Clinical Vignette Abstracts Thursday October 21, 2010

1 ESOPHAGEAL INFLAMMATION WITH EOSINOPHILIC INFILTRATION IN CHRONIC GRANULOMATOUS DISEASE B. Kessler, Pediatrics, Good Samaritan Hospital Medical Center, West Islip, NY; S. Schuval, Pediatrics, Schneider Children's Hospital, New Hyde Park, NY Chronic granulomatous disease(CGD)is a primary immunodeficiency disorder of phagocytic oxidative metabolism. Gastrointestinal involvement is common in CGD especially if the inheritance is x‐linked. Colonic disease is the most common area of involvement. Esophageal disease including stricture formation and dymotility, while reported is less common. Few studies have described the histopathology in CGD‐associated esophageal disease but nonspecific inflammation or granuloma formation has beem seen. We report a 16 month old male diagnosed with X‐linked CGD at age 1 month who presented at 16 months with rectal bleeding, abdominal pain and anemia. There was no previous history of vomiting,regurgitation or feeding difficulties. Past history was significant for rectal bleeding at age 10 days while receiving cow's milk‐ based formula and breast milk. The bleeding resolved with substitution of a hydrolyzed cow's milk‐based formula, although breastfeeding without maternal milk avoidance continued. Colonoscopy and upper endoscopy were performed at 16 months. Biopsies of the colon revealed inflammation only of the right side i.e. acute cryptitis and colitis without granuloma. Biopsies of the duodenum and stomach were normal. Esophageal biopsies revealed active esophagitis with 20 eosinophils per HPF and neutrophilic infiltrate without granuloma. GMS and PAS stains were negative for bacteria or fungi. Endoscopic appearance of the distal esophagus demonstrated scattered white plaques. Testing for serum specific IgE antibodies only demonstrated a class II response to beta lactoglobulin. A CBC did not reveal peripheral eosinophilia. Prick puncture skin tests to milk proteins were negative. This patient was not on acid suppression at any time prior to the endoscopic procedures. In conclusion, we report the first case of esophagitis with eosinophilic infiltration in a child with CGD. Whether the findings were specific to esophageal involvement secondary to CGD, gastroesophageal reflux disease or eosinophilic esophagitis is open to speculation. 2 EOSINOPHILIC ESOPHAGITIS (EE) IN A PEDIATRIC PATIENT WITH HERPES SIMPLEX VIRUS ESOPHAGITIS (HSVE). A CAUSE OR A CONSEQUENCE? THE DILEMMA CONTINUES. M. Mavers, A. Shah, S. Kumar, B.A. Becker, A.K. Jain, , Saint Louis University, St Louis, MO; B.A. Becker, A.K. Jain, Pediatrics, SSM Cardinal Glennon Children's Medical Center, St Louis, MO; Background: HSV is a rare, but well described cause of esophagitis. Its association with EE is not clear. We present a patient with HSVE who was found to have EE. Case report: A 12 year‐old previously healthy male presented with fever, emesis, dysphagia, abdominal and ‘burning’ chest pain. Physical exam was benign, except for epigastric tenderness. He had a high sedimentation rate (44 mm/hour) and mild anemia (Hgb 11.5 gm/dL). Endoscopy showed ulceration, erythema and friability of the esophagus with inflammation and basal cell hyperplasia on histology and a normal stomach and duodenum. Immunohistochemical staining and culture showed HSV in proximal and distal esophagus. Immunological workup revealed an elevated white cell count (19.2K/mm3), normal total and subtype immunoglobulin titers, complement activity, NK cell function and no peripheral eosinophilia. Infectious and parasitic workup including ELISA for HIV was negative. IgE immunocap assay was negative for an extensive panel of food allergens. Flow cytometry showed CD4 lymphocytes at 33%, a CD4 count of 786/mm3 with a CD4/CD8 ratio at 2.36. Adequate antibody titers to diphtheria, tetanus and Hib were documented with a poor IgG response to 4 of 7 Prevnar‐7 serotypes. Lymphocyte proliferation to phytohemagglutinin, concanavalin A and pokeweed was decreased but normal for candida and tetanus. He was treated with esomeprazole, sucralfate and acyclovir. Patient had recurring emesis and a repeat endoscopy two months later revealed esophageal furrowing and erythema with greater than 40 eosinophils/HPF on histology. The patient was started on oral fluticasone with an improvement in symptoms. Conclusion: The development of EE following HSVE was unusual. Whether this patient had a primary immunodeficiency or immunomodulation secondary to HSV is debatable. One could envision viral infection(s) predisposing to EE. Physicians should have a high level of suspicion for EE in HSVE patients with persisting symptoms. 3 FATAL ATTRACTION: AWARENESS OF THE MANAGEMENT OF INGESTED MULTIPLE MAGNETS OR SINGLE MAGNET AND A METALLIC OBJECT. D. Mirchandani, A. Aybar, S. Blanchard, A. Malkani, Pediatric Gastroenterology, University of Maryland, Baltimore, MD; Most ingested gastric foreign bodies will pass spontaneously from the stomach. However, if these objects are more than 5 cm long, corrosive in nature, sharp or magnetic they should be removed immediately. The purpose of this case report is to alert clinical providers to the detrimental effects on the gastrointestinal tract when more than one ingested magnet or magnetic object is not removed urgently. If not removed, pressure necrosis resulting from attraction between the magnetic objects across two bowel loops leads to perforation, hemorrhage, volvulus or even death. We describe 3 asymptomatic pediatric patients with known ingestion of multiple magnets. All had successful endoscopic retrieval avoiding possible complications. Case 1: 3 year old swallowed 16 plastic coated cylindrical magnets (resembling mint candy), part of a magnetic building set. Radiograph revealed 14 magnets arranged in a circle in the stomach and 2 in the small bowel. The gastric magnets were removed endoscopically with a snare. Other 2 magnets passed uneventfully. Case 2: 3 year old swallowed 2 magnetic toy pieces. X‐ray confirmed gastric and proximal duodenal location. Successful retrieval of both was accomplished with a Roth Net. Case 3: 9 year old swallowed 2 dumbbell toy magnets. X‐ray revealed gastric location. At endoscopy, the magnets were separated from retained food as they attracted to the biopsy forceps, and removed with a Roth Net. Conclusions: Public and physicians must be made aware of the urgency of managing patients who have ingested multiple magnets or a single magnet and magnetic object to prevent significant morbidity and mortality. In unknown ingestions, when the radiologist determines that more than one metallic object is present radiographically, the clinician should be contacted to verify if one of them is magnetic. If there is a suspicion of magnets accessible with an endoscope, they should be removed urgently, even if asymptomatic. If inaccessible endoscopically, the patient must be observed vigilantly for possible complications. 4 A RARE CASE OF CONCURRENT EOSINOPHILIC ESOPHAGITIS (EOE) AND EOSINOPHILIC GASTRITIS (EOG). M. Le‐Carlson, J.A. Kerner, Pediatric Gastroenterology, Lucile Packard Children's Hospital at Stanford University, Palo Alto, CA; R. Pai, Pathology, Stanford Medical Center, Palo Alto, CA; BACKGROUND: Eosinophilic gastrointestinal diseases are characterized by the infiltration of eosinophils into the gastrointestinal tract mucosa. EoE is the most common form with EoG being much rarer. In general, EoE and EoG are distinct entities that typically are not associated. CASE: A 13 year‐old male with asthma and multiple food allergies presented in 1978 with poor growth, hypoproteinemia and anemia. Small bowel biopsy (Crosby capsule) was negative with no esophageal samples collected at that time. Chromium‐labeled albumin was intravenously injected into the patient and levels measured in the stool. Labeled albumin levels were normal when the patient was not eating but significantly elevated when patient was placed on a normal diet. Patient initially improved with diet restriction, hypoallergenic formula and cromolyn. At 23 years of age, the patient developed dysphagia and a recurrence of hypoproteinemia. Repeat endoscopy was significant for an esophageal ulcer with otherwise normal esophageal and jejunal biopsies. At 43 years of age, the patient underwent a third endoscopy due to ongoing dyspaghia. Endoscopy findings included an esophageal stricture, narrowed antrum and severe eosinophilic infiltration of both the esophagus and stomach consistent with EoE and EoG. Patient was initially treated with oral prednisone alone. Shortly thereafter patient underwent a final endoscopy for a flare and was found to have a feline esophagus and partial gastric outlet obstruction. Patient was then treated with prednisone and swallowed fluticasone with an improvement in symptoms. CONCLUSION: Presented here is an exceedingly rare case of concurrent EoE and EoG. It is important for the clinician to be aware that although typically distinct entities, in rare instances they can occur concurrently and that multiple biopsies may be needed to make the correct diagnosis. 5 PERCUTANEOUS GASTROJEJUNOSTOMY (PGJ) TUBE FEEDINGS IN INFANTS WITH PRUNE BELLY SYNDROME (PBS) V.M. Pineiro‐Carrero, R. Shonce, R. Caicedo, J. Dranove, V. Gopalareddy, Pediatrics, Levine Children's Hospital, Charlotte, NC; Infants with chronic renal disease (CRD) are at risk for malnutrition. These infants often require peritoneal dialysis (PD) and need enteral feedings to achieve their growth potential. We report our experience of PGJ feedings in 2 infants with PBS and CRD. Case 1: JC is a 15 m/o male with ESRD and PBS. PD was initiated at 2 d/o and a PEG tube was placed for nutritional support at 25 d/o. After 5 m/o he developed persistent vomiting and poor weight gain. A fundoplication was considered high risk due to the need for changing PD to hemodialysis post op. Therefore the PEG was converted to a PEG/GJ tube. PD was resumed within 48 hours and he tolerated the PGJ feedings. The wt/ht increased from the 10th %tile to the 75th %tile. The patient continues on PD awaiting a kidney transplant. Case 2: MG is a term male diagnosed with PBS at birth. His course was complicated by CRD and failure to thrive. Despite oral nutritional supplementation his weight decreased below the 3rd %tile by 4 m/o. A surgical G‐tube was placed at 5 months. Postop, he developed significant respiratory distress and an aspiration pneumonia. An esophageal pH study was positive for severe GE reflux. Due to the high surgical risk of a fundoplication he underwent placement of a PGJ feeding tube. The patient tolerated the jejunal feedings well and demonstrated excellent improvement in his growth and development. Within months, his weight doubled, increasing from less than the 3rd %tile to the 99th %tile. Discussion: Decreased stature and poor nutritional status are common in infants with CRD. PD is a safe and effective treatment for renal failure in these infants. G‐tube feedings are often used to supplement oral intake, nevertheless vomiting and feeding intolerance are a common problem in infants with PBS and CRD. We report two cases with CRD and PBS in whom long term GJ tube feedings resulted in optimal weight gain. A PGJ is a safe and effective alternative to surgical gastrostomy placement and Nissen fundoplication in patients with CRD, including patients undergoing PD. 6 SEVERE INTRAGASTRIC GRANULATION TISSUE CAUSING BLEEDING IN AN 8 YEAR OLD GIRL RECEIVING GT FEEDINGS E. Maksimak, Medical Student, Philadelphia College of Osteopathic Medicine, Philadelphia, PA; M. Maksimak, W. Cochran, Pediatric Gastroenterology and Nutrition, Geisinger Clinic ‐ Janet Weis Children's Hospital, Danville, PA; SL is an 8 year old girl with a past history of severe developmental delay secondary to a congenital CMV infection, a feeding disorder and refractory GE reflux managed with gastrostomy feeds and a surgical fundoplication shortly after her 7th birthday. Approximately 4 months later SL presented with upper gastrointestinal bleeding and a drop in her hemoglobin from 12 to 9 g/dl. Upper endoscopy revealed the presence of severe intragastric granulation tissue around the gastrostomy button site. The granulation tissue was removed surgically and the gastrostomy site was relocated. However, GI bleeding symptoms recurred at this new site within 2 months requiring endoscopic removal of the granulation tissue with snare electrocautery. The patient has required repeated transfusions because of a drop of her hemoglobin to 7 g/dl. The patient also required repeated endoscopic procedures every several months to remove the bleeding granulation tissue. Intragastric injection of the granulation tissue site with steroids has prolonged the intervals between endoscopic treatments. Multiple pre and post treatment endoscopic pictures will be presented. 7 MAGNET INGESTIONS CAN BE MORE HAZARDOUS THAN WE THINK M. Altaf, S.N. Raju, J. Grunow, Pediatric Gastroenterology, The University Of Oklahoma, Oklahoma City, OK; Background: Literature suggests multiple magnet ingestions can lead to ischemia, necrosis, perforation and volvulus due to attraction across the intestinal walls. We report extensive ulceration with single magnets and perforation due to multiple magnets on one side of the intestinal wall possibly due to extensive ulceration. Materials and Methods: Records of foreign body ingestions were retrospectively examined over an 8‐year period. We identified 71 children between ages of 0‐18 years with foreign body ingestions. Subjects were sorted by type of ingestion. Time duration between ingestion / onset of symptoms and endoscopy, and medical or surgical complications were noted. Endoscopy reports were reviewed for extent of mucosal / tissue damage observed during endoscopy. Tissue damage was classified as 0 – no mucosal damage, 1 – erythema or erosion, 2 – ulceration or perforation. Results: 3 of the 71 children had already passed the foreign body beyond the pylorus by the time of endoscopy. 8 had battery ingestions (6 had lesion score 0, 2 had score 1), 35 had coin ingestions (28 had lesion score 0, 7 had score 1), 3 had magnet ingestions (all 3 had lesion score 2, 1 with perforation), other ingestions were 22 (21 with lesion score 0, 1 had score 1). The case of perforation was a stack of 4 magnets, on the same side of the stomach, with no other intestinal wall perforated ruling out attraction across the intestinal wall. The average time to endoscopy post ingestion was 34 hours (+/‐ 34.12) in magnet ingestions, 54.14 hours (+/‐ 69.20) in battery ingestions, 2200.54 hours (+/‐ 9908.10) in coin ingestions and 8.08 hours (+/‐ 7.91) in other ingestions. Conclusions: Newer toys with much smaller but powerful magnets can be easily ingested by an adventurous child. Previous reports suggest only observation for single magnet ingestions, but our case series suggest that extensive damage can be caused by an impacted single magnet in short time period as compared to other ingestions and may lead to perforation. Clinicians should be vigilant and include single magnet in their algorithm to remove from the stomach urgently. 8 EXPERIENCE OF ENDOSCOPIC GUIDED PLACEMENT OF MIC‐KEY GASTROSTOMY TUBE IN CHILDREN. M. Yuwono, M. Pickens, D. Lustig, Ped GI, MBCH, Tacoma, WA; R. Holland, S. Acierno, Ped Surgery, MBCH, Tacoma, WA; Introduction: Mic‐key gastrostomy tubes are convenient and preferable for conventional gastrostomy feeding tubes for pediatric patients. They are easier to use and the absence of long tubes allows for greater mobility of the patients. Gastrostomy tubes may be placed either surgically, endoscopically, or with radiographic guidance. However, Mic‐key GT tubes are usually placed through an existing gastrocutaneous stoma. We report 2 cases of Mic‐key GT tube placement endoscopically as an initial placement using Kimberly Clark Introducer Kit. No studies have been reported assessing this method in children. Methods: The patients required gastrostomy tubes for support of nutritional status and growth: neurologic impairment (n=1), feeding difficulty (n=1). They were FTT as well. The patients received most of their nutritional needs, as well as medications via the tube for an extended period of time. The Mic‐key gastrostomy tube was placed endoscopically under general anesthesia in OR vs Propofol sedation in endoscopy suite. Prophylactic intravenous antibiotics were given prior to the gastrostomy tube placement. Results: Patients mean age was 3 years. 1 female and 1 male. Direct insertion was easy, quick and no complications were encountered during placement. 1 patient, however, had a repeat placement the following day when the new tube came out after the balloon was deflated accidentally. Replacement was quick and easy as well. The duration of the tube placement either in the OR or endoscopy suite were similar. Recovery time is more rapid, however, if the sedation is done in the endoscopy suite. Summary: 1. This study provides new evidence that “Mic‐key” low profile gastrostomy tube can be placed as an initial feeding tube in children endoscopically. 2. Complications related to tube placement are not significantly different than other methods of placement. 3. Direct visualization of gastrostomy tube placement endoscopically can avoid bleeding, penetration of the bowel and partial obstruction related to the location. It is an ideal placement for any non‐surgical candidates. 9 EFFICIENCY AND COST‐EFFECTIVENESS OF GASTROCUTANEOUS FISTULA CLOSURE ENDOSCOPICALLY VS SURGICALLY. M. Yuwono, D. Lustig, T. Matthew, M. Pickens, Ped GI, MBCH, Tacoma, WA; R. Holland, Ped Surgery, MBCH, Tacoma, WA; INTRODUCTION : Persistent leakage after removal of a gastrostomy tube due to flow of gastric juice through the fistula may hamper healing. Various conservative surgical and endoscopic closures have been described in the literature. Little has been written about the cost‐effectiveness of various therapies in pediatric patients. We describe a method of endoscopic closure of gastrocutaneous fistula using an Olympus endo‐clip that is safe, quick and efficient. We compare the cost both endoscopically in ambulatory settings vs surgically in OR. METHODS : We identified 10 patients who had their gastrostomy tube removed when enteral access was no longer needed. A detailed explanation of fistula closing, both endoscopically and surgically was provided and choice was given to the family. Primary outcomes measure included complications, duration of the procedure and hospital charges. RESULTS : Of 10 candidates, 6 chose endoscopic closure and 4 had surgical closure. 1 patient had to go for repeat endoscopic and 1 repeat surgical closure after failing endoscopic closure. Endoscopic closure was accomplished using an Olympus endo‐clip in the endoscopy suite and Propofol sedation. Surgical closure, in a layer fashion, was carried out by the surgeon in the OR with general anesthesia and intubation. In a comparison, the Propofol sedation in the ambulatory setting showed quicker induction time (within 60 seconds), quicker procedure time (<5 minutes), and more rapid sedation recovery period (<20 minutes) when compared to tradition OR surgical closure. Global cost was around $ 5000 for gastrocutaneous fistula closure in the endoscopy suite and $ 15000 in the OR. CONCLUSIONS : 1.Endoscopic closure of gastrocutaneous fistula closure under Propofol sedation using Olympus endo‐clip is easy, safe to perform, and spared the patients the potential complications of surgery under general anesthesia and possible wound infections. 2.Endoscopic gastrocutaenous fistula closure is quick, allows a shorter full recovery time and provides a cost effective alternative to surgical closure. 10 A POSSIBLE ROLE FOR IMMUNOTHERAPY IN EOSINOPHILIC ESOPHAGITIS C. Hayes, V.A. Mukkada, Pediatric Gastroenterology, Nutrition, and Liver Diseases, Hasbro Children's Hospital/Brown University, Providence, RI; R. Klein, Pediatric Allergy and Asthma Center, Hasbro Children's Hospital/Brown University, Providence, RI; R. Klein, V.A. Mukkada, Pediatric Food Allergy Program, Hasbro Children's Hospital/Brown University, Providence, RI; Eosinophilic esophagitis (EoE) is characterized by esophageal symptoms with esophageal eosinophilia once other causes of eosinophilia such as GERD have been ruled out. There is significant morbidity with this condition, including social issues and the long‐term development of strictures. While swallowed corticosteroids can be effective for acute flares, evidence for maintenance therapy is limited. Investigations have primarily focused on food allergens in EoE pathogenesis, but subsequent animal models and clinical series suggest that aeroallergens can drive disease activity. Based on this practitioners generally prescribe allergen avoidance. Immunotherapy, which induces immunologic tolerance by gradually increased doses of an allergen, may also play a therapeutic role in EoE. We present a 13 year old boy who developed abdominal pain after stopping maintenance immunotherapy for seasonal allergies. At the time of presentation, he had been on proton pump inhibitors for 6 weeks. Subsequent upper endoscopy revealed histologic evidence of EoE. On follow‐up, skin prick testing failed to identify any food allergens, and discontinuation of allergy shots was identified as a potential inciting event for his symptoms. After discussing this with his family, we decided to withhold corticosteroids in lieu of restarting the immunotherapy. The patient noted resolution of symptoms shortly after immunotherapy resumed. Repeat upper endoscopy after 8 weeks revealed complete resolution of eosinophilic infiltrate. Notably, the only treatment introduced after the first endoscopy was resumption of allergy shots, and he never received any corticosteroids in this time period. The possibility that immunotherapy induced remission of EoE in our patient emphasizes the link between aeroallergens and disease activity. This suggests a new modality for management of this emerging disease. 11 TWO CASES OF H. PYLORI NEGATIVE PEPTIC ULCER DISEASE LEADING TO GASTRIC OUTLET OBSTRUCTION R.A. Patel, S.S. Baker, R.D. Baker, Digestive Diseases & Nutrition Center , SUNY at Buffalo, Buffalo, NY; W.N. Sayej, Digestive Diseases, Hepatology & Nutrition, Connecticut Children's Medical Center, Hartford, CT; Introduction: Gastric outlet obstruction (GOO) causes abdominal pain and vomiting. In children, GOO is rare and the most common cause is idiopathic hypertrophic pyloric stenosis (IHPS). Prior to the introduction of proton pump inhibitors (PPI) and H2 blockers (H2B), peptic ulcer disease (PUD) was recognized as a common cause of GOO. We present two cases of PUD leading to GOO. Case 1: An 11 year old male presented with a two year history of weight loss, vomiting and abdominal pain. The diagnosis of pyloric channel narrowing and peptic ulcer was made by upper endoscopy (EGD) and confirmed by an upper gastrointestinal series (UGI). Stool antigen for H. pylori and CLO testing were negative on three separate occasions. Despite negative testing, the patient was treated for H. pylori and started on a daily PPI. In addition, he was treated with five days of steroids with no clinical improvement. The patient underwent a Jaboulay pyloroplasty as definitive treatment. Two months after surgery the patient was symptom free and had gained weight. Case 2: A 15 year old female presented with a two month history of vomiting and weight loss. She was diagnosed with a pyloric channel ulcer and obstruction by UGI and confirmed by EGD. The patient was negative for H. pylori by serology and CLO testing on two separate occasions. Despite negative testing, the patient was treated with two courses of H. pylori eradication therapy, and placed on daily H2B. The patient underwent therapeutic pyloric dilation on six separate occasions prior to undergoing a Billroth I gastrectomy with vagal nerve preservation as definitive treatment. Two months following surgery the patient had no symptoms of GOO and gained weight, however, she now has symptoms of reflux. Conclusion: Gastric outlet obstruction secondary to peptic ulcer disease can occur in the absence of H. pylori infection. These cases suggest that surgical management after failing medical treatment can provide definitive therapy. 12 ARGON PLASMA COAGULATION IN TREATMENT OF ESOPHAGEAL SQUAMOUS PAPILLOMA M. Tempel, M. Lowenheim, A. Chawla, , Stony Brook University, Stony Brook, NY; Esophageal squamous papillomas (ESP) are benign epithelial lesions rarely reported in children. Inflammatory response to gastroesophageal reflux, mucosal irritants and minor trauma have been suggested as underlying etiology. Others have suggested the role of human papilloma virus infection although studies have not consistently identified the virus in these cases. We report of a 7 year old boy with 3 month history of abdominal pain and frequent vomiting. Blood work was revealing for antibodies for helicobacter pylori (H.pylori). Patient was treated with triple therapy with resolution of his symptoms. Repeat stool H.pylori was positive and esophagogastro‐ duodenoscopy (EGD) was performed. A warty, polypoid mass 1 cm by 1 cm was present in mid esophagus. Biopsy of the lesion confirmed benign ESP. Viral culture was negative. Gastric biopsy confirmed H.pylori gastritis. Pt was treated for H.Pylori. Repeat EGD 3 months later showed eradication of H.Pylori but esophageal lesion persisted unchanged. EGD 8 months later showed persisting esophageal lesion with 3 small adjacent lesions. Hot forceps biopsy was taken and Argon Plasma Coagulation (APC) was used on the remaining lesions. EGD 5 month later revealed almost complete eradication of the lesion with <1mm small vesicular lesion in mid esophagus. Most cases of esophageal papillomas have been either treated conservatively or endoscopically. Forcep removal and thermal ablation have been used with inconsistent results. Thermal ablation also carries a risk of perforation as the depth of tissue injury is unpredictable. In our case forcep removal of the lesion did not eradicate the lesion. Argon therapy led to almost complete eradication of the ESP. Our case suggests that APC may be the preferable way to treat ESP. With APC the approximate depth ranges from 1 to 3 mm depending on power setting, argon gas flow and can be delivered to a predictable depth. To the best of our knowledge, argon plasma coagulation has not been used for the treatment of squamous esophageal papilloma in the past. 13 15‐YEAR‐OLD GIRL WITH CHRONIC ATROPHIC GASTRITIS AND ECL CELL HYPERPLASIA A. Russell, M.J. Rosen, Pediatrics, Vanderbilt School of Medicine, Nashville, TN; H. Correa, Pathology, Vanderbilt School of Medicine, Nashville, TN; A 15‐year‐old girl presented with 8 months of epigastric post‐prandial abdominal pain unresponsive to esomeprazole. Maternal history was notable for IgA nephropathy and B12 deficiency. Physical exam was unremarkable. CBC, metabolic panel and celiac serology were normal. Endoscopy revealed nodules in body of the stomach. Pathology showed multifocal chronic atrophic gastritis (CAG), enterochromaffin‐like cell (ECL) hyperplasia, and focal intestinal metaplasia. Synaptophysin staining showed linear and nodular ECL hyperplasia. Gastrin staining demonstrated an increase in the number of antral G cells. H. pylori staining and biopsy urease testing were negative. ECL hyperplasia may be due to high gastrin levels associated with CAG (H. pylori‐associated or autoimmune), chronic proton‐pump inhibitor use, or gastrin‐secreting tumors (Zolinger Ellison syndrome) with or without multiple endocrine neoplasia (MEN) 1. Evaluation off acid suppression revealed a gastric pH of 6.6, elevated fasting serum gastrin (272 pg/ml), and negative secretin stimulation test, suggesting her elevated gastrin may be in response to chronic achlorhydria and not a gastrin‐secreting tumor. PTH and thyroid function tests obtained to evaluate for MEN 1 were normal. A B12 level was low normal (365 pg/ml) and anti‐parietal cell and anti‐intrinsic factor antibodies were negative. CAG leads to parietal cell destruction and subsequent achlorhydria. Antral G‐cells respond by increasing gastrin secretion, stimulating ECL proliferation. This feedback response can lead to diffuse micro‐nodular ECL cell hyperplasia, which may become type 1 neuroendocrine tumors (NETs). Type 1 NETs typically follow a benign course. Approaches to treatment range from annual endoscopy and B12 levels to antrectomy to somatostatin analogue therapy. CAG typically presents in the 6th‐7th decade. Gastric atrophy in children has been reported but mostly in the setting of H. pylori infection. To our knowledge, this is the first reported case of a child with CAG in the absence of H. pylori infection and associated NET. 14 DYSKERATOSIS CONGENITA AND ESOPHAGEAL WEBS: A CONNECTION THAT’S EASY TO SWALLOW S. Ali, N. Alkhouri, L. Mahajan, , Cleveland Clinic, Cleveland, OH; Dyskeratosis congenita (DC) is a rare genetic syndrome which causes bone marrow failure. The main manifestations include nail dystrophy, leukoplakia in the oral mucosa, dysphagia, along with thrombocytopenia and leukopenia. The disease can be inherited in an autosomal recessive, autosomal dominant, or x‐linked recessive form. Gastrointestinal manifestations of DC include esophageal webs, along with mucocutaneous manifestations such as oral leukoplakia and diarrhea. A 12‐year‐old female presented with a history of dysphagia. She had a history of difficulty swallowing since the age of 4 years and she received an esophageal dilatation at the age of 6 years. Lab values showed a WBC count of 3.66 k/uL, platelets of 108 k/uL, absolute neutrophil count of 690/ uL, neutrophils of 18.9%, normal liver enzymes and electrolytes, TSH of 266 uU/mL and free T4 of 0.3 ng/dL . A barium swallow showed an esophageal web at the level of C6‐C7. An endoscopy was then performed which showed a web at 12 cm from the incisors. Dilation was done by pneumatic balloon dilator with rupture of the web. A second web was noted at 15cm past the incisors (3cm past the initial web) and it was also ruptured. Two months later, the patient returned with recurring dysphagia, and on repeat endoscopy was found to have partial recurrence of the previously dilated web. The web was dilated a second time with a Savory dilator. Upon further questioning we found out that her brother had similar history with hypothyroidism, pancytopenia and difficulty swallowing due to esophageal webs. The parents of the children did not display similar findings; however the parents were first cousins, indicating an autosomal recessive inheritance pattern. Based on these findings, a diagnosis of DC was made and the family received genetic counseling. This case illustrates that DC is an important differential diagnosis in a child with pancytopenia and recurring esophageal webs. Gastroenterologists should be aware of this rare genetic syndrome in order to initiate further work up in the appropriate settings. 