion

PTLD is a lymphoproliferative disease that can occur as an uncommon complication of immunosuppression after solid organ transplantation.

While most commonly occurring within the first year after transplant, our patient presented after 16 years of his second renal transplant. Epstein-Barr virus activation of B cells is reported in 90 to 95% of PTLD cases. In our case, serum EBV specific IgM and IgG antibodies were negative, whereas EBV DNA was detected by PCR in his blood and his brain biopsied lesions.

Conclusion

Understanding nature of solid organ transplant patients’ immune status and possible complications is pivotal for the timely diagnosis and treatment of life-threatening complications of immunosuppression. High clinical suspicion and early imaging and biopsy can ensure early diagnosis of PTLD cases and hence early treatment.

Abstract 238 Figure 1

Brain MRI showing multifocal enhancing PTLD lesions Brain MRI showing multifocal enhancing PTLD lesions

239 Toxoplasmosis in newly diagnosed HIV

BL Boudreaux*

B Stevens

C Lasecki

R Robinson

D Beyer

E Prejean

S Sanders

K Ellard

LS Engel

LSU Health Sciences Center, New Orleans, LA

Case report

A 38 year old African American man with newly diagnosed HIV presented to the Emergency Department after his girlfriend became concerned about his neurologic function. She noticed he completely ignored his left side, was getting into car accidents and drifting leftward while driving. He was also noted to have issues with his gait. In addition to neurologic deficits, the patient seborrheic keratosis covering his entire face and chest. He was noted to have white plaques covering his tongue. His CD4 count was 10/cu mm and CD4% was 2.4%. MRI showed numerous ring-enhancing lesions in the cerebral hemispheres and posterior fossa. Lumbar puncture was negative except for a positive serum toxoplasma IgG. The patient was started on treatment for toxoplasmosis. After 2 weeks of intravenous sulfamethoxazole/trimethoprim (TMP-SMX), he had a repeat MRI that demonstrated slight improvement in the ring enhancing lesions. He was switched to oral TMP-SMX and was discharged to inpatient rehab. Even after 2 weeks of treatment and improvement on MRI, he had significant neurologic deficits, requiring assistance with transfers and he still had left side neglect.

Discussion

Toxoplasmosis is a parasitic infection due to Toxoplasma gondii. Humans typically contract Toxoplasma from eating undercooked meat, exposure to cat feces, or mother to fetus transmission. Initial signs of infection typically are flu-like, including fever and body aches. In the immunocompromised population, headaches, loss of balance, issues with coordination, and seizures are more common. The classic finding on imaging is ring-enhancing lesions, though malignancies, abscesses, tuberculomas, and neurocysticercosis can also present similarly on imaging. All of these are more common in immunocompromised patients, so a good history and examination of cerebrospinal fluid are imperative in the diagnosis. Our patient’s immunocompromised state placed him at high risk for toxoplasmosis.

240 Severe acne in HIV and hidradenitis suppurativa

L Colpitts*

J Willig

University of Alabama at Birmingham, Birmingham, AL

Case report

Introduction: We present a case of HS with severe facial involvement, possibly triggered by HIV acquisition.

Case

A 26 yo male, a bisexual student, was admitted with severe facial folliculitis (figure 1). His symptoms progressed in 3 weeks, despite clindamycin and TMP/SMZ. He denied constitutional symptoms. Routine HIV testing in the ED was positive (viral load 30k, CD4 531/18%). On day 3 of admission, he was febrile to 102F and a draining right groin abscess was noted. Over the next 2 days, he developed abscesses in groin and axillae bilaterally, which were surgically drained. He was discharged on metronidazole, TMP/SMZ, and antiretroviral therapy. He was seen in dermatology clinic, diagnosed with HS, and prescribed isotretinoin. Over 9 months, his folliculitis improved significantly.

Discussion

A retrospective study of 2 large CDC databases examined 3.45 million ambulatory visits for HS from 2002–15 and found a significant association with HIV (OR 6.39), relative to smoking (OR 2.65) and obesity (OR 2.86). The virus activates the immune system via specific pro-inflammatory cytokines that also play a role in HS. The severity of facial involvement seen in our patient is unique, which may represent the rare diagnosis of acne conglobata (AC), a severe form of acne causing deep burrowing abscesses. AC is seen with HS as part of the follicular occlusion tetrad, which includes dissecting scalp folliculitis and pilonidal cyst.

Our patient is a unique case HIV and HS with severe facial involvement, likely representing AC.

241 Abscess preventing gap closure in diabetic ketoacidosis

N Davison*

UF Health, Jacksonville, FL

Case report

Perinephric abscesses are caused by one of two mechanisms: hematogenous seeding or reflux from the lower genitourinary tract. Risk factors include pregnancy, female gender, diabetes mellitus and urinary tract abnormalities. Dysuria and urinary frequency are frequently absent in these patients. A 22 year old female with a history of poorly controlled insulin dependent diabetes presented with abdominal pain, nausea and vomiting. She also reported she had diarrhea, fevers and malaise and stopped her insulin due to decreased appetite. Urinalysis was remarkable for pyuria. Her chemistry and symptoms were consistent with diabetic ketoacidosis likely due to urinary tract infection. She was admitted to the intensive care unit and started on broad spectrum antibiotics, insulin infusion and intravenous fluids. However, her nausea persisted and her anion gap never definitively closed. Given her tachycardia, leukocytosis in conjunction with her persistent acidotic state and abdominal pain, decision was made to obtain a CT abdomen and pelvis. This imaging was significant for a 5.6 cm mixed cystic and solid lesion on the posterior aspect of the right kidney. She was started on empiric broad spectrum antibiotics and underwent CT guided drainage of the lesion. Cultures of aspirated fluid grew candida albicans sensitive to fluconazole. She was started on a two week course of oral fluconazole 200 mg daily. Follow up imaging during that admission showed reduction in size of the perinephric abscess but showed a new collection in the inferior aspect of the right hepatic lobe concerning for extension of adjacent perinephric abscess. She underwent drainage of this collection which again grew candida albicans sensitive to fluconazole. She finished a new course of oral fluconazole 400 mg daily. Follow up imaging one month later showed resolution of abscesses. A persistently elevated anion gap frequently prompts the search for occult abscess. Empiric coverage of renal and perinephric abscess generally includes antimicrobials aimed at gram negative, staphylococcus aureus or polymicrobial infections. Fungal genitourinary tract abscesses are uncommon and generally associated with diabetes mellitus. This case confirms the need for drainage and rule out of less common causative organisms such as fungus in patients with relative immunocompromised states such as uncontrolled diabetes.

242 Twisted ankle with a twist ending: necrotizing fasciitis from an ankle sprain

J Dickey1*

T Mupfumira1

C Rountree1

J Raposa2

A Chao1

1Medical College of Georgia at Augusta University, Augusta, GA

2Liberty University COM, Lynchburg, VA

Case report

We present a case of necrotizing fasciitis (NF) necessitating an above knee amputation (AKA) following an ankle sprain.

A 33-year-old woman with no prior medical history sustained a right ankle twisting injury while dancing at a wedding. She presented 48 hours after initial injury for progressive right leg swelling and pain. Exam showed an ecchymotic, diffusely tender and swollen ankle with no skin breaks nor crepitus. X-rays showed no fractures. She was placed in observation for possible compartment syndrome and over the next 12 hours she developed tachycardia, hypotension, and tachypnea but was afebrile. Laboratory and radiologic findings were within normal range except for a mild lactic acidosis. At 18 hours hospitalization she went to the operating room for fasciotomy and decompression. Intra-operative findings noted dishwater fluid upon incision with myonecrosis concerning for NF. At this time, blood cultures grew Streptococcus pyogenes (S. pyogenes). IV antibiotics were empirically started, and the wound was closely monitored. Two hours post-operative, the foot became progressively necrotic with ascending progression requiring AKA. Following AKA, the patient experienced a good recovery with few complications.

This case demonstrates the importance of early recognition of NF to prevent significant morbidity and mortality. NF spreads rapidly and requires prompt surgery and antibiotics. Early signs and symptoms of the infection can be difficult to recognize, especially in cases of antecedent blunt trauma where pain and swelling can mimic a musculoskeletal injury. Studies that propose a mechanism for this syndrome have shown that S. pyogenes strains associated with NF avidly adhere to vimentin, a cytoskeletal element that is highly expressed in regenerating muscle cells in vitro. Increased vimentin production in a patient with musculoskeletal injury could potentially increase vulnerability to S. pyogenes infection. This case of an ankle twisting injury leading to NF is a possible manifestation of this mechanism. Furthermore, it highlights the importance of a high index of suspicion for NF in a decompensating patient with pain out of proportion even in the absence of fever or leukocytosis.

243 The tuberculosis masquerade – disseminated tuberculosis presenting as effect of leukemia treatment

M Fasen*

University of Florida, Jacksonville, FL

Case report

Tuberculosis (TB) is an ancient disease that has become a modern-day challenge. Immunocompromised individuals, such as those with leukemia, are at great risk of not only infection but also dissemination of TB. Herein we describe a case of disseminated tuberculosis in the context of acute myelocytic leukemia and chemotherapy, thus complicating the course and therapy of both entities.

Case report

A 24-year-old male presented to the emergency department with complaints of intermittent fever for the past several weeks. His history includes immigrating from Congo one year ago. Upon admission, he was found to be febrile and neutropenic with 85% myeloblasts found on peripheral smear. Acute promyelocytic leukemia M3 was confirmed on subsequent fluorescence in situ hybridization (FISH) that showed t(15:17). He was started on all-trans-retinoic acid (ATRA), idarubicin, arsenic trioxide (ATO) plus vancomycin and cefepime for neutropenic fever. After induction chemotherapy, the patient developed transaminitis and his chemotherapy was subsequently discontinued. Steroids were also initiated due to concern for differentiation syndrome. Liver biopsy was performed due to persisting transaminitis which showed necrotizing granulomas with acid-fast bacilli. This in combination with mycobacterium tuberculosis complex in sputum and blood cultures were consistent with miliary tuberculosis. The patient was started on levofloxacin, rifampin, isoniazid, pyridoxine, ethambutol (RIPE) for concern for multidrug-resistant TB. As the patient’s neutropenia resolved and liver enzymes improved on RIPE therapy, he was started on his chemotherapy regimen ATRA and ATO at half the original dosing. He completed his full course of consolidative chemotherapy without worsening of his disseminated TB. Repeat bone marrow biopsy showed complete response to treatment.

Discussion

To prevent lethal outcomes, a high index of TB suspicion is warranted in immunocompromised patients especially during treatment of malignancies. A dilemma in treatment priority may present in such cases. In this presentation, concern for chemotherapy side effects led to TB diagnosis. TB treatment took precedence during hepatic recovery but AML treatment with complete response was ultimately achieved without worsening of the patient’s disseminated TB.

244 Malignant bordetella pertussis: an atypical presentation with leukemoid reaction in a vaccinated adolescent

Anum Fayyaz*

Robert Welliver

Donna Tyungu

University Of Health Sciences, Oklahoma City, OK

Case report

Bordetella Pertussis is known to cause severe symptoms with leukemoid reaction in infants. We describe a 16 year old vaccinated female with past medical history of mild pulmonary hypertension (group 3 diagnosed 10 years ago by a right heart catheterizarion), and idiopathic adolescent scoliosis presenting with clinical deterioration secondary to pertussis.

The patient was initially admitted to undergo a posterior spinal fusion for scoliosis but was transferred to our pediatric service on the first POD for progressive respiratory distress and hypoxia requiring oxygen. She didnot have cough, congestion or chest pain. The initial chest x-ray was concerning for pulmonary edema versus pneumonia and the patient was started on ceftriaxone. Despite antibiotics her respiratory distress progressed and she was transferred to the pediatric intensive care unit. Initially her white cell count was 29,000, C-reactive protein was 280 and early sputum culture grew Streptococcus Pneumoniae. Ultimately she experienced respiratory faliure requiring intubation and sedation. Leukemoid reaction was noted on day 4 of her hospitalization with a WBC of 60,000. Further infectious workup revealed Bordetella Pertussis. She was started on Azithromycin. She improved clinically over the course of one week and was able to discharge home on 1L of oxygen via nasal cannula.

This case illustrates the emergence of severe pertussis in the USA, even among vaccinated patients. Although malignant pertussis, the most severe form, is better identified and studied in infants, it is important to consider pertussis within the differential diagnosis in patients who present with respiratory faliure and leukemoid reaction, regardless of age and vaccination status. We hope to educate through this case, the importance to consider bordetella pertussis, even among vaccinated individuals, when evaluating patients who present with severe respiratory distress or develop respiratory faliure. Pertussis can be diagnosed in all spectrums of age, in all geographical locations, especially after the revision of vaccination schedule and formulation, especially in those patients with underlying immunodeficiency, pulmonary, or cardiac pathology to avoid lethal consequences.

245 Thrombophlebitis as an initial finding in hansen’s disease

ED Holweg*

D Dwight

Eisenhower Army Medical Center, Augusta, GA

Case report

Hansen’s Disease is an uncommon infectious disease caused by M. lepromatosis and M. leprae that classically infects the skin and peripheral nerves and can present with hypopigmented or reddish skin lesions with overlying loss of sensation as well as enlarged, tender peripheral nerves. Hansen’s disease is rare in the United States with 150–200 cases yearly. The following case is an instance of thrombophlebitis as the initial finding in Hansen’s disease.

A 21-year-old male originally from Micronesia presented to his primary care clinic with a 2 week history of left dorsal hand numbness and palpable cord on the radial dorsal wrist. He was diagnosed with thrombophlebitis of the cephalic vein, started on aspirin and referred for a left upper extremity venous duplex which confirmed superficial thrombophlebitis of the left cephalic vein. 2 weeks later the patient began to develop a reddish lesion over the dorsum of his hand that widened to about 5 cm with increased numbness. At this time the patient was referred to dermatology and started systemic and topical antifungals. The lesion did not improve at follow up and topical steroids were started with no resolution of the skin lesion. Ultimately the patient underwent a punch biopsy which showed sarcoidal granulomas with surrounding lymphocyte predominant inflammation. AFB, gram stain, and PAS stain of this biopsy were negative. Repeat biopsy revealed a single appropriately sized and stained possible organism. A vascular biopsy of the lesion was obtained, however the sample was not sent for histopathology and AFB. The patient’s clinical presentation as well as skin biopsy showing no AFB organisms was consistent with the tuberculoid form of Hansen’s disease. The patient was started on dapsone and rifampin for a planned total duration of 12 months.

Leprous phlebitis was first described in the 1800’s with several cases reported in the 1980’s. It is believed to be caused by deposition of mycobacterium in the intima of vessels which spreads to peripheral nerves via the Vasa nervosum resulting in overlying paresthesia. This case represents a rare initial presentation of thrombophlebitis in Hansen’s Disease and highlights the importance of keeping a broad differential and using clinical presentation to make a diagnosis when laboratory tools are not useful in diagnostic confirmation.

246 A toddler with refractory atypical kawasaki and concomitant enterovirus viremia

MM Hopper*

C Dye

University of Alabama Birmingham, Birmingham, AL

Case

A 2 year old previously healthy male was admitted for dehydration and symptoms of a viral illness, including 4 days of fever, emesis, bilateral conjunctivitis and rash. Initial work up revealed Enterovirus viremia, a likely source of the patient’s presentation. However during the patient’s hospital stay, he remained persistently febrile, and labs were consistent with Atypical Kawasaki (anemia, elevated ALT, hypoalbuminemia and elevated inflammatory markers.) Initial echo was normal, and he was treated with IVIG. His fever responded dramatically to initial treatment of IVIG, and the patient was discharged home after remaining afebrile 24 hours. He was readmitted for recurrence of fever the following day. He received a second round of IVIG for recurrent Kawasaki. The patient underwent repeat echo revealing a right coronary artery more dilated than in the prior study but within upper limits of normal. He developed fever again and received Infliximab for refractory atypical Kawasaki on fourth day of readmission. After Infliximab he remained afebrile and was discharged home with cardiology follow up and continued aspirin course.

Discussion

Kawasaki Disease (KD) remains a disease without a known cause. Several researchers have suggested that KD may be linked to common viral illnesses, including enterovirus. One population based cohort study in Taiwan found the incidence of KD was 56% higher in a cohort of children infected with enterovirus when compared to a cohort of children without enterovirus infection. (Weng, K.P., et al) Several other common viruses have been considered in the etiology of KD, but further studies suggest that enterovirus infection alone may be more strongly associated with KD.

Conclusion

As researchers continue to search for a cause of Kawasaki Disease, the increased association of enterovirus with KD supports claims that the virus is linked to the etiology of the disease. Our patient‘s KD may have been closely linked to his concurrent enterovirus viremia.

Reference

  1. Weng K-P, Wei JC-C, Hung Y-M, Huang S-H, Chien K-J, Lin C-C, Cheng M-F. Enterovirus infection and subsequent risk of kawasaki disease. The Pediatric Infectious Disease Journal 2018;37(4):310–315. doi: 10.1097/inf.0000000000001748

247 Acutely relapsing hepatitis a virus infection after five years of initial infection

M Ibrahim1*

A Ibrahim2

M Krolikowski1

I Haddad1

O Sheikh1

P Bhattad1

1ETSU, Johnson City, TN

2University of Jordan, Amman, Jordan

Case report

HAV infection confers a lifelong immunity, with a full clinical and laboratory recovery within 2–3 months in most patients, but some relapsing cases have been reported. Relapsing hepatitis occurs in up to 10% of the patients within 6 months of acute illness.

We are presenting a case about a 39-year old female with known chronic HCV, who presented with relapsing HAV, after five years of confirmed initial HAV infection resolution. The patient presented with severe abdominal pain, fatigue, gross jaundice and vomiting for one week. Patient also had a fever of 101.8 F, dehydration, constant vomiting with loose diarrhea and generalized itching. On physical examination, she had severe superficial tenderness over the epigastric and RUQ locations with hepatomegaly but no peritoneal signs. Ultrasound of biliary tract showed collapsed gall bladder. Labs were remarkable for transaminitis, including markedly elevated Alkaline phosphatase and hyperbilirubinemia (as shown in the table 1). Further work up showed negative HIV, CMV, EBV, ANA and HbsAg. With supportive management, liver enzymes trended down with decreased bilirubin level and marked improvement in her symptoms over a week.

Abstract 247 Table 1

Laboratory tests shows marked transminitis with positive hepatitis A IgM test Laboratory tests shows marked transminitis with positive hepatitis A IgM test

Abstract 247 Figure 1

MRCP shows hepatomegaly with collapsed gall bladder MRCP shows hepatomegaly with collapsed gall bladder

248 Meningoencephalitis: the great mimic

H Jain*

SUNY Upstate Medical University, Syracuse, NY

Case report

A 63-year-old male with a history of melanoma metastasizing to lungs started on immunotherapy two months ago with Ipilimumab and Nivolumab once every three weeks, sinus surgery for deviated nasal septum two years ago presented for a routine follow-up to oncology clinic and was found to have a fever of 101.8. Before the clinic appointment, he and his wife had gone to the Outer Banks, North Carolina along with six other couples for a vacation. The patient had been complaining of intermittent headaches there and was finding difficult to watch television with light bothering his eyes. He had chronic sinus pain and stuffiness for which he used a neti pot with a mixture of saline and tap water regularly. During the hospital course, he started spiking daily intermittent high-grade fevers along with worsening of mental status. Infectious diseases were consulted for persistent fevers who recommended lumbar puncture (LP) with routine CSF analysis and culture. The patient was started on empiric antibiotics/antivirals. His LP results returned with a mildly elevated total nucleated cell count of 17 with 100% lymphocytic predominance, an elevated protein of 126 and glucose of 70. Given extensive infectious workup and the failure to improve on appropriate treatment, other etiologies and medications were evaluated. Steroids were started eventually due to concern of chemotherapeutic adverse effects. After receiving steroids, there was a complete resolution of fevers and the patient self-extubated.

