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2018 Southern Regional Meeting

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Cardiovascular club I, 11:00 AM, Thursday, February 22, 2018

1 A possible role for genetics in cardiovascular disease among the acadians

AE Tedesco

Z Haq

KL Di Losa

MJ Ali

P Gregory

LSUHSC – New Orleans, New Orleans, LA

Purpose of study It is well documented that the Louisiana Acadians (‘Cajuns’) experience a disproportionate risk for some genetic diseases due to a genetic founder effect.Furthermore, certain founder populations show a predisposition for developing heart disease.The current study was designed to determine both the population prevalence of cardiovascular disease in the Acadian region and to determine whether the prevalence of early cardiovascular disease is increased among the Cajun population.

Methods used We obtained descriptive information via the electronic medical record of 345 consecutive patients previously diagnosed with early onset (age <50) cardiovascular disease (CVD) who presented to University Hospital and Clinics Cardiology Clinic (June 2015–July 2016). The patients consisted of 184 African Americans (97 females and 87 males) and 161 Caucasians (74 females and 87 males).For this study, cardiovascular diseases included: coronary artery disease, HTN, myocardial infarction, and CHF. Demographics, vital signs, lipid panel results, medications, family history, date of CVD diagnosis, and past medical history were collected for all patients.The data on the (161) Caucasian patients were then stratified into either Cajun-identified or non-Cajun by comparing each patient’s last name to a standardised list of most popular Cajun last names. Means for each variable between the two groups were compared using independent t-tests.

Summary of results The results of our analysis revealed that Cajun-identified patients were diagnosed with a CVD at a significantly younger age (40± 8 years) than were non-Cajun patients (44± 4 years, p= 0.03).There were no significant differences between the Cajun-identified and non-Cajun groups with regard to BMI, blood pressure, HDL and LDL levels, family history of CVD, or smoking/alcohol use history. Cajun patients had lower triglyceride levels (184.6± 140.4) than non-Cajun whites (210.3± 153.1) although whis was not statistically significant.

Conclusions Our results show that patients of Cajun ancestry were diagnosed with cardiovascular disease at a younger age than their non-Cajun counterparts.This intriguing finding suggests that genetic predisposition may contribute to environmental/behavioural factors in development of CVD. Further study is needed to determine the generalizability and the cause of this pathology.

2 Still with higher ldl levels-hispanics in puerto rico shows a lower coronary artery disease than the usa-explained by genetic admixture

JE Muñoz1

P Elosegui1

C Melendez1

PI Altieri1,2

HL Banchs1,2

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

2Cardiovascular Centre of Puerto Rico and the Caribbean, San Juan, PR

Purpose of study Coronary artery disease (C.A.D.) is one of the highest causes of death in the world. The purpose of this study is to compare Puerto Rico (P.R.), Hispanic, U.S.A. country, with the U.S.A., in coronary artery disease.

Methods used Compare a population of Hispanics with the high LDL levels with normal total cholesterol and HDL in P.R. and the U.S.A. The study population was 1000 patients. The U.S.A. health statistics and P.R. Department of Health was used for comparison.

Summary of results Studying the lipid profile of Puerto Rico population, we found that the mean value of LDL lipoprotein is high (±104 mg/dl) with similar cholesterol and HDL levels in both societies; still the coronary disease (CAD) incidence is lower than the U.S.A. (20%–30%). Investigators from the U.P.R. reported the genetic admixture of this Hispanic population. They reported the admixture consisted of 3 genes called protective against C.A.D.

Conclusions C.A.D. is an inflammatory process involving inflammation of the endothelial cells, macrophages and other cells. Probably, this admixture protects the endothelial cells against an aggressive inflammatory process and excessive oxidative stress. The observation of stitziel-Washington University which described ANGPTL3 gene which produces low cholesterol levels and absent plaques in the coronary arteries support our hypothesis.

3 Obesity is associated with aortic dialation in marfan’s syndrome

SD Grado

C Watson

WF Campbell

S Kiparizoska

A Thibodeaux

C Richards

TA Skelton

MR McMullan

ME Hall

University of Mississippi Medical Centre, Jackson, MS

Purpose of study Marfan’s Syndrome (MS), a connective tissue disorder characterised by a slender build and long limbs, is associated with aortopathy and aortic dissection. Obesity is associated with haemodynamic and metabolic abnormalities such as hypertension, hyperglycemia, inflammation and aortic stiffness that may adversely affect the aorta. We aimed to determine if obesity is associated with aortic dilation in MS patients living in an obese environment.

Methods used We retrospectively analysed anthropometric and echocardiographic data from 61 MS patients from the University of Mississippi Medical Centre from the past 5 years. Multivariable linear regression was used to assess the association of body mass index (BMI) with aortic root diameter measured on the parasternal long axis view at the sinuses of Valsalva on transthoracic echocardiogram.

Summary of results The mean BMI of our MS patients was higher compared with historically published data on MS patients (24.5 kg/m2 vs 20.1 kg/m2). This corresponds with the high prevalence of overweight (BMI 25.0 to <30 kg/m2) and obesity (≥BMI 30.0 kg/m2) in the state of Mississippi and suggests that it extends to the congenital heart population. Mean aortic root dimensions were increased in overweight (40.9±5.1 mm) and obese MS patients (51.5±12.0 mm) compared to normal weight (37.0±6.8 mm) or underweight (38.1±8.6 mm) patients. Aortic root diameter increased with increasing BMI, independent of age, sex, race, systolic blood pressure and height (p=0.06).

Conclusions Patients with MS tend to be tall and lean; however, in Mississippi, a state with high rates of obesity, they have higher mean BMIs when compared to published data. Higher BMI was associated with larger aortic root diameters in our single-centre study. Further assessment of rates of aortic dilation in MS patients with differing BMIs is needed to determine if overweight and obesity exacerbate aortic dilation in MS.

4 Barriers to early cardiac catheterization in high risk nstemi patients

C Basman

A Bhandary

J Daibes

P Sayegh

S Lebrun

N Coplan

Lenox Hill Hospital, New York, NY

Purpose of study The purpose of this study is to analyse barriers for patients with high risk NSTEMI to receive an early invasive treatment strategy.

Methods used We conducted a retrospective chart review designed to evaluate whether patients with high risk NSTEMI are receiving an early invasive (within 1 day of admission) or delayed invasive (1–3 days after admission) strategy.

Summary of results The study included 173 patients that presented to the Emergency Department with high risk NSTEMI. There were 46 patients (average age 64.5 years) in the delayed invasive arm, and 127 patients (average age 63.2 years) in the early invasive arm analysed. Patients receiving a delayed invasive strategy were more likely to have a history of atrial fibrillation (AF) and to be on anticoagulation (table 1). Patients admitted on weekend/holiday were more likely to have delayed invasive compared to patients admitted during the week (table 2).

Conclusions It is important to recognise barriers to prompt cardiac catheterization and to make hospital system adjustments for optimal treatment of patients with NSTEMI.

Abstract 4 Table 1

Chi square analysis; baseline features

Abstract 4 Table 2

Chi square analysis; patients admitted on a weekend/holiday versus weekday

5 Intravenous iron administration reduces fibroblast growth factor 23 levels in chronic kidney disease

B Panwar

O Gutierrez

University of Alabama, Hoover, AL

Purpose of study Higher fibroblast growth factor 23 (FGF23) is associated with higher risk of heart disease and mortality in chronic kidney disease (CKD). Recently, iron deficiency has been linked to elevated FGF23 levels. We examined whether treatment with intravenous iron reduces FGF23 in individuals with CKD.

Methods used 10 participants with stage 3/4 CKD (eGFR 15–59 ml/min) and scheduled to receive intravenous iron (Ferumoxytol) were enrolled in a single-arm study. The primary outcome variable was change in intact FGF23 (iFGF23) or c-terminal FGF23 (cFGF23) levels. Study samples were drawn at baseline and 2 weeks after iron administration. Paired t-test analysis was used to examine change in log transformed iFGF23 and cFGF23 over time.

Summary of results At baseline, mean estimated glomerular filtration rate, mean serum haemoglobin, mean serum ferritin, mean percent transferrin saturation and mean serum phosphorus were 29.5 (±0.5) ml/min, 8.8 (±1.5) g/dL, 54.8 (±41.6) ng/ml, 14(±11.4)%, and 3.9 (±0.5) mg/dL respectively. There was a significant reduction in mean serum iFGF23 concentration (17.7%) at 2 weeks post iron infusion (two tailed p= 0.022). (Figure) There was no significant change in mean cFGF23 concentration.

Conclusions Intravenous iron administration significantly reduced serum iFGF23 levels among individuals with stage 3/4 CKD. Our findings suggest that optimising iron status in individuals with CKD could potentially decrease FGF23 concentration.

Abstract 5 Figure 1

There was no significant change in mean cFGF23 concentration

6 High grade heart block following accidental exposure to organophosphate poisoning

GD Bedanie

D Gebremariam

K Nugent

Texas Tech University Health Centre, Lubbock, TX

Introduction Organophosphates are potent cholinesterase inhibitors capable of causing severe cholinergic toxicity following cutaneous exposure, inhalation, or ingestion. Cardiac manifestations, such as sinus bradycardia, prolonged PR interval, and prolonged QTc, occasionally occur. Complete atrioventricular (AV) block has rarely been reported in this poisoning.

Case presentation A 45-year-old man was found unconscious and brought to our emergency centre as Level 1 trauma patient after his family found him on the floor. He was in respiratory arrest with an unknown mechanism and duration. His blood pressure and heart rate were normal. He had abrasions and bleeding from forehead. He was intubated and admitted to Surgical ICU under the trauma service. On the next day, his family reported that they are suspicious of exposure to an unknown chemical. Due to our suspicion of organophosphate exposure, laboratory test was done and showed a very low level of acetylcholine esterase. On the same day, he developed severe bradycardia with recurrent 3rd degree AV block. Cardiology was consulted, and the patient was treated with atropine.

Discussion Organophosphate exposure produces clinical manifestations due to cholinergic excess. It inhibits the acetylcholine esterase enzyme. Cardiac complications develops secondary to augmented vagal influence on the sinoatrial and AV nodes.

The presentation of our case was unusual and was misleading; he had no typical manifestations of organophosphate poisoning at presentation. Cardiac telemetry monitoring helped us detect recurrent 3rd degree AV block and severe bradycardia that developed 24 hour after exposure to organophosphate. It is rarely described in the literature and complete heart block has been reported in very small number of cases. Development of life threatening cardiac conduction abnormalities may not be early. Patients with suspected organophosphate exposure should be observed closely in an acute care setting with cardiac monitoring and access to atropine, oximes, and external pacing. Careful monitoring, early recognition of this complication, and appropriate management should decrease the mortality rate in these patients.

7 Bortezomib in the management of cardiac amyloidosis-a metaanalysis

JK Bissett

CAVHS/UAMS, Little Rock, AR

Purpose of study Cardiac amyloidosis is a devastating cardiomyopathy with poor prognosis. Median survival after the diagnosis is less than 6 months after diagnosis and one year mortality is 45% if no effective treatment can be offered. Bortezomib is a boronic acid derivative competitively bind to the proteosome S and has shown improvement in the survival of these patients. As cardiac amyloidosis is a rare disease, no large randomised clinical trials has been done to study the effectiveness of different therapies. Reported clinical experience is limited to retrospective studies. Recently there are abstracts reported different therapies on Bortezomib. The purpose of this study is to review the clinical use and effectiveness of Bortezomib in the managment of cardiac amyloidosis.

Methods used Pubmed and Cochrane Evidence based medicine database search with keywords ‘ Bortezomib’ and ‘Cardiac Amyloidosis’ ‘systemic AL amyloidosis’ will be used. The listed reports/journal articles are reviewed, inclusion criteria:

  1. Patient aged >18;

  2. Study involve systemic amyloidosis AL type and Bortezomib used as an either a)induction agent; b) Part of the combination chemotherapy

  3. Studies/reports listed clinical response and survival.

Relevant data are extracted and listed.

Summary of results There is a growing interest in the therapy of cardiac amyloidosis. There are more publications involving collaboration between centres and quality studies on the use of Bortezomib. Use of Bortezomib either along or more commonly, in combination with other chemotherapeutic agents such as dexamethasone, melphalan and cyclophosphamide show promise for patients with this devastatin disesse. The reported cardiac response rate varies from 19%–30%. In addition, Bortezomib used along or in combination with other chemotherapeutic agents improves survival. The studies so far are limited in terms of number of patients and further stratification of different risk groups. However, the results so far are promising.

Conclusions Bortezomib hold promise for cardiac AL amyloidosis. Further quality study is needed to characterise its use.

8 Effects of glucagon in the conduction of system of the heart-and possible uses in the cardiovascular and metabolic system

H Nuñez1

PI Altieri1,2

HL Banchs1,2

N Escobales1

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

2Cardiovascular Centre of Puerto Rico and the Caribbean, San Juan, PR

Purpose of study The role of glucagon in the conduction system is not clear at the present time, but its role in glycemic control is more known. We decide to clarify its electrophysiologic effects and define its possible use in close-loop bi hormonal systems-artificial pancreas.

Methods used His bundle studies were done to find the effect in 10 patients (P.) in the sinus node, A-V node and ventricular conduction. B.S. levels were analysed prior and at the end of the study.

Summary of results Glucagon 5 mg was injected intravenously in an infusion which lasted 10 min. His bundle studies were done by standard techniques. Sinus node function was shortened by 16.6%, increased the atrioventricular conduction 12%, increased the heart rate by 17% (911 P<0.25). No effect in intraventricular conduction was found. The effect lasted 10 min. The baseline FBS was 90 mg/dl and at the end of the study 105 mg/dl. (N.S.).

Conclusions This shows that glucagon improves the sinus node and atrioventricular function, but not the intraventricular function. No other cardiac function was affected. Elevation of B.S. was observe, but not significant. We can conclude that the conduction system is improved by glucagon without other cardiac abnormalities. This shows that glucagon can be used in another compartment in the artificial pancreas to avoid hypoglycemia episodes, if any malfunction occurs in the device.

Adult clinical case symposium, 12:00 PM, Thursday, February 22, 2018

9 Pelvic floor muscle exercise-based behavioural therapy improves urinary symptoms in parkinson disease

CP Vaughan1,2

K Burgio3,4

P Goode3,4

J Juncos2

L Muirhead1,2

G McGwin3,4

T Johnson1,2

1Atlanta VA, Atlanta, GA

2Emory University, Atlanta, GA

3Birmingham VA, Birmingham, AL

4University of Alabama at Birmingham, Birmingham, AL

Purpose of study Determine the efficacy of pelvic floor muscle-exercise based behavioural therapy (BET) for overactive bladder (OAB) in Parkinson disease (PD).

Methods used Randomised trial of BET compared to control (CON) conducted at two VA medical centres. Participants were diagnosed with PD by a movement disorders neurologist and had ≥4 episodes of weekly UI. BET included pelvic floor muscle exercises with urge suppression training, fluid modification, constipation management, and self-monitoring with a bladder diary. CON included a bladder diary and mirrored shape drawing. Outcomes were the International Consultation on Incontinence OAB questionnaire (range 0–16, higher=worse) and bladder diary-based weekly UI eight weeks post-randomization. Outcomes analysed using generalised linear models adjusted for baseline symptoms.

Summary of results 53 participants were randomised and 47 reported outcome data including 26 randomised to BET and 21 to CON (6 dropouts in CON). BET vs CON participants were similar with respect to age (71.0±6.1 vs 69.7±8.2 years), gender (70% vs 78% male), MDS-UPDRS part 3 motor score (25.6±13.4 vs 23.8±15.7), cognition (MoCA 23.5±3.1 vs 24.9±2.4), mean weekly UI episodes (13.8±9.8 vs 15.2±11.1) and OAB symptoms (8.9±2.4 vs 8.3±2.2). BET reported greater reduction in OAB symptoms compared to CON ((−3.1±2.8) vs (−1.7±2.2), p=0.04). Weekly UI reduction was similar between BET (−7.0±8.9) and CON (−4.8±12.7) (p=0.5). QOL and bother from OAB were significantly improved in BET (p<0.0001 for both) compared to baseline.

Conclusions Behavioural therapy improved overactive bladder symptoms in Parkinson disease. Bladder diary self-monitoring was associated with urinary incontinence reduction in both groups. Providers should consider behavioural therapy as initial therapy for overactive bladder symptoms in Parkinson disease.

10 Reliability of a novel index of senescent cell abundance in human adipose tissue

L Raborn1

J Justice2

H Gregory2

B Nicklas2

J Ding2

S Kritchevsky2

J Williamson2

1LSUHSC – NO Medical School, New Orleans, LA

2Wake Forest School of Medicine, Winston-Salem, NC

Purpose of study We sought to evaluate reliability of scoring senescent cell abundance by immunohistochemical [IHC] staining of p16INK4a+ cells in adipose tissue.

Methods used Cells expressing p16INK4a were identified by IHC staining of adipose tissues obtained from women who were older (69.8±7.1 years) and overweight/obese (BMI: 32.2±2.9 kg/m2). Fields were viewed at 20 × magnification using OlyVIA pathology software; 15–20 fields per sample at were chosen randomly, and images screen-captured for offline quantification. Two independent examiners reviewed each visual field and recorded total cells counted, and cells expressing Strong, Medium, or Weak intensity positive nuclear staining for p16INK4a. Cell counts were compared between examiners, and inter-rater reliability was assessed by partial correlations adjusting for sample (r) and intra-class correlation coefficients (ICC) per sample.

Summary of results A total of 115 visual fields were analysed from 6 adipose tissue samples. Overall, an average of 38.0±27 cells were counted per visual field, and 6.6%±5.1% p16INK4a+ cells scored Strong (0.58±0.96 p16INK4a+ cells per field) or Medium (1.78±1.5 p16INK4a+ cells per field) signal intensity, and 14.7%±8.8% p16INK4a+ at any intensity. Significant differences between raters were observed in cell counts with Weak signal intensity (3.76±3.1 vs 1.83±1.2 counts per field, p<0.05), but not total cell counts, or Medium/Strong intensity cell counts (p≥0.05, all). Between-rater partial correlations for p16INK4a+ counts per field were: Weak r=0.61; Medium r=0.67; Strong r=0.83 (p<0.05 all). Between-rater consistency was observed in: total cell counts per field (ICC=0.98), and p16INK4a+ cell counts of Medium (ICC=0.89) or Strong (ICC=0.99) intensity (p<0.05 all), but not Weak (ICC=0.64, p≥0.05).

Conclusions Adipose tissue senescent cells that stain p16INK4a+ with Medium or Strong signal intensity can be reliably identified and quantified between raters. This study provides evidence for the utility of the adipose tissue p16INK4a + cell abundance as a biomarker of cellular senescence in older adults.

11 Intraepidermal blisters vs re-epithelialization of subepidermal blisters: the spectrum of histopathologic findings in bullous pemphigoid

BD Hodge

R Brodell

J Mitchell

AB Googe

T Patel

J Schulmeier

University of Mississippi School of Medicine, Jackson, MS

Purpose of study To examine the incidence of re-epithelialization of subepidermal blisters causing the appearance of an intraepidermal blistering process leading to diagnostic challenges.

Methods used A search of positive immunofluorescence reports (2006–2015) confirming the presence of autoimmune subepidermal blistering was performed and then compared to hematoxylin and eosin (H and E) findings to identify cases of bullous pemphigoid. The presence of subepidermal blistering, intraepidermal blistering, absence of blistering reflecting urticarial and eczematous pemphigoid, and evidence of partial re-epithelialization were tabulated. Cases were not counted on a per-patient basis but rather per biopsy location; therefore, two biopsies from one patient were reported as two cases.

Summary of results 77 cases were identified as having both immunoflouresence and histopathologic features indicative of an autoimmune subepidermal blistering process. Of these cases, 43 showed dermal-epidermal blisters (55.84%), 12 showed features of urticarial or eczematous pemphigoid (15.58%), 12 showed at least focal intraepidermal blistering (15.58%), and 10 showed complete re-epithelialization producing the appearance of an intraepidermal blister (12.99%). A limitation of this study is the possibility that subepidermal autoimmune processes other than bullous pemphigoid (epidermolysis bullosa acquisita and cicatricial pemphigoid) could have been included in this analysis.

Conclusions Re-epithelialization of subepidermal blisters can pose diagnostic challenges as it leads the clinician to suspect an intraepidermal blistering process. By starting with immunofluorescent findings typical of pemphigoid and then reviewing H and E findings, it was determined that 55.84% of the time, histology demonstrated classic dermal-epidermal blistering. In 12.99% of specimens, however, histology showed complete re-epithelialization of the blister floor. Thus, the presence of an intraepidermal blister does not preclude the diagnosis of a sub-epidermal blistering process such as pemphigoid highlighting the importance of clinical-pathologic correlation. Biopsies taken of newer lesions are less likely to show this phenomenon.

12 Langerhans cell histiocytosis wreaks havoc on the hypothalamus

C Bicknese

M McLemore

A Ioachimescu

Emory University School of Medicine, Atlanta, GA

Case report A 23-year-old previously healthy man presented with polyuria, polydipsia of 10 month duration. One month prior to the endocrinology visit, a polyp was removed from the right ear canal after he complained of ear pain. Pathology revealed Langerhans cell histiocytosis (neoplastic cells stained for CD45, S100, CD1a, and vimentin). Other symptoms included fatigue and decreased libido. On physical exam, the 98 kg patient appeared euvolemic. Fluid balance was negative: intake 11.49/output 11.52 L. Laboratory studies confirmed diabetes insipidus: serum sodium 144 (136–145), urine osmolality 87 mOsm/kg (300–900 mOsm/kg), and central hypogonadism: undetectable testosterone (280–1,1,00 ng/dL), luteinizing hormone 1.0 mlU/mL (1.2–8.6 mlU/mL), follicle stimulating hormone 2.62 mlU/mL (1.2–19.2 mlU/mL). Prolactin was slightly elevated (17 ng/mL; 3–13 ng/mL), cortisol, thyroid and IGF-1 levels were normal. MRI of the brain showed mass-like enhancement of the hypothalamus and superior infundibulum, and enhancing lesions of right parietal calvarium and mastoids extending to the external auditory canal. PET scan did not reveal other lesions. Ophthalmology evaluation was normal. The patient received monthly cytarabine, oral desmopressin and transdermal testosterone. After 3 cycles of chemotherapy, brain MRI and PET scans showed improved appearance of hypothalamic and bone lesions.

Discussion LCH is a rare granulomatous disease characterised by abnormal expansion of dendritic cells. The estimated incidence is 3–5/million in children and 1–2/million in adults. Sites involved usually in adults are bones, lungs and skin. CNS-LCH is rare and usually associated with multisystem disease. Both anterior and posterior hypothalamic-pituitary axis (HPA) can be involved and up to 30% of patients have diabetes insipidus. There is currently no standardised therapy for CNS-LCH. Some patients are conservatively monitored while other receive high-dose steroids, chemotherapy (vinblastine, etoposide, cytarabine) or radiation. While prospective studies are lacking, hypopituitarism persists in most cases after treatment.

Conclusion LCH should be considered in the differential diagnosis of diabetes insipidus and hypothalamic masses. Early diagnosis is important as LCH is a multisystem organ disease that may progress in absence of therapy.

13 To diagnose it, you need to think of it

S Chalasani

L Maher

University of Mississippi, Jackson, MS

Case report Immunoglobulin G4-related disease (IgG4-RD) is rare immune-mediated condition affecting multiple organs such pancreas, kidney, lung, salivary glands, lymph nodes and skin with a characteristic lymphoplasmacytic infiltrate enriched in IgG4 positive plasma cells. It occurs mostly in older males with history of allergic diseases. Here we present a patient who had multiple hospitalizations with nonspecific symptoms and extensive work up.

A 64 year old male with past medical history of hypertension, dyslipidemia and diabetes comes for evaluation of 6 months history of nausea, vomiting, abdominal pain and weight loss. Patient had multiple hospitalizations and underwent an extensive work-up which showed hepatomegaly with a pancreatic mass and intrahepatic lesions as well as lymphadenopathy and an abnormal SPEP without a discrete monoclonal spike to point to multiple myeloma. He was seen in allergy clinic 8 months prior for evaluation of hives, rhinitis, and dyspnea. He was diagnosed with asthma and was started on Albuterol. His rheumatologic and infectious work up was negative. He had endoscopy and colonoscopy with biopsies which did not show any evidence of malignancy. He underwent right axillary lymph node biopsy that showed hyperplastic features with negative flow cytometry. Since he had elevated IgG and IgG4 level of 3360, a liver biopsy was performed that showed dense portal lymphoplasmacytic infiltrates with predominance of IgG4 plasma cells. A diagnose of IgG4-related disease was made and he was started on high dose steroids with significant clinical improvement. In addition, he received 2 infusions of 1 gram Rituximab 2 weeks apart with plans to repeat imaging in future.

IgG4-related diseases is heterogeneous disorder involving multiple organs and presents with nonspecific symptoms. The gold standard for the diagnosis is dense lymphoplasmacytic infiltrates enriched in IgG4-positive plasma cells organised in a storiform pattern with fibrosis and obliterative phlebitis. Serologies are negative except for the elevated IgG4 (>135 mg/dL) seen in 60% to 70% of cases which can be a marker for a more aggressive disease course. Treatment includes immunosuppressive drugs; of these, rituximab appears to be most effective. Due to the rarity of the disease and nonspecific symptoms, a high index of suspicion is needed for early diagnosis and treatment.

14 A rare vascular cause of recurrent pneumonia

MA Alawoki

EK Addo-Yobo

B Boonpheng

N Kakkar

C Rosero

DM Pierce

East Tennessee State University, Johnson City, TN

Case report Pneumonia is the infection of the lung parenchyma that can be life-threatening if not managed appropriately. Most community acquired pneumonias have good prognosis, with majority recovering within a month. However, when it is recurrent in the same anatomical location, it is important to do further investigation to look for the cause. With this inquisitive clinical approach, an undiagnosed aberrant right subclavian artery (ARSA) was found to be the cause of recurrent pneumonia.

A sixty five year old female with a recent history of two episodes of right middle lobe(RML) pneumonia, within the previous five months, presented for a hospital follow up. Chest X-ray from both hospital visits suggested RML processes which resolved with levofloxacin. A detailed history during our encounter uncovered intermittent dysphagia to solids and liquids. The recurrent RML pneumonia and the remote history of dysphagia suggested significant pulmonary aspiration. A video fluoroscopy showed compression of the oesophagus. A CAT scan showed the presence of an ARSA that was compressing the upper oesophagus.

We discovered that an ARSA compressed the upper oesophagus, which caused recurrent aspiration pneumonia. ARSA is an uncommon congenital anatomical variant where the right subclavian artery comes directly off the aortic arch instead of the brachiocephalic trunk. It travels posterior to the oesophagus towards the right upper extremity; causing esophageal compression. If symptomatic, it will usually present in infants with respiratory symptoms or after decades as dysphagia along with chest pain, hoarseness or anorexia. Our patient had two upper endoscopies however no imaging study was done to evaluate for extrinsic esophageal compression. Despite the appropriate workup, vascular causes can be missed if not considered.

This case demonstrated a presentation of an ARSA compressing the upper oesophagus and causing dysphagia in a patient with recurrent RML pneumonia. Patients with recurrent pneumonia in the same anatomic location needs further workup and detailed questioning about dysphagia or aspiration events.

15 Acute confusion as a sequela of pepto-bismol toxicity

A Prystupa

S Ahmed

RS Urban

Texas Tech Univ HSC Amarillo, Amarillo, TX

Case 61-year-old female with PMH of hypertension presented with acute confusion, hand tremors, and gait ataxia. Workup revealed negative RPR, normal TSH and Vitamin B12. Urine toxicology, blood alcohol, salicylate, and acetaminophen levels were negative. Lab work uncovered refractory hypokalemia and normal AG metabolic acidosis. Non-contrast head CT and brain MRI showed age-related atrophy, subacute/chronic bilateral thalamic enhancement and old basal ganglia lacunar infarctions. Family disclosed her overuse of Pepto-Bismol for dyspepsia. Bismuth toxicity was suspected due to progressive neurological decline, new onset RTA and history of Pepto-Bismol use. High blood and urine bismuth level confirmed the diagnosis. Patient was treated conservatively with discontinuation of Pepto-Bismol and serial follow-up. At 4 weeks we detected resolution of tremor, gait ataxia, ankle clonus, and marked improvement of memory.

Impact Bismuth is a heavy metal and an active ingredient of a popular and usually safe OTC medication, Pepto-Bismol (bismuth subsalicylate). Its chronic use can lead to bismuth intoxication manifesting as memory change, confusion, depression, insomnia, ataxia, tremor, myoclonus, seizures and coma.

Discussion Acute or chronic ingestion of toxic dose of bismuth can lead to progressive confusion, myoclonus, lack of coordination, and speech disturbance. Bi binds to sulfhydryl groups, leading to white matter changes in CNS and proximal tubule damage in the kidney. Suggestive history, high blood and urine bismuth levels confirm the diagnosis of toxicity. Management is symptomatic with a gradual but unpredictable improvement after discontinuation of product. Extensive search for infectious and metabolic causes of encephalopathy was negative in our patient. The history of chronic bismuth use with high blood and urine levels and typical clinical findings with subsequent resolution of symptoms confirmed the diagnosis of bismuth encephalopathy. Our patient was treated conservatively and followed the typical course of Bi encephalopathy with gradual improvement at follow-up. More detailed labelling would increase awareness to avoid this potential toxicity. Bi toxicity should be considered in a patient presenting with suggestive history and symptoms.

16 Rapidly progressing htlv-1 associated myelopathy/tropical spastic paraparesis presenting as bilateral lower extremity weakness

B Nguyen

M Gutierrez

K Rizg

C Palacio

J Shah

University of Florida College of Medicine, Jacksonville, FL

Case report Patient is a 60 y/o African American male with a history of HTN and seizures who presented for a six-month history of bilateral lower extremity weakness that worsened to the point where he was unable to walk. Associated symptoms included upper extremity tremors, urinary retention and repeated falls. Remarkable labs included mildly elevated CK at 329, with negative RPR, Lyme, ANA, SSA/SSB, Aldolase, immunofixation, and HIV. MRI of neuro axis was done which showed no masses, no acute findings, and mild degenerative changes in C and L spine with minor disc bulging and mild neuro foraminal stenosis in L spine without any cord changes. Previous notes revealed an episode of uveitis in March 2015, but that there were no reported complaints of lower extremity weakness prior to the initial presentation. During outpatient Neurology follow-up, it was thought that his symptoms were consistent with a neurodegenerative process. Vitamin B12, Folate, MMA, Ceruloplasmin, and HTLV-1 were ordered and HTLV-1 was positive. He had multiple hospitalizations for progression of symptoms and was eventually diagnosed with TSP based on WHO criteria as lumbar puncture was deferred. He was sent to rehab for physical therapy but due to progression of disease leaving him mostly bedbound, he developed complications of sacral decubitus ulcers, recurrent UTI’s and died secondary to sepsis only 14 months after onset of symptoms.

Human T-Lymphotropic virus 1 (HTLV-1) has been implicated in multiple diseases such as HTLV-1 associated myelopathy/tropical spastic paraparesis (TSP), an insidious disease with progression over 12–24 years. However, we report a case of TSP with rapid progression over 14 months, which contributed to his demise. Possibly, due to the rapid course, this patient did not have cord atrophy that is often seen on MRI of patients with severe, chronic TSP. CSF serology studies should be considered during initial admission as this may help to confirm the diagnosis and to identify markers of a more rapid course of TSP in the future. We suggest that if a rapid course of TSP is suspected, Psychiatry should be involved early on as patients can become demotivated and decompensate rapidly.

17 Left ventricular non-compaction cardiomyopathy: case of a rare congenital disorder presenting with multiple organ failure

I Serrano

S Lulla

U Warden

B Al-Turk

S Maharaj

University of Florida, Jacksonville, FL

Case report A 42-year-old African American male with past medical history of chronic kidney disease and hypertension presented to the hospital with complaints of worsening dyspnea, cough and orthopnea for one month. On presentation, he was found to be volume overloaded with significant bilateral lower extremity oedema, rales and a pro-BNP of 11 500. A transthoracic echocardiogram revealed severe mitral regurgitation, an ejection fraction of 25%–30% and left ventricular enlargement with trabeculations consistent with non-compaction cardiomyopathy. After diuresis and optimisation of volume status, he underwent left heart catheterization with findings of non-obstructive coronary artery disease. Patient was initiated on goal directed medical therapy for heart failure and discharged. Months later an echocardiogram was repeated with no recovery in function. Medical therapy was optimised and a dual chamber-ICD was placed.

Two years later, the patient returned with complaints of dyspnea, palpitations, and oedema. He was found to be in atrial fibrillation with rapid ventricular response and decompensated heart failure. The morning after admission, the patient had a new onset seizure and shortly thereafter went into PEA arrest. Return of spontaneous circulation was achieved after 10 min and required endotracheal intubation. During hospital course the patient’s renal function worsened with minimal urine output, eventually requiring hemodialysis. A repeat echocardiogram revealed a worsened ejection fraction of 10%–15%.

Discussion Left ventricular non-compaction (LVNC) is a rare congenital heart disorder that occurs in-utero due to arrest in the compaction of the developing myocardium, resulting in ‘spongy’ appearance of the left ventricle and thick myocardial wall. It is a genetic cardiomyopathy that presents with heart failure, ventricular arrhythmias, systemic embolism or sudden death. It has a prevalence of 0.01% to 1.3% on echocardiogram. This case presents a middle-age man who developed rapidly worsening of heart function, arrhythmias, renal failure, PEA arrest and hypoxic respiratory failure as subsequent complications from LVNC cardiomyopathy.

18 Serial dilation for complicated extrinsic cervical esophageal strictures

CC White

Eisenhower Medical Centre, Evans, GA

Purpose of study The unconventional use of serial mechanical dilations of extrinsic cervical esophageal strictures has been attempted with promising results.

Methods used Our patient is a 63 year old female with a history of stage 3 metastatic papillary thyroid carcinoma status post total thyroidectomy with selective right neck dissection who presented to our service with progressive dysphagia and hoarseness. Two years following her initial surgical management, she had recurrence of her disease requiring right radical neck dissection and radiation. She developed vocal cord paralysis and became tracheostomy dependent for more than seven years until a cordotomy was performed. She experienced persistent dysphagia and hoarseness following the placement of her tracheostomy. Despite maximal medical therapy, she often experienced food regurgitation requiring additional chewing to facilitate swallowing. She was evaluated with upper endoscopy, which revealed extrinsic esophageal strictures. ENT assessed for the patient for symptomatic relief and was deemed unable to provide any assistance because of her pathology. Both the aetiology and location of the stricture limited intra-lesional steroid therapy and stent placement. An upper endoscopy was performed and the stricture was bypassed utilising a specialised scope. A wire was placed and the stricture was dilated to an estimated of 10 mm. She has required 12 additional dilations at about 4 weeks interval over a year. The most recent dilation was 18 mm. As expected, there has been restenosis following each procedure and the strictures appeared more open and stable. Marked clinical improvement was noted with our therapy and there has been no suggestion of significant adverse effects.

Summary of results Progressive serial dilation is a safe and effective method of treatment for complex extrinsic cervical esophageal strictures not amenable to alternative therapies. There is minimal restenosis effect after dilation as the frequency of the dilations increases. The appropriate frequency of dilations is dependent on the patient’s response to therapy. To date, progressive serial dilations appear safe and effective for severe, complex esophageal strictures.

Conclusions Progressive serial dilation is a safe and effective method of treatment for complex extrinsic cervical esophageal strictures not amenable to alternative therapies.

Neonatal case report symposium, 12:00 PM, Thursday, February 22, 2018

19 Acute alcohol intoxication and withdrawal in a premature infant

MG Johnson

ER Miller

AC Farris

University of Louisville, Louisville, KY

Case report Acute alcohol intoxication in an infant has rarely been reported, and the majority of reported cases have involved exposure after delivery in term infants. Here we report a case of a preterm infant with acute alcohol intoxication at birth.

A male infant at 27 weeks gestation was admitted to the NICU for acute alcohol intoxication, metabolic acidosis and prematurity. The pregnancy was complicated by extensive maternal alcohol abuse and scant prenatal care. Maternal blood alcohol level was 382 mg/dL prior to delivery. The infant delivered precipitously prior to NICU team arrival and Apgar scores were 1, 5, and 7 at 1, 5, and 10 min. The infant was quickly intubated and transferred to the NICU while the mother was transferred to intensive care for acute alcohol withdrawal. On admission, the infant demonstrated severe anion gap metabolic acidosis with pH 7.00 and bicarbonate 7 mmol/L. The infant’s blood alcohol level was 132 mg/dL at nine hours of life. Despite aggressive fluid resuscitation, sodium bicarbonate administration, and appropriate mechanical ventilation, the metabolic acidosis persisted with a lactic acid level of 16 mmol/L at thirteen hours of life. Liver dysfunction was noted with transaminitis and coagulopathy requiring cryoprecipitate and platelet transfusions. Lorazepam was initiated on day of life two for increased jitteriness and agitation presumed to be secondary to alcohol withdrawal and was continued for several days as the anion gap acidosis gradually resolved.

Ethanol freely crosses the placenta and is a well-known teratogen. Ethanol for umbilical cord care has been the most common reported source of acute infant intoxication, although there are also reports of ingestion related to child abuse. Only one case report from 1967 described an infant with acute alcohol intoxication immediately after birth, and no case reports were found describing acute intoxication in a premature infant. Infants with acute alcohol intoxication can present with a variety of symptoms including hypothermia, tachycardia, poor suck, lethargy, seizures and coma. Associated lab findings include anion gap metabolic acidosis and hypoglycemia. Therapy includes treatment of acidosis and electrolyte abnormalities and supportive care including antiepileptic medications and respiratory support as needed.

20 Hematochezia in a neonate: think beyond necrotizing enterocolitis

FJ Zaidi

P Agarwal

D Macariola

S Hollinger

East Tennessee State University, Johnson City, TN

Case report A 37 week late preterm female born vaginally presented at 36 hours of life with neonatal abstinence syndrome. Delivery was uneventful. Maternal history was positive for hepatitis C, polysubstance abuse and poor prenatal care. Mother denied having any genital infection during her entire pregnancy. On admission, physical examination was unremarkable except for irritability and mildly increased tone. She was treated with morphine for a total of 10 days. Withdrawal symptoms resolved but she remained in NICU due to poor feeding. Patient required nutritional support but was otherwise doing well until day of life (DOL) 16, when she developed severe rectal bleeding associated with hypothermia and respiratory distress. She was initially placed on supplemental oxygen via nasal cannula but with increasing oxygen requirements she eventually needed to be on high frequency jet ventilation. The patient was kept NPO and was started on Ampicillin and Gentamicin. Sepsis work up was obtained and infectious disease was consulted. Blood and urine cultures stayed negative throughout. CRP levels raised from <0.5 on admission to 45.0 mg/L. KUB, CXR and urinalysis were unremarkable. On DOL 17, the patient developed significant apnea, bradycardia, and hepatomegaly along with thrombocytopenia. She was treated with IV acyclovir and was transfused with platelets. Rectal bleeding worsened and was treated with fresh frozen plasma. AST and ALT were >2600 IU/L while platelet count dropped significantly from 2 53 000 to 14,000 K/uL. Haematology/Oncology was consulted who recommended parvovirus PCR, DIC panel and IVIG infusion. The patient progressively deteriorated, developed fulminant hepatitis, acute renal failure and disseminated intravascular coagulopathy. Despite assisted ventilation, vasopressor support, multiple blood products, and aggressive antiviral therapy, the patient passed away on DOL 19. The results of serum HSV DNA PCR came back positive post mortem.

Disseminated cases constitute only one quarter of HSV cases but have the highest mortality of 85% in untreated patients. In around 30% of cases, there are no cutaneous manifestations making an accurate diagnosis of HSV challenging. We report a neonate with rectal bleeding as the initial presenting symptom of disseminated HSV infection.

21 An infant with congenital myotonic dystrophy phenotype

K Jeffries

J Philips

University of Alabama, Birmingham, AL

Introduction Congenital myotonic dystrophy (CMD) is a rare condition that presents with hypotonia and often respiratory distress. We present a severe case.

Case report This 2,130 g female infant was born at 34 weeks by Caesarian for NRFHT and polyhydramnios to a 28 y/o mother with a history of a prior 35 week infant who died shortly after birth from respiratory failure. The case infant was hypotonic with no respiratory effort at birth and was intubated and placed on a ventilator. Apgar scores were 1, 1, and 2 at 1, 5, and 10 min, respectively and were primarily due to profound hypotonia. Admission exam showed Jeffries K severe hypotonia and chest X-ray showed thin ribs. The mother has a positive family history for CMD, exhibits myotonic facies,and has difficulty releasing her hand after a handshake. However, array CGH was normal and karyotype revealed a balanced translocation between chromosomes 17q and 19q13. The infant failed numerous attempts at extubation and had severe feeding intolerance for which she received a tracheostomy and feeding gastrostomy. She has developed bronchopulmonary dysplasia, persistent patent ductus arteriosus with left-to-right shunt and right ventricular hypertrophy. At 6 months of age,she is tolerating 30 min trials of continuous positive airway pressure every 4 hours in an attempt to wean from the ventilator. She is on continuous gastrojejunal tube feedings with OG tube clamping for one hour every four hours. Profound hypotonia persists.

Discussion CMD is a rare autosomal dominant genetic disorder with an incidence of about 1 in 45 to 50 thousand live births. Inheritance is almost always from an affected mother although parental inheritance has been documented. The aetiology is an unstable CTG repeat in an untranslated region of the dystrophia myotonica protein kinase (DMPK) gene. These repeats in the mRNA inhibit proper splicing and result in disease. The more CTG repeats there are, the more severe the disease. Infants with greater than 1000 repeats manifest disease in the newborn period while individuals with fewer repeats have onset later in life.

Conclusion All infants presenting with hypotonia in the newborn period should have a family history taken and be evaluated for the possibility of this disease.

22 A rare case of combined genetic syndromes mowat wilson and mbd5 neurodevelopmental disorder

B Gavan

T Chatmethakul

J Martinez

F Eyal

University of South Alabama, Mobile, AL

Introduction Mowat Wilson Syndrome (MWS) is the result of pathogenic variants and deletions of the ZEB2 gene 2q22.2 characterised by microcephaly, mental retardation, distinct facial features with or without Hirschprungs disease. MBD5 neurodevelopmental disorder is characterised by significantly below average intellectual functioning associated with impairments in adaptive behaviour.

We report a female newborn that presented with a phenotypic presentation of MWS who had a large deletion at chromosome 2q.22.2.

Case Our patient is an African American female infant born to a 29 years old Gravida 2 Para 1 mother with a past medical history of sarcoidosis. Prenatal history was significant for idiopathic polyhydramnios but otherwise unremarkable with no maternal medication exposure during prenatal period. There was no family history of mental retardation, consanguineous marriage or congenital anomalies. Patient was born at gestational age of 37 weeks by caesarean section secondary to previous maternal caesarean section with no complications.

At birth, patient was noted to be symmetrically SGA on exam with distinctive facial features including hypertelorism, prominent nasal bridge, tapered digits and acrodysplasia. On day of life 2, the patient developed increased abdominal circumference, delayed passage of meconium and decreased urine output. A contrast enema was concerning for Hirschprungs disease, which was later confirmed with tissue pathology showing aganglionic cells up to the splenic flexure.

Genetics testing with chromosomal micro array showed deletion at chromosome 2q.22.2 of 9.6 MB in size, which included 25 genes. Of these 25 genes ZEB2 and MBD5 were deleted yielding diagnosis of MWS and MBD5 neurodevelopmental disorder.

Discussion A Majority of reported cases of MWS are associated with ZEB2 gene mutation. However, our patient’s presentation is secondary to a unique large deletion, which contains deletion of 25 genes including ZEB2 and MBD5 which from the literature we have been unable to find another deletion like it. These genes play a major role in neurodevelopment, so monitoring of her neurodevelopmental milestones and seizure disorder is expected to be crucial and only time will tell.

23 Unusual breathing pattern in a neonate

ES Farnsworth

B Drabu

University of Oklahoma Health Science Centre, Edmond, OK

Introduction For most term newborns, respiratory distress is transient, with major causes being transient tachypnea of the newborn, respiratory distress syndrome, or persistent pulmonary hypertension. Although rare, anatomical causes may be an aetiology of respiratory distress. In this case we describe a patient who was in apparent respiratory distress, but later diagnosed with a genetic condition causing anatomical dysfunction.

Case report A male infant was born to a 35 year-old G4P2 at 39 week gestation via scheduled caesarean section, with no complications during pregnancy or delivery. Apgar scores were 7 and 8 at 1 and 5 min, respectively. However, at a few hours old, his chest appeared to move oddly whenever he took a breath, making it seem that he was in respiratory distress. On physical examination the infant had an asymmetric chest, with an area in right upper chest, approximately at the second rib, with paradoxical movement with inspiration. He had excellent oxygen saturation, normal respiratory rate, and no signs of increased work of breathing, including grunting or retractions. On further examination the patient appeared to be missing musculature over the right chest. The remainder of his newborn examination, including newborn reflexes and hip examination were normal. A chest ultrasound showed missing vs severely underdeveloped right pectoralis muscle, consistent with the diagnosis of Poland syndrome. An echocardiogram and renal ultrasound were obtained to rule out congenital structural abnormalities and both were unremarkable. He was admitted to the newborn unit and managed routinely. He remained stable on room air during admission. Because his mother had a history of neonatal death of a male infant from osteogenesis imperfecta, a skeletal survey was also performed on the patient, and no fractures were observed. Genetic studies had also been performed in utero with no concerns.

Discussion This case is clinically significant in that apparent respiratory distress in an infant can actually be just unusual breathing due to an anatomical reason, like Poland syndrome. Although additional imaging was done for this particular infant because of concerning family history, this case demonstrates that a full detailed physical exam at birth can decrease the need for unnecessary testing in a newborn.

24 Persistent tachypnea in a term neonate diagnosed with neuroendocrine cell hyperplasia of infancy

J Gallois

C Mumphrey

J Patrick

LSUHSC School of Medicine, New Orleans, LA

Case report Neuroendocrine cell hyperplasia of infancy (NEHI), formerly known as persistent tachypnea of infancy, is a form of childhood interstitial lung disease characterised by tachypnea, retractions, crackles, and hypoxia. Symptoms can appear at birth, but can present at 3 months of life or greater. Although the diagnostic gold standard for NEHI is increased neuroendocrine cells on lung biopsy, the diagnosis can be made with clinical and radiographic findings.

We present a term male infant who required oxygen by non- invasive positive pressure ventilation for desaturations and severe persistent pulmonary hypertension (PPHN) at birth. The PPHN resolved within two days and the hypoxia resolved within one week. The tachypnea persisted after resolution of PPHN and hypoxia. Infectious work up was negative. Inhaled albuterol, chest physiotherapy, flow from nasal cannula and oral diuretics failed to improve the tachypnea. Initial chest radiography showed bilateral interstitial densities that persisted on subsequent chest radiographs. Lung CT scan was notable for diffuse ground glass opacities consistent with NEHI. The patient was able to safely nipple and breastfeed all feeds. The patient was discharged on day of life eighteen with persistent tachypnea, and close follow up for weight gain.

The incidence of NEHI is not known, but is rare, and epidemiologic data are largely from case series reports. Increased awareness and clinical suspicion in the neonatal population are important as the diagnosis may go unrecognised. Over time, most patients show clinical improvement without intervention and radiographic changes persist regardless of the initial severity of the disease. The diagnosis of NEHI generally supports a good prognosis.

25 A bumpy road: subcutaneous fat necrosis of the newborn associated with symptomatic hypercalcemia

DL Charles

U Narsinghani

Navicent Health, Macon, GA

Case report We report the case of a 39 week female born via emergency c-section for loss of fetal heart tones. Infant received CPR. Apgars were 0 and 2 at 1 and 5 min. Initial studies revealed severe metabolic acidosis which improved after resuscitation and mechanical ventilation. Infant was subjected to hypothermia protocol for 72 hours. Seizures were noted and treated with Phenobarbital.

Patient was discharged home on day of life 11 and readmitted on day 14 with fever and lethargy. Patient underwent full sepsis workup. Initial vital signs: Temp 37.7, RR 52, Pulse 159, BP 72/54, Sp02 100%. On exam, patient had erythematous nodules on her back since day 11 of life. Total and Ionised calcium (Ca) were both elevated at 11 mg/dl and 1.32 mMol/L, respectively. Additional lab work was normal. Patient was treated with empiric antibiotics and IV hydration. Patient was diagnosed with subcutaneous fat necrosis of the newborn (SCFN) based on clinical findings. Symptoms improved with supportive care and patient was discharged home at 72 hours. Skin changes resolved by 2 months of life.

SCFN is a self-limiting panniculitis affecting newborns who have birth asphyxia, experience perinatal stress including maternal hypertension or are subjected to therapeutic hypothermia. This condition is characterised by erythematous, violaceous subcutaneous nodules, red to purple in colour which develop on the cheeks, trunk, or extremities. Although, self-limiting, it may be associated with thrombocytopenia, hypoglycemia, hypertriglyceridemia, and symptomatic hypercalcemia. Symptoms of hypercalcemia may include lethargy, poor feeding, nephrocalcinosis and fever. The specific cause is unknown, but may be related to increased levels of 1α-hydroxylase which stimulates Ca absorption in the intestine and promotes Ca mobilisation from bone which could lead to secondary hypercalcemia. Hypercalcemia is a life-threatening complication that requires close monitoring including IV hydration, use of Ca-wasting loop diuretics and restricting Ca and Vitamin D intake. Diagnosis of SCFN is clinical and most lesions resolve within weeks to months.

Reference

  1. . Del Pozzo-Magana BR, Ho N. Subcutaneous fat necrosis of the newborn: A 20-year retrospective study. Pediatr Dermatol2016;33:e353.

26 Successful angiojet® aortic thrombectomy of ecmo-related thrombus in a newborn

ME Gutierrez

M Law

J Alten

University of Alabama at Birmingham, Vestavia Hills, AL

Case report Thrombosis and systemic embolization are morbid complications of extracorporeal membrane oxygenation (ECMO). We present a 2.5 kg newborn with hypoplastic left heart who required ECMO support after a cardiac arrest. A ECMO associated thromboembolism resulting in occlusive distal aortic thrombus was subsequently managed by transcatheter Angiojet (Boston Scientific, Boston, MA) thrombectomy. The procedure successfully restored perfusion to the lower extremities, confirmed by angiography. This case is reports of the use of Angiojet thrombectomy in a newborn on ECMO support with complex congenital heart disease.

27 Central diabetes insipidus: a rare complication of intraventricular haemorrhage in a preterm infant

P Thakore

A Dunbar

EB Lindsay

Tulane-Ochsner Paediatric Residency Program, Metairie, LA

Case report A 710 g male infant was born at a referring hospital at a gestational age of 23 weeks and 2 days via vaginal delivery and was transferred to our facility at 14 days of age. His delivery was complicated by breech presentation with difficult head extraction. The infant’s initial course was significant for respiratory distress syndrome, grade III-IV intraventricular haemorrhage (IVH), acute renal failure, and large PDA. On day of life 29, a gradual increase in serum sodium level which was refractory to increase in total fluid volume was noted. The combination of persistent hypernatremia (150–160 mmol/L), polyuria (8.4 ml/kg/hr), high plasma osmolality (323 mosm/kg), hyposthenuria (75 mosm/kg) and an undetectable serum ADH (<0.8 pg/ml) confirmed the diagnosis of Central Diabetes insipidus (CDI). Serum sodium and urine output decreased and urine osmolality increased after subcutaneous DDAVP administration and the DDAVP dose was titrated to achieve normal values.

CDI is an uncommon cause of hypernatremia in neonatal period. The diagnosis can be difficult as excessive urine output and high serum sodium can often be attributed to high insensible water loss in the extremely premature newborn. Persistent hypernatremia despite increased fluid intake combined with polyuria and hyposthenuria should increase suspicion for DI. The causes of neonatal CDI include asphyxia, severe bacterial infections such as meningitis, congenital CNS malformations, and intraventricular haemorrhage. CDI in our patient was thought to be due to grade III-IV IVH complicated by post haemorrhagic hydrocephalus. There are few reports describing CDI as a complication of IVH, and the incidence of CDI following IVH is unclear. In conclusion, the diagnosis of central DI should be considered as a complication of severe IVH in the extremely premature neonate who demonstrates persistent hypernatremia, polyuria, decreased urine osmolality, and increased plasma osmolality. Serum ADH levels can be helpful in confirming central origin of DI and subcutaneous desmopressin can be an effective treatment in the preterm infant.

28 Identifying barriers to diagnosis of infants with neonatal abstinence syndrome: a case report and review of literature

M Howell1

A Smith2

S Drury1,2

1Tulane University School of Medicine, Metairie, LA

2Tulane University School of Medicine, New Orleans, LA

Background Neonatal abstinence syndrome (NAS) is a growing public health concern. Accurate identification of infants at risk for NAS is crucial to quality neonatal care. Current efforts to identify mothers at risk and their infants have poor specificity and sensitivity.

Case report A 36 2/7 week infant was born to a mother with limited prenatal care and self-reported prenatal heroin/tobacco use, last 12 hours before delivery. APGARS were 8 and 9. Infant’s and mother’s urine toxicology were negative. NAS risk protocol was initiated. Within 24 hours, Finnegan scoring criteria for NAS was met and medication withdrawal protocol began. The infant was in the NICU for 30 days, with 16 days of withdrawal medication. Cleft palate compounded feeding difficulties associated with NAS. Meconium resulted positive for opiates after one week. Per state mandate, Department of Child and Family Services was notified. Baby was discharged home with mother.

Conclusions In this case, we highlight several factors. First, mothers often fail to disclose opioid use or to obtain adequate prenatal care. Often this is the result of shame, inadequate resources, fear of removal of infant from parents’ custody or lack of education about safe options during pregnancy, coupled with inadequate provider training/support. Second, the negative urine toxicology highlights the rapid metabolism of heroin and the limitations of universal and targeted drug screening. Community level education targeting moms, soon-to-moms and providers is needed. There remains a need to expand research related to multi-substance use exposure in infants. Table 1 highlights the variable phamacokinetics of different opioids and the limited data about rates of NAS following exposure.

Abstract 28 Table 1 Highlights the variable phamacokinetics of different opioids and the limited data about rates of NAS following exposure

Paediatric clinical case symposium, 12:00 PM, Thursday, February 22, 2018

29 Idiopathic dilated cardiomyopathy: an atypical presentation

VC Diaz Vidal

JP Scimeme

University of Florida College of Medicine Jacksonville, Jacksonville, FL

Case report Dilated cardiomyopathy is one of the most common cardiomyopathies in the paediatric population, but compared to other diseases affecting children, it is quite rare. Heart disease in children presents differently compared to adults. Most symptoms are non-specific and may resemble common conditions or illnesses, like asthma or gastroenteritis. Some children may complain of fatigue or dyspnea as the cardiomegaly worsens, and signs of heart failure can be subtle with only tachycardia or tachypnea as abnormal vital signs. Most children may also present with hepatomegaly and a gallop on auscultation suggesting signs of congestion later on. These subtle findings can make diagnosis difficult.

We present the case of a previously healthy 4-year-old male with the sudden loss of consciousness due to a cardio-embolic stroke affecting the left middle cerebral artery and anterior cerebral artery territories of the brain due to a left ventricular thrombus found on echocardiogram after cardiomegaly was seen on initial x-ray. He presented to his primary care physician with vague symptoms of fatigue, abdominal discomfort and decreased activity in April 2017 after a viral upper respiratory infection, 5 months before presentation. This case is a classic example of the subtle and non-specific symptoms that make the diagnosis of idiopathic dilated cardiomyopathy difficult in the paediatric population. We believe the dilated cardiomyopathy could have been secondary to viral myocarditis, most likely parvovirus B19, based on PCR results. Having a high index of suspicion is crucial to identifying and treating dilated cardiomyopathy early before irreversible complications arise.

Abstract 29 Figure 1

Initial chest X-ray demonstrating cardiomegaly and pleural effusions

30 Seizures, meds, and v-tach: a journey to a brugada diagnosis

D Fitzgerald

S Das

M Malone

S Schexnayder

University of Arkansas for Medical Sciences, Little Rock, AR

Purpose To describe a case of Brugada syndrome in a child treated with psychotropic medications who presented with seizures and cardiac arrest.

Methods Descriptive Case Report.

Summary of results A 10-year-old male presented with fever, status epilepticus, and sudden onset of pulseless ventricular tachycardia. The patient had seizure and behavioural disorders and was receiving quetiapine, doxepin, guanfacine, dexmethylphenidate, and lamotrigine. He received CPR, IV epinephrine, and was defibrillated once with return of spontaneous circulation (ROSC). IV amiodarone was given after ROSC. Initial ECG showed Type 1 coved ST segment elevation >2 mm in V1 followed by a negative T wave, diagnostic of Brugada syndrome. Elevation of transaminases developed, consistent with post-arrest organ dysfunction. Echocardiogram demonstrated normal structure and function. Electrophysiological study with IV procainamide challenge showed increased QRS duration, prolonged QTc, J point elevation, and inverted T waves in V1 suggestive of Brugada syndrome with Type I pattern. A subcutaneous implantable cardiac defibrillator (S-ICD) was placed. Lab studies normalised, as did his neurologic status.

Conclusions Brugada syndrome is an autosomal dominant genetic disorder associated with mutations in the SCN5A gene that encodes sodium channel function, and is characterised by abnormal findings on ECG. The case illustrates complicating factors in the diagnosis being confounded by psychotropic medications that affect cardiac conduction. Recognition of this unusual ECG pattern is paramount, given the potential consequence of sudden death. While implantation of an ICD is an uncommon paediatric procedure, the potential benefit of immediate cardioversion justifies aggressive measures to reduce the risk of lethal arrhythmias.

Abstract 30 Figure 1

Initial ECG

31 Chasing fever of unknown origin into the rabbit hole

S Clark

A Alsulami

S Boppana

University of Alabama-Birmingham, Birmingham, AL

Introduction Fever of unknown origin (FUO) is a common clinical problem in children that carries with it a vast differential, including infectious, rheumatologic, and neoplastic disorders. Diagnostic evaluation can become expansive and expensive and should be guided by the history and physical exam. Tularemia is a rare but important consideration in the evaluation of FUO.

Case Patient is a 10 month old male who presented with daily fevers>102 for two weeks. He developed a skin lesion on the right thigh 3 days following fever onset, but had no other symptoms. Parents denied any known insect, tick, or new animal exposures including rabbits. Was found to have leukocytosis with elevated CRP and ESR at an outside hospital. Initial workup including blood, urine, and wound cultures, and viral panel, were negative. On admission to our hospital, he remained febrile with elevated inflammatory markers (CRP 16, ESR 99). Skin lesion persisted and became a central punched out ulcer with surrounding erythema. Biopsy of lesion showed microabscesses but stains and cultures were negative. Patient also had right inguinal adenopathy. Lymph node biopsy revealed necrotizing granulomas and universal PCR was positive for Francisella tularensis. Tularemia titers were also positive at 1:2560. Parents later recalled their dog killing a rabbit in their yard 2 weeks prior to symptom onset. Patient was treated with IV gentamicin for 10 days before being discharged home, and has remained afebrile since.

Discussion Tularemia is a zoonotic infection caused by the aerobic gram-negative bacterium F. tularensis. In the US, primary reservoirs are rabbits and ticks, and infections are typically spread to humans via skin inoculation. The most common manifestation in children is ulceroglandular disease, characterised by skin ulcers, lymphadenopathy (LAD), and fever. Diagnosis can be confirmed with serum agglutination titers>1:160 or other testing such as ELISA or PCR. Treatment of choice is gentamicin, and treatment failure rates and mortality are quite low for patients receiving recommended therapy.

Conclusion Evaluation of FUO should be tailored based on history and physical exam findings. Tularemia is a rare cause of FUO, but should be considered in children with exposure to ticks or rabbits in addition to fever, skin ulcers, and LAD.

32 Scurvy, only a disease in pirates?

SE Mayberry

University of Alabama Birmingham, Birmingham, AL

Introduction Vitamin C deficiency is often thought to be obsolete, however, prevalence among 12–17 year olds in the United States was found to be 5%–6% based on the latest National Health and Nutrition Examination Survey. Vitamin C deficiency should still be considered in certain at risk populations.

Case-report A 9 year old male with a history of epilepsy, non-verbal global developmental delay and cerebral palsy presented to the hospital with a 3 week history of worsening petechial rash on all extremities, gum bleeding, intermittent fever, leg pain and swelling. On arrival he was febrile to 101, with fussiness, refusal to straighten his legs, bilateral lower extremity oedema, gingival bruising and bleeding, and extensive follicular petechiae on his extremities. His labs were remarkable for normocytic anaemia (haemoglobin 6.5), elevated CRP (4.9 mg/dL), ESR (45 mm/h) and x-rays and venous ultrasound of the lower extremities were unremarkable. An extensive lab work-up was unrevealing of any rheumatologic, infectious, or oncologic etiologies, including a normal skin biopsy to rule out a vasculitis. Vitamin C supplements were initiated due to concern for possible Vitamin C deficiency in the setting of developmental delay and poor oral intake. Within 24–48 hours, he showed remarkable improvement in the rash, gingivitis, and leg swelling. His vitamin C level was found to be extremely low (<0.1 mg/dl) supporting the diagnosis of scurvy. He was discharged home on vitamin C supplementation, 100 mg 3 times a day for 1 week then daily for 2 months.

Discussion Vitamin C deficiency leads to impaired collagen synthesis. Manifestations include irritability, low-grade fevers, gum bleeding, petechiae (particularly perifollicular), ecchymoses, corkscrew hairs, leg swelling, arthralgia, elevated inflammatory markers, normocytic anaemia, and coagulopathy. Scurvy is diagnosed clinically by dietary history and physical findings as well as rapid improvement in symptoms after starting vitamin C supplementation. If untreated, scurvy can be fatal from infection, cerebral haemorrhage or hemopericardium.

Conclusion Although rare in the modern era of fortified foods, scurvy and other nutritional deficiencies should still remain on the differential diagnosis in a child who is malnourished, developmentally delayed, has intestinal malabsorption syndromes, or other restrictive diets.

33 Tachycardia (in a child with vomiting and diarrhoea) unresponsive to crystalloid boluses in the paediatric emergency room

R Dababneh

E Fontane

University of Florida, Jacksonville, FL

Case report A 23 month old girl was brought to the paediatric emergency department (PED) by her grandmother for non-bloody, non-bilious vomiting and watery diarrhoea worsening over a 4 day period. In addition, the child had subjective fever and was noted to be less active than usual. She had an unremarkable birth and medical history. On exam she was irritable but consolable. She was ill-appearing without signs of respiratory distress. She was small for age, her skin appeared sallow, and she had fine scalp hair. Her rectal temperature was 98.7 F. Her heart rate was initially noted to be 134 beats per minute but upon re-evaluation was noted to be 150–160 beats per minute. Her blood pressure was 112/73. Her respiratory rate and SpO2 were normal. Her weight was at the 25th percentile. Her eye exam was notable for mild proptosis. Her precordium was hyper-dynamic and her heart exam revealed a grade 3/6 harsh systolic murmur. She had normal skin turgor and moist mucous membranes. Her lungs were clear. Her abdomen was soft and non-tender. Her bedside glucose was normal. Her treatment in the PED included two intravenous 20 mL/kg crystalloid boluses and intravenous ondansetron. Upon reassessment, her heart rate and blood pressure remained elevated. Her other vital signs remained unchanged as well.

Diagnostic tests in the PED included a 12 lead EKG that was notable for sinus tachycardia with a pericarditis pattern versus early repolarization. A chest radiograph showed a normal heart size and no lung abnormalities. Echocardiography showed hyper-dynamic systolic function, normal valves and chamber size, and no septal defects. Her chemistry panel were notable for a HCO3 of 12 mmol/L and anion gap of 30 mg/dL. T3 and Free T4 were elevated; 3.30 ng/dL and 5.80 ng/dL, respectively. TSH was markedly suppressed at <0.005 mIU/L. Exam and diagnostic findings were concerning for hyperthyroidism. She was admitted and the paediatric endocrine team was consulted. Her Thyroid stimulating immunoglobulins (TSI) were 299% of reference control confirming Graves’ disease as the cause of hyperthyroidism in this patient, and she was treated with atenolol and methimazole.

34 That’s below the belt: a curious case of infantile methemoglobinemia

P Redmond

MN Frascogna

B Dillard

University of Mississippi Medical Centre, Jackson, MS

Case report Cyanosis in children can present for a variety of reasons. When presenting outside the immediate newborn phase or when accompanied by dyspnea, respiratory compromise is the leading cause. However, if oxygen administration or airway protection does not improve symptoms, other causes should be considered. In this case we present a previously healthy infant with acute onset cyanosis.

A three week old male presented in respiratory distress as an flight transfer from a tertiary care facility. He had undergone a circumcision the day prior and had subsequently developed apneic episodes and colour change in the last 12 hours. Once in our unit, the patient was noted to be hypoxemic and lethargic. He had clear breath sounds but decreased respiratory drive. His heart rate and abdominal exam were normal. His skin was blue and mottled. He was placed on oxygen with no change in oxygen saturation levels. Basic labs were obtained, including an arterial blood gas with co-oximetry. Blood was visually noted to be dark brown in appearance on phlebotomy. Initial methemoglobin level was 26.7% confirming diagnosis of methemoglobinemia. The patient was intubated for airway protection due to persistent apnea and given 3.5 mg of methylene blue. Subsequent saturations returned to normal levels and repeat methemoglobin level was 3.3%.

The patient was admitted to the paediatric intensive care unit where he was weaned to extubation and recovered completely. Further history revealed that the patient’s mother had been using a prescribed lidocaine/prilocaine gel on the circumcision site. In consultation with toxicology services, further use of this medication was halted and no further symptoms persisted.

35 An unusual case of chickenpox in a newborn

AV Agwu

AZ Solaiman

MGovern Medical School at UT Health, Houston, TX

Case report A 17-day-old infant was admitted for five day history of worsening rash. Initially, a single pustule was noted on his right fourth digit. It was small and round without erythema. On admission, he had single pustules on erythematous bases on his head, right eyelid, hands, scrotum, back, and feet. They were in various states of healing with occasional crusting.

The baby was born term via repeat caesarean section to a mother with adequate prenatal care. She denied all STDs in this pregnancy, during delivery, or prior to conception. History significant for a maternal grandmother who lived with the infant having shingles 2.5 weeks prior to the infant’s admission. She had symptoms for 4 days before initiating and completing 4 days of therapy. Also, the infant’s older brother had a cold sore on his bottom lip noted 4 days before admission. Mother had chickenpox as a child. All other contacts in the house had been vaccinated against Varicella Zoster Virus (VZV). Review of systems was negative for fevers, cough, congestion, rhinorrhea, diarrhoea, emesis, decreased oral intake, jaundice, or other clinical signs of infection.

Complete blood count, comprehensive metabolic panel, and cerebrospinal fluid analysis were unremarkable. Acyclovir and Mupirocin were ordered due to concerns for Cutaneous Varicella vs Herpes Simplex Virus (HSV). Varicella and HSV 1/2 PCR on CSF, HSV eye swab, HSV genitorectal, and blood cultures were negative. Acyclovir was discontinued. HSV wound PCR eventually resulted negative. Varicella wound PCR resulted positive confirming the diagnosis of Cutaneous Varicella.

Discussion Cutaneous Varicella acquired postnatally is rarely seen in the neonatal period due to protection from maternal antibodies. Even if a newborn does not fit the age range of those typically infected with chickenpox, their exposure history and exam should guide clinicians to consider it in their differential diagnosis. The rash of cutaneous varicella obtained postnatally can mimic HSV, and the consequences of missing HSV can be severe. As such, a complete HSV workup should be completed if cutaneous varicella is suspected in a newborn. For term infants diagnosed with postnatally acquired cutaneous varicella, VZV Immunoglobulin is not indicated.

36 Can’t hear the diagnosis? just follow your nose

AS Marshall

University of Alabama at Birmingham, Birmingham, AL

Case report A 14-year-old male presented to the ED with a 60-pound weight loss over 1 year complaining of increased respiratory effort and secretions. Previously healthy, he is now tracheostomy dependent due to apparent iatrogenic subglottic stenosis, with vocal cord paralysis, progressive hearing loss, and recurrent joint swelling also noted. He is wheelchair bound due to pain and weakness. Exam revealed a mute cachectic male with poor dentition, right knee swelling, hearing loss, saddle nose deformity, and cauliflower ear. Initial work-up was unremarkable on imaging, with normal electrolytes, kidney, and liver function. ESR/CRP were 110 and CRP 16.9. Depressed protein, albumin, and pre-albumin levels indicated poor nutrition. Patient was admitted for respiratory support and further work-up.

Right knee MRI imaging showed soft tissue inflammation consistent with synovial fluid analysis. ANA and ANCA were not detected. ACE levels were normal. Tissue biopsies of the lung and gastrointestinal tract biopsies/brushings revealed no fungal/malignant disease. Hearing loss was sensorineural in nature. Cartilage biopsies of the nose/ear revealed chronic inflammatory changes, and with the patient’s clinical picture were consistent with Relapsing Polychondritis as a diagnosis. Collagen Type II antibody levels were elevated at 51.7 U/mL. Patient received solumedrol during admission, and Cyclophosphamide and rituximab were started with a significant clinical response after discharge.

Relapsing Polychondritis is an autoimmune disease affecting cartilage types 2, 9, and 11. Exact aetiology is unknown. Most commonly, it affects whites, both genders equally, and presents typically at 40–60 years of age. Symptoms often include inflammation of the ears, nose, and eyes manifesting as cartilage deformities, audiovisual deficits, tinnitus, vertigo, and hypogeusia. Other symptoms such as arthralgias and joint swelling, and severe tracheobronchomalacia presenting as hoarseness, aphonia, wheezing, stridor, and obstructive apnea can also occur. Genetic predisposition may exist, but there is no evidence of familial inheritance.

Misdiagnosis of chronic rheumatologic diseases in children can lead to unnecessary work up, residual sequelae, and significantly reducing quality and potential quantity of life.

37 A common denominator: enterovirus-induced myocarditis and acute flaccid paralysis

MD Lisenby

V Godsey

N Tofil

University of Alabama School of Medicine, Birmingham, AL

Case report MC is a previously healthy 22-month-old male who presented to the emergency department with fever, vomiting and lethargy for one day. On arrival, he had mild respiratory distress. Chest x-ray showed bilateral opacities, concerning for pneumonia. Despite escalating oxygen therapy, he became increasingly hypoxic, requiring intubation and admission to the paediatric intensive care unit. An echocardiogram showed dilated ventricles and mitral regurgitation concerning for myocarditis. He developed recurrent episodes of non-sustained ventricular tachycardia requiring infusions of lidocaine and calcium and required multiple vasopressors to maintain adequate perfusion. On hospital day 4, his cardiac function improved enough to allow him to be weaned off vasopressors. However, despite decreased sedation he continued to have altered mental status and was unable to be weaned from the ventilator. Initial neurologic work-up with head computed tomography, magnetic resonance imaging and long-term electroencephalogram was unremarkable. Spinal fluid was notable for slight protein elevation (49) and 12 white blood cells. Despite treatment aimed at sedation withdrawal and possible delirium, he failed to improve and his exam was notable for generalised severe weakness and horizontal nystagmus. Repeat imaging showed a large area of demyelination in the cervical region and lower brainstem consistent with acute flaccid paralysis. Further testing was positive for Enterovirus non D-68. Respiratory muscle weakness persisted, requiring tracheostomy and ventilator for respiratory support.

Discussion This case represents a challenging neurologic work-up in the setting of myocarditis management. Enteroviruses are a known cause of myocarditis and acute flaccid paralysis but rarely involve cardiovascular and neurologic systems concurrently. Central nervous system involvement of enterovirus is often devastating and although polio virus has been largely eradicated, other enteroviruses may have similar impact. No antiviral treatments have been developed and evidence for intravenous immunoglobulin is somewhat controversial. Long-term outcomes for enterovirus-induced neurologic impairment are variable, but unfortunately many studies indicate ongoing symptoms at one year follow-up in most patients.

38 A case of puzzling paralysis: acute flaccid myelitis

BL Clampitt

D Hahn

University of Oklahoma, Edmond, OK

Case report Acute onset paralysis in children can represent a range of diagnoses and treatment possibilities. A more recently recognised entity, acute flaccid myelitis (AFM), should be considered in differential.

A previously healthy 12 year old girl presented with 2 days of new onset left upper extremity and facial weakness after 9 days of intermittent fevers, cough, malaise, nausea/vomiting, and headache. Exam noted significant left triceps, biceps, and deltoid weakness, dysarthria and left facial droop. Lumbar puncture was normal except for pleocytosis. She was initially treated for presumed infection with Ceftriaxone, Vancomycin, and Acyclovir, however bacterial and viral cultures and studies were negative. MRI of the brain showed leptomeningeal enhancement in the posterior fossa, with subtle T2 hyperintensity of the pons. Cervical spine MRI showed extensive T2 signal abnormality in central grey matter, most notably the anterior horns. Anterior horn disease has been described in Enterovirus infections, and a diagnosis of AFM was made given her presentation with corroborating imaging findings and prodromal viral illness, although enterovirus was not isolated. She received a 5 day course of intravenous immunoglobulin. Fluoxetine was also started, as there are reports of improvement of Enterovirus-related AFM with its use; it was later discontinued due to side effects. She did note some improvement of facial droop and triceps weakness, although had persistent severe biceps/deltoid weakness and dysarthria; she was discharged with speech, occupational, and physical therapy follow up. At recent clinic follow up 1 year post onset, she shows continued improvement.

Although AFM is very rare, it should be included in the differential for paediatric patients presenting with acute onset paralysis, even without identified Enterovirus infection. While there are limited case reports of various therapies, there is no formally recognised successful treatment for AFM. Appropriate diagnosis in this setting allows for exploration of treatment options, early services for physical and occupational therapies, and realistic expectations of potential outcomes for patients and families, as well as the opportunity to obtain valuable data and expand the literature that assists in the continued effort for surveillance and investigation of this condition.

Case reports in cardiovascular medicine, 2:00 PM, Thursday, February 22, 2018

39 Epi-pericarditis as a manifestation of acute myeloid leukaemia

D Sahni1

ML Hess2

S Vijayanagar1

1VCU School of Medicine, Richmond, VA

2Virginia Commonwealth University, Richmond, VA

Case report A 30 year old male with no significant past medical history presented with tachycardia, anaemia, thrombocytopenia and blasts consistent with acute myeloid leukaemia (AML). The patient was asymptomatic from a cardiac standpoint. Vital signs were remarkable for a temperature of 38.6°C. Chemistries were significant for a troponin of 2.5 ng/ml, normal creatinine kinase and elevated brain natriuretic peptide of 426 pg/ml. ECG findings showed normal sinus rhythm with incomplete bundle branch block and lateral diffuse ST-T wave elevations suggestive of evolving myo-pericarditis. Echocardiography revealed no pericardial effusion and an ejection fraction of 55%. The patient had a pericardial friction rub at the right lower sternal border. Cardiac MRI was recommended, but due to tumour lysis syndrome an endomyocardial biopsy was performed. The biopsy was negative for leukemic infiltration. A right heart catherization was also performed and intracardiac pressures were normal. Following a short interim, the pericardial rub subsided. The troponin level remained elevated. A cardiac MRI was obtained before discharge and revealed mild left ventricular hypertrophy, enhanced pericardium, and inferior basilar enhancement. The patient was confirmed to have epi-pericarditis secondary to AML.

Discussion Acute myeloid leukaemia (AML) is a cancer with predisposition for infiltration of the blood, bone marrow and mucosal sites. Extramedullary AML (EML-AML) is a known manifestation of the disease. Currently, less than 1% of EML-AML is associated with invasion of the myocardium. The known complications of EML-AML include the formation of a myeloid sarcoma (chloroma), sudden cardiac death, restrictive cardiomyopathy, heart failure and pericardial effusion. A cardiac chloroma consists of myocardial infiltration by leukemic cells and was ruled out with biopsy and cardiac MRI. At a 6 month interval, following intensive chemotherapy, the epi-pericarditis resolved. We have identified epi-pericarditis as a new complication of EML-AML.

Abstract 39 Figure 1

Epi-pericarditis as a new complication of EML-AML

40 Straight to the heart: unusual site of metastatic disease

PP Kyaw1

S Ahmed1

R Smalligan2

T Vo1

1Texas Tech Univ HSC Amarillo, Amarillo, TX

2UAB-Huntsville, Huntsville, AL

Case report A 54 yo African American woman with HTN and hypothyroidism presented with progressive dizziness and fatigue for 2 months along with persistent cough with blood streaks. Meds: HTCZ, levothyroxine; FH neg for cancer; SocHx-few cigarettes per day, no alcohol, no drugs. PE: T 36.7, SpO2 98% on RA, HR 113, RR 15, alert and oriented, no JVD, chest-clear, heart- RRR with no murmurs. Labs: CBC and CMP, TSH, T4 WNL. CT chest- right lower lobe pneumonia with mediastinal lymphadenopathy, cardiomegaly, small pericardial effusion. EKG – sinus tachycardia. Hospital course: patient was treated for community acquired pneumonia initially. She had recurrent episodes of atrial flutter with RVR; TTE showed a 3.3×2.9 cm mass in left atrium suspicious for myxoma. She underwent excision of the tumour and path showed undifferentiated squamous cell carcinoma (SCC). Endobronchial ultrasound showed small masses in the trachea and the right main stem bronchus. Primary site was confirmed by CT guided biopsy of the right infrahilar mass which showed undifferentiated SCC. She was discharged after clinically justable. In consultation with oncology as an outpatient, multiple metastases were found, including brain mets and patient chose palliative care.

Discussion Cardiac tumours are uncommon, whether benign or malignant, and data on the best management and outcome of these tumours is limited. Frequency of secondary cardiac tumours of any type, as in our case, is estimated to occur in up to 1.2% of cancer pateints although it is exceedingly rare to diagnose these during life. Lung cancer accounts for 36% of cancers known to metastazie to the heart. Metastatic pathways include direct extension, via the bloodstream, lymphatic system or by intracavitary diffusion from the IVC or pulmonary veins. Pericardial disease is most common (64%), myocardial and epicardial (35% combined). Endocardial mets, as in our case are rare. Histologically, adenocarcinoma accounts for 26% of these mets, SCC 23%, undifferentiated 21%, and bronchoalveolar 17%. We suspect our patient‘s left atrial mass originated hematogenously or via a small atrial septal defect that was found at the time of surgery. Although the tumour was successfully removed, the extent of the patient‘s disease dictated her ultimate demise.

41 Myasthenia gravis masquerading as a non-st-elevation myocardial infarct (nstemi)

A Richardson1

P Staiano1

CM Baldeo1

C Harris1

E Benitez2

F Franchi2

1University of Florida Jacksonville, Jacksonville, FL

2University of Florida College of Medicine Jacksonville, Jacksonville, FL

Case report A 57 year old male with a past medical history of Hypertension, Hyperlipidemia, and Myasthenia Gravis presented to the emergency department for severe shortness of breath which occurred while climbing stairs. Denied chest pain, diaphoresis or previous history of coronary disease. Vitals upon presentation revealed blood pressure of 115/85 mmHg, heart rate of 86 beats per minute, oxygen saturation of 96% on room air, and respiratory rate of 18. Electrocardiogram revealed normal sinus rhythm with no ischaemic changes. Initial troponin T was noted to be 0.04 ng/ml, with subsequent troponin T two hours later at 0.233 ng/ml. Creatine kinase-MB was 7.5 ng/ml, total creatine kinase 561 ng/ml, and pro-BNP <5 pg/ml. He was placed on a heparin infusion and admitted to the Coronary Care Unit for NSTEMI. Left heart catheterization revealed normal coronary arteries. Echocardiogram showed no abnormalities, left ventricular ejection fraction of 55%–60%, normal diastolic function. Troponin T returned to normal limits before discharge.

Troponin T is part of a regulatory protein complex located in striated muscle. It is a sensitive and specific biomarker of suspected cardiac injury. The most common cause for elevated troponin T is myocardial ischemia. Rarely it can be elevated in patients with neuromuscular disease. Although cardiac disease can occur as a result of neuromuscular disease, up to 15% of Myasthenia gravis patients, it is unusual to see elevation of troponin T in the setting of no detectable disease.

Elevated troponin T in patients with neuromuscular disease is troublesome as it can lead to misrecognition of acute coronary syndromes. Further complicating diagnosis is overlapping of symptoms such as dyspnea and fatigue. Theories for elevated troponin T in neuromuscular disease range from upregulation of embryonic myogenic pathways for repair of damaged muscle to undetectable cardiac damage. Although physicians should retain a high clinical suspicion for acute coronary syndromes, elevated troponin T must be interpreted within the entire clinical context.

42 Cannabinoid-induced symptomatic second degree av block

M Heckle

SB Latham

KT Weber

S Haji

University of Tennessee Health Science Centre, Memphis, TN

Case report Cannabinoids are the bioactive components of the marijuana plant. Over the past decade and even more so recently, medicinal and recreational uses of marijuana have progressively risen. While considered a relatively safe drug, significant cardiovascular side effects can accompany its use, which we have previously reported in the American Journal of the Medical Sciences. We present a patient with syncope from second degree AV block due to chronic daily inhaled marijuana use.

A 30-year-old male with a history of a single chamber atrial pacemaker due to symptomatic bradycardia was sent to the emergency room (ER) after having a syncopal event while conducting a grocery store robbery. On initial evaluation he was bradycardic with an average heart rate of 40 beats per minute. His electrocardiogram revealed an atrial-paced rhythm with 4:3 s degree Mobitz type II AV block. He was admitted to the cardiology service and by the second day there was no evidence of AV block. On further questioning the patient revealed he is a chronic daily smoker of marijuana. It was felt that his marijuana use was the likely culprit of his transient second degree AV block leading to his syncopal event. Due to having symptomatic second degree AV block and his addiction to marijuana his pacemaker was upgraded to a dual chamber pacemaker.

Chronic inhalation of cannabinoids can be associated with bradyarrhythmias. Although bradycardia is considered a rare side effect of marijuana, it is likely an under recognised complication. With the recent legalisation of marijuana in 29 states, physicians should be made aware of its potential side effects especially in daily users. Recognition of these cardiovascular effects can help providers avoid expensive and unnecessary testing with potential serious side effects leading to life threatening situations.