15 TEENAGER WITH NEW ONSET ASPIRATION E. Aitken, J. Screws, Pediatrics, University of TN College of Medicine‐Chattanooga, Chattanooga, TN; E. Aitken, J. Screws, Pediatrics, T.C. Thompson Children's Hospital, Chattanooga, TN; A 14 year old female presents to a pediatric GI clinic after choking on shrimp. The patient also complained of solid foods, especially meat, getting stuck in her throat and had never been on acid suppression therapy. She denied choking or difficulty with liquids. Patient had a longer history of about 2‐3 years of pyrosis and epigastric pain described as burning that was relieved by water. She also complained of a productive cough that persisted after 3 weeks of an upper respiratory infection. Secondary to persistent cough and dysphagia a swallow study done 2 days prior revealed repetitive episodes of deep laryngeal penetration and aspiration with thin and nectar consistencies only. It was recommended that she thicken all thin liquids to honey consistency. An upper endoscopy showed EOE with at least 52 intraepithelial eosinophils per high power field in the proximal, mid and distal esophagus. There were also foci of superficial eosinophilic microabscesses present. The patient was put on an oral methylprednisone burst. This was followed by fluticasone propionate twice a day that was to be swallowed and esomeprazole daily. Because of the patient’s swallowing dysfunction and concern for abnormal anatomy she was referred to ENT. An indirect laryngoscopy was normal. She was noted to be allergic to wheat and peanuts by skin prick testing and was advised to avoid them, along with all nuts. She was also noted to have perennial allergic rhinitis. Montelukast sodium was added. Seven weeks after starting treatment for EOE her dysphagia, choking, cough, and pyrosis had resolved. A follow up swallow study at 9 weeks showed significant improvement. No aspiration was noted with thin or nectar thick liquids. Laryngeal penetration was noted with thin liquids, but no penetration was noted with nectar thick liquids. A follow up swallow study was scheduled for 8 weeks later with the plan to repeat the upper endoscopy after the swallowing changes resolved. 16 HERPETIC ESOPHAGITIS IN IMMUNOCOMPETENT WRESTLERS J. Khlevner, J. Morganstern, Pediatric Gastroenterology, Stony Brook University Medical Center, Stony Brook, NY; Background: Infectious esophagitis is rare and usually self‐limited in the immunocompetent host.Common causes include Candida, cytomegalovirus(CMV) and herpes simplex virus (HSV).HSV‐1 esophagitis has been reported in immunocompetent children although the mode of transmission remains unclear.We present two patients with herpetic esophagitis who share a possible risk factor. Case 1: A 17 year old previously healthy male, member of a wrestling team, presented with a 3 day history of midsternal chest pain, odynophagia and fever.Physical exam was remarkable for epigastric tenderness.Labs, EKG and chest X‐ray were normal.Upper endoscopy revealed multiple ulcerations, exudates and friable mucosa from mid‐lower esophagus.Microscopically, severe inflammation, basal cell hyperplasia, rare intraepithelial eosinophils, and no fungal elements were seen.Oral esomeprazole and sucralfate were started.Significant clinical improvement was noted by day 3.PCR of esophageal tissue was positive for HSV‐I,negative for CMV. Case 2: A 16 year old previously healthy male, also a member of a wrestling team, presented with a 2 day history of dysphagia, odynophagia and fever.Physical exam, labs, EKG and chest X‐ray were normal.Esophagoscopy revealed friable, ulcerated mucosa with exudate.Microscopic evaluation demonstrated highly reactive squamous epithelium and rare eosinophils.No viral inclusions or fungal organisms seen.The patient was started on oral esomeprazole and sucralfate.Significant clinical improvement was noted by day 3.PCR of esophageal tissue was positive for HSV‐I, negative for CMV.Repeat esophagoscopy in 2 weeks demonstrated healing ulcerations.HSV was not detected via PCR. Conclusion: Herpetic esophagitis should be considered in any patient presenting with dysphagia, chest pain and/or fever. These cases help demonstrate that herpetic esophagitis in the immunocompetent patient is self‐limited and that supportive care without antiviral medications is sufficient for treatment.The fact that both patients were wrestlers suggests that a mode of transmission may be related to this.Further investigation is warranted. 17 MICROVILLOUS INCLUSION DISEASE (MVID) ASSOCIATED WITH PAUCITY OF THE BILE DUCTS. P.J. Palomo, R.T. Fischer, R.E. Quiros‐Tejeira, Pediatric Gastroenterology, University of Nebraska Medical Center, Omaha, NE; D.F. Mercer, Transplant Surgery, University of Nebraska Medical Center, Omaha, NE; G.A. Talmon, J.L. Wisecarver, Pathology, University of Nebraska Medical Center, Omaha, NE; Introduction: MVID is a rare,congenital intestinal disorder associated with watery diarrhea and intestinal failure. Patients require life‐long parenteral nutrition(PN)and often progress to intestinal transplantation.Findings on liver biopsy typically reflect changes associated with PN. We present a previously undescribed association of MVID with a paucity of interlobular bile ducts(PILBD). Case Report. A former 36‐week Hispanic male whose delivery was complicated by respiratory distress and presumed sepsis. On his 3rd day of life, he developed watery diarrhea and electrolyte abnormalities. The diarrhea was unresponsive to numerous formula changes and persisted when feedings were held. He was transferred to our center at 65‐days‐old for further evaluation on full PN. He was jaundice with hepatomegaly but no splenomegaly. An abdominal sonogram was normal. Initial labs demonstrated a total bili of 7.6 mg/dl(with a direct of 4.3 mg/dl), AST of 145 mg/dl, ALT of 113 mg/dl and a γGTP of 155 mg/dl. Stool studies confirmed a secretory diarrhea. Small bowel biopsy was consistent with MVID, based on periodic acid‐Schiff, CD10 staining and electron microscopy. Liver biopsy showed mild portal fibrosis,canicular fibrosis and PILBD. Discussion: To our knowledge, this is the first case of MVID associated with PILBD. Evidence points to mutations in the gene encoding Myosin Vb(MYO5B)as a major cause of early‐onset MVID. Interestingly,MYO5B is located on chromosome 18q21, a site shared by the ATP8B1 gene responsible for progressive familial cholestasis type 1 (PFIC1). Though our patient is not believed to have PFIC, mutations of MYO5B could affect a sensitive signaling genetic pathway on the liver leading to a non syndromic PILBD. Hence,patients with MVID could be at higher risk for progressive liver disease,especially in the face of PN. Further genetic studies are necessary to assess this hypothesis. 18 CHOLESTASIS ASSOCIATED WITH GRAVES' P.N. Kratimenos, N. Kerkar, R. Arnon, T. Miloh, Pediatric Hepatology, Mount Sinai School of Medicine, Mount Sinai Medical Center, New York, New York, NY; Patient is a 17‐year old female with shortness of breath presenting with jaundice, palpitations and unintentional weight loss for 2 months. A month ago she developed RUQ pain, nausea, dark urine, pruritus, weakness and was diagnosed with Hepatitis A. Her symptoms progressed and she was referred to our institution due to coagulopathy (INR 1.9). The patient was significantly jaundiced with mild exophthalmia, palpable tender thyroid with hepatomegaly. Labs at diagnosis and course are summarized in Table 1. The patient was positive for anti‐TPO, HAV (IgM), total IgG 1627. ANA, ASMA, LKM reported negative. The thyroid US revealed enlarged inflamed thyroid. Histology of the liver showed inflammatory infiltration and few plasma cells. Electron microscopy of the liver revealed significant cholestasis and hepatocytes with large numerous mitochondria. Patient was treated with methimazole, propranolol, ursodiol, rifampin, phytonadione and hydroxyzine. Total and direct bilirubin normalized 48 days after the diagnosis(Dx). The patient underwent a total thyroidectomy 3 months after Dx. Discussion: The patient presented with cholestasis, liver failure in the background of hyperthyroidism and hepatitis A. HAV may present with cholestasis, however it is prolonged (>12 weeks). Autoimmune hepatitis may be associated with Graves', but ruled out with negative serology and histology. Cholestasis resolved with normalization of the thyroid function. 19 GLYCOGEN HEPATOPATHY IN POORLY CONTROLLED TYPE 1 DIABETIC CHILDREN H.T. Vo, Y. St. Louis, Pediatrics, Bronx Lebanon Hospital Center, Bronx, NY; D.H. Pan, G.W. Klein, A. Loizides, P. Zhou, Pediatrics, The Children’s Hospital at Montefiore, Bronx, NY; Q. Liu, Pathology, The Children’s Hospital at Montefiore, Bronx, NY; Glycogen hepatopathy (GH) has been described as one of the manifestations of poorly controlled type 1 diabetes (T1DM) in children. Diabetic patients with hepatomegaly and abnormal liver enzymes have often been assumed to have non‐alcoholic steatohepatitis (NASH) rather than GH. Yet, GH in T1DM has distinct histologic features, is a benign condition and is reversible with good glycemic control, whereas NASH has been reported to progress to fibrosis or cirrhosis. It is important to recognize and distinguish GH from NASH as it impacts the management and long‐term prognosis. Aiming to expand the recognition of GH in T1DM among pediatricians, pediatric gastroenterologists and hepatologists, we performed a retrospective chart review of 5 children with poorly controlled T1DM who developed GH, and reviewed the literature. Selected clinical and laboratory data of the patients are presented in Table 1. Liver biopsy was performed on three patients, and none showed advanced liver disease such as fibrosis or cirrhosis. Hepatomegaly resolved and liver tests normalized in three children after euglycemic control was established. Histologically, GH is characterized by large, swollen, glycogen‐laden hepatocytes without significant fatty change, inflammation, or fibrosis. Although it will confirm the diagnosis of GH, a liver biopsy may not be needed in the setting of massive hepatomegaly and poorly controlled T1DM. It has been suggested that diabetic patients with hepatomegaly and elevated liver enzymes be given a trial of improved glycemic control prior to liver biopsy. 20 HIV HEPATOPATHY: HISTORY SUGGESTS THE DIAGNOSIS S. Walji‐Virani, M.A. Russo, Pediatric Gastroenterology, Hepatology and Nutrtition, Childrens Medical Center, Dallas, Dallas, TX; S. Walji‐Virani, M.A. Russo, Pediatric Gastroenterology, University of Texas Southwestern, Dallas, TX; T. Barton, Pediatric Infectious Diseases, University of Texas Southwestern, Dallas, TX; A comprehensive history and physical exam remain the cornerstone to evaluating a child with elevated aminotransferases. Human Immunodeficiency Virus (HIV) is not routinely considered in the differential possibilities in pre‐teen children both due to age and decreased likelihood for blood products exposure. There is limited literature that addresses HIV as a cause for hepatitis. A 10 year old African American boy presented with jaundice and increased fatigue. Mother had remarried after a divorce when biological father died from unknown causes; family history was positive for paternal hemophilia. The patient's past medical history was significant for two episodes of herpes zoster and hospitalization for an “infected hematoma.' On physical exam, scleral icterus and hepatomegaly were significant findings. He had no stigmata to suggest chronic liver disease ‐ lacking splenomegaly, ascites, vascular changes and palmar hyperemia. Labs revealed transaminitis with cholestasis, mild coagulopathy, severe lymphopenia and proteinuria. Anatomic, infectious, metabolic, rheumatologic and autoimmune testing were unrevealing. Abdominal sonogram showed increased echogenicity throughout the liver. Ultimately HIV screen returned positive. Under the direction of our infectious disease colleagues, anti‐retroviral therapy was instituted. A dramatic improvement was noted in his transaminitis with normalization of all liver associated enzymes within two months of therapeutic intervention. Early detection and treatment of HIV can prevent liver related morbidity and improve mortality. Obtaining a HIV screen as part of the routine work‐up, particularly in a family background of recurrent blood product exposure, should be a mainstay in the evaluation of patients with elevated aminotransferases. 21 SUPERIOR MESENTERIC COLLATERALS BYPASSING LIVER CAUSING HEPATOPULMONARY SYNDROME B. Loveridge‐Lenza, K. Furuya, Gastroenterology and Nutrition, AI duPont Hospital for Children, Wilmington, DE; K. Furuya, S. Dunn, Division of Solid Organ Transplant, AI duPont Hospital for Children, Wilmington, DE; S. Dunn, Division of Pediatric Surgery, AI duPont Hospital for Children, Wilmington, DE; 12 year old Caucasian male with significant past medical history of heterotaxy syndrome with mesocardia, several cardiac anomalies, interrupted IVC with azygous continuation s/p Mustard procedure, s/p failed Kasai portoenterostomy for biliary atresia and living related donor liver transplantation presented with complaints of worsening dyspnea and hypoxia for 3 months. Investigations included a CT scan which showed prominent numerous and markedly enlarged collateral vessels throughout the abdomen and pelvis. An echocardiogram showed normal biventricular systolic function. Cardiac catheterization showed hepatopulmonary syndrome with diffuse arteriolar and capillary intrapulmonary right to left shunt, arterial saturation of 85% with high output heart failure with preserved systolic function, small inferior Mustard Baffle (only draining hepatic veins) and multiple venous collaterals seen from SMA territory to IVC/azygous system bypassing the liver. Liver biopsy showed fibrous expansion of some portal areas with fibrous septae with focal bridging. Interventional radiology embolized some of his collaterals to try and decrease shunting away from the liver. This patient is a good example of hepatopulmonary syndrome related in part to his extensive cardiac history , interrupted IVC, and formation of large venous shunts which resulted in blood flow directed into his lungs bypassing the liver. The liver produces plasminogen and collagen XVIII, which are presursors of angiogenesis inhibitors. Blood flow bypassing the liver does not contain these substances and may lead to angiogenesis, vascular proliferation and eventual formation of arteriovenous fistulas. This supports the hypothesis that the liver is actively responsible for remodeling the pulmonary vascular bed. 22 DUODENAL HEMATOMA RESULTING IN PANCREATITIS & GASTRIC OUTLET OBSTRUCTION FOLLOWING ENDOSCOPY IN AN ALAGILLE PATIENT M.A. Bozic, A. Har, G. Subbarao, Pediatric Gastroenterology, Hepatology, and Nutrition, Indiana University School of Medicine, Indianapolis, IN; Background: Intracranial hemorrhage is a known complication among Alagille patients. Cirrhosis with coagulopathy and thrombocytopenia places these patients at an additional increased risk for bleeding. There are no known reports of hemorrhagic complications from routine endoscopy among Alagille patients. Case: 7 y.o. female with Alagille syndrome complicated by congenital heart disease, cholestasis, and portal hypertension presents for EGD for evaluation of diarrhea, failure to thrive and new onset hemoptysis. Prior to procedure, patient had an INR of 1.19 and platelet count of 99,000. Endoscopically, esophageal, gastric and duodenal mucosa was normal without evidence of varices. Biopsies were obtained from the distal duodenum and stomach. There was no active bleeding from the biopsy sites. Patient tolerated procedure well and was discharged home. Hours later, she developed vomiting and epigastric abdominal pain radiating to the back. Laboratory studies revealed a lipase of 3024 U/L and HgB of 9.8 gm/dL. Abdominal CT scan demonstrated a C‐shaped, mixed density structure in the retroperitoneum along the lateral and inferior margins of the pancreatic head following the distribution of the duodenum. It measured 6.6 x 4.3 cm in diameter. Stomach was distended suggestive of a gastric outlet obstruction. She was diagnosed with pancreatitis and gastric outlet obstruction secondary to a large duodenal hematoma. She was managed conservatively; npo, TPN, pain control and intermittent gastric suction. She was given one PRBC transfusion to maintain hemoglobin above 8gm/dl. Patient slowly improved with above measures. Conclusion: This is the first known case report of a duodenal hematoma resulting in gastric outlet obstruction and pancreatitis following endoscopy with routine biopsy in an Alagille patient. The risk of bleeding in Alagille patients, even those with normal coagulation studies and platelet levels must be taken into account before even relatively noninvasive procedures such as endoscopy. 23 HEPATOPORTAL SCLEROSIS IN A 5 YR OLD WITH A HISTORY OF MULTIPLE INTESTINAL ATRESIAS AND PERSISTENT BACTERIAL OVERGROWTH K.C. Usmani, A. Chawla, Pediatric Gastroenterology, Stony Brook University Medical Center, Stony Brook, NY; R. Scriven, Pediatric Surgery, Stony Brook University Medical Center, Stony Brook, NY; Hepatoportal sclerosis (HPS) is a rare condition characterized by portal hypertension, splenomegaly and variceal bleeding. Its etiology is unclear but it is hypothesized that it may be due to a vascular thrombotic mechanism. Coagulation and platelet dysfunction have been observed. A state of mild disseminated intravascular coagulation due to endotoxemia or portosystemic collaterals has been suggested. Intestinal bacterial infections with repeated septic embolization of the portal circulation have been proposed as a possible etiology. HPS has not been associated with TPN exposure. However, trace metals including copper sulfate, a component of TPN have been implicated as the causative agents in the pathogenesis of hepatoportal sclerosis. We present the case of a 5 yr old male with portal hypertension secondary to hepatoportal sclerosis diagnosed on liver biopsy. He was born at 32 wks gestation with 23 intestinal atresias with resulting short gut. He received TPN for 3 months. Severe cholestasis was noted with direct bilirubin of 16.5 mg/dL at 3 months of life. This resolved by 6 months. Splenomegaly noted since 2yrs of age, which has steadily progressed with a corresponding drop in his platelet count, white count and increase in INR. Endoscopy revealed grade 2 varices. Protein S and C were slightly low. Parenteral vitamin K did not correct the INR. Vitamin K level was normal. MRI revealed periportal fibrosis, splenomegaly and peri‐splenic varices. He was empirically treated with rotating antibiotics for bacterial overgrowth. He had 3 positive hydrogen breath tests over a 9 month period. Clinically the child is thriving and gaining weight. Multiple intestinal atresias are a predisposing factor for bacterial overgrowth. Persistent bacterial overgrowth with most likely recurrent bacterial embolization of the portal circulation may likely be the etiology of HPS in our patient. Liver insult may have been further compounded by TPN in the neonatal period. 24 RAPIDLY PROGRESSIVE ADENOVIRUS ASSOCIATED ACUTE LIVER FAILURE IN A CHILD WITH ALL: POTENTIAL DIAGNOSTIC UTILITY OF HEPATIC IMAGING FINDINGS A.R. Maspons, R. Himes, , Texas Children's Hospital, Houston, TX; Introduction: Adenovirus is an uncommon cause of acute liver failure. Children with malignancies and other immunocompromised states appear to be at an increased risk. Cedofovir is effective, but detection is key and challenging. We report a case of adenovirus‐associated acute liver failure in a patient with ALL in remission and highlight hepatic imaging findings that may be diagnostically useful. Case Report: A 16 month old African American male with ALL in remission on maintenance chemotherapy, was admitted for fever, neutropenia, but remained febrile despite antibiotics, antifungal and antiviral (ganciclovir) therapy. He later developed multiorgan failure, including acute liver failure (INR 2.8, later 12.8). An abdominal CT demonstrated multiple hypoechoic liver lesions that were not visualized on ultrasonography at the time of a directed liver biopsy attempt one day later. His blood, stool cultures and nasal pharyngeal viral PCR studies were negative. Serum adenovirus PCR was 3.5x10e9 DNA copies/ml, resulted after patient’s demise. On autopsy: hepatomegaly, multiple liver nodules (0.3 ‐ 1.7 cm) with central umbilication; histologically: nodules with bland necrosis, abundant viral inclusions. Cultures of lesions and grossly‐normal appearing lung grew adenovirus. Discussion:Adenovirus‐associated acute liver failure is rapidly progressive, often fatal in immunosuppressed states. Prompt identification is vital. When adenovirus is considered, consequences of delay in identification, as well as effectiveness of treatment and renal toxicity of cedofovir must be weighed. In our case, as in that reported by Rothenburg, et al. discrete lesions were noted in the liver of patient by CT scan but were not reproducible on ultrasonography. We propose that this finding, in the setting of a sepsis‐like picture in an immunocompromised patient, should raise the specter of adenovirus and prompt consideration of appropriate testing or treatment. Reference: Rothenberg,M et al., Adenovirus Induced Acute Liver Failure. Dig Dis Sci (2009) 54:218–221 25 A SUCCESSFUL LIVER TRANSPLANT FOR SEVERE CONGENITAL FACTOR V DEFICIENCY S.L. Page, Gastroenterology, Children's Mercy Hospitals & Clinics, Kansas City, MO; B. Wicklund, Hematology, Children's Mercy Hospitals and Clinics, Kansas City, MO; W. Andrews, Surgery, Children's Mercy Hospitals and Clinics, Kansas City, MO; J. Daniel, Hepatology, Children's Mercy Hospitals and Clinics, Kansas City, MO; Factor V (FV) is produced primarily in the liver, and FV deficiency is rare. Fresh frozen plasma (FFP) is the treatment of choice for FV deficiency as no single replacement factor exists for FV. A few case reports have been published regarding neonates who presented with intraparenchymal and/or subdural hematomas associated with FV deficiency. All patients were managed with FFP. We present a case of an 11 month old male who developed bilateral cephalohematomas at birth. He also had prolonged bleeding from a heelstick and was found to have FV deficiency with a level of 4%. At 3 months of age he developed unilateral subdural and intraparenchymal hemorrhage with herniation and obstructive hydrocephalus requiring emergent craniotomy. His FV level was <1%. He was treated with FFP until he developed an allergic reaction consisting of generalized hives and agitation. He was transitioned to recombinant factor 7 as therapy. At 4 months of age he presented with bilateral chronic subdural hematomas and a new left parietal intraparenchymal hemorrhage. Acetaminophen and diphenhydramine were given prior to FFP as prophylaxis. He was maintained on every other day FFP infusions while a liver transplant evaluation was performed. He remained at significant risk for further anaphylaxis and development of inhibitors to FFP. We hypothesized that liver transplant would provide adequate FV to ultimately decrease his risk of further episodes of intracranial hemorrhage. Within hours of his orthotopic liver transplant, his FV levels reached normal and have remained there since his operation. He no longer requires every other day infusions of FFP. He has had no further episodes of intracranial hemorrhage. He is developing normally. This case demonstrates how liver transplantation may be a good option for patients diagnosed with severe congenital factor V deficiency. 26 INSPISSATED BILE SYNDROME SUCCESSFULLY TREATED BY ERCP WITH SPHINCTEROTOMY AND BILIARY IRRIGATION K. Atienza, Y. Rivas, Pediatric GI, Children's Hospital at Montefiore, Bronx, NY; G. Weinberg, Pediatric Surgery, Children's Hospital at Montefiore, Bronx, NY; S. Ho, Gastroenterology, Montefiore Medical Center, Bronx, NY; K. Atienza, G. Weinberg, S. Ho, Y. Rivas, , Albert Einstein College of Medicine, Bronx, NY; Cholestasis is a common neonatal diagnosis requiring prompt evaluation. In addition to laboratory testing, imaging studies are performed to identify possible anatomic abnormalities, such as extrahepatic biliary atresia or a choledochal cyst. Inspissated bile syndrome is a rare form of obstructive jaundice in neonates, which is caused by the development of dense bile sludge or cholelithiasis resulting in intraluminal biliary obstruction. Predisposing factors include hemolysis, sepsis, total parenteral nutrition, and abdominal surgery. We describe a term baby boy who had a prenatal diagnosis of gastroschisis, which was repaired at birth and required a loop ileostomy due to ileal obstruction caused by tenacious meconium. After reanastamosis, the baby developed acholic stools, hepatomegaly, and worsening cholestasis despite full enteral feeds and ursodeoxycholic acid therapy. His abdominal ultrasound showed gallbladder sludge with fusiform dilation of the common bile duct. An MRCP showed dilation of the common bile duct and the distal left hepatic duct. An ERCP with sphincterotomy was performed. A normal common bile duct with a dilated left intrahepatic system was identified. Following biliary irrigation, gallstones and sludge emerged from the sphincterotomy site. Post procedure, the baby had yellow stools and significantly decreased bilirubin levels. A repeat ultrasound showed decreased caliber of the left hepatic and common bile ducts. Management of inspissated bile syndrome has traditionally involved percutaneous transhepatic cholangiography or cholecystectomy to lavage the biliary tree to re‐establish bile flow. We have successfully shown that ERCP with sphincterotomy and biliary irrigation is a less invasive and safe alternative. 