Discussion

Despite unprecedented efficacy across multiple tumor types, immune checkpoint inhibitor therapy is associated with a unique and wide spectrum of immune-related adverse events including neurologic events ranging from a mild headache to potentially life-threatening encephalitis. Serious and sometimes fatal neurologic adverse effects have been reported with Ipilimumab, including sensory and motor neuropathy, and myasthenia gravis. Other reported neurologic adverse effects include inflammatory myopathy, aseptic meningitis with cerebrospinal fluid (CSF) lymphocytosis, and chronic inflammatory demyelinating polyneuropathy.

Conclusion

Physicians need to be aware of the clinical presentation of serious but uncommon neurologic adverse effects associated with checkpoint inhibitors. Prompt diagnosis and management are critical to minimize serious complications and patient outcomes.

249 A not so typical presentation of lung adenocarcinoma in an HIV patient

AT Kunnumpurath*

B Huang

DG Jacob

White River Health System, Batesville, AR

Case report

46 y/o African American woman with known HIV on anti-retroviral therapy was admitted with progressively worsening dyspnea.She endorsed orthopnea, ankle swelling with NYHA class 3 symptoms. Chest radiograph showed cardiomegaly with no acute infiltrate. On examination she was tachycardic and hypoxic with distant heart sounds. She had a palpable left supraclavicular lymph node and multiple axillary lymph nodes. She also had tenderness in her right calf. Her white cell count was minimally elevated with a left shift. EKG showed sinus tachycardia. An Echo cardiogram revealed moderate pericardial effusion with a normal ejection fraction. A pericardiocentesis performed drained serosanguinous fluid which was negative for infection. Doppler of the right lower extremity revealed a deep vein thrombosis and she was started on heparin drip. A chest CTA showed no pulmonary embolism but did reveal right paratracheal adenopathy, right hilar mass and subcarinal nodes. Further imaging showed bilateral breast masses, bilateral pleural effusions, bilateral adrenal nodules, vertebral translucency and two brain lesions. Her CD4 count was 116 cells/mm3 and with T- lymphocyte CD4/CD8 ratio -0.11.She was started on prophylaxis with Bactrim in addition to her anti-retroviral therapy. An excision biopsy of the supraclavicular lymph node was consistent with metastatic adenocarcinoma. Pericardial fluid studies showed malignant large epithelioid cells, favoring metastatic adenocarcinoma. Patient continued to have worsening dyspnea prompting a repeat pericardiocentesis. However due to continued symptoms and re-accumulation of pericardial fluid, patient received a pericardial window. Patient decompensated shortly after pericardial window and was transferred to a higher center for further management, but died later.

Discussion

Lung Cancer is associated with unique HIV-related causal mechanisms, and may be associated with worse outcomes in some people with HIV. The mean age of HIV infected patients at the time of lung cancer diagnosis is 45 years. Lung cancer is an important complication associated with chronic HIV infection. The clinician must remember that patients with chronic HIV are at higher risk for solid tumors from the rest of population and include this in their differential.

250 ‘You make my hear flutter’ an arkansonian tick’s love story: an unusual cause of atrial flutter

A Kunnumpurath*

R Kamoga

White River Health System, Batesville, AR

Introduction

The number of ehrlichiosis cases due to Ehrlichia chaffeensis reported to CDC has increased steadily since the first year of disease reporting. In the year 2000, only 200 cases of ehrlichiosis were reported, while in 2017 more than 1,642 cases were reported.1 Ehlirchiosis can present with a varied clinical picture. Typical symptoms including generalized weakness, joint pain and rash within days of weeks of tick bite.

Case

46 y/o Caucasian male with past medical history of multiple sclerosis, plaque psoriasis presented with generalized weakness of 1 week duration. He also had associated fever and chills for the past day. He did admit to multiple tick bites from recent travel in Arkansas prior to the symptoms starting. He did not know the duration of tick attachment. On exam he had a fever of 100.50F and a heart rate of 64 beats/min. Significant labs included a white cell count of 4.2 k/ul with 77% neutrophils, platelets of 83,000/ul. Sodium was 131 meq/l. His Bun was 12 mg/dl and creatinine was 1.4 mg/dl. Liver enzymes were within normal limits. EKG obtained showed variable block atrial flutter with heart rate less than 100 beats/min. (Prior EKG showed Normal sinus rhythm). Patient was treated empirically Doxycycline, intravenous fluids and metoprolol tartrate. Ehrlichia chaffeensis IgM Ab was positive with titers >1:40 consistent with Ehrlichiosis. Patient’s symptoms improved over the next couple of days and eventually discharged. Repeat EKG one month later showed normal sinus rhythm.

Discussion

Ehlrichiosis is usually associated with anemia, thrombocytopenia and elevated liver enzymes. Severe cases have been associated with meningitis and seizures.3 Ehlrichioisis affecting the cardiovascular system has not been reported and not thought to be part of the typical presentation of Ehlirchia chafeensis. We found one case report of atrial fibrillation with rapid ventricular2 response due to Ehlirchia. The pathophysiology on how the bacteria causes atrial fibrillation or atrial flutter is unclear, thus warranting further understanding on this mechanism. With number of cases of ehlrichia on the rise,this case also teaches the clinician that another rare cause of new onset atrial fibrillation and flutter could be due to Ehlrichia chafeensis associated illness.

251 My my my… it’s MAI a case of diffuse cavitary lung disease

A Kunnumpurath*

N Mehta

A Kumar

White River Health System, Batesville, AR

Case report

Cavitary lung lesions found on chest radiograph are relatively common. Infectious and non-infectious etioloigies can both present with diffuse cavitary lung lesions. The differential is broad and often presents as diagnostic challenge to the clinicain.

Case

43 y/o Ex-smoker (28 pack years) who worked as a painter in car body shop was admitted with sudden onset dyspnea. He was hypoxic and white cell count was slightly elevated with a left shift. Chest radiograph showed a right sided pneumothorax of moderate volume with cavitary lesions in right and left upper lung. Chest CT showed a thick walled cavitary lesion in left upper lobe and multifocal cavitary lesions throughout the lung. A chest tube was placed, following which patients symptoms improved. Blood cultures showed no growth. Patient was started on levofloxacin, rifampin and ethambutol due to concern for tuberculosis. AFB sputum cultures were negative. Bronchoscopy was done and specimen grew Stenotrophomonas maltophilia and AFB culture from the bronchial specimen grew Mycobacterium avium intracellulare but negative for tuberculosis. Cytology and pathology was negative for malignancy. MAI was identified by 16s RNA gene sequencing directly from the acid fast bacilli smear positive broth. Patient was started on trimethoprim- sulfamethoxazole and rifampin base on sensitivity and transferred to higher center with an infectious disease service for further management and evaluation. Patient eventually did better after a prolonged course of antibiotics and was discharged home.

Conclusion

Lung imaging of Mycobacterium avium intracellulare (MAI) can include a wide range of findings including atelectasis, bronchiectasis, cavities, consolidation, emphysema, ground glass opacity, linear opacities, mediastinal lymphadenopathy, nodule and over inflation like TB with upper lobe disease.Our case shows that MAI continues to be reported in immunocompetent individuals.Patients with preexisting lung disease or immunodeficiency are at greatest risk for developing MAI infection. The clinician thinks of tuberculosis or fungal infection in the setting of upper lobe cavitary lesions; however our case shows that MAI can often mimic other cavitary lesions.

252 An unusual cause of acute pyelonephritis with hydronephrosis : aerococcus urinae pyelonephritis

A Kunnumpurath1*

RT Emery2

1White River Health System, Batesville, AR

2University of Arkansas for Medical Sciences, Little Rock, AR

Introduction

Aeroccus urinae is a rare cause of urinary tract infection in older adults. Aerococcus urinae is usually seen among males,nursing home residents or those with prostate disease.

Case

94 y/o caucasian female with past medical history of breast cancer in remission, history of intermittent urinary tract infections presented with abd pain and confusion. On exam her abdomen was soft with no peritoneal signs.Significant labs included a white cell count of 13.4 k/ul with 84% neutrophils and acute kidney injury with Bun of 34 mg/dl and creatinine of 1.5 mg/dl (Baseline creatinine of 0.9–1.0 mg/dl). Urine analysis showed 6–10 red cells, too numerous to count white cells, 1 + bacteria, small leukocyte esterase and negative nitrite. A CT of abdomen and pelvis showed left pyelonephritis and hydronephrosis with no obstructing stone. Due to unclear etiology of hydronephrosis, urology performed cystoscopy, a diagnostic left retrograde pyelogram and a left ureteroscopy. The cystoscopy revealed large amounts of mucus in the bladder. The reterograde pyelogram went up to the level of the proximal ureter. A large amount of pus was extracted from beyond this area which was sent for culture and sensitivity (including fungal cultures which were negative) and a ureteral stent was placed. Final cultures grew Aerococcus urinae and patient was treated with two weeks of intravenous Ceftriaxone and discharged home. Patient did better, had good urine output and acute kidney injury resolved.

Discussion

Our case shows that Aerococcus urinae infection continues to be reported in persons without typical risk factors and is an emerging cause of urinary tract infections and/or pyelonephritis. The presence of hydronephrosis with no obstructing stone prompted a retrograde pyelogram and ureteroscopy which helped make the accurate diagnosis. Aerococcus urinae is often difficult to isolate and initial gram positive growth often misguides the clinician to think of other gram positive organisms such as stapholycoccus and enterococcus.

253 When one infection points to another

M Magnuson*

GD Gibson

C Doo

J Jackson

I Weatherly

University of Mississippi Medical Center, Jackson, MS

Case report

Herpetic Whitlow is a painful cutaneous infection of the hand caused by HSV 1 and 2. Here we report a case of herpetic whitlow and acute bacterial paronychia in an immunocompromised patient.

Case presentation

A 21-year-old African American female with SLE on chronic immunosuppression, lupus nephritis, and atypical HUS on eculizumab presented with pain and swelling around her fingernails. She reported that the symptoms started on two digits of the left hand. She presented to local ED due to worsening pain and violaceous discoloration where she was diagnosed with paronychia. She underwent incision and drainage and was discharged on oral clindamycin.

Despite antibiotics, she had worsening symptoms and progression of involvement to multiple digits on the right hand. She was advised by local provider to transfer to University of Mississippi Medical Center for further care. On arrival to our ED, patient was noted to have erythema, edema, and crusting of the proximal and lateral nail folds on bilateral hands. Crusted erosions were noted on lower lip, and she disclosed that she had a habit of onychophagia. Regarding other exposures, she had used a chemical hair treatment the week prior.

Laboratory data revealed no leukocytosis. Xrays of bilateral hands showed soft tissue swelling and a small pocket of gas along the right fourth digit at the site of recent I&D. There was no radiographic evidence of osteomyelitis. A bacterial culture from an affected area grew MRSA with resistance to clindamycin; PCR detected HSV1. The patient was diagnosed with herpetic whitlow and acute bacterial paronychia involving multiple digits. She was started on acyclovir, doxycycline, and metronidazole with improvement in pain and swelling.

Discussion

We present a complex presentation of herpetic whitlow in the setting of acute bacterial paronychia secondary to poor hand hygiene and nail biting. Incision and drainage should be avoided as it can worsen symptoms and lead to viremia or superimposed bacterial infection. In immunocompromised patients, treatment with systemic acyclovir is indicated with any form of herpes infection, localized or disseminated. Recognizing and diagnosing herpetic whitlow promptly is important for the treatment of the affected patient and for the prevention of transmission to other individuals.

254 A peculiar presentation of parotitis

A McKernan*

TC Kingdon

A Gupta

LSU Health Sciences Center, New Orleans, LA

Case report

Neonatal suppurative parotitis (NSP) is an uncommon disease with a previously studied prevalence of 3.8:10,000 of neonatal admissions. While uncommon, its broad differential with daunting complications presents therapeutic necessities. Cellulitis-adenitis syndrome represents a possible focal manifestation of late-onset GBS infection, which is more common than parotitis, with an incidence estimated at 4%. Bacteremia is present in up to 90% of the cases and there can be meningitis associated in 33% of cases making aggressive treatment a consideration. We present the case of a 14-day old male who presented with right-sided facial swelling and erythema and was diagnosed with NSP.

A 14-day old full-term twin male born at 38 weeks without complications presented to the emergency department with one day of unilateral facial swelling. Physical exam demonstrated right-sided facial swelling with expression of purulent drainage from Stensen’s Duct following parotid massage. Initial lab-work showed a slight leukocytosis with neutrophil predominance. Electrolytes, inflammatory markers including C-reactive protein and procalcitonin, and nasal PCR for viruses were all normal. Cerebrospinal fluid (CSF) was grossly bloody but did not have white blood cells and meningitis PCR panel was negative. Head and neck ultrasound demonstrated a swollen, hypervascular right parotid gland consistent with parotitis without abscess. Empiric intravenous antimicrobial coverage was initiated with vancomycin, cefepime, and metronidazole. CSF and blood cultures remained without growth. Following clinical improvement, ENT and infectious disease agreed with the transition to oral clindamycin for an additional ten days with subsequent resolution symptoms.

Although rare, NSP should be suspected in newborns presenting with an erythematous pre-auricular mass with or without any predisposing factors. S. aureus is the most common pathogen isolated from infants with NSP. Expression of purulent fluid from Stensen’s Duct is pathognomonic of NSP and culture should be collected if possible. Most patients can be treated conservatively, provided that the empiric antibiotic treatment covers the causative agents according to the local sensitivity pattern and is started early. The prognosis of the disease is generally excellent.

255 Streptococcus viridans urosepsis in a patient with nephrolithiasis

F Mubeen*

S Islam

T Naguib

A Kamat

Texas Tech University Health Sciences Center, Amarillo, TX

Case report

Sepsis from gram negative urinary tract infection is a common cause of hospitalization in the US. While streptococcus viridians is a normal oral flora that is known to cause subacute bacterial endocarditis after dental procedures, it rarely can cause urinary tract infection. We present a case of viridans streptococcal bacteremia and urinary tract infection complicated with obstructing renal stones.

Case

Sixty-year-old female presents with fever, flank pain, dysuria, and septic shock. She has polycystic ovarian syndrome, morbid obesity, hyperthyroidism on methimazole. She was tachycardic and hypotensive with leucocytosis and left shift. Elevated serum creatinine, pyuria, bacteuria, and microscopic hematuria were noted. CT showed atrophic kidneys and large bilateral multiple calculi in the renal pelvis and the ureteropelvic junctions with modest hydronephrosis. Intravenous fluids, cefepime, and vancomycin were started. Right ureteral stent and a left percutaneous nephrostomy were placed. Blood cultures revealed viridans streptococcus Group identified by MALDI-TOF technique that also showed the same organism in urine culture. A surface echocardiogram showed no endocarditis. Intravenous cefepime yielded marked improvement and she was discharged on 4 weeks of cefuroxime.

Discussion

Women are at most risk of urinary tract infection, usually due to enterobacteriaceae and staphylococcus saprophyticus. However a viridans group streptococcus positive blood and urine cultures in a patient with symptomatic UTI with renal stones is an atypical presentation. A viridans group streptococcus urinary tract infection with streptococcus mitis strain in a diabetic patient with a urethrocele was reported earlier. Sreptococcus viridans forms biofilm on endocardium and can have similar pathological process in urinary tract providing nidus for stone formation and increase risk of systemic infections. It is appropriate to study the stone and urinary chemistry to explore potential mechanisms of stone formation in this infection.

Conclusion

Streptococcus viridans group urinary tract infection is a rare and may be associated with obstructive uropathy from renal stones or uretherocele. Evaluating urine chemistry is needed in such patients.

256 Abrupt thrombocytopenic crisis in an immunodeficient patient

CD Nance*

J Hogan

N Dunlap

A Kumar

UTHSC, Memphis, TN

Case report

53 yo male with history of AIDS (CD4+ 78), presented to the ED with flank pain and fevers for two days. On initial exam he was ill appearing and tachypneic with BP of 99/70 mmHg. Labs were significant for a creatinine of 5.0 mg/dL, AST/ALT of 156/290, and LDH of 3,965 U/L. Urinalysis, renal ultrasound, chest X-ray and chest/abdomen CT were unremarkable. The patient was started on Rocephin and Zithromax. He developed severe sepsis on day two and antibiotics were broadened to Linezolid, Cefepime, and Amphotericin B with resolution of sepsis. He was started on dialysis. Due to concern for HIV nephropathy, antivirals were started. On day five the platelet count dropped abruptly from 215k to 84k and continued to drop to 12k. Blood cultures and urine antigen came back positive for histoplasmosis. All antibiotics except amphotericin B were held. We ruled out DIC, HIT, and TTP based on labs and smears. His ferritin level came back >7k which raised concerns for HLH. Diagnostic BM biopsy was obtained and we empirically started high dose IV Dexamethasone. The platelets did not respond and IVIG was then started with subsequent improvement to 75k. The renal biopsy was finally performed that showed ATN. Bone marrow biopsy resulted showing hypercellularity of marrow and histoplasmosis, favoring both idiopathic thrombocytopenic purpura and disseminated histoplasmosis. He was discharged with improving platelets and transitioned to Itraconazole. In follow up, platelets fully recovered.

Discussion

Thrombocytopenia can have a wide differential and an immediate decline in platelet counts can delay diagnostic testing and limit medical treatments. The severe thrombocytopenia secondary to ITP complicated the management of severe sepsis, acute renal failure, and disseminated histoplasmosis in our patient with AIDS. Potential adverse effects of management often need to be expected for care in patients with multiple serious comorbidities. Thus, the appropriate pathogenesis should be identified, and therapy narrowed, to limit risks of treatment induced adverse events. The comprehensive, yet concise workup enables the most appropriate therapy to be chosen in conditions with multi-organ failure.

257 Bad to the bone

H Oddo Moise*

AL Heifner

N Masri

K Boudreaux

LSU Health Sciences Center, New Orleans, LA

Case report

Prostate cancer is the second most common malignancy in men worldwide. Treatments have generally consisted of surgical and radiation therapies. Well-known complications include urinary incontinence, cystitis, ED, osteitis pubis and voiding difficulties. Not as well known, pelvic osteomyelitis or fistulation into pubic symphysis may also be seen.

Case

A 69yo male with history of recent UTIs, prostate cancer s/p radical prostatectomy and external beam radiation therapy and urinary incontinence presented with confusion x1 day. Patient’s acute medical problems began 1 month prior to admit when he was admitted for UTI. Since that time he had noted progressive generalized weakness, difficulty getting out of bed and walking unassisted, fever, chills, purulent penile discharge and pain in right inguinal region. On admit, patient was febrile to 102.4F, altered, tachycardic and tachypneic, UA with >100 WBC, many bacteria, 40 RBCs, UCx polymicrobial. He was started initially on vanc and meropenem. CT AP noted fluid collection vs abscess in right anteromedial thigh musculature and air/hyper-density near pubic symphysis. Abscess confirmed on US as 7.4 × 3.2 × 6.4 cm heterogenous mass. IR consulted for percutaneous drainage and placed right thigh drain, cultures without growth. MRI pelvis was obtained and found findings consistent for osteomyelitis of bilateral pubic bodies and septic arthritis centered at pubic symphysis with fluid collection concerning for abscess extending into right adductor longus musculature. A CT cystogram was performed that demonstrated a vesico-pubic symphyseal fistula, likely resulting in pelvic osteo and septic arthritis with tracking into right thigh. Bilateral nephrostomy tubes were placed for urinary diversion. He was discharged in stable condition to LTAC for 6 weeks of antibiotic therapy.

Discussion

Anastomotic complications are sometimes seen as early or late sequelae of prostate cancer treatment. These may present as both pelvic osteomyelitis or symphyseal fistulation, that typically involve prolonged antibiotic therapy on the conservative end or surgical intervention and ultimately reconstruction. This case emphasizes the importance of thorough medical and surgical histories on all patients, including those who present with seemingly routine diagnoses, as well as avoidance of anchoring to a previous diagnosis.

258 Lemierre syndrome: atypical presentation of a forgotten disease

P Pastor-Perez*

M Colon

San Juan City Hospital, Bayamon

Case report

Lemierre Syndrome (LS) is a rare life-threatening condition secondary to progressive oropharyngeal infection. It may extend to pharyngeal spaces of neck with complications such as internal jugular vein septic thrombophlebitis and bacteremia. Estimated 1/1,000,000 cases are reported worldwide annually in increasing trend. Most commonly seen in immunocompetent patients within 19–22 years old. We present the case of an immunocompetent elderly female with an unresolved sore throat which progressed to left neck swelling and dysphagia.