43 A peculiar aortic valve vegetation

X Solis

SK Prieto

B Rosales

TTUHSC, Lubbock, TX

Case report Our patient was a 61 year old male with a PMH of COPD, who presented to our ER after being found wandering the streets with confusion. He did not recognise his daughter who was at bedside. He was disoriented but was complaining of extreme generalised muscular pain and weakness. Vital signs were notable for a MAP of 54, T 100.6 F. He was noted to have a grade II systolic murmur over the right upper sternal border with minimal radiation to the carotids, it was unknown how long this murmur had been present as the patient did not get regular medical care. He also had extreme tenderness to palpation over all quadrants of the abdomen and large muscle groups. Pertinent labs were WBC 15.42, platelets 28, lactate 2.04, CK 698. Urine drug screen did test positive for amphetamines and cannabinoids. Chest X-ray was notable for bibasilar oedema. CT abdomen/pelvis was notable for bibasilar pulmonary oedema. CT head was negative for acute abnormalities. He was started on pressor support as well as vancomycin and piperacillin-tazobactam.

Blood cultures from admission were positive for Staphylococcus aureus, MSSA. Antibiotic therapy was then changed to nafcillin and rifampin. Transthoracic echocardiogram was notable for a large aortic valve vegetation measuring up 1.6×2.7×2.2 cm, highly mobile, extending into in the left atrium. Moderate to severe aortic regurgitation was also noted AVA 1.6 cm, and transvalvular gradient measuring 6.3 mmHg. LVEF was estimated at 55%–59%. LHC was negative for any epicardial CAD.

He underwent bioprosthetic AVR by cardiothoracic surgery. Small vegetations were seen on the mitral valve leaflets, which were surgically resected. He did develop post-operative pleural effusions and was progressing well until he suddenly developed respiratory distress and metabolic acidosis. Repeat TTE was notable for pericardial effusion and tamponade, so he was taken to the operating room once again for chest exploration and pericardial drain placement. Aortic regurgitation did improve was now moderate, with LVEF maintained. The patient then had increased ventilator requirements, aneuric renal failure requiring CRRT, as well as hematochezia and family decided to withdraw care and forego any aggressive treatment.

44 Diabetic ketoacidosis-induced hypothermia causing cardiomyopathy

D Gill

M Hess

SUNY Upstate Medical University, Syracuse, NY

Case report Diabetic ketoacidosis (DKA) is often triggered from underlying stressors, and can rarely lead to hypothermia. The proposed mechanism of hypothermia is secondary to the inability of glucose molecules to be transported intracellularly because of lack of insulin, hence leading to lack of substrate for cellular heat production. Hypothermia serves as a stressor, which can cause catecholamine surge. Catecholamine surge is the underlying mechanism for stress induced cardiomyopathy.

25-year-old male with past medical history of diabetes mellitus type 1, presented with 3 days of nausea, vomiting and confusion. In the emergency department he was found to be hypothermic with temperature of 31.3°C, with blood pressure of 137/94 mm Hg, and heart rate of 108 beats per minute. On physical exam he appeared to be lethargic, had a dry oral mucosa and poor skin turgor. He had a regular rate and rhythm and did not have any jugular vein distension, or inspiratory crackles. His labs showed pH of 6.77, and bicarbonate of 5 mmol/L, anion gap of 29 mmol/L, betahydroxy butyrate of 13.3 mmol/L and glucose of 1396 mg/dL. His electrocardiogram showed sinus bradycardia and Osborn waves. Echocardiogram on day 1 showed left ventricular ejection fraction of 30% along with diffuse hypokinesis of all walls. Patient was admitted to the intensive care unit and started on insulin drip and aggressively hydrated for DKA, along with rewarming. Repeat echocardiogram on day 4 had shown ejection fraction of 40%. Cardiac enzymes remained benign throughout the hospital stay. Patient did not have any prior cardiac history and he was not drinking, smoking or abusing illicit drugs. He also denied autoimmune disease or radiation therapy. Hence patient was diagnosed by the cardiology service with stress-induced cardiomyopathy, a Takotsubo variant the most likely aetiology. He was started on beta blocker and an ACE inhibitor, with full recovery of his ejection fraction and wall motion abnormalities during follow up visit at 3 months.

This case is an illustration of a rare case of DKA induced hypothermia, which then caused stress induced cardiomyopathy. Electrocardiogram findings of Osborn waves were crucial to the diagnosis of hypothermia. Physicians should keep in mind that diabetic patients can present with new onset cardiomyopathy from DKA and hypothermia.

45 Septic diffuse left ventricular calcification

B Lennep

RC Long

ME Hall

CS Guild

University of Mississippi Medical Centre, Madison, MS

Case report A 54-year-old man with no cardiovascular history aside from hypertension presented with complaint of abdominal pain and shortness of breath. Computed tomography (CT) imaging revealed right-sided pneumonia with empyema. He rapidly deteriorated into septic shock requiring intubation and 3 vasopressors. He was treated with antibiotics and drainage of the empyema, with gradual recovery over 4 weeks. During this time, he had several chest CT scans to evaluate the pleural space. At discharge, he was referred to cardiology for left ventricular (LV) ejection fraction of 20% by echocardiogram. Review of the serial CT scans revealed development of diffuse calcification of the LV myocardium over a period of 6 days following his initial CT (figure A). Coronary angiography demonstrated only nonobstructive coronary disease. Cardiac magnetic resonance imaging demonstrated diffuse subepicardial late gadolinium enhancement (figure B). Three years later, the calcification and diminished ejection fraction persist.

Diffuse calcification of the LV is a rare complication of severe sepsis, with less than 10 published case reports. The mechanism has not been clearly established but is thought to represent dystrophic (as opposed to metastatic) calcium deposition. This phenomenon differs from the calcification seen post-infarct in that it is diffuse and from that seen following myocarditis in that it spares the right ventricle. Severe LV dysfunction is common and short-term mortality is very high.

Abstract 45 Figure 1

Computed tomography image (A) demonstrating diffuse epicardial calcification of the left ventricle. Short axis cardiac magnetic resonance image (B) demonstrating diffuse subepicardial late gadolinium enhancement

46 Right ventricular failure with pulmonary hypertension and progressive respiratory failure after ruptured breast implants

M Shahreyar

S Koshy

KT Weber

N Garg

UTHSC Memphis, Collierville, TN

Introduction Several reports have described an association between free silicone injections with multisystemic complications, including acute and chronic pneumonitis, silicone pulmonary embolism and connective tissue disease. Right ventricular failure and pulmonary hypertension with progressive respiratory failure after ruptured breast implants has rarely been reported.

Case report A previously healthy 42-year-old transsexual female presented with progressive shortness of breath over a 3 months period. For cosmetic augmentation, 10 years prior, she received silicone breast implants and free silicone injections to the buttocks and calves. Prior to the onset of her pulmonary symptoms, her silicone breast implants ruptured following motor vehicle accident.

At initial evaluation diffuse crackles more noticeable in bilateral upper lung fields were found. Chest radiograph showed opacities in upper lobes bilaterally, computed tomography demonstrated severe pulmonary fibrosis predominantly in upper lobe peripheral distribution. Severely dilated right ventricle with reduced systolic function and a normal left ventricular size and function were found on echocardiogram. On right heart catheterization, mixed venous saturation was 47% mean pulmonary artery (PA) pressure 47 mmhg, capillary wedge pressure 11 mmhg, pulmonary vascular resistance (PVR) 11 wood units and cardiac index 1.83 L/min/M2. Vasoreactive testing with inhaled nitrous oxide was negative. Right upper lobe transbronchial biopsy revealed interstitial multinucleated giant cells and focal small multicystic area with minimal fibrosis suggestive of silicone-induced pneumonitis.

Conclusion Silicone implants and free silicon injections have been increasing worldwide with 2/3 breast implants rupture over 11 years. This case highlights a devastating cardio-pulmonary complication such cosmetic surgery and should raise patient and physicians awareness of these life-threatening complications.

47 Casting light on plastic bronchitis: an inside look at complications following fontan palliation

EJ Schwartzenburg1

RE Herdes1

SS Maqsood2

D Edell2

A Gutierrez2

C Lilje2

1Louisiana State University Health Sciences Centre, New Orleans, LA

2LSUHSC, New Orleans, LA

Case report Plastic bronchitis (PB) is a rare complication of single-ventricle patients following Fontan palliation. Patients expectorate impressively intact ‘tree-like’ casts, which can vary in size and can cause filling defects in the lung. This can present as acute respiratory failure or hypoxia, and can lead to asphyxiation and death. Pathophysiology of PB remains unknown. It is believed to be associated with central venous congestion and subsequent lymphatic abnormalities. There are no clinical trials that demonstrate superior efficacy of any single treatment for PB beyond optimising cardiac hemodynamics. Case studies have suggested aerosolized mucolytics, steroids, bronchoalveolar lavage, bronchoscopic extraction, pulmonary vasodilators, and nebulized fibrinolytics.

We report the case of a 16 yo female with history of Tricuspid Atresia with a VSD following Fontan palliation who presented tfor recurrent tussive episodes of white casts. Oxygen saturation at time of presentation was 90% on room air. Cardiac imaging and catheterization studies revealed reasonable cardiac function. Mean pressure in the fontan system was 14 mmHg. A fenestration had closed spontaneously years ago. There was no evidence of intracardiac thrombosis, Fontan stenosis, or airway filling defects. CBC, CMP, and coagulation studies were within normal limits. Patient was treated with Azithromycin, low fat diet, and nebulized heparin. The PB exacerbation resolved and patient was sent home on day 4..

This case represents a unique complication of paediatric cardiac patients with single ventricles after definite Fontan palliation. PB should be considered in all such patients who present with expectorated casts. These casts is usually an ominous prognostic sign. The underlying cardiac status needs to be carefully investigated and optimised if possible. Symptomatic treatment options to resolve the casts are evolving, as there are very few cases documented. The successful resolution of symptoms in our patient suggests support for the use of nebulized heparin in this population.

48 Resistant prinzmetal angina due to pheochromocytoma

R Subedi

R Dean

D Villarreal

SUNY Upstate Medical University, Syracuse, NY

Case report Prinzmetal angina is caused by the spasm of the coronary arteries. Pheochomocytoma can cause release of catecholamines and vasospasm of coronary arteries precipitating prinzmetal angina in susceptible patients. This is a case of a difficult to control prinzmetal angina from underlying metastatic pheochromocytoma.

A 36 year old male presented with intermittent chest pain for 3 days and an episode of loss of consciousness. His chest pain occurred even at rest, was worse at night and relieved by nitroglycerine. Physical exam, vital signs and electrocardiogram (EKG) were normal. In the hospital, he had further episodes of chest pain. He had a heart rate of 34 beats/min and a BP of 70/46 mmHg. An EKG showed ST elevations in the inferior leads with 3rd degree atrioventricular (AV) block. Telemetry monitoring showed intermittent 3rd degree AV block. He had presented to an outside hospital with similar complaints and findings. A cardiac catheterization performed at that time revealed normal coronaries. He was started on isosorbide mononitrate and ranolazine but he continued to have nocturnal chest pain with ST elevations and 3rd degree AV block. Diltiazem and cilostazol were added. A nitroglycerin patch was applied at night. Aspirin was switched to clopidogrel and atorvastatin was started to stabilise the endothelium. His symptoms improved with this regimen. Because of his very difficult to control vasospastic angina, plasma nor-metanephrines and 24 hour urine nor-metanephrine were sent which were elevated at 3694 pg/ml and 14876 ug/24 hour respectively. Further workup revealed metastatic pheochromocytoma.

Prinzmetal angina can cause arrhythmias, including high grade AV block and sudden cardiac death. It usually occurs at rest, mainly between midnight and early morning because of the natural circadian variation of hormones. Calcium channel blockers and nitrates which promote coronary vasodilatation are commonly used. Cilostazol, a vasodilator, can be added to control the symptoms. Because endothelial dysfunction and oxidative stress are involved in the pathogenesis, statins and antiplatelets can be helpful to stabilise the endothelium. In refractory cases, endogenous production of cathecholamines by pheochromocytoma should be considered, as in our patient.

Moving your clinical case presentation into a published manuscript, 2:45 PM, Thursday, February 22, 2018

49 Adenocarcinoma of the appendix presenting as recurrent abscess formation

I Serrano

J Ruiz

A McCarthy

University of Florida, Jacksonville, FL

Case report 24-year-old African American male with a medical history of appendiceal abscess and perforation presented to the hospital with complaints of abdominal pain and an open draining wound with purulent foul-smelling fluid. His abdominal symptoms initially started eight months prior, when he was found to have an appendix rupture with a superimposed abscess at an outside institution. At that instance, he was managed with intravenous antibiotics and a percutaneous Jackson-Pratt drain was placed. In the subsequent months, the patient had 3 admissions with similar presentations requiring percutaneous catheter to drain the abscess recollection and antibiotics. On physical exam, there was evidence of purulent discharge from an open abdominal wound in the right lower quadrant concerning for recurrence of abscess with possible formation of an enterocutaneous fistula. A computed tomography (CT)-scan of the abdomen and pelvis was done with evidence of a thick-walled, multilobulated collection in the anterior lower abdomen/pelvis, which passed through the right rectus abdominis muscle communicating with the skin surface. He was started on antibiotic coverage and a drainage catheter was placed. CT-guided core biopsy of the abdominal mass confirmed the diagnosis of adenocarcinoma with mucinous features. A PET-scan revealed bilateral metastasis to inguinal and iliac lymph nodes. He was scheduled to start neoadjuvant chemotherapy with Folinic acid, Fluorouracil and Oxaliplatin (FOLFOX) with plans of reassessment for surgical resection.

Discussion Appendix cancer is an extremely rare gastrointestinal malignancy found in approximately 1 percent of appendectomy specimens and accounting for less than 0.5 percent of all tumours of gastrointestinal origin. Mucinous adenocarcinomas are one of the histologic types causing glandular invasion and mucus secretion. It commonly presents as acute appendicitis but on rare occasions, can develop an abscess and perforation of the appendix with spreading of cancerous cells into the peritoneum. We report an unusual case of recurrent intra-abdominal abscess formation masquerading a diagnosis of appendiceal mucinous adenocarcinoma. Mucinous adenocarcinomas like the one presented, can cause build-up of mucin leading to appendiceal rupture and abscesses.

50 A rare presentation of achalasia in a teenager

NS Jones

W Thomas

J Bocchini

LSUHSC Shreveport, Shreveport, LA

Case report Achalasia is a rare childhood disease. Due to its association with nonspecific symptoms, its diagnosis is often delayed. Delayed diagnoses often lead to delayed treatment. The most common symptoms in the paediatric population are as follows: weight loss, dysphagia, and regurgitation. Children presenting to their primary care physician with complaints of the aforementioned symptoms are often diagnosed with Gastroesophageal Reflux, Failure to Thrive, or other nonspecific eating disorders. When patients complain of progressive dysphagia accompanied by increasing frequency in regurgitation, it is imperative to work up the patient for this diagnosis. A 17 year old male with no pertinent past medical history presents with complaints of worsening epigastric burning pain occurring in the late evening. The patient states that his symptoms have been present for about one year. Approximately nine months prior to presentation, he began having difficulty swallowing solids such as steak, but was able to swallow chicken, bread, and liquids. Seven months prior to presentation, his dysphagia worsened, making it harder to swallow softer textured foods. The patient developed manoeuvres to aid himself with swallowing; however, over time these manoeuvres failed resulting in further progression of his dysphagia to include liquids as well. Over a two month time period, the patient lost 30 pounds. His initial physical examination was benign except for minimal epigastric tenderness to palpation. An upper gastrointestinal series demonstrated incomplete spontaneous contrast flow through the lower esophageal sphincter with significant fluid buildup in the proximal oesophagus. The study also demonstrated esophageal dilation proximal to the lower esophageal sphincter. Paediatric gastroenterology was consulted and an extensive work up was performed including esophageal manometry and esophagogastroduodenoscopy. These diagnostic tests confirmed the diagnosis of achalasia. Paediatric surgery was consulted, and after discussing with the family the therapeutic interventions available, the decision to perform a Heller myotomy with partial fundoplication was made. The patient tolerated the procedure well and his diet was advanced without complication. At time of discharge, he was able to tolerate a regular diet without any pain or discomfort.

Adolescent medicine and paediatrics, Joint plenary poster session and reception, 4:30 PM, Thursday, February 22, 2018

51 Gaps in atv injury reporting in alabama: a case for statewide trauma databases

A Burks

K Jeffries

A Klasner

M Nichols

K Monroe

University of Alabama, Birmingham, AL

Purpose of study In 1998 Alabama passed legislation requiring hospitals to submit data regarding head and/or spinal cord injuries; collection began in 1999. That same year this was extended to include all traumatic injuries but only head and spinal cord reporting was mandated. In 2007–2008 the Alabama Trauma System (ATS) was established, data is entered by EMS in the field or from hospital entry; this is optional reporting. ATS and head/spinal injuries are recorded in same database. Currently there is no state plan to expand or mandate recording of paediatric trauma injuries in Alabama. The purpose of this study was to compare data from multiple reporting agencies within the state of Alabama, including chart review from the state’s paediatric trauma centre, regarding ATV injuries to determine if accurate reporting is being achieved.

Methods used Data from 4 sources in the state of Alabama was reviewed from 01/01/16 to 12/31/16 for patients aged 0–18 years old who sustained an injury from an ATV related accident. Data was compared between sources to establish if accurate reporting of ATV injuries is being achieved. Keywords and diagnosis codes were searched through the National Electronic Injury Surveillance System (NEISS), Children’s of Alabama retrospective chart review, Alabama Trauma System (ATS), and Alabama pre-hospital EMS records.

Summary of results National Electronic Injury Surveillance System reported 30 injuries, 73% male, 27% female, median 12.5 years (mean 11.7). Children’s of Alabama chart review revealed 104 injuries, 60% male, 40% female and one unknown gender, median 12, (mean 11.5). Alabama EMS recorded 25 injuries 56% male, 44% female, median age 12 (mean 12.2). Alabama Trauma System reported 5 injuries, 80% male, 20% female, median age 14 (mean age 11).

Conclusions Injuries are the leading cause of death in paediatric patients. The current reporting of ATV injuries in Alabama demonstrates large gaps in accuracy. Without a uniform reporting system it is impossible to track the number of ATV accidents, or other traumatic injuries, occurring in the state. Prevention of injuries is key to decreasing paediatric mortality and a statewide trauma database would allow development of targeted prevention programs, education and outcome monitoring.

52 Hpv vaccine is recommended less often in the south

A Caldwell

P Darden

The University of Oklahoma, Edmond, OK

Purpose of study HPV vaccine receipt for males and females remains low. Provider recommendation in favour of the HPV vaccine is associated with increased vaccination. Regional variations in practice, including vaccine recommendations, may explain variations in vaccine uptake.

Methods used Data were from the 2008–2013 National Immunisation Survey-Teen. Provider recommendation was from parent report and vaccination status by provider report. All analyses accounted for the complex sampling design. Up to date (UTD) was defined as receipt of 3 doses of HPV vaccine.

Summary of results UTD did not differ among males in the South compared to the US. UTD increased for females in the South from 13.9% in 2008 to 35% in 2013, however, this was lower than US females who increased from 17.9% to 37.6%. Provider recommendation of HPV vaccine was also lower among females in the South compared to the US, increasing from 44.6% to 66% in the South and from 49.2% to 69.5% in the US. Among males, recommendation did not differ except in 2013. HPV vaccine was recommended 39.4% in the South and 45.7% in the US for males in 2013. The attached figure displays percent UTD by provider recommendation and sex among those who live in the South.

Conclusions Females in the South receive a provider recommendation and the HPV vaccine less often than females in the US as a whole. Male vaccine receipt did not differ in the South compared to the US and neither did recommendation except for 2013. There are regional variations in both HPV vaccine receipt and frequency of provider recommendation. Recommendation is key for HPV vaccine receipt for both sexes in the South. Improving frequency of provider recommendation for HPV vaccine could improve UTD for HPV in the South.

Abstract 52 Figure 1

UTD by provider recommendation and sex in the south

53 Trends in suicide attempt admissions 2012-present at the children’s hospital at ou medical centre

M Cooper

S Gillaspy

R Wallace

S DeLeon

Oklahoma University Health Science Centre, Oklahoma City, OK

Purpose of study The purpose of this study is to evaluate secular trends and seasonal variations in admissions for suicide attempts at the Children’s Hospital at OU Medical Centre over the last five years.

Methods used Charts of children and adolescents aged 4–18 years of age admitted to The Children’s Hospital at OU Medical Centre with the diagnosis of suicide attempt, self-harm, or intentional ingestion from January of 2012 to August 2017 were identified using ICD-9 and ICD-10 codes from the hospital electronic medical record. Records were individually examined to evaluate for appropriateness for inclusion and admissions were grouped by month. Linear regression was used to identify the overall trend in the data, and we plan to sue Auto Regressive Integrated Moving Average (ARIMA) analysis to analyse separately for seasonal variation and trend.

Summary of results Linear regression detected a significant increase in monthly suicide attempt admissions between January 2012 and August 2017. There was an average overall increase of 0.21 admissions per month over the entire time period (p<0.0001). See figure for actual admissions per month over the 68 month period and the predicted regression line. ARIMA modelling is underway to examine for seasonal variations and recent changes in trend.

Conclusions There has been a significant increase in suicide admissions at The Children’s Hospital at OU Medical Centre since January of 2012. Full analysis to define seasonal variation and changes in trend will be completed prior to meeting.

Abstract 53 Figure 1

Suicide admissinons by month

54 Assessment of a paediatric clinical asthma score’s ablity to predict disposition

L East1

K Kepple2

R Gibson1

N Lane1

1Augusta University, Augusta, GA

2Orlando Health, Orlando, FL

Purpose of study This research is part of a feasibility study for a paediatric emergency department observation unit. This study examined the ability of a clinical asthma score (CAS) to predict disposition of paediatric patients treated in a paediatric emergency department (ED). The CAS is used to assess asthma severity and is based on respiratory rate, lung sounds, retractions, and oxygen saturation.

Methods used This study was a retrospective chart review of patients ages 2–17 treated at a paediatric ED with a diagnosis of asthma between July 1, 2012 and June 30, 2013. CAS scores were obtained for patients twice: upon presentation to the ED and at the time of disposition. The CAS is rated on an evenly distributed scale of 0–12. Student’s t-tests were performed to evaluate the relationships between and CAS and age, gender, or race. Linear regression was used to analyse the relationship between patients’ CAS and length of stay. Student’s t-tests were conducted to analyse the association between patients’ CAS and disposition.

Summary of results A total of 608 visits from 499 patient charts were analysed. The CAS was not significantly impacted by age, gender, or race (p>0.05). A higher CAS, regardless of whether it was evaluated at presentation or disposition, correlated to a longer length of stay (p<0.0001). A patient’s disposition also correlated to their CAS. Patients that were discharged had the lowest CAS scores on average (Presentation CAS=4.1, CI: 3.8 to 4.3; Disposition CAS=1.4, CI: 1.2 to 1.5), while those that went to the PICU had the highest CAS scores on average (Presentation CAS=8.2, CI: 7.7 to 8.7; Disposition CAS=6.9, CI: 6.5 to 7.3) and those patients admitted to the floor had, on average, a CAS in between (Presentation CAS=6.0, CI: 5.7 to 6.4; Disposition CAS=3.2, CI: 3.0 to 3.5) (p<0.0001).

Conclusions The CAS is significantly correlated with both length of stay and disposition of patients. A higher CAS score predicts both a longer length of stay and a more acute disposition. Future work developing an algorithm inclusive of the CAS to evaluate patients for appropriate disposition including the use of an observation unit for paediatric asthma patients is planned.

55 Testing teens for hiv – are we doing what we should?

J Fernelius1,2

K Migacz1,2

A Srinivasan1,2

C Bell1,2

L Benjamins1,2

1UTHealth, Houston, TX

2McGovern Medical School, Houston, TX

Purpose of study Globally, nearly 25% of patients with HIV are 10–24 years old. In 2015, about 51% of the 61,000 HIV infected youth ages 13–24 in the U.S. were unaware of their status. Although CDC (2006), AAP (2011), and USPSTF (2013) guidelines recommend routine testing of adolescent patients, little is known about how often physicians screen for HIV per these standards. Thus, we set out to assess the prevalence of HIV testing among adolescent patients and to compare rates of testing from 2010 to 2015 in our practice.

Methods used After IRB approval, we conducted a retrospective chart review of patients aged 13–18 seen in general paediatric and adolescent outpatient clinics in a large university-based setting. Whether or not a teen was tested for HIV at these visits was the primary outcome. Additional variables included sexual history, reported condom use and HPV vaccine status. Descriptive statistics were performed.

Summary of results To date, 473 charts have been reviewed. Mean patient age was 14.9 (14.6 in paediatrics, 16 in adolescent), 49.9% were female, 67.2% African American and 16.0% Hispanic. In 2010, 7.6% of patients were tested for HIV in the paediatric clinics, 34.3% in adolescent. In 2015, rates were 6.3% and 38.2% for paediatric and adolescent clinics respectively. Of the 63 patients tested for HIV, the average age was 16. In this group, 63.5% reported sexual activity, 20.6% reported unprotected sex, 44.4% had used a condom at their last sexual encounter, and 46.0% had received at least one HPV vaccine. Among patients not tested for HIV, 15.9% reported sexual activity, however, for 28% sexual activity was not assessed.

Conclusions Overall testing rates for HIV were similar in 2010 and 2015. Teens were more likely to be tested in the adolescent clinics, however, these patients were also older. Teens who reported sexual activiity were also more likely to be tested. This study provides important information on how often teens were tested within this population, but may not be generalizable to other settings. Thus, similar studies in other practice settings to determine how and when physicians decide to test for HIV will be crucial. Understanding this is important for the future design and implementation of interventions directed towards routine, non-risk based screening.

56 Bruised buttocks: is it abuse?

JP George

R Brown

K Morgan

University of Oklahoma Health Science Centre, Oklahoma City, OK

Case report Bruising is the most common evident injury in children who have been physically abused1. Moreover, certain locations of bruising are more consistent with physical abuse. For example, in children younger than 4 years, and back, buttocks, forearm, foot and abdomen are rarely bruised from day to day activities2. Here we discuss a 7 month old child who presented with bruised buttocks and was diagnosed with Acute haemorrhagic oedema of infancy (AHEI)

K.H. is a 7-month-old male who presented with burised buttock that mother noticed when giving the child a bath. The mother states that the child had fallen asleep on a hard-plastic swing, had a low height fall onto the floor from the family sofa 3 days prior, and had been increasingly fussy.

Physical exam shows a well appearing, afebrile, non-toxic child who is noted to have a grossly benign exam except for a nontender 3 cm circular purpuric patch on the left buttock and a similar 3×2 cm patch on the right buttock with extension down the right thigh laterally. Given the vague history provided by the mother our child abuse service was consulted and agreed a non-accidental trauma work up was warranted. The work up included labs, a computerised tomography scan of the head, a skeletal survey and ophthalmological evaluation. The work up was negative except for an elevated d-dimer, which would be expected with the presence of bruising seen. Consequential, the diagnosis of AHEI was made. Child was discharge home and was followed to note complete resolution of bruising without other complications and no other situation concerns for abuse.

Acute haemorrhagic oedema of infancy (AHEI) is a rare cutaneous illness that affects children between the ages of 4 months and 2 years of age and is classically thought of as a purely cutaneous form of Henloch-Schonlein Purpura3. AHEI has classically been characterised with a triad fever, purpuric lesions and oedema which can yield the physician to be faced with a wide differential4, however in the absence of a fever and often an unclear history non-accidental trauma must also be considered and can often pose a diagnostic challenge given that bruising patterns associated with AHEI are not often associated childhood activities.

57 Improving screening and referral for postpartum depression in a paediatric primary care clinic

AL Hoppmann

M Silverberg

B Ratliff

S Hall

S Walley

C Dye

University of Alabama at Birmingham, Birmingham, AL

Purpose of study Postpartum depression (PPD) has been shown to impact maternal-child bonding and infant development. As PPD is often under-recognised and undertreated, our aims included increasing paediatric resident knowledge and comfort of screening a mother for postpartum depression by 30% and referring 50% of positively screened mothers for depression counselling.

Methods used We implemented a resident-driven quality improvement project at the University of Alabama at Birmingham (UAB) Paediatric Primary Care Clinic (Clinic) with partnership with the UAB Community Counselling Centre. Residents had an educational curriculum which included PPD and a new screening/referral process over a 1 week period in Clinic. Maternal depression was evaluated using the Edinburgh Postnatal Depression Scale at the 1 month – 6 month well child check appointments, while resident knowledge was evaluated via online survey collected pre and post educational intervention. Mothers who screened positive for PPD were referred. Strategies included process mapping and collaborative intervention planning with clinic faculty and support staff. Data was compiled and analysed using Microsoft Excel.

Summary of results Fourty-six residents completed the survey prior to intervention while 36 residents completed the post-survey. Following the educational intervention, the proportion of residents who knew how to screen for PPD increased by 30%, while the proportion of residents who felt comfortable screening and referring mothers with PPD increased by >50%. Of the 35 mothers who screened positive for PPD, 17 (49%) were referred for counselling and 3 (15%) mothers attended an initial counselling session.

Conclusions A clinic based educational curriculum improved resident knowledge and comfort of maternal screening/referral for PPD. While almost one-half of mothers who screened positive for PPD were referred for counselling, only a small proportion attended. Future aims include determining alternative strategies to assist mothers with PPD.

58 An unsusual cause of hip pain in a young girl

MN Frascogna

B Dillard

E Landry

University of Mississippi Medical Centre, Jackson, MS

Case report Hip pain is a common complaint evaluated and treated in the Paediatric Emergency Department. Even though musculoskeletal causes are the most common, practitioners must keep a broad differential diagnosis list in mind, including pain referred from nearby structures. We present a case of hip pain in a young girl with an unusual cause.

A 5 year old female presented to the paediatric emergency department (PED) with a complaint of recurrent left hip pain that started approximately 3–4 months prior to her presentation. The pain occurred infrequently and typically would resolve when treated at home with ibuprofen. She had been worked up for this complaint by her primary care physician and by a local orthopaedic surgeon with lab work and imaging reported by the patient’s mother to be normal. Prior to evaluation in the PED, she began to have worsening left hip pain and vomiting. On exam, she was afebrile, had no abdominal tenderness, and had full range of motion of her hips with no gait abnormalities. She reported pain to palpation of her left lower flank above her hip. An abdominal X-ray was performed showing moderate stool in the distal colon but no signs of obstruction or mass effect. A complete blood count, metabolic panel, and urinalysis were obtained and were noted to be unremarkable. She was given a normal saline bolus and morphine with improvement, but not resolution, of her pain. An abdominal CT was performed and showed a pelvic mass in the area of the left ovary. A pelvic ultrasound was obtained and showed no definite vascular flow within the left ovary, leading to a diagnosis of left ovarian torsion with ischemia. The paediatric surgery service was consulted and she was taken to the operating room where an exploratory laparotomy was performed with detorsion and left oophoropexy. She was monitored post-procedure and was discharged home three days after admission in good condition.

Ovarian torsion can be difficult to diagnosis, especially as it can be intermittent. Though it is much more common in reproductive aged girls, ovarian torsion should be kept on the differential in all females with unilateral abdominal, back, or hip pain.

59 Exploratory study on discussion of transition of care for adolescents during well child checks

S Leung

S Mennito

Medical University of South Carolina, Charleston, SC

Purpose of study Despite guidelines on transitioning paediatric patients to adult care, many young adults do not receive appropriate preventative care resulting in emergency department overuse, high medical costs, and low adult vaccination rates. A core factor is whether transitional care discussions occur between medical providers and patients. This study evaluated whether transition discussions are held during adolescent well child checks (WCC) and if teen health status impacts the likelihood of those conversations occurring.

Methods used We identified adolescents ages 15 and up seen in a resident continuity clinic for a well check between January 2015 and January 2016 and categorised them as being ‘overall healthy’ or ‘medically complex’ based on pre-set criteria. We reviewed charts for transitional discussion key words or for the use of an electronic medical record (EMR) transition template developed locally for teen WCC. Two-tailed Z-test was used for two statistical analyses: at the subject level, n=100, and at the visit level of each well child check, n=161.

Summary of results A total of 432 subjects were identified of whom 100 were randomly selected for review. Patients were 59% female and 65% overall healthy when seen between 2013 and 2017, ages 15–18 years old. At the subject level, transitional discussions occurred for 60% of medically complex teens compared to 49% of overall healthy teens (p=0.3; Z-score of 1.02). At the visit level, a transitional discussion occurred at 45% of WCC for medically complex teens compared to 33% of WCC for overall healthy teens (p=0.14; Z-score of 1.4). No documentation of transition discussions occurred without the use of the EMR template.

Conclusions Despite transition EMR template availability, transition of care topics are not consistently documented for any adolescent, healthy or otherwise. Use of a template encouraged documentation of transition discussions. Sports physicals and sick visits, which utilise separate EMR templates, may be missed opportunities. Additionally, transition of care topics are at the end of the WCC template, which may limit its use during visits with numerous topics to cover. Adjustment of the available template and further education to encourage earlier and more frequent talks about transition may improve rates of those discussions.

60 Impact of multidisciplinary management on comorbidities of paediatric obesity

AN Metcalf

AE Weedn

S Gillaspy

University of Oklahoma Health Sciences Centre, Oklahoma City, OK

Purpose of study 17% of US children ages 2–19 are obese, and at risk for dyslipidemia, type 2 diabetes, and non-alcoholic fatty liver disease. The United States Preventive Services Task Force recommends multidisciplinary management, which has been shown to stabilise body mass index (BMI), but studies on the impact of this treatment on obesity comorbidities are limited. This study examined the effect of a paediatric multidisciplinary weight management clinic on targeted cardiometabolic markers of health.

Methods used Participants 2–18 years of age presented to the Healthy Futures Clinic from August 2012 to October 2016 and had a BMI ≥99 th percentile or a BMI ≥95 th percentile with an obesity-related comorbidity. Patients and families received behavioural counselling from a paediatrician, dietitian, physical therapist, and psychologist at an initial visit and every 3 months, with individualised follow-up visits in the interim. BMI, fasting cholesterol, triglycerides, HDL, LDL, glucose, ALT, and haemoglobin A1c were obtained at baseline and at 6 months. Changes in BMI and lab values were analysed continuously by paired t-tests and categorically (abnormal vs normal results) by McNemar’s Test.

Summary of results 138 participants presented to the clinic; 63% were female and 61% were between 6 and 12 years of age. At baseline, 43.8% had abnormal ALT levels, 43.8% had elevated triglyceride levels, and 34.1% had high cholesterol. Among patients still participating in the clinic at 6 months (n=78), total cholesterol improved by an average of 6 mg/dL (p<0.05), and triglycerides improved by an average of 14.6 mg/dL (p<0.05). Of these patients (n=78), 32.0% had hypercholesterolemia at baseline, which improved to 19.2% after six months of treatment. Similarly, 39.7% of returning patients (n=78) had abnormal triglyceride levels at baseline, which was reduced to 25.6% at six months. Glucose, HDL, LDL, and ALT also improved at six months but no significant difference was demonstrated. These improvements occurred despite a lack of change in BMI with treatment.

Conclusions Six months of multidisciplinary clinical intervention improved dyslipidemia in obese children, and these improvements occurred without change in BMI. Future studies will examine treatment effect at 12 months.

61 Abdominal mass(es) in a paediatric patient

Y Nathani

J George

C Knoles

University of Oklahoma, Oklahoma City, OK

Case report D.J. – an obese 10-year-old premenarchal female presented with worsening RLQ abdominal pain with nausea and vomiting. ROS negative for fever, jaundice, weight loss, trauma. Initial VS were normal and exam was only impressive for RLQ tenderness without peritonitis. Ultrasound showed a 9×9×10 cm complex anechoic structure right pelvis with normal blood flow (figure 1) and a mass in RUQ. Subsequent CT scan showed a 5 cm solid, well circumscribed mass in liver (figure 2) in addition to complex mass in pelvis. Labs were normal and HCG was negative. Due to progressive symptoms, she underwent a laparoscopic right salpingectomy for paratubal cyst torsion with normal viable ovary. The liver mass was determined to be focal nodular hyperplasia (FNH).

This was diagnostically challenging as the patient had two distinct masses with progressive symptoms. Possible etiologies included ovarian or tubal cyst, primary ovarian/hepatic tumours, metastases, abscess, ovarian/tubal torsion and ectopic pregnancy.

Paratubal cysts are remnants of Wollfian and Mullerian ducts and constitute 3%–7% of adenexal masses in paediatric patients.1 Increasing size of paratubal cysts are associated with obesity, likely due to excess androgens. Most are asymptomatic; however, they can present with abdominal pain due haemorrhage, rupture and torsion. These cysts are non-physiologic and unlikely to spontaneously regress and definitive management is operative.

FNH is generally a benign and asymptomatic lesion of the liver, usually discovered incidentally. These lesions are associated with high oestrogen states, including obesity and require surgical intervention only if symptomatic.

Reference

  1. . Muolokwu E, Sanchez J, et al. The incidence and surgical management of paratubal cysts in paediatric and adolescent population. J Pediatr Surg2011;46:2161–2163.

Abstract 61 Figure 1

Ultrasound showing complex mass in right adenexa

Abstract 61 Figure 2

CT scan showing liver mass

62 Inpatient breastfeeding study: what are the barriers to inpatient breastfeeding exclusivity?

E Omoruyi1

MS Evangelista1

TA Sugrañes2

1University of Texas Health Science Centre at Houston, Houston, TX

2New York-Presbyterian/Weill Cornell, New York, NY

Purpose of study Breastfeeding is widely accepted as the best nutritional source for infants. In the United States, exclusive breastfeeding is recommended until at least 6 months of age. Although breastfeeding has been shown to have beneficial effects, studies have shown that problems and emotional discomfort experienced during early breastfeeding are risk factors for breastfeeding cessation before 6 months. Determining why mothers are unable to achieve breastfeeding exclusivity in the immediate postpartum period is important to the creation of targeted interventions.

Methods used This cohort study enrolled 510 women who were each personally interviewed using a survey tool during the immediate post-partum period in an urban county hospital. Factors assessed included breastfeeding history, perceptions regarding breastfeeding/breastmilk, socioeconomic factors, as well as hospital and family support. Breastfeeding exclusivity at discharge was assessed based on the mothers’ self-reported infant feeding behaviour during her hospital stay.

Summary of results 38% of women exclusively breastfed during their inpatient stay. Several maternal, hospital, and external factors influence exclusive breastfeeding in a hospital. While the knowledge and experience of breastfeeding other children is a determining factor (OR=3.33; 95% CI: 1.01 to 11.04), several hospital factors such as skin to skin contact, knowledge about whom to approach and ask about breastfeeding help after discharge, and encountering issues in hospital are responsible for encouraging the behaviour of exclusive breastfeeding in a hospital (p<0.05).

Conclusions This study highlights that knowledge regarding breastfeeding as well as knowledge regarding sources of support, both inpatient and outpatient, are important factors that affect immediate breastfeeding outcome. This is one of the few studies that looks at specific factors affecting exclusive breastfeeding in the inpatient setting.

63 Breastfeeding duration and bone strength in young adult females

K Perofsky

NK Pollock

Augusta University, Augusta, GA

Introduction Data on the relationship between breastfeeding and bone health are scant and equivocal. This study investigated the relationships between breastfeeding duration and indices of bone strength in young adult females.

Methods Bone mass, density, and geometry at trabecular and cortical sites of the tibia were measured in 71 white females (aged 21±0.4 years) by using peripheral quantitative computed tomography. Breastfeeding duration was self-reported by each participant’s biological mother. Fat-free soft tissue (FFST) and fat mass were measured using dual-energy X-ray absorptiometry. Relationships between breastfeeding duration and bone parameters was determined using multiple linear regression models, including height, FFST, and fat mass as covariates.

Summary of results 20% of the participants reported not having been breastfeed; 32% were breastfed 1–6 months; 24% were breastfed 6–12 months; and 24% were breastfed 12 months or longer. At the trabecular site of the tibia, breastfeeding duration was a positive independent predictor of total volumetric bone mineral density (β=0.28, p=0.045). Although breastfeeding duration was positively correlated to bone strength index (BSI; r=0.28, p=0.03), it was not an independent predictor of BSI in the regression model. At the cortical site, breastfeeding duration was a positive independent predictor of bone mineral content (β=0.24, p=0.02), cross-sectional area (β=0.23, p=0.02), and cortical thickness (β=0.36, p<0.01). There were no associations between breastfeeding duration and the other bone parameters.

Conclusions Our results suggest that a greater duration of breastfeeding may have long-term benefits on cortical and trabecular bone. Given that our findings should be considered hypothesis generating, further studies are needed to elucidate the role of breastfeeding on bone development.

64 Inappropriate prescribing of systemic steroids and opioids to paediatric patients with pneumonia or sinusitis

KG Phang1

J Roberts1

S Garner2

M Ebeling1

W Basco1

1Medical University of South Carolina, Charleston, SC

2South Carolina College of Pharmacy, Charleston, SC

Purpose of study National clinical guidelines do not recommend use of systemic steroids or opioids in treating paediatric pneumonia or sinusitis. The purpose of the study was to compare the frequency of systemic steroid or opioid prescribing for children with pneumonia and sinusitis based on location of care. We tested the hypothesis that inappropriate prescribing of systemic steroids and opioids for paediatric pneumonia or sinusitis was greater in the emergency department than other clinical sites.

Methods used The study evaluated paid claims for visits and medications using 2016 South Carolina Medicaid data. Subjects were 5 to 18 years old with a primary diagnosis of pneumonia or sinusitis, selected using the ICD-9 and ICD-10 Clinical Classification Software Category System. The prescription of a systemic steroid or an opioid dispensed 0–7 days from a visit claim was evaluated. Medicaid visits were associated with one of 3 locations: emergency department (ED), urgent care (UC), or ambulatory site. Patient demographic data avaialable included ethnicity, gender and age in months.

Summary of results A total of 16 480 visits were evaluated from all 3 settings. Of 2153 visits in the ED 273 (13%) included a systemic steroid and 98 (5%) included an opioid. Of 14 149 visits in the ambulatory setting 974 (7%) included a systemic steroid and 376 (3%) included an opioid. Of 178 visits in the UC 15 (8%) included a steroid. Too few patients were prescribed an opioid in the urgent care setting to perform statistical analysis. ED visits were associated with a higher steroid prescription rate (chi square, p<0.0001) when compared to ambulatory and UC visits. ED visits were associated with a higher opioid prescription rate (chi square, p<0.0001) when compared to ambulatory visits.

Conclusions Our results suggest that school age children and adolescents received steroid and opioid prescriptions at higher frequency when seen in the ED versus ambulatory setting. Given safety concerns of steroids and opioids in paediatric patients, improved prescribing practices for these medications are needed.

65 Knowledge of and attitudes toward hpv vaccine in preadolescents and teens

L Raney

E Owers

R Pasternak

P Prasad

R Gardner

LSUHSC, New Orleans, LA

Purpose of study Human Papilloma Virus (HPV), common in both females and males, is responsible for pathologies ranging from benign genital warts to cervical and penile cancer. Pharmaceutical companies have now developed a vaccine that will help prevent the virus-associated malignancies. The CDC recommends that females ages 11–26 years and males ages 11–21 years receive the vaccine series. Despite being widely available and highly publicised, only 40% of eligible females receive the vaccine. This study aims to assess the knowledge of HPV, the attitudes towards the HPV vaccine, and identify barriers preventing full utilisation of the vaccine. Once identified, we aim to overcome the barrier(s) and improve vaccination rates in eligible adolescents.

Methods used We distributed a standardised questionnaire to the parents of eligible female and male patients in both paediatric hematology-oncology and general paediatric clinics. It assessed the parents’ knowledge of HPV and the vaccine, their views of the vaccine, and reasons why they may oppose it. Additionally, we will compare the views of parents between these two settings, in order to determine if the patient‘s personal history of a hematologic or oncologic disease influences their decision to permit vaccination.

Summary of results 75% of parents say they have been educated about HPV, mostly by their primary care physician. However, only 25% knew what disorders HPV caused. Only 40% felt the vaccine should be added to the typical vaccine schedule. Surprisingly, eighty percent of parents intend to or already have vaccinated their child. In those that opposed the vaccine, one-third were concerned about potential side effects and nearly 20% feel they do not have enough information.

Conclusions The largest barrier to the utilisation of the HPV vaccine that we have identified appears to be lack of education. As a result, we have begun distributing the CDC’s HPV and vaccine patient guide to our patients’ families as an intervention. We will resurvey these families after implementing the intervention to assess its success in increasing both knowledge and utilisation of the HPV vaccine.

66 Separating fact from fiction: diagnosing functional neurological disorder

HA Rehman

S DeLeon

D Hahn

OUHSC, Oklahoma City, OK

Case report Patient is a 13 year old girl with a history of complex regional pain syndrome who presented with new onset visual changes and severe headaches. Ophthalmologic exam showed bilateral optic disc cupping, lumbar puncture (LP) revealed a mildly elevated opening pressure and magnetic resonance imaging (MRI) revealed bulging optic discs; she was admitted for presumed idiopathic intracranial hypertension (IIH). Multiple therapeutic LPs resulted in mild headache relief with no improvement in vision. An intracranial pressure monitor showed normal pressure, ruling out IIH. A comprehensive autoimmune, endocrine and infectious work-up was negative. She later reported back pain and spine MRI noted L1-L2 discitis osteomyelitis of unclear aetiology. She received extensive treatment with steroids, a selective serotonin reuptake inhibitor, a carbonic anhydrase inhibitor and several pain medications.

After 6 weeks with no definitive diagnosis, she was transferred to our facility per parent request. Multiple specialists were consulted including neurology, ophthalmology, psychology, infectious disease, physical therapy (PT), pain management and neurosurgery. She had various complaints including persistent headaches, changes in visual acuity and fields, and weakness and numbness of her left extremities. Her exam, however, remained inconsistent and all symptoms except for headache and vision changes spontaneously resolved. Repeat MRIs and ophthalmology exam revealed normal orbits with resolution of disc cupping. Given persistent complaints with normal exams and studies, she was diagnosed with functional neurologic disorder (FND) in addition to lumbar osteomyelitis and discitis. She worked with psychology and PT and was transferred to a rehab facility specialising in the disease process. Though potential stressors were identified, these were never fully accepted as the aetiology by the patient or family.

FND, previously known as conversion disorder, is manifested by neurologic symptoms that cannot be explained by an underlying disease pathology. Patients experience motor, visual, speech or sensory changes believed to be triggered by an emotional or physical stressor. Treatment involves a multidisciplinary approach with therapy and often medication. Early diagnosis is key to prevent unnecessary testing that can lead to a poorer prognosis.

67 Acute disseminated encephalomyelitis in a six year old male presenting as unilateral optic neuritis

AR Riggs

D Hahn

Oklahoma Health Sciences Centre, Oklahoma City, OK

Case report Our patient is a previously healthy 6 year old male who presented with a ten day history of intermittent nausea and vomiting, fluctuating periods of hyperactivity and fatigue, as well as acute onset loss of colour vision, decreased visual acuity, and appearance of scotomata in his right vision field. The patient’s primary care physician referred him to an Ophthalmologist, who noted right optic nerve swelling. The patient was then transferred to our Emergency Room, where a thorough initial lab workup yielded only mild leukocytosis on a blood sample. The only pertinent physical exam finding was fatigue. A brain Magnetic Resonance Imaging (MRI) was obtained to evaluate for optic neuritis. The MRI revealed global involvement of the brain with diffuse white matter lesions predominantly in bilateral cerebral hemispheres. These findings steered us to a diagnosis of Acute Disseminated Encephalomyelitis (ADEM). We treated our patient with a five day course of high dose steroids. Within days, the child responded with symptomatic improvement. At a one month follow up, he continued to have improvement in his visual problems.

While ADEM has certainly been well-documented in children in our patient’s age group, his clinical presentation did not initially suggest the severity of the brain involvement found on MRI. Vacillating fatigue and hyperactivity paired with unilateral optic neuritis made for an unusual and unique presentation of ADEM.

68 Hematemesis and hematuria in a healthy 3 month old male

AR Riggs

D Hahn

Oklahoma Health Sciences Centre, Oklahoma City, OK

Case report Our patient is a previously healthy 3 month old male who presented to our Emergency Department with a report of poor oral intake, decreased urine output, hematemesis, and hematuria. The initial lab work-up was unremarkable with the exception of microscopic hematuria in a bagged urine. We admitted the child for further evaluation of the hematuria. Due to concern for formula intolerance and reflux causing his hematemesis, his formula was switched to Nutramigen and he was started on a trial of Omeprazole. The child failed to respond to our interventions and continued to have guaiac positive hematemesis. An esophagogastroduodenoscopy was performed, which showed no evidence of bleeding or ulcer. While repeat bag urine specimens continued to show microscopic hematuria, an in-and-out catheterization specimen surprisingly showed no red blood cells. At this time, multiple team members noted that patient only had positive findings when his mother was alone with the child, but never had positive findings when in care of nursing alone. When we confronted our patient’s mother with our observations, she soon admitted to contaminating the child’s stool, emesis, and urine with her own menstrual. The mother further confessed that she created the ruse in order to keep her son hospitalised so they would both be safe from the patient’s father. At this point, we contacted our Child Maltreatment, and the Department of Human Services was notified for concern for Medical Child Abuse.

Medical Child Abuse, also known as Munchausen Syndrome by Proxy, is an unfortunately common condition seen in the paediatric population. The simulation of symptoms can result in procedures and hospitalizations that are not necessary and are potentially harmful, as seen in our patient. Although it is difficult to recognise and confront medical child abuse, it is invaluable to do so early in order to protect patients from unnecessary discomfort and harm.

69 Arm pain among south carolina youth baseball players

J Roberts

M Rao

E O’Brien

A Greenhouse

E Dawley

J Eichinger

Medical University of South Carolina, Charleston, SC

Purpose of study Arm and shoulder injury to young athletes is increasing in the United States, especially among baseball pitchers. Although pitch count limitations have been implemented in youth league baseball, the need for ulnar collateral ligament reconstruction is increasing at a younger age, with 56% of these procedures being performed on 15–19 year olds. The objective of this study is to describe the injury burden to young athletes in South Carolina where baseball and softball are played year round.

Methods used We asked parents who brought their 7–17 year old child to any visit to one of 13 South Carolina Paediatric Practice Research Network (SCPPRN) practices to complete a survey. Initial questions included the age of the child, position(s) played, injuries and past surgeries. Examples of throwing-specific questions included timing and quality of arm pain, occurrence of pain or fatigue during games or practice, limitations of the pain in terms of participation or performance, and pressure to play through the pain. We also inquired about the number of months they play during the year, and interest in a pitch-tracking smart phone application. Questions were based on a previous study conducted in the Northeast for comparison. Descriptive statistics were calculated.

Summary of results To date, 131 parents have completed the survey. 35 (26.7%) reported a previous injury, and 3 (2.3%) reported having previous surgery. Responses of ‘never’ and ‘rarely’ were counted as responses in the negative, and ‘sometimes’, ‘often’, and ‘always’ were counted as responses in the affirmative. 34 (26.0%) report pain when they throw or play and 29 (22.1%) report that pain the day after playing. 7 (5.3%) reported that previous arm pain has negative effect on their enjoyment of the game. 9 (6.9%) report that a coach has encouraged him/her to keep playing despite the presence of arm pain. 81 (61.8%) of parents would be interested in having access to a pitch count app.

Conclusions Approximately one quarter of young athletes report the presence of arm pain while playing. The use of a smart phone app to track pitch counts may be a helpful tool to prevent overuse injury and should be tested in a cohort of youth baseball players.

70 Adolescent weight management – is there an app for that?

C SanGiovanni

M Steffen

C Pope

J Roberts

Medical University of South Carolina, Charleston, SC

Purpose of study Adolescent obesity has quadrupled in the past thirty years from 5% to 20.5% for 12–19 year olds. Paediatricians report lack of resources as a barrier to management. Since adolescent smartphone use has increased significantly and apps are useful in managing chronic diseases in adolescents, apps that assist with weight management could be a beneficial resource. However, little is known about features that overweight/obese adolescents have considered helpful. The objective of this study was to explore adolescents’ preferences about weight management app features and willingness to use collaboratively with their healthcare provider.

Methods used Participants, recruited from an urban and rural practice in South Carolina, were eligible if between 13–17 years old, had a Body Mass Index (BMI) at or above the 85th percentile and had access to a smartphone or tablet. Semi-structured interviews were conducted from May to October 2017. Participants were presented with 3 popular mobile health apps and asked to complete tasks using each app. Likes/dislikes about each app and which features (customised workouts, logging physical activity and food/beverage consumption, and social support) would encourage further use were elicited. Content analysis was performed on interview transcripts.

Summary of results Twelve adolescents were interviewed. Average participant age was 14.7 years, and average BMI was 96.6 percentile. Eight (67%) were female, and 8 (67%) identified as White, while the remainder identified as African American. The majority had used health apps in the past, but none were recommended by healthcare providers. All adolescents felt apps would be helpful in managing their weight. Adolescents’ most favourable features were finding healthy food alternatives and customising their workouts. Additional features they would include in health apps were competing with friends to exercise more or eat healthy, incentives to work toward healthy goals, sharing customised workouts with friends, creating a music playlist for exercising, and hearing weight loss success stories from other adolescents.

Conclusions Adolescents think health apps would be helpful in weight management. The use of apps should be studied to determine their role as part of an effective weight management program.

71 Kawasaki disease outbreak in central appalachia

VP Smith

D Macariola

JW Schweitzer

ETSU, Johnson City, TN

Purpose of study In the US, Kawasaki Disease (KD) has a global hospitalisation rate of 17.1 per 1 00 000 children. Although the aetiology remains unknown, the current consensus is that KD is likely caused by an infectious trigger initiating an abnormal immune response in genetically predisposed children. In March of 2017, Central Appalachia had an outbreak of KD, with 4 patients concurrently hospitalised with this disease. It is unclear whether this outbreak was due to the genetic susceptibility of the Appalachian population, or exposure to an antigen which was particularly toxic. This study seeks to better understand the underlying genetic predisposition and pathogen antigenicity related to KD in the Appalachian region.

Methods used All data was collected via chart review and retrospective study. Subjects were all admitted to the hospital within 1 week of each other and presented with fever prior to admission. During their admission, each subject was diagnosed with KD. All patients were followed for sequelae in the outpatient setting.

Summary of results Table 1 summarises the data during each subject’s hospitalisation. All subjects met criteria for KD while in the hospital. All subjects were treated with IVIG and had resolution of fever. The mortality associated with all subjects was 0%. No subjects had long-term sequelae.

Conclusions The Appalachian population may be more susceptible to KD, however, a larger chart review of incidence of KD in the region for multiple years would be necessary to draw these conclusions. It is much more likely that there was a highly antigenic pathogen present in the population in early spring 2017 that increased the incidence of this outbreak. Although a small sample size was used, the study’s incidence was dramatically increased from the US incidence when considered on a monthly basis. Public health officials and providers should be aware that the Appalachian region may be more genetically susceptible to outbreaks of KD, or be exposed to pathogens with increased antigenicity, and thus have a lower index of suspicion for diagnosis of KD when multiple cases occur in relative proximity to one another.

Abstract 71 Table 1

Summarises the data during each subject’s hospitalisation

72 Persistent hyperglycemia secondary to second-generation antipsychotic medications

N Soulages Arrese

AB Middleman

JD Meyer

University of Oklahoma Health Sciences Centre, Oklahoma City, OK

Case report It has been shown in the literature that second-generation antipsychotics are associated with patients developing hyperglycemia and diabetes mellitus along with other metabolic changes such as weight gain and lipid profile abnormalities. This reports highlights a case of a paediatric patient with hyperglycemia while taking second-generation antipsychotics in the setting of a complex social situation and weight loss. C.K. is a 16 year old with a history of family dysfunction, depression, suicidal behaviours, and cognitive delay and prolonged psychiatric admissions. She presented at age thirteen to endocrine clinic with hyperglycemia, impaired fasting glucose, normal HgbA1c while taking quetiapine. Approximately 1.3 years later, 1 year prior to presenting to our service, the patient developed polyuria, polydipsia, hyperglycemia, elevated HgbA1c of 9.2%, without diabetic ketoacidosis, and BMI 19.7. The patient had recently been switched from quetiapine to olanzapine. Antibodies associated with Type 1 Diabetes Mellitus were negative. The patient was treated with an intensive insulin regimen including long and rapid acting insulin. At presentation to our service, an inpatient service for malnutrition, the patient had severe malnutrition, BMI of 15.3 and HgbA1c of 5.7%. The mother had discontinued insulin when the patient had stopped eating. The patient indicated that she had been too tired to eat. The mother reported she was giving the patient additional olanzapine ‘as needed’. Genetic testing for Maturity-onset diabetes of the young was completed and results were negative. The patient was transitioned from olanzapine to risperidone, as it is associated with less risk of hyperglycemia. During treatment for malnutrition, glucose levels improved, requiring rare corrections with regular insulin. This case serves as a reminder of the importance of appropriate metabolic screening for paediatric patients, regardless of weight, before and during treatment with second-generation antipsychotics; the metabolic changes may cause significant and potentially irreversible morbidity.

73 Do children seen in the emergency department for skin and soft tissue infections who are admitted to the inpatient service meet criteria for sepsis?

B Weaver

D Nakayama

JJ Burns

B Weidner

University of Florida, Pensacola, FL

Purpose of study Admission of patients with skin and soft tissue infections (SSTI’s) have increased as have hospital charges. The main treatment of these is incision and drainage (I and D) for abscesses which can take place as an outpatient or inpatient.

Systemic inflammatory response syndrome (SIRS) criteria include temperature, heart rate, breathing rate, and white blood cell count. A patient with positive SIRS criteria and source of infection qualifies as having sepsis, justifying admission.

The purpose of our investigation is to determine the frequency of positive SIRS criteria in patients admitted for SSTI management. We also wish to determine if SIRS criteria status correlated with positive blood cultures, resolution without I and D and if I and D was performed in the operating room OR.

Methods used A retrospective chart analysis was conducted on 203 patients admitted with SSTI’s from the paediatric ED during 2011–2016. Charts were excluded if patients were repeat visitors for the current SSTI, outside hospital transfers, immunocompromised state, chronic debilitating illnesses, or if affected area was the face, mouth or inside the scrotum. Age, heart rate, respiratory rate, temperature, blood culture results, WBC, SIRS status (positive or negative), I and D status (required or not), and I and D setting were recorded (OR or not).

Summary of results Thirty-seven met criteria for study entry; mean age was 24.7 (STD 28.8) months.

59.5% patients met SIRS criteria; 40.5% did not.

Two patients had positive blood cultures. Both were contaminants.

72.7% of those who met SIRS criteria went to OR vs 53.3% who did not meet SIRS criteria. (Chi-square; p=0.225).

22.7% of patients who met SIRS criteria had SSTIs that resolved without I and D vs 40% with negative SIRS criteria (Fisher’s Exact Test) 0.295.

Conclusions Most of the admitted patients with SSTIs met SIRS criteria, yet there were 40.5% who did not and may have therefore been unnecessary admissions.

This study supports previous studies that indicate that blood cultures are not useful in SSTI management.

SIRS status did not correlate with rate of resolution without I and D or need for OR I and D.

Studies with higher N are needed to confirm these findings.

Adult case reports/ageing/geriatrics, Joint plenary poster session and reception, 4:30 PM, Thursday, February 22, 2018

74 Syncope evaluation using point-of-care ultrasound

EK Addo-Yobo

J Treece

N Kakkar

V Pai

MA Alawoki

C Rosero

Shamas, S, East Tennessee State University, Johnson City and Mountain Home, TN

Case report While syncope represents a common presentation of cardiovascular compromise, it is rarely the first presenting symptom of a pulmonary embolism (PE). If syncope occurs, delay in therapy can quickly become fatal. We describe a complex presentation of syncope in which bedside point-of-care echocardiogram would have directed appropriate management of a PE.

Eighty seven year old male with no significant cardiac history presented with three syncopal episodes. There were no prodromal symptoms or evidence of seizure activity. Blood pressure of 76/42 mmHg and pulse of 118 bpm were recorded by paramedics. Electrocardiogram (EKG) showed atrial fibrillation with rapid ventricular rate. He presented hypoxemic and hypotensive. Labs showed serum troponin of 3.7 ng/mL and acute renal insufficiency. EKG showed no acute ischaemic changes. Head imaging showed no acute intracranial pathology.

Acute coronary syndrome protocol with heparin drip was initiated. The patient opted for medical management and was transitioned to apixaban for stroke prophylaxis. Transthoracic echocardiogram (TTE) showed characteristic signs of right heart strain. Chest angiogram showed a large central pulmonary embolus in the right lung with additional multifocal segmental bilaterally. Subsequent lower extremity ultrasound showed a deep vein thrombosis in the left lower extremity. Apixaban was continued since the patient was oxygenating well on nasal cannula and was hemodynamically stable.

Syncope is an uncommon presentation for PE. Even when an alternative reason for syncope exists, it is estimated that PE is present in 12% of patients with syncope. Our patient presented with syncope in the setting of ACS, new onset atrial fibrillation and hypotension. The TTE showed the Mcconnel sign, a finding which has a 94% specificity for a PE. This sign is a pattern of right ventricular dysfunction with akinesia of the mid wall and hyper contractility of the apical wall. If bedside sonography was part of the initial encounter, the patient’s presentation would have appropriately directed treatment towards thrombolysis or thrombectomy. It has been shown that prognosis for PE is best if treatment within the first hour. Prompt initiation of treatment is even more important because syncope is an ominous sign of PE.

75 Intravascular lymphoma presenting as respiratory failure

A Bokhari

D Trofimovitch

B Boonpheng

H Zaver

T Nold

J Goldstein

ETSU, Johnson City, TN

Case report Lymphoma, a great mimicker, presents with an array of signs and symptoms in almost any organ system. Here we present a case of acute respiratory failure in an otherwise healthy elderly man.

A 73-year-old man presented with four weeks of progressive dyspnea on exertion. He reported a non-productive cough, low-grade fevers, chills, and night sweats. He denied chest pain, weight loss, or lack of appetite and was hemodynamically stable on presentation. Physical examination was significant only for diffuse crackles and 2+pitting ankle oedema bilaterally. Chest x-ray demonstrated bibasilar infiltrates, mild pleural effusion and cardiomegaly. Metabolic panel revealed hyponatremia (119 mEq/L) with high urine osmolality and Na, consistent with SIADH. Serum cortisol was normal; however TSH, free T4 and rT3 were compatible with central hypothyroidism. CBC was within normal limits. Initially he was on antibiotic therapy for presumed Community-Acquired Pneumonia. CT chest showed bibasilar atelectasis, bilateral pleural effusions, and no evidence of embolism. Multiple incidental calcified mediastinal lymph nodes were aspirated, with benign pathology. Patient underwent thoracoscopic wedge lung biopsy; pathology indicated high-grade intravascular large B cell lymphoma positive for CD20, CD79a, Pax-5, CD10, Mum-1, and negative for CD3, cytokeratin AE1/AE3, S100, Cam5.2, and CD34. The lymph node histology was consistent with a remote granuloma. Gram stain, AFB, culture and fungal studies were negative. Bone marrow showed minimal presence of lymphoma. CT abdomen showed mild hepatosplenomegaly, a mildly enlarged epigastric lymph node, and no other intra-abdominal lymphadenopathy. The patient developed type 1 respiratory failure likely due to lymphangitis carcinomatosis, which improved with chemotherapy [R-CHOP].

Intravascular lymphoma is rare and aggressive. It was an unexpected diagnosis in our patient with predominant respiratory distress and pulmonary infiltrates. This subtype of lymphoma can manifest in any organ system and presents a diagnostic challenge given most information is from a paucity of case reports. Clinicians should maintain a high index of suspicion in high-risk patients presenting with an atypical constellation of symptoms.

76 Doubling down on anakinra: a case of relapsing still’s disease

S Chalasani

V Majithia

S Kishore

University of Mississippi, Jackson, MS

Case report Adult onset Still’s disease (AOSD) is a rare systemic inflammatory disease of unknown aetiology characterised by fever, arthralgia, salmon-pink rash and leukocytosis. We present as classic case of AOSD who was refractory to treatment with high dose steroids and Anakinra.

A 21-year-old African American male with no past medical history presents with 1 month history of worsening joint pains and fever. 2 weeks prior to presentation, he had a diffuse scaly erythematous rash on his trunk and extremities. Fevers were as high as 104 °F responsive to Tylenol. Patient had similar symptoms 9 months ago that resolved with doxycycline and prednisone. On exam, he was ill appearing with fever of 100.3 °F. He had peeling macular rash involving trunk, arms and legs. He complained of pain with active and passive range of motion in all joints. Pertinent labs include: haemoglobin 7.1 g/dL, white blood cells 17.8 TH/cmm with 73% neutrophils, platelets 362 TH/cmm, aspartate transaminase 163 U/L, alanine transaminase 117 U/L, sedimentation rate of 104 mm/hr, C- reactive protein 21.4 mg/dL, Ferritin >100,000 ng/ml and elevated IL-2 receptor at 4090 pg/mL. Other rheumatological and infectious work up was negative. Imaging relieved hepatosplenomegaly and diffuse lymphadenopathy. Bone marrow biopsy ruled out malignancy and there was no evidence of hemophagocytosis. Patient was started on methylprednisolone 60 mg thrice daily and noticed significant clinical and serological improvement. He was later discharged on 60 mg prednisone. Follow-up at 2 weeks, he had clinally worsened requiring admission for pulse dose steroids. He was also started on Anakinra 100 mg daily. Four weeks into therapy with Anakinra, as the steroids were being tapered he had relapsed again. Therefore, the dose of Anakinra was doubled. Patient has been doing well on the current regimen. He is now undergoing slow taper of his steroids with recent addition of Methotrexate.

AOSD is considered an interleukin (IL)−1 or IL-6 and IL-18-driven disease. Treatment in severe disease requires high-dose steroids and IL-1 or IL-6 blockers. Anakinra is an IL-1 receptor antagonist used for treatment of AOSD at recommended dose of 100 mg daily. However, our patient’s disease was not controlled on this recommended dose and required higher levels of Anakinra for stabilisation.

77 Late renal manifestations of henoch-scholein purpura in adults and its relation to immunoglobulin a nephropahy

SH El Nawaa

A Shredi

H Edriss

P Laoveeravat

TTUHSC, Lubbock, TX

Introduction Henoch-Schonlein purpura (HSP) is the small vessel vasculitis primarily seen in children with self-limiting disease, however, it is rare in adults with annual incidence of 0.1–1.8 per 1 00 000 individuals. HSP can lead to severe complications with multi-organ involvement in adults in comparison to children. Kidney is one of major organs affected which can contribute to significant morbidity and mortality.

Case presentation A 41-year-old Caucasian male from Texas, with past medical history of well controlled hypertension, type 2 diabetes mellitus, gouty arthritis, alcoholism, and hyperlipidemia, presented to his primary care physician’s office with complaints of severe colicky abdominal pain, nausea, vomiting, and an itchy purpuric skin lesion mainly on his lower extremities and the buttock area. Patient also had diffuse arthralgia, but denied having any bloody stools or gross hematuria. Labs were significant for mildly elevated liver enzymes and normal urinalysis. Metabolic profile showed a serum creatinine (0.66 mg/dl), and BUN (15 mg/dl). Patient was diagnosed with alcoholic liver cirrhosis and the skin lesions were not thoroughly investigated at the time. Almost six months later he presented to our hospital with hypertensive emergency and generalised anasarca. Labs showed AKI with a serum creatinine of 5.2 mg/dl and BUN of 35 mg/dl, nephrotic range proteinuria, and gross hematuria. There was active urinary sediment. kidney biopsy revealed mesangial expansion on LM, IgA and C3 deposits were seen on IF, no fibrin, IgG, IgM deposition were seen. Focal effacement of foot processes and numerous large deposits were seen on EM in the mesangium. The renal biopsy was diagnosed as IgA nephropathy. He was treated with high dose steroids without much improvement. With worsening renal function and anasarca, patient was eventually placed on hemodialysis with a hope of improvement in due course of time.

Discussion We describe a possible correlation between HSP and IgA nephropathy. Renal involvement of HSP with an IgA nephropathy like pathology is usually seen within 3 months of onset of rash; however, this patient’s had unusually delayed renal involvement till 6 months after the initial rash was noticed.

78 Furosemide as a tool for therapy and diagnosis

AL Heifner

Engel LS

Lo B

LSU Health Sciences Centre, New Orleans, LA

Introduction We describe a case of cardiogenic pulmonary oedema in a patient with negative biomarkers and a non-diagnostic echocardiogram.

Case A 68-year-old Spanish speaking woman with type 2 diabetes and hypertension presented to the hospital with a worsening chronic nonproductive cough and recent onset of lower extremity and periorbital oedema. She was seen by her primary care physician who prescribed furosemide and support hose one week prior. Her physical exam was significant for bilateral rales at the mid-lung zones and trace pedal oedema. Initial chest radiograph findings were suggestive of pulmonary oedema. Initial labs including troponin and BNP were unremarkable. During her admission, she became acutely hypoxic with desaturations in the 80 s requiring supplemental O2. CT angiography of her chest was negative for pulmonary embolism but showed bilateral pleural effusions and basilar airspace disease with ground glass opacities. One dose of intravenous furosemide was given in the emergency department followed by an extensive workup that included an aborted thoracentesis, non-diagnostic echocardiogram, non-diagnostic bronchoscopy with negative AFB stains, and labs for rheumatologic and infectious processes which were all negative. Cardiology recommended a repeat echo with straight leg raise to assess for occult diastolic dysfunction but this was not performed. The team then decided on a trial of aggressive diuresis which resulted in significant symptom improvement. A diagnosis of heart failure with preserved ejection fraction was made based solely on the clinical improvement.

Discussion The differential diagnosis for our patient was broad and included heart failure, infection, rheumatologic disorders, and malignancy. Her diagnosis was further impaired due to a language barrier and an inability to provide a good medical history. This led to a workup that may have been overly aggressive. A simple therapeutic trial of diuresis ultimately provided the diagnosis. This case illustrates that patients presenting with pulmonary oedema of unclear aetiology can be safely given a trial of diuresis, a non-invasive and cost-effective intervention with both diagnostic and therapeutic benefit.

79 Primary central nervous system lymphoproliferative disorder in a patient with cadaveric renal transplant

JM Hernandez1

S Maharaj1

J Chique-Figueroa2

C Isache1

1UF Health, Jacksonville, FL

2Mayo Clinic, Jacksonville, FL

Case presentation A 72 year-old African American female with history of cadaveric renal transplant in 2005 on tacrolimus and mycophenolate mofetil (MMF) presented after generalised tonic-clonic seizures. She required intubation for status epilepticus. CT brain revealed multifocal hypodensities in both cerebral hemispheres. MRI brain characterised these lesions as rim and nodular enhancing. A left cerebellar lesion was also identified. Craniotomy and biopsy of a frontal lesion was performed, histopathology revealing post-transplant lymphoprolipherative disorder (PTLD), polymorphic type. MMF was discontinued and tacrolimus dose reduced. Rituximab was commenced to be followed by whole brain irradiation. Serology for EBV was IgG (+) and IgM (-), with no evidence of infection prior renal transplant. Bone marrow biopsy had no signs of malignancy.

Discussion PTLD is a known complication of transplantation. Its incidence following kidney or liver transplant is 1%–5%. Risk factors include degree of immunosuppression, viral infection, allograft type, and therapy with calcineurin inhibitors (CIs). Primary central nervous system post-transplant lymphoproliferative disorder (PCNS PTLD) is a rare complication of solid organ transplantation representing 5%–15% of PTLD. The highest incidence of systemic PTLD occurs within one year after transplantation, whereas the median time of transplantation to CNS PTLD is 54 months. CNS disease is most often monomorphic, associated with renal transplant, EBV infection, and has a poor prognosis. Studies show that patients taking CIs have a significantly lower incidence of CNS disease, while the use of MMF has been linked to an increased risk in EBV (+) CNS PTLD. Optimal therapy is not known.

Conclusion PCNS PTLD is most often associated with renal transplant and EBV. With the upsurge in transplantations, clinicians and pathologists must be aware of this entity. CIs have been linked as a potential risk factor for PTLD in multiple studies; however recent studies postulate a protective effect for CNS involvement.

80 Complicated lung abscess with empyema thoracis in an elderly patient from primary dental infection

MK Islam

A Islam

T Vo

Texas Tech Univ HSC Amarillo, Amarillo, TX

Case report An 91 year old male with past medical history of Dementia, CAD, Type 2 DM, Hypertension; brought to the hospital due to recurrent non-resolving pneumonia. Patient was treated twice for pneumonia in last 8 weeks without complete resolution, no fever, weight loss, and hemoptysis. He was living at assisted living facility for last 8 months due to advanced dementia. Vitals: temperature 99 F, pulse 90/min, respiratory rate 18/min, BP 130/64 mmHg, SPO2%–95% on room air. On physical exam- dehydrated, bad oral hygiene including multiple dental caries, confused, oriented only to person, crackles in left lower lung, S1/S2 normal, no murmur/pericardial rub, no rash, neck stiffness, focal weakness. Initial lab: Hb 10 gm/dl, MCV 90, WBC 13 K, Neutrophil 78%, Bands 2%, Platelet 248 K, Na 138, K 4, Cl 100, HCO3 23, BUN/Cr 19/0.8, Blood sugar 81, Ca 8, Mg 2.1, albumin 2.9, AST 17, ALT 10, Alkaline Phosphatase 83, PT 13, PTT 300, d-dimer 329. CXR revealed LLL consolidation; CT chest showed right apical infiltrate, left lingular abscess and left loculated pleural effusion. Thoracentesis was performed, showed purulent fluid. Pleural fluid analysis suggestive of empyema, culture grew streptococcus anginosus. Patient was treated with ceftriaxone IV for 4 total weeks.

Discussion The Streptococcus anginosus group (also known as the S. milleri group) is a subgroup of viridans streptococci that consists of three distinct streptococcal species: S. anginosus, S. intermedius, and S. constellatus. The organisms are normal flora of the human oral cavity and gastrointestinal tract and are known for their pathogenicity and tendency for abscess formation. Oral, head and neck, and abdominal infections caused by members of the S. anginosus group are often mixed; they may involve other bacteria such as Eikenella corrodens, Fusobacterium nucleatum, or other microorganisms. Lung abscess and empyema thoracis of our patient is thought to be due to bad oral hygiene and dental caries; which cause bacteremia or aspiration pneumonia complicating with lung abscess and empyema. In resistant cases, surgical drainage may be considered if needed. Our patient reminds physicians’ about the importance to identify undiagnosed dental/oral infections which might be the source of systemic infection and abscess.

81 A case of metastatic stage 4 renal cancer and the relevance of hospice in end stages

F Mathai

R Pattabhi

TM Reske

L Ali

S Barry

EA Aguilar

LSU Health Sciences Centre in New Oreans, New Orleans, LA

Case report A 65-year-old female patient was admitted to the hospital for recurrent episodes of fever, chills and dysuria. She had no past medical history, was not on any medications, no history of alcohol use, cigarette smoking, no history of exposure to any chemicals and no family history. Evaluation revealed that she had metastatic, Stage 4, renal cancer ultimately not responsive to chemotherapy. She would have benefited from hospice. Although this option was discussed with the patient multiple times, she refused it.

In this case, despite the poor prognosis, the patient requested multiple sessions of chemotherapy, which were unsuccessful. She finally agreed to hospice the day before she passed.

In order to receive hospice, it is important for the patient to understand the course of their illness and their outlook must be directed towards symptom relief rather than cure of illness. In addition to optimising medical management, hospice would have tailored the services to help with the patients emotional and medical needs. This case serves as a great example of how the available resources are underutilised in our community.

Conclusion The goal of hospice is to provide a continuum of home, outpatient and homelike inpatient care for the terminally ill patient and their families. It consists of an interdisciplinary team that meets the special needs arising out of the physical, emotional, spiritual, social and economic stresses, which are experienced during the final stages of illness and during dying and bereavement.

The bias against hospice is a major issue that undermines its importance and prevents it from being utilised to its fullest potential. Patient and family education is as important as spreading an awareness amongst the physicians on its availability and indications

82 Complicated recurrent intra-abdominal abscesses in a patient with fabry disease

L Ngo

RD Smalligan

A Islam

Texas Tech Univ HSC Amarillo, Amarillo, TX

Case report A 29 y/o Hispanic male with Fabry disease, HTN, and ESRD on peritoneal dialysis was admitted for C.difficilediarrhoea and peritonitis and severe sepsis. CT of the abdomen showed multiple loculated intraabdominal abscesses. Peritoneal dialysis catheter was removed and patient had IR guided drainage of abscesses. Family history revealed that three cousins had been diagnosed with Fabry disease and that three male members of older generation had died of renal failure before the diagnosis of Fabry disease. Our patient was diagnosed with Fabry disease at age 25 when he was admitted to ICU for acute on chronic renal failure requiring dialysis. During childhood and adolescence, the patient had noticed impaired sweating after exercise, heat and cold intolerance, periods of intense neuropathy in knee and feet, and intermittent purple-red skin rashes on extremities. After patient was diagnosed with Fabry disease, he was on peritoneal dialysis for four years and had experienced two episodes of peritonitis and abdominal abscess. Patient was planned to initiate enzyme replacement therapy (ERT) with fabrazyme once the infections are treated.

Discussion Fabry disease (FD) is a progressive, X-linked inherited disorder of glycosphingolipid metabolism due to deficient or absent lysosomal alpha-galactosidase A activity. This results in accumulation of globotriaosylceramide (Gb3) within lysosomes in a wide variety of cells, thereby leading to the protean manifestation of multiple organs. Renal manifestations occur in approximately 50 percent of affected patients who eventually develop end stage renal disease (ESRD). Patient with Fabry disease are also prone to life threatening infections. Current treatment focuses on replacing the missing or deficient enzyme with recombinant alpha-galactosidase A. Studies have shown that enzyme replacement therapy (ERT) has effectively reduced tissue deposition of Gb3 in the heart, skin, and in most cell types of the kidneys Thus, we strongly advise all patients with strong family history of Fabry disease to have screening, so that enzyme replacement and adjunctive therapy can be initiated early, to prevent further organ damage and to reduce the severity of disease manifestations.

83 A commonly missed cause of hyponatremia

R Pattabhi

F Mathai

TM Reske

L Ali

S Barry

EA Aguilar

LSUHSC-NO, New Orleans, LA

Case report A 75 year old female, with a medical history significant for hypertension presented to the ER complaining of a three day history of bilateral lower extremity muscle cramps, nausea, vomiting, decreased oral intake, loose stools and lethargy.

She had started hydrochlorothiazide (HCTZ) two weeks prior to admission. Physical exam showed lethargy and dry oral mucosa. Lab showed sNa 108 mmol/L, sCl 70 mmol/L, glucose 104 mg/dL, AST 49 u/L and, ALT 44 u/L. Other labs were normal.

Normal saline at 100 ml/hour was started and HCTZ stopped. She was admitted to the ICU with neurochecks. sNa was 111 mmol/L after 4 hours and remained unchanged after 9 hours. One hour later she developed tonic-clonic seizures requiring Lorazepam and intubation for airway protection. Stat labs revealed sNa 113 mmol/L, sCl 84 mmol/L, and glucose 119 mg/dL. Head CT showed no acute changes and EEG did not reveal focal or epileptiform abnormalities. Post seizure her family disclosed her long history of substantial alcohol intake, with the consumption of wine nightly for many years. Unfortunately at the time of admission this information was not known.

Alcohol dependence is an important cause of hyponatremia, which is sometimes missed. This case iterates the fact that an accurate history is essential for proper diagnosis and treatment of hyponatremia. Had alcohol withdrawal been high on the differential, lorazepam would have been started earlier preventing her seizure episode and subsequent intubation. Alcohol use is associated with hypomagnesemia, hypophosphatemia, hypocalcemia, hypokalemia and hyponatremia. 22.8% of chronic alcohol users have been found to be hyponatremic at ER presentation, half of cases were found to be hypovolemic as well.1 According to the National Institute of Alcohol Abuse and Alcoholism, alcohol is the third leading cause of death in USA, it is estimated that 14 million Americans have an alcohol use disorder.2

References

  1. . Addict J. 2015;2015:541–536.

  2. . Clin North AmSeptember 2013;42(3): 593–615.

84 Autoimmune hepatitis associated chronic myelomonocytic leukaemia

A Shredi

H Edriss

SH El Nawaa

Texas Tech Univ Health Science Centre, Lubbock, TX

Case report Chronic myelomonocytic leukaemia is a rare malignant hematopoietic stem cell disorder with clinical and pathological features of both a myeloproliferative neoplasm and myelodysplastic syndrome. It occurs most commonly in older men with a median age at diagnosis of 65 to 75 years. Cytopenia and hepatosplenomegaly are common.

As many as 10% of patients diagnosed with myelodysplastic syndromes including CMML have autoimmune manifestations which range from vasculitis to glomerulonephritis.

A 62 year old man presented with an upper GI bleed. Physical examination included blood pressure 110/60 mmHg, heart rate 94 beats/minute, respiratory rate 23 breaths/minute, and temperature 98.3 F. His abdomen examination revealed a distended abdomen and a nodular liver. Computed tomography of the abdomen revealed liver cirrhosis. The patient denied any history of liver disease, drinking alcohol, using antiplatelet drugs, NSAIDs or anticoagulants. His initial lab tests showed WBC 16,500/µL, Hb 9.6 gm/dL, platelets 92 k/µL, and INR 2. His total bilirubin was 1.4 mg/dL, albumin was 2.6 mg/dL, and AST and ALT were normal. An EGD revealed a non-obstructing Schatzki ring at the gastroesophageal junction and an erythematous gastric mucosa. Biopsies were negative of H. pylori. Tests for Hepatitis B and C were negative. He had a positive smooth muscle antibody; other antibodies, including ANA, anticentromere, CCP, antiglomerular basement membrane, ANCA, antimitochondrial antibodies, were negative. He underwent bone marrow biopsy which showed a hypercellular marrow with monocytosis. His prognosis based on the MD Anderson CMML prognostic calculator was very poor with a median overall survival 10 months. The patient died after 3 months of diagnosis.