27 INTRAVENOUS IMMUNOGLOBULIN THERAPY FOR NEONATAL HEMOCHROMATOSIS: SUCCESSFUL OUTCOME IN A SIBLING REFRACTORY TO ANTIOXIDANT REGIMEN J. Shah, N. Mittal, Pediatrics, UTHSCSA, San Antonio, TX; Neonatal hemochromatosis (NH) is a rare disorder and one of the most common causes of liver failure in the neonatal period. Until recently, therapy has revolved around antioxidant cocktail regimens. Liver transplantation is indicated for patients refractory to antioxidant therapy. The role of high‐dose intravenous immunoglobulin (IVIG) in refractory cases of NH has been suggested in a recent study. Baby Girl S.M. was the 1900gm product of a 34 weeks gestation to a 21 year‐old gravida 3, para 2 mother, delivered via cesarean section. The mother lost a prior child who presented with acute liver failure and confirmed NH on autopsy. Physical examination revealed jaundice without hepatosplenomegaly. Initial laboratory findings included transaminitis, direct hyperbilirubinemia, coagulopathy, hypoalbuminemia and an elevated ferritin level. A clinical diagnosis of NH was made based on the presentation of acute liver failure, elevated ferritin levels and a positive family history. A chelation and antioxidant cocktail was initiated, which included a 7‐day course of acetylcysteine and a 10‐day course of alpha‐tocopherol, desferroxamine, selenium‐3 and low‐dose PGE‐1. This therapy was met with limited success, as there was a lack of improvement of laboratory findings and clinical status. A recent article suggests a role of IVIG in improving the outcome of patients with NH, and reducing the need for liver transplantation. While the exact mechanism by which IVIG improves outcomes is still unclear, the proposed strategy centers on displacing reactive IgG from the target antigen, and interfering with the formation of membrane attack complexes. On days of life 5 and 19, this infant received two 1g/kg doses of IVIG with clinical improvement, and was released from the NICU with outpatient follow‐up. 6 months follow‐up showed clinical and biochemical recovery of NH. 28 ACUTE HEPATITIS ASSOCIATED WITH NOVEL INFLUENZA A (H1N1) INFECTION L. Wozniak, J. Yeh, C. Fink, M. Martin, M. Ament, K. Ngo, Pediatrics, UCLA, Los Angeles, CA; G. Cortina, Pathology, UCLA , Los Angeles, CA; J. DeVille, Infectious Disease, UCLA , Los Angeles, CA; K. Ngo, Transplant Surgery, UCLA , Los Angeles, CA; CASE REPORT: A 2‐year‐old previously healthy boy presented in October 2009 with 4 days of fatigue and 2 days of jaundice. He had no fevers, URI symptoms, abdominal pain, vomiting, diarrhea, sick contacts, travel, or acetaminophen use. On exam there was a soft liver edge 3cm below the right costal margin and a palpable spleen tip. Admission labs revealed hepatitis, hyperbilirubinemia, and coagulopathy (AST 2228, ALT 1950, T/D bilis 5.5/4.6, Albumin 3.7, INR 1.4). Ultrasound showed an edematous, hypoechogenic liver, edematous gall bladder, and enlarged spleen. Liver biopsy showed severe acute hepatitis without fibrosis, steatosis, or cholestasis. Though his AST and ALT trended down, his T/D bilis and INR peaked on day #3 at 9.6/5.6 and 1.6. On day #3 he developed fever and cough. On day #6 respiratory viral culture came back positive for influenza A, and he was started on oseltamivir. PCR confirmed novel influenza A (H1N1). Extensive workup revealed no other etiology for his hepatitis. He was discharged on day #12 with improving liver function tests. DISCUSSION: H1N1 was first identified in April 2009 and rapidly became a global pandemic. Hepatic involvement is uncommon, and there are few reports of H1N1‐associated hepatitis. Interestingly, acute hepatitis has been reported in association with past influenza A epidemics (Whitworth, 2006). Influenza A has also been described to contribute to hepatic decompensation in patients with cirrhosis (Duchini, 2000). The exact mechanism of hepatic dysfunction is unclear, but influenza A appears to be hepatotropic. Our patient’s liver biopsy was tested by the CDC, and no influenza virus was detected by immunohistology or PCR, suggesting the hepatitis is indirectly mediated. The risk for hepatic involvement in cases of influenza A needs to be appreciated. Early testing for respiratory viral infections should be considered in patients with hepatitis of unknown etiology as influenza A may be an under‐recognized cause of hepatic dysfunction. 29 ALPHA 1‐ANTITRYPSIN DEFICIENCY IN A CHILD WITH TYROSINEMIA TYPE 1 P. Mohanty, M. Beg, , SUNY Upstate Medical University, Syracuse, NY; Introduction: Alpha 1‐Antitrypsin (AAT) deficiency and tyrosinemia are distinct inherited metabolic conditions with specific pathophysiology and clinical manifestations. We report an infant boy with a diagnosis of tyrosinemia type 1 who was subsequently found to have AAT deficiency. Case description: A full term baby boy born to nonconsanguineous parents presented at 2 weeks of age with bloody diarrhea, vomiting, lethargy and prolonged jaundice. Evaluation showed elevated liver transaminases, elevated direct bilirubin, coagulopathy, hypoglycemia, hyperchloremic acidosis and renal Fanconi Syndrome. Serum amino‐acid analysis revealed elevated tyrosine levels, deficient fumarylacetoacetate hydrolase (FAH) and elevated urinary succinyl acetone. Molecular analysis identified two mutations consistent with tyrosinemia type 1 (TT1). Nephrocalcinosis was noted on renal ultrasound. Liver biopsy was consistent with chronic active hepatitis. Coincidentally, AAT level was reported to be low with phenotype of SZ. Parents were found to have phenotype MS and MZ and his brothers had MM phenotype. The patient responded well to nitisinone therapy and low tyrosine, low phenylalanine diet. Discussion: TT1 is an autosomal recessive disorder caused by mutation on chromosome 15q23‐q25 resulting in deficiency of FAH. AAT deficiency is caused by a mutation on chromosome 14q32.1. Liver disease is associated with phenotype ZZ but it may occur with phenotype SZ at a relatively young age and with MZ in adulthood. We speculate that the liver disease in this case has mainly been caused by TT1; however it is difficult to ascertain whether the presence of the coexisting AAT deficiency ameliorates or accentuates the liver injury. It is interesting to note that the genes responsible for these two disorders are on different chromosomes. Conclusion: To our knowledge, there are no previous reports of tyrosinemia type 1 and alpha 1‐antitrypsin deficiency diagnosed in the same individual. Whether this association will affect the patient’s prognosis is not clear yet. 30 PHENOTYPIC VARIATIONS AND NOVEL MUTATIONS FOR PROGRESSIVE FAMILIAL INTRAHEPATIC CHOLESTASIS J.M. Stoll, M.M. Jonas, Gastroenterology, Children's Hospital Boston, Boston, MA; Progressive familial intrahepatic cholestasis (PFIC) is a heterogeneous group of cholestatic disorders which often lead to severe liver disease. Three major types have been recognized based on biochemical and genetic abnormalities. The majority of children with PFIC present with jaundice and pruritus. Two patients presented to our institution with atypical clinical characteristics and each had novel mutations in genes known to be associated with PFIC. A 4 year old girl presented with chronic, intractable pruritus which she had had since 3 months of age. The patient had never been jaundiced. She was noted to have elevated serum bile acids with a normal GGT. A liver biopsy revealed hepatocellular damage with intrahepatic cholestasis, ductal proliferation, and portal fibrosis. Genetic testing demonstrated a previously unreported splice‐site mutation in ABCB11 on one allele as well as a novel sequence variant on the other, predicted to be PFIC 2 disease‐causing. A 2 year old asymptomatic girl presented for a well‐child visit and was noted to have hepatosplenomegaly. Laboratory studies revealed elevated aminotransferases, low platelets, and elevated serum bile acids. Liver biopsy demonstrated micronodular cirrhosis with mixed portal inflammation and cholangiolar hypertrophy. Genetic testing noted a nonsense mutation in ABCB4 on one allele and a previously unreported sequence variant on the other, predicted to be PFIC 3 disease‐ causing. Parental testing revealed maternal carriage of the nonsense mutation and paternal carriage of the novel missense variant. These cases illustrate the importance of considering PFIC as a cause of cholestatic liver disease when patients present without the classic findings of jaundice and pruritus. Sequencing for the genes known to cause PFIC expands diagnostic ability, allows detection of novel disease‐causing mutations, and improves recognition of atypical phenotypes of these disorders. References 1. Davit‐Spraul, A., et al., Progressive Familial Intrahepatic Cholestasis. Orphanel J Rare Dis, 2009. 4: p1. 31 SEVERE GASTROSTOMY TUBE DETERIORATION DUE TO FUNGAL INVASION C. Wilson, Pediatric Gastroenterology, Stanford University School of Medicine, Palo Alto, CA; K. Thompson, Pathology, University of Hawaii John A Burns School of Medicine , Honolulu, HI; A 9‐month old with dysphagia associated with Rubinstein‐Taybi syndrome residing in Hawaii had a percutaneous endoscopic gastrostomy (PEG) placed using a Bard 16 French Ponsky pull‐type tube. He was fed a 24 calorie per ounce peptide‐based infant formula in a combination daytime bolus and overnight drip schedule. His only medications were cimetidine and as‐needed nebulized albuterol. Three and a half months (108 days) later the device had to be removed due to severe deterioration of the shaft of the tube, which had a irregular outside surface, multiple pinpoint holes, and a foul odor. The only area of the tube shaft spared was the 1‐2 cm closest to the stoma itself. Although preparations for endoscopic removal were made in case of tube breakage with routine removal, the tube was successfully removed in 1 piece by traction, and replaced with a low‐profile balloon‐type tube. Microscopically, sections of the PEG tube showed invasion of the tube material with numerous budding yeast. Although fungi are recognized as colonizers of gastrostomy tubes and deterioration of tube material by fungi has been reported, our case is significant due to the extent of tube damage in a short period of time. The sparing of the tube area closest to the gastric contents suggests that contamination did not arise from the stomach, and that components of gastric fluid were a deterrent to fungal growth. 32 AN UNUSUAL CASE OF FEEDING INTOLERANCE IN A 7 MONTH OLD FEMALE A. Dorros, A. Safta, C. Greene, S. Blanchard, Pediatrics, University of Maryland Medical Center, Baltimore, MD; Aromatic l‐amino acid decarboxylase deficiency (AADC) is an inborn error in neurotransmitter metabolism which leads to combined serotonin and catecholamine deficiency. Symptoms include severe developmental delay, hypotonia, muscle stiffness, athetosis, lethargy, feeding intolerance, sleep disturbances, extreme irritability, muscle spasms, and uncontrolled movements. A 7 month old Caucasian female with a history of hypotonia and developmental motor delay presented with excessive irritability and feeding intolerance. She was well until 3‐4 months of age, when she became inconsolable, had non‐bilious, non‐bloody emesis, and irritability with feeds. Symptoms worsened over the last 3 months. At evaluation, she had decreased activity, poor eye tracking, and hypotonia. Initial workup showed normal gastric emptying scan, MBS revealing frank aspiration, and pH probe showed copious reflux, so a Nissen fundoplication with gastric feeding tube was performed. Additional workup included an EEG, head CT scan, head MRI, karyotype, serum lactate level, serum ammonia level, and plasma amino acids which were all within normal limits except for a low cysteine level. Urine organic acids showed minimal dicarboxylic acids and acetylcarnitine profile showed a mildly elevated C4OH due to diet or a ketotic state. Magnetic resonance spectroscopy (MRS) was notable for nonspecific lactate spikes. Because of suspicion of a possible mitochondrial or neurotransmitter disorder, a lumbar puncture was performed. Neurotransmitter analysis and plasma enzymology confirmed the diagnosis of AADC. Pyridoxal‐5‐phosphate and Ropinirole were added, in addition to hyoscyamine, pantoprazole, and sucralfate. Two months after diagnosis, she has been tolerating feeds with less irritability. Children with feeding disorder, developmental delay and hypotonia should have a work‐up for inborn errors of metabolism. Initial laboratory workup should include CBC, electrolytes, LFTs, urine organic acids, blood amino acids and acylcarnitine profile, total and free carnitine levels, as well as an MRI and MRS. 33 FEEDING DIFFICULTIES IN STUVE‐WIEDEMANN SYNDROME: IS EARLY PEG PLACEMENT A BETTER FEEDING OPTION? S. Narwal, A. Felix, O. Yugay, , Maimonides Medical Center, Brooklyn, NY; Background: Stuve–Wiedemann syndrome (SWS) is a rare autosomal recessive disorder that was initially described as a unique syndrome by Wiedemann and Stuve in 1971. SWS was characterized as a Multiple Congenital anomalies (MCA) syndrome with distinctive clinical and radiographic features. Patients are commonly described with bowing of lower limbs, camptodactyly, scoliosis, episodic hyperthermia, feeding difficulties with an increased risk of pulmonary aspiration, and respiratory distress. It is no longer considered a fatal condition since at least 11 long‐term survivors have been reported within the last decade. Method: We present a case report of three siblings with SWS born in a consanguineous family from Pakistan. One of the affected siblings died early during infancy while the other two are currently alive at 5 years and 9 months of age. All of the siblings experienced impaired oral feeding along with respiratory complications necessitating tube feeding. Results: Feeding evaluation by Modified Barium Swallow performed by a speech pathologist and a radiologist showed poor swallow with aspiration and therefore nasogastric (NG) tube feeding was recommended. After failing NG feeds, early percutaneous endoscopic gastrostomy (PEG) tube was placed in all 3 siblings for adequate nutritional support on day of life 35, 20 and 14 respectively. No anesthesia related major side effects and complications were noted during and after the procedures. Conclusions: Patients with SWS suffer from severe feeding difficulties and they mostly develop hyperthermia after anesthesia. All three patients tolerated the procedure and anesthesia well. Both of our surviving patients are tolerating gastrostomy tube feeds and growing adequately. We suggest an early placement of PEG tube as beneficial for long‐term nutritional support in SWS patients, although other feeding options should be considered on an individual basis. 34 PERCUTANEOUS ENDOSCOPIC PLACEMENT OF LOW‐PROFILE GASTROSTOMY‐JUJENOSTOMY TUBE IN 2 YEAR OLD WITH FEEDING DIFFICULTIES P. Jhaveri, P.B. Jhaveri, A. Vasilescu, A. Darbari, Pediatric GI, Hepatology and Nutrition, Johns Hopkins Hospital, Baltimore, MD; P.B. Jhaveri, A. Darbari, Pediatric Gastroenterology and Feeding Disorders Program, Kennedy Krieger Institute, Baltimore, MD; Background: Prolonged emesis occurring in patients with feeding difficulties hinders nutritional status. Children who present with feeding difficulties often cannot tolerate adequate gastric feeds, leading to dehydration and malnutrition and reliance upon IV fluids and parenteral nutrition. The option of post‐pyloric feeds has offered a way to continue enteral nutrition without having voluminous emesis. Though jejunal feeds seem promising, there is a dearth of information on gastrostomy‐jejunostomy tubes in children. Patients who require jejunal feeds have either undergone direct percutaneous endoscopic jejunostomy, surgical jejunostomy or a conversion of a previously placed gastrostomy tube. Direct jejunostomy tubes have been used in patients with conflicting results. Previous publications describe the use of jejunal feeds in the neurologically devastated patient. Case Report: We describe the use of a low‐profile GJ tube in a 2 year old male who was initially seen for feeding difficulties, including marked emesis. Our patient was a mobile toddler who was otherwise developmentally well. His continued movement made a low‐profile device appropriate. Using the newly introduced Kimberly‐Clarke TJ Introducer Kit, we were able to endoscopically place the device, and advance the jejunal extension appropriately. Six months follow up has shown that the patient tolerated the tube well and continues to work on behavioral feeding issues. Discussion: The low‐profile GJ tube offers a well tolerated method to deliver enteral nutrition, while maintaining mobility in patients who are not neurologically devastated. Consideration can be given to those individuals suffering from chronic pancreatitis, prolonged emesis, gastroparesis or feeding difficulties. Additionally, the tube allows for trials of gastric feeds, when appropriate. To our knowledge, this is the youngest patient reported with placement of such a device for feeding difficulty. Friday October 22, 2010 35 PORTAL VEIN THROMBOSIS ASSOCIATED WITH PRIMARY CYTOMEGALOVIRUS INFECTION IN AN IMMUNOCOMPETENT CHILD V. Okwu, N. Alkhouri, R. Alkhouri, B. Rouphail, M. Elias, C. Carter‐Kent, , Cleveland Clinic, Cleveland, OH; Cytomegalovirus (CMV) infection may occur in immunocompetent children and adolescents and often follows an asymptomatic course. Portal vein thrombosis in association with acute CMV infection is a very rare condition in an immunocompetent host. An 18‐year‐old female with no significant medical history presented with a one‐week history of right upper quadrant pain and low grade fever. On examination, the upper part of the abdomen was tender and the spleen was palpable at 4 cm below the costal margin. Initial laboratory testing showed WBC of 10.1 k/uL (lymphocytes 71%), platelet count of 156 k/uL, ALT 193 U/L, AST 134 U/L, LDH 360 U/ml with normal prothrombin time, alkaline phosphatase and bilirubin. An abdominal ultrasound showed a thrombus in the portal vein trunk and splenomegaly of 19.5 cm. Serologic tests were negative for hepatitis A, hepatitis B, hepatitis C, Epstein‐Barr virus, HIV infection and antibodies to toxoplasmosis. Antibodies were detected against CMV with both positive IgG and IgM; CMV DNA was detected by PCR. Screening for thrombophilia revealed normal levels of protein C, protein S, antithrombin III and homocysteine with no prothrombin or factor V Leiden mutations and no antiphospholipid antibodies. A diagnosis of portal vein thrombosis secondary to acute CMV infection was made. The patients was started on heparin and transitioned to oral anticoagulant therapy. Repeated ultrasound in 3 months showed recanalization of the portal vein and improved splenomegaly. This case illustrates that in the presence of acute CMV infection with abdominal pain, the possibility of abdominal venous thrombosis should be always entertained in order to start anticoagulation as soon as possible. Acute CMV hepatitis should be added to the list of risk factors of acute portal vein thrombosis. 36 CHRONIC HEPATITIS B IN CHINESE CHILDREN: WHAT ARE WE WAITING FOR? S. Narwal, A. Felix, O. Yugay, , Maimonides Medical Center, Brooklyn, NY; Background: Chronic Hepatitis B (CHB) is common in Chinese children. Most of these children get CHB via vertical transmission (VT). Treatment with Interferon and Lamuvidine has not been very successful in these patients. Entecavir and Tenofovir have been used only in adults with CHB with some success. Aim: To evaluate the effectiveness and the side effects of Entecavir and Tenofovir in Asian children with CHB. Methods:34 Chinese children with CHB were treated with Entecavir aged between 1‐17 years. 18 patients were born in China and acquired CHB via VT. All of the patients were positive for HBsAg, HBeAg and had high HBV loads for more than 6 months. 29 % of the patients had elevated liver function tests (LFTs). 3 patients underwent liver biopsy showing fibrosis with CHB activity. All patients were monitored at 3 month intervals with blood tests for Electrolytes, CBC, LFTs, Hepatitis B profile and viral load. Tenofovir was added to Entecavir therapy in 7 patients after 1 year of treatment and 5 were started on both as the initial treatment. 7 patients failed to follow up after 3 months. Results: 27 of the initial 34 patients completed therapy with either Entecavir alone or in combination with Tenofovir.100% of the treated patients experienced a significant decrease in the viral load and normalization of LFTs. HBsAg and HBeAg became negative in 4 patients, HBeAb became positive in 9 patients and HBcAb became nonreactive in all the patients between 3‐12 months of therapy. Only 2 patients from this positive response group were on combination therapy and within 3 months of treatment they showed significant viral load decrease and converted to HBeAb. No side effects were noted in any of the patients. Conclusion: Significant viral load reduction, normalization of LFTs and conversion of HBsAg, HBeAg and HBcAb were noted in children treated with Entecavir alone, or in combination with Tenofovir. We suggest that these two drugs can be safely used in children with CHB, but careful monitoring is needed. Further studies with larger number of patients should be done to determine the guidelines to treat pediatric patients with CHB. 37 A NINE MONTH‐OLD WITH ACUTE LIVER FAILURE: THE APPROACH TO DIAGNOSIS OF A RARE BUT TREATABLE CONDITION J. Kaplan, E.J. Israel, Pediatrics, Massachusetts General Hospital, Boston, MA; Acute liver failure is a rare but serious cause of morbidity and mortality in children and its etiology is not found in up to 50% of pediatric cases. A recent evaluation of the PALF database showed that children with acute liver failure deemed indeterminate had a less than complete evaluation for metabolic and autoimmune causes. Here we present a case of liver failure in a young child. A thorough and expedited multidisciplinary evaluation led to a rare but treatable diagnosis. A 9 month‐old previously healthy boy presented to the emergency room with two days of intermittent fever, lethargy, rhinorrhea, vomiting and non‐bloody diarrhea. Evaluation showed a significant elevation of his transaminases and hepatomegaly. Over the next 24 hours, acute liver failure ensued with coagulopathy and encephalopathy. The patient required mechanical ventilation and multiple pressor medications. Initial evaluation for infectious, autoimmune, toxic and ischemic causes was negative. Biopsy of the liver within 48 hours of admission revealed steatosis with a microvesicular pattern in the centrilobular regions and a macrovesicular pattern in periportal regions. Out of concern for persistent lethargy, brain magnetic resonance imaging with spectroscopy was performed, revealing normal brain structure and parenchyma but two very abnormal lipid peaks, suggesting a metabolic condition. Plasma acylcarnitine and urine organic acid profiles were suspicious for a fatty acid oxidation defect. The diagnosis of carnitine‐acylcarnitine translocase (CACT) deficiency was entertained. A low‐fat diet and carnitine replacement resulted in a rapid improvement in liver function and overall clinical status. Molecular genetic testing revealed three mutations in the CACT gene confirming the diagnosis. The patient made a full recovery and has normal liver function 15 months later. This patient’s story supports the notion that with decreasing age, metabolic disorders are more likely to be found as a cause of acute liver failure. An aggressive approach to searching for that cause may very well be life‐saving. 38 RUPTURED HEPATIC ADENOMA IN AN ADOLESCENT MALE K. Romeo, M. Greifer, L. Moy, J. Levine, Pediatric Gastroenterology, Cohen Children's Medical Center of New York, New Hyde Park, NY; Introduction: Hepatic adenomas are benign liver lesions most commonly found in females with long term use of oral contraceptives. We describe an unusual presentation of hepatic adenoma found in a 16 year old male with no risk factors. Case: A 16 year old obese male was referred for elevated liver enzymes. History was notable for 10 days of high fevers, decreased appetite and 15 lb weight loss. Blood tests revealed ALT 566 and AST 82 (IU/L). He had a normocytic anemia with platelets 399 thous/mcL and albumin 4.2 g/dL, total bilirubin 1.6 mg/dL, and alkaline phosphatase 188 IU/L. Physical exam was notable for an obese male with mild pallor, anicteric sclera and no hepatosplenomegaly. Stool was guaiac negative. Sonogram revealed an enlarged liver and a large heterogeneous solid mass measuring 18.6x13.4x17.7 cm with a hypoechoic component. An MRI showed a large mass with fluid and hemorrhagic components and irregular enhancing margins. On admission, ALT was 31 and AST 21 (IU/L). On night of admission, he developed hypotension and an acute drop in his hemoglobin from 9 to 7.9 (g/dL). An angiogram revealed a right hepatic artery adjacent to a large hypovascular area consistent with the known area of the hemorrhage. No AV malformation or tumor vascularity was identified and the right hepatic artery was embolized. The patient underwent a right liver lobectomy and cholecystectomy. On pathology, a hepatic adenoma measured 20 cm in diameter with diffuse infarction and intralesional hemorrhage. Post‐ operative course was unremarkable. Discussion: While hepatic adenomas are most commonly found in young women using oral estrogens, they are unusual in a teenage male. There is a higher incidence in patients with glycogen storage disease, diabetes mellitus, hemochromatosis, and anabolic steroid use. Our patient had no known risk factors. The greatest risks of hepatic adenoma are hemorrhage and malignant transformation. Treatment is surgical. Hepatic adenoma, while rare, should be considered in the differential diagnosis of a liver mass in an adolescent male. 39 A RARE FORM OF HISTIOCYTOSIS CAUSING CHOLESTASIS P. Minar, K. Jensen, V. Goh, V. Biank, Pediatric Gastroenterology and Nutrition, Medical College of Wisconsin, Milwaukee, WI; A 27 day term, female was transferred for evaluation of tachypnea, pallor and massive hepatosplenomegaly. Exam:tachycardic, tachypnic, hypoxic requiring 1/2 L of oxygen; pallor; no jaundice, petechiae or bruising. Liver span was 9 cm with splenomegaly. Labs: WBC 75.7 K/uL; Hgb of 5.9 g/dL; platelets of 7 K/uL. Bone marrow excluded malignancy. Infectious, metabolic and immune workup was negative. Ultrasound showed hepatomegaly with normal flow, a gallbladder with sludge and wall thickening, no ductal dilatation Direct bilirubin increased from 0 to 9.5mg/dL over 24 hours and peaked at 28.7 mg/dL. U/S showed no obstruction. Liver biopsy showed mononuclear and polymorphonuclear infiltrate without fibrosis or bile plugs. Central veins and sinusoids contained cell aggregates with pale gray eosinophilic cytoplasm and abnormal nuclei. Clusters of degenerated hepatocytes were accompanied by neutrophils. Immunohistochemistry staining was positive for CD68, S100, CD163, Factor XIIIa and negative for CD1a. Histiocytes on liver biopsy were immunoreactive to Anaplastic Lymphoma Kinase (ALK) diagnosing ALK + Non‐langerhans cell histocytosis (NLCH). Initially, she required oxygen, daily platelet and twice weekly PRBC transfusions. Treatment with prednisolone (40mg/m2) for 8 weeks and vinblastine for 6 weeks resolved the oxygen need, cholestasis, anemia and thrombocytopenia. ALK+ NLCH: NLCH describes a rare group of disorders defined by accumulation of histiocytes that do not meet phenotypic criteria for diagnosis of langerhan’s cell histiocytosis and are differentiated based on their clinical presentation and immunohistochemistry. This is the 4th documented case of ALK+ NLCH. ALK is an insulin receptor family of cell membrane spanning receptors that display intrinsic tyrosine kinase activity. Aberrant expression and/or activation of ALK results in a spectrum of malignancies: ALK+ large B cell lymphomas, inflammatory myofibroblastic tumors and non‐small cell lung cancer. This case emphasizes the role of immunohistochemistry staining when confronted with abnormal liver specimens. 