Case of 77-year-old female who came to the emergency room with complaints of sore throat of one week of evolution. She self-treated with Azithromycin, however, she subsequently developed left neck swelling and dysphagia. Laboratory findings remarkable for leukocytosis. Admitted for Intravenous (IV) antibiotics (Doxycycline and Ampicillin-Sulbactam) with diagnosis of complicated-acute tonsillitis. After one week of therapy, accumulation of fluid was suspected in the neck for which Neck/Thorax CT-scan was done revealing large abscess in the anterior neck and parotid gland extending to the mediastinum with evidence of left internal jugular vein thrombosis. Incision and drainage was performed; Neck abscess culture reported Citrobacter freundii. Antibiotic therapy was optimized to Ceftazidime/Avibactam and Tigecycline. Clinical improvement was slower than expected. Neck/Thorax CT-scan was repeated with evidence of new multiple abscesses requiring second surgical drainage. Multiple complications including pleural effusions and empyema requiring chest tube placement. After five weeks of antibiotic therapy, significant clinical improvement reported.

Advanced LS is a life-threatening severe illness. Differential diagnosis for early LS is broad considering all possible causes of oropharyngeal infections. Even with antibiotics, mortality by LS has been documented. Incidence is higher in younger populations with only a few cases reported in older populations. Elderly population is at greater risk for sepsis and septic emboli. Oral infections should have thorough evaluation due to potential extension to pharyngeal neck spaces such as jugular veins. The goal of this case report is to reinforce medical awareness of possible rare complications of oropharyngeal infections due to its high mortality.

259 A rare case of serratia endocarditis

R Patel*

K Sanders

M Oye

University of Florida, Jacksonville, Jacksonville, FL

Case report

Serratia marcescens is an uncommon cause of infective endocarditis. While this disease has historically been associated with intravenous drug use, more recent reports suggest that it is now largely a consequence of opportunistic infections of the chronically ill. Our case highlights several characteristic features of this infection, including isolation of a non-pigmented strain of the organism, an antibiotic susceptibility profile suggestive of AmpC β-lactamase production, and rapid clinical deterioration with multiple embolic complications resulting in death. In this review we discuss the history, epidemiology, and management of endovascular infections due to Serratia spp., emphasizing the continued importance of considering this organism in the differential diagnosis of endocarditis among intravenous drug users and as a potential indication for surgical therapy.

Infective endocarditis from Serratia marcescens is rare. Endocarditis is typically right-sided in intravenous drug users however Serratiatends to involve left-sided valves. Coincidentally; our patient’s echo findings show only right heart-valve involvement despite the presence of a PFO. With the incidence of intravenous drug use on the rise, patients are at a heightened risk of pathogen exposures. When picking an antibiotic for therapy; it is important to remember to place susceptibilities at the forefront of medication management.

260 A case of necrotizing fasciitis due to fusobacterium necrophorum

H Rafeek*

C Nguyen

A Ammu

TTUHSC, Odessa, TX

Case report

A 24y old African American male with no known past medical history presented with 2 day history of pain, swelling and redness involving his left upper extremity and left ankle. He also reported dyspnea and left sided pleuritic chest pain for 1 day. He denied any trauma, bites, water exposure or intravenous drug use. Physical exam revealed temperature of 101.6 F, heart rate of 110/min; extreme tenderness, swelling, erythema and induration of left upper extremity and left ankle. Abnormal labs included wbc of 17000/µL, AST of 107 IU, ALT 85 IU and procalcitonin of >100 ng/ml. X-ray of left arm showed presence of gas in subcutaneous tissue. Computed tomography of the chest showed septic emboli and left sided pleural effusion. He underwent emergent surgical debridement of his left upper extremity and left ankle. Purulent material was noted to be extending from the subcutaneous tissue down to deep fascia at both sites. Wound cultures grew Fusobacterium necrophorum from the left arm and left ankle. Blood cultures grew Fusobacterium necrophorum. Pleural fluid analysis revealed ph. of 7; glucose of 11 mg/dl, LDH of 19950 units/L, protein of 3238 mg/dl suggestive of empyema. Chest tube was placed. He was treated with prolonged course of intravenous piperacillin/tazobactam and metronidazole for 6 weeks and had complete resolution of infection.

Conclusion

Fusobacterium necrophorum is a gram negative anaerobe that is usually associated with head and neck infections including septic thrombophlebitis of internal jugular vein (Lemierre’s syndrome). It can cause necrotizing soft tissue infection in rare cases. Treatment involves early and aggressive surgical debridement and antibiotics. The antibiotics that are effective against Fusobacterium are piperacillin/tazobactam; metronidazole; clindamycin; ampicillin/sulbactam and penicillin (in beta lactamase negative strains).

261 A severe case of coagulase-negative staphylococcus prosthetic valve endocarditis

JT Raposa1,2*

A Chao2

J Dickey2

C Rountree2

T Mupfumira2

1Liberty University College of Osteopathic Medicine, Lynchburg, VA

2Medical College of Georgia at Augusta University, Augusta, GA

Case report

Infective endocarditis (IE) is of continued clinical importance, occurring in up to 15 per 100,000 people per year in the United States. While bacteria like Staphylococcus aureus and viridans group streptococci remain among the most common causative organisms, we continue to see a significant amount of disease by coagulase-negative staphylococci (CONS). While often dismissed as contaminant or of no significance, CONS remain a formidable cause of extensive disease pathology, notably in patients with prosthetic valves (PV).

A 70-year-old man presented to the hospital with complaints of shortness of breath in the setting of recent bioprosthetic aortic valve replacement 3 months prior for moderate-severe aortic stenosis. He experienced progressive dyspnea on exertion for 4 days, occurring with minimal exertion as well as new orthopnea. He had been started on furosemide by his cardiologist without relief. He denied any other symptoms.

He was noted on exam to have a new 4/6 holosystolic murmur and 3/4 diastolic murmur at the right upper sternal border, but no peripheral stigmata of IE or embolic disease. Due to these new exam findings and complaint of dyspnea, he underwent transthoracic echocardiography (TTE), finding partial dehiscence of his PV with severe paravalvular aortic regurgitation.

IE was suspected in the setting of his presenting complaints as well as new TTE findings. Blood cultures grew Staphylococcus epidermidis on sequential tests and he was started on vancomycin. A transesophageal echocardiogram confirmed the TTE findings as well as a peri-valvular abscess around his PV extending up the aortic root towards his mitral valve. He was also noted to have progressive PR prolongation to over 0.4 seconds and required transcutaneous pacing.

CONS are often underestimated in the clinical setting. This patient’s course highlights how CONS infections may insidiously develop, yet still have catastrophic infectious complications. This patient required multidisciplinary care closely managed by Cardiology, Electrophysiology and Infectious Diseases services and was ultimately referred to Cardiothoracic Surgery at a quaternary medical center for advanced care.

262 Septic lumbar facet joint arthritis due to streptococcus agalactiae; a real pain in the back

JG Rodriguez Velez*

A Nieves-Ortiz

HR Cintrón-Colón

WD Marrero

San Juan City Hospital, San Juan

Case report

Back pain is one of the most common reasons for visits to emergency clinics in the United States. Often degenerative diseases are the cause of back pain, however, less frequently, it can be caused by infective processes. Rarely septic lumbar facet joint arthritis (SLFJA) have been reported as underline back pain etiology. SLFJA is a potentially fatal condition with high risk for devastating complications including spinal cord compression. Staphylococcus epidermidisis the main causative micro-organisms of SLFJA, but Streptococcus agalactiaeis extremely rare. We describe a case of a male patient who presented to our institution with complaint of worsening back pain diagnosed with SLFJA due to Streptococcus agalactiae.

58 y/o male with past medical history of hypertension and diabetes arrived at the emergency clinic complying of worsening back pain with associated night sweats, chills, and fever. Physical examination revealed tachycardia, and lumbar vertebrae tenderness. Initial workup revealed leukocytosis and elevated inflammatory markers. Further questioning revealed a recent hospitalization for left leg cellulitis. The patient was admitted with diagnosis of sepsis secondary to possible bacteremia and started on broad spectrum antibiotics. Blood culture were positive for Streptococcus agalactiae sensitive to Ceftriaxone for which antibiotics were adjusted. MRI of the lumbar spine reported L4-L5 and L5-S1 facet joint septic arthritis. Neurosurgery services were consulted but in view of absence of signs of cord compression, medical management was advised. He completed 72 days of Ceftriaxone. MRI was repeated which showed significant decrease in extension of the epidural collection. Patient was discharge with a corset for back support and ambulating with assistance.

SLFJA as the underlying cause of back pain is rarely encountered. For this reason, diagnosis might be illusive at evaluation and proper management delayed. It is imperative to identified risk factors which might point to an infection as the etiology of backpain. Having a low threshold for suspicion for hematogenous bacterial seeding to vertebrae is important because of high risk of progressive adjacent structural damage, septicemia and ultimately death.

263 The importance of pre-transplant screening in solid organ transplantation during the opioid crisis

C Rountree1*

T Mupfumira1

J Dickey1

J Raposa2

A Chao1

1Medical College of Georgia at Augusta University, Augusta, GA

2Liberty University College of Osteopathic Medicine, Lynchburg, VA

Case report

Due to the demand for kidneys for transplantations, deceased donor kidney transplants (DDKT) account for nearly 68% of all kidney transplantations. All transplants warrant a thorough history of both donor and recipient with screening for infections and exposures. This is especially pertinent during the current opioid crisis. In this case, we report the discovery and management of a polymicrobial bloodstream infection in a kidney donor who died of an acute opioid overdose.

In our case, a 31-year-old female with a past medical history of end-stage renal disease on hemodialysis had received a DDKT. She had previously undergone pre-transplant screening for infections which included testing for human immunodeficiency virus (HIV), cytomegalovirus (CMV), Epstein-Barr virus, hepatitis C virus (HCV) and tuberculosis, with only a positive CMV IgG. She tolerated the surgery well and had steady improvement in both her clinical condition and transplant function.

On post-transplant day 2 we were notified that additional microbiological data from the donor was available. The kidney donor was a 23-year-old male who had overdosed while using IV heroin 2 days prior to the surgery. Donor screening was performed as well as blood and urine culture. The donor was HIV and HCV negative, but had positive blood culture growth for E. coli, methicillin-susceptible Staphylococcus aureus (MSSA) and Pantoea agglomerans. The recipient was treated with cefepime and ciprofloxacin and serial blood cultures drawn. Fortunately, no bacteremia occurred post-transplant and the recipient did well. We recommended follow-up HIV and HCV screening 4 weeks post-transplant.

In addition to the interesting challenge of antibiotic management in this post-transplant patient, this case highlights areas of concern for transplantation as a whole. Transplant protocols are aimed at screening for chronic disease conditions, however organs from high-risk donors such as those actively using IV drugs are at risk for acute acquisition of infections like HIV and HCV in addition to more common bacterial infections. Careful attention must be paid to ensure appropriate monitoring both pre- and post-transplant.

264 A rare case of nocardia peritonitis with suspected nocardia endocarditis

A Roy*

R James

J Johnsrud

A Kothari

R Bradsher

University of Arkansas for Medical Sciences, Little Rock, AR

Case report

Nocardiosis is commonly a disease of immunocompromised; however, one-third of infected patients are immunocompetent. Although Nocardia has the potential to disseminate, it most commonly affects lungs, brain, and subcutaneous tissues. We describe a unique case of nocardia peritonitis in a peritoneal dialysis (PD) patient with infective endocarditis and aortic valve perforation.

A 40-year-old patient with End-Stage Renal Disease (ESRD) on PD presented with abdominal pain and confusion. He was afebrile, tachycardic, abdomen was diffusely tender. Peritoneal fluid cell count was 18,190/uL with 88% neutrophils. Treatment was initiated with vancomycin, cefepime, metronidazole for peritonitis. HIV, hepatitis panel, Clostridium difficile, blood culture, fungal culture were negative. Body fluid culture turned out to be positive for gram-positive, weakly acid-fast filamentous bacteria. Antibiotics were changed to meropenem and trimethoprim-sulfamethoxazole (TMP-SMX) was added. The organism found to be Nocardia nova which was sensitive to both meropenem and TMP-SMX. Chest x-ray was negative for infiltrates or abscess. MRI brain showed multiple acute punctate infarcts. CT abdomen showed multiple splenic infarcts. Physical exam then revealed a decrescendo diastolic murmur at the left sternal border. Transesophageal echocardiography showed an 8x3 mm vegetation on the left coronary cusp of the aortic valve with leaflet perforation and severe aortic regurgitation, also a concern for aortic root abscess. He underwent aortic valve replacement with bioprosthetic valve. PCR and culture of aortic valve specimen were negative for Nocardia. He already received 2 weeks of antibiotics by then for which he responded well clinically. Meropenem was changed to ceftriaxone based on sensitivities. Intravenous ceftriaxone and oral TMP-SMX was continued for 6 weeks post-surgery and plan made to continue TMP- SMX alone for 3–6 months.

CNS and cardiac evaluation should be considered in patients with nocardiosis as it has an affinity to the CNS and both native and prosthetic heart valves. Majority of cases with Nocardia endocarditis resulted in valve replacement, despite being on the potent antibiotics. Early diagnosis and prompt initiation of proper antibiotics are necessary to prevent dissemination.

265 Isolated cryptococcuria as a manifestation of disseminated cryptococcosis- a diagnostic dilema

A Roy*

A Goel

Y Pandey

H Goraya

University of Arkansas for Medical Sciences, Little Rock, AR

Case report

Disseminated cryptococcosis is a rare, life-threatening infection commonly seen in HIV patients. It is infrequently seen in immunocompetent patients. Pulmonary and central nervous system are the commonly involved sites of infection in an immunocompromised host. Here, we present a rare case of isolated cryptococcuria as a manifestation of disseminated cryptococcosis in a non- HIV patient.

A 71-year-old patient with a history of hypertension, diabetes, ulcerative colitis on steroids presented with altered mental status, vomiting, headache, shortness of breath for a month. On admission, he was having high-grade fever and was hypoxic. Lab studies showed anemia, leukocytosis, acute kidney injury and urinary tract infection. CT head was negative for acute process. CT chest showed multifocal pneumonia. His clinical condition deteriorated despite being on broad-spectrum antibiotics. Blood culture was negative. Fungal culture, urine histoplasma antigen, blastomycosis antigen were negative. Fungal Beta-glucan was elevated. His urine culture grew Cryptococcus neoformans. Lumbar puncture was not consistent with cryptococcal meningitis, opening pressure was normal, gram stain, culture were negative. CSF cryptococcal antigen was negative. In the setting of respiratory failure, multifocal pneumonia and immunosuppression from chronic steroid use, we made the presumptive diagnosis of disseminated cryptococcosis, even though his serum cryptococcal antigen was negative initially. Serum cryptococcal antigen was repeatedly tested and was finally turned out to be weakly positive. He improved drastically after starting amphotericin and flucytosine. After two weeks of therapy, antifungals changed to fluconazole and he was discharged with a plan of continuing fluconazole for a total of one year.

Disseminated cryptococcosis is very rare in non-HIV patients. Isolated cyptococcuria can be a manifestation of disseminated infection. A high index of clinical suspicion is needed as it can be easily missed in non-HIV and immunocompetent patients and delayed diagnosis leads to unfavorable outcomes.

266 Trying to stomach extrapulmonary tuberculosis

R Strength1,2*

A Broadnax1,2

N Hysmith1,2

1University of Tennessee Health Sciences Center, Memphis, TN

2Le Bonheur Children’s Hospital, Memphis, TN

Case report

E.M. is a 2-year-old previously healthy Latino male who initially presented to Le Bonheur Children’s Hospital in Memphis, TN with 2 weeks of fever. His parents also reported intermittent abdominal pain, diarrhea, and anorexia. Prior to admission, the patient had completed a course of amoxicillin-clavulanate for suspected pneumonia as well as ciprofloxacin due to history of typhoid exposure. Despite these treatments and scheduled antipyretics, his fevers persisted for a total of 16 days prior to admission. Parents denied any recent tick or tuberculosis exposure, but they did endorse recent proximity to the United States-Mexico border as well as unpasteurized cheese intake. He was born in the US and had never traveled internationally. Upon admission, we obtained extensive workup. Labs showed non-specific signs of infection with leukocytosis and elevated C-reactive protein. Given gastrointestinal symptoms with no discernable cause, abdominal ultrasound was obtained—a large mass was quickly visualized. CT chest, abdomen, and pelvis then revealed a 6 cm right retroperitoneal mass and scattered pulmonary nodules. PPD and AFB gastric aspirates were positive. A biopsy during EGD ultimately confirmed the diagnosis of Mycobacterium tuberculosis complex (MTC).

MTC comprises a slew of Mycobacterium species including organisms like M. tuberculosis, M. africanum, and M. bovis. MTC cases are uncommon in the United States, but immunocompromised patients and patients from areas with high tuberculosis burden are at increased risk. In 2018, the CDC reported an incidence of about 2.8 cases of MTC per 100,000 persons. Extrapulmonary tuberculosis is rarer still—in 2016, it accounted for only about 20% of MTC cases in the US. This patient encounter demonstrates the importance of knowing the varied presentations of tuberculosis, how to diagnose it, and how to treat it.

267 Frequency and severity of cranial ultrasound abnormalities in a cohort of newborns molecularly screened for congenital cytomegalovirus infection

CR Thompson1,2*

HL Cohen2

E Meals1,2

R Tomlinson1,2

L Harrison1,2

C Lane2

K Fan2

M Carrillo-Marquez2

SR Arnold2

J DeVincenzo1,2

1CFRI, Memphis, TN

2UTHSC, Memphis, TN

Purpose of study

Infants congenitally infected with cytomegalovirus (cCMV) may develop permanent neurological sequelae, but most are asymptomatic at birth. Cranial ultrasound (cUS) can determine gross brain abnormalities in newborns that may indicate silent cCMV damage.

Methods used

To describe cUS abnormalities in infants with cCMV, a retrospective analysis was performed on cUS obtained from 60 known cCMV-infected infants identified by universal newborn CMV screening. For blinding, we randomly added controls. A single radiologist, blinded to cCMV diagnosis and previous cUS reading, evaluated each subject’s first cUS, and systematically categorized any hyperechoic areas in the basal ganglia and/or thalamus using a grading scale (0–3) of lenticulostriate vasculopathy (LSV). Other abnormalities and the presence of classic LSV defined by the radiologist were noted.

Summary of results

91.7% (55/60) of cCMV-infected infants had some LSV hyperechoic finding (15/55 LSV grade 1, 23/55 LSV grade 2, 17/55 LSV grade 3, 8/55 classic LSV). 90% (27/30) of classically asymptomatic cCMV infants had some LSV finding. A significant difference was found between viral load Ct value and presence of classic LSV (p=0.007). 12/30 (40%) infants with classic symptoms of cCMV had grade 3 vs 5/30 (16.7%) of those with asymptomatic cCMV (p=0.084). 10/20 (50%) of symptomatic newborns detected by routine evaluation had grade 3 vs 7/40 (17.5%) of asymptomatic infants (p=0.014). The kappa values for IRR between our reader and the original report for cCMV-infected (n=60), controls (n=27), and both groups combined (n=87) were 0.373 (fair), 0.471 (moderate), and 0.401 (fair), respectively. Significant differences were found between viral load copy number and having a LSV grade ≥2 (p=0.033).

Conclusions

A high percentage of asymptomatic and symptomatic cCMV infants have some degree of abnormal LSV hyperechoic findings along the lenticulostriate vessels on cUS. Viral load correlates to more hyperechoic findings and the presence of classic LSV on cUS of cCMV infected infants. Adopting a grading system for LSV may improve accuracy of cUS in cCMV infected newborns. Whether LSV predicts later neurologic cCMV sequelae is unclear.

268 Significantly elevated procalcitonin in setting of hepatitis C virus induced cryoglobulinemia and septic shock

T Thorne*

A Burke

LS Engel

N Masri

LSU Health Sciences Center, New Orleans, LA

Case report

Procalcitonin (PCT), first described as a marker of sepsis in 1993, is a biomarker that over the past twenty years has been increasingly studied as a diagnostic tool in clinic medicine. PCT is well studied in bacterial and fungal infections, but can also be elevated in patients with noninfectious processes such as autoimmune disease, trauma, and rhabdomyolysis.