This patient likely had autoimmune hepatitis which caused liver cirrhosis and GI bleeding. This case report represents a rare association between autoimmune hepatitis and CMML. There are reported cases of association of other types of leukaemia with autoimmune hepatitis, however, none reported about autoimmune hepatitis associated with CMML. I hope reported case here could raise the awareness of the medical personnel to consider this rare association.

Allergy, immunology, and rheumatology, Joint plenary poster session and reception, 4:30 PM, Thursday, February 22, 2018

85 The sarcoid crisis: neurosarcoidosis causing pituitary-hypothalamic dysfunction

NS Harrison

ME Lindsey

A Subauste

V Majithia

University of Mississippi Medical Centre, Jackson, MS

Case report Sarcoidosis is a granulomatous disease of unknown aetiology that affects the lungs in 90% of cases. Thirty percent develop extra-pulmonary manifestations, and 5% develop neurosarcoidosis (NS). NS most often presents with cranial mononeuropathy; hypothalamic-pituitary abnormalities are rare and have been identified in 2.5% of NS cases.

Case This is a 31-year-old African American female with a long history of systemic sarcoidosis. She initially presented with incontinence and lower extremity weakness, and imaging revealed a lumbar mass. Biopsy of this mass revealed non-caseating granulomas. Despite treatment with IV steroids followed by oral prednsione and azathioprine, the disease progressed. She was noted to have poor compliance. She developed sarcoid in her lymph nodes, maxillary sinuses and GI tract. Therapy was changed to mycophenolate mofetil without success and her disease progressed to the central nervous system. Decision was made to start IV cyclophosphamide, but patient only received one infusion. Four months later she presented to ER with nausea, hypotension, and tachycardia with polyuria of 7 L in 24 hours. Laboratory data revealed hypernatremia, hypokalemia, lactic acidosis, abnormal cortisol, abnormal thyroid function, and low urine osmolality that improved with water deprivation. She was diagnosed with panhypopituitarism and diabetes insipidus and treated with hydrocortisone, desmopressin, and IV cyclophosphamide in the hospital. She was discharged on prednisone, cortef and desmopressin. She completed 6 months of cyclophosphamide. However, the pituitary mass has been unchanged on repeat imaging. She remains on prednisone 40 mg with plans to start Infliximab for further treatment.

Conclusion Less than 1% of intrasellar lesions are associated with hypothalamic pituitary dysfunction. Clinicians must consider neurosarcoidosis when these lesions are noted in a patient with pre-existing sarcoidosis. Glucocorticoids are the initial treatment followed by immunomodulators. These patients have poor prognosis, and most will require long term hormone replacement in addition to sarcoidosis therapy.

86 A case of maffucci syndrome

S Kolagatla

N Moka

S Bailey

ARH-Markey Cancer Centre, Hazard, KY

Case report Maffucci syndrome (MF) is a rare genetic disorder occur as a result of somatic heterozygous mutation of Isocitrate dehydrogenase 1 and 2 (IDH1/IDH2) genes that affects skin and bone. Enchondromas, skeletal deformities and hemangiomas are characteristic of this syndrome. We report a case of MF.

53 year old female presented to the clinic with gradual worsening of left hand swelling for the past several years. Her review of systems is positive for fatigue, musculoskeletal pains of multiple joints and long standing skin lesions. Past history of hemangiomas, enchondromas and chondrosarcoma, multiple surgeries for joints. Her family history is negative for enchondromas. Exam is significant for multiple purplish skin lesions on the extremities and multiple healed scars from remote surgeries.

MF is a rare genetic syndrome so far less than 200 cases have been reported worlwide. Differential diagnosis includes Ollier syndrome but can be distinguished by absence of cutaneous lesions and age of onset. Diagnosis of MF can be made by detailed history, examination and radiological assessment. Pathological assessment of enchondromas will support the diagnosis. Enchondromas must be distinguished from chondrosarcomas as the later possess malignant potential. No association with mental or psychiatric disorders has been reported. Multidisciplinary team management with orthopedician is encouraged in the management of the patients

Abstract 86 Figure 1

Severe deforming joint of hand due to multiple enchondromas

87 A ring-enhancing lesion by any other name

ME Lindsey

NS Harrison

J Bridges

J Blossom

V Majithia

S Kishore

University of Mississippi, Jackson, MS

Case report Systemic lupus erythematosus (SLE) affects the central nervous system in up to 20% of patients and is often termed neuropsychiatric lupus. Neuropsychiatric lupus may present with a wide array of clinical manifestations, and appropriate diagnostic workup is essential. A crucial step in this process is to rule out primary etiologies that neuropsychiatric lupus may mimic. Appropriate brain imaging can play a role in this setting, although unexpected findings may lead to misdirection of the final diagnosis. We present a 27 year old African American male with an eight-year history of SLE who presented to the emergency department with altered mental status. His symptoms had developed insidiously over the course of several days. His underlying SLE had previously been treated with hydroxychloroquine monotherapy. At time of presentation, routine lab findings were fairly unremarkable and computed tomography (CT) of the brain was normal. Lumbar puncture was performed; routine cerebrospinal fluid analysis revealed normal glucose, elevated protein, and slightly elevated opening pressure. Magnetic resonance imaging (MRI) of the brain was obtained; this revealed unusual ring-enhancing lesions reported as possible neurocysticercosis. This led to more extensive infectious disease evaluation which ultimately revealed no evidence of any bacterial, viral, fungal, or parasitic infectious processes. Further autoimmune serology testing revealed a positive anti-ribosomal-P protein. The patient was diagnosed with neuropsychiatric lupus, and he responded rapidly to treatment with high dose intravenous glucocorticoids. Cyclophosphamide was later added to his treatment regimen. The patient has continued to show clinical improvement and repeat brain MRI shows complete resolution of the previously noted ringed lesions. This case highlights the challenges of diagnosing neuropsychiatric lupus and illustrates the importance of viewing all clinical information as part of the whole patient scenario rather than making assumptions based on one abnormal finding.

88 Fixed drug eruption associated to aspirin

RM Medrano

M Orellana-Barrios

TTUHSC, Lubbock, TX

Case report A previously healthy 27-year-old female presented to Outpatient Urgent Care Clinic complaining of dark spots that appeared suddenly on both her feet and face. She had been prescribed Aspirin (650 mg PO every 6 hours as needed) the day prior to presentation as therapy for migraine-type headache. She also then recalled that these spots had appeared suddenly, in exactly the same areas approximately one year before the current episode, also associated with ingestion of an over the counter medication (Alka-Seltzer). Physical examination was unremarkable with the exception of dark, erythematous, slightly oedematous round plaques asymmetrically distributed over feet (figure 1) and left eyelid (figure 2). A complete blood count was ordered (results within normal range) and patient advised to substitute Aspirin with Ibuprofen, which controlled her headache. The skin lesions subsided and disappeared without complications within 2 weeks.

Fixed drug eruption is a cutaneous drug reaction noted to recurs in the same anatomical locations upon recurrent exposure to the offending agent. Lesions usually resolve with no further treatment, but may leave post inflammatory hyperpigmentation. Supportive treatment may include oral H1 antihistamines and short course of steroids in more severe cases. The most common drugs associated with fixed drug eruption are antibacterial agents, aspirin and other non-steroidal anti-inflammatory agents, acetaminophen, and barbiturates.

Abstract 88 Figure 1

Physical examination was unremarkable with the exception of dark, erythematous, slightly oedematous round plaques asymmetrically distributed over feet

Abstract 88 Figure 2

Left eyelid

Cardiovascular, Joint plenary poster session and reception, 4:30 PM, Thursday, February 22, 2018

89 Outcomes of coronary artery occlusion following transcatheter aortic valve replacement

O Akinseye

C Nwagbara

S Jha

UN Ibebuogu

University of Tennessee Health Science Centre, Memphis, TN

Purpose of study Coronary Occlusion (CO) is a rare but serious complication following transcatheter aortic valve replacement (TAVR) with limited published data. We sought to evaluate the immediate and short-term outcomes of CO complicating TAVR.

Methods used Studies, including case reports, case series and original articles published from 2002 to 2016 describing CO following TAVR were identified with a systematic electronic search using the PRISMA Statement. Only studies reporting data on demographic and procedural characteristics, management and follow up outcomes were analysed.

Summary of results A total of 40 publications describing 96 patients (86 native, 10 bioprosthetic) were identified. Mean age was 83±7 years and most (81%) were females. The mean logistic EuroSCORE and STS score was 23.5%±14.6% and 9.1%±3.2% respectively. TAVR access site was transfemoral in 73% and a balloon expandable valve was used in 78%. Among those with LCA occlusion, the mean LCA ostium height was 10.1±1.8 mm while the mean RCA ostium height was 10.4±2.0 mm among those with RCA occlusion. CO frequently involved the left main coronary artery (80%) and the most common mechanism was displacement of native valve leaflet (60%), and most cases occurred within 1 hour post-implantation (88%). Percutaneous coronary intervention was attempted in 82 patients and successful in 89%. Procedural death was 10.4%. CO following TAVR in native aortic valve stenosis was associated with a 30 day mortality rate of 35.3%.

Conclusions CO following TAVR is associated with a high procedural and 30 day mortality rate despite aggressive resuscitative measures including percutaneous coronary intervention.

90 Atrial fibrillation in hospitalised, non-critcally ill elderly patients

M Heckle

M Nayyar

BM Ramos

KT Weber

University of Tennessee Health Science Centre, Memphis, TN

Purpose of study Atrial fibrillation (AF) is the most common sustained tachyarrhythmia. It is associated with increased cardiovascular morbidity, mortality and preventable stroke. It accounts for a third of all hospitalizations for cardiac dysrhythmias. The incidence of AF rises with advancing age. Herein, we reviewed the profiles of elderly hospitalised, non-critically ill patients diagnosed with AF on standard 12 lead electrocardiogram to determine if certain variables were characteristic of these patients.

Methods used A retrospective review of 413 patients with AF at an urban medical centre from November 2015 to July 2017, of which 197 were >65 years of age. The following variables were analysed: sex, electrolytes (potassium, magnesium, calcium), brain natriuretic peptide, serum creatinine, race, body mass index, left ventricular hypertrophy present on electrocardiogram, corrected QT interval and presence of rapid ventricular response (heart rate >100 bpm). Variables were compared with identical features in those <65 years (n=61).

Summary of results In our elderly population there were more males (59.4%) and Caucasians (64.3%) with atrial fibrillation. When compared to the younger cohort, the elderly population had a higher level of brain natriuretic peptide (1171 vs 736 pmol/L p=0.05) and average BMI (30.2 vs 24.9 kg/m2 p=0.02), but less incidence of rapid ventricular response (49.1% vs 61.7% p=0.04). The presence of left ventricular hypertrophy on electrocardiogram was also more common, but not statistically significant in the younger population (19.6% vs 13.3% p=0.09).

Conclusions In our cohort elderly hospitalised patients with atrial fibrillation are more likely to be male Caucasians with a higher level of serum brain natriuretic peptide and BMI when compared to their younger counter parts. Younger hospitalised patients with atrial fibrillation are more likely to have electrocardiographic evidence of left ventricular hypertrophy and have a higher incidence of rapid ventricular response.

91 Heart failure with recovered ejection fraction. a new phenotype or a result of percutaneous coronary revascularisation

I Ifedili

A Pathak

O Akinseye

KT Weber

University of Tennessee Health Science Centre, Memphis, TN

Purpose of study Recent observational studies would suggest the presence of a new heart failure (HF) phenotype, termed heart failure recovered ejection fraction (HFrecEF). However, several reversible negative inotropic stimuli may explain the recovery in EF. We examined the characteristics of patients with ischaemic cardiomyopathy (ICM) who had recovery in EF following percutaneous coronary revascularisation.

Methods used Retrospective review of our cardiology clinic patients with prior ICM and reduced EF (HFrEF) (<40%) who subsequently had HFrecEF (>40%) between March, 2015 and April, 2017. The baseline characteristics, medical history and echocardiogram of these patients were analysed after percutaneous coronary revascularisation on average of 503±320 days.

Summary of results A total of 10 patients with ICM were identified. Mean age ±SEM was 50.2±8.7 years, 60% were females and all patients were African American (AA). Baseline characteristics reveal that 80% had hypertension, 50% had diabetes and 70% had hyperlipidemia. Seventy percent had acute coronary syndrome (ACS) (3 STEMI and 4 non-STEMI) at the time of their initial EF and all had percutaneous coronary revascularisation with most interventions done on the left anterior descending artery (57.1%). Baseline EF was 26.6%±6.4% and which improved to 50.0%±5.9% on average of 503±320 days. Seventy percent and 90% of patients were on aspirin and a statin, respectively, while 100%, 90%, and 60% were on a beta-blocker, angiotensin converting enzyme inhibitor and aldosterone receptor blocker, respectively.

Conclusions A substantial number of our AA patients with ACS and having ICM with HFrEF had HFrecEF following percutaneous coronary revascularisation. This would suggest hibernating myocardium that subsequently improved with revascularisation and optimal medical therapy and would further imply their HF was in remission rather than a new HF phenotype.

92 Atrial fibrillation in patients with rapid ventricular response

M Nayyar

M Heckle

M Agarwal

J Tran

KT Weber

University of Tennessee Health Science Centre, Memphis, TN

Purpose of study Atrial fibrillation (AF) is the most common sustained tachyarrhythmia. The ventricular rate of patients with AF is determined by the conduction properties of the atrioventricular (AV) node. Avoidance of rapid ventricular response (RVR) is important in preventing haemodynamic instability and tachycardia-induced cardiomyopathy. Herein, we reviewed the profiles of atrial fibrillation in patients with and without rapid ventricular response.

Methods used We performed a retrospective review of 239 patients with AF who were seen at an urban medical centre from November 1, 2015 to July 1, 2017. Rapid ventricular response was defined as a heart rate greater than 100 beats per minute. The following variables were analysed: age, sex, electrolytes (potassium, magnesium, calcium), brain natriuretic peptide, serum creatinine, race, body mass index (BMI), left ventricular hypertrophy present on electrocardiogram and corrected QT interval. Variables were compared with features found in those patients whom atrial fibrillation with RVR was not present.

Summary of results Upon review,127 patients were found to have RVR. These patients were younger with an average age of 60.5 (1.35) versus 66.4 (1.5) p<0.01 and had a higher body mass index, with an average BMI of 29.2 (1.77) versus 25.5 (1.29) p=0.09. Electrolytes were similar between the two groups except that serum calcium was significantly lower in patients with RVR, 8.42 (0.07) versus 9.03 (0.07) p<0.01. In addition, patients with RVR had a significantly longer QTc interval, 468.7 m/sec (3.64) versus 453.3 m/sec (3.89) p<0.01 and were more likely to have evidence of left ventricular hypertrophy found in 20.5% versus 9.8% p=0.02.

Conclusions In our study, patients with atrial fibrillation and evidence of rapid ventricular response were younger, had a higher BMI, lower serum calcium, longer QTc and were more likely to have electrocardiographic evidence of left ventricular hypertrophy. The importance of critical illness with a hyperadrenergic state in AF with RVR was not considered herein. BMI is a clinically important and potentially modifiable risk factor to prevent rapid ventricular rates in patients with atrial fibrillation.

93 Withdrawal of cardiotoxins and subsequent recovery in heart failure with reduced ejection fraction

A Pathak

I Ifedili

O Akinseye

KT Weber

University of Tennessee Health Science Centre, Memphis, TN

Purpose of study Heart failure with recovered ejection fraction (HFrecEF) has been considered a distinct HF phenotype. However, HF in remission due to the withdrawal of a cardiotoxin and the subsequent response to optimal medical therapy may explain such a response. Herein, we monitored patients having HFrecEF at our urban medical centre.

Methods used Retrospective review of our cardiology clinic patients with nonischemic cardiomyopathy (<40%) with history of alcohol, cocaine, marijuana and other cardiotoxin use who subsequently had recovered EF (>40%) between March, 2015 and April, 2017. The baseline characteristics, medical history and echocardiogram of these patients were analysed.

Summary of results There were 10 alcohol (7 former and 3 current), 5 marijuana (2 former and 3 current), 4 former cocaine abusers, and a former energy drink consumer. All stopped their abuse of the offending agent within 1 month of HF diagnosis. Mean age ±SEM was 52.1±9.9 years, 64.3% male, 100% African American (AA). Baseline characteristics revealed that 85.7% had hypertension, 28.6% had diabetes, 42.9% had hyperlipidemia, 28.6% had either stroke or transient ischaemic attack and 28.6% had coronary artery disease. Baseline EF was 28.7%±6.8% and improved in all patients to 52.5%±9.6% at 396.5±305.9 days. 100%, 92.9% and 57.1% of patients were on a beta-blocker, angiotensin converting enzyme inhibitor and aldosterone antagonist respectively.

Conclusions In our AA patient cohort with HFrecEF, the improvement in systolic function accompanied cessation of a negative inotropic agent and optimal medical therapy to suggest HF was in remission rather than a new HF phenotype.

94 Mechanical failure of angio-seal device needing surgical extraction

S Pruthi

M Abdelghany

D Chaudhuri

SUNY Upstate Medical University, Syracuse, NY

Case report Vascular closure devices have found their place to achieve homeostasis after percutaneous endovascular procedures, however consequences of failure are not clear. We describe a case of Angio-Seal malfunction with failure of locking system.

Case 65-year-old gentleman with history significant for peripheral vascular disease with iliofemoral-tibial-peroneal bypass and renal artery stenosis with stenting was brought after out of hospital cardiac arrest. Cardiac catheterization was attempted via right radial approach showing heavily calcified, 95% stenosed RCA which could not be crossed. For repeat cardiac catheterization left femoral artery approach was used with ultrasound guided micro-puncture needle and 10 cm Terumo Pinnacle sheath, exchanged to 35 cm 6F sheath. Multiple attempts to cross RCA lesion were unsuccessful.

A 6F VIP Angio-Seal was used as closure device. Insertion sheath and arteriotomy locator were advanced over a guide wire into femoral artery and good pulsatile flow obtained. The locator was removed and compaction tube advanced through the sheath until a click was heard. The compaction tube was pulled back, but second click, indicating tube locking was not obtained. Device was left in place, vascular surgery consulted and device extracted surgically. There was no facture or missing pieces. Analysis of the device by St. Jude Medical Company revealed mechanical failure of the device for unclear reasons.

Discussion Although Vascular closure devices are safe, several complications like pseudo-aneurysm, AV fistula formation, peripheral vascular embolization with lower limb ischemia have been reported. To treat limb ischemia authors recommend direct surgical cut down, retrieval of the device and definitive arterial repair. Though these procedures are limb sparing, they are associated with significant morbidity. We report a relatively rare complication of closure device with failure of deployment of collagen plug and sandwich mechanism which should be kept in mind when faced with such situations.

95 Chest x-ray to predict a tortuous right brachiocephalic artery prior to trans-radial catheterization. a retrospective study

S Salem

D Ardeshna

P Jagadish

S Koshy

RN Khouzam

N Garg

University of Tennessee Health Science Centre, Memphis, TN

Purpose of study Cardiac catheterization is one of the most widely used diagnostic and therapeutic modalities in modern cardiology. In recent years, there has been a paradigm shift towards percutaneous trans-radial artery catheterization as the preferred access site. Its accessibility, lower bleeding risk, lower mortality with ST elevation myocardial infarction, patient comfort, early ambulation and discharge, cost, and reduced post procedural monitoring makes it superior to femoral artery access.

A subset of patients have a tortuous right brachiocephalic (innominate) artery that makes catheter manipulation difficult, which can result in increased procedure time, radiation, patient discomfort, and radial artery spasm. We hypothesised that such patients may be identified by certain measurable parameters pertinent to the shape of their thoracic cage as measured by plain chest x-ray.

Methods used We reviewed chest x-ray films on 56 patients that had undergone radial cardiac catheterization in our lab. We prospectively identified 23 patients with tortuous innominate artery identified by fluoroscopy (cohort group), and 33 patients with no tortuosity (Control group). Chest x-ray films were analysed and several measurements were obtained between cohort and control groups using t-test and P-Value models.

Summary of results Among all measurements, we concluded that vertebrocarinal distance (VCD), identified on chest X-ray as distance from thoracic T1 spinous process to carina, as the most statistically significant, with mean distance of 9.2 cm in cohort group, compared to 11.3 cm in control group (P value of<0.001). Other parameters derived from VCD, including ratio of body height to VCD, area of rectangle formed between VCD and thoracic diameter at carinal level were also statistically significant.

Conclusions Short distance between the spinous process of T1 vertebral body and the inferior edge of carinal bifurcation measured on chest x ray is a strong predictor of tortuous right innominate artery and may be helpful in considering an alternative access site prior to trans-radial catheterization.

96 Hypoparathyroidism associated with hypomagnesemia, hypocalcemia and vitamin d deficiency

M Agarwal

YI Harper

KT Weber

University of Tennessee Health Science Centre, Memphis, TN

Purpose Hypomagnesemia (HypoMg2+) is a frequent disorder associated with gastrointestinal and/or renal losses whose pathophysiologic mechanisms have included vitamin D deficiency and impaired secretion of parathyroid hormone (PTH). Herein, we describe a case of HypoMg2+ with associated hypoparathyroidism-related hypocalcemia and vitamin D deficiency.

Case report This was one of several admissions for this 66 year old African American male with hypertension, alcoholism, diabetes and coronary artery disease who was hospitalised with the chief complaint of weakness and fatigue, but denied diaphoresis, seizures, tremors, vomiting, diarrhoea or urinary symptoms. He also reported experiencing frequent palpitations and dizziness. He was compliant with his outpatient medications: aspirin, atorvastatin, lisinopril, carvedilol, magnesium oxide (800 mg daily), pantaprazole and furosemide (80 mg BID). BP 110/64 mm Hg with regular heart rate of 94 beats/minute. Examination of all systems was noncontributory. Pertinent laboratory findings included: serum K+ 3.1 mEq/L, Mg2+ 0.2 mg/dl, Ca2+ 7.1 mg/dl, albumin 2.8 g/dl, 25-hydroxy-vitamin D 15 ng/ml and PTH 10 pg/ml with hypokalemia, HypoMg2+ and hypocalcemia also seen on previous admissions. EKG revealed sinus tachycardia without arrhythmias. Over the next 48 hours, his symptoms, serum Mg2+ and Ca2+ levels each improved, however, they remained abnormal despite cation supplementation and discontinuation of furosemide. Considering the clinical presentation and laboratory workup, we attributed his hypoparathyroidism as HypoMg2+- induced with hypocalcemia and vitamin- D deficiency. His supplementation was continued and he was begun on oral vitamin D (6000 units/daily) together with abstinence from alcohol, whereby his Mg2+ (2.1 mg/dl) and Ca2+ levels (8.5 g/dl) each further improved and remained normal thereafter.

Conclusion Hypoparathyroidism with hypocalcemia and vitamin D deficiency are under recognised in patients presenting with HypoMg2+ and should be considered in its differential diagnosis. In our African American patient with hypovitaminosis D, diuretic- induced and alcohol-related magnesium wasting led to HypoMg2+ with impaired release of parathyroid hormone and consequent hypoparathyroidism.

97 Partial anomalous pulmonary venous return

MA Alsharif

C Ashangari

D Murray

R Murray

Texas Tech University Health Science Centre Amarillo, Amarillo, TX

Background Partial anomalous pulmonary venous return (PAPVR) is an anatomic variant in which one to three pulmonary vein drains into the right atrium or its tributaries rather than into the left atrium. We present the case of PAPVR from the left upper lobe with drainage to the left brachiocephalic vein.

Case A 47 year old woman with past medical history of factor V Leiden mutation presented with sudden onset left side chest pain radiated to the back. Vitals were normal. Physical exam was unremarkable. EKG showed normal sinus rhythm with no ST changes. TTE showed LVEF of 45%. LA, RV and RA size was normal. RVSP was 30–40 mmHg. CT chest angiogram was negative for PE. PAPVR was incidentally noted involving the left upper lobe with drainage to the left brachiocephalic vein.

Discussion PAPVR is a rare congenital condition which is usually recognised in the paediatric population but may also be diagnosed during adulthood in patients who develop PAH, or in asymptomatic patients undergoing pulmonary vascular studies for other indications. For the treatment, In adult patients, the criteria for surgical repair are less clear cut. Those who have already developed symptoms due to shunting, or have evidence of right-sided volume overload, regardless of the magnitude of the shunt, are also considered for surgery. However, in asymptomatic patients with a low shunt fraction and no clinical or echocardiographic evidence of right heart overload, pulmonary hypertension, or other symptoms, surgery may be unnecessary.

Abstract 97 Figure 1

PAPVR from the left upper lobe with drainage to the left brachiocephalic vein

98 Wearable cardioverter defibrillator (wcd) – a novel treatment option in the prevention of sudden cardiac death

C Ashangari

A Qasim

N Salagundla

B Khandheria

Texas Tech Univ HSC Amarillo, Amarillo, TX

Background Implantable cardioverter defibrillator (ICD) is a life saving device ensuring protection against life threatening ventricular arrhythmias. There are certain situations which do not recommend the implantation of an ICD while the patient can still be at a risk of demise due to a life threatening ventricular arrhythmia. The wearable cardioverter defibrillator (WCD) is a device which comes to the rescue in such situations.

Case report 48 y old, Caucasian, male with significant PMH of HTN, hypothyroidism, COPD, DVT came with the C/O chest pain. EKG, troponins, nuclear stress test, cardiac cath and CTA were negative. Patient’s echocardiogram showed severely depressed left ventricular systolic function with ejection fraction less than 10% and dilated left atrium and left ventricle. Patient also had grade 3 diastolic dysfunction. In the hospital stay, patient went into nonsustained VT, AVNRT, and SVT. He was stabilised and then discharged.

Patient was readmitted after few weeks due to syncopal episodes. During this episode, there were no jerking movements, no urinary or faecal incontinence. BNP was in the range of 2000’s. Chest examination showed bilateral crepitations, there was JVD with positive hepatojugular reflux, S3. Patient was treated for CHF and discharged on WCD.

Patient was readmitted a few days later with chest pain. He noticed that the screen on the WCD showed waves of his heart rate during that episode, but he has not feel the shock. WCD interrogation showed episode of sustained VT, successfully terminated by the WCD. Patient denied any syncopal episode since he started wearing his WCD.

Discussion WCD may be used in patients in the early phase after acute myocardial infarction with poor left ventricular function, after acute coronary revascularisation procedures, reduced left ventricular ejection fraction (≤35%) and in patients with non-ischaemic cardiomyopathy of uncertain aetiology. Also, patient may not be aware of the shock delivered by the WCD as by the time shock was delivered, patient may pass out. The WCD may also replace ICD implantation in patients waiting for heart transplantation or who need a ventricular-assist device. However, patient compliance is essential for the effective use of this device.

99 One insane murmur

A Thibodeaux

MR McMullan

WF Campbell

University of Mississippi Medical Centre, Madison, MS

Introduction Atrioventricular septal defects (AVSD) are a group of congenital cardiac defects involving the atrial and ventricular septum and the AV valves. This diagnosis is usually made at a young age and has a strong association with Down syndrome. Some variations may be largely asymptomatic and not diagnosed until adulthood. Here we report a case of a non-syndromic, 20 yo female with a transitional AVSD diagnosed after a referral for a murmur evaluation.

Case description A 20 yo white female was referred to our clinic after an ENT physician heard a murmur on her preop exam. She was sent to her PCP and then set up for an echocardiogram. This showed a primum atrial septal defect (ASD) with severe tricuspid regurgitation. The patient stated that she played sports and did some cheerleading with no limitations in high school. She exercises regularly and had recently completed 8 weeks of ‘Insanity’ workout with no problem. She has noticed easy fatigability for the past 6 months. She also had a trip to Tennessee where she had difficulty completing a 6 mile hike in the mountains. Her physical exam revealed a fixed, split second heart sound, a 4/6 systolic murmur at left lower sternal border with a right ventricular heave. Electrocardiogram showed normal sinus rhythm with a right bundle branch block and left axis deviation. A cardiac MRI revealed she had a transitional AVSD with 2 separate AV valves (cleft mitral valve) and a small ventricular septal defect (VSD). Right heart catheterization hemodynamics showed her mean pulmonary pressure to be 24 mmHg, with a pulmonary vascular resistance of 0.4 Wood units and QP:QS ratio of 3.7:1. She was referred for surgical correction. She underwent patch repair of her ASD and VSD with cleft mitral valve repair. She had an uneventful recovery and reports increased exercise tolerance on follow up.

Discussion This case illustrates two key points in patients with AVSDs. First, AVSDs are not found solely in Down syndrome patients, although there is a strong association. Secondly, partial and transitional AVSDs can be well tolerated and not diagnosed until adulthood. They may present with a murmur, heart failure or atrial fibrillation. Primary complete repair is the preferred surgical approach, with overall low morbidity and mortality. It is important to remember these facts when diagnosing and treating patients with AV septal defects.

100 Dialysis: treatment for complete heart block

K Gada

L Perera

M Hess

SUNY Upstate Medical University, Syracuse, NY

Case report A 58-year-old female with history of HTN, ESRD, seizure disorder presented after a seizure like episode. She was found to have heart rate<20, was given 0.5 mg atropine with no significant response. She was started on transcutaneous pacing and glucagon was administered for potential beta blocker toxicity with no improvement. Basic lab work showed Na 136, K 6.6, Cl 93, Cr 5.3, BUN 46. EKG showed complete heart block with wide QRS. She was given calcium gluconate and insulin drip. Thereafter urgent hemodialysis was initiated with return to sinus rhythm.

Hyperkalemia leads to cardiac rhythm disturbances by altering the resting membrane potential of the cell which depends on the ratio of intracellular to extracellular potassium. In the myocardium, hyperkalemia depresses electrical conduction velocity but increases the rate of repolarization.

Our case highlights the fact that clinicians should be aware of heart block as a potential manifestation of hyperkalemia and it should be high on our list of differentials if a new onset complete heart block is seen in ESRD patients. Our case also highlights the fact that though transcutaneous pacing is usually helpful for hemodynamically bradycardia, in hyperkalemia given the underlying changes in cell excitability it is not helpful and in such patients, dialysis should be undertaken without delay to prevent adverse outcomes.

101 Diagnostic value of selective angiography approach in a female patient with chest pain

M Khalid

G Murtaza

D Hidalgo

M Kanaa

T Paul

East Tennessee State University, Johnson City, TN

Case report 62-year-old female with past medical history of hypertension was admitted with chest pain, nausea, epigastric pain and shortness of breath. Physical exam was significant for epigastric tenderness. Labs were unremarkable. Troponins were negative and EKG was normal. She had normal stress test in 2013. Computed Tomography Angiography showed severe atherosclerotic plaque with 70%–99% percent stenosis in a diagonal branch of Left Anterior Descending Artery (figure 1) and total coronary artery calcium score was 176. she had a Left heart catheterization that revealed Non obstructive mild proximal LAD 30%–40% disease and 20%–30% diagonal stenosis.

CCTA has high diagnostic accuracy for detection of obstructive coronary artery disease. Recent Studies compare Selective angiography approache; CCTA followed by invasive angiography Vs Direct invasive angiography approach showed no difference in major adverse cardiac events and has a lower cardiovascular cost with selective approach. The only disadvantage of CCTA is higher radiation dose exposure. Factors that can influence the diagnostic accuracy of CCTA include gender, age, duration of symptoms, atypical presentation and coronary artery calcification. Young female patients with atypical presentations of chest pain might have less diagnostic accuracy just like in our case leading to overestimation of coronary artery disease on CCTA.

Abstract 101 Figure 1

CCTA showed diagonal stenosis (blue arrow)

102 Iatrogenic cavernous sinus air embolism during peripheral venous catheter insertion

M Khalid

ES Josan

P Sankhyan

G Hoskere

East Tennessee State University, Johnson City, TN

Case report 86 year old male with advanced dementia admitted with worsening confusion for 2 weeks. Physical exam was significant for disoriented to place and time (baseline) but no neurological deficit. Labs and CXR were normal. Computed tomography (CT) of the head without contrast showed air in left cavernous sinus and right transverse foramen on C1 which tracked down into venous circulation of neck. He didn’t have any central line placement, however it was later discovered that he had a peripheral catheter insertion with a poorly primed line for saline administration and was the most likely cause of air embolization. He was kept in Trendelenburg position and hyperbaric therapy was indicated, but deferred due to patient non-compliance in the setting of advanced dementia. Serial follow up neurological exam showed no deficits. Repeat CT head later showed resolution of air embolism. Echocardiography with bubble study showed normal left ventricular function.

Cavernous sinus air embolism is associated with infection, penetrating trauma or complication of vascular interventions. The most common cause is peripheral or central venous catheter lines insertion. Air embolism should be consider one of the differentials if the patient has acute change in neurological function especially during venous cannulation. Diagnosis usually made by CT scan of the brain and treatment is 100% oxygen.

Abstract 102 Figure 1

Computed tomography (CT) of head without contrast revealed cerebral atrophy, air in left cavernous sinus, right transverse foramen on C1 (A) which tracked down into venous circulation of neck (B) but no traumatic injury

103 Brachial artery pseudoaneurysm: a rare complication of intravenous drug abuse

V Kohli

M Ibrahim

D Ginn

East Tennessee State University, Johnson City, TN

Introduction Trauma is the leading cause of vascular injuries in the general population. However, this case reports a rare complication of intravenous drug use (IVDU) that resulted in the development of a pseudoaneurysm (PSA) of the brachial artery (BA). Very few cases of BA PSA have been described in literature.1 To the best of our knowledge, none of these cases occurred in relation to to IVDU. In addition, the PSA’s in most the of the previously cases were not infected, while ours was.

Case presentation An 18-year-old female patient with extensive IVDU history presented to the emergency department (ED) complaining of pain, bluish discoloration, and swelling of her right (Rt.) upper extremity for 4 days. She was seen in ED 2 days prior to that for the same complaint, was not septic, had a negative roentgenogram of the Rt elbow, was prescribed oral antibiotics, and sent home. However, A computerised tomography (CT) scan of the Rt. upper extremity on her returning to the ED showed a 1.8-centimetre (cm) PSA of the BA with a surrounding 3.6 cm fluid collection, suspected to be a hematoma, abscess or injected material. Despite intravenous antibiotics for her Methicillin-resistant Staphylococcus aureus (MRSA) bacteremia, cutaneous thromboembolic complications with erosions of multiple Rt fingertips could not be avoided. Her heart valves remained intact though. The swelling and pain continued to worsen.Therefore, a magnetic resonance imaging (MRI) with contrast was ordered., It showed a significant vascular compromise at the medial aspect of her forearm where the PSA obstructed the ulnar artery. The radial artery lumen was patent and so it maintained the circulation in her forearm and hand. Considering this finding, the vascular surgery service was consulted. When her repeat blood cultures proved sterile, the BA PSA was resected and grafted with a vein from her lower extremities. The patient circulation, swelling, pain, and discoloration improved afterwards with continuation of the intravenous antibiotics.

Summary Penetrating, blunt, and hemodialysis are the leading causes of arterial pseudoaneurysms and other malformations. However, given the rarity of IVDA association with this complication, a case of BA PSA was reported here.

Reference

  1. . Tex Heart Inst J2003;30(4):293–297.

104 Ventral septal defects & ventricular arrhythmias: not so benign

K Donovan

M Nayyar

D Ardeshna

S Alsafwah

University of Tennessee Health Science Centre, Memphis, TN

Case report Ventricular septal defects (VSD) are the most common congenital heart defect and is seen in approximately 38 infants per 1000 live births. Although relatively uneventful in the first 2 decades, arrhythmias become prevalent in the 3rd decade of life.

We present the case of a 52 year-old male who presented to the clinic with a one month history of palpitations and diaphoresis. Physical exam was significant only for a grade 2/6 holosystolic murmur best heard at the right lower sternal border. Basic laboratory work up, including electrolytes and TSH were normal. ECG revealed sinus rhythm with incomplete right bundle branch block. TTE showed trace mitral and tricuspid regurgitation without evidence of elevated right heart pressures.

The patient noted palpaitations and 19 beats of polymorphic non-sustained ventricular tachycardia were captured by Holter monitor. A cardiac catheterization revealed normal coronary arteries and evidence of possible VSD by left ventriculogram.

A CT heart with contrast identified multiple VSDs. There was a 3–4 mm ventriculospetal defect at the base of the heart inferiorly, slightly anterior to the coronary sinus (figure 1A). Adjacent to this VSD, slightly more apical was a second contrast filled channel with possible right ventricle conduit (figure 1B). Also a third VSD (3.2 mm) between the left ventricle and the pulmonary outflow tract (figure 1C).

A study in UK of adults with small VSDs noted a 2% incidence of ventricular arrhythmias and symptomatic arrhythmias in 8.5% of patients. Although this patient‘s VSD was classified as small, he is at an increased risk for malignant arrhythmias. There are limited studies investigating serious arrhythmias in patients with VSDs and no studies including patients with VSDs. We conclude that it is prudent to exclude inducible arrhythmias in patients with small VSDs given their underlying diseased conduction system, increased prevalence, and increased risk of sudden cardiac death when presenting with lifestyle limiting symptoms.

Abstract 104 Figure 1

(A) There was a 3–4 mm ventriculospetal defect at the base of the heart inferiorly, slightly anterior to the coronary sinus, (B) Adjacent to this VSD, slightly more apical was a second contrast filled channel with possible right ventricle conduit, and (C) Also a third VSD (3.2 mm) between the left ventricle and the pulmonary outflow tract

105 Cardiac sarcoidosis: a lethal arrhytmia presentation

JR Rivas

KR Green

J Shah

F Rollini

University of Florida, Jacksonville, FL

Case report A 50-year-old male with history of stage four sarcoidosis, non-ischaemic cardiomyopathy with non MRI compatible implantable cardioverter-defibrillator (ICD) for secondary prevention who presented to our institution after a spontaneous ICD discharge. Interrogation of his device revealed appropriate ICD discharge for a sustained monomorphic ventricular tachycardia that failed anti-tachycardia pacing. Left heart catheterization was performed to evaluate for possible ischaemic precipitant of his arrhythmia, however, revealed normal coronary arteries. He was then started on anti-arrhythmic control with Sotalol. A PET scan was performed which showed mild to moderate increased uptake within the anterior and anteroseptal wall of the left ventricular myocardium suspicious for active inflammatory from cardiac sarcoidosis. Thus he was treated with prednisone. No other episodes of monomorphic ventricular tachycardia were observed. He was then discharged with cardiology follow-up.

Discussion This case supports that sarcoidosis with cardiac involvement is an important entity that leads to life-threatening disorders. It is found in at least 5% of patients that could be asymptomatic or present with heart block, congestive heart failure, lethal arrhythmias or sudden death. Cardiac involvement may occur at any point during the course of sarcoidosis and may occur in the absence of pulmonary or systemic involvement. Conduction disturbances and arrhythmias are the most common cardiac manifestations and reflect granulomatous infiltration within the conduction system or ventricular walls. Noncaseating granulomas in the ventricular myocardium is a potential focus for abnormal automaticity, increasing the risk for reentrant tachyarrhythmias such as ventricular tachycardia. Prognosis of cardiac sarcoidosis can account for upto 65% deaths from it.

Conclusion Cardiac sarcoidosis may occur alone or alongside systemic sarcoidosis but could be frequently clinically silent. Due to initial non-specific findings, the diagnosis can be challenging, frequently missed or delayed. Echocardiography, MRI, PET or Nuclear scan and Endomyocardial biopsy are some of the available modalities for diagnosis.Early recognition and diagnosis is imperative, especially in symptomatic patients due to the high risk of lethal complications.

106 Tetralogy of fallot and anomalous origin of the left pulmonary artery from the ascending aorta in a newborn patient with digeorge syndrome

C Schmitt1

G Pridjian1

A Dunbar1

J Caspi2

S Yang1

1Tulane University School of Medicine, New Orleans, LA

2LSUHSC School of Medicine, New Orleans, LA

Case report Anomalous origin of the left pulmonary artery (AOPA) is a rare cardiac defect that is more commonly associated with conotruncal heart defect and 22q11 deletion syndrome. The natural history of AOPA without surgical correction is the onset of congestive heart failure, pulmonary hypertension and severe pulmonary vascular obstructive disease, that has a 30% 1 year survival rate. This case reports a newborn with a prenatal diagnosis of Tetralogy of Fallot and DiGeorge Syndrome found to have an anomalous origin of the left pulmonary artery from the ascending aorta. The cardiac anatomy was confirmed by 3-D CT angiogram before the newborn underwent successful complete surgical repair. This case highlights the importance of a genetic testing for 22q11 deletions in fetuses diagnosed with conotruncal cardiac malformations, and the significance on recognition of AOPA in postnatal echocardiogram for newborns diagnosed with Tetralogy of Fallot.

Abstract 106 Figure 1

Computed tomographic angiography of the chest demonstrating the left pulmonary artery [LPA] originating from the ascending aorta [AO]

107 Collision in my heart

EK Swe

MN Alexander

ME Hall

University of Mississippi Medical Centre, Jackson, MS

Introduction The differential diagnoses of right atrial (RA) masses are normal variants, primary cardiac tumours, secondary tumours and thrombi. Collision tumour of hepatocellular carcinoma (HCC) and cholangiocarcinoma is very rare, and to our knowledge cardiac involvement has not been reported. Therefore, one needs to be aware of the possible direct extension of hepato-biliary carcinoma while evaluating an RA mass.

Case A 58-year-old white man with coronary artery disease status post CABG 8 months prior, diabetes mellitus and hypertension presented to his cardiologist’s office with extreme fatigue, dyspnea and weight loss for 6 weeks. Physical examination was remarkable for only mild hypotension and pallor. In-office echocardiogram showed normal left ventricular systolic function with a new right atrial mass, measuring 4.2 cm ×2.1 cm extended from the inferior vena cava, partially prolapsing into the right ventricle in diastole. Admission laboratory findings revealed acute renal failure (estimated glomerular filtration rate was 15) and abnormal liver enzyme levels. Chest X-ray showed a new 1.6 cm nodular density at left lung base. Venous Doppler showed venous thrombosis in the lower extremities. CT scan of chest, abdomen and pelvis as malignancy workup revealed a large infiltrating right liver mass. Labs were positive for markedly elevated alpha-fetoprotein but negative for hepatitis panel. Two-phase MRI showed a large (17 cm) necrotic mass with features consistent with a collision tumour of HCC and cholangiocarcinoma, extending via IVC cranially into the right atrium and caudally into the renal veins. After thorough discussions regarding the extensive and aggressive nature of the disease, treatment options were limited. The patient and family opted for home hospice. He was discharged home and passed away 3 days posts discharge.

Discussion A unique case of a collision tumour of HCC and cholangiocarcinoma extending into the inferior vena cava and right heart had an aggressive clinical course. Collision tumour of HCC and cholangiocarcinoma is extremely rare with few symptoms until alerted by metastasis. It is even more unusual for such tumour to have cardiac involvement. The different diagnosis of RA mass should include not only the common aetiology, but also the collision tumour of HCC and cholangiocarcinoma.

108 Giant coronary aneurysms in a patient with noonan syndrome

PR Thurmond

EM Khansur

WF Campbell

ME Hall

C Richards

G Aru

MR McMullan

University of Mississippi Medical Centre, Jackson, MS

Case report Coronary artery aneurysm is defined as a localised luminal dilation of at least 1.3 to 2 times the diameter of a normal adjacent reference segment. Giant coronary artery aneurysms (GCAA) are generally defined as dilation of the artery greater than 4 times the reference diameter. GCAAs are very rare, with an estimated prevalence of 0.02%–0.2%.

We present a 35-year-old black man with a history of Noonan syndrome variant and mental retardation, who was referred for surgical evaluation of ascending aortic root dilation and left anterior descending (LAD) GCAA. During initial evaluation for Noonan syndrome in childhood, an echocardiogram was performed revealing the presence of aortic root dilation. Due to progression of the dilation on echocardiogram in 2010, a chest CT was obtained which noted an aneurysm of the LAD. While not formally diagnosed, review of old records revealed symptoms consistent with Kawasaki disease as an infant. In 2016, CT angiography demonstrated diffuse coronary ectasia with a GCAA in the proximal LAD measuring over 50 mm with a dilated aortic root measuring 57 mm. There was also noted mild fusiform aneurysmal dilation of the left branch pulmonary artery measuring 30 mm in maximal diameter. Repeat echocardiogram showed moderate concentric left ventricular hypertrophy consistent with cardiac findings in Noonan syndrome. Prior to surgery, a coronary angiogram revealed stenosis distal to the aneurysm. In May 2016 he underwent valve sparing root placement, aortic valvuloplasty, septal myomectomy, and coronary artery bypass graft (CABG) with left internal mammary artery to LAD without ligation of the GCAA. Current post-operative management includes beta-blocker, anticoagulation, and aspirin.

GCAAs are rare occurrences most often attributed to atherosclerosis, vasculitis including Kawasaki disease, and connective tissue disease. Clinical sequelae include thrombus formation, distal embolization, fistula formation, and rupture. Surgical correction is generally the preferred treatment including CABG with or without resection or ligation of the aneurysm. In the absence of ligation of the GCAA, the decision was made to initiate anticoagulation to reduce the risk of thrombus embolization.

109 Wellen’s sign in an atypical presentation of stroke

S Werner

LS Engel

LSU Health Sciences Centre, New Orleans, LA

Introduction Wellen’s sign is an ominous EKG finding, suggestive of significant stenosis of the left anterior descending (LAD) artery, with a high risk of progression to myocardial infarction if untreated.

Case A 73 year-old man without reported medical history presented to the Emergency Department (ED) after a losing consciousness while running on his treadmill. The patient was performing his morning exercise, and without warning or prodromal symptoms, had spontaneous loss of consciousness. His wife found him on the floor and activated EMS. The patient was reportedly altered until arrival to the ED, where a Code Stroke was activated. Initial computed tomographic scan of the head was unremarkable but MRI showed an acute right frontal lobe CVA. Initial troponin was 0.94 and EKG did not suggest changes associated with ischemia. The patient was admitted to the ICU for stroke workup and monitoring. On exam, he did not have any neurologic deficits or chest pain. Troponins’ trended to 9.01 and EKG showed dynamic changes, which included deep inverted T-waves in V2-V3, and V4-V6. Echocardiogram showed apical dyskinesis, without left ventricular thrombus. Cardiology was consulted, and angiography showed a 95% stenosis in the proximal Left Anterior Descending Artery (LAD), which was opened with a single drug eluting stent. The patient was discharged on dual antiplatelet therapy, ACE inhibitor and a statin. Apixaban was also prescribed due to his apical akinesis and new onset stroke. Continuation of apixiban would be re-evaluated after a repeat echocardiogram could be performed as an outpatient.

Discussion This patient had an atypical presentation of stroke and myocardial infarction. A Wellen’s pattern was identified and stenosis in the proximal LAD was diagnosed with angiography. The characteristic biphasic T-wave or deeply inverted T-wave seen in leads V2-V3 are a result of myocardial reperfusion, and should alert clinicians to the likelihood of obstructive coronary disease.

Endocrinology and metabolism, Joint plenary poster session and reception, 4:30 PM, Thursday, February 22, 2018

110 Functions of long non-coding rna in trimethylamine n-oxide production

MA Al-Obaide

T Vasylyeva

Texas Tech University Health Science Centre, Amarillo, TX

Purpose of study We recently provided evidence of the elevated levels of trimethylamine N-Oxide (TMAO) in type 2 diabetes mellitus (T2DM) and chronic kidney disease (CKD) patients, which strongly links to cardiovascular diseases [Al-Obaide et al., 2017]. Five FMO genes are differentially expressed in liver, kidney, and other tissues and are involved in the production of TMAO that promotes vascular inflammation through NF-kB signalling. Long non-coding RNAs (lncRNAs), 200 nucleotides to many kilobases in length, are found to have critical functions in the tissue-specific regulation of gene expression and considered therapeutic targets. The objective of this study was to investigate unexplored regulatory functions of lncRNAs LOC105371611 in the expression of FMO genes.

Methods used NCBI-Gene/Nucleotide, UCSC Genome Browser, Ensembl, were used to search for the genomic setting of FMO and lncRNAs genes. The locations of the identified sequences were verified and updated to hg38 version of human genome sequence by the BLAT tool. RNA expression of FMO genes and LOC105371611 (lncRNA) were extracted from the data in the NCBI-Gene/HPA RNA-seq normal tissues. Identification of mature sequences (miR) of lncRNA transcript was performed by using the miRBase BLASTN search tool. The miRNA recognition element (MRE) of FMO transcripts, were analysed and identified by the RNA22 v2 tool.

Summary of results To date, there are no reports on the regulatory functions of lncRNAs in FMO expression. The FMO and lncRNA LOC105371611 mRNAs showed differential expression in the kidney and liver, which indicated the tissue-specific pattern. Our analysis showed the lncRNA LOC105371611 locus mapped contiguous to FMO1 and FMO3 and hosted FMO2 produce miRNA mature sequences (miR) could target the MRE of FMO transcripts and consequently, downregulate the TMAO production.

Conclusions This study provided insight into the functions of lncRNA LOC105371611 to downregulate FMO genes’ expressions at transcriptional and posttranscriptional levels in the kidney and liver and could be exploited in targeted therapy of T2DM-CKD.

111 Short-term efficacy and safety profiles of intravenous continuous glucagon infusion for the management of refractory neonatal hypoglycemia

J Bhat

A Kaulfers

M Zayek

R Bhat

F Eyal

USA, Mobile, Alabama, Mobile, AL

Purpose of study Intravenous continuous glucagon infusion is one of the treatment options for persistent neonatal hypoglycemia despite high glucose infusion rates. However, in the literature, limited published data exist on its short-term safety and efficacy. Hence, our objective was to evaluate the efficacy and safety profiles of continuous glucagon infusion for treating refractory hypoglycemia in moderately preterm and term neonates.

Methods used In this retrospective, single-centre study, neonates born at ≥30 weeks of gestational age from 2012 through 2014, treated with intravenous continuous glucagon infusion for refractory hypoglycemia (blood glucose levels persistently <47 mg/dl despite high glucose infusion rates) were included. The lowest blood glucose, serum sodium, and serum bicarbonate values twelve hours before and up to 24 hours after the initiation and stoppage of glucagon infusion were collected and compared.

Summary of results Totally, 26 neonates with mean (±SD) gestational age (weeks) of 36 (±2) and birth weights (g) of 2990 (±868) were included in the study. Among the included infants, 13 (50%) were preterm, and 18 (69%) were infants of diabetic mothers. During the first 24 hours of glucagon treatment, lowest blood glucose levels increased from a mean (±SD) of 26 (±10) mg/dl to 61 (±13) mg/dl (p<0.01). The rates of hyponatremia, thrombocytopenia and metabolic acidosis were comparable before and during the periods of glucagon infusion (table 1).

Conclusions In this study, intravenous continuous glucagon infusion was efficacious in improving blood glucose levels in neonates with refractory hypoglycemia without causing short-term adverse events.

Abstract 111 Table 1

Short-term efficacy and safety profiles of intravenous continuous glucagon infusion in 26 neonates

112 A rare case of emphysematous urinary tract infection

JA Carlson

J Verdecia

C Horn

J Ruiz

R Javb

University of Florida, Jacksonville, FL

Case report A 41 year old female with history of diabetes mellitus (haemoglobin A1c 12.4%) with neuropathy, gastroparesis, and neurogenic bladder presented with complaint of abdominal pain and intractable vomiting. On physical exam, the patient was afebrile, with tenderness at the left costovertebral angle and suprapubic area. Workup showed serum white blood cells (WBCs) of 15 125. Urinalysis had 180+WBCs, 500+glucose, and nitrites. Abdominal CT showed gas in the urinary bladder and urine culture grew Klebsiella pneumonaie. A foley catheter was placed and she was treated with 2 weeks of antibiotics.

Emphysematous UTIs (EUTIs) are infections of the lower or upper urinary tract associated with gas formation. Air seen on imaging along the urinary tract along with nitrites on urinalysis is essentially pathognomonic for EUTI diagnosis. Although extremely rare (less than 135 reported cases in English literature before 2006) they are becoming more prevalent in the United States. This is due to 2/3 of cases involving diabetics, and in the U.S. the number of diabetics increased 4 fold from 1980 to 2014, totaling over 20 million. EUTI pathogenesis is poorly understood, but it is believed increased glucose in diabetic urine provides nutrients for glucose fermenting bacteria to thrive.

Emphysematous cystitis can usually be treated with medical therapy, 90% requiring IV antibiotics until susceptibilities are returned. Bladder irrigation may be needed if blood clots are present, and catheter placement is often required in order to rest the bladder and prevent bladder tamponade. If there is still no improvement, surgery may be necessary.

The typical EUTI patient is a female (2:1 female to male ratio), over the age of 60, with a hgbA1c of 10% or higher. These patients can present asymptomatically or in florid sepsis, but most often with abdominal pain. In over 80% of cases Eschericia coli or Klebsiella are the identified culprits, however, fungal and more ominous bacterial strains (pseudomonas, staphylococcus) have been reported. It is critical hospitalists are able to recognise EUTIs as even with proper antibiotic treatment, EUTIs have a mortality rate of 7%. If the diagnosis is missed, a patient can progress to emphysematous pyelonephritis where mortality approaches 40%.

113 Does pkc mediate the high risk of preeclampsia in pregnant women with diabetes?

RP Chow1,2

J Zhao2

TM Curtis2

T Lyons1

J Yu1

1Medical University of South Carolina, Charleston, SC

2Queen’s University Belfast, Belfast, UK

Purpose of study Preeclampsia (PE) is a leading cause of pregnancy-related mortality and morbidity, and its prevalence is 4-fold higher in women with diabetes vs those without, but the underlying mechanism is unclear. The anti-angiogenic factor soluble fms-like tyrosine kinase-1 (sFlt1) plays an important role in the pathogenesis of PE. Evidence suggests that PKC activation, which is associated with the development of diabetic vascular complications, may mediate the enhanced sFlt1 release in non-trophoblast cell types; but it is unclear whether this mechanism occurs in the placenta and thus mediate the high risk of PE in diabetes. We aimed to determine:

  • the role of PKC in the regulation of sFlt1 expression in a human placental trophoblast cell line

  • whether diabetes–relevant conditions promote sFlt1 expression via the PKC pathway.

Methods used Quiescent human trophoblast HTR-8/SVneo cells were treated with the PKC activator phorbol-12-myristate-13-acetate (PMA), or the diabetes stimuli ‘heavily oxidised, glycated’ low-density lipoproteins (HOG-LDL) vs native LDL ±glucose, over 24 hours, with or without PKC inhibitor GF109203X. Both sFlt1 mRNA expression (RT-PCR) and protein release (ELISA) were measured.

Summary of results We found that 5 nM PMA increased sFlt1 mRNA expression (p≤0.001 for sFlte15a) and protein release (p≤0.001) in HTR-8/SVneo cells; this effect was abrogated by the pre-treatment of 5 uM GF109203X (p≤0.001 for sFlte15a expression, p≤0.001 for protein). Similarly, 50 µg/ml HOG-LDL increased sFlte15a expression (p≤0.001) and protein release (p≤0.001), which were attenuated by GF109203X. However, glucose did not appear enhance the effect of HOG-LDL.

Conclusions The results are consistent with the possibility that modified lipoproteins may promote PE development in women with diabetes, likely via a PKC-mediated up-regulation and release of sFlt1. These findings provide new insights into the disease mechanism, and potential targets for developing preventative and interventional measures for PE.

114 A case of cushing syndrome

K Ellard

B Hartman

LS Engel

T deSilva

LSU Health Sciences Centre, New Orleans, LA

Case report Cushing’s syndrome is characterised by over-secretion of cortisol. Three main subtypes of Cushing’s syndrome exist:

  • Cushing’s disease,

  • ACTH dependent release of glucocorticoid (ectopic ACTH syndrome),

  • Adrenal overproduction of cortisol such as seen in an adrenal adenoma or carcinoma.

A 28 year old woman presented to Endocrine clinic for evaluation of secondary amenorrhea of 2 years duration. The patient did not bleed in response to a progesterone challenge and was subsequently referred to Endocrine clinic. She was anxious and tearful. She denied any recent acne, excess hair growth, or voice deepening. She had not experienced galactorrhea or problems with peripheral vision. She noted intermittent tension headaches, fatigue, polydipsia, polyuria, easy bruising, and weight gain distributed primarily on her face, abdomen, and the back of her neck. At presentation, her BMI was 21.7 kg/m2 and physical exam demonstrated a round face with increased facial plethora, and an increased posterior fat pad. Significant labs included DHEA sulfate 15 mcg/dL (18–391 mcg/dL), FSH 6.3 mlU/mL, estradiol <15 pg/mL for a non-premenopausal woman (19–357 pg/mL), total testosterone 3 ng/dL (2–45 ng/dL), prolactin 25.9 ng/dL (3.0–30 ng/dL), ACTH <5 pg/mL (6–50 pg/mL), and AM cortisol of 22.9 mcg/dL (<2.0 mcg/dL), following administration of 1 mg of dexamethasone the previous evening, Repeat low dose dexamethasone suppression test was significant for an AM cortisol 20.7 mcg/dL (4–22 mcg/dL) and dexamethasone 258 ng/dL. CT Abdomen revealed a left side adrenal mass. Left adrenalectomy was performed and pathological was consistent with an adrenal cortical adenoma. Her post-operative midnight salivary cortisol levels were appropriately low. Her menstrual cycles gradually returned, facial plethora and sites of abnormal fat deposition resolved and she reported a significant improvement in her psychological well-being

Menstrual irregularities are very common in women with Cushing’s syndrome. Curative treatment for an adrenal source of Cushing’s syndrome is unilateral adrenalectomy. Post-operatively, patients may experience transient adrenal insufficiency due to contralateral suppression of the other adrenal gland. Post-operatively, resolution of symptoms of Cushing’s syndrome usually occurs over a period of several months.

115 Triple threat after craniopharyngioma resection requires close attention

HV Gates1

N Arshad1

N Tangutur2

R Smalligan1

1UAB, Huntsville, AL

2Huntsville Hospital, Huntsville, AL

Case report A 71 yo AAF with a h/o HTN and hyperlipidemia underwent trans-sphenoidal surgery for craniopharyngioma. On post-op day (POD) 2, she developed polydipsia and polyuria requiring DDAVP for early diabetes insipidus (DI). She went home on POD 5 with a Na of 146. POD 7 she had nausea and vomiting that worsened and was accompanied by weakness, disorientation, and an inability to keep food down. She was brought to the ER on POD 10. Home meds: aspirin, losartan, gabapentin, baclofen and diltiazem. PE: disoriented woman with a normal exam except for nonfocal, generalised weakness and decreased vision in her right visual field. Labs: Na 107 and otherwise WNL. The patient was admitted to the ICU and started on 3% saline with q2 hr monitoring of Na. Her Na and symptoms slowly improved.

Discussion Trans-sphenoidal surgery is commonly needed for symptomatic pituitary adenomas but is also required for craniopharygioma rx. Damage to the hypothalamus during surgery (or trauma) can result in a triphasic response in a minority of patients. First, the polyuric phase results from inhibition of ADH release due to hypothalamic dysfunction. It starts within 24 hour of surgery and can last 5 days. Second, the antidiuretic phase results from stored hormone being released from remnants of the posterior pituitary. This phase can last 5–7 more days. Excessive water intake during this phase can lead to severe hyponatremia due to transient ADH secretion. Third, the permanent DI phase occurs due to complete depletion of ADH from the posterior pituitary. Less than 20% of patients experience all three phases.

Each phase requires a unique treatment approach. IV desmopressin or dilute arginine ADH is used for the polyuric phase. The antidiuretic phase is treated like SIADH: stopping IVF and restricting PO fluids. If severe hyponatremia continues or the patient is symptomatic, then 3% saline may be used. Patients who enter the third phase of permanent central DI require regular desmopressin. Treatment of these patients is delicate and challenging because of variation in patient presentation and treatment responses, and few studies help guide management. However, treatment should include a multidisciplinary team approach.

116 Implementing the american academy of paediatrics recommendations on screening preterm infants at risk for metabolic bone disease

O Ginnard1

S Jain1

A Nella1

CL Blanco2

K Bonagurio2

A Moreira2

AC Calabria3

1University of Texas Medical Branch, Galveston, TX

2University of Texas Health Science Centre of San Antonio, San Antonio, TX

3Children’s Hospital of Philadelphia, Philadelphia, PA

Purpose of study Metabolic bone disease of prematurity (MBD) remains a common comorbidity in ELBW neonates. In 2013, AAP provided a Consensus Statement on screening and treating MBD in high-risk preterm infants. Our goal was to i) evaluate the feasibility of implementing a modified AAP MBD practice guideline-based protocol, ii) describe preliminary findings, and iii) identify potential barriers to implementation.

Methods used An 18 month retrospective study in ELBW neonates without major anomalies was conducted. Bone minerals and markers obtained at 4–6 weeks of age included: serum alkaline phosphatase (APA), calcium (Ca), phosphorus (P), magnesium (Mg), parathyroid hormone (PTH), and 25-OHVitamin D (25-OH-D).

Summary of results 46 neonates were included. APA, Ca, P, and Mg were available in 43 (93%) neonates, while PTH and 25-OH-D in 76%, drawn at 36±18 days of life. Mean gestational age was 26±2 weeks with a birth weight of 773±140 grams. At one month of age 52% were receiving parenteral nutrition and 87% had advanced to fortified feeds. Ca, P, Mg were all within normal limits whereas APA, 25-OH-D, and PTH values were 423±188 IU/L, 33±15 ng/mL, and 137±103 pg/mL, respectively. Of note, 3 of 43 (7%) neonates had APA levels>800 IU/L and 4 out of 36 (11%) had 25-OH-D levels<20 ng/mL, the latter normalised with supplementation. Radiographic findings were not obtained on patients with APA >800. Barriers to implementation included: volume of blood draw, cost, faculty preference, and collaboration with radiologists.

Conclusions Implementation of a practice guideline-based protocol focusing on MBD in ELBW infants is feasible. These preliminary findings suggest that serum bone analytes may demonstrate abnormalities as early as 4–6 weeks after birth and require further study. Limitations include small sample size, single-institution, and absence of bone imaging.

117 Repeatedly in rhabdo

C Gooch

L Marzullo

UAB, Birmingham, AL

Case report Repeated presentations of an uncommon symptom in a patient should prompt a physician to evaluate for rare conditions. A teenager presented to the Children’s of Alabama ED with recurrent episodes of rhabdomyolysis and weakness. He was eventually diagnosed with McArdle’s Muscular Dystrophy, Glycogen Storage Disease Type V. His rhabdomyolysis has been severe, with a creatine kinase measurement of >320,000, myoglobinuria, transaminitis and elevated bilirubin. He has a low threshold for going into rhabdomyolysis, such as doing an hour of aerobic exercise two days in a row.

McArdle’s Disease is a Glycogen Storage Disorder in which the skeletal muscle cannot turn glycogen into glucose. Unlike other glycogen storage disorders, this only affects skeletal muscle, sparing the brain and visceral organs, and leading to a vague phenotype. These patients have exercise intolerance, rhabdomyolysis, and muscle cramps. Many patients report loading with simple carbohydrates before exercise as they have learned this can increase their stamina. The vague symptoms can lead to decades of delayed diagnosis and significant mismanagement.

Rhabdomyolysis is the most severe finding in McArdle’s Disease and it can lead to acute kidney injury, requiring dialysis in the severest cases. Rhabdomyolysis has numerous causes, but recurrent episodes, especially with seemingly insignificant triggers, should prompt a broader differential and advanced testing. This testing can include specific exercise tests, genetic sequencing, and muscle biopsy. Our presentation will guide the clinician through the process of evaluating recurrent rhabdomyolysis, working through a differential diagnosis and testing options.

118 Hypothyroidism & dvts

M Henry

AW Maier

UMMC, Jackson, MS

Introduction First discovered in the nineteenth century, hypothyroidism was diagnosed after observing the effects of the surgical removal, which resulted in systemic tissue swelling. The pathology of thyroid disease, either through hypothalamic dysfunction or primary thyroid failure, results in a generalised slowing of the metabolic processes leading to a plethora of abnormalities affecting nearly all body systems. Here we report an atypical presentation of hypothryoidism.

Case report A 74 year old African American female with history of hypertension, diabetes mellitus, hyperlipidemia, and previous history of treated TB presented with a chief complaint of right leg pain for three weeks, most prevalent in her right hip and foot. She had been bed-bound for over one year after months of severe depression following the death of her brother, resulting in severe deconditioning further worsening her weakness.

On physical exam, patient was found to have significant bilateral lower extremity pitting oedema, cold feet, and decubitus ulcers on the right heel and midline sacrum. A CT lower extremity and abdomen/pelvis was obtained showing filling defect in right common femoral vein concerning for DVT, body wall oedema, diffuse lower extremity oedema. Doppler ultrasound of bilateral lower extremities showed multiple nonocclusive DVTs in both legs and one occlusive DVT in the right femoral vein. It was felt that these clots were a result of the patient’s extended immobility. Thyroid function labs were tested due to her history of depression and her significant weakness and fatigue. Laboratory evaluation resulted in a high TSH level of 20.2 mcIU/mL, low T4 measurement of 0.312 ng/dL and subsequently found to have thyroid peroxidase antibody of 443.56 IU/mL and antithyroglobulin antibody 28.94 IU/mL. She was started on Synthroid 50 mcg. During hospitalisation, her weakness improved with physical therapy and she was discharged to an acute rehab facility with outpatient follow up and thyroid ultrasound scheduled in 4 weeks.

Discussion 1. Hypothyroidism can manifest through a wide variety of clinical presentations and must never be overlooked as an underlying aetiology for a patient’s symptoms, even with an atypical picture. 2. When treating a patient with depression, even with a clear instigating factor, thyroid dysfunction should still be considered as an underlying cause.

119 ‘bi-ectopics’: thyroid cancer and parathyroid adenoma

N Jain

W Bibars

H Steinberg

University of Tennessee Health Science Centre, Memphis, TN

Background Most thyroid cancers and parathyroid adenomas are eutopic. About 400 cases of ectopic thyroid cancer have been reported while mediastinal parathyroid adenoma (MPA) is commonly identified on autopsies than during surgery. We report an interesting case of both ectopic thyroid malignancy and MPA.

Case A 54 year old Caucasian woman with heart failure and COPD stage IV presented with an enlarging left neck mass causing odynophagia and shortness of breath. Vital signs were stable. On examination, normal oral cavity, without tonsillar masses or tongue base lesions, and normal thyroid. She had an obese neck, with a palpable, firm, non-mobile, non-tender mass in left level IB/II without other palpable masses. Thyroid function normal: TSH 3.24 mcIU/ml, Free T4 1.35 ng/dL, but calcium high at 11 g/dL. Two ultrasound guided Fine Needle Aspirations were non-diagnostic and laryngoscopy normal. CT scan neck showed a 4 cm complex necrotic mass suggestive of squamous cell or salivary gland malignancy. A core-needle biopsy showed a malignant lesion of unknown primary: positive for TTF-1 and HBME-1, and negative for Napsin A and thyroglobulin. A diagnosis of papillary thyroid malignancy TxN2aMO was preliminarily made, and she underwent thyroidectomy with sentinel lymph node and neck dissection. Incidentally during surgery, a large anterior mediastinal mass was found. It was identified as thymic enlargement and was removed. Pathology showed normal native thyroid and left parathyroid gland, right parathyroid showed benign thyroid tissue. 13.4×6×5.3 cm thymic mass showed enlarged and hypercellular parathyroid gland (2 cm), with small amount of benign thymic tissue. The 1.8 cm left submandibular mass had mixed components of papillary thyroid cancer (TTF-1+) with de-differentiation into squamous cell components (p40, CK5/6+), both with psammomatous calcifications. A final diagnosis of papillary thyroid cancer of ectopic thyroid tissue and parathyroid ectopic in thymus was made.

Conclusion Our case highlights peculiarity of two ectopic glands in an adult female presenting as malignancy of ectopic thyroid and a ectopic parathyroid adenoma in the thymus gland, which is usually regressed in adults.

120 Extreme parathyroidemia

N Jain

A Wynn

A Ogunsakin

H Steinberg

University of Tennessee, Memphis, Memphis, TN

Background Parathyroid hormone (PTH) elevation is frequently associated with a parathyroid adenoma. Levels>1000 pg/ml are almost always due to parathyroid carcinoma, unless proven otherwise.

Case 67 year old African American female brought in for abdominal pain, confusion and lethargy for 3 days. She also had change in her speech and gait. Vital signs stable; exam with abdominal tenderness, disorientation, slow speech and unsteady gait. Workup for sepsis and stroke was normal. On labs, she had low potassium (3.1 mmol/L) and magnesium (1.5 mg/dL) but elevated Calcium (Ca) (14.1 mg/dl) and PTH (901 pg/ml). Albumin (3.5 g/dL) and 25(OH)Vitamin D (18.4 ng/ml) were low while renal function normal. She was treated with fluids, cinacalcet and pamidronate. A sestamibi scan showed a left lower parathyroid adenoma and nodular thyroid tissue on the right. Adenoma resection was performed. Ca and vitamin D replacement started for prevention of hypocalcemia. On one-week follow up, pathology showed 1.15 g hyper-cellular parathyroid tissue, PTH 257 pg/ml and Ca 12.9 mg/dl. The Ca-carbonate dose was reduced, cinacalcet restarted and labs repeated in 6 weeks. She was called for admission when PTH came at 647 pg/ml and Ca 15.1 mg/dl. Repeat tests confirmed elevated PTH 1999 pg/ml and Ca 14.2 mg/dl and low normal PTH-related peptide. Ultrasound revealed a new parathyroid adenoma to the right of thyroid lobe and sestamibi showed persistent nodular thyroid. Concerned for malignancy, a CT neck/chest was performed showing 5.5 cm heterogeneously enhancing mass posterior to trachea, increased in size, contiguous with right thyroid and suspicious for malignancy, but without metastasis. Subsequently, right parathyroidectomy and hemithyroidectomy were performed revealing a 2.5 cm pale, firm, well circumscribed, focally haemorrhagic mass; pathology reported a parathyroid adenoma and benign nodular hyperplasia of thyroid. A work up for Multiple Endocrine Neoplasia-1 was recommended, however the patient declined and was discharged home.

Conclusion Very high PTH (>1000) and Ca levels (>14 mg/dl) are associated with parathyroid malignancy, but can be reported with parathyroid adenomas, as highlighted by our case.

121 Mature onset diabetes of the young: a rare but important entity of diabetes

AL Marle

RE Guinto

D Dwight

Eisenhower Army Medical Centre, Fort Gordon, GA

Case report Mature onset diabetes of the young (MODY) is a group of genetic disorders manifested by non-ketotic diabetes mellitus, autosomal dominant mode of inheritance and age of onset before 25 years. Though rare, accounting for only 1% to 2% of all cases of diabetes, MODY is often misdiagnosed as type 1 or type 2 diabetes. Given this disorder is autosomal dominant, diagnosing this distinct endocrine disorder from the more common forms of diabetes has treatment and prognostic implications across generations.

Here we report a 33 year old Caucasian male with MODY subtype 5. The patient initially presented with diabetes at the age of 24. His BMI was 22 and he participated in regular physical activity. Routine labs obtained at the time of presentation showed an elevated fasting glucose, glucosuria and a haemoglobin A1C of 9.2%. Glutamic acid decarboxylase autoantibodies were obtained to evaluate for type 1 diabetes but found to be negative. He was given a formal diagnosis of type 2 diabetes mellitus and started on appropriate oral diabetic medications. After one year his haemoglobin A1C improved to 6.8%.

Over the next few years, however, the patient demonstrated worsening glycemic control and was referred to Endocrine. Interestingly, the patient reported no family history of diabetes. Additional pancreatic autoantibodies were obtained but negative (tyrosine phosphatase-like protein IA2 and zinc transporter 8). Imaging demonstrated an atrophic pancreas and multiple renal cysts. Lantus was added to Metformin and Sitagliptin which improved the patient’s fasting blood glucose levels.

In MODY 5, a mutation occurs in the hepatocyte nuclear factor 1β (HNF-1β) gene. Normally, this gene produces transcription factors that regulate insulin production. In addition to defective insulin production, the mutations in HNF-1β are associated with pancreatic agenesis, renal abnormalities, genital tract malformations and liver dysfunction. Our patient was found to have a heterozygous frameshift mutation in the HNF-1β gene confirming the diagnosis of MODY 5.

This case highlights the phenotypic manifestations and early disease progression seen in MODY 5. It also underscores the importance of accurate diagnosis in patients with autosomal dominant disorders. In this gentleman’s case, he was referred for genetic counselling.

122 Severe hypoglycemia- a case of von gierke’s disease

A Ogunsakin

H Steinberg

E Nyenwe

University of Tennessee Health Science Centre, Memphis, TN

Background Glycogen storage disorders type 1 have an incidence of 1 in 1 00 000 individuals. Presentation with hypoglycemia at initial diagnosis or periods of acute stress or illness is common. Frequent small servings of carbohydrates must be maintained throughout life to prevent hypoglycemia, lactic acidosis, hypertriglyceridemia, hyperuricemia and other long-term complications.

Case presentation A 43-year-old male who presented to the emergency room with nausea, vomiting and severe hypoglycemia. Past medical history was significant for a history of type 1 glycogen storage disease type 1a that had been managed most of his life with daily corn starch to prevent the symptoms and consequences of hypoglycemia; however, he was unable to tolerate any oral intake on the day of presentation. Laboratory data showed severe hypoglycemia in association with significant metabolic acidosis and Lactic acidosis. He had a serum glucose of 29 mg/dL, anion gap of 26, carbon dioxide level of 6 mmol/L, lactic acid level of 19.2 mmol/L and beta-hydroxybutyrate level of 2.9 mmol.

Hospital Course Hypoglycemia resolved over 24 hours in response to 10% intravenous dextrose for glucose infusion rate of ~2.3 mg/kg/min. Lactic acidosis resolved gradually.

Discussion Von Gierke’s disease, also known as glycogen storage disease (GSD) type la, is a rare autosomal recessive disorder of the metabolism in which there is an inability to break down glycogen into glucose due to the deficiency of enzyme glucose 6-phosphatase. Patients with GSD type 1 usually present at infancy with hepatomegaly and signs and symptoms of hypoglycemia, and less commonly as adults. Severe hypoglycemia is potentially life-threatening and individuals with glycogen storage disease such as Von Gierke’ s disease can present with severe hypoglycemia, when they are unable to maintain a steady source of exogenous glucose.

Conclusion Glycogen storage disorders are rare and a high index of suspicion for such disorders is warranted when severe hypoglycemia exists in combination with severe lactic acidosis, in the absence of sepsis. Prompt treatment of hypoglycemia is important to prevent significant morbidity and death.

123 Endocrine complications of lithium therapy in a critically-ill woman

C Ogwo

M Haltom

E Nyenwe

University of Tennessee Health Science Centre, Memphis, TN

Case report Lithium is an effective therapy for psychiatric disorders but has several adverse effects including endocrine dysfunction. We present a critically–ill woman with multiple endocrine abnormalities due to lithium therapy.

Case presentation A 60-year-old female with a history of bipolar disorder treated with Lithium was brought unconscious to the Emergency Room. She had tachycardia (heart rate-140/minute), tachypnea (respiratory rate-26/minute) and crackles in both lung fields. Chest imaging showed bilateral lower lobe consolidation and patchy infiltrates in the upper lobes. She was diagnosed with multifocal pneumonia and admitted to the intensive care unit. She was mechanically ventilated for acute respiratory failure. Blood chemistry showed hypernatremia-147 mmol/L, hypercalcemia-ionised Ca −1.41 mmol/L, TSH-0.236 uIU/mL, free T4-0.69 ng/mL, and free T3 <0.50 pg/mL, PTH-53.1 pg/ml, Lithium level- 0.4 mmol/L. She was treated with antibiotics and corticosteroids for pneumonia, intravenous hydration for hypernatremia and hypercalcemia. Hypercalcemia and hypernatremia improved but ionised Ca remained elevated at 1.34 mmol/L. She also developed hyperglycemia. She made full recovery after 31 days and was discharged.

Discussion We have presented a patient with severe pneumonia, respiratory failure and multiple endocrinopathies attributable to chronic lithium therapy (hypercalcemia, hypernatremia, hyperglycemia and abnormal thyroid function). These endocrine defects may have contributed to the severity of her illness. Lithium treated patients could have endocrine abnormalities commonly diffuse goitre but a constellation of endocrine defects as in our patient is not common. Since lithium is commonly used in psychiatry, physicians should be aware of the potential for endocrine abnormalities that could complicate other co-morbid conditions. Hypercalcemia is due to decreased sensitivity of the calcium sensing receptor in the parathyroid gland. Hypothyroidsm with goitre is due to inhibition of iodine uptake, coupling and thyroid hormone secretion. Lithium inhibits the expression of aquaporins in the collecting ducts leading to concentrating defects and diabetes insipidus. Hyperglycemia results from the inhibition of calcium influx into the β-cell which blocks insulin secretion.

124 Graves thyrotoxicosis in a patient with sickle cell anaemia

C Ogwo

A Ogunsakin

E Nyenwe

Uthsc, Memphis, TN

Case report Graves’ disease (GD) is a common cause of hyperthyroidism with an annual incidence of 20–50 cases/100,000 persons but GD is extremely rare in patients with sickle cell anaemia (SCA). We present a patient with SCA who developed GD that complicated the course of her disease and ultimately contributed to mortality.

Case presentation A 28 year old African-American woman with a history of SCA, presented with painful vaso-occlusive crisis. She had been hospitalised several times in our facility with bone pain crises but at this admission she complained of loose stools, palpitation and fluttering in her chest. On examination, her heart rate was 168/minute and irregular, she had mild proptosis. ECG showed atrial fibrillation with rapid ventricular response. Her thyroid function tests revealed undetectable TSH, free t4-10.42 pg/mL (2.18–3.98), free t3-3.13 ng/dL (0.76–1.46). Thyroid scintigraphy showed diffuse radioiodine uptake of 69% at 24 hours (10%–30%). She was treated with methimazole and metoprolol but treatment was complicated by non-adherence and frequent episodes of vaso-occlusive crisis. Attempts to treat with radioiodine to control thyrotoxicosis were not successful due to recurrent painful crises requiring hospitalisation, atrial fibrillation and difficulty keeping her appointments. She presented to the hospital a few months ago with acute chest syndrome, thyrotoxicosis and atrial fibrillation. She developed acute respiratory failure requiring mechanical ventilation, hypotension and cardiac arrest. Resuscitation was unsuccessful and she died. Her post mortem studies showed an enlarged thyroid of 94 grams; the cause of death was reported as cardiac arrest due to acute chest syndrome from vaso-occlusive crisis.

Discussion Hypothyroidism is uncommon in patients with SCA but GD is extremely rare. Thyrotoxicosis complicates the course of SCA due to added burden from thyro-cardiac disease. Upregulation of adrenoceptors in hyperthyroidism results in a hyperdynamic circulation and arrhythmias, which contributed to worsening episodes of vaso-occlusive crises and ultimately death in our patient. Physicians who care for patients with SCA should keep this rare but potentially fatal condition in mind. Thyroid function tests would be warranted in patients with recurrent vaso-occlusive crises and tachycardia or arrhythmias.

126 Inappropriately high parathyroid hormone with low serum calcium in a 60-year-old female with suspected albright hereditary osteodystrophy

P Ratanasrimetha

T Mingbunjerdsuk

S Thavaraputta

S Suchartlikitwong

A Rivas-Mejia

Texas Tech University Health Sciences Centre, Lubbock, TX

Background Parathyroid hormone (PTH) and vitamin D play a crucial role in serum calcium and phosphorus regulation. Pseudohypoparathyroidism is a rare condition that results from parathyroid hormone resistance. As a result, patients present with low calcium level, high phosphorus level and inappropriately high PTH level as well as characteristic phenotypic appearances.

Case report 60-year-old female with sustained hypocalcemia documented 14 years ago. She had no symptom of hypocalcemia. She had been on calcium carbonate and vitamin D replacement for several years. She had history of normal menstruation periods until menopause. She denied history of neck surgery. She did not have family history of calcium disorders.

Current medications were calcium-vitamin D 1200 mg-1600 IU per day, hydrochlorothiazide, metoprolol, rosuvastatin and paclitaxel. On physical examination, her BMI was 36.9. She was obese and had clinical features that are compatible with Albright hereditary osteodystrophy including round face and short digits. She did not have knuckles abnormality. Laboratory studies were as follow: calcium 6.4 (normal 8.8–10.5) mg/dL, phosphorus 5.7 (normal 2.7–4.5) mg/dL, PTH 140 (normal 15–65) pg/mL, thyroid stimulating hormone 3.54 (normal 0.27–4.20) IU/mL, and 25-hydroxy vitamin D 37 (normal 30–100) ng/dL. 24 hour urine calcium after discontinuation of calcium supplements was 17 mg. Genetic studies are pending.

Conclusion Prevalence for Albright hereditary osteodystrophy is 0.79 per 1 00 000. It is characterised by end organ resistance to PTH action. Albright hereditary osteodystrophy has clinical findings such as brachydactyly, round face, short stature, central obesity and variable degrees of mental retardation.

Pseudohypoparathyroidism (PHP) has several variants resulting in varying degrees of disorders. PHP type 1 is inherited as autosomal dominant. There are differenced between maternal and paternal transmission. Maternal transmission manifests in PHP type 1a expression, whereas paternal transmission associates with pseudo-pseudohypoparathyroidism with Albright hereditary osteodystrophy features but without hormonal resistance.

127 A rare case of metastasis to thoracic spine from malignant pheochromocytoma

A Reddy

WS Orr

V Manucha

University of Mississippi Medical Centre, Jackson, MS

Case report Pheochromocytoma is a rare, destructive neuroendocrine malignancy that occurs primarily in the adrenal glands and can metastasize anywhere along the sympathetic chain. The diagnosis of malignant pheochromocytoma is often problematic to analyse microscopically and thus is primarily dependent on the existence of local invasion or metastasis. The incidence of malignant pheochromocytoma with bone metastases is approximately 10%. Vertebral involvement is exceedingly rare with only a few cases reported. We present a case of malignant pheochromocytoma with metastatic lesions to the thoracic spine.