40 RECESSIVE TWINKLE MUTATION PRESENTING AS ACUTE LIVER FAILURE V. Goh, K. Jensen, K. El‐ Chammas, G. Telega, V. Biank, Pediatric Gastroenterology and Nutrition, Medical College of Wisconsin, Milwaukee, WI; 10 wk‐old presented with feeding intolerance, jaundice, and respiratory distress. She was born term without complications but hospitalized at 1wk of age for lethargy. Metabolic work up was normal and symptoms were attributed to transient hyperammonemia of the newborn. At 2 month visit, was documented as thriving without concerns. Family history: unremarkable with 5 healthy siblings. Parents not consanguineous. Exam:Weight and length at 1%. She had scleral icterus without dysmorphic features and was in respiratory distress. Her abdomen was distended with liver palpated 5cm below costal margin; no splenomegaly. Labs:Hgb 7.8g/dL, platelet 527K/µL, total & direct bilirubin 10.9/7.6mg/dL, AST 1033 IU/L, ALT 634 IU/L, GGT 77 IU/L, albumin 2.5g/dL, LDH 2633 IU/L, Alkaline phosphatase 1084 IU/L, ammonia 29µM/L, INR 15.22, PTT 60sec, fibrinogen <60mg/dL, D‐dimer 10.12µg/dL, ferritin 973ng/mL, alpha fetoprotein 84800ng/mL. Hospital Course:CD was diagnosed of acute liver failure. Infectious, toxicology and metabolic workup including hemachromatosis and hemophagocytic lymphohistiocytosis was negative. She was later noted to have dysconjugate gaze. Brain MRI was normal but she failed visual and auditory evoked potential testing. At 4 months old, she developed Fanconi syndrome. Liver biopsy showed active cirrhosis with bile ductular proliferation, cholestasis, diffuse hemosiderosis and scattered macrovesicular steatosis. Mitochondrial content in the liver was 17% of mean value. Genetic workup revealed mitochondrial DNA depletion with two deleterious mutations in the TWINKLE gene c.85C>T (p.R29X) and c.1523A>G (p.Y508C). She passed away at 6 months from sepsis and multiorgan failure. Autopsy revealed no histo‐ pathological changes in the brain, spinal cord, skeletal muscle, peripheral nerves or heart Few cases of recessive TWINKLE mutations with infantile onset spinocerebellar ataxia (IOSCA) have been reported. Our case is significant as this is the first report of IOSCA associated with a recessive TWINKLE mutation presenting with marked biliary cirrhosis and Fanconi syndrome. 41 AUTOIMMUNE CHOLANGITIS IN A 3 YEAR OLD PATIENT S.H. Ibrahim, D.K. Freese, Pediatric Gastroenterology and Hepatology, Mayo Clinic, Rochester, MN; L. Zhang, Anatomic Pathology, Mayo Clinic, Rochester, MN; Immunoglobulin G4 (IgG4)‐associated cholangitis (IAC) is a steroid responsive biliary disease, often associated with autoimmune pancreatitis. It is characterized by elevation of serum IgG4 and infiltration of IgG4 positive plasma cells in bile ducts. This condition is well‐recognized in adults, but has not been previously described in pediatric patients. We report a 3‐ year‐old female patient with IAC. The patient presented with hepatomegaly, jaundice, and abdominal distention. There was no evidence of IBD. Laboratory studies revealed markedly elevated ALT, alkaline phosphatase, and mildly increased bilirubin. ANA, SMA and IgG4 levels were increased. Liver biopsy revealed mild focal interface activity, focal mild lymphocytic cholangitis, and positive IgG4 immunostaining. GGT and alkaline phosphatase were increased out of proportion to ALT and AST. MRCP showed diffuse thickening of intrahepatic ducts, but did not have features characteristic of PSC. She had intermittent elevation of amylase and lipase, but pancreas appeared normal on MRCP. The patient responded initially to prednisone and azathioprine, but relapsed upon tapering of prednisone. Addition of vancomycin to her regimen resulted in improvement of her liver enzymes. Follow up MRCP was normal and repeat liver biopsy showed resolution of inflammation, absent IgG4 staining and no progression of fibrosis. We speculate that the incidence of IgG4 associated cholangitis in the pediatric population might be higher than previously recognized. Screening for this condition in children with suspected overlap syndrome may be indicated, especially for patients with pancreatic involvement and/or no evidence of IBD, and may identify a subset of patients more responsive to immunosuppression. The course and prognosis of IAC in the pediatric population needs to be clarified in the future. 42 ARC SYNDROME, ANOTHER CAUSE OF LOW‐GGT CHOLESTASIS M.K. Jensen, V. Goh, G. Telega, V. Biank, Pediatric Gastroenterology, Medical College of Wisconsin, Milwaukee, WI; History: EG is a 43 day‐old Hispanic female with jaundice & failure to thrive since 2 weeks‐old. Mom denied acholic stools, dark urine, fever, bleeding or bruising. EG was born at 38 weeks via repeat c‐section. She failed her hearing screen, but has been well without hospitalizations or surgeries. She lives with parents and healthy 4‐year‐old sister. Family history identified a previous sister who died at 7 months of age from Arthrogryposis, Renal dysfunction and Cholestasis (ARC) syndrome. Parents are not consanguineous. Exam: EG weighed 3.85kg(10%) with length 51.7cm(3%). She had scleral icterus, low set ears, but was not dysmorphic. Cardiopulmonary exam was normal. Her liver edge was palpable 3cm below right costal margin, without splenomegaly. Stools were acholic. Labs: Hemoglobin 8.5, INR‐1.0, Protein 5mg/dL, albumin 3mg/dL, Total & conjugated bilirubin 8.9 & 7.4mg/dL, alkaline phosphatase 1,329IU/L, Free T4‐normal with elevated TSH. GGT 24. UA‐positive glucose, protein & bilirubin. Immune workup demonstrated elevated B‐cells . Urine bile acids were elevated. Ultrasound showed normal gallbladder and bile duct. Scintigraphy demonstrated uptake but no excretion. Genetic testing confirmed a homozygous nonsense mutation (c.1498G→T; p.Glu500x) in exon 20 of the VPS33B gene consistent with ARC syndrome. Disease progression: She was frequently admitted for fever without source and passed away at 9months from respiratory failure complicated by worsening renal and hepatic function with intermittent bleeding from thrombocytopenia. Contractures never developed. ARC syndrome: An autosomal recessive condition causing low GGT cholestasis with renal dysfunction. Arthrogryposis is less frequently documented. Other common findings include: sensorineural deafness, elevated TSH with normal T4 and an increased susceptibility to infections. Patients have increased bleeding risk with >50% developing significant bleeding. Patients are developmentally delayed, have failure to thrive and average life expectancy of 7months. Death is frequently due to respiratory illnesses and/or sepsis. 43 LIVER FAILURE FROM COPPER OVERLOAD IN A 6 YEAR OLD M.G. Bartlett, Pediatric Gastroenterology, University of Minnesota, Minneapolis, MN; A six year old boy with autism presented with gradual onset of fatigue. The family had previously sought treatment for his autism from numerous caregivers including a chiropracter who provided him with dietary supplements thought to improve his communication skills. After multiple visits to primary care providers he was admitted to the hospital with hepatosplenomegaly, elevated transaminases, and anemia. At the time of presentation his ALT was 214, AST 707, GGT 353, total bilirubin 1.1 mg/dl, and hemoglobin 8.7 mg/dl. Further evaluation found positive ANA and positive F‐Actin Antibody IgG test. He also had borderline low Ceruloplasmin (24 ug/dl). A 24 hour urine copper test was elevated (218 mcg/dl)and increased significantly after penicillamine challenge test (1480 mcg/dl). Liver biopsy demonstrated pericellular fibrosis and cirrhosis and special orcein staining showed abundant copper. A dry copper weight of 1430 mg/gram (normal 10‐25) was considered diagnostic of Wilson's Disease. He was started on trientene and zinc. Within one week of admission he developed liver failure with INR rising to 6 and total bilirubin elevated to 46.3 mg/dl. He was listed status 1A for liver transplant and received a deceased‐donor split liver on day 3. Since he has 4 siblings, genetic testing was done with complete sequencing analysis of the coding region for the ATP7B gene. No known mutations for Wilson's Disease were found. One single amino acid substitution was found which may represent a previously unknown mutation, although it occurs in a region of the gene that does not code for a known functional domain. Review of his dietary history, which included 3 separate supplements all mixed with chocolate milk,show his approximate daily copper intake to be 3‐4 mg, tenfold the recommended daily allowance. This case may be classic Wilson's Disease with an undetected mutation, a heterozygote for Wilson's with a newly dicovered mutation, or copper toxicity in a child predisposed to injury from autoimmune hepatitis. 44 GLYCOGENIC HEPATOPATHY: HEPATIC COMPLICATION OF POORLY CONTROLLED DIABETIS MELLITUS V.V. Gopalareddy, M. Parker, R. Caicedo, J. Dranove, V. Pineiro, Pediatrics, Levine Children's Hospital at Carolinas Medical Center, Charlotte, NC; W. Ahrens, Pathology, Carolinas Medical Center, Charlotte, NC; A 12 year old boy presented with hepatomegaly of unknown duration. He had a past medical history significant for poorly controlled Type 1 diabetes,diagnosed two years ago. He complained of upper abdominal fullness and intermittent emesis for about 4 weeks. Examination revealed distended upper abdomen and a massive tender hepatomegaly. Lab work revealed AST 849, ALT 568, alkaline phosphatase 404, total bilirubin 1.2, direct bilirubin 0.2, albumin 4, GGTP 191. HbA1C level was elevated at 10.2%. Ultrasound abdomen performed revealed marked hepatomegaly, mild splenomegaly with no focal lesions nor ascites. Hepatic arteries, portal venous wave forms were normal. Liver biopsy showed abundant glycogenated hepatocytes with no significant inflammatory activity, necrosis nor steatosis. Better glycemic control over the next 3‐4 weeks lead to a marked decrease in hepatomegaly and normalization of transaminases. Discussion: Glycogenic Hepatopathy (GH) most often occurs in individuals with type 1 diabetis and poorly controlled blood sugar. It is also known to occur following short term high‐dose steroid therapy.GH results from excess accummulation of glycogen in hepatocytes, occuring when marked or prolonged hyperglycemia is treated with insulin. Glucose in the sinusoidal blood is rapidly taken upbyhepatocytes, followed by rapid conversion to glycogen, which is then trapped within the liver. The most severe form of GH is Mauriac syndrome, consisting of growth retardation, delayed puberty, hepatomegaly and cushingoid features. Key clinical features are hepatomegaly and mild to markedly elevated transaminases, rarely ascites, which typically return to normal with adequate blood sugar control. Histologic features include marked glycogen accumulation leading to pale, swollen hepatocytes, no or mild fatty change/inflammation/spotty lobular necrosis and intact architecture with no significant fibrosis. The pathology is distinct from steatohepatitis. GH is often under recognized by clinicians. 45 CHRONIC MALARIA MIMICKING AUTOIMMUNE HEPATITIS V.V. Gopalareddy, A. Ahmed, R. Caicedo, J. Dranove, V. Pineiro, Pediatrics, Levine Children's Hospital at Carolinas Medical center, Charlotte, NC; W.A. Ahrens, Pathology, Carolinas Medical Center, Charlotte, NC; J. Menendez, Transplant Surgery, Carolinas Medical Center, Charlotte, NC; A 7‐year old girl adopted from Liberia was referred for elevated liver enzymes. She had a firm spleen measuring 5 cm and a mild hepatomegaly. Tests revealed AST of 226 IU/L, ALT of 126 IU/L, bilirubin of 0.8 mg/dl, albumin of 3.5gm/dl. IgG level was 2.6gm/dl. Chronic hepatitis evaluation was otherwise negative. ANA was 1: 40 positive, SMA was 1: 64 positive, AMA was 1:55 positive. Abdominal ultrasound revealed a normal sized liver with an enlarged spleen. Liver biopsy revealed chronic mild lobular and portal hepatitis with no significant fibrosis. The patient was diagnosed with AIH‐Type 1 and Prednisone 2mg/kg/day was initiated. Her AST and ALT normalized within 3 weeks on prednisone therapy and autoantibody levels also improved. In the interim she developed daily fevers with headache and was diagnosed with malaria. Repeat analysis of liver biopsy revealed the presence of Hemazoin pigment, typically seen in malaria. Steroid therapy for autoimmune hepatitis was discontinued and malarial treatment with quinine 10mg/kg/dose TID for 3 days and doxycycline 100mg BID for 7 days was completed. Serial LFT monitoring over the subsequent 6 months has been normal off all immunosuppressive therapy. Discussion: Autoimmunity can be seen in malaria and other infectious processes. It can present as chronic hepatosplenomegaly, mimicking autoimmune hepatitis. This case study suggests that autoimmune hepatitis can be misdiagnosed even when International Autoimmune Hepatitis Group (IAHG) score is used. Hypergammaglobulinemia, autoantibodies and immune‐mediated liver damage can be seen in cases of infectious etiologies including viruses, leishmaniasis and malaria. This case study supports the need for histologic confirmation of diagnosis even in the setting of high‐score autoimmune hepatitis. 46 LEFT VENTRICULAR ANEURYSM IN A PEDIATRIC LIVER TRANSPLANT RECIPIENT FOLLOWING PRESUMED COCAINE INGESTION. V.V. Gopalareddy, R. Caicedo, J. Dranove, W. Tsai, D. Bailey, V. Pineiro, Pediatrics, Levine Children's Hospital at Carolinas Medical Center, Charlotte, NC; J. Menendez, L. Eskind, Transplant Surgery, Carolinas Medical Center, Charlotte, NC; L. Watts, Cardiovascular Surgery, Levine Childrens Hospital at Carolinas Medical Center, Charlotte, NC; A 23‐month old African‐American male, who underwent liver transplantation for biliary atresia, presented 5 months after transplant with low grade fever, decreased level of activity and cough. The chest examination was unremarkable, with the exception of fine crackles. Chest radiograph demonstrated an enlarged cardiac silhouette compatible with cardiomegaly or pericardial effusion. An echocardiogram demonstrated a large pericardial effusion with early diastolic collapse of the right ventricle and right atrium. The child was immediately admitted to the pediatric intensive care unit where a pericardiocentesis was performed. After insertion of pericardial drain, the patient developed ventricular dysrhythmias and hemodynamic instability requiring intravenous inotropic support. A repeat echocardiogram revealed a left ventricular apical aneurysm. Review of the patient’s social history revealed the child had been found carrying a bag of cocaine and had possible ingestion. Serial echocardiograms demonstrated increasing size of the ventricular aneurysm. Due to risk of rupture and sudden death, the child underwent surgical repair with temporary cardiopulmonary bypass. Recovery was uneventful with stable liver functions throughout hospitalization. The patient was discharged to the maternal grandmother with normal cardiac and hepatic function 9 days after his surgery. Discussion: Ventricular aneurysm in a pediatric liver transplant patient is extremely rare. We found no other reports in the literature with this association. Ventricular aneurysm due to coronary spasm and infarction with cocaine exposure is well recognized. Prompt recognition and surgical intervention in the pediatric liver transplant population can lead to uneventful cardiac recovery and stable liver function. 47 UNIQUE CAUSE OF CHRONIC ABDOMINAL PAIN T.L. Sutton, , Walter Reed Army Medical Center, Washington, DC; AD is a 13 year old white female with a long history of chronic abdominal pain. The dull, but intense, pain involves her entire abdomen and is triggered by eating. Occurring in discrete episodes, it typically lasts 2‐3 days. She is completely well between episodes. Her physical exam is unremarkable. Two extensive workups revealed normal laboratory, endoscopic, and histologic findings. Abdominal radiography showed fecal retention. She experienced transient improvements with various treatments for functional abdominal pain and constipation. Due to a change in the quality of her pain, a repeat examination was performed. Abdominal ultrasound identified a cystic mass near the gallbladder neck. MRCP confirmed a Type II choledochal cyst. She underwent a laparascopic cyst excision, cholecystectomy with biliary reanastamosis. She experienced complete resolution of her symptoms. Choledochal cysts are rare cystic dilations along the biliary tree. The current classification scheme divides them into 5 subtypes. Type II, characterized by discrete diverticula along the extrahepatic biliary ducts, account for only 2% of choledochal cysts. These cysts are unique from other choledochal cysts in their pathogenesis and outcome. Though the exact pathogenesis is unknown, they are thought to arise from true diverticula of the common bile duct or duplication cysts. Clinically, most children and adults are asymptomatic or present with only one symptom, chronic or intermittent abdominal pain being the most common. Abdominal ultrasound is very sensitive in indentifying choledochal cysts. MRCP is now considered the gold standard for confirming diagnosis and delineating the biliary anatomy prior to surgery. For Type II cysts, complete cyst excision with or without cholecystecomy is recommended. In contrast to the other types of choledochal cysts, there is minimal increased risk of subsequent cancer. This case highlights a unique cause of chronic abdominal pain and underscores the importance of maintaining a high index of suspicion in these patients. An abdominal ultrasound is safe and has a high sensitivity in identifying choledochal cysts and should be considered in this population. 48 A RARE CASE OF JUVENILE POLYPOSIS SYNDROME T.L. Sutton, P.L. Rogers, , Walter Reed Army Medical Center, Washington, DC; Juvenile polyposis syndrome (JPS) is a rare disorder defined as the presence of more than 5 juvenile polyps in the colon or multiple juvenile polyps throughout the gastrointestinal tract. Further, it is associated with an increased risk of cancer. These hamartomatous polyps have a characteristic appearance and histology. We report on a patient with JPS who presented at 8‐months‐of‐age (mo) with severe hypoproteinemia and anemia. By maternal report, he began experiencing hematochezia at 2 mo. Subsequently, he passed a polyp at 6 and 7 mo. EGD and colonoscopy revealed a few duodenal polyps but numerous distal ileal and colonic polyps; appearance and histology were consistent with juvenile polyposis. Despite regular endoscopic sessions, he continued to manifest severe protein losing enteropathy, hypogammaglobulinemia associated with numerous respiratory infections, hyponatremia, hyperaldosteronism, hypertension, anemia, and malnutrition, attributable to his polyp burden. Treatments included regular IVIG and albumin infusions. At 14 mo, a total colectomy was performed after he presented with an incarcerated inguinal hernia due to an entrapped polyp. Postoperatively, his polyp growth slowed for 4‐6 month with subsequent rapid development of new polyps throughout the remaining bowel and stomach. Of particular note, he developed 2 perianal juvenile polyps. In contrast to the clinical progression of his disease over the years, his albumin and immunoglobulin levels have stabilized and he no longer requires intervention. Developmentally, the patient has significant delays and has been diagnosed with autism. This case is unique for many reasons. First, our now 7‐year‐old patient is the longest surviving patient presenting with infantile juvenile polyposis. Second, he has no family history of polyps and has tested negative for PTEN, SMAD4, and BMPR1A mutations, which are associated with severe hamartomatous polyposis disorders. Third, this case provides information on the natural history of an often fatal condition and may offer insight on the medical management of juvenile polyposis syndrome. 49 COLON CANCER IN A 10‐YEAR‐OLD GIRL: A RARE SUSPECT V.V. Gopalareddy, C. Jacobson, R. Caicedo, J. Dranove, V. Pineiro, Pediatrics, Levine Children's Hospital at Carolinas Medical Center, Charlotte, NC; W. Ahrens, Pathology, Carolinas Medical Center, Charlotte, NC; A 10 year old Caucasian girl presented with a 3 week history of intermittent non bilious vomiting and periumbilical crampy abdominal pain. There was no history of constipation or bloody diarrhea. She also reported fatigue and six pound weight loss. She denied fever, rash or swellings. The physical exam was unremarkable. Complete blood count, metabolic panel and sedimentation rate were all normal. Fecal occult blood testing was negative. Computed tomography of the abdomen revealed fluid filled loops of distal small intestine, with dilation of right hemicolon, with a focal segment of thickened bowel at the transition point near the hepatic flexure.Colonoscopy revealed a non‐bleeding, firm, 3cm long sessile mass, completely obstructing the lumen at the level of hepatic flexure. Biopsy confirmed a signet‐ring cell adenocarcinoma of the colon. Wild‐type KRAS mutation and elevated carcino embryonic antigen (CEA) of 25.5ng/ml were noted. Genetic studies for hereditary nonpolyposis colon cancer (HNPCC) and Li‐Fraumeni syndrome mutations were negative. She underwent a laparoscopic‐ assisted right hemicolectomy for Stage IV colon cancer. Chemotherapy regimen with avastin, oxaliplastin, 5‐flurouracil and leucovorin was started. Unfortunately at the end of 12 cycles of chemotherapy, she developed local recurrence with diffuse peritoneal seeding Discussion: Colorectal cancer is rare in children and only a few cases have been described. Most cases present in 2nd decade. Only 12%‐20% of these cases occur in children 10yrs of age and under. Inflammatory bowel disease and hereditary disorders are known to predispose individuals to colorectal cancers.The most common site of primary colorectal tumors in children is the transverse colon. The majority of colorectal cancers are adenocarcinomas. Primary signet‐ring cell carcinoma, a rare subtype of mucinous adenocarcinoma, is characterized an abundance of intracellular mucin, giving the cells a ‘signet ring’ appearance. It has a poor prognosis in children with few 5‐year survivors. 50 HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS IN A PATIENT WITH CROHN'S DISEASE AND A SUBTHERAPUTIC 6‐MERCAPTOPURINE LEVEL E. Kutsch, S. Khan, Gastroenterology , AI duPont Hosp, Wilmington, DE; C. Frantz, Hematology, Al duPont Hosp, Wilmington, DE; A 14 yo girl with Crohn’s disease, on 6‐mercaptopurine (6‐MP), admitted with fever and fatigue for 3 wk, and positive monospot. Exam notable for fever 38.9C, hypotension, abdominal distention and palpable spleen. Lab work showed WBC 1.1, ANC 500, Hgb 7.1, plt 37, normal peripheral smear, EBV PCR 833. Compliance with 6MP was previously questioned. Her 6‐TGN level was 77, 6‐MMPN level undetectable. On admission, 6‐MP was held due to possibility of myelosupression. Other abnormal labs noted: albumin 2.3, fibrinogen 97, ferritin 468, TG 138, coagulopathy and persistent cytopenias. She was negative for CMV and other viral hepatitides. Abdominal CT revealed bilateral pleural effusions, splenomegly with focal infarction, gallbladder edema, moderate ascites. Bone marrow (BM) biopsy showed 1+ hemophagocytosis with significant excess of macrophages. She had low NK cell function. She was diagnosed with acquired hemophagocytic lymphohistiocytosis (HLH). Genetic testing (MUNC13‐4, STX11 and PRF1) was negative. She completed 8 wks of dexamethasone and etoposide, with HLH resolution. Six months later, EBV PCR level was 1. She is in clinical remission of Crohn’s disease on maintenance 5‐ ASA therapy. HLH is a rare, life‐threatening, but potentially reversible disease in which the immune system is inappropriately stimulated rendering it highly ineffective and overwhelming. Her clinical features, labs, and BM results are consistent with HLH. The presence of EBV and negative HLH genetics support the diagnosis of acquired HLH. Conceivably, she is at risk due to underlying Crohn’s disease and its alterations of the immune system, but it is unclear as to what role 6MP may have played as her metabolites were undetectable. To our knowledge, previously reported cases of HLH in IBD were all associated with immunosuppressive therapy. This case raises concerns about the risk for a serious immunologic complication in IBD children who are not on immunomodulators. Our patient was successfully treated with chemotherapy and did not require antiviral therapy. 51 NEW ONSET PSEUDOTUMOR CEREBRI IN A CHILD TREATED WITH ADALIMUMAB FOR CROHN'S DISEASE. P.D. Wali, H. John‐Kelly, Pediatric Gastroenterology, A. I. duPont Hospital for Children, Wilmington, DE; Adalimumab is a recombinant, fully humanized immunoglobulin‐1 monoclonal antibody which has been shown to be efficacious in pediatric Crohn’s disease.We present the first known case of pseudotumor cerebri in a child with Crohn’s disease started on adalimumab. A 7‐year‐old female presented with a 4 month history of abdominal pain, chronic diarrhea and heme‐occult positive stool. An upper and lower endoscopy was performed showing pancolitis with an ulcerated terminal ileum. She was started on oral prednisone and mesalamine. After 1 month she was weaned off steroids and started on 6‐MP. Six months later she again had abdominal pain and bloody diarrhea. She was started on infliximab but developed abdominal pain, nausea and vomiting during an infusion. Methotrexate therapy also failed. She was then started on adalimumab induction, and progressed to maintenance therapy. She had resolution of her symptoms and normal inflammatory markers. After the fifth injection, she had a localized skin reaction which resolved spontaneously. After the seventh dose she started to complain of headaches, intermittent blurry and double vision. She was evaluated by a pediatric ophthalmologist and was found to have papilledema and sixth nerve palsy. MRI of her brain was normal. Spinal tap showed an elevated opening pressure of 29 cm of H2O, CSF studies were all negative. She was started on acetazolamide and had improvement of symptoms within 24 hours. Adalimumab was discontinued. Pseudotumor cerebri is defined as elevated CSF pressure above 20 cm of H2O, with normal CSF studies, normal sized ventricles and exclusion of other underlying abnormalities. Clinical features include headache, visual disturbances and papilledema. The goals of therapy are relief of symptoms and preservation of visual function. The most effective first line therapy is acetazolamide. Permanent optic atrophy is a severe complication when patients do not respond to medical therapy. Clinicians must be aware of possible neurologic complications of anti‐TNF therapy, including development of pseudotumor cerebri. 