Case

A 25 year old man presented with a history of IV heroin use presented to the Emergency Department with generalized body aches, fevers, dark urine, left hand swelling, and bilateral plantar foot pain and discoloration of 2 days duration. He was tachycardic (122 beats/min), hypotensive (83/51 mmHg) and tachypneic (22 breaths/min). He was toxic appearing, with active rigors. He had no heart murmur but he did have bilateral plantar non-blanching/non-palpable purpura of his feet and toes. Initial labs were significant for a white blood cell count of 20.71, platelet count of 104, a creatinine of 3.9, bicarbonate of 17, and lactic acid of 3.8. He was started on broad spectrum antibiotics for presumed endocarditis and levophed for pressure support. However, echocardiogram was unremarkable. Blood cultures grew Pseudomonas aeruginosa. He had a markedly elevated procalcitonin level of 190.70 ng/ml. He was hepatitis C positive with cryoglobulinemia. His symptoms resolved with antibiotics, plasma exchange, rituximab, and steroids.

Discussion

The initial extreme elevation of PCT was thought to be due in part from sepsis, but largely attributed to underlying cryoglobulinemia. Although PCT is most frequently elevated in bacterial and fungal infections, there is an increasing amount of evidence of elevated PCT levels in noninfectious disease processes such as, pancreatitis, rhabdomyolysis, heatstroke, cardiogenic shock, trauma, and autoimmune disease.

269 A classic presentation of legionnaire’s disease in a 73 year old patient

H Tran*

C Gregg

P Lowery

E Peirce

K Wilson

LS Engel

R McCarron

LSU Health Sciences Center, New Orleans, LA

Case report

Introduction: Legionella pneumonia accounts for 2–10% of community acquired pneumonias with over 75% of cases occurring in patients older than 50 years of age.

Case

A 73 year old woman with type 2 diabetes mellitus on insulin, hypertension, obstructive sleep apnea, and hypothyroidism presented with a 2-day history of fatigue, rigors, chills, non-bloody diarrhea, and a non-productive cough. On evaluation, her temperature was 100.8 F, BP 155/68, Rate 70s, O2 Sats 96% on 2LNC. Physical exam was remarkable for bibasilar crackles, dullness to percussion in the lower lobes, and the presence of diarrhea. Laboratory studies were notable for Sodium 132 mmol/L (baseline 135–136), Chloride 91 mmol/L, BUN 29 mg/dL, Cr 2.0 mg/dL, WBC 16.14 K/uL, normal lactate, and normal liver enzymes. CXR showed a retrocardiac opacity concerning for aspiration or pneumonia. CT Abdomen & Pelvis showed mild hepatosplenomegaly and a patchy consolidative change concerning for pneumonia or aspiration. She was was started on ceftriaxone and azithromycin. Overnight, she began having fevers up to 103F and antibiotic coverage was broadened to piperacillin-tazobactam plus azithromycin to cover for aspiration pneumonia and legionella. Her clinical state improved over the next few days and her antibiotics were de-escalated to clindamycin and azithromycin. Legionella urine antigen returned positive.

Discussion

Legionnaire’s Disease is often misdiagnosed due to the clinically and radiographically similar presentation to other forms of pneumonia. Although patients may present with similar symptoms such as fever, cough, and fatigue; certain features such as gastrointestinal symptoms of nausea, vomiting, diarrhea as well as laboratory findings such as hyponatremia and transaminitis should raise the suspicion for Legionella. Studies have shown early diagnosis and appropriate antibiotic management is associated with improved outcomes; which makes it important to consider Legionnaire’s Disease.

270 Ludwig’s angina: an extravaginal infection caused by gardnerella vaginalis

H Tran*

AL Heifner

J Dubuc

B Mock

LS Engel

K Happel

LSU Health Sciences Center, New Orleans, LA

Case report

Introduction: Ludwig’s angina is defined as a bilateral infection of the submandibular space, which commonly arises from a dental infection. This is an aggressive, rapidly spreading cellulitis, and if not treated in a timely manner can lead to respiratory compromise.

Case

A 61-year-old man presented to an outside hospital with a week of progressive tooth pain and neck swelling. Prior to presentation, a dentist evaluated him but due to swelling, no intervention was performed. Despite penicillin, his pain and swelling had progressed, and he was unable to swallow. In the Emergency Department, he was found to be in respiratory distress. He was resuscitated and successfully intubated. He received vancomycin, piperacillin-tazobactam, and clindamycin. A CT head and neck showed pneumomediastinum and multiple abscesses. He underwent incision and drainage of the bilateral submandibular space, sublingual space, and submental space with extraction of teeth. Wound cultures grew Gardnerella vaginalis and parvimonas micra. Over the next month, his hospitalization was complicated by renal failure requiring intermittent hemodialysis, tracheostomy placement due to compromise of his upper airway, pleural catheter placement for a right sided pleural effusion concerning for an empyema. Additionally, patient was started on pleural lytics due to concerns for mediastinitis with abscess formation revealed on repeat CT imaging. He ultimately required a 2nd catheter placed in his right pleural space, which was complicated further by a pneumothorax. He slowly improved and was eventually discharged on day 36 of hospitalization to an LTAC to continue rehabilitation.

Discussion

Our patient’s condition progressed rapidly from dental pain to respiratory compromise, illustrating the importance of dental health. With a mortality rate of up to 8%, prompt intervention is required for suspected Ludwig’s Angina. Treatment includes airway management, antibiotics, and surgical drainage for complicated deep neck infections.

271 Paraspinal thoracic mass with histology strongly suggestive of cat scratch disease

HL Valdin1*

C Udemgba2

C Sandlin1

1LSU Health Sciences Center, New Orleans, LA

2Tulane School of Medicine, New Orleans, LA

Case report

Cat Scratch Disease (CSD) typically presents as self-limited, tender lymphadenopathy following inoculation with Bartonella henselae from an infected cat bite or scratch. The disease manifests histologically with granulomatous inflammation and a hallmark description of central stellate micro-abscesses. Warthrin starry staining confirmation occurs in approximately two-thirds of cases. Several atypical presentations of CSD have been described, including ones with neurologic manifestations and visceral involvement. We present the case of a patient with a right thoracic paraspinal mass. Pathologic findings were suggestive of Bartonella henselae infection.

A 10-year-old male presented with abnormal chest imaging at an outside clinic. He complained of a dry cough, fevers, fatigue, and increased work of breathing associated with a 15 pound weight loss. A posteriorly-located right lower lobe mass was noted on chest X-ray and was confirmed with CT imaging. The initial differential diagnosis included malignancy, bronchopulmonary sequestration, and infection. MRI of the chest revealed a heterogeneous right posterior paravertebral soft tissue mass most consistent with tumor of neurogenic origin or granulomatous infection. Quantiferon Gold was obtained, and a PPD was placed to rule out tuberculosis (TB).

A tissue sample was collected via core needle biopsy. Preliminary biopsy results were negative for malignant cells. Quantiferon Gold and PPD were negative. Final pathology report revealed stellate microabscesses and granulomatous inflammation with epithelioid histiocytes. These findings were all suggestive of CSD. Warthrin starry stain was negative. The family later reported that the patient frequently played with a kitten at his grandparents’ home. Diagnosis was unable to be confirmed by serology.

This case highlights the importance of keeping a broad differential diagnosis in patients with atypical presentations. Although neoplasm is initially suspected in a large percentage of atypical cases of CSD, it is important to explore other potential diagnoses through further lab testing and consultation with an infectious disease specialist when granulomatous inflammation is a consideration – as was the case in our patient.

272 Teeming with treponemes

O Van Gerwen*

CA Muzny

University of Alabama at Birmingham, Birmingham, AL

Case report

True to its nickname ‘the great imitator’, syphilis is easily mistaken for other diagnoses, especially in HIV+ patients. Our case of secondary syphilis presenting as oral mucous patches was initially mistaken for thrush.

A 36yo HIV+ man presented to clinic with persistent oral plaques. He was seen in clinic one week prior and treated with fluconazole for oral thrush. His HIV was well-controlled on antiretroviral therapy (CD4 619, HIV VL 44 copies/mL). He reported condomless oral and anal receptive sex with two new male partners over the past month. Upon representation to clinic, exam revealed adherent, serpiginous, white patches on the hard palate and buccal mucosa (figure 1). These were unchanged despite taking oral fluconazole. RPR drawn at his previous visit resulted as 1:128 during our clinic visit. Given his well-controlled HIV and failure to improve with antifungals, the oral lesions were thought to be mucous patches from secondary syphilis. He was treated with 2.4 MU IM benzathine PCN G and the lesions resolved.

Secondary syphilis presents in many ways, with a maculopapular full body rash most classically described. Oral manifestations like mucous patches are present in up to 1/2 of patients, but often overlooked by clinicians. These are described as ‘snail-track ulcers’ as they can coalesce together into elevated and often ulcerated plaques. A normal CD4 count made oral thrush unlikely in this patient. Consideration of his sexual history and elevated RPR titer were key in making the diagnosis of secondary syphilis.

273 A rare case of HLH triggered by MRSA

K Vedala*

M Keel

S Khan

A Kunnumpurath

K Kakkera

White River Medical Center, Batesville, AR

Case report

Hemophagocytic lymphohistiocytosis (HLH) is a rare disease that results due unregulated activation of the immune system. This activation can be because of multiple causes which are classified into Primary Familial HLH, Secondary HLH, and Malignancy-Associated Hemophophagocytic Syndrome (MAHS)[1]. Secondary HLH is usually a result of Immune activation through viral illnesses, most commonly EBV2. Here, we describe a case of HLH which was triggered because of MRSA bacteremia.

Patient is a 67-year-old male with history small cell lung cancer with Carboplatin and Etoposide, who was admitted to the hospital with fevers, mucositis, confusion, weakness and fatigue. He rapidly worsened with GI tract bleeding and septic shock requiring admission to ICU. He was initiated on Antibiotics and supportive therapy. Work up revealed hypertriglyceridemia (at 385), elevated ferritin (32,100) and severe pancytopenia. Both initial and repeat blood cultures (5/5) persistently grew MRSA raising suspicion for endocarditis.

A soluble IL2 receptor assay was sent and in view of high suspicion for HLH (met 4/5 criteria) he was initiated on high dose steroids. Despite aggressive measures including initiation of steroids for HLH, patient’s condition deteriorated and family decided to withdraw care with comfort measures only. He subsequently expired within the day. Post-mortem, an elevated soluble IL2 receptor (sIL2-r) at 22268 U/ml supports a diagnosis of HLH.

With this case we wish to draw attention to the fact that MRSA sepsis can be a trigger for HLH likely through reactivation of EBV. Although our patient was being treated with chemotherapy, his most recent cycle was four months ago and imaging from a month prior to admission showed no recurrence of disease. For this reason, secondary HLH triggered by MRSA-septicemia is suspected to be more likely than MAHS.

References

  1. Henter JI, Horne A, Aricó M, Egeler RM, Filipovich AH, Imashuku S, Ladisch S, McClain K, Webb D, Winiarski J, Janka G. HLH-2004: diagnostic and therapeutic guidelines for hemophagocytic lymphohistiocytosis. Pediatr Blood Cancer 2007 February;48(2):124–31. doi:10.1002/pbc.21039. PubMed PMID: 16937360

  2. Flipovich A, McClain K, Grom A. Histiocytic disorders: recent insights into pathophysiology and practical guidelines. Biol Blood Marrow TransplantJanuary 16, 2010.

274 Cryptococcal meningitis presenting with cranial nerve 6 palsy in a renal transplant patient

A Vellucci*

C Lopez

K Bateman

Tulane University School of Medicine, New Orleans, LA

Case report

Cryptococcus neoformans is an opportunistic fungal pathogen that infects immunocompromised individuals. With the advent of HAART, there has been an increase of cryptococcal meningitis in non-HIV infected patients, namely solid organ transplant (SOT) recipients. Patients who are HIV-negative or have non-typical symptoms may experience a delay in diagnosis, resulting in higher morbidity and mortality. Currently, Cryptococcus is the third most common invasive fungal infection in SOT recipients, with mortality reaching 50%. Therefore, it is imperative to have a broad differential diagnosis for SOT patients presenting with focal neurologic deficits (i.e. CN nerve palsy).

Case presentation

A 52yo African American woman with history of hypertension, CKD, and renal transplant presented with a 3-week history of headache and diplopia and had progressed to new onset imbalance, neck stiffness, vomiting, and blurry vision. MRI was unremarkable and an LP diagnosed cryptococcal meningitis (titer 1:320). She began induction therapy with IV amphotericin B liposome and flucytosine and was transferred to Tulane. Here, repeat antigen titer showed 1:160. Her hospital course was complicated by AKI, focal neurologic deficits, and persistently elevated LP opening pressures. As a result of her high fungal burden, persistent neurologic symptoms, and increased ICP, she required a longer length of induction therapy and VP shunt placement. Three days after VP shunt placement she began maintenance therapy with Fluconazole.

Discussion

The number of SOT recipients acquiring Cryptococcus is steadily increasing. As there is currently no prophylactic therapy, it is imperative we diagnose and begin treatment early. A more acute onset and a higher antigen level, as well as a presentation with less typical clinical manifestations has been noted in patients with T-cell suppression. Most patients are diagnosed within 17–28 months post SOT, however our patient presented 7 years after SOT. With internists normally being the first physicians to engage in a patient’s care, having an advanced organizer for diplopia (specifically CN6 palsy) can assist with correct diagnosis. With this case, we present an advanced organizer for diplopia (CN6 palsy) to assist with the differential diagnosis of CN6 palsy.

275 Free air in liberia

RM Walker1*

M Morrison1

J’ Walker2,3

1Oklahoma Baptist University, Shawnee, OK

2ELWA Hospital, Monrovia, Liberia

3Texas Tech, Amarillo, TX

Case report

A 62 yo M x ray technician came to ER in Liberia, West Africa co 2 day ho severe abdominal pain with severe rebound tenderness and w no bowel sounds.

Upright CXR showed large free air. He was taken to surgery and a 2 cm perf of the ilium was found repaired.

He recovered well.

As a senior radiology technician, he had taught the technician working in the ER that day!

Discussion

Typhoid fever is a public health challenge which is concentrated in impoverished areas of the developing world which is due to unsafe drinking water.

Typhoid intestinal perforation (TIP) is the most serious complication observed in 0.8–39%.

TIP mortality rate fluctuates from 5 – 80% due to many factors including access to care.

276 Rare cause of pulmonary empyema

U Wardan*

UF Health, Jacksonville, FL

Case report

60 year old male with past medical history of diabetes mellitus and end stage renal disease presented with respiratory distress. His blood pressure was 130/70, heart rate was 119/min, respiratory rate was 30/min and temperature was 100.8F. Significant laboratory findings included WBC count 3,000 with 10% bands and procalcitonin of 194. Computed tomography of chest revealed left fibrothorax and pleural effusion. He was admitted to intensive care unit for sepsis and hypoxic respiratory failure requiring intubation. Blood cultures and endotracheal aspirate returned positive for non-typhi salmonella. Transthoracic echocardiogram was negative for vegetations. Diagnostic thoracentesis was performed and showed LDH 20,000, protein 3.9 (total protein 7.8), 400,000 red blood cells and 378,000 white blood cells, with 100% neutrophils. Pleural fluid culture was also positive for non-typhi Salmonella. Cardiothoracic surgery was consulted and pleurectomy with thoracotomy decortication was performed. Tissue culture obtained intraoperatively was also positive for Salmonella. He was treated with Ciprofloxacin based on susceptibilities with subsequent improvement. He later reported few episodes of diarrhea before admission. However, stool culture was negative for salmonella likely as it was ordered after he had already been on antibiotics. Despite negative stool culture, our patient likely had Salmonella gastroenteritis, which led to bacteremia, seeding in his pleural space, with resultant empyema.

Salmonella is an anaerobic gram-negative bacilli which most commonly causes gastroenteritis. Other clinical manifestations include bacteremia, endocarditis, meningitis and very rarely, pulmonary empyema. Initial diagnostic workup for empyema includes stool cultures and blood cultures. Computed tomography scan of the chest with intravenous contrast should be ordered to evaluate for effusions and loculations. Diagnostic thoracentesis sample should be sent for LDH, glucose and protein. Sample should also be tested for aerobic, anaerobic, fungal and acid-fast bacilli cultures. Enteric Salmonella infection is generally susceptible to fluoroquinolones, trimethoprim-sulfamethoxazole, macrolides, and third-generation cephalosporins. In addition to antibiotics, treatment for empyema includes drainage by catheter thoracostomy or video assisted thoracoscopic surgery.

277 Lyme disease presenting as atrioventricular block

U Wardan*

H Patel

UF Health, Jacksonville, FL

Case report

A 21 year old male without any past medical history presented with chest pain. He reported substernal and non-radiating pain without any relationship to rest or exertion. He also complained of generalized myalgia and arthralgias. Electrocardiogram showed a first-degree heart block with a prolonged PR interval of 300 ms and an incomplete right bundle branch block. Cardiac enzymes were negative. Physical exam was notable for left facial droop. On further questioning patient reported that he had a rash on his right thigh with a noted ‘target’ appearance about 2 months ago which resolved on its own. He is an active member of the Navy and had recently visited Pittsburg and Pennsylvania. Lab workup was significant for positive Lyme IgG antibodies. Magnetic resonance imaging of the brain was unremarkable. Transthoracic echocardiogram showed mildly reduced systolic function with an ejection fraction of 45%. Cardiac MRI with stress was negative for ischemia, myocarditis, or fibrosis. He was diagnosed with disseminated Lyme disease, with cardiac and neurologic involvement. He was started on ceftriaxone with subsequent improvement of first-degree heart block as well as his left sided Bell’s palsy.

Lyme disease is caused by Borrelia which is a type of spirochete. It is most prevalent in the northeast region and is transmitted by Ixodes Ricinus ticks. Following exposure, patients first develop erythema migrans which is most commonly described as a bull’s eye rash. This may be accompanied with symptoms of fatigue and malaise. This can further progress to early disseminated disease which includes atrioventricular block, myopericarditis, facial palsy and lymphocytic meningitis. Late disseminated disease presents as monoarticular or oligoarticular arthritis. Diagnostic workup includes enzyme linked immunosorbent assay followed by western blot. Current guidelines recommend testing patients who have traveled to endemic areas, have been exposed to ticks and have findings concerning for disseminated disease. Localized disease can be treated with Doxycycline, Amoxicillin or Cefuroxime for 2 weeks. Early disseminated disease is treated with IV Rocephin, Cefotaxime or Penicillin. Patients will heart block should also be admitted for telemetry monitoring and pacemaker evaluation. Lyme associated arthritis is treated with oral Doxycycline or Amoxicillin.

Medical education/medical ethics/advocacy

Joint plenary poster session and reception

4:30 PM

Thursday, February 13, 2020
278 Breast is B.E.S.T. breastfeeding improvement through resident education and sustainability teaching

L Key*

JH Purvis

S Taylor

AJ Talati

University of Tennessee Health Science Center, Memphis, TN

Purpose of study

We sought to improve our rates of exclusive breastfeeding by surveying women delivering in our hospital to determine their impressions regarding breastfeeding and to use this to develop a specialized and targeted curriculum to teach pediatric residents in direct contact with post-partum mothers.

Methods used

Women admitted to the postpartum unit of our hospital were surveyed regarding the barriers they encounter when considering breastfeeding. After analyzing 500 surveys, we developed a targeted curriculum for residents rotating through the well baby nursery, that they in turn teach to post-partum women.

Summary of results

Residents now complete the AAP online breastfeeding modules, hear a lecture about breastfeeding concerns in our population, receive a hand-out on this topic to discuss with patients, and round with lactation consultants. We distributed surveys to residents and to post-partum patients to determine if the new curriculum was increasing their knowledge base. Of 162 breastfeeding women surveyed, 92% reported that they had decided on breastfeeding prenatally. 55% reported that they knew more about breastfeeding after the resident teaching and 20% reported that the teaching influenced their decision on breastfeeding.