A 60 year-old female presented with a constant cough in the setting of increasingly intense headaches, dizziness, nausea, and vomiting over a few weeks. A chest x-ray revealed a nodule. Laboratory work-up showed significantly elevated catecholamines in both blood and urine. Computerised Tomography (CT) scan demonstrated a large right-sided adrenal mass with metastatic lesions to liver, sacrum and bilateral iliac bones. A radical right adrenalectomy was performed with tumour measuring 5.9×5.6 cm extending into the peri-renal fat. Focal renal capsular invasion at superior pole and lympho-vascular invasion was identified. Multiple wedge resections of liver and right nephrectomy were performed. Immunohistochemistry demonstrated tumour cells positive for synaptophysin and chromogranin; S-100 focally highlighted sustentacular cells. Stains for cytokeratin, calretenin and MART-1 were negative. Ki-67 was approximately 8%–10%. Findings consistent of pheochromocytoma. Germ-line genetic testing revealed no mutations. Patient complained of back pain at 3 month follow-up. CT showed T11 and T12 thoracic spine lesions. Subsequent bone biopsy contained bony trabeculae with tri-lineage hematopoietic elements and interspersed tumour cells positive for synaptophysin and negative for leukocyte common antigen, consistent with a metastatic pheochromocytoma.

She underwent radiofrequency ablation and cement augmentation to the thoracic spine lesions. The surgical, radiological, and pathological overlap of this case highlights the importance of a multidisciplinary approach to these patients.

128 Adrenal insufficiency induced by megestrol acetate

HA Rehman

M George

K Shah

J Krodel

OUHSC, Oklahoma City, OK

Case report Megestrol acetate (MA), or Megace, is a synthetic progestin commonly used as an appetite stimulant in patients at high risk for malnutrition or cachexia due to chronic illnesses such as cancer or HIV/AIDS. It is often used in the adult population, with limited use in the paediatric setting. Evidence has shown the benefits of MA with respect to weight gain, however, it is not without adverse effects. Due to its affinity for the glucocorticoid receptor, MA has been reported to cause suppression of the hypothalamic-pituitary-adrenal (HPA) axis. Thus these patients can present with clinical signs of Cushing’s syndrome and/or adrenal insufficiency (AI), as seen in this case.

A 4 year old Hispanic male presented to the general paediatrics clinic for a well child visit. Review of systems was positive for weight gain and generalised weakness. His medical history was significant for myopathy of unknown aetiology and failure to thrive. He had been taking MA (300 mg/day) for over 1 year prior to presentation prescribed as an appetite stimulant due to poor weight gain. Physical exam was notable for mild moon facies, diffuse muscle weakness and decreased muscle tone. He was referred to endocrinology where an ACTH stimulation test was performed to assess for adrenal insufficiency. His basal cortisol was 0.7 mcg/dL and 0.5 mcg/dL and 0.6 mcg/dL at 30 and 60 min, respectively, suggestive of severe adrenal insufficiency. MA was slowly tapered off over a period of 2 weeks and the patient was prescribed stress-dose steroids. A repeat ACTH stimulation test 4 months later showed a normal response suggestive of recovery of the HPA axis with notable resolution of moon facies.

Significant complications, including death, have been associated with the use of Megace in adults. MA-induced adrenal insufficiency can be potentially life-threatening in children as well. AI has been noted to occur either following abrupt withdrawal or during active treatment with the medication. Although the mechanism remains unclear, it is believed to be due to suppression of the HPA axis. With the widespread use of appetite stimulants like Megace, it is important for clinicians to be aware of the adverse effects, particularly related to cortisol axis due to its severity.

129 Clinical characteristics and outcomes of paediatric patients with severe hypertriglyceridemia

TH Richardson1

A Ashraf1

S Aslibekyan2

1Children’s of Alabama, Birmingham, AL

2UAB School of Public Health, Birmingham, AL

Purpose of study Severe hypertriglyceridemia (HTG i.e., Serum TG >1000 mg/dl) is extremely rare in children. Little is known about the aetiology, management and treatment outcome of this disease in children. The primary objective was to evaluate the aetiology and outcomes of severe hypertriglyceridemia. A secondary objective was to analyse the metabolic abnormalities associated with severe HTG categorised by the aetiology.

Methods used This was a retrospective Electronic Medical Record (EMR) chart review of paediatric patients with severe hypertriglyceridemia at Children’s Hospital of Alabama, University of Alabama at Birmingham (UAB) between 1999 to 2016. Inclusion criteria were:

  • serum total triglyceride concentration >1000 mg/dL

  • adequate documentation of lipid panels and complete metabolic panels

  • a weight recorded within 6 months of when their triglycerides were over 1000 mg/dl.

Patients were excluded if they had insufficient anthropometric information, biochemical testing, or lacked documentation providing a clinical picture.

Summary of results 2987 patients had elevated triglycerides based on the ICD 9 code of 272.1 for pure hypertriglyceridemia and 272.2 for mixed hyperlipidemia. 140 had severe hypertriglyceridemia. 29 subjects were excluded. Etiologies included renal disease (n=14), diabetes (n=40) TPN related (n=27), malignancy related (n=42, ALL=24, CML=3, other malignancies=17) and miscellaneous (n=5). The average number of days for serum TG to decrease to <1000 mg/dl was 147.68±567.28 days. The average number of days for serum TG to decrease to <500 mg/dl was 136.84±230.9 days. 64 patients had persistent dyslipidemia. The triglyceride levels fell below 500 mg/dl in 73 patients.

Conclusions Severe HTG is rare in paediatrics and is often due to secondary causes rather than primary genetic abnormalities. More than half the patients continue to have persistent dyslipidemia at follow up indicating underlying metabolic abnormality. Severe HTG in children is a serious condition with serious complications that lacks specific management guidelines. We postulate that severe hypertriglyceridemia occurs when patients with genetic susceptibility to hypertriglyceridemia are in situations where there is increased biosynthesis and/or of failure to clear TG-rich lipoproteins.

130 A comparison of fluid resuscitation using 0.9% saline and lactated ringers in paediatric diabetic ketoacidosis

A Rughani

M Marin

University of Oklahoma Health Sciences Centre, Oklahoma City, OK

Purpose of study Patients with diabetic ketoacidosis (DKA) are dehydrated and require fluid resuscitation prior to insulin therapy. However, there are currently no clinical guidelines on the optimal resuscitation fluid in paediatric DKA. This study determines the effects of 0.9% saline (NS) vs lactated ringers (LR) during initial fluid resuscitation in paediatric DKA.

Methods used A retrospective cohort analysis of paediatric patients over 5 years who presented to the ED of a tertiary care centre and received NS, LR, or both NS and LR as resuscitation fluids. DKA was defined as bicarbonate <15 mEq/L. 3 cohorts (NS only, LR only, NS and LR) were analysed. Outcomes measured were time to metabolic resolution, changes in serum glucose, potassium, chloride, creatinine, and bicarbonate, and admission length. Student t statistic was used to compare the statistical significance of outcomes between NS vs LR, and NS vs NS and LR cohorts. Least squares regression analysis was used to determine the correlation between fluid volume and outcomes.

Summary of results 96 paediatric patients were studied. 69 received NS, 19 received LR, and 8 received both NS and LR. The mean age was 12.3 years; the mean fluid bolus was 23 ml/kg (95% CI: 20.8 to 25.2). At presentation, mean bicarbonate was 7.6 mEq/L, glucose was 552 mg/dL, potassium was 4.9 mEq/L, chloride was 101 mEq/L, and creatinine was 1.16 mg/dL. After correction of acidosis, mean bicarbonate increased to 17.3 mEq/L over 10.1 hours (95% CI: 9.0 to 11.2); glucose corrected to 203 mg/dL at a rate of 42 mg/dL/hr (95% CI: 35.0 to 49.0), potassium declined by 1.4 mEq/L, chloride increased by 13 mEq/L, and creatinine normalised to 0.53 mg/dL. Mean hospital stay was 1.9 days. Outcomes between cohorts (NS vs LR, NS vs NS and LR) were not statistically significant (α=0.05) and there was no statistically significant correlation between the amount of initial fluid received and metabolic outcomes.

Conclusions There were no statistically significant differences in metabolic outcomes between NS vs LR, or NS vs NS and LR as initial fluid resuscitation agents in paediatric DKA. Given cost and accessibility considerations of LR in the ED, NS is efficacious for initial fluid resuscitation in paediatric DKA.

131 Improving guideline based diabetes management in an academic outpatient clinic

S Sareen

M Agarwal

C Stanton

G Hamman

J Campbell

NA Hommos

University of Tennessee Health Science Centre, Memphis, TN

Purpose of study Diabetes mellitus (DM) is a chronic illness.Haemoglobin A1c (HbA1c) is a good marker of glycemic control over previous 3 months. Metformin monotherapy should be started in patients at time of diagnosis of type 2 DM unless contraindications are present. Furthermore, in patients with fasting blood glucose >300, A1c>10% or patients with hyperglycemic symptoms, insulin therapy must be considered. We had observed clinical inertia in initiating and intensifying insulin treatment by housestaff in our outpatient clinic. Considering this, we designed a quality improvement project to educate and update housestaff on current ADA guidelines.

Methods used We evaluated consecutive patients with type 2 DM and reviewed their baseline demographics, A1c, metformin use, and insulin dosing using our electronic record system from April 1 2016 to June 30 2016 (pre-intervention period) and from Jan 1 2017 to March 31, 2017 (post-intervention period). We collected data on what management was offered to these patients on their subsequent visits (2–3 months after the A1c was drawn in both groups). Our intervention included conducting small group discussions of current ADA guidelines, placing posters on appropriate use of oral anti-diabetic agents and insulin regimens in clinic. We performed analysis to see improvement in pre and post intervention groups.

Summary of results Our pre-intervention group included 101 DM patient], while 98 patients were included in post-intervention group. The pre-intervention group did not differ from post intervention groups in terms of percentage of patients who were offered metformin (71.6% vs 75%, p=0.58), insulin initiation for non-users (41.7% vs 21.4%, p=0.27) and increase in dose of insulin (34.4% vs 34.5%, p=0.98). The post-intervention group had significant increase in rate of life style modification intervention offered (48.5% vs 32.1%, p=0.02) compared to pre-intervention group.

Conclusions We observed a significant increase in rates of life style modification intervention offered in the post-intervention group, the most important aspect of diabetes management.

132 Transient hyperthyroidism caused by pemprolizumab: a case report

SK Prieto

J Lado

R Shroff

TTUHSC, Lubbock, TX

Background Immune checkpoint inhibitors are new, effective treatment options for some cancers. Programmed cell death protein 1 (PD-1) receptors are found on lymphocytes as well as several cancers such as melanoma, lung cancer and lymphoma. PD-1 inhibitors like pembrolizumab have been shown to prolong survival and are generally well tolerated, however, there are adverse side effects that can occur. Checkpoint inhibitors are associated with autoimmune induced endocrinopathies but the mechanism by which this occurs remains unclear. Pembrolizumab has been associated with thyroid dysfunction, more specifically hypothyroidism, that typically develops weeks after treatment is initiated. It remains to be elucidated as to which patients are most susceptible to developing these adverse side effects.

Case report A 55 year-old male with significant smoking history presented to clinic with shortness of breath, dysphagia and weight loss. A chest computerised tomography (CT) showed multiple pulmonary nodules, a right lower lobe mass, and enlarged mediastinal and hilar lymph nodes suggestive of malignancy. Biopsy of the lung mass was positive for poorly differentiated adenocarcinoma. Further imaging revealed nodules on the adrenal glands bilaterally and multiple ring enhancing masses within the brain. Pembrolizumab was started as palliative chemotherapy after whole brain radiation and thyroid function tests drawn prior to treatment were normal. Two days later he presented to the emergency department with vomiting, palpitations and constipation. Thyroid function tests showed hyperthyroidism. Pembrolizumab was stopped and the patient decided to return home on hospice after repeat CT imaging showed progression of his cancer.

Discussion This unusual case reiterates the importance of screening patients for possible endocrine related side effects while on these medications. Further research is needed to investigate how checkpoint inhibitors cause autoimmune mediated endocrinopathies. Once better understood, there may be specific factors that can be targeted to help clinicians determine which patients will likely develop these adverse side effects.

133 Skull metastasis of papillary thyroid carcinoma: a case report

SK Prieto

T Warmoth

J Lado

R Shroff

TTUHSC, Lubbock, TX

Background The calvarium is a common site for metastasis, especially for breast cancer, lung cancer and prostate cancer. While 15%–25% of all cancer patients will have metastasis to the skull, only about 2%–5% of skull metastasis are due to thyroid carcinoma. Follicular thyroid carcinoma is the most reported type of thyroid cancer to metastasize to the skull and is typically found at the base and the calvaria. Papillary thyroid carcinoma, on the other hand, does commonly metastasize to the bone but rarely to the calvaria. Due to its rarity, calvaria metastasis from thyroid carcinoma pose many diagnostic and therapeutic challenges.

Case presentation A 54-year-old man presented to his primary care physician with swelling over the scalp and a mass that he noted to be increasing in size. Magnetic resonance imaging and computed tomography showed a large destructive mass overlying the frontal convexity bilaterally involving the extra-axial intracranial space, full-thickness of the calvarium and extending into the sagittal sinus. Frontal craniectomy was preformed and pathology of the mass confirmed metastatic papillary thyroid carcinoma involving the dura with lymphovascular invasion. After thyroidectomy, craniectomy and resection of metastasis to the right humerus, he underwent radioactive iodine ablation 6 months later. Unfortunately, post-treatment I-131 Whole Body Scan showed new activity involving the pelvis, right femur and left thyroid lobe. He continues to undergo treatment for metastatic papillary thyroid cancer.

Discussion Calvarial and dural metastasis from papillary thyroid carcinoma is extremely rare and surgical resection is the treatment of choice followed by postoperative radiotherapy. Due to the rarity and complexity of such widespread metastatic cancer, a multidisciplinary approach helps to provide the best treatment outcomes.

134 Characteristics of patients with established type 1 diabetes mellitus admitted to the paediatric intensive care unit for diabetic ketoacidosis from 2012 to 2016

S Vazquez Diaz

N Bilbao

University of South Alabama, Mobile, AL

Purpose of study Diabetic Ketoacidosis (DKA) in established Type 1 Diabetes (T1D) patients is an extremely common cause of hospital admission, despite being highly preventable. In the past decade, there were remarkable improvements in insulin therapy and glucose monitoring, but the risk of recurrent DKA admission remains high. We aim to describe the characteristics of patients who had multiple hospital admissions for DKA in the last five years, in order to identify high risk groups. We hope to use the findings for future development of targeted intervention for prevention of recurrent hospitalizations for DKA.

Methods used After obtaining IRB approval, a retrospective chart review was performed using electronic health records of USA Children’s and Women’s patients ages 1 to 20 years old with established T1D, who were admitted with a diagnosis of DKA in the Paediatric Intensive Care Unit, from January 2012 to December 2016. Age, sex, race/ethnicity, type of insulin treatment (pump, basal/bolus, conventional, mixed) and HbA1c at time of admission were collected and analysed for frequency distribution.

Summary of results A total of 567 admissions were reviewed, of which 383 met the inclusion criteria. These admissions were divided by year (2012–2016). 2014 was the year with most admissions (n=84). Most admissions fall under the ages 13–17 years. There were more females admitted in every year (54%), except in 2016, in which there were more male admissions (55%). More patients admitted were Caucasian (49%), except for 2015 in which there were more African American patient admissions (58%). The most common treatment modality was basal/bolus regimen in all years, except 2012 when patients admitted were mostly on conventional (twice daily) insulin regimen. The HbA1c range of these patients was mostly 10%–16%.

Conclusions Patients with established T1D who were admitted for DKA were mostly 13 to 17 years old, with a HbA1c of 10% and above. Most patients admitted were receiving Multiple Daily Injections (basal-bolus), except in the earliest study year (2012), in which conventional dosing was the most prevalent treatment modality. Development of targeted intervention for patients with these characteristics may help decrease their recurrent hospitalizations for DKA.

135 Exploring a rare complication of diabetic ketoacidosis

M Yousif

K Shah

K Ward

S Krishnan

University of Oklahoma Health Science Centre, Oklahoma City, OK

Case report Diabetic Ketoacidosis (DKA) is typically the presenting sign of Diabetes Mellitus type I (DMI). Among the serious complications of DKA, cardiac issues are not appropriately highlighted. We report a case of a child presenting with DKA complicated by myocardial ischemia. This association has been well studied in the adult population, but there is limited data in the paediatric population, and its possible long term consequences.

A previously healthy 13 year old African American obese male (BMI-36.5 kg/m2) was admitted to the Paediatric ICU with altered sensorium. Review of symptoms revealed polyuria, polydipsia and extreme fatigability for 2 weeks. Admission labs showed bicarbonate of <10 mmol/L, pH of 7.09, glucose of 1,300 mg/dL, anion gap of 27, and elevated creatinine of 2.9 mg/dL. He was started on standard DKA protocol with insulin infusion and fluids. After acidosis resolved, he was noted to have an irregular rhythm on exam, so an electrocardiogram (EKG) was obtained and showed ST elevation in the inferolateral leads along with prolonged QT interval. Cardiology was consulted and cardiac enzymes were obtained, which revealed both troponin I and CK-MB to be elevated (2.215 ng/mL (normal <0.4 ng/mL) and 5.9 ng/mL (normal 0.0–4.9 ng/mL) respectively). An echocardiogram was normal. The EKG changes and elevated cardiac enzymes were believed to be secondary to metabolic abnormalities resulting from DKA rather than acute coronary syndrome and no additional intervention was needed. Repeat chest pain profile obtained on outpatient follow up showed complete normalisation of cardiac enzymes.

There are very few case reports describing cardiac dysfunction as suggested by elevation of cardiac enzymes and EKG changes in paediatric patients presenting with severe DKA. The aetiology of myocardial cell damage is not secondary to an acute coronary event. Metabolic abnormalities, fluid shifts, tachycardia, and increased sympathetic tone may lead to focal myocardial necrosis and troponin release. Elevations in the cardiac enzymes have been associated with increased mortality in adult population, but this is not well described in children. Paediatricians should be aware of rare complications of DKA like myocardial strain in absence of acute coronary syndrome, for appropriate management of these children.

Gastroenterology, Joint plenary poster session and reception, 4:30 PM, Thursday, February 22, 2018

136 Omelsartan induced enteropathy

MA Alawoki

A Gaba

East Tenn State University, Johnson City, TN

Case report Omelsartan is a relatively new angiotensin receptor blocker (ARB) but unlike most other ARBs, it has been associated with drug induced enteropathy. We present the case of a 75 year old female who presented to clinic with complaints of diarrhoea of 5 years duration. She had multiple episodes a day with no association with meals and stools were loose, fatty but not foul smelling or bloody. Abdominal pain and weight loss were present with onset of diarrhoea 5 years ago. Symptoms were first thought to be due to methotrexate used to treat eczema (misdiagnosed as psoriasis) but they failed to resolve on discontinuation of methotrexate. Diagnosis of irritable bowel syndrome was later made after workups and evaluation by gastroenterologist but symptoms still persisted with trial of cholestyramine and pancrelipase. On review of medications, she had been on omelsartan, omeprazole, paroxetine, and simvastatin for many years. Omelsartan was then suspected as the cause of her diarrhoea and she was switched to losartan. On return to clinic 2 weeks later, patient reported resolution of diarrhoea and she was still stable 3 months after.

Discussion Enteropathy was initially not associated with omelsartan when approved in 2002 but the FDA advised that sprue-like enteropathy be included as an adverse effect in 2013. Between 2008 and 2010, Mayo clinic reported 22 cases and the ACG reported another 40 cases by mid 2012. In general these patients had moderate to severe diarrhoea, lost significant weight, had villous atrophy, negative celiac antibodies and failed trial of gluten free diet. On stopping omelsartan, symptoms resolved and there was appropriate weight gain. Though there is a lag between initiation of therapy and onset of symptoms, it is important for clinicians to be vigilant and recognise that symptoms can occur at any point. Pathophysiology is unclear but likely due to suppression of intestinal acidity which potentiates bacterial overgrowth, effects of infectious agents and destabilisation of gut motility. Delayed cell mediated immune response is also a potential cause. In the wake of the rising awareness of omelsartan induced enteropathy, a case of valsartan induced enteropathy has also been reported. Consequently, if symptoms fail to resolve in a suspected case after switching omelsartan to another ARB, it is reasonable to switch to a new class of antihypertensive.

137 Pyogenic granuloma: rare cause of gastrointestinal bleeding

C Ashangari

N Salagundla

S Siddiqui

JP Garrido

Texas Tech Univ HSC Amarillo, Amarillo, TX

Background Pyogenic granuloma is a benign, lobular capillary hemangioma that most commonly occurs on the skin. Only a handful of cases have been reported in the gastrointestinal tract.

Case report 67-year-old, Caucasian male with significant PMH of liver cirrhosis, DM, HTN and MI presented with black tarry stools. Hb was 9.9. EGD showed 3 columns of grade 1 non-bleeding esophageal varices and moderate portal hypertensive gastropathy. Pathology report showed polypoid gastric mucosa with mild chronic focally active gastritis with acute inflammation superficially within the epithelium with areas of erosion and acute inflammation within fibrinous debris. There was underlying lamina propria granulation tissue, foveolar hyperplasia, and elongation of the muscularis mucosa. No intestinal metaplasia, dysplasia or malignancy was identified. Repeat upper endoscopy was done which showed multiple large polyps in the antrum measuring from 0.6 to 1.5 cm about 12 to 15 of them in the antrum area, status post band ligation of 6×16 and 1 polyp identified in the distal body greater curvature. Diagnosis of pyogenic granuloma was made based on the findings.

Discussion Pyogenic granuloma is an uncommon lesion of the GI tract most commonly managed by excision using a polypectomy snare, endoscopic mucosal resection, or surgical resection.

Abstract 137 Figure 1

Endoscopic appearance of multiple antral gastric polypoid nodules

Abstract 137 Figure 2

Endoscopic appearance of the 12 to 15 gastric polypoid nodules in the antrum measuring from 0.6 to 1.5 cm

138 Diagnostic dilemena in adolescent with multiple systemic complaints

JJ Burns

P Tran

R Dillard

University of Florida, Pensacola, FL

Case report

HPI A 16-year-old white female presented to clinic with multiple complaints including fatigue, weakness, abdominal pain, anorexia, headaches, depression, weight loss and syncopal episodes for 1 year. No fever, vomiting or diarrhoea.

PMH Hereditary Multiple Osteochondromatosis requiring 4 surgical interventions for excision of bony exostosis with many problems including pseudoarthrosis.

Pregnancy 8 months prior; at first prenatal visit, she had a low albumin of 1.7, a total protein of 4.5 and HGB of 9.8 with microcytic indices. She also had post-partum endometritis and haemorrhage requiring transfusions and ICU admission. After delivery, patient had significant depression, being uninterested in caring for the baby. When evaluated 6 months post-partum, she was experiencing persistent weakness, fatigue, weight loss and vegetative symptoms with nearly no memory of the pregnancy or post-partum period.

Over the past 2–3 years, she had gone to the ED eight times and had consultations from many specialists and had numerous radiological and lab procedures for the myriad of symptoms noted above.

On PE, patient appeared chronically ill, had BMI of <1 st percentile, T 96.8 and had pains over multiple osteochondromas throughout. She had otherwise an unremarkable exam except for weakness and slight tenderness in the right upper quadrant.

Pertinent labs revealed a normal CBC, ESR, phosphorous, CMP, thyroid function tests and negative HIV. TTG IgA antibody was 50 U/ml (normal <3) and she was referred to Paediatric Gastroenterology where a biopsy of the small bowel showed marked villous blunting and dense lymphoplasmacytic infiltrate to the lamina propria. A bone density DexaScan revealed osteoporosis with Z-score of −2.7.

Course Patient was placed on gluten-free diet, iron, vitamin D and calcium supplementation and is showing dramatic improvement with overall weight gain and resolution of depression and weakness.

Discussion In retrospect, this patient’s celiac disease symptoms including unexplained malnutrition, depression, anaemia, and hypoproteinemia likely contributed significantly to her difficult pregnancy, complicated post-partum course and chronic bone disease.

139 Liver failure caused by hepatitis a treated with n-acetylcysteine

JA Carlson

G Nelson

C Harris

J Cury

University of Florida, Jacksonville, FL

Case report An 88 year-old female with history of HTN and heart failure presented for evaluation of chest discomfort and abdominal pain for 3 days. On admission patient was encephalopathic with scleral icterus, RUQ abdominal tenderness, asterixis, multiple spider angiomata and 3+pitting oedema. Imaging included CXR showing pulmonary oedema and ultrasound with hepatic steatosis. Labs were significant for ammonia of 127, AST 5403, ALT 2336, ALP 77, albumin 3.2, total bilirubin 1.8, INR 1.8, AG 18, Cr 2.99, troponin 0.39, and WBC 16.18 with 12% monocytes. Acute hepatitis panel revealed hepatitis A (HAV) IgM positivity. After 5 days of supportive care and intermittent N-acetylcysteine (NAC) infusions, her mental status had improved as well as troponins and LFTs returning to normal range, creatinine approached baseline, and she was stable on room air.

HAV can be difficult to diagnose as it can be subclinical or present in fulminant hepatic failure, with a worsening course for older adults or patients with underlying hepatic disease. Patients present with vague symptoms as 70% experience abrupt nausea, vomiting, anorexia, fever, malaise and abdominal pain. HAV is diagnosed by testing anti-HAV IgM antibodies which are present 2–14 weeks after initial exposure. The overall prognosis for acute HAV in its less severe manifestations are good as long as patients are not presenting in liver failure or with underlying liver disease. If these features are absent, the course is generally self-limiting. Typically within 3 months 85% of patients achieve clinical and biochemical resolution, with almost all patients resolving by 6 months. Survival rates for Hep A patients with acute liver failure, however, are approximately 60%, with over half of these patients requiring liver transplantation.

While NAC is well known for acetaminophen toxicity treatment, it may be beneficial in other forms of acute liver failure. A placebo-controlled trial with 173 patients with acute liver failure due to causes other than acetaminophen found significantly higher transplant-free survival (40 vs 27%) in patients randomised to NAC. Thus, for patients who are unlikely to qualify for liver transplant and presenting in fulminant hepatic failure, as in this case, NAC may give these patients a better chance at survival.

140 Polysplenia syndrome with pancreas malformation; an unforeseen clinical presentation of chaudhrey’s disease

C Castillo Latorre

San Juan City Hospital, San Juan

Case report From the zygote to the lateral plate mesoderm subsequently develops a primitive organ called the spleen. A rare clinical presentation of an even rarer pathology, we present the case of a 34 year old Hispanic patient, that came to the emergency department with black stools of a few days of evolution, also associated with a recently 10 pound weight loss history. With further interrogation an interesting surgical history was evident, and an even more noteworthy past family history. Upon supplementary evaluation in the case an abdominal pelvic computer tomography was performed, multiple spleens were seen and surprisingly absence of the pancreas body. We present a rare clinical presentation of polysplenia syndrome (Incidence 1/250,000), a congenial abnormality that is characterised of two or more spleens and various organ anomalies. An extremely rare case of a 34-year-old male without past medical history or toxic habits, which presented with upper gastro intestinal bleeding, associated with severe thrombocytopenia. When embryological changes occur major clinical complications develop. Those alterations are commonly seen in childhood, however major catastrophic presentations could be seen in adulthood as in our case. It is a well-documented relationship between malposition of visceral organs and multiple anomalies such as cardiovascular malformations, bowel malrotation, pancreas agenesis, biliary atresia, and portal vein anomalies. Most of them will die during childhood, mainly due to cardiovascular anomalies. However, less than 10% of affected individuals with no major cardiac anomaly reach adulthood and are nearly asymptomatic, but a even less percentage of them will present with upper gastrointestinal bleeding.

141 Gastrointestinal stromal tumour at ampulla of vater presented with upper gastrointestinal bleeding

P Chariyawong

P Laoveeravat

A Rakvit

Texas Tech University Health Sciences Centre, Lubbock, TX

Case report Gastrointestinal stromal tumours (GISTs) is a mesenchymal tumour accounted for only one percent of primary GI cancer. An estimated 4000 to 6000 new cases of GISTs are diagnosed annually in the United States. Common locations of tumour are stomach and small intestine. GISTs at ampulla of Vater is extremely rare of only 12 cases per literature review. We presented the rare case of GISTs at ampulla of Vater presented with upper gastrointestinal bleeding.

Sixty five years old female with history of diverticulitis status post laparoscopic sigmoidectomy, and gastroparesis presented with melena for 2 weeks. Upper endoscopy showed submucosal mass at the Ampulla of Vater. Biopsy showed normal duodenum. CT abdomen showed common bile duct dilation and 1 cm soft tissue nodule with peripheral enhancement within the third portion of duodenum. Endoscopic ultrasound confirmed submucosal mass, but the fine needle biopsy was complicated with bleeding, and the biopsy showed normal duodenum. ERCP with stent was placed in common bile duct to relieve the obstruction. MRI abdomen showed enhancing 1.7×1.5 cm mass within the periampullary region. She was referred to surgery. Open pancreatic sleeve duodenectomy was perfomed. Pathology revealed 2.3 cm GISTs with free margin and positive for CD 117, CD 34 and smooth muscle actin, negative for S100. No mitosis per fifty high power fields reported. No adjuvant chemotherapy is indicated.

Melena is a common presentation of GISTs. However, diagnosis can be difficult because it is a submucosal mass which causes obtaining biopsy problematic. This patient was proved to have GISTs by expression of CD 117 which differentiated GISTs from leiomyoma and leiomyosarcoma. From previous reports, ulcerative lesion is concomitant findings in several cases but not presented in our case. Although GISTs at ampulla of Vater are rare, GISTs should be included in the differential diagnosis especially if the mass has an enhancement per CT scan which is characteristic of GISTs. Overall, prognosis is less favourable for small intestinal GISTs. Literatures reviews show that GISTs at Ampulla of Vater has fair prognosis. Most of them has no metastasis or lymph node involvement. Only one case had liver metastasis and died from hepatic failure.

142 Bird watching: a case of severe achalasia

JA Coba

PR Sanchez

Texas Tech University, Lubbock, TX

Case report Achalasia is chronic incurable motility disorder of the oesophagus characterised by loss of esophageal peristalsis and inadequate relaxation of the lower esophageal sphincter (LES).1,2 We present a case of severe achalasia and the complications associated. The patient is a 67 y/o male with PMH of achalasia s/p balloon dilation in 1995 presenting with chief complain of hematemesis. Two days prior to presentation, patient had been diagnosed with community-acquired pneumonia and was treated as outpatient. He was admitted to medical ICU for suspected GIB and schedule for EGD. Prior to the procedure, he experienced recurrent episodes of hematemesis that resulted in aspiration pneumonia. He subsequently required intubation with mechanical ventilation. He underwent an EGD that revealed dilation of the entire oesophagus. He required a repeat EGD 2 days later for balloon dilation and endoscopy-guided NGT placement. He was successfully extubated, but he required antibiotic treatment for aspiration pneumonia. He demonstrated clinical improvement, thus, an esophagram was performed which showed the oesophagus with massive dilation and tortuosity and stenosis at the gastroesophageal junction. General surgery was consulted who proceeded with outpatient manometry and planned for laparascopic Heller myotomy with Dor fundoplication. Achalasia, although rare, is the most common esophageal motility disorder. Its underlying aetiology is unknown. The proposed pathophysiological process is described as myenteric plexus and ganglion cell degeneration in the body of the oesophagus and LES, which leads to unchallenged action by cholinergic nerves and incomplete LES relaxation. This eventually leads to esophageal dilation from mechanical elongation to accommodate food accumulation.

143 Demographic predictors of health related quality of life in caregivers and patients with chronic gastrointestinal conditions

M Casper

C Pierce

M Lynch

MJ Barnes

R Dimmitt

University of Alabama, Birmingham, AL

Purpose of study This study aimed to determine what, if any, demographic variables predict health-related quality of life (HRQoL) in children with chronic gastrointestinal conditions and their caregivers. It is understood that individuals with these conditions, and their caregivers, may have impacted HRQoL. Literature suggests that certain demographic information can be used as predictors of HRQoL.

Methods used The caregivers of 203 paediatric gastroenterology patients, age 5–17 years, were recruited for the study at the time of their scheduled upper endoscopy. Caregivers provided demographic information including age, race, employment status, and annual family income. The patient’s diagnosis and current treatment were extracted from the medical record. Caregivers were asked to complete the PedsQL Family Impact Module, providing self-reported HRQoL, as well as the caregiver-report PedsQL Generic Core Scale, providing child HRQoL.

Summary of results Analysis of caregiver self-report indicates that the caregiver HRQoL scores were predicted by caregiver sex (b=0.20, t=2.79, p<0.01), annual family income (b=0.142, t=1.98, p=0.05), and caregiver employment status (b=-.26, t=−3.59, p<0.01). Further, the child HRQoL scores were also predicted by annual family income (b=0.17, t=2.34, p<0.05) and employment status (b=-.19, t=−2.66, p<0.01), as well as their diagnosis (b=0.21, t=2.98, p<0.01) and number of medications (b=-.20, t=−2.17, p<0.05).

Conclusions Analysis of data indicates that certain demographic factors, specifically socioeconomic factors, are predictive of both child and caregiver HRQoL scores. This suggests that beyond the child’s diagnosis and treatment, outside stressors impact the quality of life of both the caregiver and child. As these predictors have been identified, preemptive measures, such as involvement of social workers, could be taken. By doing so, the impact of outside stressors on the ability of a family to function when a child has a chronic illness can be greatly diminished.

144 Pruritis as a presentation of igg4 related disease

V Fonseca-Ferrer

M Miranda

San Juan City Hospital, San Juan, Puerto Rico

Case report IgG4-related sclerosing cholangitis (IgG4-SC) is a characteristic type of sclerosing cholangitis, with an unknown pathogenic mechanism. Patients with IgG4-SC display increased serum IgG4 levels and dense infiltration of IgG4-positive plasma cells with extensive fibrosis in the bile duct wall. IgG4-SC is frequently associated with autoimmune pancreatitis and is commonly observed in elderly men. Obstructive jaundice, pancreatitis, weight lost and salivary involvement are frequently observed. The diffuse cholangiographic abnormalities observed in IgG4-SC may resemble those observed in primary sclerosing cholangitis (PSC), and the presence of segmental stenosis suggests cholangiocarcinoma (CC). IGG4 levels more than 135 mg/dl are 80% sensitive and 86% specific for diagnosis. A 71-year-old man with T2DM, chronic kidney disease stage III, osteoporosis and cholelithiasis presents to his primary care physician due to pruritus of 2 weeks duration. Pruritus was constant and not associated with food intake. No fever, rash, weight loss, jaundice, dizziness, abdominal pain, nausea, or bleeding. Physical examination was unremarkable. Initial laboratory work up showed elevated liver enzymes and elevated alkaline phosphate. MRCP presented intrahepatic and extrahepatic ductal dilations that suggested primary sclerosis cholangitis (PSC). In the ERCP patient was found with strictures consistent with PSC. Cytology and biopsies from biliary strictures were negative for malignancy or PSC. IgG4 levels, schistosomiasis and strongyloides were ordered for further evaluation. IgG4 levels were found to be considerably elevated, more than 300 mg/dl. Patient was diagnosed with IgG4-SC. Azathioprine and prednisone was started with significant improvement. Even through, he did not have pancreatitis or jaundice as seen in IgG4-SC, it is imperative to identify IgG4-SC in patients with suspected PSC due different prognoses between these conditions. Among patients with bile duct radiographs that look like PSC, those who have elevated serum IgG4 levels have a much more severe course of disease; these patients are more likely to die or require transplantation than patients who have normal IgG4 serum levels. Fortunately, patients with IgG4-associated disease are often very responsive to steroid therapy, which is not the case with PSC.

145 Dark stools as an initial presentation of lung cancer

D Hidalgo

S Gaddam

M Khalid

J Phemister

M Young

East Tennessee State University, Johnson City, TN

Case report Lung cancer is the most frequently diagnosed malignancy and the leading cause of cancer mortality worldwide. While the preferred sites of lung cancer metastasis are brain, liver, adrenal glands and bone, the gastrointestinal (GI) tract is an unusual site of spreading.

The latest reports estimate that the incidence of gastrointestinal metastases from lung cancer ranges between 0.5% and 10% and only 0.2% to 0.5% are gastric metastases. As patients are usually asymptomatic, most of this information has been obtained from post mortem analysis.

We report a case of an 80 years-old male with past medical history remarkable for chronic obstructive pulmonary disease, essential hypertension and smoking, who presented to the emergency room after ‘passing out’. He had two episodes of syncope and reported he has been weaker than normal due to shortness of breath for the last couple of weeks. He also complained of a 6 months history of dark stools but no active bleeding.

On admission labs were remarkable for haemoglobin of 8.2 g/dl. Once the patient was stabilised, he underwent an Esophagogastroduodenoscopy (EGD) that showed a 5 cm large, friable and ulcerated mass at the junction of the antrum and gastric body towards the lesser curvature. Biopsies were taken and the immunohistochemistry revealed the tumour was positive for cytokeratin AE1/AE3, cytokeratin 7, TTF-1, napsin and MOC-31. These findings showed a poorly differentiated adenocarcinoma. The morphologic and immunostain findings were consistent with a metastasis from a lung primary.

When a metastatic tumour is found in the gastric tract, it is most commonly metastatic melanoma, carcinoma of the cervix uteri, ovary, or breast. A study published in 1975 reviewed 1010 autopsies of patients with cancer, 17 cases were gastric metastases (incidence of 1.7%).

More recent studies have shown a considerable increase in this number. A study of 470 patients with lung cancer identified 11.9% of GI metastasis, 5.1% of them were gastric. This same study showed that the most common histological type was adenocarcinoma, followed by squamous cell and large cell carcinoma.

Although gastric metastasis from lung cancer is very rare, GI manifestations should be always taken seriously, with EGD and PET scan being very useful in the diagnosis of GI metastasis.

146 Serious complications from a benigh condition: cecal lipoma

D Hidalgo

S Gaddam

M Khalid

J Phemister

M Young

East Tennessee State University, Johnson City, TN

Case report A lipoma is a non-epithelial benign tumour that originates from the adipose tissue. Incidence in general population is 0.2% to 4.4% and constitutes 1.8% of all the colonic lesions. Most of these are found incidentally during surgery, colonoscopy or autopsy, as they are asymptomatic or present with nonspecific symptoms. Lipomas exceeding >2 cm are more likely to be associated with symptoms that range from mild abdominal pain to gastrointestinal bleeding, intussusception or obstruction.

This is a 63 years old female with PMH remarkable for breast cancer, hypertension and diabetes mellitus 2. She presented to the emergency room(ER) due to intense abdominal pain and nausea. An abdominal CT scan incidentally showed a 3.5×3.2 cm mass consistent with a lipoma. As her symptoms resolved spontaneously and this was a benign lesion, she was sent home. Three years later she presented to the ER with melena and abdominal pain. An upper endoscopy did not reveal any acute source of bleeding and was scheduled for a colonoscopy as an outpatient. Two months later the colonoscopy revealed a 5 cm ulcerated lipoma in the cecum. A couple hour after the procedure she came back to the ER complaining of intense mesogastric pain, nausea and vomiting. She denied fever, chills, diarrhoea or blood in stools. An abdominal CT revealed a long colonic intussusception with the cecum intussuscepting into the colon up to the splenic flexure and an increased 5.8 cm cecal lipoma as the lead point. Due to persistent symptoms, she underwent exploratory laparotomy with right hemicolectomy and anastomosis. Post operative course was uneventful and symptom-free.

Although the majority of colonic lipomas are asymptomatic, treatment is warranted in symptomatic cases. Endoscopic or surgical resection can be done based on the size and location of the lesion. In our case, given the size of the lesion (>2 cm), ulceration causing bleeding, and intense pain, surgery was considered as the best option. We recommend close monitoring and timely intervention for the treatment of symptomatic lesions. Laparoscopic tumour removal is the standard-of-care for large and symptomatic colonic lipomas. Unfortunately, our patient underwent open laparotomy with hemicolectomy and anastomosis given the large area of intussusception as well as mass size.

147 A case of pancreatic neuroendocrine tumour in an adolescent with autoimmune hepatitis

A Glinky

R Zwiener

Iglesias Escabi

B Keith

LSUHSC, New Orleans, LA

Case report Pancreatic neuroendocrine tumours (PNETs) are a rare malignancy with an incidence of less than 1 case per 1 00 000 persons per year. Neuroendocrine tumours can arise in any tissue of the body where neuroendocrine cells are found but are most commonly seen in the gastrointestinal tract, pancreas and lung. They can appear in conjunction with other syndromes such as MEN1 or Von Hippel-Lindau or in solidarity. The tumours may produce and secrete functional peptides or may be inactive and produce nothing at all. Symptomatology varies based on the product secreted by the tumour.

Autoimmune hepatitis (AIH) is an inflammatory condition of the liver marked by elevated aminotransferases. Diagnosis is often made through the exclusion of other chronic liver diseases. Hyperglobulinemia, particularly of IgG, is often seen along with IgA deficiency in children.

We present a16 year old male with history of AIH, IgA deficiency and non-alcoholic steatohepatitis on 6-mercaptopurine who presented to gastroenterology clinic for routine follow-up. He endorsed a one month history of left upper quadrant abdominal pain and 3 kg weight loss over the last four months. He reported dieting as well as decreased appetite and energy. Abdominal ultrasound was performed showing a localised hypovascular 4.5 cm mass near the tail of the pancreas. CT scan and MRI showed similar findings and also noting a concern for autoimmune pancreatitis (AIP). Further workup for AIP was negative.

The patient was referred to an advanced endoscopist at an outside hospital for endoscopic ultrasound (EUS) with fine needle aspirate. The EUS showed a well-differentiated grade I neuroendocrine tumour with strongly positive synaptophysin stain. The patient is currently undergoing further work-up for neuroendocrine tumour markers and will have a distal pancreatectomy.

Our team was unable to find any previous reports of patients with AIH later being diagnosed with PNETs. However, there have been reported cases of patients with autoimmune conditions having subsequent diagnoses of neuroendocrine tumours. Given the relative rarity of neuroendocrine tumours in paediatric patients, there are limited resources to guide treatment in this case.

148 Clostridium difficile masking biloma

A Guzman

University of South Florida, Tampa, FL

Case report Sub-hepatic bilomas typically manifest as persistent right upper quadrant or epigastric pain, abdominal distention, fever and leukocytosis1. Though bile duct injuries infrequent, they are potentially devastating complications of biliary tract surgery and high index of suspicion should be maintained in patients presenting with abdominal pain post-cholecystectomy. Here we present a case of a biloma masked by Clostridium difficile colitis.

A 69 year-old male with a history of hypertension, alcohol abuse and obesity presented with shortness of breath, subjective fevers, and abdominal pain. He was recently treated for gangrenous cholecystitis requiring emergent cholecystectomy and 7 day course of Augmentin. He reported no change in abdominal pain since procedure, though developed 2 days of non-bloody, watery stools.

Physical exam showed a soft, diffusely tender abdomen with well healing incisions at the right upper quadrant, hyperactive bowel sounds and a palpable liver. Vitals were stable with temperature elevation of 100.3 F. On laboratory, there was mild leukocytosis (10.6), transaminitis, and significantly elevated inflammatory markers (CRP=27.7 mg/dL, ProCalcitonin=1.24 ng/mL). Stool studies were positive for C diff. CXR unremarkable.

Patient was admitted and placed on oral vancomycin. He developed a supraventricular tachycardia that resolved with adenosine. Further imaging revealed a large complex fluid and gas bubble collection in the gallbladder fossa suspicious for post cholecystectomy abscess or biloma. A biliary drain was placed and 250 mL bilious fluid was aspirated, cultures growing E. coli. Patient was placed on IV antibiotics. HIDA scan showed no evidence of active biliary leak. Further evaluation by ERCP demonstrated a leak from cystic duct stump and a biliary stent was placed.

A biloma is a sub-hepatic bile collection outside of the biliary tree caused by iatrogenic or traumatic causes. Bile duct injuries are infrequent but potentially devastating complications of biliary tract surgery and have become more common since the introduction of laparoscopic cholecystectomy2. Evidence of hepatomegaly on exam prompted further investigation, ultimately leading to detection of the biloma.

149 Superior mesenteric artery syndrome: a rare but real cause of abdominal pain

J Johnson1

C Cantrell2

R Smalligan1

S Malhotra1

1UAB School of Medicine, Huntsville, AL

2UAB School of Medicine, Birmingham, AL

Case report A 40 yo CM with a history of chronic abdominal pain (treated with narcotics) and malnutrition presented with recurrent severe symptoms to the hospital. Chart review included multiple upper endoscopies showing only mild gastritis, an ultrasound showing no signs of acute or chronic cholecystitis, a normal HIDA scan, an elective cholecystectomy, and multiple normal colonoscopies. Urine tox screen was negative for cannabis. Infectious causes had been excluded. He presented within 24 hours of an outpatient repeat upper endoscopy with nausea and vomiting and CT revealed a small bowel obstruction with a transition point over the 3rd portion of the duodenum. This was confirmed by upper GI series. He was diagnosed with superior mesenteric artery (SMA) syndrome with an aortomesenteric angle of 18 degrees. After the obstruction resolved he was treated with a non-surgical approach of positioning in the left lateral position after meals to promote weight gain. This allowed for weight gain and resolution of symptoms including complete cessation of narcotic use.

Discussion Recurrent abdominal pain is an extremely common complaint in both the inpatient and outpatient setting and drug-seeking behaviour is always a concern. Since the pain is frequently self-limiting, the underlying cause often goes undiagnosed. SMA syndrome has been shrouded in controversy in the literature due to its non-specific symptoms, its rarity, and the fact that some patients do not improve despite aggressive therapy. The aetiology is believed to be compression of the 3rd portion of the duodenum due to a reduced angle between the SMA and the aorta leading to compression, obstruction, and other symptoms. Treatment is conservative initially, with a variety of surgical procedures available if needed including dividing the ligament of Treitz (Strong’s procedure), gastrojejunostomy or duodenojejunostomy. This case reminds physicians to maintain and open mind and to think through a full differential diagnosis when evaluating chronic abdominal pain patients since making the correct diagnosis can be life-changing. This patient has made a remarkable recovery with conservative measures and no longer seeks narcotics.

150 Malignant transformation of type i choledochal cyst into papillary biliary neoplasm associated with a history of anabolic steroid use

TV Joshi

LS Engel

S Walvekar

LSU Health Sciences Centre, New Orleans, LA

Introduction Choledochal cysts are congenital malformations and can be classified into four types. The type 1 lesion is the usual form. Type 1 is defined as an extrahepatic ductal dilatation. Malignant transformation can occur in 10% to 30% of choledochol cysts.

Case A 27 year old man who had no previous past medical history presented with a 2 week history of pruritus, jaundice, and a notable absence of abdominal pain. He also reported dark urine, pale stools, indigestion as well as nausea and vomiting. Upon further investigation he described a rigorous diet and exercise regimen which resulted in the loss of 15 pounds, extensive anabolic steroids abuse, use of testosterone boosting supplements, and a10 pack/year smoking history. Ultrasound and ERCP revealed a dilated proximal bile duct with an irregular mass classified as Choledochal Cyst (type 1). Biopsy was unsuccessful, however cytology brushings were obtained. The patient underwent cyst excision. Intraoperatively, a mass in the medial duct attached to the portal vein was noted. Complete mass excision, portal node dissection, and partial portal vein dissection was performed. Biliary-enteric continuity was restored with a Roux-en-y hepaticojejunostomy. The cytology results from the ERCP brushings identified the mass as a malignant adenocarcinoma with a rare papillary configuration. Surgical pathology specimens confirmed this result indicating a differentiated adenocarcinoma with negative biopsied lymph nodes.

Discussion We describe investigations leading to diagnosis of intraductal papillary adenocarcinoma. Animal studies suggest the possibility of expression of androgen receptors on bile canalicular tissue and the possible carcinogenic effect of tobacco leading to the development of papillary adenocarcinoma Our patient’s history of anabolic steroids abuse, androgenic hormone boosting supplements, smoking history may have played a significant part in the development of his cancer at such an early age.

151 Interesting case of intra papillary mucinous neoplasm of bile duct

M Kanaa

EK Addo-Yobo

M Khalid

S Arikapudi

J Yorke

M Young

ETSU, Johnson City, TN

Case report 81-year-old female with no significant past medical history was found to have elevated liver enzymes on a routine follow up clinic visit followed by abdominal Computed Tomography showed evidence of intrahepatic and extrahepatic bile duct dilation with no evidence of calcified stones. She had an Endoscope Retrograde Cholangio-pancreaticography(ERCP) that revealed distal common bile duct stone (CBD) and CBD dilatation. The stone was removed and a spyglass showed a nonfriable, villas, hypertrophic appearing lesion with proximal upstream dilated left hepatic duct with normal appearing mucosa. Multiple biopsies were taken with the spybite biopsy forceps. The pathology analysis was suggestive of intra-ductal papillary neoplasm of the bile duct (IPNB), with high-grade dysplasia. Approximately 1 month after the ERCP, the patient presented with jaundice and pruritus that was refractory to medications. She had another ERCP with placement of 2 stents. A follow up MRI showed the malignancy started at the level of the common hepatic duct and extended into the proximal aspects of the right and left hepatic biliary ducts. The tumour extended into the left hepatic lobe parenchyma, the medial and lateral segments. Referral to oncology was made, however she was not a surgical candidate due to the tumour burden.

Intra-ductal papillary mucinous neoplasms (IPMN) are a very rare group of machine producing tumours that originates from epithelial cells. IPMNs of the pancreas are well known; however, subset of IPMNs that involves the bile ducts (IPNB) is unusual. Because IPNB is a precursor to invasive carcinoma, surgery remains the treatment of choice in both non-invasive and invasive once the IPN-B is suspected or diagnosed. Awareness of this condition along with diagnostic modalities such as cholangioscopy Early recognition is essential because there is evidence that complete surgical resection has a good survival rate even when malignant transformation has occurred.

152 Referral, evaluation, and management patterns at the alabama aerodigestive program

R Kassel1

AC Kraus2

B Crawford3

A Chapman3

L Boehm3

N Smith4

B Kulbersh4

G Hector1

W Brian4

R Dimmitt1

WT Harris1

1University of Alabama Birmingham, Birmingham, AL

2UT Southwestern, Dallas, TX

3Children’s of Alabama, Birmingham, AL

4Pediatric ENT Associates, Birmingham, AL

Purpose of study The Alabama Aerodigestive Program was founded in 2012 to meet the multidisciplinary needs of children with complex, chronic airway and digestive concerns. Currently available literature describing referral diagnoses and diagnostic evaluation of Aerodigestive patients is limited.

We seek to report the evaluation and management of referrals to a single-centre Aerodigestive Program.

Methods used We performed a retrospective chart review of the first 200 patients referred to the Alabama Aerodigestive Program from 2012–2014.

Summary of results Mean age at referral was 41 months. The most common indications for referral were gastroesophageal reflux (77%), chronic cough (67%), and dysphagia (56%). Most patients (83%) were evaluated by all four disciplines: otolaryngology (ENT), pulmonology, gastroenterology, and speech pathology. Approximately 2/3 of patients underwent an endoscopic airway evaluation by flexible bronchoscopy (64%), and 70% with direct laryngoscopy and bronchoscopy (DLB). Over half had an esophagogastroduodenoscopy (EGD, 58%). Endoscopic airway examination frequently revealed bronchitis (43%), tracheomalacia (41%), laryngomalacia (34%), and bronchomalacia (33%). A laryngeal cleft was identified in 12% of evaluations. EGD and pH/impedance studies were typically normal (66% and 70% respectively), but 16% of EGD biopsies found eosinophilic esophagitis (EoE). Reflux was only found in 11% of patients. Swallow dysfunction was common, present in 58% of patients undergoing video fluoroscopic swallowing evaluation. Nearly half (49%) of polysomnograms indicated obstructive sleep apnea (OSA).

Conclusions Children referred to The Alabama Aerodigestive Program represent a broad spectrum of medically complex children. Most have airway abnormalities and/or swallow dysfunction. Findings of laryngeal cleft and EoE are increased in this population.

154 A rare case of ectopic pancreatic tissue within barrett’s oesophagus

P Laoveeravat

S Suchartlikitwong

T Mingbunjerdsuk

P Chariyawong

Y Vorakunthada

A Rakvit

Texas Tech University Health Sciences Centre, Lubbock, TX

Background Ectopic pancreatic tissue can be found in stomach but rarely in oesophagus. It is reported to be in paediatric or teenaged patients with the belief that it is congenital in origin. The patients with ectopic pancreatic tissue mostly presented with epigastric pain due to pancreatic enzyme secretion or mass effect.

Case presentation We present a case of 69-year-old female presented with heartburn for more than five years. She denied any abdominal pain, weight loss, or loss of appetite. She underwent esophagogastroduodenoscopy (EGD) in the past, and was found to have 2 cm Barrett’s oesophagus at 38–40 cm from incisors. Biopsy showed columnar mucosa and chronic inflammation without dysplasia. Her GERD symptoms are well controlled with omeprazole 40 mg daily. Follow-up EGD at 2 years showed the same endoscopic findings. Biopsy of the Barret’s oesophagus showed focal ectopic pancreas at squamous-columnar junction with foveolar hyperplasia.

Discussion Ectopic pancreatic tissue at oesophagus can be found very rare in adults with Barrett’s oesophagus. Specifically, pancreatic acinar tissue, one of cell types in pancreas, is existed 3% of adults with Barrett’s oesophagus. There are some postulations that it is from metaplastic process of Barrett’s oesophagus but more likely from congenital process without correlation to Barrett’s oesophagus. The risk of development to malignant tissue is still unknown. We still need to observe and monitor the tissue changes by endoscopy and tissue biopsy.

155 Pneumoperitoneum: a case for conservative management

A Mahajan

P Sankhyan

K Muhammad

T Bhandari

East Tennessee State University, Johnson City, TN

Case report A 69 year old male was admitted for elective left foot subtalar joint fusion. His post-op course was complicated by severe constipation. He denied any nausea, vomiting and tolerated his diet well. On post-op day 4, he had mild generalised abdominal discomfort with minimal tenderness without rigidity or rebound tenderness and stable vital signs. CT abdomen (figure 1) showed pneumoperitneum with a large amount of faeces in colon and rectum. All labs were normal. Surgery recommended conservative management and he was discharged on post-op day 5.

Pneumoperitoneum generally signifies perforation of gastrointestinal (GI) tract and these patients present with an acute abdomen. It is a conditioned reflex for the surgeons to proceed with laparotomy in such cases. Pneumoperitoneum without peritonitis or non surgical pneumoperitoneum (NSP) is a rare entity, where laparotomy is not a necessity. Mularski et al identified 96 cases of NSP from MEDLINE database, of which 45 had surgical exploration without any evidence of perforated viscus. Common etiologies include tension pneumothorax, mechanical ventilation, peritoneal dialysis catheter placement and post GI endoscopic procedures. Yamana et al described first case of NSP due to severe constipation where emergent laparotomy didn’t reveal any evidence of viscus perforation.

Our case highlights the importance of conservative management in carefully selected patients with NSP and development of a good bowel regimen early in the post-op period.

Abstract 155 Figure 1

CT abdomen showed pneumoperitneum with a large amount of faeces in colon and rectum

156 Associations between changes in feeding behaviours and psychosocial functioning in paediatric patients with feeding disorders and their families following an 8-week intensive feeding program

ME Mastin1,2

MJ Barnes1

AJ Melton1

1University of Alabama at Birmingham, Birmingham, AL

2Children’s of Alabama, Birmingham, AL

Purpose of study Children with complex medical and developmental issues treated for feeding disorders in multidisciplinary intensive outpatient programs show success. Few studies have reported quantitative group results following treatment. Given the relative dearth of information regarding outcomes following intensive outpatient treatment, the present study examined associations between changes in body mass index, feeding behaviours, and psychosocial functioning in paediatric patients with feeding disorders and their families following an 8 week intensive feeding program.

Methods used Caregivers of 22 patients (mean age=5.50 years) completed pre- and post-treatment measures of feeding behaviours and psychosocial function including the Behavioural Paediatrics Feeding Assessment Scale (BPFAS), Paediatric Assessment Scale for Severe Feeding Problems (PASSFP), Paediatric Quality of Life (PedsQL) General and Family Impact Modules, and the Parenting Stress Index (PSI). Anthropometric outcomes were abstracted from the medical record. Paired samples t-tests evaluated differences between pre- and post-treatment measures. Pearson correlations assessed associations between change scores computed for relevant variables. Data collection is ongoing.

Summary of results Maladaptive mealtime behaviours improved on the BPFAS [t(21)=11.80, p<0.001] and PASSFP [t(20)=−8.84, p<0.001]. Family Impact [t(21)=−2.52, p=0.020] and PedsQL [t(17)=−3.32, p=0.004] also showed improvements. No other variables met significance although all change scores indicated improvements post-treatment with the exception of parenting stress that showed a slight increase. Correlations amongst change scores indicate that reductions in maladaptive feeding behaviours are associated with increases in family quality of life (r=−0.565).

Conclusions Results support the benefits of an intensive feeding program for children with severe feeding disorders. The only variable to show no improvement following treatment was parenting stress which may reflect the increased responsibility placed on caregivers at the end of treatment to maintain their child’s feeding protocol outside the program.

157 Liver imaging reporting and data system (li-rads) in patients at high risk for hepatocellular carcinoma

BP McCullar1,2

A Appelbaum3

JK Phillips1,3

A Weir1,3

B Waters1,3

1University of Tennessee, Memphis, TN

2Baptist Medical Group, Memphis, TN

3VA Medical Centre, Memphis, TN

Purpose of study Hepatocellular Carcinoma (HCC) may be diagnosed radiographically without the need for biopsy if the typical imaging features are present. The American College of Radiology endorsed the Liver Imaging Reporting and Data System (LI-RADS) algorithm with the goal of reducing variability in lesion interpretation through standardisation and improving communication with clinicians. This study utilised LI-RADS to retrospectively analyse screening scans prior to the diagnosis of HCC to determine if this system could provide earlier detection.

Methods used Following IRB approval, a retrospective chart review was performed at the Memphis VA Medical Centre on patients with HCC and benign liver nodules between 2009–2014. Patients with HCC who had surveillance CT images performed 6 to 13 months prior to their diagnosis of HCC were reviewed. Also identified were patients with benign liver nodules undergoing surveillance CTs who did not develop HCC with two year follow-up. Two Radiologists scored each CT according to the LI-RADS diagnostic algorithm.

Summary of results 70 nodules were reviewed by two Radiologists. 42 nodules were in patients who developed HCC and 28 nodules remained benign. The sensitivity for predicting eventual HCC was 64.3%–69% and specificity was 75%–82.1%. LI-RADS accuracy was 71.4%. The false-negative rate was 31.0%–35.7% and the false-positive rate was 17.9%–25%. The Radiologists agreed in scoring of 58 of the 70 nodules. The kappa statistic was 0.5992 which indicated moderate agreement.

Conclusions LI-RADS was shown to have a good diagnostic accuracy for surveillance CTs in terms of determining the risk of HCC. Utilising LI-RADS scoring would have provided an earlier suspicion of HCC in over 65% of nodules. Such information could lead to closer follow-up and additional imaging, resulting in an earlier diagnosis of malignancy.

158 Relations between health behaviours, body composition and disease status in paediatric patients with inflammatory bowel disease

AJ Melton

M Lynch

MD Lisenby

MJ Barnes

University of Alabama at Birmingham, Birmingham, AL

Purpose of study Lean body mass (LBM) deficits in youth with Inflammatory Bowel Disease (IBD) are well-established and persist despite achievement of remission and restoration of body mass. LBM deficits are associated with both short and long-term health outcomes including sarcopenia, metabolic dysregulation, and development of osteopenia/osteoporosis. The LBM deficits are multifactorial in nature though largely explained by inflammatory processes, inadequate dietary intake, and increased energy needs. In addition, physical activity (PA) may also play a role in LBM deficits. Unfortunately, PA and diet in youth with IBD have been understudied. The present cross-sectional study describes relations between PA, diet, body composition, and disease status in a sample of youth with IBD.

Methods used 40 patients with IBD aged 8–17 (M=14.58 years; 60% female; 78% Caucasian; 60% Crohn’s) completed the study. Measures included: moderate to vigorous PA (MVPA; Godin Leisure Time Exercise Questionnaire), Diet (4 day Food Record), Body Composition and Bone Mineral Density (BMD; Dual-energy X-ray Absorptiometry), and Disease Status (Physician Global Assessment). Analyses included independent samples t-test for between group differences and two-tailed pearson correlations to evaluate relations between variables across groups. Data collection is ongoing.

Summary of results No differences were found on variables based on group. Correlational analyses (n=40) revealed that greater MVPA was associated with higher LBM (r=0.39) and lower fat mass (r=−0.38). Average percent protein intake was associated with older age (r=0.38), lower percent carbohydrate intake (r=−0.72) and higher BMD (r=0.37). Less active disease was associated with higher BMI (r=−0.38), higher BMD (r=−0.46), and higher LBM (r=−0.48). Interestingly, disease status was not associated with health behaviour engagement.

Conclusions The present study suggests the need for further research into PA and diet to promote health and development outcomes for paediatric patients with IBD. Harnessing health behaviours to compliment treatment modalities may lead to improvements in body composition that in turn may mitigate long-term health outcomes associated with IBD.

159 An unusual aetiology of acute recurrent pancreatitis

N Menaker

DJ Askenazi

University of Alabama-Birmingham, Birmingham, AL

Introduction Acute recurrent pancreatitis is an uncommon paediatric disease which can predispose patients to chronic pancreatitis and islet cell dysfunction. Thus, it is critical to evaluate anatomic and medically correctable etiologies to reduce patient morbidity. Hypercalcemia is a recognised cause of pancreatitis particularly in the setting of primary hyperparathyroidism, but other causes of hypercalcemia including thyrotoxicosis must be considered.

Case presentation A 15-year-old Caucasian female with a history of renal sarcoma status post nephrectomy with chronic kidney disease IV presented as a transfer from an outside hospital for the second time in two months with an elevated lipase (1100 U/L) and epigastric abdominal pain, consistent with acute recurrent pancreatitis. The patient was hypercalcemic (12.7 mg/dL) with normal triglycerides and ethanol levels. Abdominal ultrasound was without evidence of gallstones. Magnetic resonance cholangiopancreatography revealed pancreatic divisum, and a genetic pancreatitis panel was collected. Medication review did not reveal any high-risk agents for drug-induced pancreatitis.

The patient’s hypercalcemia was confirmed, and an intact parathyroid hormone (PTH) was low-normal (10 pg/mL) indicating a non-PTH mediated process. Vitamin D and PTHrP levels were unremarkable, but TSH was decreased (<0.03 iU/mL) and free thyroxine (T4) was elevated (3.56 ng/dL) consistent with hyperthyroidism. Thyroid stimulating immunoglobulin was increased (12 U/L), and radioactive iodine study showed diffuse uptake consistent with Graves’ disease. The patient was initiated on atenolol and methimazole before a successful thyroidectomy. Outpatient follow-up revealed normalisation of calcium and free T4 levels, and the genetic pancreatitis panel returned normal.

Discussion This case highlights the importance of a thorough evaluation of anatomic and medical causes of acute recurrent pancreatitis. When hypercalcemia induced pancreatitis is suspected, an investigation into the aetiology of hypercalcemia that focuses on differentiating PTH-driven from non-PTH mediated processes is necessary. This report lends insight that hypercalcemia induced pancreatitis can be caused by thyrotoxicosis as suggested by the resolution of hypercalcemia and pancreatitis via medical and surgical treatment of hyperthyroidism.

160 Human immunodeficiency virus associated gastroparesis

T Mingbunjerdsuk

P Laoveeravat

S Suchartlikitwong

P Ratanasrimetha

A Rakvit

TTUHSC, Lubbock, TX

Case report Gastroparesis is a syndrome of delayed gastric emptying in the absence of mechanical obstruction. Multiple conditions have been described causing delayed gastric emptying. Among common etiologies, many viral infections have been associated with the occurrence of gastric stasis and severe dysautonomia.

We describe a 51 year-old African American woman with HIV infection for 33 years presented with abdominal bloating and esophageal dysphagia. She has been compliance with antiretroviral treatment. Barium swallow study and upper endoscopy showed normal findings. Gastric emptying study revealed 252 min emptying time.

Human Immunodeficiency Virus (HIV) associated gastroparesis has not been well demonstrated. HIV infection has been associated with autonomic dysfunction result in delayed gastric emptying. Whether mechanism is secondary to viral infection or immune systems remains unclear. HIV-associated gastroparesis can become manifest at any stage of the disease. Treatment with dietary modification and prokinetic agents have shown to improve quality of life.

161 A ‘screwed up’ case of hematemesis

A Mirza

N Salagundla

JP Garrido

TTUHSC, Amarillo, TX

Case report 43 yo male with PMH of cervical stenosis s/p anterior cervical discectomy and decompression of C3-C6. Presented with fever, dysphagia and vomiting blood; a 2 cm screw came up in his vomit. The screw was confirmed to be a part of the ACFD. X ray of the neck showed intact hardware, however one screw was missing. Gastrograffin swallow did not show any leakage. EGD showed soft tissue swelling, but no perforation. The mechanism of screw dislodgement was thought to be slow chronic extravasation. Conservative management was opted. Patient improved and was discharged. Outpatient follow up showed recovery.

Discussion ACDF is the standard treatment for cervical trauma, tumours and DJD. Complications of ACDF include donor sight morbidity, graft dislocation, etc. Pharyngeoeophageal perforation is an uncommon complication, with an incident rate of less than 2%. Most of these injuries heal spontaneously, the risk of mediastinitis, sepsis, and death are present. Most perforations are identified immediately. Delayed perforations may occur due to esophageal wall compression leading to ischemia, abscess, and ultimately perforation. For contained leaks and no systemic symptoms, conservative management recommended. The incidence of delayed complications after ACFD is low however it is important to recognise it early due to associated morbidity and mortality. Patients with hematemesis, dysphagia or neck pain who have a history of cervical spine hardware should be evaluated for hardware related complications.

Abstract 161 Figure 1

X-ray of the neck showed intact hardware, however one screw was missing, screw was confirmed to be a part of the ACFD

162 Collagenous gastritis a rare and underdiagnosed entity

A Mirza

S Prakash

N Salagundla

JP Garrido

TTUHSC, Amarillo, TX

Case report 64 yo female with PMH of diabetes, asthma, Hashimoto thyroiditis and sarcoidosis. Reports symptoms of dyspepsia for long time. EGD done 8 and 3 years prior showed chronic gastritis; biopsies were negative for malignancy. She presented again due to dyspepsia and 20 lb weight loss. EGD showed nodular atrophic gastritis. Biopsy results revealed aggregates of giant cells in the basal lamina propria, superficial chronic gastritis, subepithilial collagen deposition and surface epithilial degeneration. Colonoscopy was normal.

Discussion Collagenous gastritis is a rare clinical entity and is commonly present in the paediatric population. In adults, is associated with colleganous colitis. The aetiology of collagenous gastritis is not clear and as such there is no specific treatment. In this case, the patient has a history of hypothyroidism, and sarcoidosis. It has been previously suggested that collagenous gastritis and colitis are associated with other autoimmune diseases, but no specific association with sarcoidosis has been reported. We know from literature that collagenous gastritis may not be a primary pathology but possibly an abnormal response to an underlying trigger. Given that this patient had underlying chronic gastritis without any pathological evidence of collagenous gastritis in the previous 2 EGDs, the question remains if her autoimmune condition contributed towards her abnormal immune response leading to collagenous gastritis.

We recommend reporting more cases of collagenous gastritis so we can have a better understanding of the disease and in turn develop a successful treatment regime.

Abstract 162 Figure 1

Collagenous gastritis

163 Case report of hepatic abscess in an adolescent with crohn’s disease

J Nelson

M Shaukat

El Halabi

J Grunow

F Ramji

University of Oklahoma Health Sciences Centre, Oklahoma City, OK

Case report This patient is a 14-year-old male with past medical history significant for Crohn’s disease of the terminal ileum who presented to the ER for acute abdominal pain and fever. Four weeks prior to presentation, he had had diffuse abdominal pain, fatigue, headache, subjective fever and chills, non-bloody and non-bilious emesis, as well as constipation and an associated 10 pound weight loss after vacationing in Colorado. He was started on a prednisone taper for a presumed Crohn’s disease flare. Symptoms resolved, but on the last day of his prednisone taper he developed fever to 103F and right upper quadrant abdominal pain. He presented to the emergency room where labs were significant for leukocytosis with left shift, and an elevated ESR and CRP. Abdominal ultrasound indicated hypoechoic lesion in liver that was 6.5×4.2×7.6 cm. CT scan was consistent with hepatic abscess and showed multiple areas of wall thickening prominently in the terminal ileum, with a possible mucosal ulceration in the terminal ileum. On exam, his abdomen was mildly distended and he was tender to palpation over his RUQ with guarding. He was started on IV flagyl and IV rocephin prior to drainage of 60ccs of purulent material the following day. His abscess culture grew Streptococcus intermedius. Flagyl was stopped. He was continued on rocephin daily until repeat abdominal ultrasound showed significant improvement of his hepatic abscess, which totaled 30 days.

Discussion While hepatobiliary diseases are common extraintestinal manifestations of IBD, the development of hepatic abscesses is uncommon. Hepatic abscesses are more common in Crohn’s disease than in ulcerative colitis, and typically present in patients with active Crohn’s but may be the initial presenting complaint as well. This case illustrates the need to complete a thorough workup in a patient with Crohn’s disease presenting with abdominal pain and fever to rule out hepatic abscess prior to starting treatment for a presumed Crohn’s flare.

164 Celiac artery compression syndrome: an under-diagnosed cause of abdominal pain: presentations and outcomes

J Nichols1

B Davis1

I Al-Bayati1

M Ghaleb2

J Diaz1

R McCallum1

1Texas Tech Health Science Centre El Paso Paul L. Foster School of Medicine, El Paso, TX

2University Medical Centre El Paso, El Paso, TX

Purpose of study Celiac artery compression syndrome (CACS), an uncommon disorder due to compression of the celiac artery (CA) by the median arcuate ligament (MAL), presents unexplained severe upper abdominal pain. This study presents three cases.

Methods used Three CACS cases are summarised with chief complaint, diagnostic studies, therapies, and outcomes.

Summary of results Case 1: A 38-year-old female presented with unexplained upper abdominal pain refractory to traditional therapies. Doppler ultrasound (DU) showed a peak systolic velocity (PSV) of 213 cm/s and end diastolic velocity (EDV) of 57.8 cm/s on inspiration while expirational PSV and EDV were 323 cm/s and 103 cm/s, respectively. Abdominal aortogram (AA) was diagnostic for CACS at >70% compression of CA on expiration. Intra-operative DU velocities normalised after surgical release of MAL with expirational PSV and EDV at 178 cm/s and 52.0 cm/s, respectively. At 2 year follow-up, there was >80% pain relief, and a repeat AA was normal.

Case 2: A 55-year-old female presented with severe epigastric pain requiring narcotics and unexplained by standard diagnostic studies. DU velocities and AA findings were typical of CACS including >50% narrowing on expiration. Endoscopic ultrasound-guided celiac plexus block (ECB) provided transient relief, and surgical release of MAL was confirmed by intra-operative DU. Over a 3 year follow-up, a repeat AA study was normal with 75% relief of abdominal pain being achieved.

Case 3: A 21-year-old female presented with epigastric pain of enigmatic aetiology. DU velocities suggested CACS, and then AA revealed diagnostic CA narrowing. Surgical release of MAL was confirmed with intra-operative DU normalisation, and pain relief was 100% at 3 month follow-up.

Conclusions CACS:

  • Consider when patient’s epigastric pain is disproportionate to objective findings.

  • Initial screening by DU reveals typical CA flow velocities, while diagnosis is confirmed by >50% narrowing on AA during expiration.

  • ECB provides brief symptomatic relief, possibly predictive of full resolution after surgical release of MAL.

  • Celiac plexus compression and not blood flow obstruction explains the clinical spectrum.

165 Outcomes of incidentally found pancreatic cysts

L Parsa

S Sonti

CR Tombazzi

C Tombazzi

UTHSC, Germantown, TN

Purpose of study The prevalence of pancreatic cysts among U.S population varies between 3%–15% and increases to 15%–25% after the age of 70. The risk of malignant transformation of an asymptomatic pancreatic cyst is estimated to be 0.24%/yr. The risk is even lower if no change in characteristics over five years. The aim of our study is to find the incidence of malignancy in incidentally found pancreatic cysts and to evaluate the accuracy of endoscopic ultrasound in predicting malignancy.

Methods used We conducted a retrospective chart review of all incidentally found pancreatic cysts who underwent endoscopic ultrasound (EUS) between the 1/1/2011 until present date at our VA Medical Centre. We included 30 patients. Data gathered included demographics, cyst features on the initial diagnostic study, and changes found on surveillance.

Summary of results Out of 30 patients, we lost two patients to follow up. On initial evaluation with EUS/FNA, two cysts were found to be mucinous cystic neoplasms (MCN), one to be a serous cystic adenoma, and one pancreatic adenocarcinoma all of which were confirmed after surgery. Out of the remaining 24 patients followed, only one patient developed pancreatic cancer. This patient’s initial evaluation didn’t show worrisome criteria, however it showed increase in size over a three year follow-up; he underwent surgery and a diagnosis of malignant intraductal papillary mucinous neoplasm (IPMN) was made. An additional patient that underwent surgery due to increasing cyst size revealed serous cystadenoma. Twenty two patients with no worrisome criteria by EUS have been followed for a mean duration of 48 months. None of them have developed cancer. Overall EUS was able to differentiate benign from malignant cyst in all patients.

Conclusions Our study showed that endoscopic ultrasound is a valuable tool in both initial diagnosis as well as follow-up of pancreatic cysts. We should be concerned of even a single worrisome feature as one of our patients developed malignancy only with one worrisome feature during follow-up.

166 Symptom presentation of paediatric patients with eosinophilic esophagitis: new diagnosis vs follow-up

C Pierce

W Casper

M Lynch

MJ Barnes

R Dimmitt

University of Alabama, Birmingham, AL

Purpose of study The study examined the symptom presentation of paediatric patients newly diagnosed with Eosinophilic Esophagitis (EoE) compared to patients with EoE requiring a follow-up endoscopy. Given treatment, it was hypothesised that the group receiving a follow-up scope would present with improved symptom presentation as compared to patients who are newly diagnosed with EoE.

Methods used 49 paediatric patients with EoE (mean 11.33 years) were recruited at the time of their upper endoscopy. 18 patients were newly diagnosed, while 31 were undergoing a follow-up scope. Caregivers completed the PedsQL EoE Symptoms Scales, recording how much of a problem 10 symptoms of EoE have been for their child over the past 7 days. Medical information was gathered via medical record.

Summary of results Results indicate that while histological results (i.e., eos/hpf) were similar, patients with EoE requiring a follow-up scope reported an overall symptom experience that was significantly more severe than patients newly diagnosed with EoE after controlling for patient age, race, and sex (F(1,43)=4.55, p<0.05). Patients with EoE undergoing a follow-up scope identified chest pain (2(2)=6.75, p<0.05) and needing more time to eat (2(2)=10.71, p<0.01) as a significantly greater problems than patients newly diagnosed with EoE. There were no significant differences in the other EoE symptoms (i.e., stomach ache, dysphagia, etc.) between groups.

Conclusions The symptom presentation of paediatric patients with EoE requiring a follow-up scope was significantly worse but histological results were similar to than those newly diagnosed. While all patients requiring a follow up scope were being treated for EoE, adherence was not assessed. Given the cross sectional design of the study, it is not possible to determine the progression of symptoms. Physicians should continue to be aware of the possible change and/or increase in symptom presentation over time to monitor the degree to which treatment is successfully managing symptoms.

167 Dios: a difficult case of intestinal obstruction in a cystic fibrosis patient

A Pogribny1

R Zwiener1

P Arias2

1LSUHSC, New Orleans, LA

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

Case report Distal Intestinal Obstruction Syndrome (DIOS) is a complication in cystic fibrosis (CF), where viscid mucofecalent material becomes obstructed in the distal small bowel, typically the ileocecum. Pancreatic insufficiency, history of abdominal surgery, history of meconium ileus, and severe CF genotypes place patients at highest risk. Patients typically present with acute abdominal pain and radiographical evidence showing faecal loading in the right lower quadrant. Aggressive medical management with laxative or mucolytic therapy will often relieve the obstruction; however, the severity of obstruction, signs of perforation or intestinal ischemia, or clinical worsening may require invasive methods for relief.

We present the case of a 12 year old male with a history of cystic fibrosis with minimal lung disease, but with pancreatic insufficiency requiring exogenous pancreatic enzymes who presented on two occasions to the ER with abdominal pain and constipation. He was initially treated with a Fleet’s enema which provided little relief and returned several days later with worsening abdominal pain, distension, and persistent emesis. Due to the location of the obstruction in the distal small intestine, directed therapy was needed to reach the occlusion. Radiologically-guided gastrografin enema was attempted twice unsuccessfully. Due to the extent of obstruction, progression of abdominal distension, and limited improvement, surgery was involved. The patient was taken to the OR for a diverting ileostomy with a conduit for directed n-acetylcysteine lavage. His course was complicated by septic shock and aspiration of gastric contents leading to ARDS requiring an extended antibiotic course, multiple pressors, and mechanical ventilation. Continued n-acetylcysteine directed therapy into the ileostomy successfully relieved the obstruction. After a long ICU course requiring significant supportive therapy, the patient recovered fully.

With aggressive medical management utilising a variation of laxatives or mucolytics, most cases of DIOS may be treated medically. Progression to severe obstruction, perforation, or ischemia prompt surgical intervention. Surgical risks include intra-abdominal infection, aspiration of gastric contents, and septic shock.

168 Simple gastrointestinal bleed turned out to be a fistula from heart: very rare presentation

A Qasim

D Harper

T Johnson

M Patel

TTUHSC, Amarillo, TX

Background Penetration of the heart is uncommon which usually results from traumatic injuries.Even more uncommon is to develop a fistula between myocardium and GI tract.We describe a very rare presentation of Ventriculo-gastric fistula in a patient with esophageal cancer.

Case report A 77 years old male patient,h/o esophageal carcinoma presented with severe UGIB.had h/o esophagectomy and jejunal esophagogastric interposition along with radiation and chemotherapy in 1982.He was unstable, BP of 60/40 and HR 120. OG Tube yields 2 L of dark red coloured blood.Initial Hb was 3.9. Because of instability,he was intubated and transferred to ICU. Massive transfusion protocol was initiated to maintain BP. An emergent EGD revealed jejunum (interposed between oesophagus and stomach) full of blood. Stomach also full of blood.Source of bleeding was not clear. Emergent angiogram of superior mesentric artery and celiac trunk did not show source of bleeding.Then CTA of abdomen was done which showed a tracking of contrast from inferior wall of left ventricle through diaphragm into the stomach.The possibility of ventriculo-gastric fistula was sought. After 12 hours, patient became hemodynamically stable and stopped bleeding.Echocardiogram showed inferior wall akinesia.Cardiac cath showed normal coronaries.Ventriculogram showed inferior wall akinesia with aneurysm formation but no extravasation of contrast (Most likely as patient stopped bleeding). The site of aneurysm formation without coronary artery disease was indirectly suggestive of the site of ventriculogastric fistula. Patient was transferred to higher level of care for surgical correction of fistula.

Discussion Our case is unique because it is extremely rare to diagnose this condition in an alive patient. We think that this patient developed ventricular-gastric fistula as a complication to his previous surgery. We think that extravasation of blood happened during the diastolic phase and fistula obliterated during the systolic phase resulting in relatively slower bleeding giving time to stabilise the patient. There are only few cases reported in literature. Outcome of these cases is very poor and mostly diagnosis is made during autopsy.

Conclusion Ventriculo-gastric fistula can present as a very rare cause of severe UGIB in a patient with previous upper GI surgeries.

169 Hepatic encephalopathy with rapid degeneration into hepatcerebral degeneration

SK Prieto

T Mingbunjerdsuk

M Quirch

TTUHSC, Lubbock, TX

Background Hepatic encephalopathy develops acutely in patients with liver dysfunction and is reversible with proper treatment. Multiple episodes can cause a degeneration into a persistent irreversible condition called acquired hepatocerebral degeneration (AHD). Clinical manifestations can include neuropsychiatric symptoms, extrapyramidal symptoms or both. Magnetic resonance imaging (MRI) demonstrates cerebral, basal ganglia, and cerebellar damage.

While hepatic encephalopathy is commonly reported in patients with advanced liver failure, AHD is rare and estimated to occur in about 1% of cirrhotic patients. The pathogenesis is not clear but it has been proposed that advanced liver failure causes deposits of toxic metabolites in the brain. Ammonia, gamma-aminobutyric acid receptors, altered amino acids, neurotransmitters, short-chain fatty acids, and manganese deposition all likely play a role.

Case report A 52-year-old man with hepatitis C and alcoholic liver cirrhosis was admitted to the intensive care unit with gastrointestinal haemorrhage and haemodynamic instability. Upper endoscopy revealed gastroesophageal varices with stigmata of recent bleeding and moderate portal hypertensive gastropathy. He was scheduled for transjugular intrahepatic portosystemic shunt placement, however, his mental status declined and he developed seizure-like activity. Labs showed an elevated ammonia level. He was treated aggressively for acute hepatic encephalopathy but continued to be obtunded after the ammonia level normalised. MRI and electroencephalogram were consistent with severe metabolic encephalopathy. He remained in a state of persistent impaired consciousness and acquired hepatocerebral degeneration was diagnosed. Treatments were unsuccessful and his neurological status never recovered.

Discussion The clinical presentations of AHD vary widely making it difficult to diagnose. The most common symptoms are extrapyramidal such as focal dystonia, dysarthria and choreoathetosis. Occasionally neuropsychiatric manifestations occur like lethargy, excessive somnolence and dementia. Impaired consciousness and coma-like states have only been reported in a few case reports. This rare presentation of AHD shows that there is much more to learn about the pathogenesis of AHD.