52 AUTOLOGOUS HEMATOPOIETIC STEM CELL TRANSPLANTATION (HSCT)IN A CHILD WITH REFRACTORY CROHN’S DISEASE (CD). C.S. Huang, G. Tenjarla, A. Lobdell, R. Scherr, B. Schoen, C. Sauer, K. Chiang, S. Kugathasan, Pediatric Gastroenterology, Hepatology and Nutrition, Emory University, Atlanta, GA; Many advances have been achieved in the treatment of CD during the past decade. Biologics including tumor necrosis factor antagonist and anti‐adhesion molecules have greatly improved our treatment options in CD. However, a subset of patients with CD will fail to respond to any available medical therapy. Surgical therapy may not be an option in those when the disease is diffuse throughout the GI tract with multi‐system involvement. Autologous HSCT represents a new hope as rescue treatment for patients with refractory CD. A 15 year old female diagnosed with CD involving her entire GI tract (esophagus, stomach, small and large bowel and perianal disease). She also had pulmonary disease with altered pulmonary function and bronchoscopy with tracheal ulcerations and granulomas. She also developed Pyoderma Grangrenosum involving both legs. She was admitted 10 times within 18 months due to CD exacerbations & complications of her disease. She failed 5‐ASA, steroids, immunomodulators (6‐MP and Methotrexate), anti–TNF(Infliximab and Adalimumab) and anti‐adhesion monoclonal antibody therapy (Natalizumab). She became TPN dependent secondary to intestinal failure. She had a colonic perforation during colonoscopy that left her with a colostomy. She underwent an elective autologous HSCT for refractory CD. Peripheral blood stem cells were mobilized with GCSF. Conditioning was achieved by Cyclophosphamide. Only toxicity observed immediate post‐transplant was a culture negative fever. Her recovery was complete and was assessed by several parameters including repeat colonoscopy which showed endoscopic remission. The colostomy was closed after 3 months post‐transplant. She was able to resume her normal life style after 2 years including her schooling. Conclusions: Autologous HSCT should be considered as a reasonable option for children with refractory Crohn’s disease as it resets the immune system. Long‐term follow up will be necessary to confirm the duration of the induced clinical remission. 53 NASAL SEPTAL PERFORATION: UNUSUAL MANIFESTATION OF ULCERATIVE COLITIS P. Mohanty, M. Beg, , SUNY Upstate Medical University, Syracuse, NY; Introduction: Ulcerative Colitis (UC) involves the rectum and colon with upper gastrointestinal involvement in 15‐ 69% of cases. Ear‐nose‐throat (ENT) manifestations are seen in inflammatory bowel disease (IBD), mainly Crohn’s disease. We report a case of nasal septal perforation in a patient with UC. Case description: A 10 year old Caucasian girl initially presented in 2003 with bloody diarrhea and weight loss. Physical examination showed disabling erythema nodosum and asymptomatic nasal septal perforation. Endoscopic and histologic diagnosis of UC was made. She remained refractory to standard medical therapy with prednisone, mesalamine, azathioprine and tacrolimus. She underwent a staged colectomy with ileostomy followed by ileoanal anastomosis with subsequent improvement of her gastrointestinal symptoms. Nasal examination revealed a smooth nasal septal perforation approximately 10mm in diameter. There was no history of previous trauma, nasal surgery, application of topical agents or environmental exposure to metals. Workup for vasculitides, collagen vascular disorders and Wegener’s granulomatosis was negative. A five year follow up examination showed a gradual decrease in the size of the septal perforation. We speculate that the nasal septal perforation was an extra intestinal manifestation of her underlying severe UC. Discussion: Nasal manifestations especially septal perforation in IBD is quite rare. Asymptomatic nasal perforation rarely requires any treatment. The uniqueness of this case is the association of nasal septal perforation in UC. Review of literature showed only two cases of nasal involvement in UC in adult patients; however none were reported in the pediatric population. Conclusion: This case has been reported to emphasize the importance of a careful physical examination of ear‐nose‐throat in IBD patients, including those with Ulcerative Colitis. 54 CAP POLYPOSIS IN PEDIATRIC PATIENTS R.H. Alkhouri, S. Sendupta, S. Desai, D. Gelfond, A. Khan, H. Hashmi, R. Baker, S. Baker, Digestive Disease and Nutrition Center, SUNY at Buffalo, Buffalo, NY; Introduction: Cap polyposis (CP) was first described in 1985 by Williams Bussey and Morson. The pathogenesis of CP is not completely understood, and it has not been described in pediatric population as an isolated finding. We describe two children/adolescents with CP. A 15 year old male presented with painless rectal bleed for one month. He had no evidence of helicobacter pylori infection, or long standing constipation. At colonoscopy multiple polyps were found, one of which was removed. His stool was positive for clostridium difficile and he was treated with metronidazole. Additional polyps were removed by a repeat colonoscopy after which his symptoms resolved. A 9 year old female presented with painless rectal bleed for 2 weeks. She had no history of constipation, and there was no evidence of H.pylori infection. She underwent a colonoscopy, one polyp was found in the rectum and removed. Upon follow up a month later, her symptoms resolved. Discussion: CP, clearly differentiated from juvenile polyps, is an uncommon condition that has been described as an isolated finding in adults, but not in pediatric population. These polyps are characterized by a distinct histopathological appearance, which includes a mixed inflammatory cell infiltrate and fibrinopurulent exudates together forming a “cap” over the body of the polyp. Most polyps are located in the recto‐ sigmoid area. The pathogenesis of CP is not understood, but it has been linked to constipation, rectal prolapse, and H.pylori infection. Treatment of CP includes endoscopic removal, surgical excision, H.pylori eradication, and treatment with metronidazole. Either the antibiotic or the anti‐inflammatory properties of metronidazole could have been responsible for its effectiveness. More recently, Infliximab has been effective in few cases. Polypectomy appears to have been curative in our cases. No long term follow up seems to be necessary. 55 NEUROLOGICAL DETERIORATION AS THE PRIMARY PRESENTATION OF CROHN’S DISEASE E.J. Rothbaum Perito, M.B. Heyman, Division of Pediatric Gastroenterology, Hepatology, and Nutrition, University of California‐SF, San Francisco, CA; E.J. Rothbaum Perito, E. Zaid, K. Hoffman, E. Stumpf, S. Wilson, M.B. Heyman, Department of Pediatrics, University of California‐SF, San Francisco, CA; Introduction: Approximately one‐fourth of children with inflammatory bowel disease (IBD) have extra‐ intestinal manifestations (EIM). Lack of clear intestinal symptoms accompanying EIM may delay diagnosis. Case Report: We report on a nine‐year‐old boy with two episodes of lower extremity weakness and pain, without joint swelling, following self‐limited diarrheal illnesses. Initial evaluation by a rheumatologist led to diagnosis of post‐infectious myositis. Seven months later a neurologist evaluated him for persistent symptoms and diagnosed proximal weakness and peripheral neuropathy. Magnetic resonance imaging of his spine and legs, cerebrospinal fluid analysis, and bone scan were normal. With the third episode, 11 months from initial symptom onset, the patient developed unilateral ankle swelling, eye redness, and diffuse abdominal pain without diarrhea. He had persistent proximal weakness, peripheral hyporeflexia, and diffuse leg pain. Laboratory tests revealed elevated erythrocyte sedimentation rate, mild iron deficiency anemia, and hypoalbuminemia. These findings raised suspicion for IBD. Endoscopy with biopsies revealed gastritis and severe colitis, consistent with Crohn’s disease. Electromyography and nerve conduction velocities were normal. The patient’s symptoms—including the lower extremity pain and weakness—resolved within three days after the initial induction dose of infliximab. Discussion: While arthralgias and arthritis are common causes of extremity pain in children with Crohn’s disease, presentation with primarily neuromuscular symptoms is rare and delayed diagnosis in this case. Previously reported causes of neuromuscular symptoms without joint swelling in IBD include peripheral neuropathy, myositis, and ankylosing spondylitis. Testing for these was negative in our patient. Awareness of cases in which EIM are severe but intestinal symptoms are subtle or absent is essential to minimize diagnosis delays. 56 CASE REPORT: RECTOCELE IN THE PEDIATRIC AGE GROUP M. Osman, Department of Pediatrics, Louisiana State University Health Sciences Center, Shreveport, LA; M. Brown, Department of Surgery‐Section of Pediatric Surgery, Louisiana State University Health Sciences Center, Shreveport, LA; M. Osman, Department of Pediatric Surgery, Ain Shams University, Cairo, Cairo, EGYPT; S. Hussain, Department of Pediatrics‐Section of Pediatric Gastroenterology, Louisiana State University Health Sciences Center, Shreveport, LA; Rectocele is protrusion of the anterior wall of the rectum. More common in females than male and never been described in the literature in children younger than 18 years of age. We are presenting three pediatric cases of Rectocele who presented by the complaint of constipation that was refractory to treatment. Diagnosis was achieved by radiological studies including defecography and barium enema. Two of these cases were treated surgically and one conservatively. Surgery completely cured the problem with uneventful Postoperative course. Further multicenter studies with the aid of radiological evaluation should be considered on children with hard to treat constipation to better describe that disorder in the pediatric age group. 57 11 YEAR OLD CHILD PRESENTING WITH CROHN’S DISEASE AND AUTO‐IMMUNE HEMOLYTIC ANEMIA G. Laroche, I. Hill, K. Buckley, Pediatrics, Wake Forest University Baptist Medical Center, Winston‐Salem, NC; Learning Objectives: 1. Increase attentiveness of Autoimmune Hemolytic Anemia associated with Crohn’s Disease. 2. The cause of Anemia can be multifactorial in patients with Inflammatory Bowel Disease. Case Report: An 11‐ year old previously healthy unimmunized Caucasian male presented to ED with 3 month history of abdominal pain and diarrhea. Patient had been having fevers along with bloody stools. Pediatrician had been checking his Hgb and noticed he was getting pale and had a significantly decreased Hgb. Because of concern that he was clinically worsening he was referred to our hospital for further evaluation. On Presentation: Patient is appeared pale dehydrated but alert and oriented, tachycardic, sunken eyes, conjunctival pallor, with soft, tender, distended abdomen. Significant Labs: Na: 127; K: 2.9; ESR: >119; CRP: 136.2; Protein 5.9; Albumin 1.8; WBC 14, 300; Hgb: 7.8; Hct: 22.5%; Granulocyte 4%; Band 50%; Lymphocytes 31%; Monocytes 12%; Retic 3.7% As a type and screen was done our patient was found to be Coombs Positive with a Warm Autoantibody Colonoscopy revealed pancolitis with thick, cobblestone appearance, ulcerations, and friability. Discussion: Autoimmune Hemolytic Anemia (AIHA) has been commonly reported as occurring along with Ulcerative Colitis. It has been less commonly associated with Crohn’s Disease especially in pediatric patients. Our case report describes a previously healthy unimmunized child presenting with fever, diarrhea, and anemia eventually diagnosed with Crohn’s Disease and AIHA with a warm antibody. The pathophysiology of the association between AIHA and IBD is not yet completely understood especially for Crohn’s Disease since its rare. Anemia in this child was multifactorial, contributed by anemia of chronic disease with minor contribution from AIHA. Transfusion is indicated in symptomatic patients. Treatment for both of these diseases is immunosuppression. Our patient was started on Prednisone, Pentasa, and Azathioprine. Colectomy maybe necessary if patient becomes refractory to medications. 58 SEVERE VULVAR LYMPHEDEMA AND PERIANAL LESIONS RELATED TO CROHN’S DISEASE C.A. Camacho, V. Velazquez, A. Mercado, Pediatrics, Hospital Episcopal San Lucas, Ponce, PR; A previously healthy 12 year old girl admitted with a 2 month history of abdominal pain and bloody stools for the last 3 days, denies anorexia, vomiting, fever, dysuria, cough, or rash. Patient was pale and wasted with a heart rate, of 120 beats/min. Physical exam of genitalia showed edematous, ulcerated, excoriated vulvar area and perianal wart like lesions skin tags and openlacerations. Hemoglobin of 7.9g/dl. Therapy was started with metronidazole, methylprednisolone, mesalamine, and pantoprazole. Peribulbar biopsy showed granulomatous lesions. Colonoscopy revealed mucosal inflammation from terminal ileus to proximal ascending colon; descending colon had diffuse, edema, exudates, nodularity, pseudopolyps, stricture and ulceration. She persists with profuse rectal bleeding during 4 days. Mesalamine dose was increased; and Cyclosporine and Cefotaxime were added to treatment. Cyclosporine levels were achieved in 4 days of 8mg/kg/day dose. Her condition improved after 15 days of treatment, and gained 12 pounds when she was discharge home. Vulvar lymphaedema due to metastatic vulvar Crohn's disease is a rare complication of CD that normally responds well to metronidazole therapy. Cyclosporine and Tacrolimus are alternative therapy in this condition. In this particular case, the patient received 13 days of Cyclosporine at a dose of 4mg/kg/day followed by 4 days at a dose of 8mg/kg/day to achieve levels at 200ng/ml and rectal bleeding remission. There was clinical improvement of vulvar edema and perineal lesions. Further studies about cyclosporine as part of the treatment in metastatic Crohn’s disease could open new doors for the future. References 1.Odes S. Vardi J, Friger M, et al. Cost análysis and cost determinants in a European inflammatory bowel disease inception cohort with 10 years of follow evaluation. Gastroenterology. 2006;131: 719‐ 728.. Markowitz J, Hyanns J, Mark D, et al. Corticosteroid therapy in the age of Infliximab; acute and one year outcomes in newly diagnosed children with Crohn’s disease. Clin Gastroenterol Hepatol. 2006; 59 UVEITIS AS AN INITIAL PRESENTATION OF CELIAC DISEASE E. Iofel, K. Soula, Pediatrics, UMDNJ, New Brunswick, NJ; 10 year old girl presented with diagnosis of uveitis after failing vision test in school. She had no gastrointestinal complaints. She denied fevers, joint pains, mouth ulcerations. PMH: unremarkable. FH: significant for IDDM in father. PE revealed an obese, well appearing child in no distress. Wt 52.6 kg (above 95th percentile) Ht 135.5 cm (75th percentile). Abdomen was soft, nondistended, nontender, without organomegaly or masses. Perianal area was unremarkable. Stool was guiac negative X3. ESR 34mm/hr, CRP1.8mg/dl. Complete blood count, iron studies and complete metabolic profile were normal. In order to rule out inflammatory bowel disease small bowel follow through, as well as fecal calprotectin test were performed and found to be normal. Parents refused endoscopy at that time. She returned six months later as her eye disease progressed, and she was treated with prednisone and adalimumab. Celiac disease (CD) screening was performed by the rheumatologist and revealed positive antiendomysial antibodies. HLA test was positive as well. EGD and colonoscopy were performed. Colonoscopy and ileoscopy were normal. EGD revealed abundance of intraepithelial lymphocytes in the duodenal biopsies, consistent with CD. Gluten‐free diet was instituted. Her antiendomysial antibodies normalized. Eye changes stabilized and prednisone was weaned off. She is currently in the process of weaning adalimumab. Three cases of uveitis as a manifestation of CD were found in the literature, none in children. Unlike other cases, our patient was completely asymptomatic with the exception of uveitis. Her mucosal biopsies were performed when patient was treated with both prednisone and adalimumab. This could have ameliorated severity of the mucosal lesion. Conclusions: 1.This rare presentation of CD should be remembered by a gastroenterologist than evaluating patients with uveitis. 2.CD patients presenting with vision changes should be evaluated by an ophthalmologist to rule out uveitis, especially those with poor disease control. 3. Recognition of this association could spare patients exposure to steroids and biologic agents, commonly used in refractory cases of uveitis. 60 GASTROINTESTINAL FOREIGN BODY: A HOLE IN ONE B. Maksimak, Medical Student, Philadelphia College of Osteopathic Medicine, Philadelphia, PA; J.N. Fussell, Pediatric Residency Program, Geisinger Clinic ‐ Janet Weis Children's Hospital, Danville, PA; M. Maksimak, Pediatric Gastroenterology and Nutrition, Geisinger Clinic ‐ Janet Weis Children's Hospital, Danville, PA; GH presented as a healthy 2 year old boy for a routine primary care visit. Mom mentioned to the family physician that the patient had increased stool frequency over the past few months to 3/day with no gastrointestinal bleeding, weight loss, abdominal pain or obstructive symptoms. The primary care physician noted GH to have a slightly distended abdomen on exam. An abdominal x‐ray was obtained to look for constipation, but it revealed a small radio‐opaque metallic nail in the right lower quadrant. Parents were unable to state when the object was ingested. Repeat x‐ray 4 days later showed no movement of the object. GH was referred to the pediatric GI clinic approximately 10 days after the initial x‐ray. Another KUB showed the object still in the right lower abdominal quadrant. A colonoscopy for evaluation and removal of the foreign body was scheduled for 5 days later along with a KUB just prior to the colonoscopy. Again the x‐ray showed the presence of the object in the right lower quadrant despite the colonoscopy cleanout. However, the head of the nail was oriented 180 degrees in the opposite direction. The colonoscopy failed to detect the object within the colon. An excellent view of the appendix and IC valve was obtained. The patient was taken to surgery later that day and the nail was found at the tip of a 5 cm appendix. There was no inflammation noted. An appendectomy was performed and the patient was discharged without complication 2 days later. Multiple x‐rays, colonoscopy pictures and surgical specimen photographs will be presented. 61 CELIAC DISEASE WITH INTERCURRENT EOSINOPHILIC ESOPHAGITIS A.R. Sicolo, L.J. Wozniak, M.E. Ament, Pediatric Gastroenterology, UCLA Mattel Children's Hospital, Los Angeles, CA; Case Report: 14 year old male with type 1 diabetes mellitus and hypothyroidism diagnosed at age 3, presented with poor height (<5th %) and weight (10th %) gain, and having one large stool a day, which in the past year had a very strong and foul odor. There was no significant history of abdominal pain and vomiting. Given his history of type 1 DM, celiac markers were sent and he was scheduled for endoscopy. His anti‐transglutaminase antibody IgA was abnormal at 5 (normal <4) and endomysial IgA antibody was positive. EGD was performed and demonstrated duodenal bulb with nodularity and increased vascularity, thickened folds and white marbleized (mosaic) pattern. Interestingly, the esophagus also had white patches that were biopsied. His small bowel biopsies did not officially get classified as a type 1 Marsh lesion, but they were still considered to be abnormal (35 lymphocytes/100 enterocytes with villous blunting). Biopsies of the esophagus were significant for eosinophophilia (>30/hpf). He had no symptoms referable to his esophagus. On the basis of the biopsies and positive anti‐transglutaminase antibody, we placed the patient on a strict gluten‐free diet. There has been a major improvement in all of his GI symptoms. He is having only one bowel movement a day, bloating has resolved, and he no longer has abdominal pain. Discussion: Celiac disease (CD) and eosinophilic esophagitis (EE) are distinct disorders with specific clinico‐ pathological characteristics. Reports suggest an association between the two. A recent study demonstrated that the prevalence of EE in children with CD is at least 4%. Our patient was initially brought to medical attention because of symptoms related to his celiac disease and during endoscopy had lesions of suspicion in the esophagus which were biopsied and later confirmed to be EE. He had no dysphagia, reflux or any history of food being stuck in esophagus. Coexistent EE should be considered in children with suspected CD undergoing endoscopy. Routine esophageal biopsies may be warranted when investigating for celiac disease. 62 ACUTE PANCREATITIS AS THE INITIAL PRESENTATION OF BURKITT’S LYMPHOMA IN A SIX YEAR OLD BOY. H. Chamdawala, J. Xu, R. Gill, S. Schwarz, W.R. Treem, Pediatric Gastroenterology, SUNY Downstate Medical Center, Brooklyn, NY; J. Amodio, Pediatric Radiology, SUNY Downstate Medical Center, Brooklyn, NY; In children the most common causes of acute pancreatitis are viral infections, drug toxicity and trauma. Involvement of the pancreas with Non Hodgkin’s Lymphoma (NHL) with resulting pancreatitis has previously been reported only in adolescents and adults. We now report a case of Burkitt's Lymphoma (BL) with pancreatic involvement in a six year old boy presenting with acute pancreatitis. Case Report: A six year old boy presented with one week of abdominal pain, vomiting, jaundice and dark urine. He had abdominal tenderness and no palpable masses. Laboratory tests showed markedly elevated serum amylase 608 U/L, lipase 2484 U/L, total bilirubin 8.9 mg/dL, direct bilirubin 5.4mg/dL, ALT 272 U/L, AST 246 U/L, Alkaline Phosphatase 783 U/L, GGT 256 U/L and LDH 470 U/L. Ultrasound (US) showed dilated intra and extra‐hepatic bile ducts, a diffusely enlarged and hypoechoic pancreas with no evidence of pancreatic duct dilatation and severe left‐sided hydronephrosis. MRI showed a diffusely enlarged pancreas and two separate pelvic masses, one of which was obstructing the left ureter. The T1 and T2 signal characteristics of the entire pancreas and both the pelvic masses were identical on the MRI. CT guided biopsy of the pelvic mass demonstrated BL. The patient was started on chemotherapy based on the FAB/ LMB96 protocol which resulted in rapid resolution of his abdominal pain, vomiting and jaundice. Ten days into the treatment, laboratory tests showed normalization of his serum amylase 130 U/L, lipase 6 U/L, total bilirubin 1.4 mg/dL and direct bilirubin 0.5 mg/dL. Repeat US showed a dramatic reduction in the pancreatic size. Conclusion: We report a case of BL presenting with diffuse pancreatic involvement and clinical features of acute pancreatitis in a six year old boy. Although rare, BL should be included in the differential diagnosis of acute pancreatitis in a young child. 63 PEDIATRIC PANCREATIC PANNICULITIS A. Chawla, C. Swandal, R. Boykan, Pediatrics, Stony Brook Medical Center, Stony Brook, NY; Pancreatic panniculitis is an uncommon sequelae of both acute and chronic pancreatitis, and is even more rare in the pediatric population. It is hypothesized that the enzymes released with pancreatitis permeate into the body, leaking into areas containing subcutaneous fat, causing necrosis. Regardless of the etiology of the pancreatitis, the development of an associated panniculitis appears to retain similar, distinct properties. The initial manifestations include the development of red, painful nodules on the lower extremities that may ulcerate, leading to drainage of a clear to brownish fluid. We present a 10‐year‐old female with multiple medical problems including holoprosencephaly, diabetes insipidus, temperature instability and chronic pancreatitis. She presented with progressive abdominal pain, nausea and vomiting of 3 to 4 weeks duration. She was found to have acute pancreatitis originating from recent abdominal blunt trauma, amylase level of 902 IU/mL (normal 40‐125 IU/mL) and a lipase level of 3090 IU/mL (0‐59 IU/mL) were noted. A CT revealed a 4.0 cm pseudocyst in the area of the pancreatic neck. Pancreatic enzyme levels remained elevated with amylase of 889 IU/mL and lipase of 2409 IU/mL. A month later, the patient presented with painful, red‐brown nodules on the extensor surfaces of her lower legs as well as acutely inflamed joints in her fingers and toes with severe swelling and areas of spontaneous skin rupture. All cultures from these sites were negative. Abdominal MRI showed inflammatory changes around the pancreas, an enlarging pseudocyst, and dilation of the main pancreatic duct distal to the cyst cavity. Repeat amylase and lipase levels were 1897 IU/mL and 3885 IU/mL respectively. Biopsy of the left great toe revealed findings consistent with pancreatic panniculitis. Following endoscopic decompression of the pancreatic pseudocyst, and starting of G‐J tube feeds, repeat imaging at 6 months showed a normal pancreas, with complete resolution of the panniculitis. Though rare in the pediatric population the treatment of pancreatic panniculitis is still supportive and directed at the underlying pancreatic disease. 64 COMBINATION OF CFTR GENE MUTATION AND AUTOIMMUNE PANCREATITIS PRESENTING AS NECROTIZING PANCREATITIS H. Patel, J. Levine, T. Weinstein, Division of Pediatric Gastroenterology and Nutrition, Cohen Children's Medical Center, NSLIJ Health System, New Hyde Park, NY; Autoimmune pancreatitis (AIP) and idiopathic pancreatitis secondary to decreased function of cystic fibrosis transmembrane conductance regulator (CFTR) gene have been reported as 2 distinct etiolgies of pancreatitis. AIP is frequently described as a mild recurrent pancreatitis. CFTR gene mutations have been associated with pancreatitis when no other etiology is found. AIP with concurrent CFTR gene mutation appears to worsen symptoms as shown by our case. A 16 yo male with recurrent acute pancreatitis presented with severe epigastric pain, fever, emesis, and elevated amylase and lipase. The patient was diagnosed with necrotizing pancreatitis by CT scan and MRI which showed pancreatic phlegmon with necrosis. Initial workup revealed heterozygous CFTR gene polymorphism R75Q, a pancreatic sufficient variant with undetermined clinical significance. His cationic trypsinogen (PRSS‐1), secretory trypsin inhibitor (SPINK‐1) and sweat test were nl. He was diagnosed with idiopathic pancreatitis attributed to his CFTR gene variant and treated with pancreatic enzymes and dietary restriction with resultant improvement. Repeated bouts of pancreatitis and multiple hospitalizations led to further investigation, including an ERCP demonstrating a small and fibrotic major papilla and a stricture in the supra‐ampullary portion of the pancreatic duct. ERCP findings raised suspicion for AIP. Further work up revealed a positive ANA and elevated IgG4 level (3x upper limit nl). The diagnosis of AIP was established based on Japan Pancreas Society criteria given the patient's ERCP, lab and clinical findings. AIP is an uncommon cause of pancreatitis in the pediatric population, as is idiopathic pancreatitis associated with CFTR gene polymorphism. It has been suggested that the presence of polymorphism R75Q may predispose patients to pancreatitis when present with another gene mutation. The combination of a CFTR gene mutation and AIP may lead to severe pancreatic disease as seen in our patient who presented with necrotizing pancreatitis. 