Conclusions

Many women decide if they will breastfeed prior to giving birth which emphasizes the importance of encouraging breastfeeding prenatally. Both mothers and residents are likely to have their knowledge surrounding breastfeeding increased with a standardized breastfeeding teaching.

Abstract 278 Table 1

Resident opinions pre and post rotation Resident opinions pre and post rotation

Abstract 278 Figure 1

Rates of any and exclusive breastfeeding over time Rates of any and exclusive breastfeeding over time

279 Evaluation of procedural sedation curriculum in pediatric emergency medicine fellowships

MJ Sims*

B Jackson

O Titus

L Robinson

MUSC, Charleston, SC

Purpose of study

Pediatric procedural sedation has been performed with increasing frequency by pediatric emergency physicians over recent years. ACGME Pediatric Emergency Medicine fellowship core competency requirements do not specify the manner in which fellows should become proficient in pediatric procedural sedation. We surveyed the variety of training experience provided during fellowship and whether those surveyed felt their training was sufficient.

Methods used

A 35-question survey offered to pediatric emergency fellows and recent (within 10 years) graduates collected data on pediatric procedural sedation training during fellowship. A follow-up questionnaire was sent to fellowship directors at programs where fellow or graduate respondents stated that a sedation curriculum existed asked details of their program.

Summary of results

There were 95 respondents to the survey, 62% of which had completed PEM fellowship training. Of respondents, 43% reported having a formal sedation curriculum during fellowship. 54% of all respondents reported feeling comfortable in performing pediatric procedural sedation after formal training. 66.6% of responding programs include evidenced-based medicine literature review and 6/9 (66.6%) include simulation. Ketamine was the most frequently used agent for sedation (87%). Propofol use, both as a primary agent and adjunct, was limited (84% reported using the drug in less than 10% of sedations) and only 56% of respondents reported that they expect to feel or did feel proficient in propofol use upon completion of fellowship.

Conclusions

Pediatric emergency medicine fellowship requirements lack a clearly defined pathway for training in procedural sedation. Data collected from both current and former fellows depicts inconsistency in training experience and suboptimal comfort level in performing these procedures. We suggest that fellows receive a more comprehensive and varied experience with multiple teaching modalities in order to improve proficiency with this critical and complex aspect of emergency pediatric care.

Neurology and neurobiology

Joint plenary poster session and reception

4:30 PM

Thursday, February 13, 2020
280 Concurrent acute inflammatory polyneuropathy and transverse myelitis of unknown etiology in an adolescent with cerebral palsy

A Agarwal*

AF Bowman

Sacred Heart Hospital, Pensacola, FL

Case report

Background: Concurrent acute inflammatory polyneuropathy and transverse myelitis (TM) occurs very rarely in childhood. Only a few cases of these conditions occurring simultaneously have been reported and represent a diagnostic challenge. Both conditions should be suspected when, in addition to the symptoms of acute inflammatory polyneuropathy, urinary retention at the onset of symptoms and sensory level deficits are present.

Case presentation

17-year-old male presented with acute onset numbness and tingling starting in his feet that rapidly ascended to his legs with associated weakness and inability to ambulate. The numbness progressed leading to acute urinary retention and halted once sensory deficits reached the level of T4 with associated chest pain. He reported an episode of diarrhea preceding onset of symptoms, and a recent history of the family dog with diarrheal disease. Initial neurological examination was significant for decreased to absent sensation in both lower extremities with normal sensation above the nipple line. Patellar, ankle and abdominal reflexes gradually became absent. CSF analysis was suggestive of acute inflammatory polyneuropathy with 2 WBC/cumm, the high protein content of 183 mg/dL with a normal glucose level of 57 mg/dL, negative bacterial cultures, and elevated albumin and IgG. Blood immunology/serology and non-culture microbiology tests to evaluate for infectious etiology were negative. MRI spine with and without contrast revealed lesion concerning for transverse myelitis at C7 level. Treatment with steroids and IVIG improved the strength and sensation, and bladder control was eventually regained.

Conclusion

Overlapping cases of acute inflammatory polyneuropathy and TM without clear etiology represents a diagnostic challenge. Further reports are necessary to understand the pathophysiology and establish optimal treatments.

281 Positive ice pack test in patient with wound botulism

D Chachkhiani*

AJ Velander

C Smith

Louisiana State University, Metairie, LA

Case report

Wound botulism is a rare disease and if not diagnosed and treated early, can be fatal. Given the rarity of wound botulism, it is common to consider alternative diagnoses such as myasthenia gravis. The ice pack test is an evaluation tool for myasthenia gravis of debatable sensitivity and specificity. We describe a case where a patient with early botulism had a positive ice pack test.

A 33-year-old man with history of heroin abuse presented with a 2-day history of ptosis, diplopia, blurred vision, dysarthria, dysphagia, and dyspnea. An initial diagnosis of myasthenia gravis was entertained, and an ice pack test was performed. A dramatic improvement of ptosis was noted after the ice pack test on the day of admission. On the next day, the descending paralysis in the context of intravenous drug abuse and suspected skin abscesses changed the focus to wound botulism. Wound debridement and antibiotics followed by botulinum antitoxin were promptly given. An ice pack test was repeated without reproducible improvement in ptosis. One week after admission, botulism was confirmed by the Centers for Disease Control via PCR and a repetitive nerve stimulation was performed over the patient‘s right median nerve, which showed decrementing response consistent with neuromuscular disease that can be seen in botulism.

This is a rare case of wound botulism associated with intravenous heroin use and multiple skin abscesses. In this patient, a false positive ice pack test was demonstrated early in the disease. This argues that the specificity of the ice pack test to myasthenia gravis merits further evaluation.

282 A missed case of osteomyelitis

M Chavez*

F Abu-Farsakh

L Ali

R Pattabhi

TM Reske

EA Aguilar

LSU Health Sciences Center in New Oreans, New Orleans, LA

Case report

Non-hematogenous osteomyelitis occurs as a result of bone infection from adjacent soft tissue such as diabetic wounds or decubitus ulcers. Stage IV pressure ulcers can lead to osteomyelitis especially in the elderly costing millions of dollars to our healthcare system. Many times, these ulcers are preventable if they care detected in the early stages.

Case description

92-year-old Caucasian male nursing home resident has a history Parkinson disease, Alzheimer’s dementia was evaluated for right 3rd digit worsening redness for 2 weeks. Patient had a 2-month history of recent right-hand contracture. 3rd digit examination revealed a stage IV ulcer once adjacent 2nd finger was lifted. 3rd digit showed a 1 × 1 cm bone exposure on the 3rd digit where the recent contracture had been reported and also had erythema, discharge and tenderness to palpation. Patient’s vital signs were stable. After discussion with family, decision was made to send to wound care for father debridement and ID consult. The patient’s labs showed a normal WBC and elevated ESR at 70 and CRP at 58. The rest of the labs were unremarkable. X Ray showed acute osteomyelitis. No biopsy was obtained per ID recommendation. Patient’s hand was placed in a splint so that fingers would not rub on each other to cause blisters. Patient was treated with Bactrim DS twice a day for two weeks. His ESR/CRP levels showed improvement as well. Wound healed in 4–5 weeks.

Discussion

Non-hematogenous osteomyelitis can be preventable with frequent assessment of patient’s conditions, in our case a recent right-hand contracture. Non-hematogenous 0steomyelitis costs our healthcare millions of dollars each year, puts patients at high risk for re-admission, and increases morbidity/mortality. With good education to all nursing home staff members on dealing with new contractures, we can possibly decrease the incidence of non-hematogenous osteomyelitis.

283 Enzyme replacement therapy in hunter syndrome does not prevent ischemic stroke

J Cummock*

P Maertens

University of South Alabama, Mobile, AL

Case report

Enzyme replacement therapy (ERT) in Hunter syndrome (HS) has limited effect on neurologic, skeletal and cardiovascular lesions. Acute ischemic stroke is rarely reported in HS: it is difficult to recognize due to progressive mental regression and epilepsy. We report an 11 YO male with HS diagnosed at age of 4 years of age and treated weekly with ERT idursulfase (Elaprase) presented while awake with acute right hemianopia and right sided flaccid palsy. Brain MRI not only showed restricted diffusion in the cortex of the insular ribbon and left cerebral hemisphere as well as centrum semiovale in the left frontal periventricular white matter consistent with recent infarction. In addition, there was diffuse atrophy. MRA diagnosed of left M2 occlusion was too late for IV tPA or intra-arterial treatment. Septicemia due to port infection was thought to be a contributing factor. Extensive etiologic work-up was otherwise negative as demonstrated. Echocardiogram showed aortic root dilation and moderate aortic valve insufficiency. Our case suggests the importance of glycosaminoglycans in the activation of toll-like receptor 4 pathway as a cause of stroke in HS.

284 Cefepime-induced generalized triphasic waves: pathophysiological implications

V Losada*

JC Baity

MM Nguyen

EC Mader

LSUHSC, New Orleans, LA

Case report

Generalized triphasic waves (GTWs)–now known as generalized periodic discharges with a triphasic morphology–were historically viewed as a distinctive electroencephalographic (EEG) feature of hepatic encephalopathy. It is now clear that GTWs may occur in the EEG of patients with toxic, metabolic, post-anoxic, or epileptic encephalopathy. GTWs have also been detected during isolated seizures and during nonconvulsive status epilepticus (NCSE). Although certain characteristics of GTWs (e.g. blunted waves, low discharge rate, symmetry, reactivity) make NCSE less likely, no features alone or in combination can reliably distinguish between GTWs due to simple diffuse encephalopathy and GTWs due to NCSE.

Case report

A 78-year-old right-handed woman presented with a large right hemisphere infarct. Her hospital course was complicated by hemorrhagic conversion of the infarct and by aspiration pneumonia requiring intubation. Vancomycin and cefepime were started, the latter at a dose of 2 mg q12h. Ten hours after the first cefepime dose, she had a convulsion followed by isolated myoclonic jerks. EEG showed GTWs with anterior preponderance. Levetiracetam and valproate were administered. The lack of electroclinical improvement after 2 days prompted the addition of propofol. The GTWs started to break up at a propofol rate of 10 mcg/kg/min and GTWs were fully suppressed at 20 mcg/kg/min. A rate of 60 mcg/kg/min was required to maintain burst suppression at 12 bursts/min. When propofol was put on hold, the EEG reverted back to baseline showing the same GTWs. Cefepime was discontinued after 3 days and propofol was weaned off after 36 hours with no recurrence of GTWs. With resolution of encephalopathy, the patient was extubated and transferred to the floor.

Conclusion

Toxic encephalopathy and seizures, with or without GTWs, are well-known complications of cefepime therapy. Because cefepime is a direct antagonist of GABAergic neurotransmission, it is reasonable to hypothesize that epileptic mechanisms are responsible for generating and perpetuating cefepime-induced GTWs. In our patient, GTWs were easily suppressed by propofol and reemerged when propofol was put on hold. This finding supports the notion of GTWs as epileptiform discharges that represent ongoing NCSE.

285 Ischemic stroke following plasma donation- a case report

H Mallah*

J Makram

D Vangipuram

K Creed

J Abdelmalek

E Islam

Texas Tech University Health Sciences Center, Lubbock, TX

Case report

Plasmapheresis is a procedure that involves the separation of all cellular elements of blood. It is considered relatively safe and used frequently for donation and therapeutic purposes.

We report a rare case of a patient who developed a fatal ischemic stroke after plasma donation.

Case report

A 53-year-old male, known to have well-controlled hypertension, and light tobacco smoking, was transferred from the plasma donation center due to seizure, immediately after plasma donation. CT head without and CTA head/neck were negative. His labs showed Hemoglobin 19.5 g/dl, and serum creatinine 1.7 mg/dl, with baseline hemoglobin 15 g/dl, and serum creatinine 1.1 mg/dl, last done four months prior. He was intubated due to unresponsiveness and respiratory failure. Head MRI showed an extensive area of restricted diffusion involving cerebellar hemispheres, vermis and right occipital lobe, compatible with ischemic stroke in the distribution of the posterior cerebral arteries. Head CT scan 18 hours following the event showed extensive infarction in the cerebellar hemispheres, vermis, pons, midbrain, and right occipital lobe, with posterior fossa edema, obstructive ventriculomegaly, and inferior tonsillar herniation. His clinical evaluation was consistent with brain death.

Discussion

Even though Apheresis is a very safe procedure, fatal complications have been reported. They are extremely rare and can occur as a result of cardiac arrhythmia, adult respiratory distress syndrome, venous thrombosis, and hemorrhagic events. However, few cases of stroke were reported. These patients were found to have thrombophilia. Unfortunately, no thrombophilia workup was done on our patient due to his rapid demise.

His labs with relative polycythemia and acute kidney injury reflect volume depletion, likely due to the plasma donation, which is thought to have contributed to the stroke, as elevated levels of hematocrit is independent risk factor for stroke.

The timing strongly suggests that the procedure may have disturbed the homeostatic balance in favor of arterial thrombosis.

This case suggests that there may be an increased tendency to arterial thrombosis in donors following apheresis. Therefore, the effects of apheresis on the coagulation system should be studied thoroughly.

286 A curious case of internuclear ophthalmoplegia that neither infarction nor demyelination can explain

M Nguyen*

V Losada

EC Mader

LSUHSC-NO Department of Neurology, New Orleans, LA

Case report

Disruption of the medial longitudinal fasciculus (MLF) in the dorsal paramedian pons or midbrain will manifest as internuclear ophthalmoplegia (INO). The two most common causes of INO are brainstem stroke (BS) and multiple sclerosis (MS). Other diseases can also cause INO but these are rare. Thus, the most common mechanisms of MLF injury are infarction and demyelination. We present a case of INO in which neither infarction nor demyelination is sufficient to explain the patient’s clinical course and magnetic resonance imaging (MRI) findings.

Case report

A 42-year-old woman with well-controlled mild hypertension experienced a 20-minute episode of blurred vison in the left eye, horizontal gaze diplopia, dizziness, and mild gait imbalance. The symptoms resolved completely but reoccurred 8 hours later. She was diagnosed with left INO due to BS in the emergency room. CT head was normal. The next day, she was transferred to another hospital. MRI brain showed DWI hyperintensity in the left paramedian midbrain at the exact location of the left MLF. The lesion was not seen on ADC map, T2-weighted, FLAIR, and contrast-enhanced images. MRA brain and neck did not show arterial stenosis or occlusion. Repeat MRI brain 3 days later showed the same DWI hyperintensity with corresponding hyperintensity on T2 FLAIR images. Three days after onset of symptoms INO was barely detectable but the patient continued to experience diplopia with oblique gaze. Inpatient work-up was significant for anti-SSB/La autoantibodies. She received 3 doses of methylprednisolone 1g q24h intravenously. After the first dose, she was totally asymptomatic and neurologic exam was normal. She was discharged after 5 days on aspirin with plans to complete the work-up in the outpatient clinic.

Conclusion

BS can explain the initial 30-minute episode but not the 4-day time course of normalization and the absence of restricted diffusion on MRI. MS can explain the MRI findings but not the initial brief episode and the rapid time course of normalization. As an isolated immunologic finding, anti-SSB/La positivity is of uncertain clinical significance. A possible mechanism of MLF dysfunction in our patient is reversible intramyelinic edema in response to transient ischemia, neuroinflammation, or both.

287 Back to basics: history and physical examination in the diagnosis of a psychiatric emergency

JG Rodriguez Velez*

San Juan City Hospital, San Juan, PR

Case report

Neuroleptic malignant syndrome (NMS) is a rare potentially life-threatening iatrogenic reaction to antipsychotic drugs characterized by hyperthermia, altered mental status, autonomic dysfunction, and Parkinsonian-like muscle rigidity, and tremors. It has been associated with virtually all neuroleptics. Despite being a well describe condition, it can be easily overlooked. However, NMS should remain a critical consideration in the differential diagnosis of patients with fever and altered mental status because it carries a mortality as high as 10% if not promptly recognized and managed.

29-year-old male with past medical history of hepatitis C and schizophrenia brought to the urgency clinic from a psychiatric hospital due to hypoactivity. Transfer documentation explain patient had presented frequent episodes of aggressiveness in prior days for which multiple psychotropic drugs were given. Physical findings were remarkable for fever, tachycardia, upper extremities resting tremor and rigidity, as well as alter mental status. Laboratory workup was significant for elevated CPK but otherwise unremarkable. Head CT scan, brain MRI and lumbar puncture were inconclusive. In view of clinical findings concerning for NMS patient was admitted to the intensive care unit. After excluding other possible causes for symptoms, NMS was confirmed as offending etiology. All psychotropic agents were discontinued and supportive measures were followed. Neurology services concurred with diagnosis and recommended management with dantrolene, bromocriptine, carbidopa and levodopa. The patient began improving clinically and treatment was tapered once the patient was stabilized. He was then discharge back to psychiatric hospital to complete 10 days of Bromocriptine.

Diagnosing NMS represents a challenge for clinicians because of its low incidence and its presentation overlapping with multiple medical conditions which occur more frequently. It is often overlooked and, as in our case, its diagnosis tends to be one of exclusion. Maintaining awareness of the clinical features of NMS is crucial for early diagnosis and a complete history, including medication review, and physical examination remains the most important tools for diagnosis.

288 A treatable cause of progressive weakness

MH Roy*

Our Lady of the Lake, Baton Rouge, LA

Case report

Cobalamin, Vitamin B12, deficiency can be precipitated by any malabsorption syndrome or surgical alteration to the gastrointestinal tract. It may take years for cobalamin deficiency to manifest clinically and neurological manifestations may occur in the absence of hematological findings. Common neurological symptoms are symmetric paresthesias and gait disturbance. Children often present with nonspecific manifestations such as developmental delay, irritability, weakness, and failure to thrive. Early detection and correction of the deficiency allows for proper development and prevention of serious sequelae. We present a child who underwent gastrointestinal surgery as an infant, with a delayed diagnosis of Cobalamin deficiency.

An 11-year-old female with past medical history of short gut syndrome presented to the ED with lower back pain and leg weakness leading to difficulty ambulating without assistance. Physical exam revealed an unsteady gait and decreased strength in her lower extremities. Labs were significant for Hgb/Hct 7.6/24.0, MCV 91, ferritin 435.1 (range 7.1–140 ng/mL), and a negative lumbar spine x-ray. She was sent home after reported improvement following Ibuprofen and IV fluids. She returned to ED with persistent difficulty ambulating and worsening bilateral leg weakness. She had a normal MRI of her brain and spinal cord and normal CSF studies. Upon admission for further work-up, it was noted that the patient had decreased proprioception and ataxia. Notable labs were Hgb/Hct 7.5/22.8, MVC 95, Vitamin B12 <146 (213–816 pg/mL), methylmalonic acid 129 (<0.40 nmol/mL), and homocysteine level 147.2 (3.4–20.4 umol/L). Her exam and labs were consistent with vitamin B12 deficiency and she started daily IM injections of 500 mcg of cyanocobalamin. Her macrocytic anemia and neurologic exam began to improve after the initiation of therapy.

Vitamin B12 deficiency may happen at any time in life for patients who have undergone gastrointestinal resections during infancy or childhood. Laboratory work up including methylmalonic acid and homocysteine are adjuvant diagnostics to confirm suspected cobalamin deficiency. Pediatricians should consider Vitamin B12 deficiency, with or without macrocytic anemia, for any patient at risk of malabsorption in order to promptly diagnose and avoid symptomatic anemia, thrombosis and severe neurologic sequelae.

289 Gratification disorder in the setting of rett syndrome: two pediatric cases

K Singhapakdi*

P Maertens

University of South Alabama, Mobile, AL

Case report

Gratification disorder (GD) is a form of masturbatory behavior the exact mechanism of which is poorly understood. However, it is usually considered benign and self-limited. This case report presents two pediatric patients with Rett syndrome that were eventually diagnosed with gratification syndrome, which has never been reported. This is significant because GD may be an early sign of Rett syndrome.