170 A rare case of gastro splenic fistula arising from diffuse large b cell lymphoma

N Salagundla

S Siddiqui

A Mirza

A Islam

Texas Tech University HSC Amarillo, Amarillo, TX

Case report A 55 year-old Caucasian male with PMH of HTN, MDD and GERD presented with melena for 3 weeks with LUQ abdominal pain and diarrhoea, up to 10 bowel movements/day, 13 lbs weight loss along with frequent subjective fever and night sweats. Social history/habits: Brief smoking history of 8 pack-years. No chronic alcohol or drug use. Vitals were within normal limits. Exam revealed fullness of LUQ without tenderness. Labs: include WBC: 17 100 with 85%neutrophils, 7% lymphocytes. Hgb 6.5 g/dL, Plt 648 k, LDH 501. CT scan of chest/abdomen/pelvis has shown 11×11.8 cm necrotic mass near stomach, spleen, and pancreatic tail with large air-fluid level in spleen and gastric wall. Bulky retroperitoneal lymphadenopathy present. CT-guided retroperitoneal para-aortic lymph node biopsy pathology and histology:100% Ki-67. Negative stain for BCL2. Flow cytometry indicated CD20, CD19, and CD10 positivity, consistent with diffuse large B cell lymphoma of monoclonal origin. Surgical exploration with drainage of intra-abdominal abscess was performed, notable gastro splenic fistula and tumour involvement of left lobe of liver, posterior wall of stomach, and distal pancreas, arising from spleen.

Discussion Gastrosplenic fistulas are rarely reported, but when occurring, tend to be associated with malignant processes. Abdominal mass with air-fluid levels can indicate tumour necrosis but can often indicate abscess collection due to GI tract communication. Here we report a middle-aged male who presented with upper GI bleeding found to have a large upper abdominal mass involving adjacent organs. CT-guided lymph node biopsy indicated non-Hodgkin lymphoma (NHL), diffuse large B-cell lymphoma (DLBCL) subtype. Patient had subsequent resection of tumour and involved organs. After recovery from surgery, Initial treatment involved R-EPOCH (rituximab, etoposide, prednisone, Oncovin, cyclophosphamide, and doxorubicin). R-EPOCH shown to be superior to R-CHOP for high-intermediate IPI. Patient showed initial response with decreasing LDH. Surgical drainage with initial IV antibiotics and correction of fistula are typically necessary. Gastro splenic fistulas, while rare, may present with symptoms of GI bleeding. Current literature suggest primary resection of tumour involvement and treatment with chemotherapy.

171 Aberrant right subclavian artery: a rare cause of dysphagia

C Saraceni

W Kwan

TV Joshi

M Spera

S Landreneau

LSUHSC, New Orleans, LA

Introduction Aberrant right subclavian artery is the abnormal involution of the right 4th aortic branch and proximal right dorsal aorta. The retroesophageal course of the right subclavian artery behind the oesophagus, although usually asymptomatic, may cause compression and a type of dysphagia known as dysphagia lursoria.

Case description A 50 year old female with a medical history of von Willebrand disease and hypertension presents with a complaint of several weeks of progressive dysphagia. Her dysphagia began with solid foods and progressed to include liquids. Three days prior to presentation, she could not tolerate any solids or liquids by mouth and would have immediate regurgitation. The patient admits to about 60 lbs weight loss in the past two months.

Follow-up course CT neck angiography revealed a congenitally anomalous aortic arch with a retroesophageal aberrant right subclavian artery reaching as far superiorly as the T1 vertebral body. Barium esophagram revealed mild extrinsic mass effect on the posterior aspect of the upper oesophagus EGD revealed a normal appearing oesophagus, stomach, and duodenum with no eosinophilic components on biopsy. Esophageal manometry revealed a normal LES with normal relaxation.

Discussion Most patients with aberrant right subclavian arteries remain symptom-free throughout their lifetimes. Childhood disease usually presents as recurrent pulmonary infections and respiratory abnormalities. Possible mechanisms of adult onset disease including age related increased esophageal rigidity, right subclavian aneurysm formation, and elongation of the aorta.

Barium swallow may reveal a characteristic diagonal impression in the oesophagus at the level of 3rd-4th vertebra. EGD may reveal a pulsating mass at around the same level. Esophageal manometry may reveal a high-pressure zone 25–30 cm from the nose. CT angiography, angiography of the aortic arch, or endoscopic ultrasound may be used for definitive diagnosis.

Initial treatment should be conservative management using prokinetic or antireflux drugs. Surgical treatment may be attempted in those who do not respond to conservative management. In patients unsuitable for a surgical procedure, endoscopic dilation may temporarily relieve symptoms.

172 Aberrant right subclavian artery: a rare cause of dysphagia

C Saraceni

W Kwan

LS Engel

M Spera

S Landreneau

LSU Health Sciences Centre, New Orleans, LA

Introduction Aberrant right subclavian artery (also known as Arteria Lusoria) is the most common congenital anomaly of the aortic arch occurring in 0.5% to 1.8% of the population. The aberrant artery crosses midline behind the oesophagus (80%), between the trachea and oesophagus (15%), or anterior to the trachea (5%). The retroesophageal course of the right subclavian artery, although usually asymptomatic, may cause compression and a type of dysphagia known as dysphagia lursoria.

Case A 50 year old woman with a medical history of von Willebrand disease, hypertension, asthma, and peripheral neuropathy presented with several weeks of progressive dysphagia associated with heaviness in her chest and 60 lb weight loss. Her dysphagia began with solid foods and progressed such that three days prior to presentation, she could no longer tolerate solids or liquids. CT neck angiography revealed a congenitally anomalous aortic arch with a retroesophageal aberrant right subclavian artery. Barium esophagram did not reveal any intrinsic mass, polyp, diverticulum, or stricture. There was mild extrinsic mass effect on the posterior aspect of the upper oesophagus however this caused no limitation of contrast passage. An EGD was unremarkable and esophageal manometry was unremarkable with normal LES with normal relaxation.

Discussion Most patients with aberrant right subclavian arteries remain symptom-free throughout their lifetimes.. Various proposed mechanisms for dysphagia include age related increased esophageal rigidity, right subclavian aneurysm formation, and elongation of the aorta.

Barium swallow remains an effective tool for initial evaluation usually showing a characteristic diagonal impression in the oesophagus at the level of 3rd-4th vertebra. EGD may reveal a pulsating mass around the same level. Esophageal manometry may reveal a high-pressure zone 25–30 cm from the nose. CT angiography of the aortic arch, or endoscopic ultrasound are often used for definitive diagnosis. Initial treatment with a prokinetic or anti-reflux drug may be followed by surgery or endoscopic dilation if conservative therapy fails

173 Large mass in duodenum secondary to pancreatic divisum

Y Vorakunthada

A Rakvit

Texas Tech University Health Sciences Centre Lubbock, Lubbock, TX

Case report Pancreas divisum (PD) is a common anatomical variation when there is failure of fusion of dorsal and ventral pancreatic buds during embryogenesis. Malignancy of minor duodenal papilla (MDA) is extremely rare, but cases of adenoma and carcinoid tumours have been reported. So, mass in the duodenum warrants further investigation. Here, we present a case of large mass at MDA due to PD.

A 57-year-old female presented to clinic for screening endoscopy. She had epigastric pain for many years. Laboratory investigation was remarkable for elevated WBC of 16.99 K/UL and alkaline phosphatase of 200 INT units/L. Upper endoscopy revealed large mass, suspicious for adenoma, in the duodenum. Biopsy revealed acute duodenitis. Subsequently, she underwent Endoscopic Retrograde Cholangiopancreatography that revealed PD and significant bulging of the minor papilla (MP) that prevented cannulation. Biopsy from MP was negative for malignancy. The Plan of our patient is to investigate further with Magnetic resonance cholangiopancreatography with secretin and pancreatic enzymes.

MDP is composed of accessory pancreatic duct and remnant of pancreatic tissue from dorsal pancreas. In PD, the dorsal pancreatic duct becomes prominent vessel for drainage through the MDP, resulting in hypertrophy and recurrent pancreatitis.1 However, our patient had no evidence of recurrent pancreatitis, possible from early diagnosis.

The correlation between PD and pancreatitis remained unknown but studies have demonstrated increase incidence of post ERCP pancreatitis.2 MDP enlargement should be suspected in any patient with underlying PD who presents with recurrent abdominal pain.

References

  1. . Terumi Kamisawa, et al. Clinical implications of accessory pancreatic duct. World J Gastroenterol2010;16(36):4499–4503.

  2. . Jinru Shia, et al. Adenocarcinoma of the Minor Duodenal Papilla and its precursor lesions a clinical and pathologic study. Am J Surg Pathol2014;38:526–533.

Abstract 173 Figure 1

(A) Duondenal mass, EGD, (B) Mass at minor papilla, ERCP, and (C) Stent at major papilla

174 Cytomegalovirus induced hepatitis in a patient with systemic lupus erythematosus

M Zhang

S Arikapudi

K Devani

M Young

East Tennessee State University, Johnson City, TN

Case report Human Cytomegalovirus (CMV) is a member of the herpesvirus family that is ubiquitous in the environment, causing flu like symptoms in immunocompetent patients. However, in immunocompromised patients, CMV infection may lead to the development or flare up of autoimmune diseases like Systemic Lupus Erythematosus (SLE) (Hsieh AH et al. 2011, 13: R162-10). Here, we report a 25-year-old female with history of discoid lupus admitted to hospital due to flare up. Patient presented with complaints of abdominal pain and rash. Exam revealed shallow oral ulcers and scattered patch of macular rash on face and extremities. Lab revealed pancytopenia and elevated transaminases. Serology revealed high titers of anti-nuclear antibody, anti-double stranded DNA (anti-dsDNA) antibody and low complement levels, consistent with SLE flare up. Antibody panel also revealed positive anti-smooth muscle and anti-ribonucleoprotein antibodies. Viral panel was negative for viral hepatitis and Human Immunodeficiency Virus, but revealed very high titers of CMV IgM of 236AU/ml (Normal <29.9) and positive CMV IgG. Patient was treated for SLE flare up with intravenous steroid. Patient‘s symptoms improved significantly within few days and transaminases trended down to the normal limits. CMV infection is frequently seen in patients with SLE. It may cause the onset of SLE, flare up of SLE or the virus may present as an isolated infection in patients with underlying SLE. This is due to a C terminal peptide of CMV protein pp65 that is highly immunogenic in the serum of patients with SLE and other autoimmune conditions (Hsieh AH et al. 2011, 13: R162-10). It is important for physicians to realise that there are many similarities of symptoms and overlaps between CMV infection and SLE flare (Ramos-Casals M et al. 2008 Nov;87(6):311–8). Although the consensus on treatment guideline is still not clear, it is important to identify underlying infections since the majority of SLE patients are immunocompromised and takes immune suppressive therapy. With early detection of treatable infections including herpesvirus and viral hepatitis, physicians can better treat many detrimental opportunistic infections and may shorten the flare up of rheumatologic diseases.

Health care research, quality improvement and patient safety, Joint plenary poster session and reception, 4:30 PM, Thursday, February 22, 2018

175 Utility of index hospitalisation characteristics in predicting 30-day hospital readmission in the united states

M Agarwal1

M Shah2

R Bradsher1

1UTHSC, Memphis, TN

2Montefiore Medical Centre, Bronx, NY

Purpose of study 30 day readmissions after patient’s initial hospitalisation are broadly accepted as a negative utilisation of healthcare resources. Despite this, impact of index hospitalisation characteristics on readmissions is poorly understood. Hence we studied patient characteristics and readmission outcomes in 18,520,527 patients who survived the index hospitalisation across US.

Methods used Using the 2013–2014 National Readmission Database, patient demographics, hospital and admission characteristics, and clinical comorbidity burden (measured by the Charlson Comorbidity Index) recorded at the time of admission were studied to describe the impact on 30 day readmissions. We then randomly selected 50% population to derive a predictive model and validated it in other 50% cohort.

Summary of results There were 18,520,527 hospitalizations with top five primary reasons for admissions as septicemia (5.1%), heart failure (5.0%), acute respiratory failure (3.1%), atrial fibrillation (2.8%) and acute myocardial infarction (2.7%). Among whole cohort, 10.5% (n=1,936,236) patients were readmitted within 30 days of discharge. When compared with single-admitters, re-admitters were older (mean: 62.8 years vs 55.4 years), males (46.7% vs 38.2%), public insurers (73.5% vs 59.2%) and belonged to lower socioeconomic status (29.9% vs 27.3%) (all p<0.001). Higher readmission rates were seen in patients who were admitted on weekends (21.1% vs 19.6%), emergent basis (84.0% vs 73.0%) and to non-teaching facilities (56.2% vs 55.5%) (all p<0.001). Higher comorbidity burden and longer length of stay were both significantly associated with higher 30 day readmission (all p<0.001). Our model had a good prediction ability (area under curve, c-statistics 0.70, 95% CI : 0.69 to 0.71) for 30 day readmissions in both derivation and validation cohort.

Conclusions Patient demographics, admission characteristics, and clinical comorbidity burden at the time of index hospitalisation significantly predicted 30 day readmissions. The proposed readmission predictive model can be used to guide healthcare resources and target interventions for reducing readmission among the highest-risk patients.

176 Chronic peritoneal in-dwelling catheters for the management of malignant and non-malignant ascites

J Caldwell

H Edriss

K Nugent

Texas Tech University Health Sciences Centre, Lubbock, TX

Purpose of study Ascites is a debilitating condition affecting many patients with end-stage liver disease and advanced abdominal malignancies. Serial paracentesis can reduce the symptoms of refractory ascites, but this procedure requires frequent trips to a clinic. Indwelling peritoneal catheters are an alternative which can allow these patients to manage their symptoms at home. This review aims to assess the safety and efficacy of these devices.

Methods used A literature database search was conducted to identify relevant studies which reported indwelling catheter placement in patients with chronic ascites. Inclusion criteria were articles which included at least 20 adult patients and had been published within the past 15 years. Patient demographics, indication, complication rates, and survival times were analysed.

Summary of results Fourteen studies comprising 957 patients (687 with malignancy [71.7%], 270 without [28.3%]) were reviewed. Symptom improvement was reported in all cohorts. The most common complication in patients with malignant ascites was catheter dysfunction (39/687, 5.7%). Overall infection rate for patients with malignancy was 5.4% (37/687); patients with pancreatic malignancy made up at least 70.2% (26/37) of these infection cases. The infection rate for patients with nonmalignant ascites was 12.2% (33/270), while catheter malfunction was 1.1% (3/270). In general, infection risks significantly increased for devices in place for longer than 12 weeks. Average survival time after catheter placement was 7.2 weeks for patients with malignancy and 164 weeks for patients without malignancy.

Conclusions Indwelling peritoneal catheters are an effective alternative to paracentesis for the palliation of ascites in patients with certain malignancies. Peritonitis is a concern with these devices in patients with ascites due to nonmalignant etiologies in whom prolonged use is usual, but proper implantation technique and maintenance may greatly reduce infection risks.

177 Reducing unplanned extubations in the neonatal intensive care unit – a quality improvement project

SK Chilakala1,2

AJ Talati1,2

K Willis1

K Smith2

K Conlee2

1UTHSC, Germantown, TN

2Regional One Health, Memphis, TN

Purpose of study Unplanned extubation (UE) in the Neonatal Intensive Care Unit (NICU) is a serious safety hazard and can be tied to increased risk of mortality and morbidity.There is no established best practice of securing an Endotracheal tube (ETT). The incidence of UEs within NICUs is not well described in the literature, but the associated rates are thought to range between 2–4.8 per 100 ventilator days.We aime to estimate our baseline UE rate, and implement a quality improvement initiative in our Level IV NICU to reduce our unplanned extubation rate by 50% within 6 months of implementation. We also aim for a stretched goal of achieving an UE rate below 2 per 100 ventilator days with in 1 year.

Methods used In April 2016, UE Task Force was formed in our unit and developed an interdisciplinary cause analysis tool to identify root causes and calculate incidence of UE. In November 2016, the QI was initiated in which we implemented standardised securement approach of Endotracheal tube (ETT). This included using right size Neobar, spiral taping of the ETT, re securing the tape every 3 days, 2 care givers for all the patient procedures, documentation of the position of ETT and verification with airway alert card. In addition, increasing the awareness amongst the staff by using display board showing the number of days since last UE, extra caution in airway management for infants with higher risk of UE, tracking all events and debriefing after every UE, multidisciplinary weekly respiratory rounds have been implemented.

Summary of results The Key measure used was median UE rate per 100 ventilator days. Data was collected and abstracted for 6 months prior to intervention and 11 months following the intervention. During the pre-intervention period the UE rate was 6.5. During the post intervention period, the UE rate remained 3.6 which is a 46% decrease.

Conclusions In addition to the standardised securement approach of ETT which has been shown to be effective previously, our approach of resecuring the ETT every 3 days and identifying and implementing extra caution in infants with higher risk of UE has been effective in reducing the UE rate. With ongoing staff training, education, detailed documentation and debriefing, we plan to achieve and sustain our stretched goal.

178 Piloting universal health literacy precautions in spanish speaking parents of paediatric patients

M Cooper

S Gillaspy

R Blucker

M Dunlap

Oklahoma University Health Science Centre, Oklahoma City, OK

Purpose of study The purpose of this study is to evaluate acceptability of institution of universal health literacy precautions consisting of standardised visual aids, instruction, and teachback targeting dosing of liquid medicines by Spanish speaking caregivers of paediatric patients.

Methods used We are currently in the process of recruiting a convenience sample of twenty Spanish speaking caregivers of paediatric patients between one month and twelve years of age who are receiving liquid medication at their visit. Following a visit at which liquid medication is prescribed, caregivers will be consented and administered the Newest Vital Sign (NVS) and the Short Test of Functional Health Literacy in Adults (S-TOFHLA) by a research assistant in Spanish. Caregivers will undergo a brief tutorial using print materials based on the HELPix program from NYU School of Medicine consisting of an illustration of their medication dosing in a syringe, and a log tailored to their dosing schedule. Pictogram/teachback sessions will be timed to test for possible clinic integration acceptability. Each caregiver will be given a survey regarding the universal health literacy precautions procedures. We will invite each participant in the intervention arm to return for a focus group, with the goal of having four focus groups of five participants each to review the procedures and suggest improvements to the process.

Summary of results As of October 2017 we have recruited four caregivers, and we anticipated having our full complement for the study within two months.

Conclusions From the surveys and focus groups sessions we expect to gauge acceptability of an intervention which has been shown to be effective in other settings. With this pilot data we plan to adjust the intervention if needed and transition into a larger scale efficacy trial.

179 Using electronic health records to identify adverse drug events in ambulatory care: a systematic review

C Feng1

AB McCoy2

1Tulane University School of Medicine, New Orleans, LA

2Tulane University School of Public Health and Tropical Medicine, New Orleans, LA

Purpose of study Previous work has established a high prevalence of adverse drug events (ADEs) in the ambulatory setting. We aimed to identify the methods used and determine the roles of electronic health records (EHRs) in detecting and assessing ADEs in the ambulatory setting through systematic review of published literature.

Methods used We performed a systematic literature review by searching PubMed and Google Scholar for studies on ADEs detected in the ambulatory setting involving any EHR use published before June 2017. We extracted study characteristics from included studies related to ADE detection methods for analysis.

Summary of results We identified 30 studies that evaluated ADEs in an ambulatory setting with an EHR. In 27 of the studies, EHRs were used only as the data source for ADE identification. In 2 studies, the EHR was used as both a data source and to deliver decision support to providers during order entry. In 1 study, the EHR was a source of data and generated patient safety reports that researchers used in the process of identifying ADEs. Methods of identification included manual chart review by trained nurses, pharmacists, and/or physicians; prescription review; computer monitors; electronic triggers; ICD codes; natural language processing of clinical notes; and patient phone calls and surveys. Seven of the studies provided examples of search phrases, lab values, and rules used to identify ADEs.

Conclusions Tools such as computer monitors and electronic triggers are replacing traditional chart review as means of detecting ADEs. These tools can be used with EHRs to enable researchers to better measure, characterise, and detect ADEs in the ambulatory setting. Further research is necessary and underway to measure ease of chart review in the EHR versus with traditional paper charts to determine which is most effective.

180 A global health trip examined – rural peru

H French

C Studdard

A Herren

J Cavo

T Littmann

S Ashley

D Caton

R Smalligan

UAB, Huntsville, AL

Purpose of study Designing a health care trip to a developing country can be difficult if patient needs and expectations are not known. As more students and faculty take part in short-term experiences to developing countries, having an idea of what to expect can improve patient care, satisfaction and outcomes. This study summarises recent experiences from such a trip to rural Peru.

Methods used Prior to the trip the team reviewed a global health trips ethics module. The team included 5 medical students, 1 FM resident, 1 IM resident, and 1 IM/Peds attending. Dose packs of multiple meds were prepared with instructions in Spanish. Clinics were held in five villages within a 2 hour drive of the small town of Curahuasi, Peru. Medical and vision stations were set up in a school, community centre or church as available. Patients‘ age, sex, chief complaints, BP and meds given were recorded and analysed using Excel.

Summary of results A total of 541 patients of Quechua lineage were seen during the 5 clinic days: 306 female, 186 male, (43 not recorded) with a F:M ratio of 1.7:1; average age 32, (6 mo-92 years); average adult BP 100.2/68.9 mmHg. Common complaints included parasites (101), back pain (99), vision/eye problems (91), musculoskeletal pain/OA (89), abd pain (88), gastritis/GERD (79), headaches (69), and UTI (34). Most dispensed meds were albendazole, acetaminophen, ibuprofen, omeprazole, ranitidine, multi-vitamins, iron, tinidazole among others. Less than 10 patients were referred to a local mission hospital for further rx or w/u (CHF, HTN, gall bladder dz, cervical CA). 90 pairs of reading glasses (+1-+4) were provided and over 100 pairs of sunglasses (to slow pterygium).

Conclusions The team had prepared for infectious diseases, GERD, DM, HTN, CHF, vision problems, and pain issues. Few patients had chronic diseases, likely because of their active (mostly agrarian) lifestyle, no obesity, low BPs and low-fat diet. The patients truly appreciated the personal evaluation and symptomatic rx. Many were thrilled with their reading glasses which allowed them to resume sewing, tapestry making or reading. Only a few were sick enough to be referred out. It is recommended that groups investigate and discuss expected common conditions with a local clinician if possible prior to such a trip to improve planning.

181 Anti-epileptic medication availability in rural oklahoma

BC Gruenberg

P Johnson

J Miller

I Kumar

M Deen

C Knoles

University of Oklahoma, Oklahoma City, OK

Purpose of study It’s imperative that rural hospitals have access to appropriate medications to effectively abort status epilepticus as treating status epilepticus is time-sensitive. The objective of this study was to identify the availability of anti-epileptic drugs (AEDs) on formulary, and quantify the number of Stage I, II, and III AEDs available.

Methods used All Oklahoma hospitals in towns of less than 50 000 people were contacted and asked to participate in an anonymous phone survey about the availability of AEDs in their hospital.

Summary of results In rural Oklahoma only four hospitals have all of the recommended medications; although 96% have at least one preferred medication from each Stage. The most common medications available were from Stage I and III with 19% of responding hospitals reporting one or less preferred Stage II medications.

Conclusions There is a disproportionate availability of Stage I and III AEDs compared to Stage II.

Abstract 181 Table 1

Treatment stage and name of each medicine in this study

Number of hospitals which have the medication in parentheses.

Abstract 181 Figure 1

Number of medications available per stage as reported by hospitals (responses rate=95.5%)

182 Identification of high blood pressure during well-child and obesity encounters in a paediatric teaching clinic

C Hays

N Connolly

University of Oklahoma Health Sciences Centre, Oklahoma City, OK

Purpose of study Approximately 3.5% of children have clinical hypertension; this prevalence increases to nearly 25% in those overweight or obese. Identifying and appropriately managing elevated blood pressure in children can help reduce serious adverse health effects related to hypertension in both childhood and adulthood. The 2017 AAP Clinical Practice Guideline outlines recommendations for routine blood pressure screening during well-child visits and other healthcare encounters to identify primary hypertension and asymptomatic secondary hypertension. Clinical practice guidelines should guide routine clinic processes to insure quality health care and to provide evidence-based education regarding blood pressure management for paediatric residents. The purpose of this study is to evaluate blood pressure measurement practices and identification of elevated blood pressure during well-child and obesity follow-up appointments in the University of Oklahoma (OU) Sooner Paediatrics first-year resident outpatient teaching clinic.

Methods used Using 2016–2017 billing records, 20 well-child appointments and 20 obesity follow-up appointments will be selected for each month. Retrospective chart review using the electronic health record will be used to determine the following for each visit: if blood pressure was measured, if elevated blood pressure was present, and if elevated blood pressure was acknowledged. Standard statistical methods will be used to analyse the data. Qualitative data involving the initial management of any identified elevated blood pressure will also be recorded.

Summary of results Results from the study will identify any missed opportunities in several areas: routine screening of blood pressure, identification of elevated blood pressure, and management/follow-up for patients with elevated blood pressure. Data from this study is currently being collected.

Conclusions The analysis of the survey results will help the OU Sooner Paediatrics first-year resident clinic recognise missed opportunities for identification and management of elevated blood pressure. This will open discussion regarding potential quality improvement related to nursing/clinic procedures and resident education in order to meet clinical guidelines, provide quality care, and help prevent co-morbidities associated with childhood hypertension.

183 Super-utilizers: characteristics of paediatric patients that frequently visit the emergency room

C Jacobs

A Tufton

B Casey

K Wojcicki

M Benak

W Morgan

LSUHSC, Metairie, LA

Purpose of study Providing healthcare to children in the United States amid rising costs has become a highly debated issue. ‘Hot-spotting’ is a method of analysing patterns of super-utilizers of the healthcare system consume and receive services in an effort to decrease costs and improve the quality of patient care by providing super-utilizers with alternative, affordable healthcare options.

Methods used We reviewed 200 patients with four or more visits (defined as super-utilizers) to one paediatric ER over the course of one year. Patients seen by the hematology-oncology service, or who were admitted to the intensive care unit at any point over the course of the same year were excluded from the study. Data were collected on time of visits, diagnosis, whether the patient had a PCP, history of chronic medical conditions, demographic information, and other medical information, and were compared to a separate group of patients who had only one ER visit over the course of the same year.

Summary of results Super-utilizers had an average age of 5.5 years, with 50% of the patients under the age of 3. 45% of the patients lived in the same 3 zip codes. The average time of visit was 2:30 on a weekday, and less than 5% were admitted to the hospital for closer monitoring. Over 80% had a documented primary care physician, and 30% had at least one chronic medical condition; 20% had a prior diagnosis of asthma. There was no statistical significance in admission rates, ages, or time of visit between super-utilizers and patients with a single ER visit over this one year period.

Conclusions More data and further analyses are needed to determine the reasons these patients consistently chose an ER visit instead of going to their PCP for minor health concerns. By understanding why these super-utilizers are not selecting to visit primary care physicians we hope to improve physician-patient communications, and patient education by addressing the issues that are currently influencing their decisions to go directly to the ER. The goal is to lower healthcare costs by ensuring these patients make appropriate healthcare choices to receive affordable, quality healthcare from a primary care physician.

184 All-terrain vehicle injuries in 2006 and 2016: determining demographics to initiate interventions

K Jeffries

A Burks

M Nichols

W King

K Monroe

University of Alabama, Birmingham, AL

Purpose of study Over the past decade, the number of all-terrain vehicle (ATV) related injuries treated in United States emergency departments has decreased by thirty-three percent with nearly 1 00 000 injuries in 2016. AAP guidelines state that ATV operators should be at least 16 years of age. Yet, children under the age of 15 continue to represent nearly a third of all ATV-related injuries.

Methods used In this retrospective study, medical records of an urban paediatric children’s hospital were queried for ATV-related injuries. Relevant demographic information including sex, age, ethnicity, type of injury, month of injury and zip code of residency were obtained using EMR queries and chart review from 2006 and 2016. Inclusion criteria included children less than 18 years of age and an Emergency Department visit for an ATV-related injury. Data were entered into Excel spreadsheet for comparisons. Pearson correlation coefficient was used to compare rates of ATV-related injuries and rates of ED annual census.

Summary of results A total of 47 children were seen in the study ED in 2006 and 105 were seen in same ED in 2016. The median age of children seen in 2006 was 9 years old (SD ±3.99) while the median age in 2016 was 12 years old (SD ±3.88). These children were primarily males (78% in 2006, 60% in 2016) and nearly half of them live in the same region (43% in 2006, 56% in 2016). Based on the dramatic increase from 2006 to 2016, we looked at ATV-related injuries from 2000 onward and found strong positive correlation between year and ATV injury visit rate (adjusted for number of ED visits in that particular year) (r=0.86, r^2=0.74), with a significant increase from 2012 to 2016.

Conclusions The total number of children evaluated for ATV injuries in study ED more than doubled within 10 years. This demographic information illustrates a dramatic rise in ATV-related injuries seen in our ED which interestingly appears to be counter to the gradual decline in national treated injuries over the same time period. We also note a higher than expected increase in ATV-related injuries in our ED per volume. Using this information, we can help define where and with whom specific interventions will be most effective in reducing the number of ATV-related injuries.

185 Characterisation of young children presenting to the emergency department for mental health complaints

K Mallicoat

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

Purpose of study The primary objective of this study was to characterise children less than 10 years of age who presented to a paediatric emergency department for mental health complaints.

Methods used One researcher reviewed medical records of children less than 10 years old who presented to Children’s of Alabama emergency department between January 2016 and May 2016 with a mental-health-related chief complaint. We then categorised patients based on demographic information, characteristics of the emergency department visit, and past medical and social history. Descriptive analyses were run using SAS version 9.4.

Summary of results 222 patients ages 10 years and under were seen between January and May 2016. This age group makes up 20% of all children seen in the ED for mental-health-related complaints. In this group of patients, 73% were male (n=162) and ages ranged from 3–10 years with a mean age of 7.8 years. Patients were 55% Caucasian (n=122), 42% African-American (n=94), 1% Hispanic (n=2) and 1% other ethnicity (n=3). Patient’s insurance coverage was 76% Medicaid (n=168), 18% private insurance (n=39), and 6% uninsured (n=14). Of the 219 patients treated in the ED (3 left without treatment), 45% of patients were admitted (n=100). Univariate analyses showed increased odds of admission for children with 2 or more prior psychiatric diagnoses (OR=2.33, p=0.03), a family history of psychiatric illness (OR=2.53, p<0.01), history of any previous psychiatric care (OR=2.61, p=0.01), and a history of trauma (OR=1.84, p=0.03).

Conclusions The paediatric emergency department sees a significant amount of children under age 10 for mental-health-related complaints. Nearly half of these children were admitted for psychiatric care. Several factors were found to predict admission, which reflect psychosocial influences.

186 Use of cell phones while driving among parents

K Massey1

G Spears2

W King2

K Monroe2

1Childrens Hospital of Sacred Heart, Pensacola, FL

2University of Alabama, Birmingham, AL

Purpose of study Motor vehicle crashes (MVCs) are the number one cause of death for adolescents. Distracted driving has been implicated as a major contributor to MVCs. Previous studies show that parental behaviours impact teen driving behaviours. This study evaluates cell phone use of parents at a community paediatrician office versus cell phone use of parents of adolescents in an urban Emergency Department (ED).

Methods used After approval from the UAB Institutional Review Board, parents (of children birth to 18 years) at community paediatrician offices (n=150) were surveyed as they brought their child for paediatrician visit (GROUP 1) while parents (of children 13–18 years) were surveyed during an ED visit (GROUP 2). Survey questions included how often do you use your cellphone while driving?

Summary of results A total of 150 participants were enrolled in Group One with 76% (n=114) females, 24% (n=36 male). A total of 42 participants were enrolled in Group Two with 95% Female (n=40) with 5% male (n=2). A total of 132 (88%) parents in group one reported using the cell phone while driving versus 17 (41%) of parents in group two reported using the cell phone while driving. More parents in Group one reported cell phone use while driving than parents in Group two (X2=42.6, p<0.0001).

Conclusions Previous studies have shown that parental behaviours have an impact on adolescent driving behaviours. We also know that distractions such as cell phone use put adolescent drivers at increased risk of mvc. This study shows that while many parents engage in cell phone use while driving, more parents of attending community paediatrician visit reported engaging in this high risk behaviour than did parents of adolescents in the ED. Outreach efforts to decrease high risk driving behaviour among parents is needed.

187 Donor-recipient matching in deceased donor liver transplants: analysis of outcomes using united network for organ sharing match sequence data

MG Park

J Seal

L De Gregorio

Ochsner Medical Centre, New Orleans, LA

Purpose of study A large proportion of the liver allografts at our centre are imported in many cases after being turned down by other transplant centres. We hypothesise that outcomes of transplantation with expanded criteria allografts can be optimised by selecting lower MELD low surgical risk recipients, by minimising blood loss, cold and warm ischaemic times and by maintaining haemodynamic stability during reperfusion period. To assess the effectiveness of this strategy, we analysed outcomes at our centre based on UNOS match sequence number to identify the cohort of expanded criteria grafts turned down by other transplant centres.

Methods used We conducted a single centre retrospective review of liver transplants performed at Ochsner Medical Centre from Jan 2012-Mar 2015 (n=533). The MSN, obtained from the match run for each donor, ranged from 1–7536 and was divided into quartiles. The 4th quartile (MSN 26–7536, n=133) was defined as the High MSN group and compared with quartiles 1–3 as a control group (MSN 1–25, n=400). Primary outcomes were patient and graft survival and early graft function as assessed by AST, ALT, total bilirubin and INR in the first week post-transplant.

Summary of results Higher rate of early allograft dysfuction was observed in the High MSN group as defined by Peak AST or ALT>2000 or bilirubin day 7>10 or INR day 7>2.0. The cold ischemia times for the High MSN group were significantly longer than the control. Nearly 70% of control group livers were from local OPO with the majority import livers from Share 35. Over 90% of the High MSN livers were imported from regional or national share. There was no significant difference in mean AST, ALT, bilirubin or INR between the control and High MSN groups at post-transplant day 7. There were no significant differences in patient and allograft survival between the control and High MSN groups during the early and follow-up periods. There was no significant difference in graft survival between local, regional and national share livers.

Conclusions Increasing marginal liver allografts utilisation in lower MELD recipients is possible without compromising post-transplant outcomes. Minimization of cold and warm ischemia and avoiding surgically complex recipients are important factors to avoid intra-operative and post-transplant complications.

188 Jet-injection of lidocaine for peripheral venous access in the paediatric emergency department

P Redmond

J Davis

MN Frascogna

University of Mississippi Medical Centre, Jackson, MS

Purpose of study Peripheral venous access is one of the most common causes of pain in the paediatric emergency department (PED). Among the available techniques for local anaesthesia prior to peripheral venous access, jet-injection of 1% buffered lidocaine (or ‘J-tip’) is a promising modality because it is rapid and doesn’t involve a needle. In most but not all reports in the literature, jet-injection of lidocaine has been shown to be effective for pain associated with peripheral venous access, however first attempt success rate has not been studied by means of a large prospective cohort. The primary objective of this study is to determine if using J-tip for local anaesthesia for peripheral venous access in the paediatric emergency department is associated with a reduction in first attempt success rates versus peripheral access performed without local anaesthesia. Our hypothesis is that there will not be a difference in first attempt peripheral venous access success rates between the two groups. Secondary objectives are to measure pain score differences and post-procedure complications between the cohorts.

Methods used Parents of children 6 months to 18 years old presenting to the ED and requiring intravenous (IV) access completed a survey. Nurses performing the IV placement were separated into control and exposure groups with equal ranges of experience and expertise and also completed surveys. Patients in the exposure group received 0.25 ml of 1% buffered lidocaine via jet injection prior to IV placement. Patient’s in the control group received no local anaesthesia prior to placement. First attempt success rate and pain scores were recorded. A three day follow up survey of patients was also conducted with both groups and post-procedure complications documented.

Summary of results To date, over 70 patients and nurses have been enrolled in the exposure group and 20 in the control group. Data collection is ongoing. Analysis is also to be undertaken in the next few months and preliminary results are anticipated to be ready for presentation at the SSPR conference.

Conclusions No conclusions as of today.

189 Physician overprescribing: one drug fits all?

R Renacci1

C Rolle2,3

E DeJesus2,4

1Georgetown University School of Medicine, Washington

2Orlando Immunology Centre, Orlando, FL

3Emory University, Atlanta, GA

4University of Central Florida-School of Medicine, Orlando, FL

Purpose of study Many clinical practices lack oversight of the preference and pattern of prescriptions (RXs) written by their healthcare providers (HCPs). Currently, there are no formal quality assessment (QA) tools for healthcare organisations to identify overprescribing of non-opioid drugs. We describe the impact of a single HCP-of-interest’s (HCPOI) RX behaviour at a large HIV clinic in Central Florida.

Methods used Dolutegravir (DTG) was FDA-approved as a single agent to be used in combination with other antiretrovirals (ARVs) for the treatment of HIV infection in August 2013 and as a single DTG plus abacavir/lamivudine tablet (ADL) in November 2014. A routine QA in 2014 revealed an unexpectedly high proportion of patients were being treated with a DTG-containing regimen (DCR). We subsequently conducted a retrospective chart review of all patients seen from the date of DTG approval to 12 weeks after ADL approval. We used descriptive statistics to quantify the amount of DTG RXs written by the HCPOI, and compared it to 2 other clinic HCPs.

Summary of results We reviewed 6168 clinic charts; 4096 patients had an HIV diagnosis, and 3150 met inclusion criteria. The median age was 49 years, 83% were males, and the median duration of ARV therapy was 10 years. ARVs were initiated or changed in 971/3150 (31%) patients, and 670/971 (69%) were prescribed a DCR. Of these, 511/670 (76%) were prescribed by the HCPOI (who was caring for 38% of all HIV patients). At week 12 following ADL approval, 43% (511/1197) of all HCPOI patients were on a DCR, and he had an estimated 1.9% of the nationwide ADL market share.

Conclusions The impact of a single HCP on new drug sales can be easily missed and underestimated within the flow of patients seen at a large practice. Though, the novelty of new agents could be perceived as beneficial, an appropriate risk-benefit analysis must always be conducted to protect the welfare of patients. This study describes the experience observed at an HIV clinic; however, similar scenarios could be encountered at other specialty practices and highlights the need for a practical method to identify inappropriate prescribing of non-opioid drugs.

190 Geaux well: an initiative for resident wellness

R Schexnayder

A Severio

M Lemoine

C Diaz

C Sandlin

LSUHSC, New Orleans, LA

Purpose of study Burnout is the feeling of emotional and physical exhaustion and depersonalization. There have been strong associations between physician burnout and suicidal ideation. The purpose of this study was to determine if implementation of a formal wellness program would improve resident well-being and prevent burnout. We hypothesised that through establishment of the ‘Geaux Well’ program, residents would develop an increased resilience to overcome stressors both emotionally and physically.

Methods used LSU Paediatric residents were anonymously and voluntarily surveyed using the Physician Well-Being Index, a questionnaire that evaluates for resident fatigue, burnout, stress, and overall quality of life. Residents were surveyed prior to program implementation and five months after implementation. The ‘Geaux Well’ program consisted of monthly wellness activities and conferences. Lectures focused on educating residents on stress management, coping techniques, and healthy lifestyle habits. Wellness activities served as physical and psychological outlets for residents to engage in and take a break from the work routine. In addition, residents had the opportunity to participate in social activities outside of work. Index scores pre and post-implementation were analysed to determine if overall resident wellness was improved after initiation of the ‘Geaux Well’ program.

Summary of results Pre-implementation survey results revealed that majority of residents felt burnt out from work and had been bothered by emotional problems. These results also demonstrated that most residents felt neutral to the notion that our residency program fostered an environment of wellness. Post-implementation survey results showed an improvement in residents’ total index scores. Most residents also felt the implemented curriculum showed that the residency program fostered an environment of wellness.

Conclusions Nearly fifty percent of physicians report symptoms of burnout during their career. Due to the association between physician burnout and suicidality, especially among medical trainees, many residency programs are developing curricula that promote physician wellness. Our survey results suggest that implementation of a formal resident wellness program improves resident well-being and resilience.

191 The role of patterns in follow-up testing for liver function test abnormalities

A Schreiner

P Mauldin

V Durkalski-Mauldin

W Moran

J Zhang

D Rockey

Medical University of South Carolina, Charleston, SC

Purpose of study In this study, we compared liver function test (LFT) follow-up by patterns of original LFT panel abnormality. We studied LFT panels with single versus multiple abnormalities, as well as clinical patterns: cholestatic, hepatocellular, and mixed.

Methods used We performed a retrospective cohort study of patients with abnormal liver function tests (LFTs) in a primary care clinic. LFT abnormalities were categorised by the number of abnormal analytes (single vs multiple) and the patterns of those tests. Outcomes were repeat LFT testing and the time to repeat testing.

Summary of results Of the 9545 unique patients included, 6155 (64.5%) had 1 abnormal test and 3390 (35.5%) possessed multiple abnormalities.

Patients with only one abnormal LFT component were more likely to lack follow-up (12.56%) than those with multiple abnormalities (10.53%, p=0.003, figure 1).

Patients with only abnormal AST had the lowest rates of follow-up (10.7%, table 1). For combinations of abnormal LFTs, the AST-ALT combination of initial abnormality was most often lacking repeat assessment (12.6%).

Conclusions LFTs are not only frequently abnormal, but abnormalities clinically notable by test (i.e. bilirubin) or degree (i.e.,>4 fold abnormal) are often not followed up.

Abstract 191 Table 1

Proportion of patients missing follow-up LFTs, by test with initial abnormality

Abstract 191 Figure 1

Proportion of patients missing follow-up LFT testing by number of abnormal LFT components

192 Implementating electrocardiographic monitoring during neonatal resuscitation in the delivery room: a new challenge

BA Shah

A Wlodaver

M Blunt

A Foulks

A Makkar

M McCoy

M Escobedo

E Szyld

University of Oklahoma Health Sciences Centre, Oklahoma City, OK

Purpose of study As neonatal heart rate is a vital sign used to assess the need for and response to resuscitation, measuring it rapidly, accurately and affordably is important to clinicians around the world. Therefore neonatal resuscitation program (NRP) has recently revised the guidelines regarding heart rate monitoring by electrocardiogram (ECG). Feasibility of this implementation in the delivery room has not been systemically studied. Our objective of this project was to demonstrate the processes related to implementation of cardiac monitoring by ECG as per revised NRP guidelines in the context of a quality improvement (QI) project.

Methods used Focus groups were conducted with responsible parties at a tertiary regional perinatal centre and level IV neonatal intensive care unit. An interdisciplinary needs assessment was used to identify the barriers. Implementation approaches were discussed.

Summary of results Collaboration, sharing experience, education and a dedicated leader were key to implementing NRP changes. Barriers were issues related to obtaining new equipment at the different geographic location, technological coordination, new or inexperienced staff and amount of time needed to educate staff, and the delivery room culture (table 1).

Conclusions Overcoming the barriers to implementing cardiac monitoring by ECG during neonatal resuscitation in the delivery room required changes in administrative, budgetary, logistical, educational and personal practices, and successfully accomplished through a QI multidisciplinary team approach.

Abstract 192 Table 1

Barriers and multidisciplinary team approach used to facilitate implementation of revised NRP guidelines

193 A quality improvement project to reduce bronchiolitis readmissions

J Cleavenger1

LL Sisterhen1

D Goad2

J Holt2

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

2Arkansas Childrens Hospital, Little Rock, AR

Purpose of study The aim of this project is to reduce bronchiolitis readmissions by 0.7%, from 3% to 2.3% by July 1, 2017 (PHIS data).

Methods used An interdisciplinary team identified key drivers to impact readmission rates: all patient education is delivered on a second grade level during hospital stay and caregivers should be comfortable with suctioning their infant’s nares. Interventions developed included:

  • nursing education on teaching nasal suctioning and documenting teach–back

  • an educational video was developed to teach caregivers about bronchiolitis diagnosis and management including instructions on nasal suctioning

  • posters and reminders of teach–back were displayed on the unit and

  • staff on the unit were asked to stock admission supplies with bulb syringe and saline for all respiratory admissions, to ensure that caregivers had access.

7 day readmission rates were collected monthly using data extracted from Solutions for Patient Safety (SPS) for DRG-138 Bronchiolitis and RSV Pneumonia. The data source changed from PHIS to SPS due to the delay by three months of PHIS data availability. The electronic medical record was reviewed for nursing documentation of bronchiolitis education, teach-back of nasal suctioning, and video viewed by caregiver.

Summary of results In June 2017, the median 7 day readmission rate was 1.65% (SPS). In addition, the median 7 day readmission rate over a 4 year period is 2.67% in quarter 1 of 2017 (PHIS). Nursing documentation of bronchiolitis teaching was 100% the entirety of the project. Teach-back documentation increased from 11% to 33% with a peak of 40%, and documentation of caregiver video education went from 0% to 9% with a peak of 18%.

Conclusions Linked improvement cycles resulted in modest improvements in discharge instructions, with a corresponding decrease in the median readmission rate from 3.0% to 2.67%. It is desirable that parents feel comfortable with care of an infant with bronchiolitis upon discharge. Implementing teach-back methods improves understanding and may reduce readmissions. Lessons learned included that key driver diagrams are useful tools to guide plans for interventions, and annotating run charts helps to communicate the current project status.

194 Teen driving education in a paediatric emergency department

G Spears1

B Schoen2

L Maloney2

J Smelcer2

T Thompson2

E Remington2

M Nichols1

W King1

K Monroe1

1University of Alabama, Birmingham, AL

2Childrens of Alabama, Birmingham, AL

Purpose of study In the US, the leading cause of death for adolescents is motor vehicle crashes.The aim of this project is to determine efficacy of education within a paediatric emergency department (ED) and assess current driving habits and knowledge of state law of teenagers and parents.

Methods used This IRB-approved pre-post intervention study of English-speaking, non-critically ill teenagers 13–19 years old and their parents was conducted in an urban paediatric emergency department. Pre-surveys assessed current driving habits and knowledge. Intervention was a ‘Safe Driving Toolkit’ followed by post-surveys to measure education outcomes. Pre and post survey data were then analysed and compared using Epistat

Summary of results 40 parents, 2 grandparents, and 44 teenagers were enrolled. Of the 19 participants who responded that they knew the specifics of the GDL Law, only 1 was correct on all three counts. The most common item missed was the curfew with n=4 believing it to be 8 p.m., n=14 believing it to be 9 p.m., n=23 believing it to be 10 p.m., and n=7 believing it to be 11 p.m. Sixty-nine percent of respondents correctly answered that there was to be no cell phone use while driving for teenagers under the GDL law. Sixty-three percent of participants had never heard of the law, and of that sixty-three percent, 37% had been enrolled in a driver’s education course. Chi square analysis revealed no significant difference between parents and teenagers having taken a drivers’ education course (z=1.16, p=0.11), and there was no significant difference in parents and teens who reported learning new information from this study (97.2% of surveyed reported learning new information).

Conclusions The majority of participants were not aware of GDL Law, which has been in place since 2002. Over 97% of those surveyed were given new information in intervention. There is a need for further public education of the law and safe driving habits. 61% of respondents believe curfew is earlier than the current law’s curfew. which support strenghtening the current law. Night-driving restrictions starting at 10 p.m. or earlier have been shown to have greater reductions in motor vehicle crashes involving teenagers.

195 Improving the inpatient discharge process for spanish-speaking patients: a quality improvement initiative

S Williams

AM Wolf

M Adams

B Bawa

J Freeman

ME Gutierrez

SE Mayberry

T Moore

S Stagno

University of Alabama at Birmingham, Birmingham, AL

Purpose of study As the Spanish-speaking population in the US grows, efforts to provide adequate translation of medical information must also increase. Patients and their families, regardless of which languages they speak, should always receive informative and understandable instructions, especially after an inpatient admission. In order to improve access to health care information for Spanish-speaking patients, our objective is to identify current barriers in our institution that prevent patients from receiving discharge instructions in Spanish.

Methods used This effort will be the first PDSA cycle of several which will seek to ensure that all Spanish-speaking families at our institution are provided bilingual discharge instructions. We retrospectively collected data over a four-month period on all inpatients whose families identified Spanish as their preferred language. We examined this data to identify (1) how many patients received written instructions in Spanish and (2) how many patients‘ charts had documentation of a Spanish interpreter being present during the discharge process. We then worked with Language Services and Nursing Informatics in focus groups to identify potential barriers in the discharge process.

Summary of results Of 171 patient visits in which families identified Spanish as their primary language, written Spanish discharge instructions were provided in 35.7%. Only 17.5% had documentation of a Spanish interpreter being present. There was no clear standardisation of the discharge process to ensure that patients with Spanish as their preferred language received comprehensive and intelligible instructions in Spanish.

Conclusions Spanish-speaking patients and their families are not currently being provided with adequate discharge instructions. Our second PDSA cycle will involve working with Language Services to develop standardised Spanish discharge instructions for the ten most common discharge diagnoses from our General Inpatient Paediatric Service. We will also develop a comprehensive program to increase physician and nursing awareness of the need for appropriate language interpretation for non-English-speaking patients. Data collection will begin again after implementation of the new discharge instructions.

196 Knowledge sustainment among providers after regional cme: an quality improvement initiative

C Lail

E Tomlin

C Dunn

M Elrod

D Adams

C Rogers

SM Marchegiani

Naval Medical Centre Portsmouth, Suffolk, VA

Purpose of study Knowledge sustainment is a challenge for active duty, contract, and General Schedule (GS) providers caring for patients in military treatment facilities (MTFs) who have variable access to paediatric continuing medical education (CME). Re-organising some MTFs into enhanced multi-service markets provided uniform clinical and business operations, however quality patient care also depends on provider knowledge sustainment. To meet this need and CME access as a quality improvement (QI) project for the Tidewater eMSM, we created a regional one-day paediatric symposium for MTF providers. We assessed knowledge of attendees in paediatric sub-specialty areas and measured the impact of our event on knowledge improvement and retention.

Methods used Participants were assessed prior to the symposium with paper versions and at one and two months after with provided electronic assesments. The infectious disease quiz had 7 items, while the quiz for adolescent medicine had five. All corresponded with lecture objectives. Due to absent provider names on initial assessments, differences in individual scores could not be calculated and samples were assumed independent.

Summary of results Average scores are shown in the table. One way analysis of variance yielded significant differences for each assessment between the three time points. Post hoc comparisons with Tukey’s paired test found significant differences between the pre and first post tests for both assessments (ID: p<0.01, Adol p=0.03), but sustained improvement from pre-test to second post-test for the adolescent medicine assessment only (ID p=0.12, Adol: p<0.01). Of post-test respondents, over 45% reported <10 hours of paediatric CME annually at their MTF.

Conclusions Provision of regional CME provides one method to improve medical knowledge sustainment for providers of military beneficiaries.

Abstract 196 Table 1

Average scores

Haematology and oncology, Joint plenary poster session and reception, 4:30 PM, Thursday, February 22, 2018

197 An unusual course of a recently diagnosed systemic lupus erythematosus (sle) thrombotic microangiopathy

S Ahmed

PP Kyaw

L Ngo

JP Garrido

T Vo

TTUHSC, Amarillo, TX

Case report A 28 yo Hispanic female diagnosed of SLE, IV lupus nephritis 2 weeks back from admission, on prednisone, mycophenolate, and hydroxychloroquine presented with severe anaemia, fluid overload and respiratory distress requiring intubation. Physical exam significant for BL basilar rales and pedal oedema. Lab: Hgb 7.8, BUN/Cr 58/2.7, platelets 135 k, LDH 294, haptoglobin 13, UA: 3+protein;2+hematuria, peripheral smear showed schistocytes. Patient developed anuric renal failure and fall of Hgb and platelet count shortly after admission. Emergency plasmapheresis and hemodialysis was initiated due to concern for TTP. Further workup was consistent with lupus flare with high anti-DS DNA(284) and low complement level (C3 29.6, C4 14.2) prompting initiation of high dose IV steroid and mycophenolate. Following a course of prolonged plasmapheresis and immunosuppressive therapy, cell counts and inflammatory markers improved. Ultimately ADAMTS13 level returned slightly low at 52%. Presence of lupus flare and normal ADAMSTS13 activity excluded diagnosis of TTP and therefore was more suggestive of SLE associated thrombotic microangiopathy.

Discussion Microangipathic hemolytic anaemia with thrombocytopenia immediately raises concern for TTP. Other etiologies include severe sepsis, DIC, Shiga Toxin associated HUS, ITP, SLE, malignancy, HELLP syndrome or adverse drug reaction. Our patient did not have bloody diarrhoea, mental status was difficult to assess, Beta HCG, HIV, DIC panel, HIT and Coombs tests were negative. TMA is a rare but known hematologic manifestation of SLE. Patients with SLE associated TMA often have significant proteinuria, severe renal insufficiency with mildly low ADAMTS13 activity; all as seen in our patient. TTP in contrast is characterised by milder proteinuria, less severe renal failure and very low ADAMTS13 activity. These two conditions can be difficult to distinguish. Case reports suggest survival benefit to early treatment with plasmapheresis despite normal ADAMTS13 activity. Early recognition and initiation of plasmapheresis along with high dose immunosuppressive is crucial to reduce morbidity and mortality in SLE associated TMA.

198 An unusual case of nonbacterial thrombotic endocarditis secondary to metastatic urothelial carcinoma

N Amilineni

S Bhogal

P Sankhyan

C Cook

East Tennessee State University, Johnson City, TN

Case report Nonbacterial thrombotic endocarditis (NBTE) is a constellation of noninfectious valvular lesions, most commonly on the aortic and mitral valves. It is most often seen in the setting of systemic lupus erythematosus or late stage malignancy, with the highest prevalence among those with adenocarcinoma of the colon, lung, ovary, prostate and biliary tract.

A 60 year old male with a past medical history of bilateral pulmonary emboli diagnosed four months earlier and subsequent diagnosis of urothelial carcinoma with bilateral renal masses, presented to the hospital 3 days after underdoing renal biopsy, with complaints of progressively worsening exertional dyspnea since his biopsy.

On physical exam he was tachycardic at rest with heart rate in the 130 s and electrocardiogram evidence of new atrial flutter with rapid ventricular response. Transthoracic echocardiogram showed evidence of masses on valves (tricuspid and aortic), with a confirmatory transesophageal echocardiogram (TEE) consistent with two separate 1.5 cm echodense masses on his tricuspid and aortic valves. Initial and multiple repeat blood cultures showed no evidence of pathogen growth. Retroperitoneal lymph node biopsy results at that time were positive for metastatic urothelial carcinoma. Based on TEE findings with negative blood cultures, in the setting of advanced urothelial carcinoma, the diagnosis of NBTE is considered most likely. During hospitalisation, his clinical status deteriorated rapidly with significant hypotension and pulmonary oedema resulting in acute respiratory failure. The patient expired due to hypoxemic respiratory failure.

This case illustrates an interesting presentation of a malignancy associated NBTE, with pulmonary emboli and multiple endocardial lesions without evidence of pathogens. The increased concentration of circulating cytokines seen in malignancy damages endothelium, ultimately leading to platelet activation and deposition of inflammatory molecules. These sterile platelet thrombi often affect previously undamaged valves, and up to half of NBTE cases will present solely with embolic phenomena. Patients with NBTE may appear otherwise asymptomatic without the classical symptoms of cardiac murmurs or fevers seen in infective endocarditis.

199 Tyrosine kinase inhibitor associated dilated cardiomyopathy in chronic myeloid leukaemia

N Amilineni

S Bhogal

P Sankhyan

C Cook

East Tennessee State University, Johnson City, TN

Case report Prior to the introduction of tyrosine kinase inhibitors (TKIs) as a means of therapy for chronic myeloid leukaemia (CML), outcomes were poor and the prognosis grave. While these drugs have drastically improved outcomes, they come with significant adverse effects.

A 53 year old man with a past medical history of Philadelphia chromosome-positive CML, diagnosed 14 years ago and previously managed with imatinib, presented to the hospital with acutely worsening dyspnea and peripheral oedema. 3 months earlier, he had suffered an acute blast crisis and was switched from imatinib to second generation dasatinib.

On physical exam he was noticeably short of breath with minimal exertion. Previous echocardiograms from the time of his initial CML diagnosis showed normal atrial and ventricular size and function, with an ejection fraction (EF) ranging 50%–60% and grade I diastolic dysfunction. Recently he was found to have an EF 25%–30%, with significant bi-atrial and left ventricular dilation, after which he had undergone cardiac catheterization with no evidence of coronary artery disease (CAD). This was further decreased on presentation with an EF <15% with significant grade III diastolic dysfunction. As he had no prior history of cardiovascular disease and current cardiac workup did not show any evidence of CAD or ischemia, it was determined that his cardiomyopathy was not ischaemic but instead secondary to tyrosine kinase inhibitor toxicity.

He failed to improve after initial management with aggressive intravenous diuretics and his hospital course was complicated by thrombocytopenia and anaemia requiring transfusion, after which dasatinib was discontinued. Despite maximal medical management to optimise cardiac function, his condition continued to deteriorate and he eventually entered hospice care.

Cardiotoxicity, characterised as dilated cardiomyopathy, is a rare but potentially devastating adverse effect from TKI’s used to treat CML. Up to 2%–4% of those taking TKI’s, specifically second generation dasatinib, will develop complications of cardiomyopathy with diastolic dysfunction. While the hematologic complications of TKIs have been well studied, this patient’s case illustrates some of the rarer cardiotoxic effects that may be irreversible and potentially fatal.

200 Breast cancer with metastasis to gastrointestinal tract

MR Arevalo

SK Prieto

S Ball

C Jones

Texas Tech University Health Sciences Centre, Lubbock, TX

Case report Breast cancer is the most common cancer in women. Though recent advancements have improved early diagnosis and therapy, many patients ultimately develop metastases. The most common metastases are to lymph nodes, bones, lungs, liver, and brain. Metastasis in the gastrointestinal tract has been reported as low as 1%. Despite this low overall incidence, breast cancer is the second most common cancer metastasizing to the gastrointestinal tract. Even so, the metastatic involvement of the gastrointestinal tract is not well known nor well described in medical literature.

Here we review the case of a 69-year-old African American female, whose case highlights gastrointestinal metastasis. Initially the patient presented with severe neck and back pain. X-rays were consistent with degenerative changes only. Shortly afterwards she developed postprandial diarrhoea and reportedly lost 80 pounds in a three month period. Computed tomography (CT) scan of the abdomen and pelvis showed extensive bony abnormalities suggestive of metastatic disease and magnetic resonance imaging (MRI) revealed extensive marrow infiltration suggestive of metastatic disease. Bone marrow biopsy was positive for metastatic carcinoma most consistent with breast primary (positive CK-7, GATA-3 and ER, HER2 equivocal). Anastrozole was started and the patient initially responded well to aromatase inhibitor therapy. However, the patient developed recurrent profound anaemia prompting endoscopic evaluation. Biopsies of the stomach body, ascending colon and rectum were compatible with metastatic breast cancer. Within one month, the patient developed gastrointestinal bleeding and elevated levels of CA-15–3, indicating progression of her gastrointestinal metastasis, prompting the decision to begin chemotherapy with paclitaxel.

Gastrointestinal metastasis of breast cancer poses a significant diagnostic challenge due to the nonspecific nature of symptoms. This case highlights the necessity of consideration of anaemia and gastrointestinal symptoms as potential diagnostic indicators in patients with breast cancer. Despite its rarity, additional research outlining a standard of care for patients with gastrointestinal metastasis of breast cancer is needed to improve clinical outcomes for this group of patients.

201 A rare case of optic neuritis in early stage cll

KC Arthur

S Elkins

C Bigelow

University of Mississippi Medical Centre, Jackson, MS

Case report Chronic Lymphocytic Leukaemia is the most common leukaemia in the Western world. CLL is considered a disease of the elderly with the average age of 72 years at diagnosis. CNS involvement of CLL is rare, usually occurring late in the disease course. Optic involvement is even less common, and when reported, is associated with hematologic relapse. There are few cases in the literature of optic neuritis in early CLL.

We present a 40-year-old Caucasian male with no medical history who was referred to the neuro-ophthalmologist for optic disc oedema and progressive blurry vision of several months’ duration. Extensive work up included negative ANA, CRP, ESR, ACE, HIV, RPR, Bartonella, Lyme, Zoster, ANA, and HSV. MRI brain did not show an abnormality. CSF studies were normal, and cytology was negative for malignancy. CBC revealed a WBC of 19.7 with lymphocytic predominance. Peripheral flow was consistent with CLL, and FISH revealed 13q14 deletion, portending a favourable prognosis; otherwise negative for high-risk markers. Optic involvement of CLL is definitively diagnosed by optic nerve sheath biopsy showing leukemic infiltrates. However, this procedure is not without significant risks. Given the extensive negative work-up, optic neuritis secondary to CLL was a diagnosis of exclusion.

He was then referred to our Haematology clinic where he was staged as a Rai 0 Binet A, and observation was recommended. He started 60 mg of prednisone daily by the neuro-ophthalmologist. However, after no response to steroids and rapidly progressive neuritis, the decision was made to initiate systemic treatment for CLL with fludarabine and rituximab. Optical Coherence Tomography (OCT) was used to measure Nerve Fibre Layer (NFL) loss. After 3 cycles of chemotherapy, OCT showed cessation of progression and stabilisation of disease. He completed 4 cycles of fludarabine/rituximab with no significant side effects.

Case reports of optic neuritis in early stage CLL are limited for review. Radiation therapy and intrathecal methotrexate have traditionally been used to treat leukemic infiltration of the optic nerve, but more recently, chemotherapy alone has produced good results in the return of visual acuity. We present a rare case of optic neuritis in early stage CLL in a young patient with good risk cytogenetics.

202 Diffuse large b-cell lymphoma involving lung parenchyma

C Ashangari

A Qasim

R Bharadwaj

P Tumula

JP Garrido

Texas Tech University HSC Amarillo, Amarillo, TX

Background Diffuse large B-cell lymphoma (DLBCL) is the most common form of NHL, accounting for 30 percent of newly diagnosed cases. Usual presentation of DLBC involves nodal or extranodal mass with systemic features. Lung Parenchymal involvement is an uncommon presentation of DLBC.

Case report 56 y old, male with PMH of RA and DM type 2, presented with on and off fever and night sweats since 1 month. He also had unintentional weight loss of >20 lbs for 6 months. His home medications consisted of metformin and abatacept. He was a smoker 1.5 PPD since 40 years. He was exposed to copper sulphate at work. On examination there was generalised tenderness on palpation of abdomen and spleen palpated 8 cm below the costal margin. Lab work showed pancytopenia with WBC-1.0, Hb-9.9, Platelets-49, ANC-0.1, AST-98, ALT-62, ALP-233. Hepatitis panel, quantiferon test and HIV were negative. CT thorax showed bilateral upper lobe and bibasilar infiltrates, multiple small nodules all over the lung. A 1.3 cm large nodule was found in the posterior aspect of right lung base. Patient was initially was started on empiric antibiotic treatment with cefepime, vancomycin, metronidazole and micafungin. Bone marrow biopsy and aspiration was done which was consistent with DLBCL. CT guided needle biopsy of lung nodule showed large atypical cells positive for LCA, and CD20, BCL6 positive in >30% of cells, Ki67 shows semi-quantative proliferative index of 98%. FISH negative for c–MYC. These findings consistent with diffuse B-cell lymphoma involving bone marrow, lung and liver parenchyma activated B-cell type, negative for BCL2 and negative for EBER. Patient was started on rituximab followed by gemcitabine and oxaliplatin.

Discussion Patients with RA are at increased risk of malignant lymphomas, most pronounced for diffuse large B cell lymphomas (DLBCLs), characterised by relatively frequent extranodal presentation, the most common are in stomach, CNS, bone, testis and liver. Simultaneous detection of multiple extranodal involvement at presentation is quite uncommon, with the majority of these cases characterised by gastric or intestinal disease localization. Our case is unique as we report a patient with an unusual presentation of DLBCL with significant visceral involvement including lung.

203 Concurrent jak2 positive myeloproliferative disorder and chronic myelogenous leukaemia: a novel entity?

G Bader1

D Hansen1

B Dreiling2

1University of Mississippi Medical Centre, Ridgeland, MS

2GV Montgomery VA Medical Centre, Jackson, MS

Introduction JAK2 mutation and BCR-ABL translocation have been considered mutually exclusive. However, few cases of coexisting JAK2 positive myeloproliferative disorders (MPD) and chronic myelogenous leukaemia (CML) have been reported. We describe a case of concurrent JAK2 positive myelofibrosis and CML that we have recently diagnosed.

Case Report A 75 y old male presented with weight loss, night sweats and left upper quadrant abdominal pain. Physical exam was pertinent for hepatosplenomegaly. His WBC count was 23.2×103/ mcl with neutrophil count of 21.3 and no blasts. Haemoglobin was 14.3 g/dl and platelet count 741×103/ mcl. Neutrophilia and thrombocytosis started in 2013 and 2014 respectively. Quantitative RT-PCR was positive for both b2a2 and b3a2 transcripts at 2.1% and 1.2% respectively. Imatinib was started for CML. Eight weeks later, WBC count was 16.8×103/ mcl and Platelet count 649×103/ mcl. RT-PCR was negative for BCR-ABL. The complete molecular response (CMR) without hematologic response triggered further testing. JAK2 V617F mutation was positive on peripheral blood. Bone marrow was hypercellular with proliferation of atypical megakaryocytes and widespread grade 2 reticulin fibrosis. No BCR-ABL translocation was detected by FISH. DIPSS PLUS score revealed intermediate 1 risk disease. Hydroxyurea was added while waiting mutational profile. Neutrophilia and thrombocytosis resolved four weeks later.

Discussion The patient had both CML and myelofibrosis as featured above. Persistence of neutrophilia and thrombocytosis despite CMR was due to myelofibrosis.

In most reported cases of concurrent MPD and CML, JAK2 mutation preceded BCR-ABL translocation. JAK2 mutated clone appeared to expand with BCR-ABL clone suppression. We suggest JAK2 mutation testing in CML with atypical course where major or complete molecular response are achieved without hematologic response. Alternatively, we suggest checking BCR-ABL translocation in cases of JAK2 positive MPD with CML-like features.

Conclusion Diagnostic criteria of MPD probably need to be revised to account for the possibility of co-occurrence of JAK2 mutation and BCR-ABL translocation which might be a novel clinical entity.

204 Nasopharyngeal squamous cell carcinoma presenting as chronic sinusitis

B Bajric

S Maharaj

J Verdecia

C Palacio

University of Florida, Jacksonville, FL

Case report A 54 year old African-American male with no past medical history presented with sinusitis of three months. He complained of right-sided nasal congestion, maxillary sinus tenderness, and a diffuse headache. The headache was worse when lying down. He intermittently experienced ear fullness over the same time period. He had completed three separate courses of amoxicillin, amoxicillin-clavulanate, and doxycycline with no improvement. Fluticasone and decongestants gave minimal symptomatic relief. Physical exam revealed swollen nasal turbinates, worse on the right. A right-sided middle ear effusion was indicated by a bulging tympanic membrane. Palpation over the right maxillary sinus elicited subjective tenderness. No neck mass, lymphadenopathy, or neurological deficits were appreciated. Given the chronicity of symptoms and failed antibiotic treatment, a Computed Tomography scan of the sinuses was obtained. This showed a 5 centimetre wide by 5 centimetre long mass arising from the posterior wall of the right nasopharynx, extending into the masticator space, the right cavernous and maxillary sinus, and the pterygopalatine and right middle fossa. Magnetic Resonance Imaging confirmed the mass and the skull base invasion. A biopsy of the tumour showed poorly differentiated squamous cell carcinoma. The plan at the time of discharge was primary radiotherapy.

Discussion Nasopharyngeal carcinoma is uncommon in the United States. Instead, it is endemic in Southeast Asia and North Africa. The symptoms are often difficult to recognise and can lead to a delay in diagnosis. Risk factors include tobacco and alcohol use, exposure to Epstein-Barr Virus, and early exposure to carcinogens. Tumours often remain asymptomatic for prolonged periods. The most common presentation for this cancer includes headache, double vision, facial numbness, and a neck mass. Due to varying presentations, there is often a delay or misdiagnosis for nasopharyngeal carcinoma. The tumours tend to metastasize quickly and typically invade the skull base. This case demonstrates that when a patient presents with symptoms of chronic sinusitis not responding to treatment, it is imperative to consider malignancy as a possibility.

205 Hepatoblastoma in a 9-year-old child: considerations in an older paediatric patient

L Powell

L Raney

L Caleon

M Benak

MC Velez

Louisiana State University Health Sciences Centre, New Orleans, LA

Case report Hepatoblastoma (HB) is the most common primary hepatic malignancy of early childhood, occurring mostly in those less than 5 years of age. From 1997–2012, only 13 cases were reported worldwide in children older than 5 years. While surgery remains the mainstay of therapy, less than 50% of tumours are resectable at diagnosis. Neo-adjuvant chemotherapy has led to many non-metastatic, initially unresectable tumours becoming resectable. While liver transplant offers a last resort to unresectable cases, only 25 transplants were performed from 1993–2007. The 5 year event-free survival is approximately 90% for completely resected tumours; it falls to <70% when resection is unfeasible. A 9-year-old male presented with right upper quadrant abdominal pain, anorexia, and emesis for several days. Physical exam revealed a palpable liver 10 cm below the right costal margin. A complete blood count and complete metabolic profile showed mildly elevated liver transaminases, and coagulation studies assessing liver function were normal. Computed tomography and magnetic resonance imaging demonstrated a large 9.8×10×16.2 cm hepatic mass in the right lobe of the liver, additional hepatic involvement in all segments, and inferior vena cava and portal vein intraluminal tumour extension, without distant metastasis. His alpha-fetoprotein (AFP), a tumour marker for HB, was over 195,000 ng/ml. An ultrasound-guided biopsy confirmed the diagnosis of HB, mixed fetal and embryonal epithelial type. The tumour was initially unresectable, and he received three cycles of neo-adjuvant chemotherapy with vincristine, doxorubicin, cisplatinum, and 5-fluoruracil following the Children’s Oncology Group protocol for non-metastatic HB, aiming to decrease tumour size. Unfortunately there was no significant change. He was then transferred to another institution where he successfully received a liver transplant in order to achieve remission. This case exemplifies a more extensive, aggressive disease associated with the atypical age of presentation that appears resistant to standard chemotherapy. This suggests a potentially different tumour biology and clinical behaviour in older patients with HB.

206 Choriocarcinoma syndrome

S Bhama

JC Henegan

University of Mississippi Medical Centre, Flowood, MS

Case report Choriocarcinoma syndrome is a rare condition in which patients with metastatic nonseminomatous germ cell tumours (NSGCT) with multiple large metastases and a high serum HCG concentration (>50,000 IU/L) often present with haemorrhage from metastatic sites.

To promote early recognition of this rare, life threatening condition, we report a case of a patient with choriocarcinoma syndrome who presented with symptomatic anaemia, melena and hemoptysis.

A 29 year old Caucasian male presented with left testicular swelling in June 2016. A left orchiectomy showed a localised mixed germ cell tumour (75% teratoma, 25% embryonal carcinoma). He was lost to follow up.

In August 2017 he was admitted to an ICU for melena and hemoptysis with symptomatic anaemia (Hgb 2 g/dL). Scans showed multiple tumours in his chest and abdomen. He was transfused blood and asked to follow-up with medical oncology. Two weeks later, he had hemoptysis and a haemoglobin of 3.5 g/dL. Scans showed extensive liver and lung metastases, necrotic lymphadenopathy and 7 haemorrhagic brain metastases consistent with relapsed, Stage IIIC (pT1b cN3 cM1b S3), poor risk NSGCT. Esophagogastroduodenoscopy and bronchoscopy did not show any areas of active bleeding or mass. Beta human chorionic gonadotropin was 319,520 IU/L. He was treated with cisplatin 20 mg/m2 and etoposide 100 mg/m2 on days 1–3 with bleomycin and two more days of cisplatin and etoposide added later in to cycle 1 per the GET-UG 13 protocol.

His course was complicated by a spontaneous pneumothorax requiring a chest tube placed prior to cycle 2. He tolerated the first five days of cycle 2 well but unfortunately died suddenly in his sleep and the cause of death is unknown.

Logothetis initially described choriocarcinoma syndrome in 1984, with haemorrhage at sites of metastasis containing high volume choriocarcinoma elements with significantly elevated B-HCG levels. The bleeding is hypothesised to be the result of extensive vascular invasion by syncytiotrophoblasts and cytotrophoblasts leading to early hematogenous dissemination. Haemorrhagic complications can occur immediately after the start of chemotherapy and/or in patients with rapid disease progression. Prompt recognition and early multimodal intervention including chemotherapy and surgical control of bleeding may prevent fatalities from this rare complication of NSGCT.

207 Argatroban dosing in heparin-induced thrombocytopenia in the setting of an elevated aptt due to antiphospholipid syndrome

Z Bhatti

D Manta

SUNY Upstate Medical University, Massapequa, NY

Case report Heparin-induced thrombocytopenia (HIT) is a serious, life-threatening complication which occurs in 1%–3% of patients receiving heparin. Treatment is based upon clinical suspicion, stopping heparin therapy and initiation of anticoagulation with a rapidly acting alternative non-heparin anticoagulant, such as argatroban. Argatroban is a synthetic direct thrombin inhibitor that reversibly binds to the thrombin active site. The recommendation for initial dosing of argatroban in HIT is 2 µg/kg/min, adjusted to achieve aPTTs>60 s. Our case highlights the unusual circumstance of dosing argatroban in the setting of a falsely elevated aPTT due to antiphospholipid syndrome.

A 36-year-old male with a recent history of iliac artery stenting presented to the hospital with complaints of shortness of breath. A computerised tomography angiography (CTA) of the chest showed a large acute saddle embolus. He was started on intravenous heparin. On day three, his platelet count fell over 50% of his baseline count. HIT was suspected and he was switched to argatroban. Argatroban was monitored using aPTT with a goal aPTT of >60 s. Anticardiolipin antibodies were elevated and mixing studies suggested a factor inhibitor. Due to these findings, there was concern for antiphospholipid syndrome. Approximately four days later the patient abruptly went into cardiac arrest and was prononced dead.

In antiphospholipid syndrome (APS), the autoantibodies, anticardiolipin antibodies, anti-B2-glycoprotein antibodies and lupus anticoagulant bind to the phospholipid part of the PTT reagent and the patient’s specimen does not clot. The coagulation tests are therefore falsely prolonged. In our patient with undiagnosed APS, aPTT was falsely elevated and therefore was an inaccurate measurement of anticoagulation. This case demonstrates the limitations of current treatment recommendations of argatroban in patients with antiphospholipid syndrome. One case report recommended the use of a fixed-dose argatroban regimen without laboratory monitoring as a management strategy.

Limited and inconsistent data exist about dosing patterns, efficacy, and safety of argatroban therapy in patients with HIT and an elevated baseline aPTT due to APS.

208 An obstructing duodenal mass with peritoneal involvement: not always adenocarcinoma!

C Busack

M Zaarour

R Munker

Tulane University School of Medicine, New Orleans, LA

Case report Primary small bowel neoplasms are uncommon tumours with overall poor prognosis. The predominant histological type is adenocarcinoma followed by carcinoid, lymphoma, and sarcoma. These tumours have often similar clinical, radiologic, and morphologic features making the distinction difficult without tissue examination. Peritoneal involvement can be a manifestation of adenocarcinoma known as peritoneal carcinomatosis. More uncommon is the peritoneal involvement by lymphoma, known as lymphomatosis. Herein, we report a rare case of small bowel obstruction secondary to a duodenal mass along with peritoneal involvement. Surprisingly, the diagnosis was not adenocarcinoma but lymphoma.

In August 2017, a 41 year-old Hispanic woman with no significant medical history presented with worsening nausea, abdominal pain, and 60 pound weight loss over a period of 7 months. The patient was initially admitted to the ICU with severe metabolic derangements. A contrast CT of the abdomen and pelvis showed a mass-like thickening at the level of the 2nd portion of the duodenum resulting in severe luminal narrowing. Also, peritoneal ‘carcinomatosis’ was noted. Serum CEA and Ca 19–9 levels were normal. Upper endoscopy visualised a friable obstructing mass in the 2nd portion of the duodenum that was biopsied. Pathology showed a CD20 +diffuse large B-cell lymphoma with germinal centre immunophenotype. Treatment was initiated with Rituximab, Cyclophosphamide, Doxorubicin, Vincristine and Prednisone (R-CHOP). The patient tolerated cycle 1 well, and she was discharged home with outpatient oncology follow-up.

Although, the gastrointestinal (GI) tract is the leading extranodal site of non-Hodgkin lymphoma (NHL), primary GI lymphomas remain uncommon. Most cases occur in the stomach, followed by the colon and small intestine. Primary duodenal lymphoma account for less than 2% of all cases of GI NHL. Peritoneal involvement is an extremely rare presentation of lymphoma and is often highly indistinguishable from the more common carcinomatosis seen with adenocarcinoma. Therefore, a high index of suspicion is warranted to prevent delay in diagnosis.

209 Ischaemic stroke in a child with down syndrome and acute lymphoblastic leukaemia

L Caleon

L Powell

M Benak

VA Cruz Flores

R Gardner

LSUHSC, New Orleans, LA

Case report Patients with Down Syndrome (DS) and leukaemia (ALL) have a higher incidence of treatment-related toxicity. ER, a sixteen-year-old girl with known DS, presented after 2 weeks of low grade fever, malaise, myalgias, and pallor. Her white blood count was 8970, haemoglobin was 4.6 gm%, and platelet count was 9,000, with 67% lymphoblasts. A diagnosis of pre-B ALL was made. ER was started on the COG high-risk arm for DS patients (vincristine, dexamethasone, intrathecal (IT) cytarabine (ARA) on day 1; pegylated-asparaginase (PEG-ASP) on day 5; day 8, IT methotrexate and leucovorin). Two days later, ER acutely developed right lower extremity weakness. Computed tomography (CT) of the brain had no focal findings, but magnetic resonance imaging (MRI) of the brain revealed acute infarcts in bilateral fronto-parietal areas with extensive vasculitis involving internal carotids, anterior, proximal, and posterior cerebral arteries without haemorrhage. The patient then developed dysarthria, dysphagia and right-sided paresis. Repeat brain CT showed right frontal lobe oedema. After intensive rehabilitation and high dose steroids, ER progressively improved, regaining speech and use of her extremities. She completed induction therapy without further use of PEG-ASP, and consolidation with systemic ARA and cytoxan.

Patients with DS have an incidence of ALL 10- to 20-fold greater than the general population in conjunction with worse outcomes. Treatment of ALL in DS can be problematic since there is increased sensitivity to chemotherapy. It is uncertain to what extent methotrexate played a role in causation of ER’s neurologic complications. Although PEG-ASP has also been proposed as a possible cause, there was no hypofibrinogenemia, d-dimer elevation, or other indicators of thrombotic risk. Our patient had no evidence of pre-existing neurologic problems, but it is entirely possible that preexisting conditions such as MoyaMoya, seen in DS, could have predisposed her to vasculitis once treatment started. The case highlights difficulties of treating those with DS and ALL, and underscores a need for thorough neurologic exam, and poses the question of need for brain imaging before starting therapy.

210 Graft versus host disease after syngeneic transplant – a case report and review of the literature

C Capra

N Patel

J King

R Brodell

C Milner

UMMC, Jackson, MS

Introduction Syngeneic bone marrow transplant for multiple myeloma is known to decrease treatment-related mortality while increasing progression free survival (PFS) and overall survival (OS) as compared to autologous transplant.1–3, 7 Previous studies cite the incidence of syngeneic graft-versus-host disease (sGVHD) from 0% to 20%, yet prophylaxis is not routinely used in syngeneic transplants.1–5 Herein, we report sGVHD affecting the gut and skin.

Case presentation A 63 year-old Caucasian male with an IgG Kappa Multiple Myeloma previously treated with Lenalidomide with Bortezomib and Dexamethasone and subsequent Carfilzomib with Dexamethasone treated to a complete response was referred for transplant evaluation. He had a monozygotic identical twin brother and the decision was made to proceed with syngeneic bone marrow transplant. Human leukocyte antigen (HLA)-typing confirmed a 10/10 match between the patient and donor. The patient underwent a myeloablative regimen with Melphalan followed by a transplant of 3.35×10e6 CD34 cells/kg taken directly from the donor’s unstimulated marrow. GVHD prophylaxis was deemed unnecessary. By day +10, the patient was having more than two litres of stool per day and developed a diffuse, erythematous macular rash without bullous formation. Biopsies of the duodenum, colon, and skin were all consistent with acute GVHD. With appropriate immunosuppression, the rash and diarrhoea resolved by day +19 and patient was suitable for discharge from the hospital on day +23.

Discussion Death attributed to GVHD after HLA-matched sibling and matched unrelated donor stem cell transplants are as high as 9% and 10%, respectively.8 We routinely administer GVHD prophylaxis for every allogeneic transplant, yet we do not when performing a syngeneic transplant. There are identifiable risk factors for sGVHD4 as well as a previously utilised model to predict GVHD and individualise prophylaxis.9–11 We propose the need for further investigation into which patients would benefit from GVHD prophylaxis to preserve the benefit of syngeneic transplant.

211 Testicular seminoma metastasis presenting as cardiac arrhythmia

JA Carlson

I Serrano

K Seegobin

P Reddy

University of Florida, Jacksonville, FL

Case report Testicular cancer is the most common cancer among males aged 15–35 years, with seminomas accounting for approximately 45% of all primary testicular tumours. Diagnosis is established with radical orchiectomy, which also serves as the initial treatment. In patients with seminoma limited to the testes (Stage I), orchiectomy provides a cure rate of greater than 85% if conducted with active surveillance after surgery.

A 59-year-old male smoker with history of right testicular pure seminoma, status post-radical orchiectomy one year prior, presented to the hospital with complaints of mild chest pain, palpitations, and shortness of breath. Patient stated he had experienced these symptoms intermittently for 5 months, and was unable to correlate clear precipitant factors and noted to have spontaneous resolution of these symptoms.

On exam, he was found to be tachycardic in the 130 s and an electrocardiogram showed atrial fibrillation with rapid ventricular rate. Chest x-ray revealed a new rounded, soft tissue density superior to the left hilum. Follow-up with CT-chest confirmed the 6.2 cm mass located in the left anterior/middle mediastinum which was concerning for malignancy. CT-guided biopsy was performed and cytology was sent, affirming the diagnosis of recurrent metastatic seminoma.