65 ACUTE RECURRENT PANCREATITIS IN A CHILD HETEROZYGOUS FOR THE N34S SPINK1 GENE MUTATION S. Honigbaum, V. Weerasooryia, M.L. Loscalzo, M.J. Wilsey, , University of South Florida College of Medicine, Tampa, FL; S. Honigbaum, V. Weerasooryia, M.L. Loscalzo, M.J. Wilsey, , All Children's Hospital, St. Petersburg, FL; Acute recurrent pancreatitis is defined as at least two episodes of acute pancreatitis per year or more than three episodes over a lifetime in patients without chronic pancreatitis. Pancreatic enzyme levels decline over 3–4 days and often no specific cause is found. Mutations in the secretory trypsin inhibitor (SPINK1) gene have been found to be associated with both hereditary and chronic pancreatitis. However, there are no previous reports of SPINK1 mutations in pediatric patients with either acute pancreatitis or acute recurrent pancreatitis. We present the case of a child with acute recurrent pancreatitis found to be heterozygous for the N34S mutation of the SPINK1 gene. CASE REPORT: Patient is a previously healthy 12 yr old female who presents with a two‐day history of acute onset nausea, vomiting, and abdominal pain. She denied constipation, diarrhea, or bloody stools and there was no family history of pancreatitis. She first presented to an outside hospital with an amylase of 266 and lipase of 1036. Abdominal U/S was normal and her pancreatic enzymes quickly normalized with supportive care and she was discharged home. Patient was re‐admitted 12 days later with similar presenting symptoms and an amylase of 573 and a lipase of 330. EBV titers were equivocal, and CMV titers, triglycerides and MRCP were normal. The patient was discharged home three days later. Gene testing showed heterozygous change in pN34S in exon 3 of the SPINK1 gene. CFTR, PRSS1 and CTRC gene testing were normal. CONCLUSIONS: SPINK1 plays important role in protecting the pancreas against excessive trypsinogen activation and gene mutations have been associated with both hereditary and chronic pancreatitis. Because SPINK1 is a modifying gene, N34S mutations alone do not necessarily cause pancreatitis, but may act together with other genetic or environmental factors. We present a child with acute recurrent pancreatitis found to be heterozygous for the N34S mutation of the SPINK1 gene. 66 PANCREATITIS ASSOCIATED WITH ALPHA 1‐ ANTITRYPSIN DEFICIENCY O.F. Almadhoun, T. Rossi, Pediatric GI, University of Rochester Medical Center, Rochester, NY; Y. Lee , Pediatric Surgery, University of Rochester Medical Center, Rochester, NY; Alpha 1‐antitrypsin (A1AT) deficiency is a genetic disorder commonly associated with pulmonary and hepatic injury. While AIAT is frequently considered in the differential diagnosis of chronic pancreatitis, the literature contains conflicting support for this association.In a comparison study, low levels of this glycoprotein have been detected in adult patients with chronic pancreatitis than in healthy controls. We report a child with Pi‐ZZ phenotype A1AT deficiency and chronic pancreatitis complicated by chronic abdominal pain and poor weight gain. Our patient is a 5‐year‐old boy who had one year history of recurrent episodes of pancreatitis. He underwent multiple admissions and would exhibit very elevated amylase and lipase levels. In between episodes, he remained active, but he would rapidly relapse with severe pain for several days. He underwent extensive testing including viral studies, genetic tests for CFTR mutations,familial pancreatitis including SPINK‐1 and PRSS1 genes,IgG4 level, lipid profile were all normal. Abdominal CT scan and ultrasound and MRCP during the episodes demonstrated edematous pancreas. At ERCP a stent was placed and post procedure severe pancreatitis occurred. A subsequent ERCP for stent removal revealed mildly dilated main pancreatic duct with stricture in the neck of the pancreas. Endoscopic ultrasound (EUS)demonstrated pancreatic changes consistent with chronic pancreatitis. Fecal elastase was extremely low consistent with this condition. In completing the evaluation for an etiology of chronic pancreatitis, an alpha‐1 antitrypsin level was found to be low at 47 (N= >100 mg/dL).Protease inhibitor typing revealed ZZ phenotype indicating homozygous alpha‐1 antitrypsin deficiency.He underwent a pancreaticojejunostomy, which was a modified Puestow operation for treatment of the stricture. After the surgery, he overall improved clinically but continued with recurrent episodes of pain in a milder form. This case suggests that AIAT may be a definitive cause of acute recurrent pancreatitis in childhood. 67 SOLID PSEUDOPAPILLARY TUMOR OF THE PANCREAS IN AN ADOLESCENT FEMALE S. Goli, L. Pan, W.R. Treem, S.M. Schwarz, Division of Pediatric Gastroenterology, SUNY Downstate Medical Center, Brooklyn, NY; Background: Pseudopapillary tumors of the pancreas are rare neoplasms, primarily seen in young women presenting with abdominal pain and a palpable mass. Herein, we report an adolescent female with cholelithiasis, in whom an incidental pancreatic mass was found. Immunohistochemical staining techniques on the resected specimen confirmed the diagnosis of a pseudopapillary neoplasm. Case Report: An 18 year old obese female presented with nausea, vomiting and epigastric pain radiating to the back. Abdominal U/S demonstrated cholelithiasis with mild dilatation of the common duct (CBD) but no pancreatic abnormality. MRCP showed a 5 mm stone in the distal CBD and a non‐cystic, 2x2 cm mass in the tail of the pancreas. Endoscopic ultrasound (EUS) could not differentiate this mass from normal pancreatic tissue. ERCP with sphincterotomy achieved clearance of the CBD prior to exploration and cholecystectomy. At surgery, distal pancreatectomy and splenectomy (secondary to splenic involvement by tumor) were carried out. Routine histology could not differentiate between a pseudopapillary and a neuroendocrine tumor. However, monoclonal antibody immunohistochemical staining (beta‐catenin, CD10, CD56) clearly identified the mass as a pancreatic, pseudopapillary neoplasm. Conclusion: Primary pseudopapillary tumors of the pancreas may present incidentally on radiological exams. Failure of standard techniques, such as EUS or abdominal U/S to clearly delineate these masses is likely related to similar densities of neoplastic and benign tissue. Immunohistochemical staining techniques are required to permit differentiation between pseudopapillary and neuroendocrine pancreatic tumors. 68 ACUTE PANCREATITIS AS THE INITIAL PRESENTATION OF MELAS SYNDROME E.D. Rosas Blum, F. Navarro, Department of Pediatrics, Division of Pediatric GI, Hepatology and Nutrition, University of Texas HSC Houston, Houston, TX; BACKGROUND: Pancreatitis has been reported in metabolic diseases including organic acidurias, and rarely in patients with respiratory chain disorders. The mitochondrial myopathy, encephalopathy, lactic acidosis with stroke‐like episode (MELAS) syndrome is a multisystem disorder caused by mitochondrial DNA (mtDNA) mutations in patients between the ages of 5–15 years. Clinical features consist of stroke‐like episodes with brain CT or MRI abnormalities, lactic acidosis, seizures, dementia, short stature, recurrent headache, and ragged red fibers on muscle biopsy. CASE PRESENTATION: An 8 year old Hispanic male with no relevant past medical history was found unresponsive while playing; regained alertness without any intervention and function normally. His mother reports that later that day he complained of abdominal pain, non‐bilious emesis, headaches and an episode of nocturnal enuresis. Because of worsening lethargy he was taken to the ER where he presented with an episode of apnea where he was intubated and transferred to the PICU. PE showed weight and height below the 3rd percentile, and a mildly tender abdomen with no hepatosplenomegaly. Laboratory results showed bicarbonate 9 mmol/L, lipase 2,805 U/L, amylase 646 U/L, and normal results for: toxicology, triglycerides, CK, cultures, serum and urinary amino acids, and plasma carnitine/acylcarnitine profile. Radiological evaluation revealed a right occipital lobe infarct on MRI and negative abdominal CT and MRCP. The mtDNA whole genome sequencing revelaed a heteroplasmic m.3243A>G (in tRNA Leu) mutation and a homoplasmic m.4317A>G (in tRNA lle) mutation. CONCLUSION: We present a case of acute pancreatitis as the initial presentation of a mitochondrial disorder. The mtDNA mutation confirmed the disorder. Patient was treated with IV fluids and bowel rest. The patient was able to tolerate a normal diet with normalization of pancreatic enzymes within 72 hours of admission. Mitochondrial disorders should be part of the differential diagnosis in patients presenting with acidosis and pancreatitis.

Saturday, October 23, 2010 69 A RARE CASE OF PHELAN MCDERMID SYNDROME WITH INTESTINAL LYMPHANGIECTASIA S.N. Raju, Z. Yu, K. Wierenga, S. Marilyn, Pediatric Gastroenterology, The University of Oklahoma, Oklahoma City, OK; 7 week old term female infant was admitted for FTT (below birth wt), severe hypoalbuminemia, and ascites. She was exclusively breast feed with a history of large, foul smelling stools and increasing abdominal girth. Family history was noncontributory. Physical exam revealed a cachectic infant with dysmorphic facies and dystrophic toenails. Abdomen was soft and distended with a small fluid wave. Arms and phalanges were slightly puffy. Laboratory tests: albumin <0.7, lymphopenia, low IgG, severe anemia, and elevated fecal A1AT. Imaging studies revealed distended bowel loops, ascites, and thickened bowel wall prominent on the mesenteric side. Initial EGD and flexible sigmoidoscopy were not diagnostic. While NPO and on TPN albumin levels, ascites and weight improved. Repeat SBFT showed improved bowel wall thickening. Numerous formula trials resulted in worsening enteric protein loss, increased ascites and poor weight gain. Repeat fecal A1AT was normal when LCT were removed from diet. A colonoscopy revealed a IC valve mass. Histology of mass demonstrated focal polypoid mucosa with dilated lymphatics packed with T–cell lymphocytes. Exploratory surgery found a 1 cm mass at the IC valve which was resected. Histology demonstrated ileal lymphangiectasia. She is currently on po Monogen (90% fat as MCT). Given her history of intestinal lymphangiectasia, dysmorphic features, and developmental delay, chromosomal analysis was done. A 22q13.3 deletion was found resulting in the diagnosis of Phelan McDermid syndrome(PMS). PMS is characterized by hypotonia, developmental delay, dysmorphic features, certain behavioral characteristics, and normal growth. Although lymphedema has been observed in >25% of individuals, it generally does not become problematic until the teenage/adult years. This is the first case of PMS to be reported with intestinal lymphangiectasia. Phelan, K. 22q13.3 Deletion Syndrome. GeneReviews, 2007. Phelan, M. Deletion 22q13.3 syndrome. Orphanet Journal of Rare Diseases, 2008. 3:14. 70 COMPLICATED DIVERTICULITIS WITH COLOVESICULAR FISTULA IN AN ADOLESCENT S.N. Raju, M. Altaf, J. Grunow, Pediatric Gastroenterology, The University of Oklahoma, Oklahoma City, OK; Acute colonic diverticulitis is common in adults, but rare in children. We report a morbidly obese 14‐year old male who developed complicated sigmoid diverticulitis with colovesicular fistula requiring prolonged IV antibiotics and surgical resection. He presented with a 3‐week history of left side abdominal pain, diarrhea and dysuria. After UTI treatment, he developed hematuria, bubbles with urination, and blood in stools. Labs were remarkable for UTI and elevated inflammatory markers. IBD panel was negative. Initial imaging was remarkable for air in the bladder, extensive sigmoid inflammation with mucosal irregularity and diverticulosis. After prolonged antibiotics and liquid diet, repeat imaging demonstrated a walled‐off sigmoid perforation, a multi‐loculated abscess, air fluid level with bladder thickening, and localized sigmoid thickening. A flexible sigmoidoscopy showed erythematous mucosa with focal, mild active colitis on biopsy. Abscess was drained, IV antibiotics continued, and then the colovesicular fistula was closed and sigmoid colon was resected. Pathology showed acute diverticulitis, multiple diverticulum, and serosal adhesions. He has done well since. In the English literature, there have been no reports of complicated left‐sided diverticulitis in otherwise healthy children. Rare reports of children with diverticulitis had Williams‐ Beuren syndrome, Ehlers‐Danlos syndrome, Marfan syndrome, and cystic fibrosis; syndromes associated with genetic alterations in the colonic wall composition. Some studies have suggested aggressive course in young adults (<40 years) and some believe it may be due to delayed diagnosis. The correlation between obesity and acute diverticulitis is controversial. Afzal NA, Thomson M. Diverticular disease in adolescence. Best Practice & Research Clinical Gastroenterology 2002. 16:621‐634 Lahat A, et al. Diverticulitis in the young patient‐Is it different? World Journal of Gastroenterology 2006. 12:18):2932‐2935 Sorser SA, et al. Obesity and complicated diverticular disease: Is there an association? The Southern Medical Association, 2009. 71 REVERSAL OF LINEAR GROWTH ARREST IN A PATIENT WITH SHORT BOWEL SYNDROME USING GROWTH HORMONE V. Busoni, G. Alonso, M. Orsi, R. Vagni, N. Granados, F. Mateo, P. Lobos, R. Sanchez Claria, D. D'Agostino, , Hospital Italiano de Buenos Aires, Buenos Aires, ARGENTINA; One of the goals in short bowel syndrome (SBS) is to meet all caloric needs for growth via the enteral route. However, this is not always possible and parenteral nutrition (PN) needs to be used in conjunction. STEP is a bowel lengthening procedure aimed to improve nutrient absorption. Recombinant human growth hormone (GH) has been shown in adults with SBS to reduce dependence on parenteral nutritional support. We describe a case of a 3.5 yr old with SBS secondary to gastroschisis, who after STEP had 12 mo of catch up growth but subsequently experienced linear growth arrest that responded to GH. Because of total loss of venous access intestinal transplantation was not feasible. The patient presented to us at 14 mo of age with enteral feeding intolerance and PN dependency, weight Z‐score ‐6.64 and height Z‐score ‐7.29. STEP procedure was performed increasing small bowel length by 137.5 % (16 to 38 cm). One yr post op 73% of caloric intake was enteral, with marked reduction of cyclic PN. Catch‐up growth occurred with a delta weight Z‐score of +4.75 and delta height Z‐score +2.45. However, 1 yr later growth plateaud. IGF1 and IGFBP3 were borderline low. GH (50 μg/kg/d) was administrated during 10 mo resulting in a delta weight Z‐score of +1.73 and delta height Z‐score of +0.9 with improved IGF1 and IGFBP3’s levels. Nevertheless the patient remains dependent on PN but with reduced requirements. Conclusions: The success of GH therapy in this patient could be explained in part by GH resistance observed in patients with chronic diseases. Levels of IGF1 and IGFBP3 should be measured when growth is arrested during the management of patients with short bowel syndrome. 72 RECURRENT INTUSSCEPTION – A RARE MANIFESTATION OF ALLERGIC EOSINOPHILIC GASTROENTEROCOLITIS R. Ramraj, A.P. Olive, , Baylor College of Medicine, Houston, TX; Objective: We report a case of eosinophilic gastroenterocolitis with intussception as the initial manifestation. Case Summary:The patient is a 3 year old female who presented to the ER with 10 days of intermittent, diffuse, abdominal pain and frequent, watery stools. Patient had a history of food allergies diagnosed by RAST and was on a diet eliminating all offending agents. Abdomen was mildly tender and no masses were felt at the time of examination. Ultrasound of the abdomen was done on arrival and repeated three hours later that showed self resolving ileoileal intussceptions at three different sites and a right ileocolonic intussception .The intussception episodes coincided with episodes of abdominal pain. Supportive therapy was given and no surgical intervention was needed. Diagnostic upper and lower endoscopies were done and the biopsies showed chronic inflammation with increased eosinophils (15‐25/HPF) in the stomach, duodenum, transverse and descending colon. The diagnosis of eosinophilic gastroenterocolitis was made and patient was started on cryptohepatidine with resolution of symptoms. Discussion: Diffuse, recurrent intussception in pediatrics is a rare entity and is usually associated with inflammation affecting the gastrointestinal tract. Eosinophilic gastroenterocolitis is a chronic inflammatory disorder characterized by eosinophil infiltration in the mucosa or in deeper layers of the gastrointestinal tract. Clinical manifestations depend on the extent and severity of inflammation and diagnosis is confirmed by histology. It is associated with immune mediated food allergies about 50% of the time and food elimination is often an important part of therapy in adjunct to steroids and other antiallergic medications. Intussusception is a rare manifestation of eosinophilic gastroenterocolitis as in this patient where the subsequent endoscopy confirmed the diagnosis. This case report illustrates the importance of recognizing the clinical spectrum of eosinophilic gastrointestinal disorders and initiating appropriate medical management to prevent surgical complications. 73 CONTINUOUS TOPICAL TACROLIMUS THERAPY IN ORAL MANIFESTATON OF CROHN'S DISEASE A. Aybar, A. Malkani, A.M. Safta, Pediatrics, University of Maryland, Baltimore, MD; Mucosal oral lesions are well described in Crohn's disease with prevalence rates as high as 20%. Recurrent aphthous oral ulcerations may be the sole manifestation, more common in young people and notably resistant to oral or intravenous corticosteroids, immunosuppression, enteral nutrition and antibiotics. There is increasing evidence that topical tacrolimus may be effective in these therapeutically challenging lesions. We report a patient with Crohn's disease with frequent oral exacerbations without significant gastrointestinal symptoms responding to topical tacrolimus solution. A six year old girl presented with progressively worsening tongue ulcers, fever and poor feeding. Infectious workup was negative.Upper gastrointestinal tract,terminal ileum, colonic and tongue biopsies showed evidence of Crohn's disease with terminal ileitis, acute ulcerative esophagitis acute and chronic inflammation extending to lamina propria and muscularis of the tongue. She was treated with systemic corticosteroids,nasogastric elemental diet and induced with infliximab with no response. She had recurrent severe tongue ulcers with inability to swallow resulting in dehydration while on systemic steroids, infliximab and elemental diet. During this episode, topical Tacrolimus 0.5 mg/ml solution, 2.5 mg swish and spit twice a day and clobetasol 0.05% twice a day were added to the regimen. Her lesions improved within 7‐10 days and she was weaned off systemic steroids. Despite systemic immunosupressive treatment she continued to have recurrence of tongue ulcers as non‐compliance was encountered with topical treatment. Upon reintroduction of topical tacrolimus tongue ulcers healed. Random tacrolimus serum levels were undetectable on multiple occasions. Topical tacrolimus is shown to reduce cytokine generation and T cell activation in both the epidermis and draining lymph nodes. In those children with isolated oral ulcerations related to Crohn's disease we recommend using continuous topical tacrolimus early in the course, especially since there is no evidence of systemic absorption and recurrence is common if discontinued. 74 CROHN’S DISEASE DIAGNOSED AFTER A MOTOR VEHICLE ACCIDENT IN AN ASYMPTOMATIC BOY. R.A. Gomez, R. Dimmitt, S. Saeed, Pediatric Gastroenterology, University of Alabama at Birmingham, Birmingham, AL; R. Kejzer, O. Muensterer, Pediatric Surgery, University of Alabama at Birmingham, Birmingham, AL; K. Harrison, Laboratory Medicine, Children's Hospital of Alabama , Birmingham, AL; Presentation of Crohn’s disease can be subtle, leading to challenges in its diagnosis. Furthermore, it is well recognized that some manifestations of Crohn’s disease such as growth failure, and pubertal delay, may precede the onset of gastrointestinal symptoms. The prevalence of Crohn’s disease in asymptomatic population is also not known. We present a case of a 14 year old previously healthy boy who was taken to the operating room due to blunt abdominal trauma after a motor vehicle accident (MVA). His physical examination and his growth parameters were normal at the time of presentation, except for findings of an acute abdomen. Intraoperatively, bleeding from a devascularized terminal ileum with perforation was found, which was treated by ileocecal resection with the intention of primary reanastomosis. However, because the terminal ileum showed an unusual firmness, an extra 15 cm of terminal ileum was also resected. The macroscopic picture was consistent with mucosal ulceration and presence of abundant pseudopolyps, and creeping fat on serosal surface. The ileal mucosal histology was consistent with edema, and hemorrhage with increased cellularity of the lamina propria, scattered crypt abscesses, and patchy ulceration. The cecum showed crypt abscesses and several epithelioid granulomas. A subsequent upper and lower endoscopy showed presence of serpiginous ulcers in the stomach, and apthous ulcers in the duodenum and rectum. Microscopically, active inflammation was noted in the stomach, duodenum, colon and rectum. He was treated with Mesalamine, 6‐mercaptopurine and Budesonide. His Prometheus serology 7® profile was normal. He remains asymptomatic now, as he was prior to his presentation. This is the first reporting of a trauma‐related fortuitous diagnosis of a case of Crohn’s disease following blunt abdominal trauma in an otherwise asymptomatic child. 75 GASTRIC OUTLET OBSTRUCTION DUE TO BRUNNER’S GLAND ADENOMA IN A CHILD S. Schroeder, J. Soden, Pediatric Gastroenterology, Hepatology, and Nutrition, The University of Colorado Denver, Aurora, CO; M. Dishop, Pathology, The University of Colorado Denver, Aurora, CO; D. Partrick, Pediatric Surgery, The University of Colorado Denver, Aurora, CO; Introduction: Brunner’s Gland adenoma (BGA) is a rare lesion in the pediatric population. We report a case of BGA in a 10 year old male who presented with abdominal pain, vomiting, and gastric outlet obstruction. Case Report: A 10 year old previously healthy male presented to The Children’s Hospital with a chief complaint of abdominal pain. There was no prior history of surgery or illness. Physical examination and laboratory evaluation were normal. The patient underwent attempt at endoscopy under general anesthesia. The stomach was fluid filled,and the physician was unable to advance an 8.6mm outer diameter endoscope into the duodenum. One month later, the child returned for an endoscopic balloon dilation of the duodenum. Subsequent radiologic evaluations revealed a duodenal mass. The patient was referred for surgical resection,where a 1.5 cm mass was resected. The pathology was consistent with a Brunner’s gland adenoma. The patient was asymptomatic after the procedure,and a repeat endoscopy several months later was normal. However,two years postoperatively,the patient returned with subacute post‐prandial pain and nonbilious emesis. Imaging studies revealed a hugely dilated and obstructed gastric lumen. Endoscopic evaluation was performed, and a 6mm OD endoscope was advanced into a narrowed duodenum, where a round, protruding mass was identified, thought to be suspicious for recurrence of the BGA. Surgery was again performed. Operative findings included a narrowed duodenum,questionable for recurrence of mass versus stricture. A diverting gastrojejunostomy was performed. At 6 month follow up, the patient is well, with no clinical or radiologic suspicion for recurrence. Discussion: BGA is a rare cause of gastric outlet obstruction. It is considered a benign tumor, and there is minimal evidence of malignant potential. This interesting lesion should be considered in the evaluation of pediatric gastric outlet obstruction. 76 ENDOSCOPIC RESECTION OF A MUCOSAL WEB IN A PATIENT WITH CROHN'S DISEASE C. Moran, J. Biller, Pediatric Gastroenterology & Nutrition, MassGeneral Hospital for Children, Boston, MA; P. Kelsey, Gastroenterology, Massachusetts General Hospital, Boston, MA; G. Lauwers, Pathology, Massachusetts General Hospital, Boston, MA; Intestinal webs are an uncommon cause of abdominal pain and when found in children, they are usually congenital. We present a 16 year old with fistulizing, ileocolonic Crohn's disease with a colonic web. This patient required catheter drainage of an intra‐abdominal abscess, and later brought to ileocecectomy after antimicrobial therapy. The drain was found adherent to the transverse colonic wall. The drain was dissected off the colon, and a small wedge resection was performed with oversewing of the colonic margins. Histologic analysis of the resected wedge showed mucosal ulceration and subserosal abscesses consistent with active Crohn's disease. Ten months later, the patient had intermittent left upper quadrant pain in the setting of a normal laboratory evaluation. Colonoscopy found an intraluminal web in the transverse colon at the previous wedge resection site. This web contained two distinct openings; each permitting scope passage into the proximal colon. Mucosa from the proximal colon was observed prolapsing through an opening of the web. The web was incised os to os using an ERCP needle knife. The involved mucosa spontaneously retracted allowing for complete opening of the lumen. The patient’s abdominal pain resolved. This case appears to be the first of a mucosal web in a child with Crohn’s disease. Congenital webs usually require surgical excision, and NSAID‐induced webs (commonly found in adults) frequently also requires surgery. However, given the concern for repeated surgical interventions in Crohn’s disease, endoscopic therapy provided an attractive alternative. After visualization of the bowel directly proximal to the web proved that the caliber of colon was normal and therefore not a stricture, endoscopic therapy allowed for web resolution and allowed the patient to avoid additional surgery. We feel that endoscopic therapy of a mucosal web in Crohn's disease should be strongly considered prior to proceeding with surgical resection. 77 POOR RESPONSE TO HEPATITIS B VACCINE IN PEDIATRIC CELIAC DISEASE A. Angirekula, M. Jatla, Pediatric Gatroenterology, Texas A&M College of Medicine, Round Rock, TX; Objective: Children with celiac disease often fail to produce a response to the hepatitis B vaccine. We examined the prevalence of hepatitis B non‐immunity among patients with newly diagnosed celiac disease. Patients: We retrospectively reviewed patients diagnosed with celiac disease in the last 12 months at our institution. All were assessed for hepatitis B immunity by determining hepatitis B surface antibody (HBSab) levels. We reviewed their charts for patient demographics, celiac disease antibody titers, and histology. Results: A total of 8 subjects were identified with a mean age of 8.2 ± 1.4 years; 75% of the patients were female. We found that 7 out of 8 patients failed to respond to the hepatitis B vaccine. Among the non‐responders, the mean HBSab level was 2.9 ± 1.0 IU/mL. The mean tissue transglutaminase IgA (TTG) and endomysial antibody IgA (EMA) titers were 67.1 ± 10.4 IU/mL and 190 ± 75.6 dilutions respectively. The histology ranged from Marsh II –IIIB. Conclusion: We found a high prevalence (87.5%) of hepatitis B non‐immunity among patients with recently diagnosed celiac disease compared to the historical non‐response rate (5‐10%) among healthy children. DQ2 haplotype presence is a similarity between celiac disease and hepatitis B non‐response. Assessing hepatitis B immunity in newly diagnosed celiacs is essential. Re‐immunization in non‐responders may be more effective once histologic remission occurs. Further studies are needed to determine if this phenomenon occurs with other vaccines in this population. 