The first patient presented at 35 months with paroxysmal stereotypic crossing of the right leg over the left with leg straightening and curling of the toes. During episodes, there was a vacant facial expression but eye movements were intact. Over the following 8 months, episodes increased in frequency and length and became more complex, frequently associated with breath-holding, body shivering and hand stereotypies. Concomitantly there was gradual loss of ambulation. The second patient presented at 12 months with paroxysmal, stereotypic straightening of both legs with toes curling down and frequent crossing of one leg over the other. There was occasional elevation of the legs. Change in facial expression and stereotypic hand twirling frequently accompanied these episodes. In both patients, MECP2 sequencing revealed a pathogenic mutation diagnostic of Rett syndrome. EEG showed no epileptiform activity during GD phenomena. We conclude that GD may be an early sign of Rett syndrome and can be associated with other lower extremities stereotypies.

290 Primary lateral sclerosis: enhancing diagnostic confidence by carefully inspecting magnetic resonance images

MY Soliman1*

EC Mader1

JP Fernandez2

LA Branch1

R El-Abassi1

1LSUHSC-NO, New Orleans, LA

2Hattiesburg Clinic, Hattiesburg, MS

Case report

Primary lateral sclerosis (PLS) is characterized by slowly progressive upper motor neuron (UMN) degeneration in the absence of lower motor neuron (LMN) involvement. PLS is suspected based on history, UMN signs on exam, and evidence of intact LMN function on electromyography (EMG). Other causes of UMN dysfunction must be excluded before diagnosing PLS. Although PLS is difficult to diagnose, magnetic resonance imaging (MRI) may provide additional evidence in favor of PLS.

Case report

A 65-year-old woman with a 1.5-year-history of slowly progressive leg stiffness, gait instability, and frequent falls was referred to our clinic. Her right leg became stiff first followed by her left leg 6 months later. Past medical evaluations, including brain and spine MRI and EMG were unrevealing. Examination of the right lower extremity revealed moderate spasticity with mild weakness of knee and plantar flexors, central foot drop, and Babinski sign. There was some spasticity in the left lower extremity but weakness was not detected. Upper extremity, cranial nerve, and bulbar functions were normal. Sensory loss and cerebellar signs were absent. Our workup, including EMG, CSF studies, autoimmune panel, tests to rule out stiff person syndrome and familial spastic paraparesis, and spine MRI, were also unrevealing. Her past brain MRI and the one we requested (~1.5 years after disease onset) were reported as normal. However, when we carefully reviewed the images, we noted focal areas of hyperintensity in the cerebral peduncles on T2-weighted and FLAIR images.

Conclusion

MRI can show focal signal abnormalities in the corticospinal tract during the early stage of PLS. The signal change can be subtle and easily overlooked. We carefully inspected our patient’s MRI and found T2 hyperintensities in the left and right cerebral peduncles. The location (corticospinal tract projection to the legs) and asymmetry (left>right) of the MRI lesions perfectly matched our exam findings of asymmetric UMN signs (right>left) in the patient’s lower extremities.

291 Genetic implications of familial chiari malformation: a case report

S Swinney*

O Garcia

J Lee

T Dang

M Amodeo

BC Baronia

Texas Tech University Health Science Center, Lubbock, TX

Purpose of this study

Highlight the prevalence of a large occurrence of familial Chiari I Malformation in a single family, suggesting a genetic predisposition to disease formation.

Methods used

This case report involves an analysis of various family members’ clinical presentation and MRI findings that led to a diagnosis of Chiari I Malformation (CM-I). Additionally, a literature review provides more information on CM-I pathogenesis, symptoms, and diagnosis.

Summary of results

Within one family, both parents and 3 out of their 4 biologic children were diagnosed with CM-I. The mother was a 43-year-old female with history of migraines, hypertension, nausea, bilateral shoulder pain, and numbness and tingling of both upper extremities. Her MRI showed 6 mm cerebellar tonsil extension below the foramen magnum, and she was subsequently diagnosed with CM-1 secondary to pseudotumor cerebri. The father was a 42-year-old with history of increasing neck and shoulder pain; his MRI showed a cerebellar descent of 4 mm. Three of their four sons were symptomatic and met CM-1 criteria. The second eldest child was a 22-year-old male who had a history of intermittent neck pain, headaches, and paresthesia of upper and lower extremities since middle school; MRI showed a 10 mm descent of the cerebellar tonsils. The third eldest child was a 21-year-old male with hypertension and presented with occipital headaches as well as chronic neck and shoulder pain; MRI showed cerebellar tonsils 6 mm below the foramen magnum. The youngest son was a 16-year-old who was incidentally found to have cerebellar tonsil herniation 12 mm below the foramen magnum on MRI. All four children were born without any congenital disorders and were healthy prior to CM-1 diagnosis.

Conclusions

While CM-1 is considered to be a sporadic condition, there are two genetic theories that postulate that CM-1 arises from defects in hindbrain segmentation or from collision between the caudally-directed cranial growth and rostrally-directed cervical growth. A case with such large clustering of CM-1 within one family has not been previously reported. It raises concern of a mutation induced by an exogenous teratogen that warrants further investigation.

292 Catastrophic simultaneous bilateral ischemic stroke in a young male

MT Torres Torres1*

KC Padilla Rodriguez1

JL Ayala Rivera1

G Torres Torres2

A Rojas Figueroa1

1San Juan City Hospital, San Juan, Puerto Rico

2Universidad Autonoma de Guadalajara, Guadalajara, Mexico

Case report

In adults younger than 45 years old, incidence of ischemic stroke (IS) ranges from 3.4 to 11.3/100,000 per year. Recent data reports that while overall stroke incidence is declining, there is an increased incidence in the young. Stroke in young adults has major social and economic impact as it impairs the ability to work during the peak of their most productive years.

A 24-year-old male with history of benzodiazepine, opioid, tobacco, and alcohol abuse was brought to the emergency department (ED) after being found unresponsive at home. Previous to event, patient consumed oxycodone, alprazolam and alcohol at a party. Opioid reversal agents were administered by EMS without response. In the ED, he had rapidly declining oxygen saturation and Glasgow Coma Scale of 7/15 for which he was endotracheally intubated and admitted to Intensive Care Unit. Initial head computed tomography (CT) was grossly normal. After 48 hours patient was extubated, but right sided hemiplegia and aphasia were noted. Repeated imaging showed worrisome findings of acute malignant infarction involving the anterior and middle cerebral territories of the left cerebral hemisphere and similar infarction areas contralateraly. He was successfully managed with osmotic therapy and emergent decompressive craniectomy. Transesophageal echocardiogram remarkable for patent foramen ovale. Initial hypercoagulability testing was positive for lupus anticoagulant. Patient made a good intrahospitalary recovery progress and was discharged to a skilled nursing facility to continue physical rehabilitation.

IS are classified as malignant when there is presence of space occupying cerebral edema that is severe enough to produce brain tissue shifts and herniation leading to rapid neurological deterioration. Less than 3% of the middle cerebral artery IS are classified as malignant, with up to 80% mortality rate in conservatively treated patients. Reported cases about simultaneous bilateral cerebral hemisphere ischemic events are very limited and cases with malignant features are even rarer. Awareness is warranted as we must expand research efforts regarding prevention, early recognition, and successful treatment on this fatal condition.

293 Vertebral artery dissection in a young patient secondary to cocaine use

H Yousuf*

A Mohammedzein

RI Hazam

G Kibuule

S Prakash

S Almas

TTUHSC, Amarillo, TX

Case report

Cocaine use is well known for its detrimental effects on the neurovascular system. To increase awareness, aid faster diagnosis, and treatment we report a case of vertebral artery dissection in a young, 39-year-old female with a history of cocaine use, which is a rare and preventable presentation of cocaine abuse.

Case description

A 39-year-old female with a history of migraine, presented with the two-week duration of progressively worsening headache, noted after using cocaine and cannabinoid. She had only minimal improvement with analgesics. Five hours before admission she developed left-sided hemiparesis and dysarthria, after snorting cocaine. On initial evaluation, blood pressure was 127/73 mmHg, other vitals were normal. The neurological exam revealed mild left side weakness, strength (4 out of 5) while cranial nerves, sensation, and coordination were intact.

Despite unremarkable MRI, CT Angiogram revealed the absence of intraluminal contrast within the right and left proximal mid V4 segments of the vertebral artery, consistent with acute or chronic occlusions/dissections with opacification of prominent right posterior communicating artery, retrograde opacification of the basilar artery and upper vertebral arteries with no significant stenosis involving the anterior circulation. She improved significantly with intravenous heparin and was discharged to home on warfarin with a target INR of 2–3. The exact mechanism by which cocaine causes dissection may be related to the stimulation of apoptosis in cerebrovascular smooth muscle cells, greatly reducing vessel wall integrity. Our patient‘s initial insult may have happened two weeks before this admission, but despite enough collaterals, as she continued to use cocaine, her disease progressed and led to complete dissection of the vertebral arteries.

Long term management aims at identifying and correcting risk factors by rehab and counseling programs. Currently, there is no strong evidence to suggest an added benefit of anticoagulation compared to antiplatelet therapy for secondary prevention. Physicians should be aware of intracranial arterial dissection as a rare complication of cocaine use to prevent morbidity and mortality. Persistent headache disproportionate to the physical findings is an important clue.

Pediatric clinical case reports

Joint plenary poster session and reception

4:30 PM

Thursday, February 13, 2020
294 Dehydration, intractable vomiting, and metabolic acidosis in a 6-year-old female

SZ Afzal*

M Martinez

Ochsner Louisiana State University Health Science Center, Shreveport, LA

Children with metabolic acidosis can present with an array of clinical signs and symptoms. It’s important to distinguish between high and normal anion gap acidosis. High-anion-gap metabolic acidosis often occurs in cases of ketoacidosis, toxic ingestion and renal failure. In their absence, further workup is required to exclude unlikely causes.

Case report

6-year-old female presented to the emergency room with a 2-day history of episodic non-bilious non-bloody vomiting. On initial presentation, she was neurologically intact, GCS 15, HR 132 and BP 124/78; >95%. Her episodes failed to resolve after oral and intravenous (IV) ondansetron which prompted further evaluation and admission. Laboratory was significant for a bicarbonate level of 14.2 mmol/l, anion gap of 23. A urinalysis demonstrated increased ketones 159 mg/dl. Toxicology panel including salicylates level, alcohol level and drug screening were negative. A review of possible ingestions were discussed. An electrocardiogram demonstrated sinus tachycardia. Abdominal x-ray was noncontributory. She received IV ondansetron and promethazine which failed to resolve her emesis. A nasogastric tube was placed. On hospital day 2 her emesis and acidosis resolved after aggressive fluid replacement and cause was attribuated to viral gastritis.

Conclusion

Metabolic derangements can be a common finding in pediatric patients. It’s important to incorporate history and physical exam along with interpretation of lab values to help identify the cause.Keep in mind that not all ‘common causes’ present in a similar manner.

Abstract 294 Figure 1

Electrolyte trend: Comparison of Sodium, Potassium, Chloride and Bicarbonate Electrolyte trend: Comparison of Sodium, Potassium, Chloride and Bicarbonate

295 A case of gianotti crosti syndrome associated with enterovirus

OA Akhiwu*

A Dixon

H Adib

R Steele

Tulane University School of Medicine, New Orleans, LA

Case report

Gianotti Crosti Syndrome (GCS), also known as papular acrodermatitis, is a syndrome involving an acute-onset rash following a viral infection. Historically, it was linked to Hepatitis B hence, defined by three cardinal manifestations: maculopapular rash, lymphadenopathy, and acute hepatitis. In the United States, GCS is no longer defined by these, as EBV is now the most common cause. Other viruses that are linked to the syndrome include CMV, enterovirus, coxsackie, adenovirus, and parvovirus, amongst others. The rash is a 1–10 mm symmetric, erythematous papules on the extensor surfaces of limbs, buttocks, and cheeks lasting 2–4 weeks. Management is supportive. This case study highlights an association of the syndrome with enterovirus.

Our case involves an 18-month-old, African American boy who presented with a worsening, non-pruritic, erythematous rash on both arms, thighs, and cheeks that had been present for 5 days after he was given Amoxicillin for Acute Otitis Media (AOM). When the rash erupted, Amoxicillin was discontinued due to concern for medication side effects. On presentation, he also had a fever, cough, and rhinitis. On physical exam, the patient’s left tympanic membrane was bulging, consistent with AOM. He was found to be positive for enterovirus and negative for CMV, EBV, and influenza with negative blood cultures. He was given IV fluids, antipyretics and restarted on amoxicillin for his AOM.

Identification of the cause of this rash was important as management varied by etiology. In this case, the patient’s AOM was sub-optimally treated due to concern for cutaneous drug eruption. The immediate identification of GCS could have prevented this. Also, if the patient was mistakenly diagnosed with Kawasaki disease, he may have received unnecessary IVIG. GCS is likely underdiagnosed due to the shift in the most common viral etiologies. GCS is a clinical diagnosis that only requires recognition of the characteristic rash.

296 Taser lodged in zygoma

M Asal*

R Butchee

University of Oklahoma Health Science Center, Oklahoma City, OK

Case report

3 yo M with taser probe lodged in face. Event unwitnessed, child found pulling at probe.

No known loss of consciousness/seizure/respiratory distress. Mother works at a jail.

Exam

vitals normal, awake/alert/stoic, 2-cm probe firmly lodged 90° to pre-auricular zygoma), regular rate & rhythm, CN II-XII intact, 2 × 0.5 cm linear abrasion to mid-frontal scalp.

Work-up

CK/troponins/EKG (normal); XR face.

Secondary risks associated with tasers introduce complexity in treatment. A shock was emitted but without witness of its impact. Tasers are easily available, delivering ranges of high-voltage, low-amperage current. With impulse exposure ≤15 seconds, adverse effects are unlikely, but there are reports of immediate cardiac arrest, arrhythmia, & tissue damage resulting from electric to thermal energy conversion (cutaneous burn, rhabdomyolysis, ocular injury, or neurologic damage.) Bone has the highest tissue resistance, increasing heat generation & potential damage, including facial/trigeminal injury in this case. Manifestations of neurologic damage can be delayed by hours to months. The taser was police-grade with toothed barbs, mechanically complicating removal from bone & increasing risk of structural damage with removal.

Abstract 296 Figure 1

Probe lodged in zygoma with barbs buried Probe lodged in zygoma with barbs buried

Abstract 296 Figure 2

X-ray AP facial bones showing penetrative depth of probe X-ray AP facial bones showing penetrative depth of probe

297 Vitamin B6 deficiency anemia in a pediatric patient on hemodialysis

R Babriwala*

S Patel

B Wilmore

M Jeroudi

R Baliga

LSU-Shreveport, Shreveport, LA

Case report

Vitamin B6 is a water soluble vitamin that functions as a coenzyme of erythroid specific 5-aminolevulinate synthase (ALAS2) which is involved in the synthesis of heme. Vitamin B6 deficiency can occur in patients with chronic renal insufficiency and end-stage renal disease, particularly those who require dialysis. Here we report a pediatric patient who developed an erythropoietin stimulating agent (ESA) resistant anemia that significantly improved following vitamin B6 supplementation.

A 16-year-old African American male with end stage renal disease secondary to obstructive uropathy, on chronic hemodialysis, experienced a decrease in his hemoglobin over a 3-month period from 11 to 6.5 g/dL, despite therapy with maximum dose of intravenous darbepoietin alfa (ESA) [0.9 mcg/kg/week]. His transferrin saturation was 41%, ferritin level 706 [80–388] ng/mL, mean corpuscular volume 87 [78–98] fL. His corrected reticulocytes count was 2.3% [0.2–1.8%]. Patient’s direct antiglobulin testing and stool for occult blood were negative. Additional laboratory findings were: Vitamin B12 635 [193–986] pg/ml; folate 8.4 [3.1–17.5] ng/mL; copper 1413 [665–1480] mcg/l; zinc 77 [60–120] mcg/dL and Ceruloplasmin 31.4 [15–30] mg/dL. PTH was elevated at 258 [9–69] pg/ml. Vitamin B6 level was low at 1.2 [5.3–46.7] ug/L. Bone marrow biopsy was normocellular (65%) with erythroid hyperplasia and rare dyserythropoiesis. Prussian blue staining showed increased iron storage. Supplemental Vitamin B6 (100 mg daily) was initiated and his labs improved to hemoglobin was 11.6 g/dL with transferrin saturation of 18%.

Vitamin B6 clearance is increased with standard hemodialysis and a further 50% increase in vitamin clearance is noted when receiving high flux high efficiency hemodialysis as seen in our patient. Vitamin B6 deficiency anemia should be considered in any pediatric patient on high flux hemodialysis who is not responding to standard ESA and iron therapy.

298 Not just your grandparent’s illness: a case of herpes zoster in an immunocompetent pediatric patient

A Balachandran*

A Meraj

A Wahba

H Huh

University of Texas Health Science Center Houston, Houston, TX

Case report

Introduction: Herpes zoster (HZ) is a well-known viral infection to commonly affect the elderly; however, it can also happen in the pediatric population, accounting for only 1% of all zoster cases - most of which have a history of Varicella infection. It presents as a vesicular rash in a unilateral dermatomal distribution and is caused by reactivation of the Varicella zoster (VZ) infection that remains dormant after the Varicella infection or vaccine. When HZ presents in a pediatric patient, the clinician should consider an underlying immunodeficiency or malignancy as it more commonly occurs in an immunocompromised state. However, there have been reported cases of HZ occurring in the immunocompetent child.

Case presentation

An otherwise healthy 9-year-old Hispanic female with no past medical history presented to our ED with an acute onset of painless left-sided vesicular facial rash and oral lesions 3 days prior to admission associated with fevers and decreased oral intake. The patient’s mother and patient denied history of Varicella, and immunizations were up-to-date. On exam, the patient‘s lesions followed a clear dermatomal distribution of V2 and V3 along the left side of her face and the oropharynx. There was no sign of bacterial superinfection or optical involvement. Given concern for possible Herpes simplex virus (HSV) versus VZ infection, she was started on acyclovir. She was evaluated by a dermatologist who agreed with the differential. Polymerase chain reaction (PCR) of patient’s vesicles confirmed VZ infection. She was discharged on a 7-day course of oral acyclovir.

Conclusion

Children developing HZ without a history of Varicella can be explained by a mild episode of chickenpox going unnoticed by the parent, acquiring a primary Varicella infection in utero or in infancy, or reactivation of the live attenuated virus administered in the varicella vaccine. In general, the course of the disease is milder in children with complete resolution in approximately 2–3 weeks, often without symptoms of acute sharp pain which is the hallmark of HZ in adults. Although HZ is a rare disease in childhood, it should be considered in the differential diagnosis of vesicular eruptions, even in children without an underlying immunodeficiency or malignancy.

299 A curious cranial collection in a child

J Becton1*

C Marbrey1

S Depaepe2

MN Frascogna1

1University of Mississippi Medical Center, Jackson, MS

2University of Mississippi, Jackson, MS

Case report

Common causes of scalp swelling in neonates often presents as cephalohematomas, caput succedaneum or subgaleal hematomas due to birth trauma. In young infants, however, scalp swelling in the absence of a defined traumatic event is not as common. We present a case of a healthy 10-week-old infant who presented to the pediatric emergency department (PED) for a spontaneous, rapidly expanding scalp swelling with no known history of acute trauma.

The patient was a term female born via urgent cesarean section (C-section) secondary to prolonged rupture of membranes and maternal exhaustion. The C-section was complicated by deep engagement in the vagina requiring hand ‘push technique’ from the vaginal canal and a ‘T’ hysterotomy incision. No instrumentation or scalp electrodes were used. She was intubated for poor respiratory effort and had an exam notable for moderate cranial molding with caput succedaneum. After a brief stay in the neonatal intensive care unit she was discharged. There was no scalp swelling at the time of discharge.

She presented to the PED due to the sudden emergence of a fluid filled collection underneath the scalp that initially appeared at 8 weeks of age and had rapidly expanded over the previous week. The collection was soft, mobile, positionally dependent, easily trans-illuminated, and ‘rippled’ with gentle palpation. Neurologic exam was appropriate and without deficits. Computed tomography showed a 9-centimeter fluid collection at the convexity of the scalp with radiodensity consistent with cerebral spinal fluid (CSF). The fluid collection crossed midline and multiple sutures without signs of fracture, intracranial communication or congenital anomalies. Imaging and physical exam were consistent with a sub-aponeurotic fluid collection (SFC) composed of CSF.