Oncology diagnosed this as stage III mediastinal seminoma, and the patient was started on chemotherapy with VIP (Vinblastine, Ifosfamide and Cisplatin) for 4 cycles. On day 5 of VIP treatment, he spontaneously converted to normal sinus rhythm. He was discharged on day 7 of chemotherapy with close outpatient follow-up with hematology-oncology.

This case illustrates the uncommon presentation of metastatic seminoma after treatment with radical orchiectomy. This procedure can help avoid adjuvant medications and therapies due to its excellent cure rate, as long as there is close follow-up. After careful chart review of this patient, oncology had ordered a CT abdomen/pelvis and repeat tumour markers 4 months after surgery to ensure full resolution, however, this patient did not follow up. As a result, 1 year later he was found to have stage III metastatic seminoma. This case emphasises that even though seminoma prognosis is excellent, active surveillance is required to monitor for long-term remission.

212 Cascades in coagulation and compliment

D Chander

P Rigby

LSU-HSC, Metairie, LA

Introduction Both the complement system and coagulation pathway proceed in a stepwise fashion via the activation of various soluble factors. The interaction between the complement and coagulation cascades is increasingly being seen as an important mediator in the pathophysiology of diffuse intravascular coagulopathy.

Case report A 56 year old male with a history of colon cancer presented to the Emergency Department with 2 months of abdominal pain and progressive loss of appetite. Prothrombin time (PT) was 14.6 and partial thromboplastin time (PTT) was 56.8. His white blood cell count (WBC) was elevated at 27 thousand. The day after hospital admission a CT scan of the abdomen was ordered which demonstrated a contained perforation and intraabdominal abscess. He was begun on antibiotics and his intraabdominal abscess was drained. His WBC and coagulation parameters normalised over the following hospital days.

Discussion Ratnoff and colleagues first proposed a ‘waterfall’ sequence of coagulation in 1964. The elucidation of the complement system dates even further to the end of the 19th century. Traditionally these pathways have been thought of as separate, however increasing research demonstrates key interactions between the two pathways play a role in the pathogenesis of dysfunctional coagulation. In particular, the anaphylatoxin C5a has been shown to be an important mediator of coagulation via increased tissue factor expression. We aim to describe the characteristics of Cascades and update the well-described waterfall sequence to reflect our modern understanding of the coagulation and complement pathways.

213 When things aren’t as they seem: a case of a rare lung cancer

E Dauchy

W Baumgartner

M Modica

LS Engel

LSU Health Sciences Centre, New Orleans, LA

Case report A 67 year old woman with chronic neck pain secondary to known cervical stenosis presented with acutely worsened neck pain and headache. She reported painful swallowing and palpitations, but denied chest pain or shortness of breath. While in the Emergency Department, she was noted to be in atrial fibrillation and was admitted for further workup. Physical examination was notable for a frail woman who appeared older than her stated age, an irregularly irregular pulse, and unremarkable musculoskeletal and neurologic findings. With the exception of an alkaline phosphatase of 163 U/L, labs were normal. EKG demonstrated an irregularly irregular rhythm consistent with atrial fibrillation and a chest x-ray revealed mediastinal lymphadenopathy that was new when compared to previous imaging. This was explored further with a CT of the chest and demonstrated a 2×3 cm mass in the right upper lobe of the lung with innumerable pulmonary nodules as well as a 7.5×6.2 cm mass in the right mediastinum with multiple enlarged lymph nodes. A CT of the abdomen and pelvis revealed likely metastatic disease in the liver, adrenals, kidneys and ovaries. MRI of the brain noted a mottled appearance of the cervical spine concerning for metastasis to the bone. Interventional Radiology performed a biopsy of a kidney lesion and pathological findings suggested a neuroendocrine growth pattern with high mitotic growth rate, high proliferation index (50%–60%) and positive neuroendocrine markers (CD56 and synaptophysin); consistent with metastatic large cell neuroendocrine carcinoma.

Discussion Large cell neuroendocrine carcinoma is a rare pulmonary malignancy, representing approximately 3% of all lung cancers. Diagnosis relies on careful attention to neuroendocrine features on light microscopy and immunohistochemical staining. LCNEC, like small cell lung cancer, carries a very poor prognosis. Unfortunately, there is little data available to define a standard treatment due to lack of clinical trials.

214 Catch-22: anticoagulation in a patient with itp and venous thromboembolism

E Dauchy

J Decuir

B Mathew

LS Engel

LSU Health Sciences Centre, New Orleans, LA

Case report A 62 year old man with ITP presented with three weeks of right leg swelling and pain. He denied chest pain or any recent travel. He reported chronic dyspnea on exertion, without change from baseline. He also denied any bruising or bleeding. Physical examination demonstrated a swollen right lower extremity with pitting oedema from the ankle to knee. Admission labs demonstrated a normal white blood cell count and haemoglobin and hematocrit. Platelet count was 36 000. Venous ultrasonography of the right LE was notable for deep venous thrombosis involving the popliteal, femoral and deep calf veins. CT angiogram with PE protocol demonstrated multiple bilateral pulmonary emboli. Haematology was consulted to assist with the discussion of anticoagulation in this patient. Using recommendations from the 4th Intercontinental Cooperative ITP Study Group that follow VTE treatment recommendations in cancer patients with high bleeding risk, the patient was started on prednisone 1 mg/kg and half of the therapeutic dose of low molecular weight heparin. Within three days, the patient’s platelet count increased to 1 01 000 and he suffered no bleeding events. He was eventually tapered off of steroids and was increased to full therapeutic dosing of low molecular weight heparin with no adverse events.

Discussion ITP is a rare hematologic diagnosis that affects both young and elderly patients. Some studies have suggested that the risk of VTE in ITP patient is just as high as in those with cancer, which poses treatment issues as anticoagulation is routinely contraindicated when the platelet count is less than 50 000. Furthermore, ITP patients with thrombocytopenia have been excluded from pivotal studies investigating newer anticoagulation agents and clinical data is lacking. Although more clinical investigation is needed, this case demonstrates that patients with ITP can be safely treated with anticoagulation.

215 An uncommonly considered cause of hemolytic anaemia

E Dauchy

A Muhs

LS Engel

C Hebert

LSU Health Sciences Centre, New Orleans, LA

Case report A 46 year old woman with previously treated Grave’s Disease presented to the Emergency Department with gingival bleeding when brushing her teeth, dark coloured urine, easy bruising and an unintentional 10 pound weight loss in the last six months. She also admitted to drinking 48 ounces of beer three times weekly. Admission labs were notable for a haemoglobin and hematocrit of 6.2 g/dL and 17.6 g/dL, respectively, platelet count of 163, MCV of 104 and RDW of 44%. The differential was notable for schistocytes, tear drop cells, and many hypersegmented neutrophils. Her AST was 84, ALT was 24, alkaline phosphatase was 60 and total bilirubin was 1.4. Faecal occult blood was negative. Her serum iron was 56, TIBC was 287, ferritin was 18, vitamin B12 level was 55 and folate level was 12.2 with a normal thyroid profile. Reticulocyte percent was 1.3. Haptoglobin was <30 and LDH was markedly elevated, consistent with hemolytic anaemia; direct antiglobulin test was negative. She was started on parenteral vitamin B12. EGD was notable for diffuse atrophic gastric mucosa. Intrinsic factor antibody level was equivocal, but gastrin level was elevated at 366 and parietal cell antibody level was elevated at 57.9 making this consistent with a pernicious anaemia. Biopsy samples taken during EGD demonstrated intestinal metaplasia and the presence of ECL cell hyperplasia in segments negative for gastrin staining, suggestive of autoimmune gastritis.

Discussion Pernicious anaemia is a common cause of vitamin B12 deficiency and is often associated with autoimmune gastritis. Although the precise cause of autoimmune gastritis is unknown, individuals with this condition are more likely to have a pre-existing autoimmune condition. What makes our case interesting is that our patient presented with a hemolytic anaemia picture, which was thought to be due to ineffective erythropoiesis. Our case highlights the importance of considering vitamin B12 deficiency in a patient presenting with severe anaemia and hemolysis.

216 Long term need of iv iron in patients with hereditary haemorrhagic telangiectasia

E Ellent

P Rigby

LSUHSC, New Orleans, LA

Case report A 45-year-old woman with HHT presented to the ER with recurrent epistaxis, which had been worsening over the past 6 years despite outpatient standard of care treatment. Over this course in time, this patient had 16 ER visits with 10 admissions secondary to epistaxis directly or IDA induced chest pain. The patient’s baseline haemoglobin (Hg) level is approximately 10 grams/DL, however, an average of her Hg upon presentation to the ER is 7.48 grams/DL. Ferritin ranged from 1.3 ng/mL to 75.6 ng/mL, with an average of 16.24 ng/mL. During this time, she has also had 15 units of pRBC transfused. Oral iron failed and the patient was started on intravenous (IV) iron infusions. Initially, the patient received IV iron infusions sporadically, which did not decrease her visits to ED for HHT complications. More recently, the patient was placed on scheduled bimonthly IV iron infusions. Since establishing care, this patient has received 5100 mg of IV ferumoxytol and 7300 mg of Iron Dextran in 40 months’ time.

Discussion There are 6 main forms of IV iron and pharmacokinetics vary based on preparation. Regardless of the preparation, IV iron has significant potential adverse effects such as anaphylactic reactions and infections. Clinicians must weigh the risks versus benefit, over time. Patients with HHT do have increased morbidity, however, mortality is generally not affected. In 2015, women had an average life expectancy of 81.2 years. If this patient lives to her anticipated life expectancy and continues IV iron infusions then she will need 869 more IV infusions which would total 443,088 mg of elemental iron. If her care is transitioned to monthly infusion then she will require 435 IV infusions and 221,544 mg of elemental iron.

Conclusion Patients with HHT may be destined to a lifetime of IV iron infusions. This can have an adverse quality of life as well as economic implications.

217 Isolated myelosarcoma treated as aml, with progression to mds/mpn

A Fuentes1

G LaBier2

B Boulmay1

1LSU Health Sciences Centre, Metairie, LA

2LSU, New Orleans, LA

Case report A 67-year-old woman with stage IIB lung adenocarcinoma in January 2014, in remission after cisplatin, etoposide, and radiation, presented January 2017 with lower extremity skin lesions, dismissed as insect bites. One month later, she developed persistent fevers, splenomegaly, and Coomb’s-negative hemolytic anaemia requiring recurrent transfusions. Labs included WBC 3,300/uL (3% metamyelocytes, no blasts), Hgb 6.6 gm/dL, Plt 456,000/uL, CRP 3.4 mg/dL. Rheumatologic and infectious workup was non-revealing. Bone marrow biopsy showed mild non-specific megakaryocytic and myeloid dysplasia, normal FISH/cytogenetics, negative for mutations in JAK2, CALR, BCR-ABL.

In June, the lower extremity skin lesions enlarged; biopsy revealed myelosarcoma with monocytic differentiation. Labs showed WBC 3,600/uL (3% blasts), Hgb 6.5 gm/dL, Plt 555,000/uL. Repeat bone marrow biopsy showed 16% blasts, 2.5% monocytes, background dysplasia, 7q31 deletion in 19% of the nuclei by FISH. She was refractory to induction chemotherapy with idarubicin and cytarabine. After re-induction with mitoxantrone, etoposide, and cytarabine, her blood counts did not fully recover, and bone marrow biopsy showed blasts<5%, worsened dysplasia including abnormal monocytes (37.5% of differential), marked reticulin fibrosis, and more prominent 7q31 deletion in 61% of the nuclei. Cytopenias worsened with WBC 500/uL with monocytic predominance (28%), Hgb 5.3 gm/dL, Plt 29,000/uL. She was started on azacitidine, to which she is thus far clinically responding with improving blood counts.

Myelosarcoma is rare and most often an extramedullary manifestation of acute myeloid leukaemia (AML). Isolated myelosarcoma without bone marrow involvement is exceedingly rare, but in the scarce literature it is thought to seed the bone marrow, which is why treatment is the same as that for AML – with induction chemotherapy. This case highlights how myelosarcoma can start as a primary extramedullary lesion, with bone marrow dysplasia occurring thereafter. The evolution of her bone marrow findings resembles that of myelodysplastic/myeloproliferative neoplasm, and she is currently responding to treatment with azacitidine. Her prior history indicates that the myelodysplasia is likely secondary to etoposide.

218 Symptomatic pericardial effusion and cytologic diagnosis with hodgkin’s disease

Z Ghandour

Tulane Medical Centre, Metairie, LA

Purpose of study Hodgkin lymphoma (HL) accounts for approximately 10% of all lymphomas and is diagnosed predominantly during two age peaks, 20 and 65 years. Approximately 65% of HL patients present with mediastinal involvement. Pericardial effusion is seen in only approximately 5% of HL patients and is rarely symptomatic. Histologically, HL has Reed-Sternberg (RS) cells in a background of inflammatory cells. Cytologic examination of pericardial fluid usually only show inflammatory cells.

Methods used A 72-year-old female patient, recently diagnosed with mixed cellularity HL on an excisional axillary lymph node biopsy, presented to the emergency room with shortness of breath and bilateral lower extremity oedema. Her echocardiogram revealed a decreased ejection fraction of 45% and a large pericardial effusion. Diagnostic pericardiocentesis was performed. The pericardial fluid cytologic examination showed large binucleated cells (figure A) in a background of a mixed inflammatory cell infiltrate. Immunohistochemical stains showed CD30 positivity (figure B) and CD45 negativity in the binucleated cells, consistent with RS cells.

Summary of results HL has four classical subtypes, nodular sclerosing being the most prevalent. HL has been genetically identified as a B-cell neoplasm with RS cell expression of CD30 and CD15 with lack of CD45 expression by immunostains. RS cells only compose approximately 1% of the cellular content of HL, so it is rarely seen in cytologic preparations. Selection of initial treatment for HL is usually based on presenting stage and prognostic factors, including extranodal involvement, such as pericardial involvement

Conclusions Symptomatic pericardial effusions are rare with HL patients. RS cells make up a small percentage of cells present in HL and may not always be present in cytologic preparations. Accurate diagnosis in this setting is needed, as the consequences may be dire if the patient is not treated properly

Abstract 218 Figure 1

The pericardial fluid cytologic examination showed large binucleated cells (A) in a background of a mixed inflammatory cell infiltrate. Immunohistochemical stains showed CD30 positivity (B)

219 Testicular myeloid sarcoma: a rare manifestation of acute myeloid leukaemia

D Hansen

S Elkins

C Milner

University of Mississippi Medical Centre, Jackson, MS

Introduction Myeloid sarcoma of the testis is a rare entity and may occur de novo or concomitantly with bone marrow disease. Myeloid sarcoma (MS) is a neoplasm of myeloid blasts that involves an extramedullary anatomic site. MS can arise in lymph nodes, skin, gastrointestinal tract and central nervous system. The incidence of MS in adults is approximately 2% and it has been linked to a poor prognosis.

Here, we report a case of a patient who developed a late recurrence of acute myelogenous leukaemia (AML) in the form of testicular myeloid sarcoma twelve years after initial treatment.

Case report A 59-year-old white man was admitted for management of relapsed AML in the form of testicular MS. Patient was initially diagnosed with AML in June of 2005 with unknown diagnostic cytogenetics. He was treated with induction chemotherapy consisting of cytarabine plus idarubicin and three cycles of consolidation chemotherapy with high-dose cytarabine. He remained in remission until July of 2017 at which time, he began to experience weight loss, fatigue and left testicular swelling. Testicular ultrasound revealed orchitis for which he was treated with ciprofloxacin without improvement. He was then evaluated by urology and had a left orchiectomy with pathology revealing MS. Bone marrow biopsy had no evidence of involvement by AML.

Examination of cerebrospinal fluid revealed no evidence of leukaemia. Patient has received induction chemotherapy with cytarabine plus daunorubicin with complication of febrile neutropenia. He is now in remission and awaiting allogeneic hematopoietic stem cell transplantation (alloHSCT).

Discussion Isolated extramedullary relapse in the form of testicular MS is rare with only a handful of cases described. Testicular MS remains a therapeutic challenge as no established treatment strategy exists due to lack of prospective trials. Treatment recommendations are based on retrospective studies with available options including surgical resection, systemic chemotherapy, local radiotherapy, alloHSCT or a combination of these methods. The role of alloHSCT has been highlighted in several studies, in which it has been shown that alloHSCT improves overall survival in patients with MS and may be the most beneficial element of MS therapy.

220 Thalessemia complicated by moyamoya cured with unrelated donor hematopoietic stem cell transplantation

CL Harper

A Baker

D Gunda

D Crawford

University of Oklahoma School of Medicine, Oklahoma City, OK

Case report Our patient was diagnosed in infancy with β-thalassemia trait due to anaemia and paternal family history. Since his haemoglobin was lower than expected, a more thorough evaluation was performed revealing a triplicated α-globin gene from his mother. He remained asymptomatic until age 4 when he required a transfusion for an aplastic crisis associated with a Parvovirus B19 infection. He infrequently received transfusions until he presented with ataxia, headaches, and vomiting at 6 years of age. MRI revealed numerous acute and subacute strokes in watershed areas of the anterior and middle cerebral arteries. MRA demonstrated vascular abnormalities consistent with Moyamoya syndrome. He was subsequently treated with aspirin and chronic transfusion therapy. Several months later, he underwent two synangiosis revascularisation procedures to reduce the risk of further ischemia. Chronic PRBC transfusions, aspirin, and oral iron chelation were continued for 6 more years. The patient proceeded with hematopoietic stem cells transplant (HSCT) using a well-matched unrelated donor. Since graft failure and graft-versus-host disease (GVHD) are major complications of HSCT for thalassemia, he was enrolled on a protocol investigating pre-treatment with hydroxyurea and post-transplant Abatacept to minimise the risk of these complications. His transplant course was complicated by prolonged neutropenic fever and respiratory distress with opportunistic pneumonia leading to a 17 day PICU stay. Another complication was stage II skin aGVHD. After discharge, he has full donor chimerism, is transfusion-independent, and doing well more than 4 months after transplant. Moyamoya is recognised as a complication of sickle cell anaemia including sickle/β-thalassemia. However, it has only been reported in 9 thalassemia patients, the majority of which are thalassemia intermedia patients, like ours. Another report has shown a high rate of silent infarcts in thalassemia intermedia patients demonstrating that Moyamoya is not the only cerebrovascular manifestation of thalassemia intermedia.

221 Race-specific genetic mutations in paediatric patients with b-acute lymphoblastic leukaemia

A Reddy1

I Espinoza1

D Cole1

J Schallheim1

E Bhanat1

Y Zhou1

J Zabaleta2

G Megason1

C Gomez1

1University of Mississippi Medical Centre, Jackson, MS

2LSUHSC, New Orleans, LA

Purpose of study The most common form of cancer in paediatric patients is B-acute lymphoblastic leukaemia (B-ALL) and comprises more than 30% of all childhood malignancies. The survival of patients was found to be significantly lower in African American (AA) children compared to European (EA) children in previous studies. This disparity is not related to socioeconomic variables, suggesting a molecular basis for the lower survival rates of AA. Here we present a study showing race-specific genetic aberrations (GA) that may play a role in health disparities in B-ALL in AA and EA children.

Methods used Twenty newly diagnosed paediatric patients were enrolled in our study (5 AA and 15 EA). Ages range between 1 and 18 years with a median age of 4 years. None of the patients had a relapse. Median percent of blasts was 94.8% (64.5%–99.9%). Frozen bone marrow aspirates were used to extract DNA and whole exome sequencing (WES) was performed, focusing on race and B-ALL specific germline mutations.

Summary of results Specific germ-line mutations were identified within the most widely accepted cancer-related genes related to B-ALL. Most GA (339) were shared between AA and EA, for example those present in the Anaplastic Lymphoma Receptor Tyrosine Kinase (ALK) gene. Some GA were specific for AA (58) such as Lipoma Preferred Partner Gene (LPP) and others specific for EA (52) such as, Leukaemia Inhibitory Factor Receptor (LIFR). The ingenuity pathway analysis revealed these genes clustered in race-specific canonical pathways. In AA, the pathways were related to telomerase signalling and cancer signalling. While in EA, it was related to stem cell pluripotency and hereditary cancer. Our findings suggest the value of WES as a tool for development of individual gene signatures and gene scores for AA and EA children afflicted by B-ALL.

Conclusions Aberrant biological networks revealed by our study, provide information on GA and signalling networks that may be involved in race-specific leukemogenesis. Our findings suggest that it may be possible to develop a WES gene signature in B-ALL to help define a race-specific prognosis. These findings may ultimately impact disease management and contribute to the elimination of disparate outcomes in B-ALL in AA children.

222 From metastatic melanoma to complete response in seven months: the power of combined checkpoint immunotherapy

SM Jeong

N Sheehan

University of Mississippi Medical Centre, Jackson, MS

Introduction Treatment for advanced melanoma has improved considerably since the introduction of checkpoint immunotherapy. Recently, combination therapy with nivolumab and ipilimumab has demonstrated increased antitumor activity compared with ipilimumab alone and has become a first-line treatment for metastatic melanoma. Here we report a case of a patient with advanced melanoma who achieved complete response to combined checkpoint immunotherapy.

Case report A 62-year-old Caucasian male with history of malignant peripheral nerve sheath tumour (MPNST) of the scalp presented for evaluation of a pathologic fracture of the right humerus. Two weeks prior, he was found to have a lung mass on computed tomography of the chest; biopsy revealed a high grade malignant epithelioid and spindle cell neoplasm that was diffusely S-100 positive. PET-CT revealed metastatic disease in the brain, lungs, heart, pancreas, abdominal mesentery, soft tissues, and throughout the osseous structures. Bone biopsy of his fracture site was consistent with metastatic melanoma. He underwent whole-brain radiation as well as radiation therapy to his right humerus.

It was felt that the features of the patient‘s disease were most characteristic of malignant melanoma.The biopsy was negative for BRAF V600 mutation. LDH was 456 U/L. In 12/2016, he was started on therapy with ipilimumab 3 mg/kg and nivolumab 1 mg/kg every 3 weeks.

After the fourth cycle, the patient experienced significant immune-mediated side effects, including hypothyroidism, colitis, dermatitis, and hepatitis, requiring hospitalisation. However after recovery, a repeat PET-CT in 7/2017 showed no residual, recurrent, or metastatic disease. MRI of the brain showed a small cavity in the right thalamus at the sight of treated metastasis with no new intracranial metastases. LDH returned to normal at 183 U/L. The patient remains in complete response.

Discussion The treatment and prognosis for advanced melanoma continues to improve as further immunotherapy combinations are explored. Clinicians should be aware of the significant improvement in progression-free and overall survival that combination immunotherapy offers compared to monotherapy alone, yet be cautious in monitoring for higher rates of grade 3 or 4 immune-related adverse events.

223 Plasmacytoma testis – rare extramedullary lesion of plasma cell myeloma

ES Josan1

M Zayko1

T Bhandari1

R Vililla2

T Tamarro2

1East Tennessee State University, Johnson City, TN

2Mountain Home VAMC, Johnson City, TN

Case report A 45 year old male with stage III Plasma Cell Myeloma (PCM) previously treated with Bortezomib, Thalidomide, Dexamethasone and maintenance Thalidomide and Zoledronate presented with back pain and left testicular swelling for 1 month. Imaging showed multiple skeletal lytic lesions but no abdominopelvic lesions or lymphadenopathy. AFP, βhCG and LDH were normal. A radical orchiectomy performed after ultrasound revealed a 3 cm pale yellow, firm, homogenous and partially lobulated tumour (figure 1A). Histopathology showed sparing of epididymis and spermatic cord (figure 1B); partial tubular effacement, diffuse proliferation of small/intermediate size plasma cells and occasional prominent bi/multinucleate cells (figure 1C). The atypical plasma cells were strongly positive for CD138, λ-light chain and negative for CD20 by immunohistochemistry (figure 1D). A neoplastic plasma cell process was confirmed by flow cytometry with 60% of abnormal cells expressing CD38, CD56 and sIg-λ.

Extramedullary lesions in PCM indicate poor prognosis with a survival rate of 1 month-2 years. Testicular lesions are seen in 0.1% cases, although the testis is a suspected sanctuary site. Solitary lesions warrant orchiectomy while diffuse disease requires chemotherapy. Palliative radiation is used in advanced disease. Plasmacytoma is an important differential diagnosis of testicular neoplasms, especially in patients with PCM. A thorough physical examination is crucial for early detection and treatment.

Abstract 223 Figure 1

(A) A radical orchiectomy performed after ultrasound revealed a 3 cm pale yellow, firm, homogenous & partially lobulated tumour. (B) Histopathology showed sparing of epididymis & spermatic cord. (C) Partial tubular effacement, diffuse proliferation of small/intermediate size plasma cells & occasional prominent bi/multinucleate cells. (D) The atypical plasma cells were strongly positive for CD138, λ-light chain & negative for CD20 by immunohistochemistry

224 Thyroid malt lymphoma: a rare cancer with a good prognosis

R Khalaf

S Singal

E Spradling

D Jaishankar

ETSU, Johnson City, TN

Case report Marginal Zone lymphoma (MZL) accounts for 10% of all Non Hodgkins lymphomas (NHL). MALT (Mucosa associated lymphoid tissue) Lymphoma is the commonest subtype of MZL. Over half of all MALT Lymphomas are seen in the gastrointestinal tract (80% in the gastric area). MALT Lymphomas can be seen less commonly in other organs such as skin, lung, ocular adnexa, and rarely in small bowel, salivary glands, thyroid, breast and bladder. Lymphomas account for 1%–5% all thyroid neoplasms. A seventy three year old female with multiple medical problems presented with progressive neck swelling of a few months duration. No fever, sweats or weight loss reported. No dysphagia or dysphonia noted. Imaging studies revealed a thyroid nodule/mass. Patient underwent a thyroidectomy. Pathology revealed diffuse, dense atypical lympho-plasmacytic cells infiltrating around residual reactive thyroid follicles. Neoplastic cells were positive for CD20, CD79a, Pax-5, and BCL2. Extensive chronic lymphocytic thyroiditis with Hurthle cell change and adenomatous nodular hyperplasia was also noted. PET scan revealed no other involved sites. Postoperative radiation was delivered for positive margins. Thyroid lymphoma is associated with chronic inflammation, autoimmune diseases, Hashimotos’s thyroiditis or chronic infections. It occurs in the seventh decade equally among both sexes. Immunophenotypically, MALT cells express Ig, B cell markers (CD19-CD20, CD22 and CD 79a) and are negative for CD5, CD10, CD23 and Cyclin D1. Cytogenetic abnormality t(11,18) are commonly reported. MALT lymphomas often present with early stage disease without B symptoms or bone marrow involvement. HCV, HIV, H.Pylori and Myeloma panel testing is recommended. MALT lymphomas can be treated with resection or radiation with curative intent. Early stage gastric MALT lymphomas often regress with H.Pylori treatment. Systemic treatment with rituximab based immuno chemotherapy regimens offer reasonable control in advanced stage disease. The ten year survival with Thyroid MALT lymphomas is 95%. There are very few cases of MALT lymphoma in the thyroid, which account for less than 0.1% of all thyroid neoplasms and we are reporting one such interesting case.

225 A rare case of uros gene negative congenital erythropoetic porphyria

S Kolagatla

N Moka

S Bailey

ARH-Markey Cancer Centre, Hazard, KY

Case report Congenital Erythropoietic Porphyria (CEP) also known as Gunther disease is due to autosomal recessively inherited deficiency of uroporphyrinogen III synthase (UROS) an enzyme needed for heme synthesis that leads to accumulation of porphyrins in tissues along with hemolytic anaemia and cutaneous photosensitivity. CEP is always due to mutations in the UROS gene. Rarely due to other genes that affect UROS gene expression. In few cases UROS coding region and the intron-exon boundaries can be unrevealing suggesting additional sites of mutations.

48 year old Caucasian male with long standing history of blistering skin lesion on sun exposed areas presented to the clinic with worsening fatigue. His past medical history include multiple staphylococcal infections, hemolytic anaemia. One of his brothers had blistering lesions. Exam showed blistering of left arm and healed lesion on both arms and head otherwise no mucosal blistering appreciated. Quantitative porphobilinogen are consistently elevated in the plasma, urine and stool. UROS gene testing was performed which was unrevealing for any mutations.He developed Normocytic anaemia with low retic count and extreme neutrophilic leukocytosis. Bone marrow biopsy which demonstrated evidence of myelodysplatic/myeloproliferative neoplasm. Within a few months he had deceased with sepsis.

In CEP excess porphyrins in the urine turn red upon exposure to sunlight (Image 1). Mainstay of treatment is to avoid sunlight especially in patients with high levels of porphyrins, also include vitamin D replacement as this patients are usually deficient in Vitamin D because of sun avoidance, blood transfusions, iron chelation, skin and eye care. Case reports describing cure with allogenic stem cell transplant with suitable donor. CEP could potentially treated with gene therapy as over expression of UROS gene is possible in cultured hematopoietic stem cells but no studies no demonstrate the efficacy and feasibility have been reported. Our case is interesting because of UROS gene was unrevealing and development of myeloproliferative/myelodysplatic syndrome.

226 Familial polycythemia likely due to novel haemoglobin variant- haemoglobin hyden

S Kolagatla

N Moka

S Bailey

ARH-Markey Cancer Centre, Hazard, KY

Purpose of study In order to identify the Haemoglobin variant resulting in familial polycythemia in a family from Hyden in Eastern Kentucky.

Methods used p50 analysis, Haemoglobin electrophoresis, Bi-directional sequence analysis to test for mutation in all coding regions and non-coding portions of the beta haemoglobin gene.

Summary of results In the course of work up of familial polycythemia that is negative for Jak-2 with reflex Exon 12 and CALR mutation. Oxygen dissociation p50 of 19 which is low indicating left shifted dissociation curve. Electrophoresis cascade demonstrate Haemoglobin A 61.2%, Haemoglobin A2 3.0% and Variant 35.8% of Beta variant.

Bi-directional sequence analysis for Molecular alterations Gene: HBB, DNA change: Codon 39, heterozygous CAG >CCG Protien change: P.G1n39Pro. [glutamine (Q) to proline (P)].

HGVS: c.119A>C, p.Q40P, Classification: Likely deleterious variant. (GenBank accession number NM_000518.4).

Conclusions This is a previously unreported beta chain haemoglobin variant present. This particular haemoglobin variant is named as Haemoglobin Hyden based on the place where this is found in Hyden, Kentucky. There have been four variants reported at codon 40 of the beta globin gene, which is an external contact site between beta globin and alpha-2 globin. One variant, Hb vassa, is associated with mild hemolytic anaemia. The three other variants (Hb Alabama, Hb Tianshui and Hb San Bruno) are not asscociated with clinical or haematological abnormalities.

In our opinion p.Q40P is likely a cause of erythrocytosis. In order to further establish the causality it may be beneficial to test first degree relative to in this family in order to determine whether the p.Q40P alteration tracks with disease and is not present in unaffected individuals. Haemoglobin threshold for phelebotomy to lower the risk of thrombosis and cardiovascular events is yet to be defined.

227 Graft-versus-host disease presenting with pancytopenia after orthotopic liver transplant

E Long

University of Mississippi Medical Centre, Jackson, MS

Case report Graft-Versus-Host Disease (GVHD) is a devastating complication of bone marrow transplantation. GVHD is caused by the activation of donor T-cells by the antigen presenting cells of the recipient. This case report describes GVHD presenting as pancytopenia after solid organ transplant, highlighting a rare presentation of this disease.

A 68-year-old female presented with fever to 101, shortness of breath, fatigue, and diffuse rash; she had undergone an orthotopic liver transplant one month prior. She was found to leukopenic, with a white blood cell count of 1.2. Her TMP-SMX and mycophenolate mofetil were held, and she was given a dose of filgrastim. CMV and EBV titers were negative. On follow-up a worsening leukopenia was noted, with WBC now 0.2.

She was admitted to the hospital. Physical exam was notable for the absence of rash, and the absence of erosions of mucous membranes. Labs revealed marked pancytopenia, with WBC 0.1, Hb 22.8, and platelet count 129.

Bone marrow biopsy and aspirate revealed markedly hypocellular marrow with ten percent cellularity and marked hypoplasia of granulocytes. There was no evidence of acute leukaemia. Peripheral blood showed marked leukopenia and pancytopenia. Likely causes were considered to be medication or infection.

Tacrolimus was stopped in favour of cyclosporine. Filgrastim was begun. Counts began to slowly recover, with WBC 2.0, Hb 9.4, and platelets 105. Patient was discharged home.

Patient represented several days later with altered mental status and seizure. CSF studies were positive for HHV-6. WBC was 0.1. Concern was raised for GVHD as a cause of her pancytopenia. Post-transplant analysis of the peripheral blood showed chimerism, with 27% donor cells and 73% patient cells, consistent with a diagnosis of Graft-versus-Host disease. Unfortunately, the patient died rapidly thereafter as a result of her disease.

Acute GHVD is rarely seen after solid organ transplant, with the number of reported cases in the hundreds. Furthermore, GVHD usually affects the skin, liver, and digestive tract. GVHD presenting as pancytopenia is quite rare. We present this case to raise awareness of the GVHD as a cause of profound pancytopenia in the post-transplant setting. We hope this will allow the diagnosis to be made more expeditiously in future presentations.

228 Impella induced hemolytic anaemia

S Maharaj

K Seegobin

B Bajric

F Rana

University of Florida College of Medicine, Jacksonville, FL

Case report A 72 year old male with a history of non-ischaemic cardiomyopathy with reduced ejection fraction (20%) was admitted with electrical storm from ventricular tachycardia (VT). He was intubated, sedated and managed with antiarrhythmic therapy (amiodarone, lidocaine) but with recurrent VT required support with placement of an Impella. He did not have any hemoglobinopathy or valvular disease and there was no infection. After Impella placement, the haemoglobin (Hb) was 10.0 g/dL. The following day, Hb declined to 8.8 g/dL with no source of bleeding. Acute anaemia continued to worsen with a decline to 7.0 g/dL by day 5. Leukocyte and platelet counts were normal. Hemolytic indices revealed elevated LDH of 671 IU/L, haptoglobin below the detectable limit of assay and increased unconjugated bilirubin. Review of the peripheral blood smear confirmed the presence of helmet cells. The patient was transfused and the Impella device was removed shortly after with no further drop in haemoglobin.

Anaemia in patients with acute heart failure is important as it leads to worsening disease. The Impella microaxial flow device is placed across the aortic valve and generates blood flow out of the left ventricle into the aorta by revolving at high speeds. Impella is increasingly used in cardiogenic shock, VT ablation and high-risk coronary intervention. A recent case report suggested improper placement leads to hemolysis, but in this case even with proper placement there was significant hemolysis. We postulate that turbulent flow through leads to excessive shear stress on the erythrocytes. The interaction with foreign surfaces also lends to mechanical trauma. Large scale data is lacking but one retrospective study found that Impella therapy was associated with a significant decrease in haemoglobin after 24 hours, and two thirds of patients underwent transfusion. Patients on Impella therapy should be monitored closely for hemolysis which can be significant. These patients are often in cardiogenic shock and we suggest that the need for transfusion should be anticipated. Serial monitoring of hematologic and hemolytic indices is therefore advised to allow early detect and management to prevent haemodynamic compromise.

229 Use of gene expression profiling in the evaluation of patients with uncommon cancer presentation: a rare case of metastatic breast cancer to the uterine cervix

J Manalac1

A Garcia2

1LSUHSC, Lafayette, LA

2LSUHSC, New Orleans, LA

Purpose of study To describe an unusual presentation of metastatic breast cancer (BC) in a 63 year old woman and the clinical application of tumour gene expression profiling in the diagnostic evaluation.

Methods used Case report and literature review.

Summary of results We present a 63 year old female with a new cervical mass. In 2002 she was diagnosed with stage II oestrogen receptor positive BC. She was treated with surgery, chemotherapy and endocrine therapy. She was in remission until 2007 when she developed isolated left hip metastasis treated with radiation therapy, and a second recurrence to the right supraclavicular lymph node in 2015 with receptor positive disease. She was subsequently placed on fulvestrant. Two years later, routine staging studies revealed interval development of a cervical mass with uterine, vaginal and bladder wall extension clinically and radiologically highly suspicious for locally advanced cervical cancer. Biopsy of the cervical mass revealed a poorly differentiated carcinoma. Immunohistochemistry stains were positive for CK7, GATA-3, oestrogen receptor, P16 (weak) and negative for Pax-8, CK5/6, p63, CK20 and CD10, consistent with metastatic BC. Gene expression profiling using a validated 92-gene real-time PCR assay was used to distinguish a new primary tumour of the cervix from an unusual presentation of isolated metastasis to the cervix. This gene assay revealed a 96% probability of BC and ruled out carcinoma of the cervix with 95% confidence.

Conclusions Metastases to the cervix occur very rarely and represent a diagnostic challenge for clinicians and pathologists. It has been reported that up to 42% of metastatic lesions to the cervix are mistaken for primary tumours. Information on the tumour type is crucial in guiding treatment decisions.. Clinicians should be aware of these new platforms which, in conjunction with clinical and pathologic evaluation, can aid in the identification of the primary site in selected patients who present with carcinoma of unknown primary or unusual clinical presentations. These assays can help the treating physician to estimate patient prognosis, select the most appropriate therapy, and determine whether tissue-dependent biomarker testing is indicated.

230 Patient-derived triple negative breast cancer xenografts to discover novel kinase pathways

M Matossian

M Burow

B Collins-Burow

Tulane University School of Medicine, New Orleans, LA

Purpose of study Triple negative breast cancers (TNBCs) have an aggressive clinical presentation due to high rates of metastasis, recurrence and chemoresistance; Louisiana, specifically New Orleans, has among the highest incidences of TNBC in the country. Targeted therapy remains elusive in TNBC and discovery of novel therapeutic targets are necessary. Current models in target discovery research cannot accurately recapitulate the complex architecture and heterogeneity of TNBC. Immortalised cell lines have been selected in a 2D environment and may have lost important features of original tumours. Our primary objective was to dissect and evaluate the various components that drive complex interactions within TNBC tumours using patient-derived xenografts from New Orleans hospitals.

Methods used We analyse relevant transcript (qRT-PCR) and protein (flow cytometry, immunohistochemistry) expression patterns that are unique to each PDX model. Using qRT-PCR and 3D culture, we examine effects of the pan-deacetylase inhibitor, LBH589 on mammospheres, in vivo tumorigenesis and collagen expression. We also generated cell lines and mammospheres (TU-BcX-2K1, TU-BcX-2O0, TU-BcX-49S, TU-BcX-4IC) from each PDX model. Finally, we utilise novel techniques such as tissue decellularization to examine extracellular matrix components and evaluate the necessity of the scaffold in TNBC tumorigenesis.

Summary of results Our laboratory has established four TNBC PDX models representing various patient ethnicities, response to chemothearpy, and TNBC molecular subtypes and metastatic behaviour. We demonstrate these models can be used in therapeutic discovery research and recapitulate results observed in preliminary studies using immoralized TNBC cell lines. Finally, we dissect these models using various techniques to examine aspects of this complex tumour that can be targeted by developing therapeutics, including specific cell populations, the extracellular matrix, and cancer stem-like cells.

Conclusions Our aim is to leverage novel patient-derived models from under-studied patients with a range of clinical presentations to guide the selection of therapeutically targetable pathways in specific molecular subtypes of TNBC.

231 Recurrent diffuse large b cell lymphoma presenting as a solitary spiculated lung nodule

RM Medrano

A Fuller

E Islam

TTUHSC, Lubbock, TX

Case report Diffuse large B-cell lymphoma (DLBCL) is the most common type of extra-nodal lymphoma. The majority of extra-nodal cases occur in the gastrointestinal tract, with pulmonary involvement is very rare.Here we present a case of pulmonary lymphoma presenting as a solitary spiculated lung nodule.

A 61-year-old male with a history of long standing tobacco use and DLBCL diagnosed 6 months prior, presented with shortness of breath and cough productive of clear sputum. He had completed 7 of 8 R-CEOP chemotherapy cycles with apparently good response. Chest CT at the time of DLBCL diagnosis is shown in figure A. On admission, chest CT revealed a 1.9 cm rounded, spiculated nodule in the right upper lobe (RUL) as seen in figure B (arrow). Given the spiculated appearance of the nodule and his long standing tobacco use, there was concern for a second primary malignancy. Navigational bronchoscopy and transbronchial biopsy of the RUL nodule was completed. Histopathology revealed high grade B cell lymphoma. Bacterial and fungal cultures were negative.

Pulmonary involvement in extranodal lymphoma represents less than 1% of cases. Clinically, it can present with non-specific symptoms such as shortness of breath, cough, fatigue, weight loss and fever. Radiographically, it can have various presentations such as consolidation, a well-defined mass, single or multiple nodules, interstitial infiltrates, cavitary or endobronchial lesions. There was significant concern for a second primary in our patient given his long standing tobacco abuse and the presence of a solitary spiculated lung nodule. Our patient represents a rare presentation of extranodal lymphoma, and highlights the importance of considering a wide array of diagnoses when completing work-up for a solitary pulmonary nodule.

Abstract 231 Figure 1

Chest CT at the time of DLBCL diagnosis is shown in figure A. On admission, chest CT revealed a 1.9 cm rounded, spiculated nodule in the right upper lobe (RUL) as seen in figure B (arrow)

232 A rare case of transformation from mycoses fungoides to hodgkin’s lymphoma

N Moka1

S Arikapudi1

B Boonpheng1

M Janelle1

T Tamarro1,2

1ETSU, Johnson City, TN

2Mountain Home VA, Johnson City, TN

Case report Primary cutaneous lymphomas are non-Hodgkin lymphomas that can be divided into three major subtypes based on neoplastic cell of origin. The three subtypes include cutaneous T-cell lymphomas (CTCL), cutaneous B-cell lymphomas (CBCL), and natural killer cell lymphomas. Mycosis fungoides (MF) is a variant of CTCL that can present with a variety of skin lesions ranging from eczema or psoriatic-like patches in the early phases to papular or nodular cutaneous tumours in later stages. The natural history of untreated MF is to evolve into systemic disease. MF can transform from a CD4 +dominant to a more malignant CD30 +variant. However, there are rare case reports of transformation between cell lineages from a CTCL to Hodgkin lymphoma.

A 70 year old male with a 10 year history of MF presented to the haematology/oncology clinic with worsening skin lesions and cervical lymphadenopathy. Pertinent to his history is the clinical trajectory of his diagnosis of MF. He first presented with cutaneous plaques on the extensor surfaces of his legs and was diagnosed with psoriasis. Treatment with narrow band UV light and topical steroids was initiated without improvement in his symptoms. He underwent treatment with etanercept and concurrent photo-chemotherapy for 3 years, after which new lesions were biopsied that were consistent with folliculotropic MF. He was lost to follow-up for 2 after his diagnosis of MF and presented to the clinic to reestablish care complaining of a new neck mass. Excisional lymph node biopsy was consistent with nodular sclerosing Hodgkin lymphoma (CD15+, CD30+, CD20-, CD45-). He received 6 cycles of ABVD and is currently in remission.

The clinical distinction of this case ultimately lies in the aetiology of the patient’s Hodgkin’s lymphoma. Our first hypothesis is that the MF underwent transformation between cell lineages and the Hodgkin’s lymphoma was an extension of his pre-existing neoplasm. A competing theory is that two distinct neoplastic processes occurred simultaneously. The major confounding variable is his 3 year treatment with etanercept, a TNF-alpha antagonist with a known association with the development of lymphoma.

233 16 year old girl diagnosed with caps and probable hit

M Nguy1

V Agarwal2

C Gauger3

1UFCOM, Jacksonville, FL

2Nemours, Pensacola, FL

3Nemours, Jacksonville, FL

Case report Catastrophic antiphospholipid syndrome (CAPS), also known as Asherman syndrome, is an uncommon complication of antiphospholipid syndrome that occurs in <1% of patients that causes thrombus in multiple organs.1 In treating CAPS, it is important to recognise heparin induced thrombocytopenia (HIT), as it can occur in up to 5% of patients taking unfractionated heparin.2 We present a case of a 16-year-old Caucasian female with concurrent diagnosis of CAPS and probable HIT. There are few case reports on these diagnoses in tandem in adults however none previously reported in paediatrics.

References

  1. . Asherson RA, Cervera R, de Groot PG, et al. Catastrophic antiphospholipid syndrome registry project group. Catastrophic antiphospholipid syndrome: International consensus statement on classification criteria and treatment guidelines. Lupus2003;12(7):530–534.

  2. . Triplett DA, Asherson RA. Pathophysiology of Catastrophic AntiphosPholipid Syndrome (CAPS). American Journal of Haematology2000;65:154–159.

Diagnostic criteria for CAPS

Review of literature of concurrent cases of CAPS and HIT

234 What came first: the ovary or the lymphocyte?

H Oddo Moise

A Coulon

J Doan

S Sanne

LSUHSC, New Orleans, LA

Case A 31 year old woman with history of daily IVDU and untreated Hepatitis C presented to the emergency department with 3 months of non-specific progressive ailments including left knee pain, shortness of breath with exertion and three-pillow orthopnea, twelve pound weight loss, abdominal ‘tightness’ greatest in the left lower quadrant, early satiety, dysphagia to liquids and solids, left axillary node swelling and bilateral supraclavicular lymph node swelling. She denied fever, chills, night sweats, recent travel, sick contacts or family history of cancer. Chest CT showed a substantial left pleural effusion with lobulated pleural thickening and mediastinal and hilar lymphadenopathy consistent with sarcoma versus metastatic disease. Subsequent abdominal and pelvic CT showed enlarged retroperitoneal lymph nodes of the left pelvis and groin with a solid mass in the left deep pelvis concerning for ovarian malignancy, metastatic disease, lymphoma or sarcoma. Transvaginal ultrasound revealed a solid left adnexal mass measuring 5.6×3.4×5.2 cm. Workup was begun to ascertain the primary site of malignancy suspicious for lymphocyte versus ovarian source. A right supraclavicular lymph node biopsy was performed during which she was intubated for increasing left pleural effusion with compression of mediastinal structures. Thoracentesis was performed with removal of 1.5 L of blood-tinged pleural fluid and a chest tube was placed. Flow cytometry of the lymph node biopsy showed 95.6% T lymphoblasts positive for CD2, CD3, CD7, TdT and CD99 consistent with the diagnosis of Non-Hodgkin T-cell lymphoblastic lymphoma. CA125 was mildly elevated at 47, not suggestive of ovarian malignancy. Despite numerous attempts at discussing the importance of a bone marrow biopsy and cancer treatment options, the patient declined all medical intervention or palliative resources.

Discussion Malignant lymphoma involvement of the female genitourinary tract, including the ovary, is not commonly seen. While ovarian involvement is relatively rare, non-Hodgkin Lymphomas such as T cell lymphoblastic lymphoma (T-LBL) represent a frequency of approximately 7% to 26% of those diagnoses and should be considered in the differential diagnoses of young females with ovarian masses.

235 Poems: an ode to a syndrome

S Ozair

B DeBoisblanc

S Subbiah

R Jetly

Louisiana State University Health Sciences Centre, New Orleans, LA

Case report A 45 year-old man with normal coronary arteries on angiography was referred for evaluation of thrombocytosis (platelet count of 812×103/UL) following two separate acute myocardial infarctions. Testing for JAK2 and CALR mutations were negative. He also described symptoms of worsening polyneuropathy and anasarca. POEMS (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal protein, Skin changes) syndrome was suspected. Physical exam was additionally remarkable for splenomegaly and newly developed hyperpigmentation of both hands with white nails. On laboratory testing he was found to have adrenal insufficiency and hypothyroidism. Both kappa and lambda free light chains were elevated but there was no M-spike on serum protein electrophoresis. Bone marrow biopsy was consistent with an increased population of plasma cells that formed atypical aggregates rimming non-paratrabecular lymphoid aggregates. These plasma cells were noted to have a lambda predominant expression pattern, suspicious for monoclonality. Imaging studies confirmed osteosclerotic lesions in T10 and mediastinal lymphadenopathy. VEGF (vascular endothelial growth factor) was elevated at 264 pg/ml.

The cause of POEMS syndrome is unknown, although chronic overproduction of pro-inflammatory cytokines (eg, IL-1, IL-6, and VEGF) appears to be a major contributor to the microangiopathy, increased vascular permeability, thrombocytosis, and neovascularization that leads to thrombosis, tissue oedema, polyneuropathy, and pulmonary hypertension.

The International Myeloma Working Group (IMWG) criteria for the diagnosis of POEMS requires the presence of two mandatory criteria (polyneuropathy and monoclonal protein), and at least one major and one minor criterion. Major criteria include sclerotic bone lesions, elevated VEGF, and Castleman disease. Minor features include organomegaly, endocrinopathy, characteristic skin changes, papilledema, extravascular volume overload, and thrombocytosis.

Patients with POEMS syndrome are best treated with chemotherapy with or without autologous stem cell transplantation similar to that used for the treatment of multiple myeloma.

236 Failed screening of advanced myeloma in an asian woman

T Dickerson

S Bautista

S Palumbo

S Hebert

LS Engel

LSU Health Sciences Centre, New Orleans, LA

Introduction The incidence of multiple myeloma (MM) varies with ethnicity and age; Asian populations have a lower incidence. The mean age of onset of MM in Asian population is 62 years.

Case A 51-year-old Asian woman without significant medical history had multiple Emergency Department visits with progressive, non-radiating low back pain of two months duration. Spinal radiographs were unrevealing and no screening labs were obtained. Eventual referral to an ambulatory clinic identified substantial hypercalcemia. Upon reassessment, the patient endorsed weakness, cramps, anorexia, fever, abdominal pain, constipation, urinary frequency, and a 15-pound weight loss. Physical exam revealed a thin female with tenderness over L4-S1 vertebrae and a 1 cm firm left axillary node. Labs showed corrected calcium 14.52 mg/dL, haemoglobin 10.1, protein gap 8.2 g/dL, and creatinine 1.4 mg/dL. Lumbar CT revealed numerous lytic foci throughout the spine and pelvis supported by skeletal survey revealing lytic foci of the cranium, axial and proximal appendicular skeleton. Her other labs showed elevated IgG, B2-microglobulin, Kappa light chain, and M spike, UPEP with monoclonal band in gamma region, and bone marrow biopsy of >50% plasma cells. FISH analysis showed translocation (11;14) with fusion of CCND1 (BCL1) at 11q13. The patient was diagnosed with multiple myeloma (MM) Stage III by ISS criteria. Hypercalcemia was treated with IV fluids, zoledronic acid, and calcitonin. Radiation was utilised for spinal compression at S1. Lastly, bortezomib and dexamethasone were initiated with plans for stem cell transplant.

Discussion A lower incidence of MM in the Asian population has been partly attributed to genetic polymorphisms with doubled incidence in the last 10 years in South Korea and Taiwan. Analysis of genetic versus environmental risk factors is lacking. MM is ‘a disease of the elderly’ but in an Asian cohort, 58.5% of those diagnosed were <65 years-old. Based on these findings, prevalence in this cohort at earlier ages is likely to continue and research should be sustained.

237 Acute liver failure caused by multiple myeloma

T Poosarla

S Elkins

A Reddy

University of Mississippi Medical Centre, Jackson, MS

Case report Multiple Myeloma (MM) is a plasma cell neoplasm that accounts for about 15 percent of all haematological malignancies. It is a disorder that involves clonal proliferation of neoplastic plasma cells in the bone marrow with an increase in monoclonal paraprotein. It is uncommonly associated with extraosseous involvement such as spleen or liver, especially at initial presentation. Reports of liver involvement in MM are usually secondary to amyloidosis rather than plasma cell involvement. Here we present a rare case of a patient with acute liver failure (ALF) who was found to have MM.

A 56-year-old African American female with history of hypertension and hypothyroidism who was admitted to the hospital with complaint of 2 weeks of abdominal swelling, difficulty ambulating secondary to bilateral lower extremity pain, lower extremity swelling, and shortness of breath. She also notes a 15-pound weight loss over the last 2 months. In the emergency room, she was found to have ascites, globulin gab, elevated serum total protein, hypoalbuminemia, anaemia, and renal failure concerning for cirrhosis. CT of abdomen and pelvis showed a large volume of ascites, abnormal appearance of liver parenchyma, and enlargement of the IVC and hepatic veins suggestive of cirrhosis. She underwent further workup including liver biopsy which showed sinusoidal involvement by plasma cells concerning for MM and no evidence of hepatitis or cirrhosis. Cytology on peritoneal fluid also showed malignant plasma cells. Serum protein electrophoresis consistent with IgG lambda with M protein of 10. Bone marrow biopsy revealed hypercellular marrow with 80% plasma cells. There were multiple subcentimeter lesions throughout the skeleton consistent with MM. She was started on treatment with bortezomib, revlimid, dexamethasone with marked improvement in symptoms and resolution of ascites.

MM presenting as ALF is extremely rare and only a few cases have ever been reported. Liver involvement can be seen and is discovered incidentally but is clinically silent. It is associated with poorer prognosis as treatment is limited with liver failure. It is important to consider MM in patients who present with ALF, especially with no risk factors for cirrhosis, since prompt diagnosis can lead to quicker treatment and theoretically improve survival.

238 Blood cells: tolerance, autoimmunity, modulation

EC Quintin1

KN Rezkallah2

S Ozair1

TM Reske1

H Boulares1

P Rigby1

1LSUHSC New Orleans, New Orleans, LA

2Presence Saint Joseph Hospital, Chicago, IL

Purpose of study All haematological cells originate from a multipotent mesenchymal stem cell that are produced via myelo- and lymphopoietic pathways in the niches of the bone marrow. The marrow structure plays a key role in the mixing of cells and cytokines resulting in cell production, maturation, kinetics, homing and circulation in the niches and sinusoids. The purpose is to enumerate, identify, and access crosstalk of blood cells related to Myeloid Derived Suppressor Cells (MDSC).

Methods used By identifying blood cells using fluorescence-activated cell sorting (FACS), we aim to locate pairs or clusters utilising standard procedures and studying the function and relationship of these cells.

Summary of results The stem cells are held in place by surface receptors and divide under a stimulus. They mature and travel to a sinusoid where they mix with other cells before being released into the venous circulation. Certain cells seek out specific partners according to their own specific surface receptors to crosstalk, exchanging particles and messages causing downhill haematological and immunological cascades.

As individuals grow and age from birth, tolerance is arranged for all self-antigens and tissues. If tolerance is lost, disease may follow. The number, types and proportions of different cells change throughout a lifetime. The mix of MDSC with Tregs, memory cells, NK cells, stem cells, and other lymph cells maintain tolerance for self but changes in the numbers of cells can affect their function, maturity or host characteristics.

Conclusions When autoimmune disease is found, cell crosstalk changes depend on appropriate numbers, proportions, and cross-functions. They may be exogenously altered to regain a tolerant state through modulation of the immune systems via immunosuppressive medications. Specific pairs are linked through cell surface marker/receptor anatomy, relating cells to activity producing certain cytokines. The relationship of cell pairs or other groupings will likely determine inhibition or activation of certain processes that may lead to pathology.

239 A case of concurrent renal cell carcinoma and hodgkin’s lymphoma

M Quirch

RE Gavidia Quezada

S Phisitkul

Texas Tech University Health Sciences Centre, Lubbock, TX

Background The association between developing secondary malignancy after treatment for a primary malignancy is well known, particularly with leukemias and lymphomas such as Hodgkin’s lymphoma. Patients treated for Hodgkin’s Lymphoma are known to have an increased risk of developing a second malignancy such as breast, thyroid, bone, colorectal, or stomach cancer. In a small retrospective study, multiple primary malignancies have been reported, particularly in women from the ages 43–68, with concurrent malignancies typically being breast, uterine, or cervical.

Case presentation This is a 71 year old female admitted for generalised weakness, hyponatremia, pancytopenia, and lactic acidosis with no verifiable signs or source of infection. One month prior to admission, the patient was undergoing work up with her private urologist for a recently discovered renal mass per CT scan that was suspicious for renal cell carcinoma of 2.3×2.0 cm with multiple small lymph nodes. Decision at the time of discovery of the mass was to proceed with surgery however she became acutely ill and that is what led to her admission to our hospital.

Initial blood cultures and urine culture were negative however lactic acid remained persistently elevated and LDH was also elevated so a bone marrow biopsy was done revealing Hodgkin Lymphoma. The patient rapidly deteriorated after bone marrow biopsy. She developed acute respiratory failure, altered mental status, and hypotension which required intubation, vasopressors, and admission to the medical intensive care unit. Broad spectrum antibiotics were started with cultures being drawn seven days after admission. Urine cultures after transfer were positive for pansensitive Klebsiella pneumonia and E. coli, and respiratory culture was positive for Staphylococcus aureus. Blood cultures remained negative throughout her stay. Despite aggressive care, the patient did not recover and the patient’s family pursued comfort measures.

Conclusion While there is an established relationship between developing secondary malignancy after treatment with chemotherapy, concurrent malignancies tend to be very rare. Until now, there has been one other reported case of Hodgkin’s lymphoma occurring concurrently with renal cell carcinoma.

240 A case of incomplete carney triad

A Ismail

M Quirch

S Mousa

MR Arevalo

TTUHSC, Lubbock, TX

Case report Carney triad is a synchronous or metachronous association of gastric gastrointestinal stromal tumours (GIST), pulmonary chondroma and extra-adrenal paraganglioma. Most patients have only one or two components of the triad, all three tumours being found in only about 2% of the patients.

We present a case of a 28-year-old woman that came to the emergency department for epigastric pain with regurgitation. She has a medical history of rheumatoid arthritis, Hashimoto’s thyroiditis and Celiac disease. She reported 6 month history of 20-pound weight loss and loss of appetite. She denied dysphagia or changes in bowel habits. She had a CT of the abdomen showing a large exophytic gastric mass, concerning for malignancy and incidental calcified masses were seen in the lungs. EGD and EUS showed a subepithelial lesion, suspicious for malignant stromal cell neoplasm. Biopsy confirmed it to be GIST, positive for CD117. Molecular diagnosis was negative for Kit and platelet-derived growth factor alpha (PDGFRA) mutations. Lung mass biopsy was consistent with pulmonary chondroma. Curative surgery was planned. Intraoperatively, the tumour was found invading the liver and lymph nodes. She had total gastrectomy with esophago-jejunstomy, celiac lymphadenectomy, and partial liver resection with negative margins. She was discharged 2 weeks after surgery and follows at the cancer centre.

GIST is a mesenchymal neoplasm affecting the gastrointestinal tract typically presenting as a subepithelial neoplasms. Although the majority of GISTs appear to be sporadic, 5% of patients have one of the familial autosomal dominant syndromes, including neurofibromatosis type 1, Carney triad, and primary familial GIST syndrome. Most GISTs are characterised by KIT or PDGFRA activating mutations. There are 10%–15% of GISTs lacking KIT and PDGFRA mutations, called wild-type GISTs. Among these WT GISTs, a small subset is associated with succinate dehydrogenase (SDH) deficiency, known as SDH-deficient GISTs. GISTs that occur in Carney triad represent specific examples of SDH-deficient GISTs. SDH-deficient GISTs locate exclusively in the stomach, showing predilection for children and young adults with female preponderance. The tumour generally pursues an indolent course and exhibits primary resistance to imatinib therapy in most cases. Surgical resection is the preferred mode of therapy.

241 Diplopia and proptosis with a pituitary mass equals a macroadenoma? think again!

D Reddy

M Ibrahim

K Krishnan

D Jaishankar

ETSU, Johnson City, TN

Case report Acute Myeloid Leukaemia (AML) is an aggressive hematologic malignancy. Central nervous system (CNS) involvement is rare among adult patients with AML. We describe a patient with AML presenting with visual disturbances and a pituitary mass which resolved after systemic AML chemotherapy.

A seventy one year old female with a history of AML treated successfully (without allogeneic hematopoietic stem cell transplantation {allo-HSCT}) sixteen years ago, was hospitalised with a two week history of fatigue, weight loss and intermittent blurry vision. Clinical exam was notable for diplopia and right sided proptosis without visual field defects. MRI Brain revealed a 2.4 cm sellar mass (suggestive of a macroadenoma) with cavernous sinus invasion, right carotid artery encasement, and mass effect on right optic chiasma. Peripheral blood smear documented 28,000 WBC count with 84% blasts. Pituitary function assay was normal. Bone marrow biopsy reported hyper cellularity (60%) and 70% myeloblasts. Molecular studies t (8;21) established a new clonal leukaemia distinct from her previous AML (normal cytogenetics). Lumbar puncture revealed monocytes with rare Auer rods. She completed induction chemotherapy with Idarubicin and cytarabine achieving first complete remission (CR1). She received one dose of intrathecal methotrexate and 3 cycles of cytarabine consolidation chemotherapy course. Her diplopia and proptosis resolved completely.

AML is characterised by a rapid clonal proliferation of immature hematopoietic cells in the peripheral blood and bone marrow. The overall survival of AML is dictated by cytogenetics/molecular markers and age. When CR1 is achieved, most relapses occur in the first two years. Extramedullary involvement with CNS leptomeningeal infiltration can be noted in acute lymphoblastic leukaemia and in 2%–10% of patients after allo-HSCT. Leukemic infiltration of the pituitary gland with AML is extremely rare and documented in isolated case reports. Our patient represented an extremely rare and unique case of a second-primary AML with pituitary involvement that responded to induction and consolidation chemotherapy.

242 ‘dose escalated’ chemotherapy for agrresive lymphoma in the elderly

D Reddy

F Tawadros

D Jaishankar

ETSU, Johnson City, TN

Case report Diffuse large B-cell lymphoma (DLBCL) is an aggressive Non Hodgkins Lymphoma (NHL). The anti-CD20 antibody rituximab anchors various immuno chemotherapy regimens, including R-CHOP (Rituximab,cyclophosphamide,adriamycin,vincristine,prednisone) a gold standard for DLBCL. Management of DLBCL in the elderly poses unique challenges given increased risk of toxicity. Anthracycline based regimens are avoided in the elderly leading to lower response rates. We present a case of an elderly patient who achieved complete response with a unique protocol of Adriamycin based therapy.

An eighty-five year old female with multiple medical problems was hospitalised with dyspnea and weakness. Non-tender cervical and inguinal adenopathy was noted. Nodal excision biopsy demonstrated high grade (Ki-67 >90%), B cell ‘double-hit’ lymphoma with MYC/BCL-2 rearrangement and grade IIIB follicular lymphoma. PET/CT revealed splenomegaly and diffuse hypermetabolic adenopathy above and below the diaphragm. A positive bone marrow biopsy established stage IV disease. CSF analysis was negative. The patient and family were very keen on treatment despite her advanced age and borderline performance status (ECOG II). She received 2 cycles of R-CVP (Rituximab, cytoxan, vincristine, prednisone). Adriamycin at 50% of full dose (akin to mini R-CHOP) was introduced at cycle 3 and continued at 75% of full dose for cycles 4–6. Post treatment PET-CT demonstrated excellent response.

DLBCL is the commonest type of NHL, usually presenting in the seventh decade. With improving life expectancy, its incidence in the elderly is predicted to rise. DLBCL is curable with a long term survival rate of 40% with anthracycline based regimens. Multiple non-anthracycline based regimens are available for frail patients. mini-CHOP is a variant with 50% dose reduction of Cytoxan, Vincristine and Adriamycin. Our protocol differed in maintaining full doses of Cytoxan and Vincristine while introducing Adriamycin at 50% of full dose and escalating doses as tolerated. Our patient completed therapy successfully without serious complication with clinical and radiographic improvement during therapy. Complete response was noted at the end of treatment. We propose ‘dose escalated’ mini R-CHOP as an intermediate regimen for the elderly.

243 Bilateral otitis externa masking extramedullary relapse of acute promyelocytic leukaemia

C Rivera-Franceschini1

V Vestal1,2

A Cruz1

1San Juan City Hospital, Ponce, PR

2VA Hospital San Juan

Case report Acute promyelocytic leukaemia (APL) is a subtype of acute myelogenous leukaemia that is characterised by the translocation of chromosomes 15 and 17. The introduction of all-trans retinoic acid (ATRA) as an early therapy has increased the overall remission rates in these patients but relapses still occur. Most relapses are limited to the bone marrow and blood. However, APL can also relapse to extramedullary sites involving the skin, central nervous system (CNS) and other organs. A 48-year-old man with APL on remission for 2 years presented to the emergency room with bilateral ear pain of 3 weeks duration. Pain began after swimming in a pool. It was 8/10, constant and associated with tinnitus, bloody secretions and decreased bilateral hearing acuity. Upon evaluation patient was found with a bilateral swollen and erythematous ear canal with granulating tissue formation. He was evaluated by an otorhinolaryngologist who recommended IV antibiotic piperacillin/tazobactam and dexamethasone/ciprofloxacin ear drops. A maxillofacial CT scan of the head showed opacified external auditory canals bilaterally, which could correlate with mucormycosis infection. Ear infection improved with antibiotics and biopsies of the external ear canals were taken. The left external ear canal biopsy was extensively involved with immature myelocytes, compatible with involvement by acute promyelocytic leukaemia. Bone marrow biopsy was negative for leukaemia. Cerebral spinal fluid analysis was negative for malignancy. Patient was started on ATRA, arsenic trioxide and radiotherapy. Even though CNS involvement is the most common cause of extramedullary relapse in APL, a biopsy of the ear canal should be considered in a patient with suspected otitis externa since it could be a rare presentation of APL. It is imperative to identify this type of extramedullary relapse considering that patients could benefit from induction chemotherapy.

244 Ldh as an early marker for primary myelofibrosis?

EB Saul

B Dreiling

University of Mississippi Medical Centre, Madison, MS

Introduction Primary myelofibrosis (PMF) is a type of chronic myeloproliferative neoplasm with an estimated incidence of 1.5 per 1 00 000 per year occurring mainly in middle aged and older adults. There are various different clinical manifestations of the disease some of which include fatigue, low-grade fever or night sweats, weight loss and splenomegaly.

Case A 68 year old Caucasian male presented with a mild hypoproliferative anaemia of obscure origin that dated back to 2012. He has had no fevers, night sweats or weight loss. In 2012, his haemoglobin and hematocrit (H/H) were 11.6 and 35.2 respectively however has gradually trended down to 9.1/29.2. WBC and platelet counts are normal. Interestingly, his LDH was elevated in 2012 at 670 and has continued to increase over the years to a current level of 1057. LDH fractionation was done revealing fraction 2>1>3>4>5 suggesting a bone marrow, cardiac or renal aetiology. CPK, renal function and cardiac workup were all normal. Peripheral blood smear revealed nucleated red blood cells, moderate large platelets, elliptocytosis, and teardrop cells. Bone marrow biopsy was done given concerns for ineffective hematopoiesis. Pathology returned as a myeloproliferative neoplasm, best regarded as primary myelofibrosis. A JAK2 V617F mutation analysis was sent and returned positive. His spleen measured 14.14 cm in length on ultrasound. He remains asymptomatic with a DIPSS-plus score of 2 and a median overall survival of 2.9 years. However, given that he is asymptomatic with stable blood counts he has chosen observation at this time.

Discussion There are various nonspecific abnormal laboratory tests in patients with primary myelofibrosis. Since there is no ‘gold standard’ for the diagnosis of PMF, there have been diagnostic criteria that have been proposed by the WHO. LDH is an enzyme found in the marrow and blood precursors. With intramedullary hemolysis associated with PMF, one could presume elevations in LDH may actually precede changes in the CBC assisting in the early detection of PMF.

245 Cutaneous lymphoma: the non resolving nodule

P Shelley

KR Green

UF Health, Jacksonville, FL

Introduction Primary cutaneous anaplastic large cell lymphoma is rare malignancy which characteristically exhibits CD30 positivity. Usually presenting in the sixth decade with spontaneous solitary or grouped nodules that fail to resolve. Diagnosis is made by skin biopsy which demonstrate characteristic large cells with eosinophilic cytoplasm and occasional horseshoe shaped nuclei with evidence of epidermal hyperplasia. Treatment modalities include localised surgical excision and systemic chemotherapy. The latter isreserved for those cases with widespread lesions.

Case report A forty three year old female with a history of non-hodgkin’s lymphoma and sarcoidosis presented to an outside institution with a complaint of progressive swelling in her right axilla for the past five weeks. The mass was both itchy and tender to palpation. She also complained of fevers, night sweats, and a thirty pound unintentional weight loss. Due to concern for potential abscess an incision and drainage was attempted but upon incision no spontaneous drainage was seen. She was prescribed a course of antibiotics and instructed to follow up wither her primary care physician. She then presented to our institution with complaint of multiple flesh coloured skin lesion which spontaneously erupted over the past week in sporadic areas from head to toe sparing the palms and soles. Further exam revealed numerous five to ten millimetre, firm, well circumscribed lesions on the breasts, arms, thighs, abdomen, back, neck, and face. Along with a large five by three tender and firm mass in the right axilla. A punch biopsy was performed of the lesions on the left breast and right clavicle. Dermatopathology of the aforementioned samples were positive for anaplastic large cell CD30 positive lymphoma. She was then referred to an oncologist to discuss treatment options and to begin staging of her malignancy.

Discussion This case highlights the necessity of prompt evaluation of skin lesions via biopsy, especially in those patients with previous history of malignancy. Most cases of primary cutaneous anaplastic large cell lymphoma have a ten year survival rate of ninety percent if treated appropriately. However recurrence is common. Systemic chemotherapy is typically reserved for those with disseminated disease or those with multiple recurrences with surgical excision.

246 Extended thirty month progression free survival with third line eribulin for metastatic breast cancer

S Singal

R Khalaf

D Jaishankar

East Tennessee State University, Johnson City, TN

Case report Eribulin is a non-taxane microtubule inhibitor approved for treatment of metastatic breast cancer after two prior chemotherapeutic regimens. Eribulin as third line agent in the palliative setting has shown a median overall survival (OS) of 13 months and median progression free survival (PFS) of 3 months. We report a patient with extended (PFS) of more than 30 months with metastatic breast cancer treated with Eribulin in the third line setting. A forty-eight year old lady was diagnosed with stage IIA (T2N0M0), high grade, triple negative, invasive ductal carcinoma (IDC) of the left breast. She underwent neo adjuvant chemotherapy with Adriamycin and Cytoxan followed by a negative sentinel lymph node biopsy. At mastectomy she was noted to have a 2.5 cm tumour, high grade, triple negative IDC with three additional lymph nodes negative for metastatic carcinoma. She subsequently pursued further chemotherapy and was treated with 6 cycles of Cytoxan, Methotrexate and Fluorouracil (CMF). She then transferred care to our cancer centre and eight months into her surveillance program developed a 2.8 cm right lower lobe (RLL) pulmonary mass with SUV of 27 on a PET-CT. A fine needle biopsy was consistent with metastatic triple negative breast cancer with sheets of poorly differentiated carcinoma similar in morphology to previous breast pathology. Imaging studies revealed oligometastatic disease. She commenced single agent Taxol in the 1 st line metastatic setting with dramatic decrease in RLL pulmonary mass to less than 1 cm with SUV of 1.7 and resolution of other sub cm pulmonary nodules. The response was short lived lasting only six months. She started 2nd line Gemcitabine with subsequent largely stable disease for a period of 11 months. Progression of RLL pulmonary nodule measuring 2.1 cm with SUV of 10 noted. She then started 3rd line Eribulin with a dramatic response on imaging studies within three months and has maintained no evidence of disease (NED) on scans over the subsequent 30 months. She is clinically stable and her tumour markers have plateaued. She has required Eribulin dose reductions on account of neuropathy. Our patient has shown excellent response and tolerance to Eribulin with PFS of over 30 months (ten times the norm) which is rare.

247 Intravascular large b cell lymphoma presenting as chronic cough

S Singal

R Khalaf

D Jaishankar

E Spradling

East Tennessee State University, Johnson City, TN

Case report Intravascular large B cell lymphoma (IVLBCL) is a rare and aggressive subtype of large cell lymphoma that is characterised by proliferation of lymphoma cells within the lumina of small blood vessels, particularly capillaries and post-capillary venules most commonly reported as cutaneous and CNS lesions. We report a rare case of IVLBCL involving the lungs. A 73-year-old male, never smoked, presented with persistent cough and hyponatremia. Chest imaging showed calcified mediastinal adenopathy. Pulmonary function testing showed no restrictive or obstructive pattern. Bronchoscopy with endobronchial ultrasound guided biopsy was non-diagnostic. Patient underwent video assisted thoracoscopy with right upper middle and lower lung wedge biopsies. Pathology showed IVLBCL with large B cells located mainly intracapillary and intra-small artery and veins. Tumour cells were positive for CD20, CD79a, Pax-5, CD10, Mum-1. Tumour cells were negative for CD3, CK AE1/AE3, S100, Cam5.2, CD34. Bone marrow biopsy and aspiration showed minimal involvement of large B-cell lymphoma. PET scan revealed focal uptake at T4, T9, L2, L4 vertebrae concerning for lymphomatous involvement of the bone. Cerebrospinal fluid was negative for malignancy. Brain imaging was negative. Patient was diagnosed with Stage IV IVLBCL. During recovery from right lung wedge biopsies he developed acute respiratory failure requiring oxygen at 10 L/min. Urgent chemotherapy with R-CHOP cycle 1 was initiated providing complete resolution of all symptoms. The final treatment plan includes 6 cycles of R-CHOP with CNS prophylaxis.

Our case demonstrates extranodal disease, aggressive biology, and the requirement of urgent treatment. The rarity of this disease and difficulty of detecting intravascular infiltration often contributes to delay of diagnosis and high mortality.

248 Penile lymphomatous disease portends poor prognosis?

F Tawadros

D Reddy

K Krishnan

D Jaishankar

ETSU, Johnson City, TN

Case report Diffuse Large B Cell Lymphoma (DLBCL) accounts for 25% of all non-Hodgkin Lymphomas. DLBCL presents as a rapidly enlarging mass, usually nodal enlargement, or less often as a mass lesion anywhere in the body. We present a patient with DLBCL with highly atypical extra nodal presentation. A sixty-two year old male presented with persistent right upper quadrant abdominal pain and weight loss over six weeks. Imaging studies suggested gallbladder adenomyosis and patient underwent a cholecystectomy. Pathology reported DLBCL, activated B cell type, CD20 +and MUM1 +with Ki67 90%. Molecular studies ruled out double and triple hit lymphoma but were positive for additional copies of IgH, amplification of BCL-2 gene and BCL-6 gene deletion. Clinical exam revealed significant penile induration. PET-CT revealed mediastinal and abdominal adenopathy with mild tonsillar, parotid, and massive penile involvement. CSF cytology, MRI Brain, Testicular Ultrasound and Bone marrow biopsy were negative. Chemo immunotherapy with R-CHOP and intrathecal methotrexate was initiated. Dramatic regression of penile induration which served as an index lesion, was noted within days. Post treatment PET-CT documented complete response. Three months after therapy he developed a biopsy proven intracranial relapse in the left occipital lobe. He succumbed to his illness while receiving palliative salvage chemotherapy with high dose cytarabine and methotrexate. DLBCL is a heterogeneous entity derived from germinal centre B cells or post-germinal centre B cells. Extra nodal disease (liver, lung, skin) is a less common but well known occurrence. Penile involvement may present as primary penile lymphoma or secondary to systemic disease. While testicular lymphoma is well reported, secondary penile involvement is rare accounting for less than 1% of extra nodal disease. Prognosis in DLBCL is dictated by clinicopathologic factors and extra nodal involvement portends higher relapse risk. Paramedian lymphoma is associated with a risk of CNS relapse. Our case highlights a high risk variant of DLBCL with atypical presentation, multiple rare extra nodal sites of disease, unusual molecular features, excellent response to therapy culminating in CNS relapse.

249 A suspicious case of pernicious anaemia and vitamin b12 deficiency

A Traina

J Dubuc

H Oddo Moise

A Coulon

A Bourgeois

J Doan

S Guillory

S Sanne

LSUHSC, New Orleans, LA

Case report A 56 year old man with a past medical history of hypertension was brought to the emergency department with progressively worsening dyspnea on exertion and fatigue for 2 months. He endorsed subjective fever for 2 weeks and his family noted confusion over several days. He also reported 15 pound weight loss for several months. He was febrile to 105.5F on arrival. The physical exam was significant for a systolic flow murmur, mild confusion and slowed speech with unclear baseline neurologic exam, and normal sensation and proprioception in the distal extremities. Initial labs revealed pancytopenia and a macrocytic anaemia with WBC of 2.3 103/UL, ANC of 100 cells/µL, haemoglobin 8.7 g/dL, hematocrit 26%, MCV 113.9 FL and platelets 41 103/UL with schistocytes and tear drop cells. He underwent further studies for pancytopenia and fever with negative infectious work up including HIV, blood and urine cultures. His vitamin B12 level was profoundly deficient at 64 PG/mL with normal folate and iron studies. His reticulocyte count was inappropriately normal at 0.9%. Haematology was consulted due to concern for hematologic malignancy. There were no blast cells identified on peripheral blood smear. Bone marrow biopsy and flow cytometry revealed hypersegmented neutrophils, but there was no definitive immunophenotypic evidence of hematopoietic neoplasia with normal CD markers, immunophenotyping and cytogenetics. There was no identifiable source of fever or malignancy on CT images of the chest, abdomen or pelvis. The patient was started on intramuscular vitamin B12 supplementation with improvement in all cell lines. Intrinsic factor antibody test was elevated at 32.6 AU/mL and anti-parietal cell antibody was also elevated at 71.6 units, consistent with pernicious anaemia.

Discussion Although pancytopenia may be caused by severe vitamin B12 deficiency, it is not usually accompanied by fevers and warrants and infectious and malignancy work up. We present a case of profound B12 deficiency due to pernicious anaemia, an autoimmune disorder characterised by the destruction of parietal cells of the gastrointestinal system resulting in vitamin B12 deficiency. Lifetime intramuscular B12 supplementation will be required and can prevent progression of irreversible neuropsychiatric changes.

250 Chemotherapy toxicity confirms diagnosis of urachal carcinoma

D Vesselinovitch1

K Thomas1,2

M Matrana1,2

1University of Queensland, Ochsner Clinical School, New Orleans, LA

2Ochsner Health System, New Orleans, LA

Purpose of study The urachus is a fibrotic remnant of the allantois, a canal that collects liquid waste and exchanges gases from the fetal bladder with the umbilical cord. Urachal cancer is a rare and aggressive cancer that often presents as adenocarcinoma at the dome of the bladder. If found early and confined to the urachus, cystectomy with en bloc re-section of the urachal ligament and umbilicus can be curative. For inoperable patients, chemotherapy generally has low efficacy. Gem-FLiP (gemzar, 5-FU, leucovorin, and cisplatin) is undergoing clinical trials but its efficacy and adverse effects with urachal carcinoma have not been confirmed.

Methods used A 76-year-old male with a long history of genitourinary disease including renal stones, urethral stricture, and transitional cell carcinoma in situ of the bladder presented with gross painless hematuria. A flexible cystoscopy showed a sessile tumour at the dome of the bladder. A CT Urogram of abdomen and pelvis confirmed an enhancing, exophytic mass at the dome of the bladder. A CT guided biopsy revealed adenocarcinoma consistent with urachal cancer.

Summary of results The patient began chemotherapy with Gem-FLiP. He developed pedel oedema and drainage of the umbilicus. A CT of abdomen and pelvis showed an anterior extension through the abdominal wall just below the umbilicus as well as central hypo attenuation within the mass in the bladder; this represented a fistula between the bladder and umbilicus, confirming the diagnosis of urachal cancer. Following completion of the second cycle, restaging showed partial response, but the patient was intolerant of the regimen. The patient began FOLFOX treatment (leucovorin, fluorouracil, and oxaliplatin). FOLFOX has been supported for adenocarcinoma in peer reviewed literature. He tolerated this but was hospitalised shortly after for recurring anaemia.

Conclusions In this case, Gem-FLiP produced an impressive partial response. The formation of a fistula along the anatomic location of the urachus confirmed the diagnosis of urachal cancer that was strongly suspected. It is highly unusual that a response to chemotherapy confirms a diagnosis.

251 Primary lung diffuse large b-cell lymphoma with hepatic metastases: a rare neoplasm

Y Vorakunthada1

W Lilitwat2

1Texas Tech University Health Sciences Centre Lubbock, Lubbock, TX

2University of Iowa Hospitals and Clinics, Iowa City, IA

Case report Primary pulmonary lymphoma is very rare. Here, we present a 66-year-old male with primary lung diffuse large B cell lymphoma (DLBCL) who presented with productive cough and shortness of breath for a week. He had weight loss of 40 lbs. over 2 months. His exam showed decreased breath sounds with dullness on percussion on the right side of chest. Contrast-enhanced CT of chest and abdomen revealed 6.6×4.5 cm right hilar mass, 3 and 4 mm nodules within right upper lobe, nodular thickening within the right middle lobe (figure 1), and multiple hypodensity lesions throughout the liver. Bone marrow biopsy was negative for malignancy. However, both transbronchial needle aspiration and liver biopsy showed diffuse, high-grade B cell lymphoma, confirmed by positive immunohistochemistry of CD20, CD79a, CD10, BCL-2 and Ki-67. He responded well with rituximab, cyclophosphamide, doxorubicin, vincristine, and prednisone (R-CHOP).

Primary pulmonary DLBCL represents only 0.04% of all lymphoma cases. In our case, hilar mass, multiple pulmonary and hepatic nodules raise a concern of lymphoma. We do not find radiographic characteristics of intratumoral bronchial translucency.1 For prognosis, positive CD99 DLBCL has a better 2 year survival. However, prognostic factor for primary lung DLBCL has not been reported. R-CHOP was started due to strong CD 20 expression for rituximab. Nonetheless, the outcomes are contradicting between R-CHOP and CHOP regimen.

Pulmonary lung DLBCL with hepatic metastasis should be suspected in lung mass and multiple hepatic nodules. This rare entity needs to be studied for effective chemotherapy treatment.

Reference

  1. . Saitoh Y, et al. Unique radiological features of two cases of primary pulmonary diffuse large B-cell lymphoma. Thorax2017;72(9):859–60.

Abstract 251 Figure 1

CT chest with mediastinal (A) and lung (B) window settings

252 A rare case of epidural follicular lymphoma

MH Williams

C Capra

C Milner

UMMC, Jackson, MS

Case report Back pain is one of the most common complaints encountered in medicine, with more than 80% of adults experiencing back pain during their lifetime. The most frequent aetiology of back pain is lumbar strain. Malignancy accounts for less than 1% of cases of back pain, with follicular lymphomas comprising less than 10% of those. Here we present a very unusual case of follicular lymphoma presenting as worsening low back pain.