78 SINGLE BALLOON ENTEROSCOPY TO CONFIRM CROHN’S DISEASE IN A PEDIATRIC PATIENT A. Miller, R. Shutka, N. Channabasappa, B. Barth, , UTSW Medical Center/Children's Medical Center, Dallas, TX; M. Dave, , Digestive Health Assoc. of TX, Dallas, TX; Background: Due to the location and tortuosity of the small bowel, its visualization and biopsy has historically been difficult. Crohn’s Disease (CD) frequently involves the small bowel, and diagnostic lesions may not be accessible by traditional endoscopy and colonoscopy. While capsule endoscopy has expanded our ability to diagnose and determine the extent of small bowel involvement of CD, its inability to obtain biopsies limits its utility. The development of single and double balloon enteroscopy (SBE/DBE) now allows direct visualization, biopsy, and therapeutic endoscopy of a significant length of small bowel. Case: We describe a 14 yo boy who presented with a 3 week history of weight loss and fever, persisting after multiple courses of antibiotics with no source identified. He denied diarrhea and bloody stools, with exam only remarkable for a thin appearance. Serologically, he was anemic, hypoalbuminemic, and had elevated inflammatory markers. He underwent EGD and colonoscopy which were grossly normal. Biopsies showed mild reactive esophagitis and chronic gastritis, with normal colonic and ileal tissue. Subsequently, fevers persisted and he developed vomiting, abdominal pain and anorexia. Abdominal CT revealed small bowel thickening, and capsule endoscopy showed bleeding, cobblestoning and ulceration in the jejunum suspicious for CD or lymphoma. For histologic confirmation, the patient underwent SBE with advancement of the enteroscope 150cm into the small bowel until the abnormal region was reached. Biopsies revealed chronic active inflammation with architectural distortion supporting the diagnosis of CD. Discussion: Balloon enteroscopy, a relatively new diagnostic and therapeutic modality, allows visualization, biopsy and endotherapy of all segments of the small bowel, and appears to be safe in pediatric patients. Likely underutilized in children, SBE in this patient was performed safely and well tolerated, allowing evaluation of abnormal capsule endoscopy findings and histologic confirmation of Crohn’s Disease. 79 EOSINOPHILIC GASTROENTERITIS IN A TODDLER S.S. Desai, R. Alkhouri, H. Hashmi, R. Baker, D. Gelfond, S. Baker, Digestive Diseases and Nutrition Center , SUNY at Buffalo, Buffalo, NY; Introduction: Eosinophilic gastroenteritis (EGE) is a rare disorder characterized by eosinophilic inflammation of gastrointestinal tissue. EGE is classified as serosal, mucosal, or muscular based on area of infiltration. The etiology of EGE is unknown, but postulated to be due to atopy. EGE can occur at any age, but more commonly found in adults. There is no consensus on diagnostic histological criteria for EGE, thus clinical and endoscopic findings are also needed. Treatment modalities are limited. Case: A 20 month old male with history of iron deficiency anemia presented with a 2 week history of watery diarrhea. The patient had facial swelling from allergies to eggs, milk, and peanut butter and was treated with a week of oral steroids. The patient was recently treated for H. Pylori, but his symptoms of hypoalbuminemia, anemia and hematochezia persisted. The only endoscopic finding was duodenal bulb nodularity. Histopathology showed chronic, eosinophilic gastritis,eosinophils in distal esophagus and prominent eosinophilia from terminal ileum to rectum. A free amino acid formula and systemic steroids were started. Fourteen days later, a gastrostomy tube was placed for decreased intake and biopsies were obtained. No eosinophils were found in duodenal, gastric or esophageal biopsies and the duodenal nodularity had resolved. Discussion: Our patient met the criteria for EGE, with gastrointestinal symptoms, biopsies showing eosinophilic infiltration of 1 or more areas from esophagus to rectum, and absence of parasitic infection or other causes of eosinophilia. Significant points about our case are the rapid resolution of histologic and endoscopic findings and the young age of presentation. Eosinophilia and duodenal bulb nodularity resolved after only 2 weeks of therapy, compared to 2 months noted in literature. EGE is more prevalent in second to sixth decade of life, and our patient was only 20 months old. Clinical symptoms are consistent with mucosal layer infiltration. First line treatments are elimination diets and steroids. Long term outcomes are not known. 80 RESPIRATORY DISTRESS SECONDARY TO BRONCHIOLITIS OBLITERANS ORGANIZING PNEUMONIA (BOOP) IN A PATIENT WITH NEW ONSET INFLAMMATORY BOWEL DISEASE (IBD) J. Dranove, R. Caicedo, V. Gopalareddy, Pediatric Gastroenterology, Levine Children's Hospital, Charlotte, NC; G. Vidwan, Pediatric Hospitalist Medicine, Levine Children's Hospital, Charlotte, NC; K. Adlakha, Pathology, Carolinas Medical Center, Charlotte, NC; Introduction: Although infrequent, pulmonary manifestations presenting with IBD are well documented in adults, but very rarely reported in children. Case Report: A previously healthy 12 YO AAF presented with 3 months of abdominal pain, bloody diarrhea, weight loss, anemia, hypoalbuminemia, and markedly elevated ESR, clinically concerning for Crohn’s Disease (CD). Interestingly, the patient did not seek medical attention until she developed a productive cough, chest pain, and striking dyspnea. An EGD showed moderate chronic gastritis, and a colonoscopy revealed a grossly and histologically normal terminal ileum and a nonspecific chronic pancolitis which grossly looked more like CD than Ulcerative Colitis (UC), with histologic findings of chronic colitis without granulomas. A chest CT on admission showed multiple bilateral pulmonary nodules and a right middle lobe (RML) consolidation. A CT angiogram ruled out a pulmonary embolism. Bronchoscopy and Bronchoalveolar Lavage were normal. PPD was negative. She started IV corticosteroids and broad spectrum antibiotics. Respiratory distress continued, and a CT guided fine needle aspirate (FNA) was unrevealing. Thoracoscopic lung biopsy was performed, showing florid BOOP. The patient’s GI and pulmonary symptoms rapidly improved on steroids. An extensive rheumatologic workup was negative. UC is the working diagnosis. Discussion: Pulmonary findings in IBD range from subclinical to severe respiratory distress, with both small and large airways and interstitial disease reported, most commonly BOOP. BOOP is almost exclusively found with UC. As far as we know this is the first report of a child presenting with IBD and BOOP. Timely thoracoscopic lung biopsy can be informative and help rule out primary or co‐existent rheumatologic, infectious, or vaculitic conditions in the setting of colitis. 81 CONCOMITANT USE OF TACROLIMUS FOR CHILDREN WITH CROHN'S DISEASE INTOLERANT TO INFLIXIMAB K. Arai, H. Shimizu, T. Yanagi, T. Kakiuchi, S. Koinuma, Gastroenterology, National Medical Center for Children and Mothers, Setagaya, Tokyo, JAPAN; Infliximab (IFX) takes important role in management of children with Crohn's disease (CD). The long term use of IFX may require dose intensification or frequent administration. Early or late infusion reaction is also a challenge. Concomitant use of immunomodulators (IMs) had been applied less frequently with emerging cases of malignancy. With limited availability of adalimumab in Japan, tacrolimus (FK‐506) was used in 3 Japanese children who were intolerant to IFX. Case 1. 13 y.o female with ileocolonic CD was induced and maintained in remission with IFX. After the 4th dose, duration between doses had been shortened for early relapse. When the effect of IFX did not last more than 2 weeks, FK‐506 was initiated. The duration of response to IFX was extended up to 6 weeks. Patient has tolerated to concomitant use of FK‐506 and IFX for 22 months. Case 2. 15 y.o. female with extensive CD was corticosteroid (CS) dependent and required IFX. With discontinuation of IFX, her CD relapsed and IFX was re‐ started. She responded poorly with upper gastrointestinal symptom. After CS was used to induce remission, FK‐ 506 was initiated with fair response. IFX was re‐started 4 weeks after FK‐506 was initiated. Patient tolerated to IFX with good response. CS was discontinued within 2 months, and patient has remained in remission without adverse reaction for 14 months. Case 3. 12 y.o male with colonic CD was resistant to conventional management, and required total parental nutrition (TPN). IFX had been used with good initial response followed by late reaction 1 week after the administration of IFX. FK‐506 was initiated with decreased disease activity. IFX was administered 9 weeks after FK‐506 was started. Patient initially responded to IFX, however, late reaction occurred in 1 week. IFX was terminated, and patient remains on TPN despite the use of FK‐506 for 4 months. Conclusion : Concomitant use of FK506 may be safe and useful in children with CD whose maintenance therapy with IFX is difficult to continue due to loss of response or infusion reaction. 82 POSTERIOR REVERSIBLE ENCEPHALOPATHY SYNDROME IN PEDIATRIC INFLAMMATORY BOWEL DISEASE:2 CASE REPORTS K. Mehta, D. Kawatu, N. LeLeiko, , Brown University, Providence, RI; M. Integlia, , Barbara Bush Children's Hospital, Portland, ME; Background:Posterior Reversible Encephalopathy Syndrome (PRES) is a clinical syndrome characterized by headache, confusion, hypertension, possible visual impairment, seizures and posterior white matter changes on neuroimaging. Common precipitants are hypertension, renal impairment or immunosuppressants. PRES is now increasingly recognized among IBD patients. We report 2 ulcerative colitis patients who developed PRES at different stages of their disease and treatment course. Case1:15 yo girl with newly diagnosed UC was started on iv methylprednisolone. After one week of steroids, she had mildly elevated BP. On day 12, she developed 2 generalized seizures, BP prior to seizures was 150/80. T2 weighted MRI showed hyperintensities in fronto‐parietal and occipital lobes consistent with PRES. Steroids were tapered and she required infliximab to induce remission. Levetiracetam was begun and she continued to receive maintenance infliximab. 4 months after diagnosis, she continues to do well on every 8 weeks infliximab. Case2: 13 yo boy with UC, unresponsive to high dose steroids and infliximab. Remission induced with 6MP, FK‐506 and steroids. 4 months into diagnosis, he had flares needing colectomy. Prior to colectomy, he was on minimal steroids and off 6MP and FK‐506. On post‐op day 12, he developed 3 generalized seizures. Head CT showed parietal and posterior lobe hypodensities consistent with PRES. BP was normal throughout the treatment course. Discussion: The literature suggests infliximab as a cause of PRES in a Crohn's patient. However, our 2 cases developed PRES in different clinical settings, one of which did not involve infliximab. Infact, in case 1, infliximab was used successfully in established PRES without worsening or recurrence of PRES. Case 2 developed it months after infliximab exposure and post colectomy. These 3 cases illustrate that PRES in children with IBD is multifactorial. Based on current knowledge, we cannot conclude whether this is a neurological association with IBD or a rare side effect of immunosuppressant therapy. 83 PSORIASIS DUE TO ANTI TNF ALPHA THERAPY IN IBD. ONE CENTER'S EXPERIENCE AND MANAGEMENT. B.P. Regan, A. Flores, Pediatric GI, Tufts Medical Center, Boston, MA; Background: Anti TNF alpha therapy has many clinical indications including RA, IBD, and refractory psoriasis (1). We report a series of 6 IBD patients in a pediatric gastroenterology group with new onset psoriatic lesions after treatment with Anti TNF alpha therapy. Case Series 6 patients age 16‐23, 5 males 1 female. 5 CD 1 UC. 5 developed psoriasis while on IFX 1 on adalimumab. IFX changed to MTX in one patient. IFX switched to certolizomab, then MTX after gluteal abscesses developed in 1 patient. Adalimumab was DC’d and placed on MTX in the primary adalimumab patient. IFX changed to Humira in one patient. UC patient went to colectomy. One patient had resolution while continuing IFX. Discussion There is not accepted explanation of the mechanism of onset of anti TNFa induced psoriasis. Possible mechanism of actions involve Plasmacytoid Dendritic Cells PDCs and their production of interferon. TNF normally inhibits PDC maturation and IFN production. The inhibition of TNF may allow unregulated production of IFN by PDCs. TNF inhibition also mimics infection or injury increasing IFN expression at the in predisposed individuals. PDCs are found in early psoriatic lesions and even in normal‐ appearing skin of psoriatic patients, prior to the appearance of the psoriatic plaque. Increased IFN expression has been shown in the lesions dermal vasculature of patients who develop psoriasis while receiving TNF antagonist therapy. In the setting of TNF inhibition, the cytokine profile increases the IFN production originating from PDCs. (2) Conclusion These cases indicate the importance of recognizing the development of psoriasis in pediatric patients treated with anti‐TNF alpha therapy, a medication used in the treatment of refractory psoriasis. Management options in IBD patients who develop this complication include discontinuing biologic therapy and changing to immunomodulators, treating through the lesions if mild, other Anti‐TNF agents in the same class, and colectomy. 1 Cohen, JD et al Journal of Rheumatology 2007 380‐85 2 Collamer et al Arthritis and Rheumatism June 2008 996 ‐ 1001 84 AN UNUSUAL CAUSE OF GASTROINTESTINAL BLEEDING IN A TEENAGER DETECTED BY CAPSULE ENDOSCOPY: MECKEL’S DIVERTICULUM C. Woods, M. Zayat, , The Wichita Center for Graduate Medical Education (WCGME), Wichita, KS; Meckel’s diverticulum is a congenital, intestinal blind pouch that results from an incomplete obliteration of the vitelline duct during the fifth week of gestation. It typically presents in the first decade of life with painless rectal bleeding and is rarely reported in teenagers. We present a case where a diagnosis of Meckel’s diverticulum was made in a teenage male. A 15 year old otherwise healthy male presented with two week history of intermittent burgundy red blood in his stool. He reported one soft bowel movement per day and denied abdominal pain, nausea or vomiting. Two days prior to admission, he reported heavy hematochezia followed by decreased energy and pounding headaches. He was seen by his pediatrician who subsequently admitted him to the hospital and consulted the GI team. No history of alcohol or NSAID use. The patient had a history of gastrointestinal bleeding at the age of 18 months. At that time, he received a blood transfusion and had a negative colonoscopy and esophagogastroduodenoscopy. His past medical history was otherwise only significant for bilateral hearing loss requiring hearing aids, acne, and seasonal allergies. His medications were minocycline, fexofenadine, and prn Tylenol. Physical exam showed stable vital signs except for a moderately elevated heart rate and significant pallor but was otherwise unremarkable. His laboratory studies revealed hemoglobin of 6.7 g/dl (MCV 80.9, RDW 14.8) on admission. Subsequently the patient was transfused with two units of packed red blood cells which stabilized his hemoglobin at 8mg/dl. An EGD, colonoscopy, and tagged red cell scan were negative. Capsule endoscopy revealed an “ulcerated polypoid mass in the distal jejunum or ileum”. He then underwent a exploratory laparotomy where a Meckel's diverticulum with an ulcerated ectopic gastric mucosal lining was identified and resected. He did well postoperatively and was sent home with no further GI bleeding. 85 EARLY ONSET NEPHROTIC SYNDROME IN PEDIATRIC CELIAC DISEASE H.A. Kader, A. Fassano, M. Martin, E. Binnie, Division of Pediatric Gastroenterology & Nutrition, The University of Maryland Hospital for Children, Baltimore, MD; L. Jinadu, Division of Pediatric Nephrology, The University of Maryland Hospital for Children, Baltimore, MD; Celiac disease (CD) is an autoimmune self‐destructive enteropathy triggered by the immune system's response to gliadin with development of specific antibodies. Glomerulonephritis has been reported to occur in CD rarely, 0.2% of CD patients vs 0.1% of the general population with a median age of onset being 28 years‐old (range 4‐86 years‐ old). IgA nephropathy is the most common form of glomeruloneprhitis associated with CD. There are only 6 reported childhood onset cases of renal disease occurring in pediatric CD in the literature, one in a recent 2009 case report and 5 via a 2002 letter to the editor. Here we report a 17 ½ month‐old male diagnosed concurrently with CD and nephrotic syndrome after presenting with a 5 month history of emesis, several large, pale and frothy appearing daily bowel movements, abdominal distension, lower extremity and periorbital edema, microcytic anemia but normal iron and low TIBC and hypoalbuminemia. He had a normal spot stool alpha‐1‐ antitrypsin level and 2+ proteinuria in a first morning urinalysis. Nephrotic syndrome occurring at this age has not been previously reported in children with CD. He was started on a gluten free diet and by his nephrologist also systemic corticosteroids. His emesis, diarrhea, edema, anemia and hypoalbuminemia resolved after 2 months though his proteinuria continued. This case is presented to demonstrate that not all hypoalbuminemia in CD is due to a protein losing enteropathy and other etiologies should be excluded to permit adequate patient care. 86 PRIMARY BURKITT’S LYMPHOMA OF THE COLON: AN UNCOMMON CAUSE OF PEDIATRIC CONSTIPATION AND ABDOMINAL PAIN C.T. Meyer, P.D. Danielson, G.H. Hale, H.L. Monforte, M.J. Wilsey, , All Children's Hospital, St. Petersburg, FL; C.T. Meyer, P.D. Danielson, G.H. Hale, H.L. Monforte, M.J. Wilsey, , University of South Florida College of Medicine, Tampa, FL; Constipation is a common problem in children, accounting for over 25% of all visits to pediatric gastroenterologists. Approximately 95% of childhood constipation is functional in nature. Gastrointestinal neoplasia is uncommon in childhood and adolescence and the majority are stromal tumors and carcinomas. Primary gastrointestinal tract lymphomas are rare. We present a case of primary Burkitt’s lymphoma of the colon in an adolescent presenting with constipation, abdominal pain, and later, rectal bleeding and progressive weight loss. CASE REPORT: A previously healthy fourteen year old Caucasian male with past history of childhood constipation presents to the gastroenterology clinic with a 5 week history of worsening constipation and abdominal pain. He denies melena, hematochezia, mouth sores or joint pains, but reports a 2‐3 kg weight loss attributed to exercise. The patient's weight was 108 kg (BMI 30; >97%) and the physical examination was unremarkable except for positive fecal occult blood. Screening labs were normal but stool studies demonstrated Dientamoeba fragilis. Symptoms improved with PEG 3350, but he developed hematochezia and further weight loss (7 kg) at his one month follow‐up visit. Colonoscopy revealed a 10 cm x 6 cm x 3 cm flesh‐colored near‐obstructive luminal mass involving transverse colon. Endoscopic biopsies revealed Burkitt’s lymphoma. The patient underwent transverse colectomy with primary anastomosis; further evaluation demonstrated Stage II disease and chemotherapy was initiated (COG ANHL01P1). CONCLUSIONS: Constipation and abdominal pain are common problems evaluated by pediatric gastroenterologists. We present a case of a patient with a seemingly benign initial presentation who developed progressive hematochezia and weight loss and was ultimately diagnosed with primary Burkitt’s lymphoma of the colon. Primary colon lymphomas are very rare tumors and early diagnosis and treatment are of fundamental importance to improve patient outcomes. 87 14 YEAR OLD PATIENT WITH CVID‐ASSOCIATED ENTEROPATHY RESPONSIVE TO IVIG THERAPY M.I. Abbas, A. DeLorimier, T. Banks, Pediatrics, Walter Reed Army Medical Center, Washington, DC; C. Mikita, Allergy/Immunology, Walter Reed Army Medical Center, Washington, DC; C. Grove, Pathology, Walter Reed Army Medical Center, Washington, DC; We present a case of common variable immunodeficiency (CVID) which was only associated with chronic diarrhea, growth failure and inflammatory changes on endoscopy that failed standard Crohn’s disease therapy. Our patient demonstrated significant weight gain and resolution of diarrhea after treatment with metronidazole and intravenous immunoglobulins (IVIG). CVID is described a heterogeneous disease that results from an immune dysfunction that leads low gammaglobulins, altered ability to produce antibodies to infection or immunizations and a propensity for infections. Diagnosis is made by noting a reduction in IgG and/or IgA and IgM at least 2 standard deviations below the age‐specific mean values and by demonstrating impaired specific antibody production to protein or polysaccharide antigens. Classically, patients go undiagnosed for several years presenting only with recurrent sinopulmonary infections, however, heterogeniety of the disease results in a variety of clinical presentation with sometimes no history of recurrent infections. Various associated manifestations have been described including autoimmunity, granulomatous disease, neoplastic, and GI diseases. Pediatric patients with CVID‐associated GI manifestation demonstrate findings that are consistent with a chronic malabsorptive or inflammatory state. Certain subset of patients are diagnosed with inflammatory bowel disease and show response to standard treatment including antibiotics, 5‐aminosalicylic acid, steriods, immunomodulators or anti‐TNFalpha therapy. CVID treatment is rooted around a few principles: immunoglobulin replacement, control and prevent infections, and monitor closely for associated disease manifestations. However, disease heterogeneity demonstrates a variable response to immunoglobulin replacement. Several case reports, including our own, have demonstrated that, if IVIG is not initiated, GI infections (i.e., Giardiasis) are more likely to be refractory. 88 PNEUMOMEDIASTINUM ASSOCIATED WITH A RELAPSE OF ULCERATIVE COLITIS IN A PEDIATRIC PATIENT J. Athayde, L.S. McKenzie, , University of Calgary, Calgary, Alberta, CANADA; Pneumomediastinum is a rare complication of inflammatory bowel disease. Postulated mechanisms include an increase in mediastinal pressure or insufficient decompression of air along fascial planes. There have been 6 cases of pneumomediastinum associated with IBD in adults reported in the literature. We present the first case of a pediatric patient with a severe flare of ulcerative colitis who experienced acute decompensation related to pneumomediastinum. The patient was a 16 year old male who presented with bloody diarrhea, abdominal cramping, and significant straining. Ulcerative colitis had been diagnosed a year before and he was on 5‐ASA for maintenance therapy when his symptoms flared. Oral prednisone failed to control his symptoms. The patient was treated with IV steroids, bowel rest, and parenteral nutrition with minimal improvement. He developed fever, acute chest pain, dyspnea, and difficulty swallowing. Physical examination was significant for tachycardia, muffled heart sounds and infraclavicular subcutaneous emphysema. Bloodwork was consistent with DIC. CT showed a pneumomediastinum with air surrounding the esophagus. There was no evidence of pneumoperitoneum, no contrast leak from the esophagus, no toxic dilatation of the colon and no evidence of colonic perforation. The patient was hemodynamically stable and was treated conservatively with oxygen, IV fluids, steroids, antibiotics, blood products, and parenteral nutrition. The pneumomediastinum completely resolved after 9 days, however the colitis worsened and he required an urgent colectomy with ileostomy for persistent bleeding. The cause of pneumomediastinum in our patient is unknown, however it is surmised that the patient’s significant and prolonged straining led to air dissection along the fascial planes from the retroperitoneal space into the mediastinum. Clinicians should consider the presence of pneumomediastinum in patients with colitis who present with Hamman’s sign and subcutaneous emphysema on physical exam, even without free intraperitoneal air. This case highlights a very unusual complication in the hospitalized patient with colitis. 89 HEMOPHAGOCYTIC LYMPHOHISTIOCYTOSIS IN A CHILD WITH CROHN'S DISEASE. M.E. Gabel, M.R. Brown, Pediatric Gastroenterology, University of Rochester, Rochester, NY; Hemophagocytic lymphohistiocytosis (HLH) is a rare, potentially life threatening disorder. It is characterized by fever, splenomegaly, and rash. Laboratory features include pancytopenia, hypertriglyceridemia, hyperferritinemia, and hypofibrinogenemia. There have been case reports of HLH occurring in patients with Crohn’s disease in the context of treatment with azathioprine or inflixamab, or with evidence of active CMV infection. We report a case of newly diagnosed Crohn’s disease, treated only with prednisone, who developed HLH without evidence of CMV or EBV infection. Our patient is a 9 year old girl who initially presented to an outside hospital due to acute onset of bloody diarrhea and abdominal pain. Colonoscopy showed severe inflammation of the colon and pathology revealed granuloma, confirming the diagnosis of Crohn’s. Her course was complicated by intestinal perforation requiring a subtotal colectomy with colostomy. After a 2‐week hospital stay, she was discharged home on piperacillin/tazobactam, prednisone and mesalamine. Shortly after her return home, this patient presented to our hospital with fever and bloody ostomy output. Laboratory studies were significant for pancytopenia, coagulopathy, transaminitis, and hyperammonemia. EBV and CMV PCRs were negative. Triglyceride level was obtained and found to be 735 mg/dl. Given the laboratory data and the critically ill nature of the patient, the diagnosis of HLH was entertained. Bone marrow biopsy confirmed the diagnosis. Our patient was continued on high dose methylprednisolone and clinically improved over the following week. Secondary HLH is a rare entity that has been associated with severe infection and autoimmune disease. To our knowledge, this is the first report of a patient with Crohn’s disease developing HLH without active EBV/CMV infection or concurrent treatment with inflixmab or azathioprine. This suggests that Crohn’s disease alone is a risk factor for HLH. HLH is a multisystem disorder that, unrecognized, can lead to significant risk of mortality, therefore a high degree of clinical suspicion is necessary. 