SFC’s are a rare and poorly understood phenomenon that occurs at around 2 months of age. Though postulated to occur secondary to birth trauma associated with vacuum assisted delivery, microfractures in the skull, scalp electrodes, or CSF leak, the condition is benign. Clinicians should be aware of this condition so as to avoid unnecessary procedures and testing.

300 Hypertrophic cardiomyopathy: role of gene-dose effect on disease severity

MH Bell*

K Gajewski

R Zambrano

LSUHSC, New Orleans, LA

Case report

Hypertrophic cardiomyopathy (HCM) is a genetically heterogeneous disorder characterized by unexplained enlargement of the myocardial wall in the absence of other cause. Familial hypertrophic cardiomyopathy tends to occur in the interventricular septum. Several genes have been associated with HCM, but MYH7 and MYBPC3 are responsible for most cases.1,7

We report a 19-year-old female with multiple congenital cardiac malformations including pulmonary valve stenosis, atrial septal aneurysm and defect, and membranous VSD. Later in life she had moderate ventricular septal hypertrophy with mild dysfunction complicated by significant restrictive physiology and 1st-degree AV block. Her family history is negative for heart-related disease. Molecular testing identified a homozygous likely pathogenic variant in MYH7 (c.1727 A>G; p.His576Arg). Additionally, two heterozygous variants of uncertain significance were found in FLNC and LMNA.

Pathogenic variants in MYH7 are associated with different phenotypes including cardiomyopathies and skeletal muscle diseases4.

The variant identified has been reported in the literature in individuals with HCM and in one HCM relative with myocardium deficiency2,3,5,6,8. Pathogenic variants in MYH7 are usually inherited in an autosomal dominant manner, but the proband is homozygous for the variant in MYH7. On examination, the proband was found to have mild myopathic face and joint hypermobility. This patient’s phenotype demonstrates a severe cardiac muscle pathology with possible mild skeletal muscle involvement.

Identification of a homozygous variant in MYH7 is likely the explanation to her presentation. Homozygosity may have a dose-dependent effect on severity of disease. Further evaluation of the patient’s family may help determine if this likely pathogenic variant in MYH7 in a heterozygous carrier causes mild, asymptomatic, or no form of this disease.

Pathogenic variants in FLNC and LMNA are responsible for mostly AD and some AR skeletal and cardiac muscle disorders, the variants identified in the proband were classified as VUS. Their significance and contributions to the phenotype is unknown.

References

  1. Alfares AA, Kelly MA, McDermott G, et al. Results of clinical genetic testing of 2,912 probands with hypertrophic cardiomyopathy: expanded panels offer limited additional sensitivity. Genet Med 2015;17(11):880–8. doi:10.1038/gim.2014.205

  2. Berge KE, Leren TP. Genetics of hypertrophic cardiomyopathy in Norway. Clinical Genetics 2014;86(4):355–360. doi:10.1111/cge.12286

  3. Homburger JR, Green EM, Caleshu C, et al. Multidimensional structure-function relationships I human β-cardiac myosin from population-scale genetic variation. PNAS 2016;113(24):16701–6. doi:10.1073/pnas.1606950113

  4. Lamont PJ, Wallefeld W, Hilton-Jones D, et al. Novel mutations widen the phenotypic spectrum of slow skeletal/β-cardiac myosin (MYH7) distal myopathy. Hum Mutat 2014;35(7):868–79. doi:10.1002/humu.22553

  5. Michels M, Soliman OI, Phefferkorn J, et al. Disease penetrance and risk stratification for sudden cardia death in asymptomatic hypertrophic cardiomyopathy mutation carriers. Eur Heart J 2009;30(21):2593–8. doi:10.1093/eurheartj/ehp306

  6. Perrot A, Schmidt-Traub H, Hoffmann B, et al. Prevalence of cardiac beta-myosin heavy chain gene mutations in patients with hypertrophic cardiomyopathy. J Mol Med 2005;83:468. doi:10.1007/s00109-005-0635-7

  7. Richard P, Isnard R, Carrier L, et al. Double heterozygosity for mutations in beta-myosin heavy chain and in the cardiac myosin binding protein C genes in a family with hypertrophic cardiomyopathy. J Med Genet 1999;36(7):542–5

  8. Walsh R, Thomson KL, Ware JS, et al. Reassessment of Mendelian gene pathogenicity 7,855 cardiomyopathy cases and 7,855 cardiomyopathy cases and 60,706 reference samples. Genet Med 2017;19(2):192–203. doi:10.1038/gim.2016.90

301 A cold case of risperidone induced hypothermia and multi organ failure treated with veno-venous extracorporeal membrane oxygenation

K Bohannon*

S Mathew

C Watson

Augusta University, Augusta, GA

Case report

Atypical antipsychotics are being increasingly prescribed in pediatric populations for a variety of mood and mental health illnesses. Side effects range from mild to severe. The 3 most common adverse effects of risperidone use are weight gain, extrapyramidal symptoms and daytime hypersomnia. Hypothermia is a rare side effect of risperidone with one adult case report leading to cardiac arrest. To date one pediatric case report demonstrates a case of mild hypothermia associated with risperidone without significant clinical sequelae. We report a rare case of a 15-year-old on risperidone therapy presenting with severe hypothermia and acute hypoxic respiratory failure and cardiac arrest with progression to multi-organ failure necessitating VV ECMO.

A 15 year-old male with a history of severe autism presented to an outside facility with acute encephalopathy and respiratory failure. On initial presentation he was moderately hypothermic to 29 Celsius with deterioration in neurologic status and progressive hypoxemia requiring endotracheal intubation. Chest radiograph demonstrated opacification consistent with pulmonary edema. Patient was actively rewarmed with persistent hypoxemia refractory to conventional mechanical ventilation. High frequency oscillatory ventilation attempted resulting in 1 minute of ventricular fibrillation with return to sinus bradycardia following chest compression. Due to refractory hypoxemia and failed attempt at HFOV patient was cannulated for VV ECMO in the right IJ with a 28Fr ECMO catheter. Upon obtaining a more detailed history it was appreciated that patient’s dose of risperidone was increased 2 weeks prior. Following this increased dose he was noted to become ‘unlike himself,” refusing to ambulate and attempting to climb into a warm bath indicating a more insidious set of symptoms as compared to the abrupt nature of encephalopathy and hypothermia that was initially appreciated. Patient was decannulated on ICU day 8, successfully extubated on ICU day 11 and transferred to the floor on day 13.

Hypothermia is not a common adverse effect of risperidone however prescribers and clinicians should be aware of this life-threatening drug reaction. A thorough history should be obtained including dose, duration and titrations of these medications.

302 Niemann-pick type a presenting as failure to thrive

M Boothe1*

R Kassel1

M Descartes1

J Dean1

J Dennis2

A Hurst1

1University of Alabama at Birmingham, Birmingham, AL

2Children’s of Alabama, Birmingham, AL

Case report

Failure to thrive (FTT) is a common pediatric symptom, accounting for 1.4–5% of pediatric hospitalizations annually. FTT is multifactorial and can be caused by both non-organic and organic etiologies. Laboratory investigations rarely reveal a medical cause for FTT and are reserved for cases in which an underlying etiology is suspected based on the child’s history or physical exam. Here we discuss an 8-month-old female who presented to gastroenterology for FTT. She had previously been well until an episode of gastroenteritis resulted in weight loss at 4 months old. After, she had feeding difficulties and did not regain weight. At presentation, she had hypotonia and motor delays attributed to malnutrition. She was admitted to the hospital, at which time she was below the growth chart for weight and height with a normal head size. Additional evaluation revealed mild transaminitis, hepatosplenomegaly, and decreased white matter bulk on brain MRI. On physical exam she had frontal bossing, bitemporal narrowing, and a prominent occiput. Genetics was consulted and advised screening for mucopolysaccharidosis, peroxisomal biogenesis disorders, and chromosome anomalies, which returned with normal findings. Gastrostomy tube was placed and weight gain improved; however, her transaminitis and hepatosplenomegaly persisted, and she developed tachypnea with signs of interstitial lung disease on X-ray. At one year of age she underwent genome sequencing which returned with two pathogenic variants in SMPD1, consistent with a diagnosis of Niemann-Pick Type A (NPA). NPA is a rare lysosomal storage disorder caused by a deficiency of acid-sphingomyelinase and accumulation of sphingomyelin within cellular lysosomes. Infants may have a period of normal development before symptom onset. Initial symptoms may include hepatosplenomegaly and FTT, and cherry-red maculae may be seen at diagnosis. As disease progresses, interstitial lung disease develops, causing tachypnea and respiratory insufficiency. Development regresses and neurologic decline is progressive, with a life expectancy of 2–4 years. This case demonstrates the importance of considering genetic etiologies in the differential diagnosis of FTT.

303 A deceitful case of right lower quadrant abdominal pain: an unusual presentation of crohn’s disease

VT Bray1*

M Liu1

D Young2

1University of South Florida, Tampa, FL

2Johns Hopkins All Children’s Hospital, St. Petersburg, FL

Case report

A 17 year old female presented with acute on chronic right lower quadrant abdominal pain. Appendix ultrasound showed findings concerning for acute appendicitis as well as collapsed bowel with thickened walls ‘raising a question of ileitis’. She was taken to the OR for an appendectomy. Due to continued abdominal pain over the next few months, an EGD and colonoscopy were completed which were grossly normal except for an ulcer at the ileocecal valve. Biopsies from the scopes were negative. A MR Enterography showed inflammation and wall thickening of the cecum and terminal ileum. Pathology of the appendix was revisited and showed mural fibrosis related to a site of gangrenous inflammation with active sub-acute and chronic inflammation. She was also found to have an elevated fecal calprotectin. Five months after her appendectomy, she was admitted for fevers, persistent right lower quadrant abdominal pain, right hip pain, 30 pound unintentional weight loss, and intermittent bloody stools. CT abdomen showed a right psoas muscle abscess with inflammatory changes involving the terminal ileum and cecum. Intravenous antibiotics were started and the patient underwent incision and drainage with external drain placement. Her clinical course was complicated by abscess recurrence requiring a second drainage, ileocecectomy secondary to fistula formation, and a prolonged antibiotic course and hospital stay. Upon resolution of the psoas abscess, the patient was started on Humira with subsequent resolution of her abdominal pain and a new diagnosis of Crohn’s disease.

Abstract 303 Figure 1

Right psoas muscle abscess Right psoas muscle abscess

304 Localized mitral valve injury following blunt chest trauma

M Burroughs*

E Landry

B Dillard

MN Frascogna

University of Mississippi Medical Center, Jackson, MS

Case report

Trauma is the most common cause of childhood morbidity and mortality. Trauma associated with all terrain vehicles (ATV) accidents is increasing in incidence. We present a case of blunt chest trauma in a 3 year old sustained while riding an ATV which resulted in a rare cardiac injury.

A 3 year old, previously healthy male was transferred to the pediatric emergency department (PED) from an outside hospital (OSH) for evaluation of blunt chest trauma sustained while riding a side-by-side ATV. He was thrown against the dashboard when the ATV struck a ditch, resulting in blunt chest trauma. He was reported to have immediate onset of difficulty breathing, and emergency services were called who transported him to the OSH. There, he received a non-contrast computed tomography (CT) scan of the chest which showed bilateral pulmonary contusions. He was then transferred to the PED where he was noted to be in respiratory distress with tachycardia. Physical examination showed no external signs of trauma or deformity to the chest or abdomen. Auscultation of the chest was only significant for bilateral rhonchi. Bedside extended focused assessment with sonography for trauma (eFAST) was negative. A chest x-ray was obtained which suggested bilateral pulmonary contusions. Shortly after arrival in the PED, he developed hypotension and worsening respiratory distress. He was given two normal saline boluses and a blood transfusion with continued decline in his blood pressure, thus was started on an epinephrine infusion. As fluid was given, his respiratory status declined and he was intubated for hypoxia and respiratory distress. After intubation, pulmonary edema began to come from the endotracheal tube. He was transferred to the intensive care unit where an echocardiogram was performed for further workup, showing a flail posterior mitral valve leaflet and severe mitral valve regurgitation.

Cardiac injury is a rare but potentially life-threatening event associated with blunt trauma to the chest. Physicians should keep myocardial and cardiac valve injury in the differential list for any patient with blunt chest trauma, especially when the patient’s clinical course progresses in a fashion that does not match expectations.

305 A rare diagnosis made through genomic sequencing

J Cassady1*

C Gooch1

K Bowling2

S Hiatt2

G Cooper2

A Hurst1

1University of Alabama at Birmingham, Birmingham, AL

2HudsonAlpha Institute for Biotechnology, Huntsville, AL

Case report

Case: A 2-month-old male presented to genetics with large body habitus, unilateral cryptorchidism, pectus excavatum, failed hearing screens, feeding difficulties, and dysmorphic features. On exam, he had plagiocephaly, micrognathia, thin lips, hypertonic extremities, and truncal hypotonia. A chromosomal microarray was normal. By 6 months of age he failed multiple modified barium swallows, prompting G-tube placement.

Genome sequencing performed through the Alabama Genomic Health Initiative identified a variant of uncertain significance in ACTG1 (c.1000C>G; p.Glu334Gln) associated with autosomal dominant Baraitser-Winter Syndrome Type 2 (BWCFS). Though the variant remains classified as a VUS, the patient’s presentation is consistent with BWCFS.

Discussion

BWCFS is caused by variants in ACTB or ACTG1. ACTG1 accounts for >20% of the clinical cases of BWCFS. The disease is characterized by typical craniofacial features and intellectual disability, though there is a large range of phenotypic variability with reports of sensorineural deafness, seizures, renal anomalies, or pectus among other findings. The variant in this case has been reported once in a patient with BWCFS, who was reported to have a milder phenotype (Di Donato, et al. 2016). Further genotype-phenotype correlation may provide improved prognostic information.

Since its inception, genetic testing has evolved from karyotypes to sequencing entire genomes. As sequencing technology becomes faster and more cost-effective, widespread use of genome sequencing may become routine in clinical care. A rising challenge includes classification of variants. Laboratories apply evidence codes (Richards, 2015), and rare missense variants may fail to reach pathogenic status, especially when patients’ parents are not available for testing to confirm if a variant is de novo. Clinical evaluation and result interpretation in light of phenotypic findings is needed to provide appropriate counseling of patients about genomic variants.

306 A case of gradenigo syndrome in a seven-year-old

A Curta1,2*

CR Antonetti1,2

1University of Florida, Pensacola, FL

2Studer Family Children’s Hospital at Ascension Sacred Heart, Pensacola, FL

Case report

A 7-year-old previously healthy female was admitted to our institution for a two-week history of worsening ear pain, headache, and ocular abnormalities. Her symptoms began after swimming in a creek. A day later she complained of ear discomfort and subjective fever. She then developed right sided otorrhea, a bilateral temporal headache, diplopia, and exhibited an unsteady gait. She was evaluated at a local hospital, where she was diagnosed with otitis media and prescribed both amoxicillin and neomycin/polymyxin B sulfates/dexamethasone otic drops. A sinus CT showed chronic mastoiditis with no intracranial findings. Two days later, her diplopia worsened, with inability to abduct her right eye and pain in the right maxillary sinus area. She presented to our emergency room, where she was found to have bilateral purulent otorrhea and right abducens nerve palsy. Infectious studies showed a normal WBC count, mild normocytic anemia, reactive thrombocytosis, and elevated inflammatory markers with ESR 114 mm/hr and CRP 2.2 mg/dl. MRI brain showed bilateral otitis media, mastoiditis, enhancement of the right 7th/8th cranial nerve complex, and enhancement of the right petrous apex concerning for petrous apicitis. MRV showed patent dural venous sinuses. Ceftriaxone was initiated. ENT, ID, and Neurology consultants assisted in management. She underwent a complete right mastoidectomy with opening of the retrolabyrinthine air cell tract to the petrous apex and bilateral tympanostomy tube placement. Blood and right mastoid fluid cultures were negative. Antibiotic therapy was transitioned to ciprofloxacin and clindamycin due to water exposure history for pseudomonal coverage. She also received methylprednisolone 30 mg/kg daily for three days given her cranial nerve involvement. She clinically improved, and transitioned to oral antibiotics and a prednisone taper to complete a four week treatment course. Gradenigo syndrome is a rare but serious complication of otitis media, characterized by a triad of symptoms including otitis media, pain in the trigeminal nerve distribution, and ipsilateral abducens nerve palsy. Gradenigo syndrome should be considered in a patient who presents with otitis media and ocular findings.

307 Methicillin-resistant staphylococcus (MRSA) aureus retropharyngeal abscess with mediastinitis in a 13 month old female

M DeOliveira*

E Broussard

Duke University Hospital, Durham, NC

Case report

A previously healthy 13-month-old female with no significant past medical history was transferred from an outside hospital with 4 days of fevers, decreased oral intake, and dehydration. Upon arrival, physical examination revealed left neck swelling with limited neck mobility. Given the degree of left neck swelling, a CT neck with contrast was done which revealed a retropharyngeal abscess extending into the superior mediastinum. She went to the operating room with ENT for incision and drainage of the neck abscess with placement of a penrose drain. She was empirically started on vancomycin and unasyn. Gram stain and culture from the operating room were positive for MRSA; in addition, a blood culture collected on admission was positive for MRSA. On POD 2 she developed worsening fevers with rising CRP and a chest CT showed progression of the mediastinal fluid collection to involve the great vessels with extension into the right pleural space with concern for internal jugular vein thrombosis versus external compression. She went to the OR again for re-excision and drainage with mediastinum wash out. After this, she was switched to daptomycin with improvement in her CRP and resolution of her fevers. Given the CT findings along with intraoperative findings of left internal jugular vein thrombus she was started on a heparin drip, however repeat neck US showed no thrombus so heparin was stopped. Echo was negative for endocarditis. She was discharged 9 days after admission with IV daptomycin. At discharge she had 3 negative blood cultures. Retropharyngeal abscesses in young children are easy to miss. Prompt recognition and treatment of the abscess and any concurrent extensions into the mediastinum are necessary for successful recovery. Through this case, we want to highlight the importance of early recognition of retropharyngeal abscess.

308 A one in ten million case of thrombotic thrombocytopenic purpura

T Dinh*

C Willis

C Joseph

E Klepper

E Hauck

OLOL Children’s Hospital, Baton Rouge, LA

Case report

Thrombotic thrombocytopenic purpura (TTP) is a condition characterized by microangiopathic hemolytic anemia, thrombocytopenia, and severe deficiency of ADAMTS13 (activity <10%). It can also be associated with neurologic, gastrointestinal, renal, and cardiac involvement. Typically associated with adults, it is considered rare in the pediatric population with a reported incidence of 1 per 10 million children less than 18 years old.

A 15-year-old male with a history of DRESS syndrome and IBD presented to the emergency department with vomiting, back pain, and dark urine for two days. Initial labs showed a creatinine of 1.9 mg/dl and a urinalysis significant for blood >100/hpf, protein of 100 mg/dl, and large hemoglobin. There was initial concern for nephritic syndrome with his urine protein to creatinine ratio 5,586 mg/g. However, additional labs including complement levels, ANA, and IgA were all unrevealing. He subsequently underwent a renal biopsy which showed thrombotic microangiopathy, acute tubular injury, and IgA nephropathy. Following the procedure, he was found to be anemic with a hemoglobin of 8.5 g/dL and thrombocytopenic with platelets of 10,000/m3. His hemoglobin continued to decline requiring PRBC transfusions, but his platelets stabilized. With no active GI symptoms to suggest infectious colitis, the working diagnosis become atypical hemolytic uremic syndrome. A few days later, new onset seizures required transfer to the intensive care unit. He received one dose of eculizumab before his ADAMTS13 activity level resulted as <5%, consistent with thrombotic thrombocytopenic purpura. Exchange transfusions and prednisone were started with initial improvement in his hemoglobin, platelets, and LDH. Three additional exchange transfusions and increased steroid dosing were eventually required. Rituximab was also initiated before his labs remained stable.

This case of a rare condition in pediatric patients highlights the importance of considering thrombotic thrombocytopenic purpura when evaluating patients for hemolytic uremic syndrome. While the two conditions share many similar features, the treatment for each is very different. As clinicians we must remember to consider thrombotic thrombocytopenic purpura in pediatric patients with anemia and thrombocytopenia.