A 46-year-old male with a previous traumatic back injury with mild, chronic cauda equina syndrome and severe chronic back pain presented with a new and different constant dull ache in his back. He denied any new weakness, difficulty ambulating, bowel or bladder incontinence, weight loss, night sweats, fever, chills, lymphadenopathy, and headaches. Neurologic examination was normal and there was no palpable adenopathy or splenomegaly. Magnetic resonance imaging (MRI) of the lumbar spine revealed an 8.5 cm epidural mass extending out multiple neural foramen and causing severe spinal cord stenosis, greatest at T12-L1. Additional imaging, including MRI of the brain, computed tomography (CT) of chest, abdomen, and pelvis, and bone scan, demonstrated extensive osseous metastases involving the left frontal skull, right distal humerus, and thoracolumbar spine. There was also adenopathy in the left axillary region and multiple large masses in the mediastinum and paraspinal soft tissues. Patient then underwent bilateral laminectomies at T12-L3 for partial resection of the lumbar spinal cord mass. Histopathology revealed a grade 2 follicular lymphoma with fluorescence in-situ hybridization (FISH) detecting a t(14;18). He was treated with four cycles of Bendamustine and Rituximab with subsequent positron emission tomography/computed tomography (PET/CT) demonstrating a complete response.

Although it is uncommon for follicular lymphoma to involve organs outside of the lymphatic system and bone marrow, cord compression may develop when epidural tumours do occur. Follicular lymphoma uncommonly involves the spinal cord, with an epidural location for lymphoma occurring in only 0.9%–6.5% of previously undiagnosed non-Hodgkin lymphomas. Even though epidural follicular lymphoma is rare, and malignancy in general is a less common cause of low back pain, clinicians should consider it in the differential diagnosis since treatment delay can adversely affect outcomes.

253 Recurrent cervical cancer: a unique path to diagnosis

MH Williams

RA Williams

D Hansen

L Puneky

K Wilkinson

UMMC, Jackson, MS

Case report Cervical cancer is the third most common gynecologic cancer in the United States and human papillomavirus (HPV) is the etiologic agent of 99.7% of cases. The most common histologic type of cervical cancer is squamous cell carcinoma. Here we present a case of cervical cancer originally thought to be urothelial carcinoma with squamous differentiation given its immunohistochemical profile.

A 53 year old black female presented with progressively worsening back pain, anorexia, and a 40 pound weight loss. She had a history of stage 1B squamous cell carcinoma of the cervix treated definitively with neoadjuvant chemoradiation and hysterectomy. Computed tomography (CT) of the abdomen and pelvis revealed a large retroperitoneal mass that extended into multiple lumbar vertebra, with extensive osseous destruction and spinal canal involvement. The mass also invaded the psoas muscle and encased the inferior vena cava, aorta, and right ureter. Pathology from biopsy of the mass was thought to be consistent with high grade urothelial carcinoma with squamous differentiation given that p40, p63, CK 5/6, and GATA3 were positive, and PAX8 was negative. She was initially treated with 5-fluorouracil (5-FU), mitomycin-c, and radiation for stage IV urothelial carcinoma. However, due to her history of cervical cancer, we were concerned for recurrent disease. Subsequently, her pathologic specimen was stained for p16 and tested for in situ HPV. Both returned positive, supporting our suspicion. After further review of imaging and pathology, her tumour was felt to be consistent with recurrent cervical cancer rather than urothelial carcinoma. She will be treated with cisplatin, paclitaxel, and bevacizumab.

GATA3 is a transcription factor that is expressed in >90% of primary urothelial carcinomas; however, it is not specific. Squamous cell carcinoma of the cervix less commonly displays this marker with an estimated 33% expression. PAX8 is positive in 91% of cervical lesions, but was negative in this instance. Although the pathologic profile seen in this case is less common in cervical cancer, it highlights the importance of considering the overall clinical picture when making a diagnosis. Additionally, p16 positivity helped support the diagnosis of cervical cancer as it is an immunohistochemical marker strongly associated with high risk HPV subtypes.

254 Chop-r is an efficient treatment for primary dural diffuse large b-cell lymphoma (pd-dlbcl): a systematic review of 45 cases

K Zakharia

ZL Quinn

L Yin

TC Brown

JJ Schmieg

H Safah

NS Saba

Tulane University, New Orleans, LA

Purpose of study PD-DLBCL is an aggressive lymphoma that affects the Dura mater, imitating other central nervous system tumours, and remains with unclear optimal management.

Methods used We conducted a retrospective review of the literature on pathologically confirmed PD-DLBCL and analysed data on biology, treatment outcomes, and survival.

Summary of results Out of 245 screened cases, 45 cases of PD-DLBCL were detected. 16 cases were intra-cranial and 29 were intra-spinal. Median age at diagnosis was 59 years. Incidence was nearly equal between women (22/45) and men. When tested, CD20 was positive in each instance (21/21). Using Hans criteria when possible to determine cell of origin, 3 cases were classified as ABC-DLBCL and 5 as GCB-DLBCL, confirming the representation of both subtypes in PD-DLBCL. All cases were stage IE and 6 of the 9 cases which provided Ki-67 data were less than 70%, reflecting an overall less aggressive behaviour. Survival data available from 40 cases showed an OS of 84% at 1 year, and 81% at 5 years, which compares favourably to PCNSL and matches early-stage DLBCL. Tumour location (intracranial vs intra-spinal) did not impact OS (p=0.82). Treatment was reported in 19 cases with available survival data. 11 patients received CHOP, 6 of which additionally received rituximab (CHOP-R). Eight patients received high-dose methotrexate (MTX)-based therapy. Interestingly, no difference in OS was observed between CHOP vs MTX-based therapy (p=0.97), suggesting that PD-DLBCL should be treated as DLBCL rather than PCNSL. Moreover, all patients who received CHOP-R remained disease free and alive. Radiation therapy was given often (25/29) in treatment of spinal disease, but rarely (4/16) when treating cranial disease, but did not impact OS.

Conclusions The good outcomes associated with CHOP-R eliminate the need for applying more toxic treatment regimens such as High-dose MTX or radiation therapy, and are consistence with the PD-DLBCL location outside the blood brain barrier.

Infectious disease, HIV, and AIDS, Joint plenary poster session and reception, 4:30 PM, Thursday, February 22, 2018

255 Outbreak of penicillin-resistant meningococcemia in rural appalachia

S Albracht

D Macariola

JW Schweitzer

DL Wood

East Tennessee State University, Johnson City, TN

Purpose of study In Spring 2017, multiple N. meningitidis cases erupted in rural Appalachia. N. meningitidis in the United States is typically penicillin-sensitive. It was hypothesise N. meningitidis isolated among paediatric patients in the Appalachian outbreak would be penicillin-sensitive and patients would have identical clinical presentations and CSF studies.

Methods used Data was compiled via qualitative retrospective case series. Inclusion criteria were age <18 years plus positive CSF studies, or presence of meningismus plus petechiae, fever, and antibiotic treatment prior to lumbar puncture. Three subjects met inclusion criteria. Subjects received 7 day course of Ceftriaxone or Meropenem if Ceftriaxone-allergic.

Summary of results Table 1 presents subject findings. All subjects improved with treatment. Mortality rate was 0% with no sequelae to date.

Conclusions Although subjects had identical classic meningococcal symptoms (meningismus, fever, and petechiae), exact skin findings varied. CSF studies were not identical among subjects, even among those who did not receive pretreatment with antibiotics. Ceftriaxone and Meropenem adequately treated subjects. The penicillin-resistant N. meningitidis recovered in this small study and variety of subject findings did not support the original hypothesis. Health care providers in Appalachia should strongly consider non-penicillin therapy with future meningococcal outbreaks and recognise that not all cases may have identical findings.

Abstract 255 Table 1

Subject findings

256 Mycoplasma cerebellitis with hydrocephalus

P Ameta1,2

VH Nayak1,2

JJ Burns1,2

1Sacred Heart Children Hospital, Pensacola, FL

2University of Florida, Pensacola, FL

Introduction CNS manifestations of mycoplasma pneumoniae occur in approximately 0.1 percent of all patients, which includes aseptic meningitis, encephalitis, cerebellar ataxia and cranial nerve palsies, this can be early onset or late onset.

Case 9 yo M with history of cough and fever (Tmax of 101F) for 2 weeks, was initially evaluated by PCP, where he was diagnosed with pneumonia and was prescribed 10 days of amoxicillin. His fever and cough resolved in 3–4 days, but a week later, he developed headache, in occipital region, moderate to severe in intensity associated with unsteady gait, poor appetite and non-bilious vomiting, 2–3/day. As he wasn’t improving, mom brought him to the hospital. His initial labs were: CBC showed reactive thrombocytosis (plt-575,000), BMP was normal, RPP was positive for mycoplasma pneumoniae, CT head – normal, CSF – 55 wbc with lymphocytic predominance, proteim and glucpse normal, enterovirus CSF PCR was negative, and chest x ray was negative.

He was admitted to floors with working diagnosis of aseptic meningitis. He continued to worsen even after a day of fluids and symptomatic management. He was requiring opioids every 1–2 hours for his headache. Hence, an MRI of his brain was obtained, which showed cerebellar oedema suggestive of cerebellitis with changes of early acute obstructive hydrocephalus. He was transferred to PICU and was started on high dose steroids (dexamethasone at 1 mg/kg) to decrease cerebellar oedema and azithromycin for 5 days. He showed dramatic improvement with in 24 hours. His steroids were tapered gradually and stopped prior to discharge.

Additional labs were obtained – CSF encephalitis panel including mycoplasma pneumonia was negative, CSF culture and blood culture were negative, serum IgM for mycoplasma was negative but serum IgG was positive.

Discussion Exact pathogenesis of neurological manifestations caused by mycoplasma is unknown. However, it is postulated that early onset is due to direct invasion and late onset is due to immune mediated process. In our patient, latency between respiratory and neurological symptoms, lack of any other aetiology, absence of mycoplasma in CSF, and with positive mycoplasma in RPP led to the conclusion that he might have had late onset CNS disease due to mycoplasma.

257 A deleterious combination of intravenous drug usage with a chronic viral infection; how renal replacement therapy emanated?

C Castillo Latorre1,2

1San Juan City Hospital, San Juan

2Centro Medico De Puerto Rico, San Juan

Purpose of study Intravenous drug usage of substances like cocaine and heroin; encompasses a spectrum of generalised state of illness. A continuum insult which predispose patients to chronic viral illnesses, bacterial infections and subsequently end organ damage due to multiple factors. The renal structure is one of the target organs involved in this process, by which a majority of them will lately developed end stage renal disease and as a result renal replacement therapy. However, the spectrum of complications of this population is enormous starting with acquire infections like HIV, Hepatitis C, Hepatitis B, severe skin infections, pneumonia, cardiovascular diseases, endovascular complications as the well known Lemierre’s syndrome, central nervous system infections, systemic complications like renal failure ending up in hemodialysis and most of them with a low expectancy of life. Our purpose was mainly in finding most common conditions associated with intravenous drug usage and compared them.

Methods used Electronic medical record was used to reach patients with documented to be active intravenous drug users. Of the patients been study the admission diagnosis that lead to renal replacement therapy was recorded, and later in the process compared.

Summary of results Our population of 69 patients, 16 of them ended up in hemodialysis 23% (16/69), of this patients that had renal replacement therapy 4 of them had Hepatitis C 25% (4/16), Hepatitis B 19% (3/16), HIV 13% (2/16), Hypertension 6% (1/16) and Diabetes mellitus 6% (1/16) respectively. There were several precise findings that lead to hemodialysis in the population, with the majority been infected ulcers 25% (17/69), multilobar pneumonia 20% (14/69),upper gastrointestinal bleeding in 10%(7/69) and symptomatic anaemia 10% (7/69) respectively. All of this patients did not had good social support, none of them knew about the long term consequences of renal failure and most of them did not had positive approach of stopping drug usage.

Conclusions As a whole, ilicit intravenous drug usage is associated with a broad spectrum of diseases, all of them creating a rapid deleterious clinical picture; mostly debuting to medical assistance with an infectious aetiology and almost 25% will require lifelong hemodialysis.

258 Intracranial hypertension secondary to eosinophilic meningitis by angiostrongylus cantonensis

RA Cruz

C Smith

AB Ramos

BJ Copeland

PS Seal

LSUHSC, New Orleans, LA

Purpose of study Increase awareness to practitioners about A. Cantonensis in patients with eosinophilia specially if patients have exposure history, or have recently travel to endemic areas now including Louisiana, Texas, or Florida.

Methods used Single case report.

Summary of results Angiostrongylus Cantonensis, a nematode, is a well-known cause of eosinophilic meningitis in endemic areas such as Southeast Asia, the Pacific Islands, and Hawaii. Nevertheless, an increasing number of cases in the southeast of the United States have been documented recently, specifically in Louisiana, Texas, and Florida. Infection is acquired after the undercooked fresh water snails, mollusks, or undercooked vegetables contaminated by the slime from infected snails or slugs. Typical signs and symptoms include fever, general malaise, meningeal signs, headaches, photophobia, nausea and vomiting. Here we present a 23 year-old female that presented to our emergency department with signs and symptoms consistent with intracranial hypertension, malaise, mild photophobia, and without fever, or meningeal signs.

Conclusions Angiostrongylus Cantonensis, is the most common cause of eosinophilic meningitis, presentation can be atypical including intracranial hypertension signs and symptoms without significant meningeal signs. It is important for practitioners to consider A. Cantonensis in patients with suspected intracranial hypertensions, or meningitis if eosinophilia is present. Diagnosis is made with PCR for A. Cantonensis. The treatment is focus on decreasing intracranial pressure, and corticosteroids to reduce meningeal inflammation. Anti-helminthics are not recommended as they can induce a larger immune response and therefore worsen the symptoms.

259 Chylous ascites in intra-abdominal mycobacterium avium complex immune reconstitution inflammatory syndrome

R Dean

R Subedi

A Karkee

Upstate Medical University, Liverpool, NY

Case report Chylous ascites, a rare form of ascites, is defined as leakage of lipid-rich lymph into the peritoneal cavity. It has been described very rarely in HIV/AIDS patients related to intra-abdominal Mycobacterium avium complex immune reconstitution inflammatory syndrome (MAC-IRIS). We present a 51 year old male with a history of AIDS and a colonic MAC infection that presented with abdominal distension and chylous ascites.

A 51 year old male with a history of AIDS and colonic MAC infection presented for abdominal distention. One year prior, he had been diagnosed with colonic MAC on biopsy and at the same time, with AIDS, and had an initial CD4 count of 9 cells/µL. He was started on HAART therapy with ritonavir, darunavir, lamivudine and efavirenz as well as levofloxacin, ethambutol and rifampin for treatment of his MAC infection. His CD4 count increased to 150 cells/µL and undetectable viral load at the time of presentation. Physical examination showed abdominal distention with tense ascites. A paracentesis removed 9050 mL milky white ascitic fluid. Fluid analysis showed a triglyceride level of 1487 mg/dL with a high lymphocyte count. Analysis revealed no signs of infection or malignancy. There was no history of trauma or signs of cardiac or autoimmune causes. The patient was continued on his MAC and HAART medications, as well as intermittent therapeutic paracenteses.

Chylous ascites is rare in HIV/AIDS patients and its association with MAC-IRIS has been rarely reported. The diagnosis is made by fluid analysis, which shows a milky white ascitic fluid with triglyceride levels greater than 110 mL/dL and a high leukocyte count with a mononuclear predominance. The etiologies include neoplastic, congenital, acquired cardiac or gastrointestinal causes, inflammatory causes, and most commonly manipulation of lymph drainage in surgery or trauma. Infectious causes from tuberculosis and MAC in HIV patients have been described, though rarely in MAC infected patients as a result of IRIS. It is caused by well-formed granulomas in the lymphatics. Because granuloma formation is dependent on CD4 cells to stimulate macrophages, it is not until the patient demonstrates increasing CD4 counts in IRIS that we see chylous ascites, making it a late complication of disseminated MAC infections.

260 Chronic treatment of cutaneous chromoblastomycosis in a paediatric patient

VC Diaz Vidal

A Mirza

University of Florida College of Medicine Jacksonville, Jacksonville, FL

Case report Chromoblastomycosis is a chronic fungal infection which typically affects the skin and subcutaneous tissue. It can be found normally in the environment, in soil, wood and decaying material. Chromoblastomycosis can be due to several organisms from the dematiaceous group of fungi, with Phialophora verrucosa, Cladosporium carrionii, and Fonsecaea species, among the most common. Infection is usually due to traumatic inoculation of the organism and generally presents as papillomatous, verrucous or vegetative lesions. Chromoblastomycosis is one of the most difficult mycotic infections to eradicate, despite multiple treatment options which include prolonged courses of oral antifungals, surgical removal, and destructive physical therapies. It occurs more commonly in tropical and sub-tropical areas, but world-wide cases have been reported. We present the case of a 13-year-old female with cutaneous chromoblastomycosis, who presented initially with an erythematous pinpoint lesion on her left cheek back in 2014. She was started on oral antifungals of which itraconazole proved to be successful. She has been on oral itraconazole for 13 months or so, with marked improvement in size and contour of the lesion, proving that proper compliance, follow-up and anticipatory guidance are key in the treatment of chronic infections.

Abstract 260 Figure 1

Facial lesion after being on oral terbinafine for 5 months

261 Hhv-6 cns infection in a young infant

ML Dietrich1,2

A White3

J Schieffelin1

K Queen4

1Tulane Hospital for Children, New Orleans, LA

2Ochsner Hospital for Children, New Orleans, LA

3Tulane School of Medicine, New Orleans, LA

4Lakeview Regional Medical Centre, Covington, LA

Case report This case presentation will review the course and treatment of an infant with the rare finding of HHV-6 CNS infection, the current literature available on the subject, as well as touch on the increasing challenges we may face in clinical decision making as more advanced and rapid diagnostics are becoming available. H is a 25 day old baby boy, previously healthy, who presented with 1 day of fever and runny nose. Full sepsis work-up was initiated, and meningitis/encephalitis PCR panel of the CSF demonstrated Human Herpesvirus-6 (HHV-6). Initial symptoms included an episode of apnea and bradycardia in the emergency room, and a possible staring spell on day two. MRI demonstrated small non-specific area of restriction. On day two HHV-6 quantitative serum PCR was sent and resulted with 12 000 copies per mL of virus. He was treated with intravenous ganciclovir for 14 days without incident. Repeat HHV-6 quantitative serum PCR demonstrated presence of virus, but too low to quantify, and confirmed HHV-6 subtype B. HHV-6 is a common infection in young children presenting with rash and fever, coined roseola infantum. While this illness is well described, the CNS manifestations of the virus are less well understood. With the fairly recent advent of the widespread use of rapid, sensitive diagnostics presumably diagnosis of this infection in the CNS will become a more common occurrence. The literature on CNS HHV-6 is for the most part limited to severely immunosuppressed patients, as well as those presenting with obvious signs and symptoms consistent with encephalitis. Efficacy of ganciclovir is limited to demonstration of decrease in viral load, without paired controls. There also exists chromosomal integration of HHV-6 in the gametes, which can be passed to offspring, which occurs in an estimated 0.2% to 0.8% of births. A better understanding of the significance of the presence of the virus in CSF is needed in order to dictate treatment and prognosis for the febrile, immunocompetent infant in whom this virus is identified in the CSF.

262 Nocardiosis exacerbated by immune reconstitution inflammatory syndrome

J Doan

L Damioli

R Lillis

LSUHSC, New Orleans, LA

Case report A 62 year old woman with past medical history of HIV/AIDS with CD4 48/mm3 and noncompliance with combination antiretroviral therapy (cART) was admitted to an outside hospital 2 months prior and diagnosed with cavitary Nocardia farcinica pneumonia. She was treated with imipenem and amikacin, but developed a morbilliform drug eruption concerning for DRESS; dermatology determined that the rash was more likely secondary to imipenem. Her treatment was discontinued after only twelve days of imipenem and seven days of amikacin. Repeat CT chest showed a new cavitary lesion in the right upper lobe of the lung. She was discharged with oral trimethoprim/sulfamethoxazole (TMP-SMX). She followed up with her HIV provider and was started on cART, which she took consistently. She felt well until the following month when she suffered two tonic-clonic seizures and left sided weakness. She denied diplopia, headache, nausea or vomiting. CT brain scan demonstrated a right frontal lobe ring enhancing mass consistent with a brain abscess. She was started on amikacin, moxifloxacin and TMP-SMX with improvement of weakness. She was continued on cART with significant increase in CD4 T-cell count from 48 to 92/mm3 and decrease of viral load from 1 95 128 to 1955 copies/mL. A MRI brain was later repeated with increased size of the brain abscess and vasogenic oedema. She was transferred for evacuation of the abscess. Gram stain from the brain abscess grew filamentous, branching, beaded gram positive rods that were modified acid fast positive that speciated as Nocardia farcinica. She was treated with six weeks of induction with TMP-SMX and amikacin followed by oral treatment with TMP-SMX and minocycline for at least one year given her HIV/AIDS status. cART was held due to concern for immune reconstitution inflammatory syndrome (IRIS) with plans to resume based on continued radiologic improvement.

Discussion Pulmonary Nocardiosis with neurological deficits should prompt evaluation for CNS involvement. Perhaps, early initiation of cART along with inadequate treatment of pulmonary Nocardia resulted in ‘unmasking’ her brain abscess consistent with IRIS, a paradoxical inflammatory response that may result when a patient with HIV/AIDS has regained an immune response.

263 Enlarged thyroid-neither a goitrenor a nodule or a tumour

PK Errabelli

UAMS, Little Rock, AR

Case report A 26 y/o male without significant PMH presented with nausea, vomiting, diarrhoea and decreased urine output for several days. He also complained of a diffuse neck swelling 1 day before presentation. He denied fever, chills, cough, dysuria, sick contacts, recent travel, and tick/bug bites. He was afebrile with stable vital signs. On examination he had B/L thyroid enlargement with tenderness (R>L) and axillary lymphadenopathy. Labs revealed, WBC 12.5, Hb 9.4, Hct 28.2, platelets 143, Na 125, K 3.9, BUN 136, Cr 21, Phos 7.9, AG 18, glucose 104, LDH 1030. The patient was admitted for work-up. He was found to be HIV positive on a screening test. CT showed cardiomegaly w/small/mod pericardial effusion, enlarged kidneys w/perinephric stranding compatible with renal failure, lymphadenopathy, and soft tissue masses w/in lower anterior neck, mildly dilated proximal descending aorta of 3.6 cm.

CD4 count of 113 and HIV RNA copies 4 73 536. Work-up for other sexually transmitted infections was negative. Dialysis was required for the poor kidney[KN1] function(AKI). USG revealed B/L enlarged kidneys with increased cortical echogenecity suggestive of medical renal disease. Kidney biopsy was suggestive of HIVAN (HIV associated nephropathy). Blood culture grew E.coli. The same organism was also isolated from his thyroid aspirate sample which showed suppurative thyroiditis. The thyroid pain and swelling improved on treatment with IV antibiotics and a 2 week course was completed. He is being discharged on PO antibiotics in the appropriate renal dose. He remained dialysis-dependent at discharge. ID f/u for starting HAART as outpatient was suggested at discharge.

We present a rare case of acute infective thyroiditis with E Coli. There are only few case reports in literature.

264 Unusual opportunistic co-infections in aids patient

LO Gerena Montano

MA Farinacci Vilaro

San Juan City Hospital, San Juan, PR

Case report Mucormycosis is an opportunistic fungal infection, 3 genera are known to be human pathogens, Rhizopus, Absidia and Mucor. The incidence is 1.7 per million people per year in the USA. This infection occurs due to the inhalation of fungi spores and results in the rapid progression of pneumonia or endo-bronchial disease and is most common on immunocompromised patient with neutropenia and malignancy.

Mycobacterium avium complex (MAC) is most commonly observed in AIDS patients with a CD4 <50. One survey estimated that 3000 cases of MAC pulmonary disease occurred annual in the USA in the early 1980s.

We present the case of a 53-year-old male with PMHx of HIV not on antiretroviral therapy, crack smoker, former jail convict who presented to ER complaining of general malaise, chills, productive cough, yellow sputum, and SOB of 3 days duration and a previous admission with pneumonia 1 month prior. Physical exam was relevant for: cachectic appearance and bilateral ronchi on lung auscultation. ABGs with hypoxemia and metabolic acidosis +respiratory alkalosis; chest CT with left upper lobe cavitation, nodular opacities on both lower lobes and right upper lobe, bilateral ground glass infiltrates and left sided pleural effusion. Patient was placed on airborne respiratory isolation; started on IV antibiotic therapy for hospital acquired pneumonia (HAP) and Pneumocystis carinni pneumonia (PCP). In view of patient’s clinical deterioration and persistent hypoxemia, sputum samples were sent for cytology and stain which results came back positive for Mucor sp. Also Acid Fast Bacilli stain and PCR samples results were negative for TB but positive for MAC in 2 different samples. PPD was negative.

Pulmonary mucormycosis is a rapidly progressive infection that can spread to contiguous structures or disseminate hematogenously with a mortality of 87%. Widely disseminated mucormycosis have a mortality rate of 90%–100%. MAC has a 5 year mortality of approximately 18%. There is no documented pulmonary co-infection with both Mucor and MAC. Due to the aggressiveness of this conditions and high mortality rate in which patient survival is dependent on an early diagnosis, physicians should be aware of this entity and the possibility of a co-infection in an AIDS patient.

265 Is it a mole? think again!

MF Habib1

D Zheng2

A Islam1

T Naguib1

1Texas Tech Univ HSC Amarillo, Amarillo, TX

2University of Texas at Houston, Houston, TX

Introduction Kaposi’s Sarcoma (KS) is an AIDS defining malignancy in HIV-positive patients associated with HHV-8 co-infection. The incidence of KS has declined drastically since the use of highly active antiretroviral therapy (HAART). Cutaneous involvement is most common but it can also affect visceral organs. In present-day, pulmonary KS is a rare finding. However, high clinical suspension with prompt diagnosis and treatment can lead to improved prognosis and outcome.

Case report 36 y.o. HIV positive male not on HAART therapy presented with a 2 months history of SOB. He was diagnosed with HIV 8 years ago and KS of left nose 7 months ago. CD4 count was 32 and viral load was 1480. Chest X-ray and CT scan of the lung showed bilateral infiltrates, lung biopsy confirmed Pulmonary KS. Patient was started on chemotherapy and HAART.

Discussion Pulmonary KS is seen in low CD4 count patients. The clinical features are non-specific; hence, making the diagnosis indistinguishable from other opportunistic infections involving the lungs. Diagnosis is often based on clinical presentation, imaging studies, and findings on bronchoscopy; lung biopsies are often needed. Histology shows angiogenesis and spindle cells proliferation. Immunohistology: CD31, CD34, and HHV-8 latent nuclear antigen −1. HAART is associated with improvement/resolution of pulmonary symptoms and increased survival time. In more advanced cases, chemotherapy plus HAART is associated with significant reduction in disease progression than HAART alone.

Conclusion With the advent of HAART and decline of incidence over the past 2 decades, young physicians may lack the necessary exposure to suspect pulmonary KS. We suggest, keeping a high clinical suspicion for pulmonary KS, early initiation of HAART with proper patient education regarding disease progression and management would lead to better prognosis and outcomes.

Abstract 265 Figure 1

Lesion on the nose, CT scan and lung biopsy

266 Streptococcus constellatus causing soft tissue necrosis surrounding the trachea in an immunocompromised patient

J Norsworthy

M Hess

SUNY Upstate, Syracuse, NY

Case report Streptococcus constellatus is a member of the Streptococcus milleri group, and this family of bacteria is particularly known to cause pyogenic infections in the oral cavity, as well as the head, neck and abdomen. Complications of pyogenic head and neck infections include mediastinitis, airway obstruction and septic shock. We describe an uncommon case of S. constellatus phlegmon formation resulting in soft tissue necrosis surrounding the trachea.

A 50 year-old male with a history of a tracheostomy secondary to metastatic small cell thyroid cancer presented in septic shock with tenderness and erythema adjacent to his tracheostomy site. He also reported foul smelling drainage that had been ongoing for several weeks. One week prior to presentation, he was started on a new chemotherapy regimen and subsequently was found to have severe neutropenia. A CT scan of the neck demonstrated a defect with multiple foci of air in the anterior soft tissues overlying the right proximal clavicle extending into the superior aspect of the mediastinum, as well as an ill-defined hypodensity and multiple foci of air within the infra-glottic posterior pharyngeal space causing severe narrowing of the subglottic airway. The patient was taken for immediate surgical debridement and a 7 by 2 cm area of necrotic tissue was debrided. The infection was found to have formed a tract communicating with the tracheostomy stoma. His tracheostomy tube was exchanged. Intraoperative wound cultures grew Streptococcus constellatus. The patient did not require further debridement and after several days was discharged on long term antibiotics.

S. constellatus is a known pyogenic pathogen and has been implicated in severe head and neck soft tissue infections. It is important to recognise and treat these infections as they can cause rapid clinical deterioration, especially in immunocompromised patients. Our patient had a favourable outcome because of early diagnosis, surgical intervention, airway stabilisation and antibiotic therapy.

267 An unusual case of brachyspiradiarrhoea in an immunocompetent adult during middle east deployment

L Hoang1,2

A Minocha1,2

M Constantinescu1

V Mekala1,2

R Washburn1,2

1Louisiana State University Health Sciences Centre, Shreveport, LA

2Overton Brooks VA Medical Centre, Shreveport, LA

Background Brachyspira aalborgi and B. pilosicoli are nontreponemal spirochetes capable of causing persistent diarrhoea in paediatric patients and individuals with HIV infection. Otherwise these spirochetes are usually asymptomatic and do not require treatment. Carriage rates are high in developing countries including those in the Middle East (11%–64%) but lower in developed countries (1%–5%). We now report an unusual case of chronic Brachyspiradiarrhoea in an immunocompetent adult.

Case report A previously healthy 33 year old Caucasian male presented with eight years of chronic epigastric and lower abdominal discomfort that began during deployment to the Middle East, associated with bloating, gas, diarrhoea, fatigue, and an involuntary seventy pound weight loss. Physical examination was normal and noninvasive gastrointestinal evaluation was negative, but colonoscopy revealed multiple nodules. Mucosal biopsies showed lymphoid aggregates, and spirochetes were visualised by Steiner and periodic acid Schiff stains as well as immunohistochemistry. Rapid plasma reagin and HIV testing were negative.

Clinical course Treatment with metronidazole 500 mg by mouth thrice daily for ten days led to complete resolution of all previous gastrointestinal symptoms and restoration of normal energy levels.

Discussion Onset of symptoms during Middle East deployment suggested that Brachyspira exposure occurred in that region. This case highlights (1) the importance of performing mucosal biopsies for evaluation of chronic unexplained diarrhoea followed by special stains when morphologically indicated, and (2) the efficacy of metronidazole in treating Brachyspiradiarrhoea.

268 Rothia mucilaginosa native tricuspid valve endocarditis in a neutropenic patient

M Ibrahim

J Yorke

Quillen College of Medicine/ETSU, Johnson City, TN

Introduction Rothia mucilaginosa, while long known to be of a low virulence,1 is being reported here as the culprit of native tricuspid valve endocarditis in a patient with a dental absces. To the best of our knowledge, this is the first case report of right2 sided native valve endocarditis caused by Rothia.

Case presentation A 31-year-old female patient with history of sarcoidosis, treatment-naïve hepatitis C, and remote intravenous drug abuse (IVDA) presented to the emergency department with progressive right-sided back pain of 4 days’ duration. Intermittent right sided toothache, palpitations, shortness of breath, and fatigue were also reported for the last 2–3 months. Physical exam revealed a septic, cachectic, very pale patient in moderate pain and respiratory distress. Labs revealed pancytopenia with mild neutropenia, microcytic anaemia with a haemoglobin of 4.0 g/dl, iron saturation of 3%, and two out of two blood cultures positive for Rothia mucilaginosa. X-ray of the right mandible revealed an apical abscess. Chest x-ray revealed right middle and lower lobes consolidation with an associated effusion. Coronal tomography exam of the chest, abdomen, and pelvis revealed massive hepatosplenomegaly, pancreatic atrophy, diffuse soft tissue oedema, and signs of portal hypertension but no emboli to the lungs. Echocardiography revealed moderate right sided heart failure with tricuspid valve mobile density consistent with vegetation 1.4×0.9 centimetre in size.The patient continued to do better after the thoracentesis, starting her on iron therapy, and continuing intravenous vancomycin. The haematology service was consulted for her pancytopenia who emphasised the likelihood of her hypersplenism besides iron deficiency being the culprits of her pancytopenia and that bone marrow exam seemed unnecessary at that time.

Summary Rothia mucilaginosa continues to emerge as an opportunistic organism of variable virulence in neutropenic patients. The full potential of this organism is yet to be identified. However, this case adds right sided, native valve endocarditis to its potential in the medical literature.

References

  1. . J Clin MicrobiolMay 2013;51(5):1629–1632. doi:10.1128/JCM.03173-12

  2. . J Clin MicrobiolSeptember 2014;52(9):3184–3189. doi:10.1128/JCM.01270-14

269 Not everything that vomits is reflux, an atypical presentation of an atypical infection in a neonate

SR Induru

C Poole

University of Alabama, Birmingham, AL

Case report We present the case of a 1 month old female with three weeks of worsening emesis. This patient was born full-term by vaginal delivery with an uncomplicated pregnancy and delivery. All antenatal testing including Group B streptococcus were negative. Baby developed emesis around a week of life which increased in frequency and forcefulness. She had an ultrasound at 2 weeks old which excluded pyloric stenosis. Parents were given feeding recommendations for reflux. She however became more difficult to feed and her weight gain was suboptimal, falling from the 50th percentile to the 5th percentile while length and head circumference remained in the 75th and 65th percentiles. On the day of presentation to our ER, parents noted some blood in the emesis together with 10 min episode of rhythmic jerking movements of her upper extremities en route to the ER. She had remained afebrile throughout. On arrival to the ER her vital signs were T 98.3 F, heart rate 159 bpm, BP 99/65, respiratory rate 23, but she was noted to have a bulging anterior fontanelle.

Clinical course and management

Given her neurologic findings she had an emergent non-contrasted head CT revealing holoventriculomegaly and a subsequent emergent MRI showing communicating hydrocephalus. The patient emergently had an EVD placed by Neurosurgery with CSF studies revealing profound leukocytosis, elevated protein and low glucose consistent with bacterial meningitis and gram stain positive for gram-positive cocci CSF cultures grew Streptococcus infantarius, a bacterium from the S. Bovis family.

Discussion The pathways of care of infants failed to pick up this patient’s presentation of a serious infection. Although benign reflux is common in infants, failure to thrive would warrant further work-up. Streptococcus infantarius has rarely been described as a cause of neonatal sepsis and to our knowledge this is the first case presenting with hydrocephalus. We report this case to identify not only an atypical pathogen, S. infantarius, but also to highlight the importance of maintaining high clinical suspicion of infection in infants even without presentation of fever.

270 Vancomycin and linear iga bullous dermatosis

A Ismail

RE Gavidia Quezada

K Iwuji

M Tarbox

TTUHSC, Lubbock, TX

Case report A 56-year-old man presented to the hospital complaining of generalised skin rash with blisters. He has a medical history of alcoholic cirrhosis with pancytopenia, chronic pancreatitis and chronic osteomyelitis. Prior to this presentation, he was admitted for osteomyelitis of his left arm. He had a hardware removed from his arm and was discharged 3 days prior to his presentation on intravenous vancomycin, oral ciprofloxacin and metronidazole. Initially, he developed an itchy purpuric rash on his trunk. The rash progressed rapidly to involve his extremities, back, lips, and tongue. Dermatology was consulted and a skin biopsy was done. The biopsy showed sub-epidermal blister with neutrophils and eosinophils. Immunofluorescence showed linear IgA and IgG deposition at the dermal/epidermal junction. Dermatology recommended supportive treatment. He also had acute on chronic thrombocytopenia not responding to transfusion. Haematology was consulted and he was treated for immune thrombocytopenic purpura with one dose of 85 g of Intravenous immunoglobulin and 100 mg of prednisone, followed by 40 mg of prednisone daily over the next 3 days. His platelet count improved to his baseline after 1 unit of platelets. The patient was hospitalised for a week and his skin lesions continued to improve daily. It is unclear how the IVIG and prednisone altered the course of his skin disease. He was re-admitted at our hospital 2 months later and his skin findings had completely resolved.

LABD is a sub-epidermal bullous disease defined by the presence of homogeneous linear deposits of IgA at the dermal-epidermal basement membrane on direct immunofluorescence. Histologically, it is characterised by sub-epidermal bullae with a predominantly neutrophilic infiltrate and basal cell vacuolization. Patients with drug induced LABD have spontaneous resolution of skin lesions within 1 to 3 weeks after removal of offending agent. They will need supportive care including pain control, fluid and electrolyte management, and nutritional support. After the vancomycin is discontinued, the patient should have prompt improvement without residual skin lesions. If patients are re-challenged with vancomycin, they may have a more severe recurrence, including a shorter latency and a longer course.

271 An unusual ‘black eye’ and shortness of breath in a 27 years old male

K Iwuji

D Payne

S Thakolwiboon

Texas Tech University Health Science Centre School of Medicine, Lubbock, TX

Purpose of study To describe a unique presentation of Kaposi Sarcoma in a newly diagnosed HIV/AIDS patient.

Methods used Clinical observation and physical examination.

Summary of results Acute human immunodeficiency virus infection may present with constellation of nonspecific symptoms which can sometimes be missed by clinicians. An estimated 10% to 60% of individuals with new HIV infection will not experience any symptoms1. Some patients diagnosed with HIV infection already have evidence of AIDS defining illnesses (e.g. Kaposi sarcoma, Lymphoma, mycobacterium infection, pneumocystis jirovecii and others) at the time of diagnosis as seen in the images of this patient with disseminated kaposi sarcoma. Despite occasional late presentation at the time of diagnosis, patients with no other commodities who are treated appropriately and are compliant with their antiretroviral medications are expected to have same life expectancy as the general population.

Case A 27-year-old male presented to a hospital with a one month history of black eye and shortness of breath. He had recently been released from jail where he had experienced progressive shortness of breath and fatigue. His black eye had appeared near the same time and patient related it to recent dental work. Examination revealed an oval shaped lesion 3 cm in length below the right eye (Panel A) with multiple similar lesions on the chest back and lower extremities. Intraoral examination revealed violaceous non-blanching plaque along hard and soft pallet (Panel B). Biopsy of skin lesions revealed atypical vascular proliferation consistent with nodular Kaposi’s sarcoma. Computed tomography of the chest showed multifocal airspace disease suspicious for Pneumocystis Jirovecii pneumonia versus Kaposi’s sarcoma. HIV screening returned positive with a CD4 count of 41/MCL. He was started on treatment for Pneumocystis Jirovocii and anti-retroviral therapy. He tolerated therapy well and continues to follow in the outpatient HIV clinic.

Conclusions The diagnosis of acute HIV infection requires a high level of clinical suspicion, detail history and physical examination.

272 A rare case of varicella zoster meningitis

R Kamat

R Helm

E Machen

LS Engel

S Sanne

LSU Health Sciences Centre, New Orleans, LA

Introduction Aseptic meningitis can have various etiologies, although viruses remain the most common cause. Historical clues, seasonality, and regional variation can be helpful in narrowing down the specific viral pathogen.

Case A 54-year-old man with a past medical history of hypertension, type 2 diabetes, cervical stenosis and hyperlipidemia presented to the Emergency department with complaints of headache, nuchal rigidity, and shoulder pain for the past three days. The patient has chronic neck pain from his stenosis, but he felt that this nuchal rigidity differed from his previous pain crises. He also described intermittent subjective fever, chills, and nausea. One week prior to presentation he developed vesicular lesions on the right C4-C5 dermatome distribution on his shoulder, but he believed they were insect bites from being outside while gardening. He denied any previous history of chicken pox, but admits he may have had it as a child. Following a lumbar puncture, he was started on broad spectrum antibiotics and acyclovir to empirically treat bacterial meningitis and HSV encephalitis. He had an unremarkable CT scan of the head. CSF studies showed WBC count of 121 cells/mm3 with 94% lymphocytes, glucose of 90 mg/dL, and protein of 104.2 mg/dL. Gram stain at that time was negative. MRI of brain was obtained to rule out temporal involvement, and based on CSF data antibiotics were discontinued. PCR studied of CSF eventually returned positive for varicella zoster virus, and the patient was discharged on a two-week course of acyclovir.

Discussion In this patient, empiric therapy for both bacterial meningitis and HSV encephalitis was initiated at the time of initial exam after obtaining CSF and blood cultures. After CSF studies showed a relatively low white count with lymphocyte predominance, treatment was de-escalated to acyclovir alone. Careful physical examination, history taking and proper diagnositic testing helped identify a clear cause and treatment course for this patient’s VZV meningitis.

273 Splenic abscess in paediatric patient

CS Keith1

C Trieu2

S Srialluri1

S Boppana1

1Children’s of Alabama, Birmingham, AL

2Mercer School of Medicine, Macon, GA

Introduction Splenic abscesses are uncommon in children with a reported incidence of 0.03%–0.7%. The diagnosis is not frequently considered due to its rarity and lack of specific clinical findings. The most common findings include fever, splenomegaly and left upper quadrant pain.

Case presentation A 14 yo previously healthy male presented to an outside hospital (OSH) with 10 days of fever and abdominal pain. A CT scan showed a complex splenic laceration with large hematoma and possible abscess. The patient denied trauma. He was admitted and started on pipercillin-tazobactam prior to transfer to our institution.

On physical exam he had splenomegaly with tenderness in the left upper quadrant. He had a WBC of 21.74 with 84% neutrophils and an elevated CRP (21.02 mg/dl). Paediatric radiology read his abdominal CT as a 16 cm ×14 cm × 16 cm fluid collection in the spleen. Ultrasound showed a large complex cystic mass.

Patient underwent a laproscopic-assisted drainage of the abscess. Cultures grew methicillin susceptible staphylococcus aureus. He was treated with nafcillin but the abscess persisted requiring 3 additional drainage procedures. He ultimately required splenectomy.

Immunology work up revealed an abnormal neutrophil respiratory burst assay concerning for autosomal recessive chronic granulomatous disease. Genetic evaluation is pending.

Discussion Splenic abscesses are uncommon in paediatrics. Predisposing factors include metastatic or contiguous infection, haematological disorders, trauma or immunodeficiency. A retrospective review of data at our institution from 04/2007–03/2017 showed three other cases of splenic abscess in which contiguous infection was the cause. The clinical features of splenic abscesses include fever, localised or diffuse abdominal pain and splenomegaly. Ultrasound shows hypoechoic or anechoic lesions in the spleen and additional imaging such as CT or MRI may be needed. The most common organisms are gram-positive cocci. Splenectomy is usually required but conservative drainage procedures can be effective.

Conclusion Splenic abscesses are incredibly rare in paediatrics. It is important to investigate for predisposing factors, especially immunodeficiency, if no other source is found. Although splenectomy may be required, it is prudent to try percutaneous drainage first.

274 Respiratory failure due to persistent thoracic fungal empyema

M Khalid

ES Josan

A Mahajan

R Akella

W Shams

East Tennessee State University, Johnson City, TN

Case report 48-year-old male with poorly controlled insulin dependent diabetes mellitus was admitted with septic shock due to pneumonia. He was intubated for acute respiratory failure. Respiratory cultures obtained by bronchoscopy grew Candida dublinesis and Escherichia coli, which were treated with Micafungin and Ceftriaxone. A thoracentesis revealed an exudative pleural effusion with lactate dehydrogenase >1800 U and culture grew the previous organisms as well. A pig tail catheter was inserted and alteplase was administered due to persistence of emphysematous pleural effusion on follow up imaging which led to adequate drainage. The patient failed weaning trials on multiple occasions due to excessive respiratory secretions with persistent lung pathology. He underwent right thoracoscopic lung de-cortication and eventually tracheostomy placement for prolonged weaning.

Fungal Empyema Thoracis is rare most common nosocomial acquired life threatening infection with high mortality up to 60%–73%. Candida species are the most common pathogens, followed by Aspergillus species. The incidence of Candida pneumonia in mechanical ventilated patients is much higher than non-ventilated patients. Candida Albicans is the most frequently encountered infections, especially after abdominal or thoracic procedures, gastroesophageal fistula. Treatment includes systemic antifungal, chest tube drainage, decortication, and salvage therapy with micafungin in refractory empyema.

Abstract 274 Figure 1

Right sided pleural effusion with air-fluid level (blue arrow)

275 Interesting case of leptospirosis presented as weil’s disease

M Khalid

I Khan

D Hidalgo

L Ibrahim

J Moorman

East Tennessee State University, Johnson City, TN

Case report 26-year-old healthy male admitted with compliant of generalised non pruritic rash associated with high grade fever, chills, headache, nausea, vomiting, myalgias, dark urine and discoloration of eyes and skin. Denied sick contacts, tick bites. Patient reported exposed to mosquitoes. Physical exam showed jaundice, pallor and faint diffuse macular rash that spared palms and soles. Labs showed anaemia, thrombocytopenia, high bilirubin and renal failure with creatinine of 1.41 mg/dl. Blood cultures were negative. Peripheral smear showed moderate thrombocytopenia with no schistiocytes. Detailed work up for infectious causes include HIV, viral hepatitis panel, EBV, CMV, monospot test, Dengue test, chikungunya, Ehrlichia, Anaplasmosis, RMSF which were negative. Computed Tomography abdomen and pelvis showed hepatosplenomegaly (figure 1). Leptospirosis serology was sent to a laboratory which came back positive and patient was started on doxycycline with improvement in the symptoms.

Leptospirosis is zonosis transmitted by domestic animals or contact with water, soil and food contaminated with urine of infected animal. Weil’s disease is a severe form of presentation characterised by kidney, liver dysfunction and Jaundice with a high mortality rate. This case highlights the importance of recognition of leptospirosis in patient with rash with liver and kidney dysfunction.

Abstract 275 Figure 1

CT scan showed hepatosplenomegaly (blue arrows)

276 Candiduria due to candida krusei in a patient with prolonged icu stay: can we treat this resistant infection with echicandins?

V Kohli

P Sankhyan

L Ibrahim

East Tennessee State University, Johnson City, TN

Introduction Candiduria is common in high risk patients in the hospital. A recent trend of non albicans candiduria is being reported and among those Candida krusei is a rare species to be isolated. A very small percentage of non albicans candiduria is blamed on this species, known to be inherently resistant to triazoles. This report aims at finding out if micafungin is effective to clear UTI caused by Candida krusei which does not have a good urine concentration, in a setting of azole resistant candida infection.

Case report A 47 year old male who was admitted to the ICU with multiple comorbidities and stayed for 23 days was shifted to the ICU step down as he was improving, with a foleys catheter in place. After two days in the step down,patient started spiking fevers of 102F and with leukocytosis (WBC 17.2 K/uL) with unknown origin of foci. He was not on any antibiotics at the time. Piperacillin-tazobactam and daptomycin were considered to treat the patient empirically. His urine and blood was sent for cultures. The blood cultures were negative but his urine culture was positive for Candida krusei (CFU>105/ mL). The sensitivity test showed the organism to be resistant to tri azoles (eg. fluconazole and itraconazole). The echinocandins does not have good urine concentration but we did not have more options so we tried to treat the patient with micafungin. Repeat culture was positive on the day we started the patient on micafungin again with Candida krusei (>105 CFU/mL). After 8 days of initiation of the treatment, we sent in another urine sample for culture. The results came back with no growth of Candida krusei. The patient did not have any signs of infection and the leukocytosis resolved.

Conclusion The patient required a minimum of 1 week of micafungin until negative cultures and was continued to complete the course for 14 days. We were able to treat the UTI with micafungin completely and thus we believe echinocandin is a good option in candiduria with candida resistant to tri azoles. More studies are needed to confirm this finding as the number of cases seen with this infections are very limited.

277 The mitis touch

D LaChance

Dwight David Eisenhower Army Medical Centre, Augusta, GA

Case report 58 year-old male with essential hypertension presented to the ED after development of acute chest pain. Pain was sharp, constant, fluctuating in intensity, exacerbated by swallowing and deep inspiration, and radiated to his neck. Ibuprofen did not relieve his symptoms prior to presentation. Patient also noted a recent URI which was self-limited. Further ROS was also positive for odynophagia. On presentation, his vital signs were stable and he was afebrile. Cardiac biomarkers were negative; however labs were notable for a leukocytosis. Chest xray was unremarkable. Electrocardiogram was normal sinus rhythm and demonstrated no changes consistent with ischemia. Sublingual nitroglycerin and full dose aspirin in the emergency department did not relieve his symptoms. Given concern for acute aortic dissection, a D-Dimer was obtained which returned positive. A CT pulmonary angiogram was obtained which revealed a soft tissue density mass within the superior mediastinum adjacent to the oesophagus with adjacent free air. Concern for esophageal neoplasm versus esophageal perforation arose. An esophogram with gastrografin ruled out esophageal perforation. Subsequent EGD with esophageal ultrasound revealed a lesion consistent with an infected bronchogenic cyst anterior to the oesophagus was identified with clear delineation between the lesion and the oesophagus. Cardiothoracic surgery performed a video assisted thorocoscopy for incision and drainage of the abscess. Purulent fluid was aspirated and sent for gram stain and culture and antibiotic therapy with vancomycin and piperacillin-tazobactam were started. Culture data revealed pan-susceptible Streptococcus mitis. The patient was started on course of amoxicillin clavulanate with full resolution of infection and chest pain syndrome.

Bronchogenic cysts are an uncommon congenital anomaly of the foregut which can lead to tracheobronchial compression and infection, with a prevalence of 1/42,000–68 000. They predominantly develop in the lung parenchyma, however about 24% develop in the mediastinum, as in the case. They are mostly discovered as incidental radiologic findings. They can become symptomatic with chest pain being the most common presenting complaint. This represents an unusual aetiology of chest pain due to bronchogenic cyst infection in an immune-competent individual.

278 An atypical presentation of a typical mycobacterium

T Liddell

J Lucar Lloveras

R Webb

University of Mississippi Medical Centre, Brandon, MS

Case report Mycobacterium tuberculosis is a common pathogen which infects one third of the world’s population. Often clinical suspicion is high when patients present with typical symptoms of pulmonary tuberculosis, however, tuberculosis is often left out of the differential when it presents atypically.

We present a 65 year-old Vietnamese female living in the US for two years with no known past medical history who presented with left ankle pain for six months. She had been treated for gout though foot pain continued to progress. On admission, ankle was inflamed, and imaging revealed destruction of the talus and an enlarged left inguinal lymph node. She underwent resection of her left talar head and neck and left inguinal lymph node. Infectious Diseases was consulted due to osteomyelitis. Patient then reported symptoms of fever, night sweats, and weight loss of 20–25 lbs that began around the development of her left ankle pain. Laboratory studies revealed mild leukocytosis, thrombocytosis, anaemia, and elevated inflammatory markers. She was also found to have undergone biopsy of her talus prior to her arrival at our hospital. Slides were obtained and reviewed with our pathologist who reported granulomatous inflammation with some granulomas displaying central necrosis. Chest imaging was unremarkable. She was started on active tuberculosis treatment with rifampin, isoniazid, pyrazinamide, and ethambutol. Pathology of talus and lymph node were consistent with biopsy, and cultures eventually grew Mycobacterium tuberculosis in six of six samples.

Of the 8.7 million cases of tuberculosis reported each year, musculoskeletal tuberculosis accounts for 1%–5% or 87 000 to 4 35 000 cases each year. Osteoarticular tuberculosis, particularly outside the spine, requires a high level of suspicion for prompt diagnosis. Our case has multiple characteristics that should cause consideration for tuberculosis including visiting from an endemic area, negative bacterial cultures, biopsy with caseating granulomas, and imaging with irregular cavities of destruction with little surrounding sclerosis. Despite a few significant diagnostic clues, our patient’s symptoms were present for six months before she received appropriate treatment underlining the importance of maintaining a high suspicion for tuberculosis despite an atypical presentation.

279 Visual deficits as a late manifestation of congenital toxoplasmosis: familiar sign in an unfamiliar setting

WJ Lindsey1

V Habet1

S Lefevre2

1Children’s Hospital, New Orleans, LA

2LSU Health, New Orleans, LA

Purpose of study While Toxoplasma gondii is generally asymptomatic in immunocompetent hosts, severe disease can manifest in congenital infection or immunocompromised hosts. 70%–90% infants acquiring Toxoplasma infection transplacentally may be asymptomatic initially. It’s persistence can end in reactivation leading to clinical disease. The most common late manifestation is retinochoroiditis. Approximately 90% of untreated children will acquire retinal lesions per year. The prevalence of ocular toxoplasmosis in the United States (US) ranges from 0.6% to 2%. Infection rates are higher in regions that are at lower altitudes with a tropical climate with high prevalence in South America. We discuss a case of congenital toxoplasmosis in an immigrant with singular, anterior cervical lymphadenopathy and worsening vision.

Methods used Case Study.

Summary of results A 5 yo male with developmental delay presented to general paediatric clinic for routine well-child check. The patient had recently emigrated from Brazil with his family and had not been examined by a physician in over one year. The parents’ primary concerns were assessing a ‘lump’ on the patient’s neck, receiving necessary immunizations to attend school and obtaining visual examination. Physical exam revealed a 5 cm nontender, mobile rubbery mass of the anterior cervical chain which had been present for over a year. Visual acuity and ocular alignment tests were attempted, but the patient was unable to identify characters at 10 feet. The patient was referred to ophthalmology but was evaluated by an optometrist due to lack of insurance. Retinal images revealed bilateral macular and peripapular scarring consistent with congenital toxoplasmosis. The patient was referred to ID who recommended close follow up without antimicrobial treatment but has since been lost to follow-up.

Conclusions In the US, prenatal testing for toxoplasmosis is not done. This contrasts other countries with higher prevalence of disease where prenatal testing is of significant value. Because toxoplasmosis can be asymptomatic paediatricians must have a high index of suspicion in children presenting with visual disturbance. This is particularly important for immigrants. Visual disturbances in this population should have a broader differential diagnosis including congenital toxoplasmosis.

280 Dress syndrome in a patient with ards

R Mangat

Z Jones

A Eranki

SUNY Upstate Medical University, Syracuse, NY

Case report Drug reaction with eosinophilia and systemic symptoms (DRESS) syndrome is a relatively rare drug hypersensitivity reaction that can present with organ dysfunction and skin symptoms two to six weeks after starting a new drug. In this case, a middle-aged male was diagnosed with DRESS syndrome when he developed fever, elevated transaminases and worsening eosinophilia after being restarted on antibiotic therapy.

A 53-year-old male with a past medical history significant for Becker’s muscular dystrophy presented to the ICU with acute respiratory failure secondary to ARDS in the setting of community acquired pneumonia and septic shock. His oxygenation improved after an initial 7 days treatment with vancomycin, zosyn and azithromycin, but he remained febrile with worsening leukocytosis. Chest x-ray showed bilateral infiltrates and he was restarted on vancomycin and zosyn for recurrent pneumonia. He developed rash and was found to have a white count of 26 000 with 61% eosinophils, ALT of 133 and AST of 122. After the antibiotics were discontinued, the patient’s clinical syndrome improved in 2 days.

DRESS syndrome is an important and under-recognised differential diagnosis to consider in patients with fever, unexplained leukocytosis and rash. In this case, the patient was restarted on the offending agents due to concern for infection several days prior to a diagnosis being made. Skin eruptions typically involve greater than fifty percent of the body surface area including facial oedema, infiltrative lesions, scaling and purpura. Abnormal laboratory values that can help diagnose DRESS include leukocytosis with an eosinophil count >700, atypical lymphocytosis, increased transaminases and reactivation of human herpesvirus-6. The most common offending drugs include antiepileptics, sulfonamides, allopurinol with beta lactams being increasingly reported. Removal of the offending agent along with supportive care is the mainstay of treatment in these patients. Prompt diagnosis of DRESS syndrome is clinically important as patient deaths have been reported due to multi-organ system failure in unrecognised cases.

281 A case of aerococcus and globicatella bacteremia

D Markabawi

R Mangat

Z Jones

H Singh Gambhir

SUNY Upstate, Syracuse, NY

Introduction Globicatella sanguinis are catalase-negative, gram-positive cocci that were first discovered in 1992. There have been multiple case reports of Globicatella sanguinis isolated from blood, urine and cerebrospinal fluid. The clinical significance of this bacteria is still unknown. Aerococcus viridans, is a very rare microorganism, which causes invasive infections in immunocompromised patients, but rarely in immunocompetent patients. It has been associated with bacteremia, septic arthritis, and especially infective endocarditis.

Case A 72 year old male with a past medical history of CVA, hypertension, and dementia, was brought to the emergency department for complaints of increased fatigue and lethargy. The patient was constipated with the last bowel movement being several days prior to presentation. In the ED, the patient was noted to be afebrile and hemodynamically stable. A CT abdome/pelvis was positive for stercoral colitis. The patient underwent manual dis-impaction. Patient was also noted to be dehydrated with an elevated lactic acid, this improved with IV hydration. Two sets of blood cultures were obtained in the emergency department. One out of the two sets grew Globicatella sanguinis and Aerococcus viridans. Upon consultation with the infectious disease service, it was decided to treat patient and not deem this a skin contaminant as this bacteremia might have been a result of bacterial intestinal translocation due to colitis and faecal dis-impaction. Both organisms were susceptible to Penicillin and third generation Cephalosporins. Patient was successfully treated with Ceftriaxone 2 g IV daily for 2 weeks.

Discussion While Aerococcus viridans is known to cause invasive infections in humans, the pathogenic role of Globicatella sanguins remains partially known. Human carriage of both species has been established. There have been reports of infective endocarditis due to Aerococcus viridans and meningitis due to Globicatella sanguins. In our case, the bacteremia likely resulted from bacterial intestinal translocation. Both species were susceptible to third generation Cephalosporins.

Conclusion Although human carriage for both Aeroccous viridans and Globicatella sanguins is well known, it is important to consider the pathogenic role of these bacteria in the right setting.

282 Back pain

BE O’Brien1

M Bahn1

R Steele1,2

1Tulane University, New Orleans, LA

2Ochsner Medical Centre, New Orleans, LA

Case report 11 yo caucasian male presented w/fracture of L4. He was in usual state of good health until 3 months prior when he developed fever, myalgia, HA, and left cervical LAD. He was dx’d w/flu, treated with Oseltamivir/Azithromycin. His brother was flu positive 2 weeks prior.

2 weeks s/p tx for flu- he developed acute, intermittent left-sided flank pain went to an ED, CTAP was normal. Patient had a kitten, Bartonella titers were drawn, and showed B. henselae IgG ≥1:1024 (normal <1:128) and IgM ≥1:20 (normal <1:20).

He was dc’d home on Azithromycin/toradol. He continued to have fatigue, decreased appetite. 10 days later- went to urgent care for flank pain, was treated empirically for UTI w/Augmentin. Labs (cbc, cmp, UA) were normal. S/P treatment, he went to ED for continued flank pain, RUS was normal. 2 weeks later- he went to his paediatrician w/fatigue, decreased appetite. Xrays of spine obtained for concern of MSK aetiology of pain. He was dx’d with scoliosis and referred to Ortho. Flank pain resolved.1 month prior to presentation, he heard a ‘popping sound’ and had sudden, severe pain. Was seen by Ortho the next day. MRI of spine showed pathological fracture of L4. He was referred to Hem-Onc to r/o malignancy.

His CBC was normal except for elevated eos (7.4%) on diff, CRP (7.32 mg/L). Otherwise labs (CMP, uric acid, ESR) were normal. Quant. TB Gold was negative. Repeat MRI of CTL spine obtained showed heterogeneously enhancing lesion measuring 2.7 cm within left central portion of L4 vertebral body with mild height loss and associated fracture.

PE:+small palpable lymph nodes in his anterior cervical triangle/inguinal regions. No bone pain, restriction in mobility or ROM, or neurologic deficits.

Biopsy of lesion was sent for gram stain, aerobic/anaerobic culture/AFB/fungal culture (all negative). Path showed necrotizing granulomatous inflammation in bone and bone marrow, no neoplasm. Biopsy sent to Mayo Clinic for Bartonella PCR, which was negative.

1 month later,labs were normal including crp.No hx of recurrent infections, but due to severity of presentation, concern for CGD. Neutrophil oxidative burst assay was negative.Patient has returned to baseline activity level, now has nightly lower back pain and was fitted for a brace.

283 An unusual presentation of disseminated varicella zoster in an immunocompetent adult

P Pachariyanon

S Thakolwiboon

D Payne

Texas Tech University Health Science Centre, Lubbock, TX

Case report A 63-year-old man with a history of bladder cancer status post transurethral resection of bladder tumour presented with 5 days of fever, progressive diffuse headache, and photophobia. Initial physical examination revealed multiple discrete erythematous papules on his left post auricular area, trunk and both thighs. A neurological examination revealed neck stiffness without any focal neurological deficit. A computerised tomography scan did not demonstrate any intracranial pathology and cerebral spinal fluid analysis showed a mild lymphocyte-predominated pleocytosis. He was initially empirically treated by intravenous vancomycin, ceftriaxone, ampicillin, and acyclovir. On the third day of admission, he developed left facial droop and hearing difficulty. A repeat physical examination revealed multiple groups of vesicles in the left C2 and C3 dermatome and diffuse erythematous vesiculopapular rash on his trunk and both thighs and peripheral left facial palsy. Weber’s and Rinne’s tests suggested left sensorineural hearing loss (SNHL) which was confirmed by audiogram. Magnetic resonance imaging was done and did not reveal any structural lesion. Blood and CSF cultures did not grow any microorganism. CSF PCR for HSV-1, HSV-2, and enterovirus were negative as well as HIV and Syphilis screening. Subsequent immunological study revealed positive serum IgG for HSV-1, VZV, and CMV while negative for HSV-2. However, serum IgM was positive for VZV. Additionally, lymphocyte subset panel did not suggest any immunodeficiency disorder. All anti-bacterial medications were discontinued. Intravenous acyclovir 10 mg/kg was continued for total 14 days. At 1 month follow up, the skin lesions were crusted over and had begun to recede. However, left facial paralysis and remained.

Herein, we described the unusual presentation of disseminated varicella zoster which included both chickenpox-like rash and typical zoster skin lesion as well as neurological involvement in an immunocompetent host.

284 Eating your way to meningitis: why maternal placentophagy might be a bad idea

C Parrish

R Welliver

University of Oklahoma College of Medince, Oklahoma City, OK

Case report Our patient is a previously healthy unvaccinated 15 day old female who presented with decreased oral intake (PO) and fever for 1 week. Patient was a full term infant born at home. Maternal laboratory investigations including group B streptococcus (GBS) were negative. Birth was uncomplicated and patient was healthy until day 7 of life, when she began having decreased PO and fevers. On exam she was alert with no focal neurological deficits. A septic workup showed cerebral spinal fluid (CSF) cultures positive for GBS; blood cultures were negative. A 14 day course of IV ampicillin was started. Initial MRI showed purulent material within subarachnoid spaces, and antibiotic course was extended to 21 days. MRI prior to discharge showed stable purulence. She was discharged home after completing full antibiotic course. At her 6 week follow up, she had age-appropriate development. Of note, mom had sent her placenta to an outside facility to be processed into supplemental pills. Mom began taking these two days prior to admission, and was advised to stop. Our laboratory recommended testing the pills for GBS be completed by the Health Department, however at this time we are unsure whether the family did this.

Late onset meningitis in neonates is often associated with horizontal transmission, one such transmission that we may not consider is maternal ingestion of the placenta. Placental ingestion has been promoted for its potential physical and psychological benefits, although there is no scientific evidence to support this. In a previously reported case in Oregon the mother of an infant with recurrent GBS meningitis had ingested her placenta in the form of pills that were tested and found to be GBS-infected; the final diagnosis: ‘late onset GBS disease attributable to high maternal colonisation secondary to consumption of GBS-infected placental tissue’.

These cases show us the importance of expanding our clinical questions and differential diagnoses as the culture we practice medicine in also expands. There is little research into the number of women who choose to ingest their placentas, and even less scientific research on the risks of doing so. These cases illustrate the need for further investigation and insight into the risks of placental ingestion as a means to better educate families.

285 Severe and progressive cellulitis following a dog scratch in a neutropenic patient

D Pithadia1

E Weathers1

R Colombo1

S Baer1,2

1Medical College of Georgia, Augusta, GA

2Augusta VA Medical Centre, Augusta, GA

Introduction Skin and soft tissue infections occur in over 20% of patients with chemotherapy-induced neutropenia. Gram positive bacterial infections predominate early in neutropenia, and incidence of resistant bacteria and fungi increases with prolonged neutropenia. Past infections and exposures influence the risk of of rare pathogens.

Case A 55-year-old woman, who was neutropenic from chemotherapy for primary CNS lymphoma, was scratched on her forearm by a dog. She cleaned the wound with isopropanol and was treated empirically as an outpatient with amoxicillin-clavulanate. Over the next 4 days, she developed fever plus erythema and swelling of the forearm without purulence, crepitus, or significant pain. The wound had contacted tap water, and she denied other exposures. She was admitted and started on intravenous vancomycin, piperacillin-tazobactam, and tobramycin. Cultures of blood and urine grew Pseudomonas aeruginosa with identical susceptibilities. Vancomycin and piperacillin-tazobactam were continued, and all subsequent cultures were negative. However, daily fevers persisted and the inflammation in her arm progressed resulting in restricted flexion of her elbow and digits. MRI of the arm showed myositis and an elbow joint effusion. Voriconazole was added for empiric fungal coverage. A bulla developed at the wound site as her neutrophils recovered, and culture of the fluid grew Serratia marcescens. Antibiotics were switched to cefepime based on susceptibility. She became afebrile with substantial improvement of cellulitis within 48 hours and was discharged on oral ciprofloxacin to complete a 14 day course.

Conclusion Serratia marcescens is a gram-negative bacillus that thrives in damp environments and causes opportunistic nosocomial infections. Serratia skin infections are rare and, based on our review, this may be the first report of Serratia cellulitis associated with dog contact and trauma. This case highlights the need to consider unusual pathogens based on exposure history in cases of treatment-resistant soft tissue infections in immunocompromised patients. It also emphasises the importance of obtaining cultures from skin lesions to establish a microbiologic diagnosis for targeted therapy.

286 Double trouble and a delay

JP Pruitt

B Johnson

L Gilliam

R Smalligan

UAB-Huntsville, Huntsville, AL

Case report A 20 yo woman presented with 12 hours of progressive abd pain, lightheadedness, dyspnea and chills 5 days after C-section performed at 35 weeks for preterm labour with breech presentation. On exam vitals were T 103.3, BP 87/44, HR 175, RR 18, O2 sats 100% on RA. She appeared ill and had diffuse abd pain from the umbilicus to her Pfannenstiel incision though it was clean and dry. Labs: WBC 15.4 k, Hgb 11, CRP 15, and lactic acid 1.0 (<1 nl). Abd CT showed some subq fluid and scant air, c/w post-op changes. She received 2 L of IVF but no antibiotics initially apparently due to the low lactic acid level and was sent to the OB ED. Hours after presentation, antibiotics were started with further IV hydration. She improved initially, but by morning the pain had worsened and lactate had risen to 3.4. At this point her abd pain was out of proportion with exam and the incision had become erythematous. Surgery was consulted and she underwent emergency I and D of what was to be confirmed by pathology as necrotizing fasciitis. Wound cultures grew MRSA. She required repeated debridement and an eventual abd wall mesh but recovered and went home in 2 weeks.

Discussion This patient had two related life-threatening conditions. First was sepsis which must be addressed aggressively with early IV fluids and broad spectrum antibiotics. Treatment was delayed initially because of a normal lactic acid. Sepsis is a clinical diagnosis based on the vitals, exam, lab, and imaging findings as opposed to meeting a long list of diagnostic criteria. While an elevated lactate is associated with a poorer prognosis in sepsis, it is not a diagnostic marker.

Necrotizing fasciitis is also a medical emergency which requires a high clinical suspicion as the dx is not always obvious. In our case the physical exam was initially misleading due to the infection being deeper in the tissues, thus the erythema, greyness and bullae often seen in the condition were not present. Typical CT findings of necrotizing fasciitis are subq inflammation and air which can also be seen post-op. Close f/u of this patient with early surgery when she deteriorated were keys to prompt dx and treatment of her second life-threatening condition. It is important to remember that pain may precede skin changes and surgical debridement is the only definitive treatment.

287 Travel-related leishmaniasis and tumour necrosis factor inhibitors: 2 cases of misdiagnosis

E Rabold

RJ Feldman

J Lutgring

A Moanna

H Wu

Emory University, Atlanta, GA

Case report An association between leishmaniasis and the use of tumour necrosis factor (TNF) inhibitors has been suggested by previous reports, and immunosuppression is a known risk for severe disease. Though leishmaniasis is rarely transmitted in the U.S., travellers are at risk of infection when visiting endemic areas. We present two patients with cutaneous leishmaniasis complicated by initial misdiagnoses and TNF inhibitor therapy. Patient A is a 43-year-old healthy American male who presented with a chronic, non-healing ulcer on his right leg and a smaller lesion on his right arm after travel to Mexico. Histopathology of the leg ulcer was inconclusive, and a presumptive diagnosis of pyoderma gangrenosum was made. Due to non-response to topical treatments, he received various immunosuppressive regimens, including oral glucocorticoids, systemic cyclosporine, and adalimumab. A subsequent biopsy of the arm lesion demonstrated parasitized histiocytes, and PCR testing was consistent with Leishmania mexicana. After cessation of all immunosuppressive medications and initiation of oral ketoconazole, both lesions resolved. Patient B is a 59-year-old American male with psoriasis treated with adalimumab who presented with non-healing ulcers on his right chest and right thigh after travel to southern Spain. Squamous cell carcinoma was diagnosed from a biopsy of the thigh lesion, and the patient received MOHS surgery. A subsequent biopsy of the chest lesion demonstrated amastigotes in the dermal layer, which was also seen on reevaluation of the first biopsy. PCR testing was consistent with Leishmania infantum/chagasi. His chest lesion resolved with liposomal amphotericin B and cessation of the adalimumab. In these cases, the delayed diagnosis of leishmaniasis resulted in unnecessary or prolonged exposure to immunosuppressive drugs, including TNF inhibitors. Physicians should consider the possibility of leishmaniasis when starting TNF inhibitors in patients with non-healing wounds, particularly if they have travelled to endemic areas.

288 Acquired tracheoesophageal fistula as a complication of acquired immunodeficiency syndrome (aids)

A Richardson

KR Green

J Perez-Downes

CM Baldeo

SF Shah

N Najjar

V Seeram

University of Florida College of Medicine Jacksonville, Jacksonville, FL

Case report A 52 year old male with past medical history of HIV/AIDS (CD4 count of 5) presented to the emergency department with a complaint of weakness, fatigue, and dysphagia. He had been non-compliant with antiretroviral therapy for 3 previous years. Two days into his admission he became septic thus chest X-ray was ordered revealing a pneumonia. Given no clinical improvement with antibiotics, a CT chest was ordered. The CT scan revealed a communication between the oesophagus and trachea with gas in the mediastinum. Given severe cachexia, no surgical intervention was recommended. A gastrojejunal feeding tube was inserted, intravenous broad spectrum antibiotics and antifungals started, and serial esophagrams were ordered to evaluate healing and closure of the tracheoesophageal fistula. Fistula formation was attributed to candida esophagitis given oral candida was present on admission. Patient was discharged to follow up for initiation of antiretroviral therapy

Acquired tracheoesophageal fistulas (TEF), although uncommon, usually occur as a complication of mechanical ventilation, trauma, and malignancy. However, the immunocompromised can acquire TEFs secondary to complications from opportunistic infections. Although TEFs were more common early in the AIDS epidemic, acquired TEFs are rare today given advances in antiretroviral therapy. Causative infectious agents of acquired TEFs include Mycobacterium, Candida species, Cytomegalovirus, and herpes simplex virus. If left untreated, progression to perforation, necrosis, or TEF formation can occur.

TEFs are a serious condition with significant morbidity and mortality. Treatment is necessary to avoid aspiration, sepsis, and pulmonary failure. Surgical repair is the preferred treatment and typically involves esophageal closure with resection of the tracheal fragment. Tracheal or esophageal stents are other treatment options but considered a palliative measure. TEFs must be considered in severely immunocompromised individuals given their life threatening prognosis.

289 Kaposi sarcoma presenting as dysphagia

J Ruiz

M Ganji

R Jacob

UF Health Jacksonville, Jacksonville, FL

Case report A 25 year old African American male with past medical history of HIV and neurosyphilis who presented to the hospital for dysphagia associated with facial and throat swelling. Physical exam was notable for significant submandibular and supraclavicular lymphadenopathy with concern for possible Castleman’s syndrome. During mouth evaluation a 3 centimetre hyper vascularized violaceous submucosal lesion below the palate was noticeable. CD4 absolute count on admission was 607. Patient was empirically started on fluconazole for suspected candida esophagitis and gastroenterology was consulted for upper endoscopy which revealed multiple large hyper vascularized violaceous submucosal nodular lesions seen on the soft palate and pharynx. Surgery was also consulted for suspicious soft palate lesion as well for excisional lymph node biopsy. Histopathology of lymph node biopsy was negative for Castleman’s disease but notable for spindle cells consistent with Kaposi’s sarcoma along with positive CD31 and HHV-8 stain. Dysphagia got progressively worse in a matter of hours. He eventually was transferred to the intensive care unit due to increasing respiratory distress. During the intubation, the patient desaturated, became bradycardic,and experienced cardiac arrest. Unfortunately, the patient expired after multiple rounds of resuscitative efforts with no evidence of cardiac activity on ultrasound.

Discussion Kaposi Sarcoma is angioproliferative disorder that requires infection with human herpes virus-8. Skin lesions are the most common manifestations of Kaposi sarcoma. It can also present in other organs as lymph nodes, oral mucosa and the gastroenterology tract. Usually presented in immunocompromised patients. Patient atypical presentation and findings on oral mucosa and upper endoscopy made this case interesting. Kaposi sarcoma usually is diagnosed by biopsy. Treatment can be either chemotherapy or radiotherapy along with antiretroviral therapy.

Conclusion Kaposi sarcoma should always be kept as a possible differential diagnosis on patients with past medical history of HIV/AIDS despite decreased prevalence due to evolution in medical therapy. This case can lead to further evaluation of dysphagia by upper endoscopy on patients that present with dysphagia and oral lesions since only handful of cases of Kaposi sarcoma are seen now a days.

290 Paradoxical response to antituberculous therapy in an hiv negative tb patient

N Salagundla

S Siddiqui

A Mirza

A Islam

Texas Tech University HSC Amarillo, Amarillo, TX

Case report A 50 yo F immigrant from Sudan with PMH, presented with symptoms of generalised weakness, nausea, vomiting and chills for 2–3 months. On P/E she appeared cachectic with bilateral diffuse crackles. CT chest: diffuse bilateral nodular infiltrates with cavitary changes in left upper lobe and right pleural effusion. Further CT evaluation showed: diffuse hypodensities in liver, spleen, kidney and adnexa, left parietal lobe ring enhancing lesion. IGRA was positive, HIV was negative. Sputum and pleural fluid AFB were negative and CT guided lung biopsy showed positive for AFB. Patient was started on four drug antituberculous therapy (ATT) and started improving. She was discharged after 2 days and continued with directly observed ATT. After one month, patient was readmitted for worsening symptoms and WBC count of 3500. CT chest: extensive bilateral, predominantly upper lobe increased cavitary lung lesions. MRI brain: enhancing lesions in right cerebellar and bilateral parietal regions. Drug sensitivity testing of previous specimens showed pan-sensitivity to standard ATT. Therefore, paradoxical response to treatment was diagnosed. ATT treatment was continued with the addition of a quinolone and steroids, but eventually the patient required occipital craniotomy for right cerebellar mass resection. Patient improved on full ATT and steroids over a one year period.

Discussion IRIS (Immune Reconstitution Inflammatory Syndrome) is a widely-recognised cause of paradoxical response in HIV positive patients, but PR can be seen both HIV + and HIV- patients, most commonly extrapulmonary infections associated with lower lymphocyte counts at baseline. Respiratory and CNS TB are most commonly involved. Diagnosis is by exclusion. Exact mechanism is uncertain but immune restitution may play a role even in HIV negative patients. In TB, an imbalanced TH2 to TH1 ratio can impair type IV hypersensitivity reaction and can lead to immunosuppression and ATT can stimulate previously suppressed immune response as a proposed mechanism. Activation and accumulation of lymphocytes and macrophages at the site of bacterial destruction can produce pro-inflammatory substances causing paradoxical response. Steroids are added with continued ATT, with the intent of decreasing the hyper-inflammatory response.

291 Bugs in my head: a case of intraventricular neurocysticercosis causing acute hydrocephalus

N Salagundla

S Siddiqui

A Chandralekha

A Islam

Texas Tech University HSC Amarillo, Amarillo, TX

Case report A 50 YO male with no known PMH presented with C/C of headache which started in the morning, lasted for more than 12 hours gradually became confused with nausea and vomitings. He often had headache in the past which usually resolve with Ibuprofen. Patient came to USA 4 years ago, grew up in Mexico, and denied smoking, alcohol or drug history. P/E and Vitals WNL except for confusion. Initial labs were normal. MRI has shown nonspecific foci of calcifications in the parenchymal and third ventricle which consisted proteinaceous material, Cyst in 3rd ventricle obstructing drainage of CSF causing hydrocephalus. Serological studies were positive for T. Solium antibody. Neurocysticercosis was highly suspicious based on symptoms, imaging and social history. Neurosurgeon immediately performed ventriculostomy with shunt to relieve increased ICP pressure. Initially patient responded well with improvement in mentation. Infectious disease specialist started antiparasitic therapy with Albendazole and Dexamethasone. As 3 days progressed, patient had persistent headache and developed diplopia. Repeat CT head showed increased dilation of 3 rd ventricle. Patient was transferred to tertiary care for further management, underwent uncomplicated endoscopic removal of intraventricular cyst and subsequently discharged home in stable condition. On follow up, patient has recovered well.

Discussion Cysticercosis is a neglected parasitic infection caused by the larval form of the pork tapeworm Taenia solium. Neurocysticercosis is the most serious clinical manifestation of cysticercosis, leading cause of acquired epilepsy in disease-endemic counties. Intraventricular neurocysticercosis commonly presents with acute obstructive hydrocephalus secondary to either cyst entrapment in foramina or ependymitis. Headache is the most common presentation along with symptoms like nausea, vomiting, decreased visual acuity, altered mental mental status and CN palsies. The diagnosis is based on imaging with MRI, but serological tests are important supplementary diagnostic tools. Treatment is mainly surgical, preferably using a neuroendoscopic technique, but patients should receive antihelmintic treatment with corticosteroids to reduce incidence of shunt failure and to treat undiagnosed viable lesions elsewhere.

292 Severe pan-sinusitis in an aplastic anaemia patient successfully treated with multi-agent therapy

R Samannan

Z Yu

R Welliver

D Crawford

C Lawrence

J Argo

University of Oklahoma, Norman, OK

Introduction Acute invasive fungal rhinosinusitis (AIFRS) is an often fulminant disease in immunosuppressed patients with mortality rates of 50%–90%. We report a patient with severe aplastic anaemia with invasive Aspergillus rhinosinusitis who responded to multimodal therapy with endoscopic sinus (ES) surgery, granulocyte infusions and multi-agent antifungal therapy.

Case description A 5-year-old girl with severe idiopathic aplastic anaemia presented with unilateral periorbital swelling, nasal congestion and tearing of right eye 2 months after initial diagnosis. Hematopoietic stem cell transplant was not done due to lack of HLA matched donor. Due to recurrent infections, immunosuppressive therapy(IST) was not initiated. Upon transfer to our facility, a CT head was done which demonstrated severe right sided pan-sinusitis. Sinus cultures was +ve for Aspergillus flavus. Broad spectrum coverage including antifungal therapy was initiated and then modified to amphotericin, micafungin, and voriconazole based on sensitivity. Extensive ES debridement was performed repeatedly over the ensuing weeks. With ongoing evidence of AIFRS, application of Ambisome-imbedded gauze was initiated along with daily donor granulocyte infusions with resulting trough ANC of more than 500 cells/µL. After 4 weeks of combination therapy, no further re-accumulation of sinus debris was noted and fungal elements were absent on histopathologic evaluation.

Conclusion AIFRS is increasing due to advances in diagnosis and treatment of paediatric malignancies and immune deficiencies. Aspergillus flavus is the most common organism. Prolonged neutropenia and Aspergillus infection are associated with a high mortality, especially in patients with no prospect for neutrophil recovery like our patient. Early institution of therapy with aggressive sinus debridement and multi-agent antifungals are of paramount importance in reducing mortality.

293 Varicella zoster meningitis with shingles in a young immunocompetent adult

P Sankhyan

A Mahajan

M Khalid

V Kohli

D Pierce

East Tennessee State University, Johnson City, TN

Case report A 39 year old man presented with sudden onset burning pain in the right flank, followed a few hours later by a fever (101 Fahrenheit) with headache and associated photophobia and phonophobia. He had no medical problems predisposing him to an immune compromised state and had a self-limited varicella zoster infection during childhood. On physical examination, he had mild neck stiffness and a vesicular erythematous rash in the T7-T8 dermatome (figure 1). Lumbar puncture showed increased protein, pleocytosis with lymphocyte predominance (94%) and normal glucose. All his other tests were unremarkable. Computed Tomography and Magnetic Resonance Imaging of the head was normal. He was diagnosed with aseptic meningitis and started on Intravenous (IV) Acyclovir followed by oral valcyclovir. His cerebrospinal fluid (CSF) returned positive for Herpes zoster polymerase chain reaction (PCR) 4 days after beginning treatment. On follow up, he had improved significantly with minimal fatigue and no sequelae. CSF findings for varicella and herpes simplex are similar, but the incidence of varicella meningitis is much lower (range 3%–20%). It can also cause meningitis preceding the rash or without the characteristic rash in upto 40% cases. Most data suggests treating immunocompromised patients with IV antivirals but guidelines for treating immunocompetent patients are unclear. But timely start of antiviral treatment can help prevent complications like motor neuropathy, herpes encephalitis, post herpetic neuralgia and the vision-threatening complications herpes zoster ophthalmicus and acute retinal necrosis.