90 WHAT'S ALL THE BUZZ ABOUT CAPSULE ENDOSCOPY? AN UNEXPECTED FINDING SOLVES A MYSTERY OF RECURRENT ABDOMINAL PAIN A.P. Eidelwein, J.M. Mills, K.F. Butcher, Y.S. Al‐tawil, Pediatric Gastroenterology/GI for Kids, East Tennessee Children's Hospital, Knoxville, TN; D.L. New, Pediatric Infectious Diseases, East Tennessee Children's Hospital, Knoxville, TN; A 13‐year‐old female presented with recurrent abdominal pain, nausea, vomiting, and bloody diarrhea for 8 months. Blood work, radiologic studies, endoscopy/colonoscopy were normal, except for gastritis. Despite treatment, symptoms continued leading to exploratory laparoscopy with cholecystectomy; repeat endoscopy/colonoscopy showed gastritis and mild colitis. Capsule endoscopy revealed unexpected findings of multiple insects throughout the small bowel(SB). Infectious Disease identified between 20‐30 insects adherent to the wall of the SB. All appeared to be honeybees (Apis Mellifera). Dead honeybees can continue to sting, and if the mucosa of the SB is not strong enough to remove the stinger from the bees, that would explain the recurrent abdominal pain. It was unclear if the colitis could be reactive to the bees’ sting. Patient/family denied any knowledge of the ingestion. Social work investigated the case; patient admitted periods of black outs where ingestion could have occurred. The father reported severe reaction to bee stings. Family bought jars of honey with combs, but patient reported no ingestion of combs. There were no bees in their property, but there was a beekeeper nearby. Ingestion of the combs does not explain presence of adult bees since the brood is in a different part of the beekeeper’s hive than the honey. Recommendation was made for a high residual diet to help dislodge the bees, since the bees remained despite clean out for the colonoscopy and capsule endoscopy. The patient was recommended to follow‐up with social work services and psychology to further investigate the case. Symptoms slowly resolved overtime and she had no further follow‐up. This case demonstrates, while considering psychological evaluation, the importance of concurrently considering capsule endoscopy in children with severe, recurrent, abdominal pain when the findings from other studies do not explain the severity of symptoms. 91 CELIAC DISEASE AND CARDIOMYOPATHY V. Goh, K. El‐Chammas, N. Tipnis, Pediatric Gastroenterology and Nutrition, Medical College of Wisconsin, Milwaukee, WI; Case Report: A healthy 16 year old male presented with progressive fatigue and was diagnosed with anemia with hemoglobin of 6.6g/dl. He was given iron supplementation. Hemoglobin 1month after starting iron was 7.5g/dL. Tissue transglutaminase antibody (IgA TTG) was >100 u/mL and duodenal biopsies were consistent with celiac disease. He was started on a gluten‐free diet (GFD) with continued iron supplementation. After 6 weeks, his symptoms improved and hemoglobin had reached 14g/dL. Six months after diagnosis, he presented with shortness of breath, fatigue, and hemoptysis. Chest X‐ray showed a dilated cardiac silhouette. His B‐type natriuretic peptide (BNP) was 1680 pg/mL suggesting heart failure. An echocardiogram showed an ejection fraction of 15%. He was diagnosed with non‐obstructive dilated cardiomyopathy and started on diuretics, and inotropes. Repeat IgA TTG was >100u/mL, although dietary history suggested compliance with GFD. Infectious and autoimmune workups were negative. He received a 3‐day high‐dose pulse of methylprednisolone for presumed autoimmune cardiomyopathy associated with celiac disease. Repeat echocardiography 4‐days later showed mild improvement with ejection fraction (23%) and BNP 1000pg/mL. A nutritional screen with copper, thiamine, zinc and selenium showed borderline low thiamine 9nmol/L. He received thiamine supplementation, but continued to be dependent on inotropic support. Because of non‐responsive heart failure, he was listed for cardiac transplant. Discussion: Celiac disease has been associated with cardiomyopathy through an autoimmune process or malabsorption. Immunosuppressive therapy has not proven beneficial in isolated cardiomyopathy, but there are mixed reports in cases of celiac disease associated cardiomyopathy. This patient’s cardiomyopathy could be due to either malabsorption or an autoimmune process. He did not respond to GFD, immunosuppression, or nutritional supplementation, suggesting idiopathic dilated cardiomyopathy. Nutritional screen and immunosuppression, however, should be considered in patients with cardiomyopathy who have celiac disease. 92 PATIENTS WITH RETAINED VIDEO CAPSULES AT A LARGE PEDIATRIC CENTER. N. Channabasappa, B.A. Barth, Pediatric Gastroenterology, UT Southwestern Medical Center, Dallas, TX; Introduction: Capsule endoscopy (CE) has made a tremendous impact in the evaluation of diseases of the small bowel. CE is generally well tolerated and safe in adults, with capsule retention being the most commonly reported adverse event. Retention rates in adults are low (0.75%‐2%), however data in pediatrics is limited to small case series. We present our experience focusing on characteristics of patients suffering capsule retention requiring endoscopic or surgical removal. Methods: Records of patients referred to our institution from 2004 to 2008 for small bowel capsule endoscopy were reviewed focusing on medical and surgical history, patient age, size and fate of ingested capsule. Results: Of 103 capsule studies performed over 4 years, three patients (2.9%) were identified in whom the capsule was retained. Two patients had a complicated surgical history prior to CE, and one had known stricturing Crohn’s disease. Patient #1 was a 9 year old male (32 kg) with a history of total colonic as well as small bowel Hirschsprung's disease. He had multiple laparotomies due to bowel obstruction. He underwent surgical removal of the capsule during a bowel lengthening procedure after it was retained for 6 months. Patient #2 was a 6 year old female (14 kg) with cloacal exstrophy and short gut syndrome who had severe anemia of unknown etiology. The capsule was retained in a cavernous portion of bowel proximal to a tight ileostomy, and was removed with a Roth net during ileoscopy 20 days later. Patient #3 was a 16 year old male (62 kg) with severe Crohn’s disease and a history of strictures who underwent capsule study to evaluate the extent of small bowel Crohn’s in preparation for surgical intervention. The patient remains asymptomatic with the capsule in a dilated portion of jejunum after 5 months. Conclusions: 1. Medical and surgical history appear be more important than patient size in anticipating risk of capsule retention in children. 2. Retained capsules appear to be safe for at least 6 months. 3. Retention rate in pediatrics appears to be similar to published reports in adults. 93 COW'S MILK PROTEIN‐INDUCED ENTEROCOLITIS MIMICKING SEPSIS IN A NEONATE R. Manley‐ Markowski, A. Leiby, , Goryeb Children's Hospital, Morristown, NJ; Food protein‐induced enterocolitis syndrome (FPIES) is a severe non‐IgE‐mediated allergic reaction. The resultant severe GI symptoms can mimic more common life‐threatening conditions including sepsis and surgical emergencies. We present a twenty five day old female who presented with occult blood positive diarrhea, dehydration, feeding intolerance, metabolic acidosis, and lethargy. She was breastfed and supplemented with a lactose‐free, cow protein formula. Family history revealed fish allergy and asthma. On exam, she was hypothermic, pale, somnolent and dehydrated. Laboratory tests showed leukocytosis with bandemia, thrombocytosis, hypoalbuminemia and a non‐anion gap metabolic acidosis. A complete sepsis evaluation was performed. The baby received intravenous fluids and empiric antibiotics, all followup cultures were negative. Abdominal ultrasound demonstrated mildly dilated, fluid filled small bowel loops. Additional diagnostic tests, including urine electrolytes and serum amino acids did not reveal significant abnormalities. FPIES secondary to cows milk was suspected and a hydrolyzed formula was initiated. Stools continued to be frequent and positive for occult blood. Elemental formula was started on day three which heralded a rapid decrease in stool output, and metabolic improvement. Iron supplementation and famotidine were initiated on discharge. At four month followup, the baby was clinically well with good growth and resolving anemia. CONCLUSION: This case supports the need for greater awareness of FPIES and the need to include FPIES in the differential diagnosis of toxic‐ appearing, acidotic infants who have a history of feeding difficulties and gastrointestinal symptoms. 94 COLORECTAL ADENOCARCINOMA AND SOLID PSEUDOPAPILLARY TUMOR OF THE PANCREAS IN AN ADOLESCENT GIRL D. Mogul, M. Trucco, B. Barger‐Kamate, D. Loeb, F. Giardiello, A. Scheimann, , Johns Hopkins, Baltimore, MD; BACKGROUND: Patient is a 15 year old girl with a 3 month history of anorexia, abdominal pain, bloody diarrhea and 30 lb weight loss. INITIAL EVALUATION: Exam revealed a cachetic girl with tachycardia and two 4‐5 cm irregular pigmented areas on the right thigh. Initial labs were significant for Hgb 7.8 gm/dl (MCV 69), platelets 670 K/cc, and ESR 35. Abdominal CT scan showed a 5 x 7 cm mass of the pancreatic tail and thickened rectal wall. ADDITIONAL WORKUP: Sigmoidoscopy revealed a well demarcated area beginning at 7 cm from the anus with severe colitis. Rectal biopsy showed atypical glands in the background of inflamed granulation tissue, suggestive of adenocarcinoma. IBD serology 7 was significant for (+) ASCA IgA (35.1 EU/ml), borderline anti‐CBir1 (20.3 EU/ml; negative is <21), (‐) ASCA IgG, (‐) Anti‐OmpC, (‐) pANCA. CA 19‐9 was 38.8 U/ml; and CEA was 8.6 ng/ml. Patient underwent resection of pancreatic mass and full thickness rectal biopsy. The pancreatic mass was a solid pseudopapillary neoplasm positive for CD10 and β‐catenin. The rectal mass was confirmed as an infiltratring adenocarcinoma with prominent mucin production. MANAGEMENT: Patient underwent sigmoid colostomy, ovary pexy with stage IV disease. She received chemotherapy (cisplatin, 5‐fluorouracil, leucovorin, Avastin) and external beam radiation therapy. Five months after diagnosis, she underwent complete tumor resection with negative margins. The plan for her is to receive 6 months of concomitant therapy, followed by takedown of her colostomy. Through the high‐risk colorectal cancer clinic, evaluation for germline mutations in β‐catenin, Trimbath variant HNPCC, and MYH‐associated polyposis are underway. CONCLUSION: We report on the first case of a patient with both colorectal adenocarcinoma and solid pseudopapillary tumor of the pancreas. A germline mutation in β‐catenin may link the two tumors, although such a finding has never been reported. Workup for germline mutation is pending. It is difficult to determine the role for positive IBD serologies in the setting of confirmed colorectal adenocarcinoma. 95 FAMILIAL CURRARINO'S TRIAD PRESENTING AS ENCOPRESIS IN A 3 YEAR OLD FEMALE A.F. Har, M. Pfefferkorn, Pediatric Gastroenterology, Riley Hospital for Children ‐ IUSM, Indianapolis, IN; Background: Currarino’s Triad is a rare AD inherited syndrome consisting of an anterior sacral bone defect, presacral mass, and hindgut anomaly – only about 250 cases have been reported in the literature. The presacral mass is most commonly a myelomeningocele, which may be associated with an enteric fistula thereby necessitating emergent surgical intervention. Teratomas are also common and there have been cases of malignant transformations. Other associated defects include spinal cord, renal, or gynecologic anomalies. The defects are caused by mutations in the homeobox gene HLXB9 located in the 7q36 region. Expression of the syndrome is highly variable; however most will have a sacral bone defect. Constipation is the most common presenting symptom, but many may remain asymptomatic. Case: A 3 yo girl presented with fecal incontinence and a bowel pattern of ≥10 stools a day since birth. Her physical exam was normal. She was hospitalized at 2 years of age for fecal impaction and rectal biopsies showed normal ganglion cells. Anorectal manometry documented the rectoanal inhibitory reflex. KUB revealed a scimitar sacrum and spinal MRI revealed a sacrococcygeal teratoma. During resection of the teratoma, a small meningocele was found intraoperatively. Family History: Upon identification of our index case, an extensive family history consistent with incomplete Currarino’s Triad was revealed. MGM has a scimitar sacrum. Two maternal aunts have sacral dimples, 1 of whom complains of limb paraesthesia along with spinal anomalies and a malformed uterus. The mother, who has a bicornuate uterus, also experiences limb paraesthesia leading to a diagnosis of a tethered cord. The maternal uncle had anal stenosis requiring surgery as a neonate. Conclusion: Currarino’s Triad should be considered as a potential diagnosis in the setting of constipation and a sacral bone defect. A MRI of the lumbosacral spine would help identify any presacral mass or spinal cord anomalies. Identification of an index case may aid in the diagnosis of other family members. 96 NOVEL PULMONARY MANIFESTATIONS IN A CASE OF ATYPICAL CROHN'S DISEASE S.S. Lusman, S. Zaidi, D. Volpert, J. Isgro, N. Ovchinsky, Pediatrics, Columbia University, New York, NY; Background: Approximately 25 to 30% of patients with Crohn’s disease (CD) present before the age of 20 years. Presentation before the age of five is extremely unusual. Pulmonary manifestations of CD have rarely been described. We report an atypical case of CD with airway stenosis. Case Report: A three‐month old girl presented with hematochezia and epistaxis. Infectious and hematologic workups were negative. Initial endoscopy was inconclusive; however, subsequent biopsies revealed cryptitis and granulomas consistent with CD. Serologic testing was also consistent with CD. She continued to be symptomatic and corticosteroids and azathioprine were started with limited response. At age two she underwent induction with infliximab and her disease was brought into remission. One year later she developed diarrhea and increased stool frequency. She was switched to adalimumab with resolution of her symptoms. Repeat endoscopy demonstrated only mild residual chronic inflammation. At age three she was evaluated for dyspnea and wheezing refractory to inhaled steroids. CT of the chest revealed right lower lobe collapse. Bronchoscopy showed a membranous lesion in the right lower bronchus; biopsies of the lesion showed chronic inflammation without granulomas. Subsequent bronchoscopies revealed bronchial stenosis with epithelialization in multiple lobes. There was no evidence of parenchymal involvement. Nasal septal biopsy showed acute and chronic inflammation without vasculitis or granulomas. Immunologic evaluation revealed no evidence of a known underlying immunodeficiency, and testing for sarcoidosis and chronic granulomatous disease was negative. Adalimumab was continued and a regimen of systemic steroids was initiated to prevent further airway stenosis. Conclusions: We report an atypical presentation of CD with significant pulmonary involvement. The gastrointestinal symptoms responded to anti‐TNFα therapy; however, the pulmonary disease worsened. This case illustrates that the extra‐intestinal manifestations of CD may be difficult to manage with currently available therapies. 97 CELIAC CRISIS WITH SEVERE HYPOKALEMIA AND PARAPLEGIA AS A FIRST PRESENTATION OF CELIAC DISEASE IN A CHILD E.M. Altamimi, Pediatric, Mu'tah University, Alkarak, Alkarak, JORDAN; Celiac disease is an autoimmune disorder, triggered by an immunologic reaction to ingested gluten that results in cytokine and antibody mediated damage to the small intestine primarily,leading to severe villous atrophy. Celiac crisis is a rare complication, characterized by explosive watery diarrhea,impressive abdominal distension, dehydration,hypotension,lethargy and accompanied by profound electrolyte abnormalities including dangerously low potassium levels and severe acidosis.Our patient was admitted first time with watery diarrhea with severe weakness, which progressed to inability to walk.Both lower limbs were weak with hyporeflexia.Her labs showed severe hypokalemia and acidosis.The patient developed ventricular tachycardia and required admission to the PICU.She received multiple boluses of potassium chloride.Patient discharged after normalization of her K on stable condition. She was readmitted with an identical clinical picture.The patient found to be anemic with prolonged prothrombin time, hypoalbuminemic and normal liver enzymes.Bleeding tendency was corrected.Upper endoscopy showed features of celiac disease in the duodenum which was confirmed by histopathology.The patient was started on a gluten free diet along with iron and multivitamins and treatment dose of Vit D.She returned after 2 weeks and had added weight but now had carpopedal spasm due to hypocalcemia. She responded to Calcium supplementation. Last follow up 4 months after discharge, the patient had gained 6.5 kilograms and her Blood Count, Albumin, INR and Electrolytes were all normal. In summary, we described a young girl with celiac disease presenting with celiac crisis‐acute diarrhea leading rapidly to dehydration, severe acidosis, and hypokalemia and paralysis. Her long‐standing clinically mild celiac disease was not diagnosed prior to and even after the first hospitalization. The case demonstrates the variable presentation of the disease, which can include acute and life‐ threatening situations for which early diagnosis and treatment are mandatory. 98 COLONIC PERFORATION IN PATIENT WITH POSTTRANSPLANT LYMPHOPROLIFERATIVE DISORDER (PTLD) TREATED WITH RITUXIMAB E. Kutsch, H. John‐Kelly, K. Furuya, Gastroenterology , Alfred I. duPont Hospital for Children, Wilmington, DE; K. Furuya, Solid Organ Transplant, Alfred I. duPont Hospital for Children, Wilmington, DE; D. Consolini, DRS & Solid Organ Transplant, Alfred I. duPont Hospital for Children, Wilmington, DE; 3 yo female, with history of a heart transplant in 2008 for hypoplastic left heart syndrome whose immunosuppresion consisted of tacrolimus, was admitted for dehydration and hemoccult positive diarrhea. Her EBV PCR level was 2307 genomes. In response to her clinical presentation and EBV PCR, her tacrolimus dose was decreased with the presumptive diagnosis of PTLD. Her watery stools then turned melenic. Magnetic resonance enterography of the abdomen demonstrated changes consistent with PTLD and colonoscopy showed ulceration with overlying melenic stool in her sigmoid colon. Biopsies from this area confirmed PTLD. She was treated with steroids, bowel rest and rituximab. Repeat colonoscopy 4 weeks later revealed a large perforation in her sigmoid colon. This segment was resected and the pathology again showed persistent PTLD. Tacrolimus dosing was further reduced. One month later, her repeat EBV PCR was 2. PTLD is a rare but potentially fatal complication of immunosupression after solid organ transplantation. Development of PTLD is often linked to primary infection or reactivation of EBV. Our patient had clinical, radiological and pathologic changes consistent with PTLD. With cases of PTLD in the GI tract, there is a higher propensity for ulceration and perforation. Rituximab, a recombinant chimeric anti‐CD20 monoclonal antibody, has been used successfully to treat PTLD. However, it has been associated with spontaneous gastrointestinal perforation due to the rapid chemotherapeutic lysis of tumors in the GI mucosa. In our case, it is believed that our patient’s bowel perforation was due to both underlying PTLD and use of rituximab. In patients with PTLD treated with rituximab who present with GI symptoms, a high index of suspicion of bowel perforation should be considered to prevent further gastrointestinal complications and mortality. 99 OBSCURE CAUSES OF SEVERE GASTROINTESTINAL BLEEDING (GIB): ILEAL HEMANGIOMAS M.M. Fisher, Pediatrics, University of Nebraska Medical Center, Omaha, NE; P.J. Palomo, F. Zapata, Pediatric Gastroenterology, University of Nebraska Medical Center, Omaha, NE; Introduction: Vascular anomalies are common in infants but are rare when found in the small intestine. Ileal hemangiomas (IH) are among the rarest causes of GIB in children. We present 2 cases of ileal hemangiomas as the main cause for GIB. The severity of the GIB can be life threatening and patients can require blood transfusions prior identification of the hemangiomas. The cases: The first case is a 44 day old female with cystic encephalomalacia, seizures, poor oral skills and 3 day history of melanotic progressing to bright red bloody stools. Initial hemoglobin (Hb) was 7.8 mg/dl. Upper and lower scopes were negative. A gastrostomy tube was scheduled to be placed due to poor neurologic prognosis and failure to thrive. During surgery, a red, thickened area on the serosal surface of the ileum was found and resected. It was diagnosed by pathology as an IH. She had no other vascular anomalies. The second case is a 25 day old female infant who presented with two days of melanotic stools. The initial Hb was 3.8 mg/dl. Again, endoscopies and radiologic imaging studies were negative. A capillary hemangioma of the ileum was found on the biopsy during laparotomy assisted by small bowel endoscopy. Additionally, this patient had one small vascular stain on her lower lip and one left sublingual hemangioma. Both patients required packed red blood cell transfusions prior to diagnosis. The first patient had resolution of GIB after surgical removal of the IH. The second patient had continued with GIB and required treatment with systemic steroids for hemangioma regression. The patient has not had any other episodes of GIB. Discussion: These cases illustrate rare causes of GIB and the diagnostic and management strategies employed. Gastrointestinal hemangiomas can present with abdominal pain, intermittent GIB, anemia, obstruction or intussusception. They must remain in our differential as they are significant and require a thorough work up to diagnose. 100 MANOMETRIC IDENTIFICATION OF SEGMENTAL HYPOGANGLIONOSIS IN THE DISTAL ILEUM OF A CHILD WITH CHRONIC INTESTINAL PSEUDO‐OBSTRUCTION. K. El‐Chammas, M. Sood, Pediatric Gastroenterology, Medical College of Wisconsin, Milwaukee, WI; R.P. Kapur, Department of Laboratories, Seattle Children's Hospital, Seattle, WA; Introduction: Severe myenteric hypoganglionosis is a recognized cause of chronic intestinal pseudo‐obstruction (CIP), but primarily has been described as colonic or pan‐intestinal. We report a 6 year old with CIP and segmental hypoganglionosis of the distal ileum. Case: A female infant was born full term and passed meconium within the first day of life. She presented with bilious emesis and abdominal distension at 5 days of age. She underwent exploratory laparotomy and rectal and colon biopsies, which showed ganglion cells. Vomiting continued with episodes of dehydration and she was started on parenteral nutrition (PN). She had repeat laparotomy at 6 weeks of age and ileostomy with mucus fistula was created. She was referred to our Motility Center at 8 months of age; antroduodenal and colon manometry studies revealed neuropathic CIP. The distal 25 cm of the ileum showed continuous phasic contractions with no antegrade propagating contractions. Contrast study showed a transition zone in the distal small bowel with narrow distal ileum. The ileostomy was revised and the narrow distal 35 cm of ileum was resected. Histological evaluation of the resected ileum showed severe myenteric hypoganglionosis and the colon biopsies had normal ganglion cell density. Following this she tolerated enteral feeds and was weaned off PN. Five years later she is eating normally and apart from occasional episodes of vomiting she is doing well. Conclusion: Myenteric hypoganglionosis restricted to a segment of the bowel, as in our patient, is unusual. Like aganglionic bowel, the hypoganglionic segment in our patient was narrow and showed abnormal manometric findings indicative of an underlying neuropathy. Severe intestinal hypoganglionosis may be diagnosed histologically, but requires generous full‐thickness biopsy or resection. Manometry studies in our patient helped identify the affected bowel and to plan a surgical intervention, which allowed the child to be successfully weaned off PN. 101 IDIOPATHIC GANGLIONEUROMATOSIS OF GASTROINTESTINAL TRACT L. Yoon, R. Sanghavi, Gastroenterology, CMC, Dallas, TX; A. Kaul, Gastroenterology, Cincinnati Children's Hospital, Cincinnati, OH; Intestinal ganglioneuromatosis is a rare condition and defined as a benign neoplastic process of proliferating ganglion cells, schwann cells, and nerve fibers that can affect the gastrointestinal tract. There are pediatric case reports of intestinal ganglioneuromatosis associated with neuroendocrine tumors (VIPoma, MEN IIB, NF‐1); however, there are no known pediatric case reports of idiopathic ganglioneuromatosis of gastrointestinal tract. Herein we report a 13 y.o. asian‐american female who presented with vomiting, abdominal pain, and secretory diarrhea. Initial abdominal CT revealed thickened stomach, small bowel, and rectum suspicious for inflammatory bowel disease, yet endoscopy/histology were not consistent with IBD. Exploratory laparotomy was performed, but revealed no source of abdominal pain and appendix was removed prophylactically. Pathology of appendix revealed ganglioneuromatosis. Due to her persistent dysmotility symptoms, repeat laparotomy to obtain full thickness biopsies was performed and revealed ganglioneuromatosis of stomach, small bowel, and colon. The workup for diseases known to cause intestinal ganglioneuromatosis was negative (no RET/PTEN gene mutations and neuroendocrine workup negative). Antral‐duodenal/colonic manometry of patient revealed neurogenic and myopathic dysmotility. Octreotide was empirically started and therapeutic. Patient also developed renal failure from collapsing variant focal segmental glomerulosclerosis, which has no known association with ganglioneuromatosis. Immunosuppression (prograf and prednisone) was initiated for renal disease. To our knowledge, this is the first reported pediatric case of idiopathic ganglioneuromatosis of gastrointestinal tract. Her management has been mostly supportive. Interestingly, immunosuppression has allowed the patient to steadily wean TPN, antiemetics and narcotics with improvement of bowel wall thickening on repeat CT scans as well. The role of immunosuppressive agents in ganglioneuromatois is unknown. 102 POST‐INFECTIOUS GASTROPARESIS: A CASE SERIES OF THREE ADOLESCENT FEMALES J. Yeh, L. Wozniak, A. Sicolo, M. Ament, Pediatrics, UCLA, Los Angeles, CA; BACKGROUND: Post‐infectious gastroparesis is a subgroup of idiopathic gastroparesis that is rarely reported in adolescents. We describe 3 adolescent females with severe post‐infectious gastroparesis. CASE PRESENTATIONS: Three previously healthy girls (ages 13‐15 years) were referred for persistent nausea, vomiting, and abdominal pain. All lived in the same area in southern California and developed acute onset of symptoms after preceding viral syndromes between November 2009 and January 2010. Gastric emptying scans in all 3 showed severely delayed emptying, with a mean t1/2 of 263 minutes (range 199‐373, normal <90). Electrogastrography was abnormal in 2 of the 3. All were diagnosed with post‐infectious gastroparesis after extensive workup including labs, imaging, and endoscopy ruled out other etiologies. All had only partial response to metoclopramide and erythromycin, and 2 required parenteral nutrition and tube feeds. One patient ultimately underwent placement of a gastric electrical stimulator which improved her symptoms. DISCUSSION: Though no pathogen was identified in our patients, they all had preceding viral illnesses. This in combination with the patients’ close geographic proximity, similar timing of presentation, and near‐identical symptomatology suggests the same infectious agent may have been responsible for all 3 cases. It is unclear if the pathophysiology of post‐infectious gastroparesis is due to inflammation, an immune‐mediated phenomenon, or exacerbation of an underlying dysmotility. Symptoms usually improve spontaneously over time, however pro‐motility agents may be needed. Use of gastric electrical stimulation has been well‐reported in adults, but has only recently been described in pediatrics (Islam, 2008; Hyman, 2009). Post‐ infectious gastroparesis may be an under‐recognized disorder in pediatrics, particularly in adolescents, and if untreated, can lead to significant morbidity. Therefore clinicians should screen for a recent history of viral syndromes and consider testing for impaired gastric emptying in patients with persistent nausea, vomiting, or abdominal pain.