309 Chronic granulomatous hsv encephalitis in a preschool child

NI Eboh1*

S Dadashazar1

J Khalid1

A Healy1

M Toon1

D Hurst1

R Jacob1,2

F Levent1,3

1Texas Tech University Health Science Center, Lubbock, TX

2University Medical Center, Lubbock, TX

3Adventhealth for Health, Orlando, FL

Case report

We present a case of a 4-year-old girl who came with 1 minute tonic-clonic seizure, right-sided weakness, transient slurred speech and left eye ptosis. She was born vaginally at term to a mother with no prenatal care and was diagnosed with neonatal herpes simplex virus (HSV) encephalitis in infancy leading to acquired right sided cerebral palsy, epilepsy, developmental delay, and recurrent herpes labialis despite appropriate treatment. Admitted at 16 months after a follow up brain magnetic resonance Imaging(MRI) revealed worsening inflammatory or infectious process concerning for possible HSV relapse or autoimmune response to HSV but cerebrospinal fluid (CSF) studies and quantitative antibodies were normal. Tests for other etiologies were also negative. She had received intravenous acyclovir which was stopped after negative results. She was followed as outpatient and continued with no new concerns until index visit.She was alert with decreased right upper extremity and lower extremity strength with no other neurological deficits or abnormal exam findings.Extensive workup ruled out an infectious or autoimmune etiology but MRI revealed linear tracts of increased T2 Flair signal in the left periventricular matter extending into the left thalamic region and scattered areas of calcification in the brain.She was subsequently diagnosed with chronic granulomatous HSV encephalitis based on history,exam findings and MRI results.She was treated with intravenous acyclovir followed by oral acyclovir which she continued for three months and then prophylaxis with clinical improvement.Chronic granulomatous HSV encephalitis is a rare complication of neonatal HSV encephalitis that causes significant neurological sequelae in children. There are no current guidelines on treatment for this condition. We present this case in order to create awareness of this complication and the need to further investigate potential treatment options.

310 Hematuria after correction of congenital heart disease

A Fayyaz1*

S DeLeon2

WF Campbell2

1University Of Health Sciences, Oklahoma City, OK

2The Children Hospital at OUHSC, Oklahoma CIty, OK

Case report

We describe a 3 year old F with a h/o complicated CHD, specifically L- Transposition of the Great Arteries, TAPVR and dextrocardia with heterotaxy. She had previously undergone multiple surgical corrective interventions including a BD Glenn procedure, right pulmonary artery-plasty, PDA ligation, and most recently a non-fenestrated Fontan procedure several weeks prior to this presentation for a new onset hematuria.

She was doing well clinically after her Fontan procedure when she was brought in for evaluation of a 1 day h/o a large amount of bright red blood in her diaper. Her medications include furosemide, chlorthiazide, baby aspirin, and sildenafil. In the ED her PE was normal for age except for a notable murmur and healed surgical scars. There was no evidence of bleeding on an external GU exam. Her labs showed normal Hg and normal renal function, but her urinanalysis was significant for >100 RBC/hpf. Urology was consulted and recommended renal ultrasound which showed multiple dilated varices in the right lower quadrant. A follow up CTA of the A/P to rule out any inferior vena cava obstruction displayed a patent IVC but showed multiple extremely large varicosities of the abdomen and renal veins. Her hematuria was subsequently attributed to the significantly dilated venous network in her pelvis. Cardiology then decreased her aspirin dose by half and added tadalafil to her medications in an attempt to decrease her central venous pressures. She had no further hematuria and was discharged home to be followed outpatient by cardiology.

The Fontan operation is a palliative procedure performed in patients with a functional or anatomic single ventricle. Patients with renal dysfuction related to decreased glomerular filteration have been described as a post-operative complication of Fontan; additionally, bleeding manifested as hemoptysis and intracranial bleeding have been reported and are more common if patients are anticoagulated. However, hematuria secondary to pelvic venous dilation is uncommon and not frequently reported in the literature. It is important for non-cardiology clinicians to be aware of this relatively rare complication after a Fontan procedure.

311 Successful physostigmine reversal of severe anticholinergic toxidrome in a child with diphenhydramine intoxication

DP Fitzgerald*

E Bennett

MM Mitchell

S Schexnayder

UAMS, Little Rock, AR

Case report

Purpose: To describe a case of severe anticholinergic poisoning from diphenhydramine successfully treated with physostigmine.

Methods

A three-year-old female ingested approximately 120–180 ml diphenhydramine elixir (2.5 mg/ml), resulting in a potential dose of 16–25 mg/kg. Approximately 30 minutes after ingestion, she developed tremors and visual hallucinations. On hospital arrival, she had tacyhcardia (heart rate 144), mydriasis (pupils 4–5 mm), cutaneous flushing, and normothermia. Central nervous system (CNS) manifestations included severe agitation and hallucinations. Lorazepam was given with no improvement. She was admitted to the PICU where her agitation and screaming were unresponsive to additional IV lorazepam and IV dexmedetomidine. Because of her marked CNS symptoms, 0.02 mg/kg physostigmine was administered after consultation with a toxicologist and poison control center. She calmed and nearly immediately fell asleep. Her tachycardia and cutaneous flushing improved.

Conclusions

Diphenhydramine is a widely used pediatric antihistamine with a wide margin of safety, but may result in severe anticholinergic toxicity in overdose. Anticholinergic drugs competitively inhibit muscarinic acetylcholine receptors in the central nervous system, heart, peripheral postganglionic nerves in smooth muscle, salivary and sweat glands, and the ocular ciliary body. These effects cause the anticholinergic toxidrome with symptoms of cutaneous vasodilation, anhidrosis, anhidrotic hyperthermia, nonreactive mydriasis, and mental status changes including delirium and hallucinations. Physostigmine is an acetylcholinesterase inhibitor that binds reversibly in both the peripheral and central nervous systems, causing an increased concentration of acetylcholine at muscarinic receptors. Physostigmine crosses the blood brain barrier, making it effective for reversing both peripheral and central effects of anticholinergic toxicity. While most ingestions do not require such therapy, this case describes the successful use of physostigmine in a pediatric patient with the anticholinergic toxidrome after ingestion of 12–20 times the therapeutic dose of diphenhydramine.

312 A rare case of a tongue mass in a 17-month-old male

BO Foxworthy*

E Alva

University of Alabama at Birmingham, Birmingham, AL

Case report

We present the case of a 17-month-old patient who presented with a tongue lesion found to be ACTB-GLI1 fusion positive sarcoma.

Patient is a 17-month-old who presented with a lesion on his tongue noticed by his parents shortly after birth. He was referred to oral surgeon who initially suspected a vascular lesion or hemangioma. Lesion increased in size to 0.5 cm and was surgically resected. Preliminary pathology showed mixed mesenchymal neoplasm concerning for BCOR-associated sarcoma. Additional molecular genetic testing from Memorial Sloan-Kettering Cancer Center and Foundation One revealed ACTB-GLI1 fusion malignant neoplasm. This rare diagnosis was only recently described in the literature with only 10 cases of this particular translocation reported, three of which were in the tongue. All patients in the initial study underwent surgical resection and were alive with no cases of metastatic spread. A subsequent study including four patients with ACTB-GLI1 fusion included two with metastatic disease to locoregional lymph nodes, both of whom were alive with no distant metastatic spread. Metastatic work-up in our patient did not show any clear evidence of metastasis, though PET scan showed some low-level SUV uptake in his neck, which could be reactive given patient’s age. Initial surgical resection with grossly positive margins as well as evidence of vascular invasion. Second surgical resection was undertaken with microscopically positive margins. Discussions with family led to decision not to pursue third resection given favorable outcomes in patients with positive margins in limited available literature. Patient is currently undergoing surveillance imaging every 3 months to monitor for disease recurrence.

References

  1. Antonescue CR, et al. A distinct malignant epitheliod neoplasm with GLI1 rearrangements, frequent S100 protein expression, and metastatic potential: expanding the spectrum of pathologic entities with ACTB/MALAT1/PTCH1-GLI1 fusions. Am J Surg Pathol 2018 April 42(4):553–560

  2. Dahlen A, et al. Activation of the GLI oncogene through fusion with the beta-actin gene (ACTB) in a group of distinctive pericytic neoplasms: pericytoma with t(17;12)Am J Pathol, 2004;164:1645–1653.

313 A rare case of infantile osteopetrosis

K Gutermuth*

EN Bufkin

AM Coleman

Univeristy of Alabama at Birmingham, Birmingham, AL

Case report

Here we describe a 10woF, ex-36wk twin, with brief NICU stay for jaundice, who presented to the ED for abnormal lab evaluation. The patient had been followed by her PCP for failure to thrive & reflux. At her GI visit, she had labs obtained that noted anemia, thrombocytopenia, & hypophosphatemia. She had imaging which demonstrated cupping, and fraying at the distal physis of the radius & ulna consistent with rickets.

Upon admission, the infant weighed 3.4 kg (1%, -2.35), length 48cm (<1%), & HC of 37 cm (38%) Exam revealed her to be small for stated age, chronically-ill appearing, pale, frontal bossing, & mild spenomegaly. The differential included congenital rubella, CMV, syphilis, & osteopetrosis. Upper GI revealed GER, abdominal U/S with mild ascites & splenomegaly, & a normal swallow study. Electrolytic disarray was noted with hyperchloremia, mildly low bicarbonate & albumin, normal calcium & magnesium, & hypophosphatemia. She had hypocellular marrow, negative for malignancy. Flow cytometry showed leukoerythoblastic anemia consistent with infantile malignant osteopetrosis (MO). Genetic testing revealed a homozygous pathogenic change in TCIRG1 gene.

Discussion

She was diagnosed with infantile MO. MO has an incidence of 1 in 250K live births. 10 genes have been recognized to account for 70% of cases. Pathogenesis is due to failure of osteoclast development & function, resulting in increased bone density which weakens the bones & restricts growth. Typical presentation includes macrocephaly, frontal bossing, choanal stenosis, & nerve compression. Hypophosphatemia & hypocalcemia can cause seizures & secondary hyperparathyroidism. Due to abnormal bone expansion & interference in medullary hematopoiesis, causing pancytopenia.

She underwent stem cell transplant at 4 mo. Unfortunately, she is deaf and blind secondary to optic nerve canal compression & narrowing of her internal auditory canals. She required a gastrostomy tube due to failure to thrive. She tolerated her stem cell transplantation well with no evidence of GVHD, & her immunosuppression is being weaned. Our presentation of infantile MO may increase awareness of this disease, expand our understanding of the way in which this disease can present, & therefore, assist in earlier clinical suspicion, diagnosis, & potential treatment.

314 An infant with familial cerebral cavernous malformation

S Hari Gopal*

W Gaylord

SE Sterner

University of Tennessee College of Medicine at Chattanooga, Chattanooga, TN

Introduction

Familial cerebral cavernous malformations (FCCM) are rare, with a strong genetic predisposition.

Case report

A 19-month-old male presented to the Emergency Department (ED) with a history of staring off, lip smacking, and shaking of extremities for one month. He was diagnosed with febrile seizures in the context of sinusitis. The night prior to presentation, he had fever and vomiting, with worsening of these episodes. In the ED, he developed a fixed right-sided gaze with left-arm shaking. An MRI of the brain showed hemorrhagic, multiloculated lesion in the left frontal lobe measuring 3.4 cm, along with other patchy, punctate vascular lesions (figure 1). He underwent resection of a hemorrhagic cavernous malformation, and was started on Oxcarbazepine for seizure control.

Further questioning revealed a significant familial history of cerebral cavernous malformations on the maternal side, which prompted referral to genetics for further work up.

Discussion

Familial CCMs have a prevalence of 0.35–0.53%. They have an autosomal dominant inheritance pattern with mutations in CCM1 (KRIT1), CCM2 (MGC4607) and CCM3 (PDCD10), affecting junction formation between vascular cells. Familial cases are typically small, asymptomatic, multiloculated lesions, scattered in different areas of the brain. Symptomatic episodes can be treated conservatively, unless the lesions are surgically accessible.

Conclusion

In patients with focal neurological signs and imaging concerning for a cavernous malformation, a family history is vital. Genetic testing can confirm the clinical diagnosis of familial CCM, which can help in prenatal testing and counseling.

Abstract 314 Figure 1

MRI brain showing hemorrhagic, multi-loculated lesion in the left frontal lobe with other patchy areas of internal enhancement. MRI brain showing hemorrhagic, multi-loculated lesion in the left frontal lobe with other patchy areas of internal enhancement.

315 Loxoscelism: unlocking the bite of hemolysis

A Hassan*

KM Galipp

R Sebastian

O Khan

K Sagdeo

University of Oklahoma Health Sciences Center, Oklahoma City, OK

Case report

Systemic manifestations and hematological abnormalities following brown recluse spider bite, termed loxoscelism, is a rare entity. We present a case with systemic features including acute hemolytic anemia with discovery of a preceding spider bite.

A 12-year-old boy presented with left shoulder pain, left arm paresthesia, and diffuse myalgias. Exam revealed a tender left axillary lymph node and shoulder contusion. He progressed and developed severe sepsis with leukocytosis and fever, and was started on IV vancomycin and ceftriaxone. He developed a diffuse exanthem that evolved into a scarlatiniform rash with sandpaper texture and later desquamation. The shoulder lesion developed increasing ecchymosis and a central eschar, which denuded to reveal an ulcerative necrotic lesion. On day 5, he developed lethargy and acute anemia with hemoglobin of 5.7 mg/dL, hyperbilirubinemia at 5.5 mg/dL, and low haptoglobin. Coombs direct antiglobulin test (DAT) was positive, including positive IgG and C3 on red blood cell surfaces, consistent with autoimmune hemolytic anemia. Peripheral smear revealed schistocytes and microspherocytes. He was started on high dose IV steroids and received PRBC transfusions for symptomatic anemia with good clinical response. At discharge he was transitioned to an oral steroid wean, as well as oral amoxicillin.

Loxoscelism refers to systemic effects resulting from the bite of a spider of genus Loxosceles. The brown recluse spider, Loxosceles reclusa, is the common endemic species. Although rare, loxoscelism can cause significant morbidity from hemolytic anemia, disseminated intravascular coagulopathy, rhabdomyolysis, and acute kidney injury. In this case, features are consistent with autoimmune hemolytic anemia with positive DAT and RBC surface C3 and IgG. The pathophysiology remains poorly understood with proposed mechanisms of erythrocyte membrane lysis caused by sphingomyelinase toxin and complement mediated immune destruction due to membrane disruption. Given the direct toxin-mediated damage along with autoimmune hemolysis, features are consistent with both intravascular and extravascular hemolysis. The standard treatment is supportive. Given the proposed mechanisms of hemolysis, steroids are a reasonable adjunct to therapy.

316 Primary chylopericardium: blood is red, green is bile. if its white, it must be chyle!

A Hassan*

C Sperrazza

University of Oklahoma, Oklahoma City, OK

Case report

Primary chylopericardium is a rare entity, as the name suggests it is accumulation of chyle in the pericardium with no secondary cause.

A 14-year-old girl came to our emergency department with chest pain for the past 3 months. Pain was sharp, midline and worsened with deep breaths and lying flat but not on palpation. She denied dyspnea, fever, dizziness, rash, trauma or recent viral illness. At presentation, she was vitally stable. Initial labs and chest x-ray were unremarkable. EKG showed diffuse ST segment elevation with electric alternans pattern of QRS wave. Echocardiography showed large pericardial effusion with no tamponade physiology. She was taken to cardiac catheterization lab for immediate pericardiocentesis, roughly 700cc of fluid was drained and she was transferred to floor with a 5-french pig-tail catheter. Fluid appeared turbid with analysis showing triacylglycerides (TAG) 1121 mg/dl, Lactate Dehydrogenase 152 mg/dl and negative gram stain and culture. Computed Tomography (CT) Chest and Magnetic Resonance Imaging (MRI) of Abdomen and Pelvis were negative for any mass. Lymphoscintigram did not reveal any lymphatics malformation.

By definition, chylous effusion should appear milky and opaque, with TAG more than 500 mg/dl, Cholesterol/TAG ratio of less than 1, negative cultures, lymphocyte predominance on cytologic examination and Fat globules seen on Sudan 111 staining. Primary Chylopericardium occurs due to malformations of thoracic duct which are usually picked up on lymphoscintigraphy but rarely, they are so miniscule and not seen at all. Pericardiocentesis with fat restriction in diet or by starting Total Parenteral Nutrition is first step. Somatostatin constricts lymphatics and has shown benefit by decreasing chyle formation. Surgical options include thoracic duct ligation with pericardial window or emobolization. Pericardio-peritoneal shunt placement can also done.

Our patient responded to low fat diet with complete resolution of effusion.

317 There’s a fungus among us! pulmonary blastomycosis in an immunocompetent child

IB Hemphill*

K Schneider

University of Mississippi School of Medicine, Jackson, MS

Case report

Pulmonary blastomycosis is a rare condition characterized by cough, fever, sputum production, and chest pain. It is usually seen in patients who are immunocompromised and live in the Ohio and Mississippi River valley basins. Disease presentation in immunocompetent children is rare. Blastomycosis is a slow growing organism and can manifest as broad respiratory symptoms but may present with systemic pyogranulomatous infection and may spread to skin, bones, joints, and the central nervous system. Cutaneous lesions are a more typical presentation in children.

We herein report a rare case of pulmonary blastomycosis in a well-appearing, immunocompetent 8-year-old African American female who presented with a dry cough and persistent fever for seven days. A previous admission for pneumonia and multiple rounds of antibiotics failed to resolve her symptoms.

On presentation, she had leukocytosis (WBC of 17.4) with neutrophilia and an elevated CRP (5.8 mg/dL). Her chest x-ray was consistent with a consolidation in the left lower lobe with left sided pleural effusion/empyema of the posterior upper pleural space. A CT of the chest showed scattered ground glass and tree-in-bud opacities throughout the lungs. A bronchoscopy revealed inflammation with greenish fluid, and bronchoalveolar lavage was performed for further testing. Preliminary results were negative, with further cultures and studies pending, and the fungal culture resulted on day 51 of the illness positive for Blastomycosis dermatidis. The patient was given itraconazole treatment for six months. Fever and cough resolved shortly after initiation of treatment.

Pulmonary blastomycosis is rare in children, and especially those who are immunocompetent. However, several studies have shown that pulmonary blastomycosis is more evident in childhood cases than originally expected. When the lung is involved in blastomycosis cases, the most common patterns of involvement are consolidations, medium sized lung nodules, and the interstitial ‘tree-in-bud’ pattern. A culture is the definitive diagnostic standard but is typically known to delay treatment due to its slow growing nature. Because of this slow growth, it is important to consider fungal etiologies in pediatric fever of unknown origin patients in the appropriate regions.

318 Just a picky eater? A case of a germinoma masquerading as a common pediatric problem

S Howell1,2*

A Siller1,2

V Parikh1,2

1Baylor College of Medicine, Houston, TX and

2Texas Children’s Hospital, Houston, TX

Case report

Tumors affecting the pituitary gland may cause a cascade of symptoms related to endocrinopathies resulting from compression of the pituitary stalk. Symptoms can often be subtle and attributed to more common pediatric problems. We present a case emphasizing the importance of keeping a broad differential diagnosis and of a complete and thorough physical examination for arriving at a correct and timely diagnosis.

The patient is a 6 year-old previously healthy male who presented with excessive weight loss. He fell from the 90th to the 32nd percentile in weight over the course of a year, initially attributed to being a picky eater. He then fell to the 5th percentile over the ensuing month despite attempts at dietary interventions, prompting referral to the emergency room for evaluation for a gastrointestinal cause. History obtained in the emergeyncy room was notable for headaches for 6 months, which occurred randomly and did not wake him from sleep, intermittent emesis for the past 6 months, occurring primarily in the morning and shortly after waking, polyuria, polydipsia, and anorexia. He had developed diarrhea in the preceding week, although his family attributed this to consuming solely Gatorade during that time. Mother and patient denied gait or vision changes. Physical exam was notable for tanner stage 3 coarse pubic hair, a phallus and scr