Abstract 293 Figure 1

Vesicular erythematous rash in the T7-T8 dermatome

294 Central nervous system aspergillosis a medical challenge

RM Medrano1

D Sotello1

K Nugent1

S Alvarez2

1TTUHSC, Lubbock, TX

2Mayo Clinic, Jacksonville, FL

Case report A 56 year old male with fever and headache 3 days of duration. Four days later he developed generalised tonic-clonic seizures, which improved with anti-seizure medications and steroids. Had history of end-stage liver disease secondary to alcoholic cirrhosis and underwent orthotopic liver transplant 2 years prior. MRI revealed a lobulated enhancing lesion involving the right fusiform gyrus with extension into the ependymal surface of the right temporal horn and atrium, figure A. Cerebrospinal fluid (CSF) examination revealed clear CSF,WBC 2 cells/HPF (lymphocytes 86%) glucose 109 mg/dL,protein 82 mg/dL, negative cultures and VDRL. He had normal WBC, chemistry panel, and negative serum Aspergillus antigen. MRI guided stereotactic brain biopsy showed focal necrosis and septate fungal hyphae, culture was positive for Aspergillus fumigatus. Was treated with voriconazole 400 mg every 12 hours (indefinitely). Surgery was not recommended due to location of the lesion and related risk. The patient had excellent response after 20 months of therapy, figure B. CNS aspergillosis represents the most severe presentation of all forms of aspergillosis. Risk factors: neutropenia, hematologic malignancies, bone marrow or solid organ transplants, and chronic use of steroids. The diagnosis requires a positive CSF culture; CSF Aspergillus antigen and/or PCR may be useful, although brain biopsy may be required. Therapy requires combination of surgery and antifungal therapy; mortality without surgical management has been described from 60.4%–100% vs 25%–28.6% with combination therapy. Voriconazole is the antifungal drug of choice.The patient favourable outcome despite the absence of surgical intervention may be due to prompt diagnosis, aggressive antifungal therapy, and decrease of the intensity of immunosuppression.

Abstract 294 Figure 1

(A) lobulated enhancing lesion involving the right fusiform gyrus with extension into the ependymal surface of the right temporal horn and atrium; (B) after 20 months of therapy

295 An unusual case of peritonitis in a peritoneal dialysis patient

B Stadsvold1

M Varghese1,2

S Baer1,2

1Medical College of Georgia at Augusta University, Augusta, GA

2Augusta VA Medical Centre, Augusta, GA

Case report Peritoneal dialysis (PD) is a convenient alternative to hemodialysis with a decreased risk of blood stream infections for end stage renal disease patients. However, the risk of peritonitis is significant and when caused by unusual organisms, diagnosis and treatment may be difficult. This is the case of a 60 year old female with a past medical history of diabetes mellitus, hypertension, and end stage renal disease on peritoneal dialysis for 7 years, who presented with diarrhoea and diffuse abdominal pain not improving on amoxicillin/clavulanate as an outpatient. Four days prior, she had received a blood transfusion for symptomatic anaemia and consequently missed a round of PD. She was admitted for sepsis, suggested by tachycardia and leukocytosis of 30 000. Peritoneal fluid analysis revealed 4000 leukocytes with 90% neutrophils, and gram stain of the fluid revealed no organisms but many neutrophils. Two days after the peritoneal fluid was collected, cultures had growth of mould. Due to her altered mental status and electrolyte abnormalities, anti-fungal therapy with intravenous isavuconazonium sulfate was initiated. The PD catheter, removed the following day, was noted to have gross mould contamination. Aspergillus niger was identified in culture of the fluid. Serum galactomannan levels were positive and repeated to trend efficacy of treatment which showed questionable improvement from 6.5 to greater than 5.48 (normal value:<0.5) within a week. The patient’s hospital course was complicated by aspiration pneumonia and septic shock requiring multiple vasopressors. Approximately two and a half weeks after admission, due to the patient‘s worsening clinical status, the family decided to pursue comfort care, and the patient expired. Peritonitis is a common complication in chronic peritoneal dialysis. While the majority of cases are of bacterial origin, fungi are responsible for up to 15% of cases, with a fraction of those caused by filamentous fungi such as Aspergillus, Fusarium, Penicillium, and dematiaceous moulds. This case highlights the difficulties in diagnosis and treatment of mould peritonitis.

296 Stari night: southern tick associated rash illness after autologous stem cell transplant

N Summers

Z Chai

M Shums

M Lechowicz

R Friedman-Moraco

Emory University, Atlanta, GA

Case report A 57 year-old male from Georgia with Stage IV Mantle Cell Lymphoma underwent autologous hematopoietic stem cell transplant conditioned with busulfan/cyclophosphamide/etoposide. On day 5 post-transplant, he developed fevers to >40 C, headache, and multiple large erythematous skin lesions with central clearing on his right arm, right chest, left scapula, and right ankle. He had no other systemic symptoms. Fevers persisted despite >48 hours of broad spectrum antimicrobial therapy. He endorsed multiple tick bites prior to admission. It was felt that his clinical presentation was most consistent with STARI (southern tick associated rash fever). Treatment with doxycycline 100 mg twice daily was initiated and the patient defervesced within 24 hours. His rash resolved over the course of several days. He completed a 14 day course of doxycycline. Additional work-up, including acute and convalescent antibodies for Ehrlichia and RMSF (Rocky Mountain Spotted Fever) were negative. He was doing well at post-transplant follow-up with bone marrow engraftment and resolution of symptoms.

Discussion STARI causes a similar clinical presentation to that of Lyme disease (Borrelia burgdorferi) transmitted by Ixodes scapularis, however the vector is the Lonestar tick (Amblyomma americanum). Skin lesions tend to be larger and multiple in STARI compared to Lyme disease, and patients may have more systemic symptoms. The causative agent is not known, but is felt to be a spirochete. STARI in immunocompromised patients has not been widely reported. Immunocompromised patients may be at risk for more severe manifestations.

Abstract 296 Figure 1

Patient’s back

297 Loasis manifested as an eye worm in an active duty soldier native of nigeria

T Tobin

K Davis

Dwight D Eisenhower Army Medical Centre, Fort Gordon, GA

Case report Ocular loaisis is a subclinical syndrome involving the migration of an adult L. loa worm across the sub-conjunctiva. Typical symptoms include eye pruritis and pain in addition to transient angioedema characterised by localised subcutaneous swellings. While common in endemic African countries, it is rarely seen in the United States.

A healthy, 28 year-old male from Nigeria was seen at sick call for left eye pruritis, erythema, and clear discharge for four days. He had no significant medical history and was taking no medications. He was referred to ophthalmology at Balboa Medical Centre when a worm was visualised in the sub-conjunctiva. On further history, he had returned to Nigeria in 2015 but did not recall any specific insect exposure or if family members were affected. He was transferred back to his home duty station, Fort Stewart, to allow him to be closer to family while receiving treatment. Ophthalmology re-examined him on arrival, but they were unable to visualise the worm again. Physical exam was only positive for left conjunctival injection, but no signs of anterior eye fibrosis, retinal involvement, or concomitant onchocerciasis were noted. Blood tests drawn at noon local time to quantify microfilariae were negative at the reference laboratory. Serologic testing for loiasis was positive and negative for onchocerciasis. He was begun on diethylcarbamazine (DEC) for treatment.

L. loa is transmitted by horse flies, most commonly Chrysops dimidiate and C. silacea, and it can present years after initial inoculation. Diagnosis is made by visualisation of a worm or by serologic testing at the NIH. Treatment is with DEC. Quantification of microfilariae in the blood is important in the management of this infection. Microfilariae are released by adult worms into peripheral blood and can be found circulating between 10 am and 4 pm. Measurement of microfilariae greater than 2500 per mL increases the risk of developing fatal sequela as an adverse effect of DEC treatment. Plasmapheresis prior to DEC treatment may decrease the risk of adverse effects when elevated microfilarial numbers are present. It is also important to assess for co-infection with onchocerciasis prior to initiating DEC as this can worsen with DEC treatment alone.

298 ‘eye’ know this is syphilis

A Traina

J Dubuc

H Oddo Moise

A Bourgeois

A Coulon

J Doan

S Sanne

LSUHSC, New Orleans, LA

Case report A 34 year old homosexual man with no past medical history presented to the emergency department with left eye pain and redness for 7 days. Upon further questioning, he reported that his last sexual encounter was 2 years ago followed by a flu-like illness. His physical exam was remarkable for diffuse left eye scleral injection, patchy alopecia, and thick hyperkeratotic plaques with hyperpigmented lesions on his palms and soles. Screening tests in the emergency department revealed that he was positive for Human Immunodeficiency Virus (HIV) which prompted further work up for other infections including syphilis. RPR titer was strongly positive at 1:256 with positive FTA-ABS. Although cerebral spinal studies were negative for VDRL, he was started on intravenous penicillin G for treatment of tertiary syphilis given ocular involvement. The following day, he patient became febrile to 103F, consistent with Jarisch-Herxheimer reaction. He was also started on trimethoprim/sulfamethoxazole and azithromycin as prophylaxis for opportunistic infections given his CD4 T-cell count of 16/mm3 with 2.9% CD4 cells. Other diagnoses such as Acute Retinal Necrosis (ARN), Progressive Outer Retinal Necrosis (PORN) and Cytomegalovirus (CMV) retinitis were less likely given his weakly positive antibodies for Herpes Simplex Virus (HSV), Varicella-zoster virus (VZV) and CMV with negative HSV DNA. Ophthalmology performed serial retinal exams without signs of retinal necrosis, but recommended continuing acyclovir since the entire retina was unable to be visualised. His ocular symptoms and palmar and plantar skin changes improved with antibiotic and antiviral treatment. He was discharged with 14 days of intravenous Penicillin G with outpatient referrals to infectious disease and ophthalmology.

Discussion Patients with severe immunodeficiency may not only be unaware of their HIV status but may also present with complicated presentations of other sexually transmitted infections and opportunistic diseases. This case highlights the importance of screening for HIV infection at least once for individuals ages 13 to 75 years or more frequently for those with increased risk factors to ensure early treatment and to reduce transmission to others.

299 Polymicrobial endocarditis caused by abiotrophia defectiva, bacillus cereus, bacillus subtilis and bacillus megaterium in the setting of injection drug use

TT Tran1

M Varghese1

S Baer1,2

1Medical College of Georgia, Augusta, GA

2Augusta VA Medical Centre, Augusta, GA

Case report Polymicrobial infective endocarditis (IE) is more commonly found in the setting of injection drug use (IDU). We present a case of a 52 year-old female with ongoing IDU. She initially presented to the emergency department with lower back and right hip pain. She was discharged with oral trimethoprim/sulfamethoxazole for a presumed urinary tract infection. Blood cultures obtained during the visit later returned positive for Abiotrophia defectiva. She was lost to follow up until two months later when she presented with dysarthria, altered mental status, and atrial fibrillation. She was noted to have IE on both mitral and aortic valves and was started on intravenous vancomycin and piperacillin/tazobactam. Preliminary results of her blood cultures were consistent with Bacillus species, which were sent to reference lab for further identification. Final identification found A. defectiva, Bacillus cereus, Bacillus subtilis and Bacillus megaterium, so antibiotics were simplified to Vancomycin. She defervesced and her bacteremia cleared. Due to her extensive mitral and aortic valve vegetations, she underwent mitral and aortic valve replacement. Her hospital course was further complicated by persistent arrhythmia and critical left leg ischemia requiring a permanent pacemaker and emergent thrombectomy. She was discharged after 38 days to an acute long term care hospital.

This case highlights the risk of polymicrobial IE in the setting of IDU as many variables can introduce common organisms from the oral cavity and the environment into the blood stream such as the type and purity of the drug and the use of unsterile procedures and paraphernalia. A. defectiva, part of the normal human flora, is a rare but aggressive cause of IE due in part to its propensity to develop large vegetations and shed septic emboli. Bacillus species are common soil microorganisms and are uncommonly associated with bacteremia or IE. Thus isolation of rare or unexpected organisms from endocarditis supports the importance of asking patients about the practices associated with their intravenous drug use.

300 Soft tissue infections following water exposure

JP Walther

R Welliver

University of Oklahoma College of Medicine, Oklahoma City, OK

Case report A previously healthy 12 yo female presented to ED after a labial laceration. She feel off a raft and was injured by the rope attached to the boat. Patient was seen by the gynaecological team who repaired the laceration which measured 8 cm ×4 cm on R labia majora. Twenty hours after presentation, she developed a fever up to 39.2 C. The incision had appropriate swelling with no induration. Patient was started on ceftazidime. Patient became afebrile and was transitioned to oral Bactrim. Wound culture grew moderate gram positive cocci; blood cultures with no growth. Later that day, patient developed significant pain, was unable to move right leg, and was febrile to 38.3 C. On exam, labial incision had a 1 cm opening at the top with purulent fluid, and the right vulva was erythematous and indurated. Patient was taken to the OR for I and D which found purulent material with necrotic debris at the base of the labial ulceration and purulent drainage tracking 10 cm into the right thigh. After washout, patient was started on Vancomycin, Zosyn, Doxycycline, and Ceftazidime covering MRSA, Gram Negative rods, Pseudomonas, and Vibrio. Wound cultures grew Aeromonas veronii, Edwardsiella tarda, Streptococcus anginosus, and coagulase negative Staphylococcus. Tissue cultures grew Edwardsiella tarda, Plesiomonas shigelloides, Streptococcus anginosus, Streptococcus gordonii, and Prevotella bivia. Vancomycin and doxycycline were discontinued after 24 hours. A PICC line was placed. She received 8 days of Zosyn and Ceftazidime, which covered all the bacteria grown in culture. Prior to discharge, patient was afebrile, tolerating regular diet, and able to ambulate. Plan is to continue IV antibiotics for 14 more days.

Soft tissue infections caused by water organisms are uncommon but potentially fatal. The 5 organisms that should be thought of when a patient presents with a wound exposed to water are Aeromonas spp, Edwardsiella tarda, Erysipelothrix rhusiopathiao, Vibrio vulnificus, and Mycobacterium marinum. Empiric treatment includes Ceftazidime or clindamycin, Levofloxacin, and Metronidazole or Doxycycline depending on exposure. This case illustrates the importance of recognising a potential infection from a soft tissue wound exposed to water with the potential for infection and the importance of empiric antibiotic treatment.

301 Invasive disease caused by haemophilus influenza type a

AM Wolf

S Boppana

University of Alabama Birmingham, Birmingham, AL

Case report Historically, paediatricians have feared the haemophilus influenza type B bacteria due to its ability to cause life-threatening disease in our patients. However since the widespread use of the H. influenza type B vaccine, invasive infections caused by H. influenza type B have been almost completely eradicated. Yet invasive infections caused by other strains of the haemophilus influenza bacterial class have become more common in its absence. Here, we present two children with meningitis due to H. influenza type A at Children’s of Alabama in the last few months.

The case of an 8 mo F and a 2 yo M with h. influenza type A meningitis were examined. The first child an 8 mo female, initially presented to an outside hospital, was diagnosed with hand, food, and mouth disease, and discharged to home. She returned the following day with fever and lethargy at which time CSF studies showed meningitis. She was started on treatment with ceftriaxone, then transferred to COA PICU after seizure activity. CSF cultures grew H.influenza type A and the patient quickly recovered, was extubated and transferred out of the ICU. Her course was complicated by persistent fevers and further seizure activity prompting an MRI brain, which revealed a right frontal empyema. The infant was treated with ceftriaxone monotherapy with eventual full recovery. The second case involves a 2 yo M with no past medical history who presented to the ED with multiple days of fever, vomiting, and lethargy and was found to be in septic shock. CSF studies were concerning for bacterial meningitis and the patient was admitted to the ICU, although never required intubation. His CSF cultures also grew H. influenza type A and he was treated with ceftriaxone monotherapy. He was placed on seizure prophylaxis due to possible seizure activity. He also had prolonged fevers, but repeat lumbar puncture was negative and MRI brain was consistent only with meningitis. He recovered and was discharged to home.

These two cases of meningitis in addition to another child with unrecognised splenic heterotaxy who had severe sepsis caused by H. influenza type A at our institution in the past 18 months highlights the need for awareness of the importance of this organism causing CNS and systemic infections. A retrospective review of charts at COA over the past 10 years is in progress.

Neurology and neurobiology, Joint plenary poster session and reception, 4:30 PM, Thursday, February 22, 2018

302 Chiari-i malformation: challenge in diagnostic and therapeutic decisions

D Gebremariam

K Nugent

G Bedanie

TTUHSC, Lubbock, TX

Introduction Chiari malformations refer to a spectrum of congenital hindbrain abnormalities and listed as a rare disease by the National Institute of Health. Diagnostic differentiation from other causes of headache like migraine used to be a challenge. However, with routine use of magnetic resonance imaging, it is discovered with increasing frequency. Chiari type-I is the most common and least severe form. Its hallmark is caudal displacement of cerebellar tonsils below the foramen magnum referred to as congenital tonsillar herniation.

Case presentation A 31 year old man with chronic headaches treated as migraine presented with acute occipital headache of 3 days duration. Headache was exacerbated when bending down with blurring of vision in left eye. He had no motor or cranial nerve deficit. His CSF and opening pressures were normal. MRI showed low lying cerebellar tonsills and syrinx at C6–7; phase contrast cine MRI showed absence of CSF flow posterior to the tonsils. He was readmitted two times within a month due to recurring headaches, loss of sensation, and tingling in his upper extremities. Chiari-I as cause of headache and not migraine was strongly considered; he had a decompression craniectomy leading to resolution of his headaches.

Discussion Chiari-I malformation can present in adulthood and may be misdiagnosed as in this patient. Most common symptom is headache exacerbated by cough and Valsalva manoeuvre. Syringomyelia can present together like in this patient. MRI is the most useful imaging study. CSF flow analysis through foramen magnum with phase contrast cine MRI provides further supportive evidence. However the real diagnostic and therapeutic challenge is encountered in patients like ours who was thought to have migraine for years. He was initially assumed to have his symptoms from his migraine when he was admitted for the first time. He was not responding to medical management. He finally had a decompressive craniectomy with resolution of headache. Our case demonstrates diagnostic and therapeutic challenges when a rare cause of headache like chiari-I malformation which may need a more invasive and aggressive treatment presents like migraine.

303 Hydranencephaly: a rare case of cortical absence in a neonatal patient

HH Patrick

RE Herdes

LM Lasseigne

E Smith

JM Volk

Louisiana State University Health Sciences Centre, New Orleans, LA

Case report Hydranencephaly is a rare disorder characterised by the near-total absence of cerebral hemispheres. Caudal brain structures are usually present. Aetiology is unknown, hemispheric absence is believed to be caused by a prenatal vascular insult. It is suspected that damage to vasculature at the base of the cranium leads to the destruction of the cerebral hemispheres. Frequently diagnosed in-utero during prenatal screening with ultrasound, MRI after birth is the best modality for diagnosis. Patients can develop irritability, hypertonia, and seizures. Absence of sellar structures leads to panhypopituitarism requiring endocrine workup and treatment. They generally have a poor outcome and survivability, and many children with this disorder expire before one year. Treatment is supportive and palliative.

We report the case of a 39 1/7 WGA term infant who presented to NICU shortly after birth secondary to prenatal concerns for alobar holoprosencephaly on maternal ultrasound. Physical exam revealed macrocephaly with a full anterior fontanelle and splayed sutures. HUS at time of delivery was consistent with hydranencephaly. And the diagnosis was confirmed with MRI. A palliative shunt was placed on DOL 5 due to family’s desire to proceed with maximum medical and surgical treatment. Endocrine work-up was within normal limits. Not surprising, the patient initially required respiratory support and had inconsistent nippling skills requiring gavage feedings for adequate nutrition. Developmental evaluation showed an overall poor prognosis for gross motor development secondary to minimal cortical tissue.

This case represents an unusual case of cortical absence in a neonatal patient. While suspected diagnosis is usually made prenatally with ultrasound, clinicians should be suspicious of neonates who present with macrocephaly, respiratory distress, widened cranial sutures, irritability, seizures, hypertonia, or neurological deficits. Head US should be utilised as an initial imaging modality for diagnosis but should be confirmed with MRI. These patients represent a unique population of paediatric patients who receive palliative care with poor prognostic outlooks.

304 Lateral medullary syndrome; resulting from unexplained thrombocytosis

MK Islam

A Islam

Texas Tech Univ HSC Amarillo, Amarillo, TX

Case An 53 year old gentleman came to the hospital because of dysphagia, vertigo and facial numbness that has been getting worse over 1 week. Denies any focal weakness, seizure, vision problem. He is a previous cocaine and methamphetamine abuser; quit 10 years ago, but continues to take marijuana in a daily basis. Vitals: temperature 36.7 C, pulse 65/min, respiratory rate 24/min, BP 143/91, SPO2%–100% on room air. On physical exam: CTA B/L, RRR, S1/S2 normal, alert and oriented ×3, no motor weakness, left sided facial numbness was present and unable to differentiate hot and cold sensations over right upper and lower extremity. Left cranial nerves V, IX, X were involved. Babinski and Pronator Drift were negative, gait couldn’t appreciate due to vertigo and unsteadiness. Initial lab: Hb 13.9, MCV 88, WBC 17.2, Platelet 901, Na 139, K 3.5, Cl 109, HCO3 23, BUN 11, Cr 0.70, LFT normal, urine toxicology positive for amphetamine and marijuana. Initial CT scan head was negative for stroke, subsequent MRI of brain showed multiple infarct involving left side of medulla, posterior portion of lateral ventricle and right temporal-occipital region. CTA of head and neck showed left intracranial vertebral artery thrombosis. TTE didn’t show any vegetation or structural heart disease. Extensive evaluation for thrombocytosis including BCR-ABL, JAK-2, PBF, thrombophilia screen; which came back negative.

Discussion Wallenberg’s syndrome (WS) is well defined clinically, and the lateral medullary infarction (LMI) is the most frequent cause. Although the combinations of the various signs and symptoms are helpful for the clinical diagnosis of WS, the presence of the different signs and symptoms may vary from patient to patient. Dysphagia has been reported in 51% to 94% of the patients with WS. It has been widely accepted that, the dysphagia in WS is initially severe enough to require nothing per oral but often improves rapidly. Unexplained thrombocytosis and drug abuse could play a pivotal role for development of LMI of this patient. Our case reminds the physician to consider unexplained thrombocytosis as a potential factor for developing stroke. It is very important for a careful neurological examination which can recognise atypical presentation of stroke very early, which could be lifesaving.

305 Non-sspe related periodic long-interval diffuse discharges and widespread diffusion restriction in the grey matter

JK Jones1

AB Ramos1

J Newsom2

EC Mader1

C Barton1

1LSUHSC-New Orleans Neurology Department, New Orleans, LA

2Ochsner, Kenner, LA

Background The electroencephalogram (EEG) of patients with diffuse encephalopathy may show generalised periodic epileptiform discharges (GPEDs). GPEDs repeating every 0.5–4 s are periodic short-interval diffuse discharges (PSIDDs) and GPEDs repeating every 4–30 s are periodic long-interval diffuse discharges (PLIDDs). PLIDDs have been reported mostly in patients with subacute sclerosing panencephalitis (SSPE) and a few patients with acute encephalopathy. By contrast, PSIDDs are not an uncommon finding in critically ill patients with acute encephalopathy. We report a case of non-SSPE related PLIDDs in a patient with acute encephalopathy and widespread MRI diffusion restriction in the grey matter. Within a week, the PLIDDs were superseded by PSIDDs.

Case report A 55-year-old man with chronic liver disease and portal hypertension presented with bleeding esophageal varices and depressed sensorium requiring transfusion and intubation. On day 4, he had a tonic-clonic seizure prompting treatment with lorazepam and levetiracetam. EEG showed PLIDDs with no clinical correlate. Brain MRI showed bisymmetric diffusion restriction in the insula, temporal neocortex, thalami, and other grey matter structures. After treatment of metabolic disturbances, he started responding to simple commands and was extubated. On day 11, he became stuporous and EEG showed PSIDDs. Lacosamide was added to levetiracetam. EEG the next day showed PSIDDs with shorter interval. On day 15, he developed respiratory failure and passed away.

Conclusion It is not clear why PLIDDs is rare in encephalopathic non-SSPE patients. A possible explanation, suggested by this case, is that PLIDDs represent an unstable state in brain neurodynamics that inevitably transitions to a more stable state, such as PSIDDs. It is also unclear what brain structures must be compromised to produce PLIDDs. In SSPE, MRI lesions usually develop in the cortex and subcortical regions and spread to the periventricular white matter. In the case presented, the MRI obtained on the same day the PLIDDs were recorded showed bihemispheric grey matter diffusion restriction with prominent involvement of the insula, temporal neocortex, and thalami.

306 Idiopathic intracranial hypertension and leukoencephalopathy as an initial presentation of systemic lupus erythematosus

DH McVadon

P Craddock

J Ingram

University of Mississippi Medical Centre, Ridgeland, MS

Case report Idiopathic intracranial hypertension (IIH) is a rare neuro-ophthalmic manifestation of Systemic Lupus Erythematosus (SLE). Diagnosis of IIH in SLE patients can be challenging often with delayed treatment; however, prompt diagnosis is critical to early and appropriate treatment. Reported cases of IIH with diffuse white matter changes on imaging are sparse, especially in the paediatric population. SLE should be considered in patients with unexplained neurologic symptoms especially females of reproductive age to ensure prompt and optimal treatment. We present a case of a 13-year-old female who presented with symptoms of IIH and significant white matter changes, subsequently found to have SLE with lupus cerebritis.

307 Triple whammy – ischaemic stroke due moya moya disease in a patient with graves and sickle cell disease

R Samannan1

M Allee2

1University of Oklahoma Health Sciences Centre, Oklahoma City, OK

2University of Oklahoma, OKC, OK

Introduction Moya moya disease (MMD) is a chronic occlusive cerebrovascular disorder characterised by bilateral stenosis of the supra-clinoid portion of the internal carotid arteries with collaterals. The disease is rare among black population. Here we describe a young African American patient with sickle cell trait who presented with right middle cerebral artery (MCA) territory infarct and in thyrotoxic crises.

Case description 34-year-old African American male with a history of poorly controlled Grave’s disease presented with history of acute onset of left sided weakness and chest pain. Magnetic resonance angiogram (MRA) of brain showed near occlusion of both distal supra-clinoid internal carotid artery (ICA) with patent bilateral proximal MCA and right ischaemic infarct diagnostic of ischaemic type of MMD. Investigations revealed patient to be in thyrotoxic crises with a low thyroid stimulating hormone, elevated free Thyroxine and free tri-iodo-thyronine. Sickle cell screen was positive. Due to intolerance of antithyroid medications due to agranuloctyosis, he underwent thyroidectomy. Right superior temporal artery (STA) to middle cerebral artery (MCA) bypass was done later with some improvement of symptoms. At discharge continued to have residual left side weakness.

Discussion Although prevalent in all races, MMD is extremely rare in black population. Various etiologies including hemolytic anemias, genetic and graves disease are for attributed to causing MMD. Cellular proliferation, vascular dysregulation, immunologic stimulation, enhanced sympathetic activity are postulated causes of MMD in graves while and subedothelial anoxia is postulated cause in hemoytic anemias

Cerebral angiography is the gold standard both for diagnosing MMD. Surgical bypass is recommended with superficial temporal artery to MCA (STA-MCA) anastomosis.

Conclusion This presentation of MMD with both Graves disease and sickle cell trait in an African American patient is new and has not been reported in literature.

308 Transient global amnesia and b12 deficiency; is there a relationship?

S Siddiqui

N Salagundla

S Wan

A Islam

Texas Tech University HSC, Amarillo, TX

Case A 64 year-old Caucasian farmer with no past medical history presented to ER with acute confusional state. He was in good health and went out for buying parts for his tractor. On his way back he forgot what he was doing. EMS was called in and they found patient to be confused and was brought to ER for evaluation. On arrival he was evaluated for possible stroke or TIA. On examination patient was vitally stable, neurological examination disclosed loss of memory, orientation, swallowing function and repeated questioning with no focal neurological deficits. He underwent further workup with MRI brain, spinal tap and EEG; all of which did not show any pathology. He was started on artificial tube feeding for nutritional purposes for acute dysphagia. He was also started on B12 replacement therapy as his levels were low. Patient was diagnosed with Transient Global Amnesia and over days of supportive treatment patient clinically improved and returned back to his baseline functional status.

Discussion Transient global amnesia (TGA) is a sudden and temporary loss of memory. It consists of anterograde amnesia as well as some retrograde amnesia. However, the patient retains executive functions and procedural memory. TGA can be preceded by trauma and stress but those factors are not necessary. The event normally resolves within 24 hours and the patient returns to baseline memory function. There are no clear tests to confirm diagnosis of TGA. This case presents an atypical look at TGA; while it normally resolves within 24 hours, our patient took more than 48 hours to recover and he had swallowing difficulty merely because he was not sure what to do with his food. Patient received B12 supplementation during his hospital stay. There has been no proven link between B12 deficiency and TGA, but there has been cases reported in which association of TGA with hyperhomocysteinemia is seen. To date TGA is thought to be primarily a diagnosis of exclusion but we may need to explore more in relation to B12 deficiency and hyperhomocysteinemia. We describe a rare case of TGA which required more than 24 hours to resolve and patient had B12 deficiency.

Paediatric clinical case, Joint plenary poster session and reception, 4:30 PM, Thursday, February 22, 2018

309 That’s not osteomyelitis

M Adams

S Narayanan

M Orr

UAB, Birmingham, AL

Case report An 11 year old male was seen in our ED with 3 weeks of daily fevers, migratory joint pains, hematuria, and a 10 lb weight loss. Two months prior, he was admitted to an outside hospital with migratory joint pain and diagnosed with septic arthritis of the left hip, treated with surgical washout and 2 weeks of clindamycin. After completion, he had continued left leg pain and was treated with Trimethoprim-Sulfamethoxazole for 3 days, after which he developed a generalised petechial rash with rosettes. Labs on admission revealed elevated CRP/ESR, gross hematuria, and an elevated protein/creatinine ratio. A bone scan revealed uptake in the left proximal femur.

Initial differential included drug reaction, vasculitis, osteomyelitis with systemic inflammatory process, and chronic recurrent multifocal osteomyelitis. A CBC, BMP, LDH and uric acid were normal. MRI showed unclear left proximal femur enhancement that could not rule out osteomyelitis; an orthopaedic surgery consult felt that the findings could be related to post-surgical heterotopic ossification. On hospital day 3, he became hypertensive and hypoxic; a chest X-ray revealed small bilateral pleural effusions. At this point labs returned with a positive cANCA concerning for a systemic vasculitis with glomerulonephritis. Non-contrast sinus and chest CT revealed minor sinus disease without bony destruction and diffuse pulmonary opacities. Renal biopsy revealed necrotizing glomerulonephritis with interstitial neutrophils and eosinophils confirming the diagnosis of Granulomatosis with Polyangiitis (GPA). Femoral biopsy revealed no signs of acute osteomyelitis. Following the biopsy, he developed hypoxemia and pulmonary haemorrhages. He was treated with pulse dose steroids, Rituximab and Cyclophosphamide infusions with symptomatic improvement.

GPA, formerly known as Wegener’s Granulomatosis, is a rare progressive necrotizing granulomatous vasculitis that commonly affects the respiratory tract, kidneys, and systemic small vessels. It is important to keep in the differential as its presentation may be non-specific but may rapidly progress to overt renal failure or diffuse pulmonary haemorrhage and can be fatal without prompt treatment. Unique to this case is the concern for joint infection and its demonstration of the importance of quickly ruling out infection so that immunosuppressant medicines may be started early in the course.

310 Complex regional pain syndrome of the abdominal wall in an 8-year-old female

O Altun1

B Faynberg2

1Karolinska Institutet, Stockholm, Sweden

2Florida State University, Pensacola, FL

Case report Complex regional pain syndrome (CRPS) is a rare disease characterised by pain out of proportion to stimulus. Generally associated with trauma or surgery, a small percentage of CRPS cases present spontaneously. With no gold standard test for diagnosis, is a difficult condition to diagnose and manage in the paediatric population.

An 8-year-old female presents with diffuse, progressively worsening abdominal pain and distention that developed over the past 10 days. Mild and periumbilical at first, the pain became severe, diffuse and extremely sensitive to touch, easily triggered by clothing or a blanket. Parents report poor appetite, but no other symptoms. Other than some recent constipation, there is no significant medical history. The patient seemed distressed, with a diffusely tender abdomen and slightly mottled abdominal skin. Vitals were normal and the rest of the examination was unremarkable. Initial labs were only significant for HCO3 of 12, glucose of 59, and WBC count of 14.2 with a slight elevation in neutrophils and no bands. Contrast CT only showed possible mesenteric lymphadenitis and a distended urinary bladder. Persistent symptoms prompted extensive laboratory workup and ultimately an exploratory laparoscopy, but a definitive cause was not identified. Pain control, meanwhile, was very challenging. Medications were largely ineffective and an epidural finally provided some relief. In coordination with Neurology she was then started on gabapentin, which helped to some degree, suggesting possible neuropathic origin. With no better explanation for her symptoms, findings were compared to the Budapest criteria, which consider sensory, vasomotor, sudomotor, and motor/trophic characteristics. This patient had two signs and three symptoms among four different categories, which can diagnose CRPS with a sensitivity of 0.99 and specificity of 0.68. Following diagnosis, this patient may require multispecialty care at a paediatric inpatient pain centre for desensitisation and rehabilitation.

CRPS remains a challenging diagnosis and extensive workup is required to exclude other causes. Though it most commonly affects extremities, it can present on anywhere and is a valid consideration in patients with abdominal pain, but no identifiable origin.

311 An unreported clinical feature of classic menkes disease: naturel killer cell dysfunction

J Bhat

P Maertens

USA, Mobile, Alabama, Mobile, AL

Case report Menkes disease (MD) is an X-linked, multisystem lethal disorder of copper metabolism resulting from mutations in the ATP7A gene. Features such as Ehler Danlos syndrome, trichopoliodystrophy, urologic and skeletal changes have been reported. We present a case of classic MD treated with copper infusions who suffered from naturel killer (NK) cell dysfunction.

Case description A 2-year-old, Caucasian male child presented at 8-month-old of age with persistent hypotonia, kinky hair and developmental regression. Diagnosis of MD was based on low serum levels of copper {5 mg/dL (18–37)} and ceruloplasmin {18 ug/dL (75–153)} and gene sequencing studies revealing exon 12 deletion in ATP7A gene. Brain MRI showed mild hypoplasia of the cerebellar vermis and vascular tortuosity typical of MD. Copper chloride treatment was immediately initiated. Child became more alert with excellent eye contact and purposeful movements. The child was hospitalised for recurrent respiratory infections, each time caused by enterovirus as confirmed by multiplex PCR. In addition, he was also admitted for multiple episodes of fever of unknown origin. Extensive immunologic studies were negative, except for a severe NK cell dysfunction (0.6 NK lytic Units; n>2.6). We consider that NK cell dysfunction in classic MD can be explained by the deficient incorporation of copper in endoplasmic reticulum leading to an abnormal Fenton chemistry within phagosomes.

Conclusion NK cell dysfunction in classic MD has never been reported. It is known to result in recurrent viral infections, thus increasing morbidity in MD. Therefore, evaluation of NK cell function should be considered in patients with classic MD.

312 A case of recurrent contispation

J Blanding

N Zeky

E Mcdonough

E Klepper

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

Case report Choledochal cysts are dilations that form along the biliary tree. The most classical presentation is the triad of abdominal pain, jaundice, and abdominal mass. However, only 20% of patients will present in this manner. Most will present in the first decade of life.

A three year old female with chronic constipation presented to the Emergency room for abdominal pain and nonbloody, nonbilious emesis that did not resolve with enema or laxatives. On initial lab testing, she was found to have elevated transaminases, gamma glutamyl transpepitase, and lipase. Total bilirubin was within normal limits. Because of the abnormal labs, computerised tomography of the abdomen was obtained that showed intra and extrahepatic dilation of the bile ducts concerning for choledochal cyst. A magnetic resonance cholangiopancreatography was obtained and confirmed a type IVA choledochal cyst. Surgery was consulted. Additional laboratory testing showed an elevated Ca 19–9 but normal alpha fetoprotein levels. Ultimately, she was taken to surgery for excision and roux-en-y with hepaticojejunostomy. During surgery, the choledochal cyst was found to encompass the entire length of the extrahepatic biliary tree and bilateral intrahepatic systems. At follow up, transaminases normalised. Pathology was negative for malignancy, though the possiblity for malignancy remains if residual cyst tissue was left. She is currently being monitored for post-surgical complications including stenosis of the anastomosis site with annual labs.

While constipation is a common diagnosis in young children with abdominal pain, this case shows that with clinical suspicion and abnormal labs, paediatricians should think about other etiologies for abdominal pain in a young child.

313 Polymicrobial pott’s puffy tumour: a paediatric predicament

KS Booker

S Boppana

Children’s of Alabama, Birmingham, AL

Case An eleven-year-old African-American male with haemoglobin SC disease presented with facial swelling and fever with recent positive rapid influenza test. On exam, he had profound swelling over his left forehead. The left eye was swollen shut, with yellow exudate. Initial computed tomography (CT) showed preseptal cellulitis and sinus disease. Inflammatory markers were markedly elevated with ESR of 125. Despite intravenous antibiotics, swelling progressed. Repeat CT demonstrated abscess formation in the preseptal and frontal tissue with an intraorbital component. Patient underwent surgical drainage and was found to have disease extension to the subdural and epidural space. This required intraoperative consult and drainage by neurosurgery. Cultures from the forehead abscess grew Gemella morbillatum, Bacteroides uniformis, and Streptococcus constellatus. Orbital cultures grew S. constellatus, and intracranial abscesses grew B. uniformis and S. constellatus. The presentation is consistent with Pott’s puffy tumour.

Discussion Pott’s puffy tumour was originally described by Sir Percival Pott in the 18th century. In the era of antibiotics, it is now a rare complication of frontal sinusitis. Clinical features include headache, fever, and frontal swelling. The condition is defined by forehead oedema, thus ‘tumour’, due to subperiosteal abscess accompanied by frontal bone osteomyelitis. It can be complicated by life-threatening intracranial infection.

This is a case of a rapidly progressive invasive polymicrobial infection of the sinuses, with intracranial extension and orbital involvement. The causative organisms are unusual. G. morbillatum is a microaerophilic gram positive coccus. S. constellatus is a species of viridans strep of the anginosus group, which has microaerophilic or anaerobic growth. This group of streptococci has a propensity to form abscesses. Finally, B. uniformis is a gram negative anaerobe. Although these bacteria have not been previously described in the literature as occurring together, it is not surprising that these anaerobic and microaerophilic organisms thrived in the patient’s sinuses, which were inflamed due to the influenza virus, creating the environment suitable for a secondary bacterial infection.

314 Rash and renal failure in a teenage male

NA Booth

N Chiriboga

T Chadha

University of Florida, Jacksonville, FL

Case report A 15-year-old boy with morbid obesity presented to his paediatrician for a rash on his thighs and was prescribed oral and topical steroids. Two days later, he developed swelling in his extremities, arthralgias, fever to 103F and no improvement of his rash. He returned to his paediatrician who prescribed amoxicillin-clavulanate for a left acute otitis media. As his rash and arthralgias continued to worsen over the next 4 days (bilateral lower extremities down to his feet), he presented to the emergency room and was admitted. While inpatient, his rash was noted to be purpuric and bullous. His initial lab values revealed a normal BMP, elevated WBC of 22.5 K/mcL, elevated ESR and CRP of 95 mm/hr and 24.3 mg/L, respectively and a urinalysis revealed microhematuria. He was pan-cultured and started on vancomycin and piperacillin-tazobactam. Over his 6 day admission, his skin lesions continued to spread over his bilateral upper extremities, trunk and lower back. A culture of the lesions was positive for coagulase negative staphylococcus and he developed progressive renal failure with oliguria, a potassium of 6.5 mEq/L and BUN/Cr of 73/5.5 mg/dL. He was then transferred to our paediatric intensive care unit for further management (figure 1). He received emergent hemodialysis and underwent a complete rheumatological, infectious, and renal work-up including skin and kidney biopsies. While his skin biopsy revealed a leukocytoclastic vasculitis, his kidney biopsy showed acute interstitial nephritis without vasculitis. After a multidisciplinary discussion, he was started on high dose steroids for vasculitis of unknown aetiology. He eventually had resolution of his rash and recovery of renal function. While rashes are commonplace in paediatrics, this case stresses the importance of early recognition of a vasculitic rash as it should prompt serial monitoring of kidney function and potential evaluation by specialists.

Abstract 314 Figure 1

Sample of widespread rash, on admission to our institution’s PICU

315 Sarcoidosis in an adolescent male presenting with hypercalcemia and weight loss

J Bridges1

C Glendye1

N Washington2

1University of Mississippi Medical Centre, Jackson, MS

2Mississippi Centre for Advanced Medicine, Madison, MS

Introduction Sarcoidosis in older children usually presents with lymphadenopathy, pulmonary involvement, fever, weight loss, and malaise; it rarely presents with hypercalcemia. Diagnosis is made by histopathologic findings of non-caseating and giant cell granulomas with exclusion of other causes of granulomatosis. In this case, an adolescent male with hypercalcemia was diagnosed with sarcoidosis.

Case description A 14 year old male presented with weakness and 70 lb weight loss over one year, elevated transaminases, thrombocytosis, and severe hypercalcemia. Thyroid stimulating hormone and C-reactive protein were within normal limits; erythrocyte sedimentation rate was elevated and parathyroid hormone and magnesium were low. Chest X-ray and computed tomography lacked hilar lymphadenopathy or visible lung pathology. Aggressive hydration improved the patient’s hypercalcemia and acute kidney injury. Angiotensin converting enzyme, lysozyme, and vitamin D-1,25-OH levels were elevated. Concern for sarcoidosis was confirmed via lymph node biopsy revealing granulomatous inflammation without caseating necrosis. Dilated eye exam revealed anterior uveitis and pulmonary function tests showed a restrictive pattern. Symptoms improved with pulse-dosed steroids and methotrexate. He was discharged with rheumatology follow up.

Discussion Only 5% of paediatric patients with sarcoidosis have hypercalcemia at presentation. Hypercalcemia in sarcoidosis is thought to be secondary to autonomous 1-alpha-hydroxylase activity within granulomas. In addition to hypercalcemia, the absence of pulmonary findings at time of diagnosis makes this presentation of sarcoidosis unique. Sarcoidosis is classically associated with bilateral hilar lymphadenopathy, with only 10% of children that present with sarcoidosis having a normal chest X-ray. This patient did not have any abnormal pulmonary imaging findings at time of presentation, though pulmonary disease was evident on pulmonary function testing. Sarcoidosis, while rare, can have serious complications and should be considered in the differential diagnosis of hypercalcemia, even in the setting of normal pulmonary imaging.

316 Osseous metaplasia in a paediatric kidney allograft biopsy

K Butler1

A Nayak1

M Turman2

A Snead3

N Mathews1

1OU Children’s Hospital, Oklahoma City, OK

2Phoenix Children’s Hospital, Phoenix, AZ

3AmeriPath, Oklahoma City, OK

Introduction Osseous metaplasia (OM) is the presence of heterotopic bone in any soft tissue. To our knowledge, only one case of renal osseous metaplasia has been reported in a paediatric patient, which was discovered after allograft nephrectomy. We present the first case of OM in a biopsy of a functioning paediatric kidney allograft.

Case report Our patient is an 11 year-old female who underwent deceased donor kidney transplant at age 8 for end-stage renal disease secondary to autosomal recessive polycystic kidney disease. Her course was complicated by multiple episodes of acute cellular rejection and chronic antibody mediated rejection (AMR) treated with various immunosuppressants including steroids, rituximab, IVIG, thymoglobulin, plasmapheresis and bortezomib. On presentation eGFR was decreased to 21 ml/min/1.73 M2 from a baseline of 36. A repeat biopsy of the kidney allograft demonstrated ongoing acute and chronic AMR and focal mature bone formation consistent with OM. CT showed pericapsular bony changes with extension into the renal parenchyma and radionuclear bone scan confirmed mild activity in those areas. After treatment, eGFR increased to 31 ml/min/1.73 M2 and has been stable.

Discussion The pathophysiology of OM is not well known, but is hypothesised to be induced by chronic ischaemic conditions in the setting of vascular and parenchymal scarring. In this case, the OM is likely secondary to the chronic inflammation from AMR, which created a suitable environment for the metaplasia to occur.

Bone metaplasia associated with chronic allograft nephropathy. Tousignant, K. et al. Kidney International, Volume 70, Issue 3, 407.

317 Food protein induced enterocolitis syndrome (fpies) induced methemoglobinemia

RC Calderone1,2

VA Harrison1

JL Stewart1,2

1The University of Mississippi Medical Centre, Pearl, MS

2The University of Mississippi Medical Centre, Jackson, MS

Case report A 4 week-old female with sickle cell trait was brought to the Paediatric Emergency Department for a 1 week history of vomiting and diarrhoea.

Emesis reportedly occurred within 1 hour of feeds and stools were 6–7 episodes of diarrhoea daily. Mom transitioned from breastfeeding 2 weeks prior to presentation. In the ED, a venous blood gas revealed a methemoglobin level of 14.3%. Patient admitted for evaluation.

Clinical course The patient was noted to have decreased on growth curve form 24th to 3rd percentile. A failure to thrive work-up was benign. The patient started on a hypoallergenic infant formula, with improved diarrhoea, increased weight, decrease in methemoglobin level. She was discharged home with 1 week follow-up.

At follow-up, the patient was pale with mild weight loss. A repeat methemoglobin level was 40.6%. Paitent was sent to ED where methylene blue was given and the patient admitted. Her diarrhoea continued, and stool culture was positive for campylobacter. Cow’s Milk IgE returned elevated and patient diagnosed with Food Protein-Induced Enterocolitis Syndrome. Total peripheral nutrition was given until stool losses were controlled and weight gain sustained. Methemoglobin level trended to normal without intervention. The patient discharged home on elemental formula.

Discussion In methemoglobinemia, nitrates are converted to nitrites in the GI tract. Nitrites oxidise iron is oxidised to the ferric state, forming methemoglobin, that binds oxygen poorly. Methemoglobinemia typically presents with cyanosis, irritability, tachypnea, and altered mental status can develop with higher levels. Methylene blue is the treatment.

Abstract 317 Figure 1

Methemoglobinemia: methylene blue is the treatment

318 Pott’s puffy tumour & epidural empyemas as complications of sinusitis in a teenager with jia

V Chandwani

Emory, Atlanta, GA

Background Acute bacterial sinusitis often follows a benign course with outpatient antibiotic treatment but in some cases, has the potential to progress to serious life-threatening complications including epidural abscesses and the rarely seen Pott’s Puffy Tumour.

Case A 14-year-old female with Juvenile Idiopathic Arthritis (on an immunosupressive study drug) and history of cranial reconstructive surgery for multisuture synostosis nine years prior to presentation who presented with two weeks of rhinorrhea and nasal congestion, three days of headache, fever and forehead swelling associated with vomiting. Although she was neurologic\ally intact on presentation to the paediatric emergency room, there was a high suspicion for facial abscess. Therefore, a CT scan of her head and sinuses with contrast was performed and revealed pansinusitis, bifrontal epidural empyemas, with subperiosteal abscesss concerning for Pott’s Puffy Tumour. She was admitted for IV antibiotics and taken to the OR for endoscopic sinus surgery with incision/drainage of her subperiosteal abscess. Her cultures grew out Streptococcus anginosis and she was later discharged home with a PICC line for parenteral systemic antibiotics of six weeks.

Conclusion A high clinical suspicion given this patient‘s exam, history and mutliple risk factors allowed for early recognition, prompt intervention and successful treatment of this patient‘s complicated sinusitis.

319 Interscapular pain progressing to leg weakness and ataxia: a case of non-traumatic epidural haemorrhage

A Chaphekar

S Kaneaster

University of Oklahoma Health Science Centre, Oklahoma City, OK

Introduction Non-traumatic epidural haemorrhage can be difficult to diagnose due to non-specific symptoms at presentation. Additionally, epidural haemorrhage is rare in children. We describe a case of non-traumatic epidural haemorrhage presenting as upper back pain progressing to neurologic deficits.

Case presentation A six year old healthy female, with no history of trauma, was initially treated conservatively for upper back pain. Six days later, she presented to an emergency department with worsening back pain, located between the scapulae, and new onset gait changes. She endorsed numbness and tingling of bilateral feet and had a wide based gait with bilateral hip instability. She had midline tenderness to palpation over T2-T4. Neurologic exam revealed bilateral decreased proximal lower extremity strength, numbness of the feet and lower legs, and ataxic gait.

Patient had an MRI done, which showed an epidural haemorrhage from T2-T5. The haemorrhage was displacing the spinal cord laterally and cord oedema was noted to be present. Orthopaedic spine team was consulted and patient was taken to the operating room for laminectomy. The surgeons visualised a possible arteriovenous malformation (AVM) enclosing the spinal cord. The compressing lesion was removed from the dorsal side, but was not safely accessible from the ventral aspect.

Post-operative imaging showed a stable ventral epidural haemorrhage. Concerningly over the next 48 hours, patient had progression of the numbness and significant worsening of her weakness. Angiogram revealed a vascular epidural fistula from the left supreme intercostal artery. After embolization, flow through the fistula ceased.

Post-embolization, bilateral leg strength quickly began to improve, but paresthesia persisted. She was soon transferred to a rehabilitation facility for ongoing therapy with hopes for full recovery.

Discussion Non-traumatic epidural haemorrhage, especially in a ventral location, is a rare condition in children. As with our patient, pain is often the presenting symptom followed by neurologic changes. An AVM was the aetiology of our patient’s epidural haemorrhage. Prompt laminectomy and interruption of the fistula is the treatment of choice. Early recognition of this condition is vital to neurologic recovery.

320 Massive congenital intracranial teratoma, a rare cause of bulging fontanelle and macrocepahly in term infant

M Choe

T Chatmethakul

B Merritt

H Imran

University of South Alabama, Mobile, AL

Case report Perinatal brain tumours are uncommon with a prevalence of 1.7–13.5 per 1 00 000 per live births. Intracranial teratoma comprises one third of the total reported cases, mostly diagnosed postmortally. Prognosis worsens with increase in size and decreasing gestational age at diagnosis. Advanced disease at the time of diagnosis and inability to resect these tumours makes them fatal in almost all cases. Current literature is evident of two case reports with chemotherapy treatments to date. We report a case of term female newborn with massive immature teratoma.

A female infant was born at a gestational age of 37 weeks with an uneventful prenatal and perinatal period. At birth, she was appropriate for gestational age with normal head circumference of 30.5 cm. At day 9 of life, at her first newborn follow up, she was found to have a bulging anterior fontanelle and marked increase in head circumference to 39 cm (>98% ile) which prompted hospital admission. She rapidly deteriorated with apneic episodes necessitating PICU transfer where emergency imaging of the brain showed a huge intracranial supratentorial mass with compression of the posterior fossa and brain stem. Emergent decompressive craniectomy and excision biopsy yielded histopathologic diagnosis of an immature teratoma. After complicated perioperative course with prolonged cardiac arrest requiring cardiopulmonary support, chemotherapy was then discussed. Carboplatin, Etoposide and Vinblastine were started on post op Day 5 which led to a dramatic improvement with ability to wean off cardiopulmonary support, removal of the external ventricular drain and only minimal cytopenia were experienced.

Our case emphasises the importance of recognition of rapid increment in head circumference and identification of uncommon causes of macrocephaly in an infant with a normal delivery. Although most immature teratomas are detected prenatally, some may be missed due to technical difficulties. Albeit with some neurologic deficits, only two survivors were reported in literature who benefited from chemotherapy. Neoadjuvant chemotherapy was effective in initial management and facilitated reduction in size and ultimately allowed for successful resection in both cases. We are hoping the same for this infant.

321 Cutaneous polyarteritis nodosa associated with streptococcus pyogenes infection

T Chatmethakul

N Hameed

J Chalam

B Merritt

H Custodio

University of South Alabama, Mobile, AL

Case report A 3 year old female who was treated with intramuscular penicillin for scarlet fever, was reevaluated because of suspected toxic shock syndrome. Erythematous macular rash was noted on the trunk and arms and new purpuric lesions on the legs and thumbs. Empiric therapy with vancomycin, clindamycin and ceftriaxone was started with aggressive hydration and administration of IVIG. However, despite overall improvement, she continued to have fever, arthralgia and persistently elevated inflammatory markers. Blood and throat cultures were negative. ASO titer was elevated. A skin biopsy was consistent with vasculitis (figure 1). RF, ANA, ANCA and C3 and C4 were unremarkable. With no systemic involvement, a diagnosis of cutaneous polyarteritis nodosa was made. Methylprednisolone (1 mg/kg/day) was started resulting in resolution of symptoms.

This report underlies the importance of considering nonsuppurative complications of Group A Streptococcus pyogenes (GAS) infections. While GAS infection can be self-limiting, nonsuppurative complications such cutaneous polyarteritis nodosa (cPAN) can be seen. cPAN is a vasculitis affecting medium sized vessels. In contrast to systemic PAN, cPAN is limited to the skin. However, extracutaneous symptoms may also be seen. Severe cPAN can lead to digital infarction and autoamputation. The aetiology remains unclear. Aside from GAS infections, tuberculosis, hepatitis B and noninfectious illnesses i.e. Crohn’s disease have been associated with cPAN. Establishing the diagnosis of cPAN can be difficult given its rarity, similarity of presentation, and association with infection.

Abstract 321 Figure 1

Skin biopsy was consistent with vasculitis

322 Ganglioneuromatous polyposis in a patient with irritable bowel syndrome

AW Cohen

JR Mestre

University of Alabama, Birmingham, AL

Introduction Colonic polyps and associated syndromes in the paediatric population serve as a clinically significant source of morbidity and potential mortality. Often associated with familial origins, sporadic cases can be more challenging to diagnose or determine clinical significance.

Case A 14 year old female sought care from her physician with history of chronic periumbilical abdominal pain, diarrhoea, and episodes of sweating, palpitations, and nausea. She was referred to a paediatric gastroenterologist where a clinical diagnosis of irritable bowel syndrome was made after basic laboratory testing was not suggestive of an organic cause of her symptoms. Due to length of symptoms and findings of iron deficiency anaemia, patient was scheduled for upper and lower endoscopy. Upper endoscopy was without abnormality. Colonoscopy revealed innumerable firm polyps throughout the entire colon and rectum without involvement of terminal ileum. Pathologic examination of polyps was consistent with ganglioneuromatous polyps. Genetic evaluation was negative for common mutations associated with polyposis syndromes (BMPR1A, NF1, PTEN, RET, and SMAD4). Patient continued with similar intermittent symptoms. Follow up endoscopy performed one year after was unchanged with ganglioneuromatous polyps without signs of dysplasia of sampled polyps. Continued follow up planned as well as additional evaluation by a specialist in paediatric polyposis.

Discussion This case demonstrates an abnormal and relatively rare endoscopic diagnosis in a patient who meets clinical criteria for irritable bowel syndrome. The finding of iron deficiency anaemia is a significant finding as it represents the most common presentation for polyps in paediatric patients, painless rectal bleeding. Ganglioneuromatous polyposis can be seen in association with neurofibromatosis type 1, Cowden disease, or juvenile polyposis or as an isolated finding. In the evaluation of juvenile polyps, a detailed family history is of particular importance as guidance for additional testing, intervention, or follow up. Detailed physical exam and review of systems should be performed to evaluate for coexisting symptoms and physical exam findings that could point to one polyposis syndrome or another and modify future screening and evaluation.

323 Parxoxysmal complete heart block in a child with a structurally normal heart

JE Cooper

ST McClanahan

P Glaeser

University of Alabama, Birmingham, AL

Case report A previously healthy 11 year-old African-American male presented with concern for seizure and arrhythmia. On the morning of presentation he had a syncopal episode at school. While his mother was driving him home he had two episodes of generalised body shaking, chest pain and emesis. EMS was called and noted seizure activity and periods of absent pulse and asystole on 3-lead electrocardiogram (ECG).

The patient denied illicit drugs, caffeine or energy drinks. He also denied history of travel outside the state of Alabama, sick contacts, bug bites, tick exposure, rashes, fever or ill symptoms other than headache and nausea. The mother denied family history of sudden cardiac death, drowning, single car accidents, early myocardial infarction or seizures.

Initial vitals were heart rate of 88, blood pressure 135/78, oxygen saturation of 98% on room-air and afebrile. On exam the he was ill appearing but alert with no apparent distress when not having an episode. He had a regular heart rate with 2+femoral pulses and no murmur noted. The remainder of his exam was unrevealing. His baseline ECG showed normal sinus rhythm with a heart rate in the 80–90 s (figure 1). Episodes began with vibration in his head, loss of consciousness, loss of pulses, flexion of the arms and legs, generalised body shaking for 3–4 s with subsequent return of normal vitals and consciousness. During the episodes his ECG showed complete heart block without ventricular escape rhythm (figure 1). He was found to have normal cardiac anatomy, no signs of infection or electrolyte abnormalities. He underwent emergent placement of a dual chamber pacemaker for paroxysmal complete heart block. Subsequent work-up did not reveal a pathologic aetiology.

Abstract 323 Figure 1

Electrocardiogram of normal sinus rhythm and complete atrioventricular block

324 Infectious fasciitis of the chest wall after strenuous activity

JE Cooper1

P Tomeny2

CM Pruitt1

1University of Alabama, Hoover, AL

2University of Alabama, Birmingham, AL

Case report A 15 year-old white female presented to the emergency department (ED) after 48 hours of right shoulder pain. The pain started after a week of colour guard tryouts. She described sharp, severe, constant pain located mainly in her axilla. Intramuscular ketorolac and an intramuscular steroid received the day before prior provided temporary pain relief, but her discomfort worsened by the day of presentation.

On physical examination, the patient was febrile to 38.5 degrees Celsius, with a heart rate of 95 and blood pressure of 115/58. She kept her right arm propped on a pillow, internally rotated with 30 degrees of abduction. She had exquisite tenderness to palpation in the right axilla. There was no crepitus. She refused active range of motion of the shoulder. Passive range of motion was limited to less than 90 degrees. A macular, erythematous, blanching rash along her chest, back, and groin developed during her ED course.

In the ED, the patient was administered two boluses of normal saline. Laboratory testing was significant for a white blood cell count of 15.44 103/uL, with 86% neutrophils; platelets 118 103/uL; and C-reactive protein 5.37 mg/dL. The total bilirubin was 1.1 mg/dL, alanine aminotransferase 143 U/L, and aspartate aminotransferase 142 U/L. International normalised ratio was 1.5, fibrinogen 390 mg/dL, and lactate dehydrogenase 850 U/L. Intravenous vancomycin and piperacillin-tazobactamwere initiated after obtaining blood cultures. She was subsequently hospitalised. Magnetic resonance imaging revealed extensive T2 hyperintensity in the fascial planes of the right chest wall along the axillary region, extending from the infraclavicular location and along the lateral aspect of the chest wall. The patient was diagnosed with acute bacterial fasciitis with secondary toxin-mediated compensated shock. She was successfully treated with IV antibiotics prior to transitioning to an oral regimen and did not require surgical debridement. This case highlights the need for early recognition of necrotizing infections to prevent morbidity and mortality.

325 Sclerosing cholangitis in kabuki syndrome: an unusual complication of a rare disorder

MA Corbera-Hincapie1

S Safder2

R Gonzalez-Peralta3

1University of Florida, Gainesville, FL

2Arnold Palmer Hospital, Orlando, FL

3Florida Hospital, Orlando, FL

Background Kabuki Syndrome (KS) is a rare disorder characterised by distinct facial and skeletal anomalies, cardiac defects, short stature and developmental delay. Hepatic involvement occurs only rarely. Herein, we describe a child with KS and liver dysfunction that was eventually attributed to sclerosing cholangitis.

Case report A 3-year-old female with KS, repaired VSD and hernia, was evaluated for persistent transaminitis over the preceding 1 year (discovered incidentally). Findings on physical exam were normal except facial dysmorphia consistent with KS. She had no jaundice, hepatosplenomegaly or stigmata of chronic liver disease. Initial laboratory tests showed elevated aspartate transaminase (440 U/L), alanine transaminase (281 U/L), and gamma-glutamyltransferase (408 U/L). Results of hepatitis A, B and C serology, alpha 1 antitrypsin level and Pi phenotype, antinuclear, liver-kidney-microsomal, antineutrophil cytoplasmic and tissue transglutaminase antibodies were negative; she had detectable smooth muscle antibody (titer 1:20). Abdominal sonogram did not visualise gallbladder but otherwise normal. Liver biopsy showed bile ductular proliferation with pericholangitis and minimal lobular inflammation consistent with sclerosing cholangitis. We initiated ursodeoxycholic acid (URSO; 20 mg/kg/d) with gradual improvement of transaminase levels that have remained normal for 5 years.

Discussion In a series of KS, 2%–21% of patients developed liver disease, ranging from neonatal hyperbilirubinemia, biliary atresia, hepatic fibrosis and sclerosing cholangitis. Clinical data is limited but severe hepatic disease requiring liver transplantation occurs. Our patient’s course suggests that URSO can be successfully used to treat sclerosing cholangitis in KS. Our case also highlights the importance of monitoring for liver dysfunction in patients with KS and proceeding with thorough evaluation to arrive at a specific diagnosis and institute appropriate therapy.

326 A rare case of epstein barr virus-associated hemophagocytic lymphohistiocytosis in an immunocompromised paediatric patient

CM Cox

R Herdes

M Shapiro

B Desselle

C Sandlin

LSUHSC, New Orleans, LA

Case report Kaposiform Lymphangiomatosis is a rare disorder of lymphatic vessels and is characterised by abnormal lymphatic clusters in various regions of the body. Prognosis can be poor, especially when there is pulmonary involvement. Complications can include pleural effusions, pericardial effusions, and haemorrhage. Current treatment includes chemotherapy and/or immune modulators, specifically vincristine and sirolimus. Patients taking sirolimus have increased risk of infections, including Epstein-Barr Virus (EBV) infection. EBV is a leading cause of hemophagocytic lymphohistiocytosis (HLH), which is a life-threatening disorder of the immune system.

We report a case of a 6-year-old male with a severe form of Kaposiform lymphangiomatosis who developed EBV associated HLH. The patient presented with persistent fever, intermittent rash, and worsening respiratory distress. He was started on broad spectrum antibiotic therapy for pneumonia, and his Sirolimus was continued upon admission. Due to his worsening clinical status, continued fevers, and progressive pancytopenia, haematology and oncology specialists recommended the initiation of vincristine.

The patient’s fevers continued despite these therapies, and his hospital course was complicated by an acute gastrointestinal haemorrhage. His clinical status and pancytopenia continued to deteriorate, prompting consideration of alternative diagnoses. After several days of hospitalisation, viral studies were positive for EBV. A significantly elevated ferritin level (33,000 ng/ml), in conjunction with his other clinical and laboratory findings, led to a diagnosis of EBV associated HLH. Etoposide was given on hospital day 15, but the patient further declined and died the following day.

This case highlights the invasive nature of EBV, especially in immunocompromised individuals. It is important for clinicians to recognise the risk factors associated with immune-modulating therapies and consider infectious etiologies early in the patient’s clinical course. Though rare, HLH should be considered in patients with persistent fever, pancytopenia, and worsening clinical status in the setting of certain conditions that place patients at increased risk for the disease.

327 Tetraphocomelia: a case report and literature review

J Deitrick

D Nguyen

W Sessions

M Naqvi

H Heather

Texas Tech Health Sciences Centre School of Medicine, Amarillo, TX

Case report Tetraphocomelia is a condition characterised by severe symmetrical limb reduction in utero. Several syndromes are associated with this finding: Robert’s syndrome, Grebe Syndrome, Waardenber syndrome, Holt-Oram syndrome, and Thrombocytopenia with Absent Radius syndrome. Furthermore, certain in utero exposures, such as thalidomide, alcohol, and cocaine, are also associated with this and similar musculoskeletal deformations. A 24 year old female presented for her 24 week prenatal ultrasound, which revealed incomplete limb development in the fetus. Subsequent to a caesarean section at 38 weeks and 6 days gestation, the newborn female presented with gross musculoskeletal deformities of all four limbs. She was diagnosed with tetraphocomelia but lacked features of a concomitant syndrome and relevant family or birth history associated with this anomaly. Due to the lack of ancillary symptoms, this case did not fit into any specific syndrome and was thought to be the result of a sporadic, non-hereditary limb deficiency involving all four limb buds. While the cause of tetraphocomelia is not fully understood, current literature provides insight into possible genetic and environmental factors contributing to the pathophysiology of this condition. This unique presentation of a rare congenital anomaly was possibly caused by amniotic bands or a vascular accident in utero, resulting in an isolated erroneous occurrence during the critical period of limb development.

Abstract 327 Figure 1

Unique presentation of a rare congenital anomaly was possibly caused by amniotic bands or a vascular accident in utero

328 A case of toxic epidermal necrolysis caused by cefadroxil

TT Doan

LSU Health Sciences, Shreveport, LA

Case report Toxic epidermal necrolysis (TEN) is a rare and severe adverse mucocutaneous drug reaction characterised by haemorrhagic erosions, erythema, and epidermal detachment due to immune mediated destruction of the epidermis. Drugs such as allopurinol, Trimethoprim-Sulfamethoxazole, cephalosporins, quinolones, and anticonvulsants are assumed to be the main cause of TEN in most case. Early diagnosis and aggressive treatment of TEN is important for the reduction of morbidity and mortality associated with his condition. We present a rare case of successfully recovered TEN caused by cefadroxil, a first-generation cephalosporin. A 4-year-old girl presented to the emergency room with progressively worsened blisters on trunk after the second dose of oral cefadroxil prescribed for otitis media. The lesion started as a diffuse erythematous, maculopapular rash over body, progressed into painful blisters, followed by diffuse exfoliation of the skin involving face, back, abdomen, and bilateral upper and lower extremities over the course of 3 days. Past medical history was significant for chronic otitis media. She has no known allergies and immunisation was up to date. The initial physical exam revealed a listless child with fever of 102.2F. She was tachycardic with heart rate of 160. Ophthalmologic exam showed bilateral periorbital oedema, skin blisters with haemorrhagic crusts involving both upper eyelid, but no injection of conjunctiva and no corneal involvement. She had denuded oral lesions and cracked lips with haemorrhagic crusts. There were multiple, thick bullous lesions filled with turbid fluids and epidermal detachments on face, oral mucosa, neck, abdomen, genital, arms, legs, and feet covering approximately 60% of body surface area. Nikolsky sign is positive. Initial laboratory investigations were unremarkable including negative Herpes simplex virus and Mycoplasma pneumoniae culture. She was admitted to the PICU with burn surgery assistance. Cefadroxil was discontinued immediately. She was managed with intravenous fluids, prophylactic antibiotics, IVIG, hydrotherapy and proper wound care. Her haemodynamic status was monitored continuously in the unit. She successfully recovered after 2 weeks of hospitalisation and was discharged to home in great condition.

329 Pain in the neck: a case of infantile tumoral calcinosis

B Ducote

C Jacobs

SN Epps

R Rao

M Murphy

LSUHSC, New Orleans, LA

Case report A four month old full term female presented to her paediatrician after her mother noticed that over the past 3 weeks she lost the ability to roll over and hold her chest up 45 degrees while prone. She had recently been diagnosed with torticollis and plagiocephaly and was seen by occupational therapy, but the onset of this regression prompted her paediatrician to refer her to neurology, who then admitted her for a full workup.

On exam she was difficult to console, head lag was noted, she was unable to actively turn her head, and she cried with passive movement. Initial labs of CBC, CMP, TSH, and FT3 were normal, while ESR and CRP were elevated. MRI of the brain and CT of the neck showed abnormal calcifications at the articulation of the C1/C2 vertebrae likely secondary to tumoral calcinosis. Skeletal survey showed only abnormal calcifications around the cervical vertebrae. Endocrine was consulted and completed a workup for secondary causes of tumoral calcinosis which was negative, with normal values of PTH, calcium, vitamin D, and phosphorus. Hematology-oncology was consulted to rule out neoplasm and suggested a biopsy. ENT performed a biopsy of the lesion which showed calcified material without inflammatory changes confirming our presumed diagnosis of tumoral calcinosis.

The patient was started on gabapentin for pain management per neurology recommendations. She was given NG feeds until her pain improved and she could tolerate full feeds by mouth. She was also given 5 days of steroids to decrease inflammation in the area, and the range of motion of her neck improved throughout her hospital stay. MRI after biopsy showed decreased residual calcifications around the C1/C2 vertebrae without evidence of spinal cord impingement. Over the next two months, she regained head control and better range of motion of her neck.

Tumoral calcinosis consists of calcium crystal deposits in periarticular soft tissues. It can be hereditary with hyperphosphatemia, a complication of dialysis, or a rare isolated finding which was likely what our patient had. In one previously reported case study in an infant, biopsy was found to be curative. Tumoral calcinosis is a rare diagnosis and cause of torticollis and loss of developmental milestones especially in infants.

330 Unusual osteomyelitis in an infant

M Duplantier

SN Epps

M Heffernan

A Bauchat

R Gardner

A Messer

LSUHSC, New Orleans, LA

Case report Leg pain in an infant can be difficult to detect and diagnose. Osteomyelitis is rare in a clinically well-appearing infant, though more common when Pseudomonas is the underlying aetiology. In this case, initial concern for nonaccidental trauma (NAT) in an otherwise healthy infant led to a diagnosis of osteomyelitis secondary to an unusual organism. Based on benign initial evaluation, there was potential for this patient’s infection to go undiagnosed and untreated. However, our Orthopaedic Surgery team reviewed her case and uncovered her diagnosis.

Here we describe a 10-month-old female initially diagnosed with a proximal femur buckle fracture and found to have osteomyelitis and Pseudomonas stutzeri bacteremia. She presented with refusal to move left leg as well as upper respiratory symptoms. There was no history of trauma and, on exam, she was febrile with pain on left hip manipulation.

Evaluation was significant for a subacute proximal femur buckle fracture seen on x-ray. Subsequent MRI findings were consistent with surrounding osteomyelitis. On presentation, white blood cell count was 12.32 × 103/UL, C-reactive protein was <0.5 mg/dL, which later rose to 1.4 mg/dL, and erythrocyte sedimentation rate was 37 mm/hr. Clinically, the patient was well-appearing with no external abnormalities. Blood culture later grew Pseudomonas stutzeri at 1.05 days. A respiratory viral panel revealed influenza A and adenovirus.

The Infectious Disease team recommended IV clindamycin and ceftazidime for 7 days to treat susceptible Pseudomonas as well as typical infectious etiologies. She was then transitioned to oral clindamycin and ciprofloxacin to complete a 4 week course. A Spica cast was placed; no surgical intervention was required.

The differential diagnosis for leg pain in an infant is broad, which may delay prompt diagnosis. Trauma and osteomyelitis are occasionally competing diagnoses due to similarities on initial evaluation and imaging, particularly in an otherwise well-appearing, non-ambulatory infant. This case serves to increase initial indices of suspicion for both diagnoses and demonstrates that these diagnoses are not mutually exclusive and require an interdisciplinary team of experts for best care.

331 Neonatal conjunctivitis: an unusual cause of a common complaint

MN Frascogna

L Moore

University of Mississippi Medical Centre, Jackson, MS

Case report Infectious conjunctivitis is a common presentation in neonates and young children. The disease course is often benign and can be easily treated in most cases. However, it is important to be aware of the more serious etiologies that can become lethal if not identified early and promptly treated. In this report, we present a rare case of Neisseria meningitidis as the primary cause of conjunctivitis in an otherwise healthy infant.

A 20 day old African American female born at 39 weeks via normal spontaneous vaginal delivery presented to the paediatric emergency department with a two day history of right eye swelling and discharge. The patient’s mother denied any fever, irritability, rash, or upper respiratory symptoms. Pregnancy and delivery were largely uncomplicated and screening labs were reportedly negative for any sexually transmitted infections.

In the emergency department, patient was noted to have significant mucopurulent discharge from the right eye with associated eyelid swelling and erythema. Slit Lamp and fundoscopic exam showed conjunctival injection and chemosis, but was otherwise normal. The remaining exam was unremarkable. The patient underwent a full septic workup including ocular, CSF, blood, and urine cultures and was admitted for empiric antibiotic treatment of the presumed causative organisms, Niesseria gonorrheae and Chlamydia trachomatis.

The patient was started on intravenous Ampicillin and Cefotaxime and Erythromycin ophthalmic solution. Symptoms improved after twenty-four hours of treatment and infectious workup remained negative; however, on day two of hospitalisation, ocular cultures returned positive for Neisseria meningitidis. Ampicillin was stopped and intravenous Cefotaxime was continued. The Health Department was notified per protocol, and chemoprophylaxis for contacts was not recommended due to strict confinement of the infection to the eye. The patient completed five days of intravenous antibiotics with marked improvement. She was discharged home to complete a 14 day course of oral and ophthalmic Erythromycin. At a 2 week follow up appointment with ophthalmology, symptoms had completely resolved and no ocular abnormalities were noted.

332 Salmonella in the scrotum: not your typical neonatal infection

E Friedrich

R Welliver

S DeLeon

University of Oklahoma Health Sciences Centre, Oklahoma City, OK

Case report A 17 day old male, born at 38 weeks without complications, presented with a 7 day history of irritability, and fever on day 2 of illness. On the day of fever, his paediatrician prescribed amoxicillin/clavulanic acid for unknown reasons, and he then remained afebrile until admission. Two days prior to presentation, his scrotum became swollen and tender, which prompted the family to seek further care. In the emergency room, a sepsis work up and testicular ultrasound (US) with doppler was performed. The work up yielded no evidence of systemic infection. His only abnormal lab was a C-reactive protein of 32 mg/dL. The US showed a right scrotal abscess with blood flow to both testes. Urology recommended admission for incision and drainage (I and D) of the abscess. A renal US and voiding cystourethrogram (VCUG) were obtained to evaluate for vesiculo-ureteral reflux or abnormal fistula connexion and were negative. He was started on intravenous vancomycin, ampicillin, and gentamicin. The I and D was performed with removal of 50 cc of purulent material, along with a right orchiectomy due to necrosis. The tissue culture grew a Salmonella species susceptible to ampicillin, which coverage was then narrowed to. He received 14 days of antibiotics. His mother denied exposure to raw chicken, reptiles, or sick contacts. A granulocyte dihydrorhodamine fluorescence test showed normal NADPH oxidase activity, decreasing the likelihood of chronic granulomatous disease.

Salmonella typically affects the gastrointestinal tract and is most often associated with exposure to raw chicken or reptiles. Most cases are seen in children under the age of 5. All other reported cases of Salmonella orchitis are in immunocompromised patients or occur in areas where Salmonella is an endemic infection. Extra-intestinal focal infections typically result from hematogenous spread, though anatomic urologic abnormalities can predispose to isolated genitourinary (GU) infections. Management of extra-intestinal infections includes I and D and antibiotics. This case is unique because our patient’s GU imaging was normal, blood culture was negative (although he had received antiobiotics prior to admission), had no history of exposure, and had no risk factors for immunodeficiency other than age.

333 Case report cyclic neutropenia and associated amyloidosis

KM Galipp

C Ilonze

K Shah

A Rughani

S Travis

A Nayak

WF Kern

K Wierenga

WH Meyer

University of Oklahoma Health Sciences Centre, Oklahoma City, OK

Study purpose Cyclic neutropenia is a rare hereditary disorder, characterised by recurrent neutropenia, cycling at about 3 week intervals, with variable associated symptoms including oral ulcers and fever. There are 4 reported cases of cyclic neutropenia associated with chronic inflammation leading to development of reactive AA amyloidosis. One patient also presented with amyloid goitre. We report a new case of cyclic neutropenia with associated renal and thyroid amyloid.

Method (case report) A 12-year-old female presented with a 1 month history of thyromegaly, and recurrent aphthous ulcers associated with fevers. Laboratory workup showed severe neutropenia, anaemia, azotemia, and abnormal thyroid function, with an absolute neutrophil count – 0/µL, haemoglobin – 9.0 g/dL, serum creatinine – 1.89 mg/dL, and uric acid – 9.0 mg/dL. Thyroid stimulating hormone was elevated – 12.5 µIU/mL, and normal free T4. Urinalysis showed 2+protein, 2+blood, and 5–10 urine red blood cells/hpf. Chest radiograph showed mild narrowing of the trachea from thyroid compression. Bone marrow biopsy showed a hypocellular marrow, with tri-lineage hematopoiesis, left shifted myeloid maturation with very rare mature neutrophils. Both renal biopsy and thyroid fine needle aspiration revealed abundant amyloid. Of note, her father had AA amyloidosis, resulting in end-stage renal disease (ESRD) requiring hemodialysis, and recurrent aphthous ulcers. The family history suggested a familial predisposition. Genetic testing revealed a pathogenic ELANE c.358 A>T gene mutation with autosomal dominant inheritance confirming the diagnosis of cyclic neutropenia. We treated our patient with daily granulocyte colony stimulating factor to reduce the burden of chronic inflammation induced by cyclic neutropenia, and to preserve renal and other end organ function affected by further amyloid deposition.

Summary of results Proband with ELANE gene mutation positive cyclic neutropenia, amyloidosis of thyroid and kidney, with a positive paternal history of AA amyloidosis resulting in ESRD.

Conclusions Cyclic neutropenia may result in chronic inflammatory states leading to secondary amyloidosis.

334 Alveolar capillary dysplasia presenting as refractory pulmonary hypertension

J Gallois

J Patrick

J Surcouf

LSUHSC School of Medicine, New Orleans, LA

Case report Alveolar capillary dysplasia with misalignment of the pulmonary veins (ACDMPV) is a developmental anomaly of the pulmonary vasculature that takes place in the late canalicular to early saccular phase of lung development. This maldevelopment results in the failure of the formation of the normal air-blood diffusion barrier. It is almost universally fatal. 90% of those effected are term gestation, 60% present with cyanosis within the first 48 hours of birth, and 80% of cases are associated with other congenital malformations. Ninety percent of cases are associated with a genetic mutation on the FOXF1 gene on chromosome 16, and 90% of cases are de novo mutations. Diagnosis is confirmed by lung biopsy or autopsy, where pathologic features include a paucity of alveolar capillaries, widened alveolar septae, muscularization of pulmonary arterioles, and usually misalignment of pulmonary veins in the bronchovascular bundle. No current supportive therapies have been shown to change mortality.

We present a term female infant born to a 33 year old mother with an unremarkable prenatal course. The infant was initially stable, then at six hours of life she was transferred to the neonatal intensive care unit for cyanosis and hypoxia with oxygen saturations in the 50’s. She was ultimately transferred to a tertiary care facility for concerns of a congenital heart defect versus persistent pulmonary hypertension (PPHN). The echocardiogram at admit revealed a structurally normal heart and evidence of PPHN. Other congenital malformations were not found. Although she initially stabilised with medical management including inhaled nitric oxide, her PPHN progressed despite maximal support. She significantly decompensated with a prolonged code while being placed on extracorporeal membranous oxygenation (ECMO) on day of life 2, requiring simultaneous initiation of therapeutic hypothermia. After two failed weans off ECMO, a lung biopsy was performed on day of life 25 which revealed ACDMPV. With the family at the bedside, support was withdrawn.

The incidence of ACDMPV is rare, but is increasingly recognised. Early diagnosis may prevent unnecessary therapies and prolongation of treatment. Neonates with refractory PPHN, especially if associated with anomalies, should prompt evaluation for ACDMPV.

335 Actinomyces epidural abscess: a virtually unheard of process in the virtual age

AL Gibson

S Liu

M Naifeh

OU Children’s, Edmond, OK

Case report Actinomyces has been termed the ‘great pretender’ in many case reports due to its ability to invade various parts of the body causing abscesses. Actinomyces epidural abscesses are virtually unheard of in the paediatric population, and only sparse literature exists in the adult population, with often only a few spinal segments involved. As a slow growing anaerobic gram positive bacteria, it typically spreads through the body via sinus tract formation.

Here we report a 3 year old female with multiple congenital anomalies including scoliosis, repaired tethered cord, history of syrinx and hydrocephalus at birth with a ventriculoperitoneal (VP) shunt, who on MRI was found to have a highly invasive epidural abscess extending from the cervical spine to the sacrum and into the surrounding scalenes and psoas muscles. The patient initially presented with clear signs of meningitis including fever, lethargy and decreased activity and was too ill to undergo imaging on admission. Cerebrospinal fluid from her VP shunt did not reveal an infectious cause for her symptoms, so a lumbar puncture was performed which collected purulent fluid from her epidural abscess. This fluid grew actinomyces turicensis and actinomyces europaeus. After clinical improvement, spinal imaging showed a dorsal to dural sinus tract at L4 which is likely the point of initial bacterial invasion.

After thorough literature review, this is the largest epidural abscess that has been reported. While the liklelihood of treating another patient with the same complex medical history is low, this case underscores the importance of prompt and repeat imaging in any patient with symptoms of meningitis who does not show clinical evidence of improvement on broad spectrum antibiotics as Actinomyces can present this way.

336 A novel de novo heterozygous mutation in the pcdh17 gene with microcephaly, developmental delay, and acute lymphoblastic leukaemia

KM Gleditsch

R Gardner

Y Lacassie

Louisiana State University Health Science Centre, New Orleans, LA

Case report We present an 11 year old girl evaluated by genetics at birth due to microcephaly. Chromosomes and a microarray were normal. At the age of 3 she developed pre-B-cell acute lymphoblastic leukaemia (ALL). She completed treatment in 2012 and has been doing well in the interim. During and after treatment she exhibited significant developmental delay and neurocognitive deficits. At age 11 her height and weight were at or below the 5th centile and head circumference was well below the 2nd centile (approximately 6 standard deviations below the mean and corresponding to the 50th centile for a 9-month-old girl). Bone age was appropriate. She had a distinctive triangular face with micrognathia and a pointed nose resembling a Seckel-like syndrome. The patient also had clinodactyly of the fourth toes, zygodactylous triradius involving the 2nd and 3rd left toes, tendency to Sydney line in the