Clinical Vignette Abstracts NASPGHAN Annual Meeting October 10-12, 2013

Poster Session I Eosphagus Stomach

11 Successful Use of Biliary Ducts Balloon Dilator in Repairing Post-Surgical Esophageal Stricture in premature infant. I. Absah, Department of Pediatirc and Hepatology, Mayo Clinic college of Medicien, Rochester, Minnesota, UNITED STATES. Absah, M.B. Beg, Department of Pediatirc Gastroenterology and Hepatology, SUNY Upstate Medical University, Syracuse, New York, UNITED STATES. Background: (EA) with or without tracheoesophageal fistula (TEF) is the most common type of gastrointestinal atresia with occurrence rate of I in 3,000 to 5,000 births. There are 5 major varieties of EA. The most common variant of this anomaly consists of a blind esophageal pouch with a fistula between the trachea and the distal , which occurs in 84% of the time. Treatment is by extra-pleural surgical repair of the esophageal atresia and closure of the tracheoesophageal fistula. Complications may include leakage to the mediastinum, fistula recurrence, GERD, and stricture formation at the anastomosis site. Benign stricture occurs in 40% of the cases after surgical repair. These benign post-surgical strictures cause feeding difficulties and require treatment. Esophageal dilation is 90% effective, but strictures that do not respond to dilation must be resected surgically. Case report: A 52 days old premature baby that was born at 35 weeks presented with feeding difficulty, due to benign esophageal stricture 44 days after surgical repair of EA and TEF. The luminal diameter of the stricture was ≤ 4mm, for that regular balloon esophageal dilators (smallest = 6mm) couldn’t be used. Then a 4mm biliary balloon dilator over 0.035 inch guidewire was successfully used without complications. Dilation was repeated using the biliary balloon dilators 3 times, then regular esophageal dilators were used until feeding difficulty and the stricture completely resolved. Conclusion: Biliary duct balloon dilators can be safely used to dilate narrow esophageal strictures with diameter of 6mm or less. This safe and effective method should be considered prior to seeking other complex alternatives, which may involve retrograde esophageal access or surgical repair

12 Eosinophilic Esophagitis and Esophageal Achalasia in a Teenager with Chronic . E. Barfield, A. Kesavan, T. Ciecierega, Pediatric Gastroenterology and Nutrition, New York Presbyterian-Weill Cornell , New York, New York, UNITED STATES. Introduction: Dysphagia is defined as difficulty. It can present with gagging, coughing or painful swallowing. The differential diagnosis in children is broad and includes anatomical anomalies, eosinophilic esophagitis (EoE), psychogenic causes and esophageal dysmotility. Co-occurrence of multiple pathologies is rare. Prompt diagnosis and treatment is crucial in these patients. We present the case of a 15 year old male with chronic dysphagia caused by co-existing etiologies. Case Description: A 15 year old African American male with repaired congenital esophageal atresia presented to our practice with the complaint of chronic dysphagia since birth. Nissen fundoplication was performed at three years of age for severe gastro-esophageal reflux symptoms not amenable to pharmacotherapy. Recently he complained of worsening dysphagia, odynophagia, burning in the esophagus and postprandial nausea. Barium esophagogram showed distal esophageal narrowing with pooling of contrast and significantly delayed emptying of the esophagus. Upper endoscopy showed active esophagitis in the proximal and mid esophagus with up to 30 eosinophils (eos)/high power field (hpf) suggestive of EoE. He was treated pharmacologically with Fluticasone and acid suppression and subsequently underwent esophageal dilatation of the stenotic area. Because of persistence of dysphagia despite appropriate treatment of EoE (less then 15 eos/hpf) he underwent esophageal manometry testing which demonstrated total esophageal aperistalsis and poorly relaxing lower esophageal sphincter consistent with esophageal achalasia. A repeat esophageal manometry was performed a few months later and remained consistent with esophageal achalasia. Discussion: While EoE is a common diagnosis in children with dysphagia, esophageal achalasia is not. Their co-occurrence is rare and has only been reported on a few occasions. It is important to consider rare causes of dysphagia including esophageal achalasia in children with chronic dysphagia in the setting of appropriately treated EoE. Esophageal manometry should be considered in these patients to exclude concomitant motility disorders.

13 Collagenous Gastritis in a young adolescent male. A. Chan, J. Screws, D. Tolosa, D. Laman, Pediatrics, UT Chattanooga, Chattanooga, Tennessee, UNITED STATES. Collagenous Gastritis was first described by Coletti and Trainer in 1989 and remains extremely rare. Treatment is empirical without defined treatment guidelines. Upon review of the literature, there appears to be two generalized patterns for presentation of Collagenous Gastritis: a pediatric type associated with anemia and abdominal pain and an adult type associated with collagenous colitis and watery diarrhea. We describe a 16-year- old male who presented with intractable postprandial emesis and periumbilical / epigastric pain. Laboratory studies included complete blood count, metabolic panel, urinanalysis, H. pylori, celiac panel, and quantitative immunoglobulins – all normal except for an elevated IgE. Radiographic studies included a normal gallbladder ultrasound and normal CT scan. Initial EGD revealed a and a moderately severe gastritis with concern for NSAID gastropathy, with biopsies showing markedly thickened subepithelial collagen and a conspicuous amount of eosinophils in the fundus with increased intraepithelial lymphocytes in the duodenum. There was erythema of the esophagus as well as erosions and medium sized polyps in the stomach on a repeat EGD three weeks later, with pathology showing moderate chronic gastritis with eosinophils and subepithelial collagen. Colonoscopy had a normal appearance, but biopsies showed increased eosinophils in the colon with a normal terminal ileum. He was tried on multiple medications initially without success, including sucralfate, PPIs, oral budesonide, as well as scheduled antiemetics such as ondansetron and benadryl. IV methylprednisone was given for several days but stopped due to severe side effects. Nitazoxanide / fish oil was trialed but the medicine was not tolerated due to emesis. Biofeedback was initiated for pain control. Prednisolone was initiated and he achieved both symptomatic improvement and histopathologic remission. There is a need for further investigation of this rare and poorly understood disease. Our patient does not fit clearly into either of the previously seen patterns of collagenous gastritis, and further cases and/or case series are needed to help establish treatment guidelines.

14 A 15 year old female with Plummer Vinson Syndrome. S. Edwards, Gastroenterology, Children's Mercy Hospital, Kansas City, Missouri, UNITED STATESM. Manalang, Hematology, Children's Mercy Hospital, Kansas City, Missouri, UNITED STATES. Background Plummer Vinson Syndrome (PVS) is the triad of iron deficiency anemia, dysphagia and esophageal webs. It is typically a disease of middle age to elderly adults. There are few reports of PVS in the pediatric literature. The pathogenesis is not clearly understood. It is important to identify these patients early because there has been evidence, from the adult population, that there is an increased risk for the development of esophageal squamous cell carcinoma. Therefore, it is recommended that patients with PVS be scoped yearly. Case Presentation A 15 year old female with a lifelong history of anemia, thought to be due to alpha thalassemia presented to the hematology department for profound fatigue. She was anemic with a hemoglobin 9.3 g/dL, MCV 58 fL and RDW 17.8%. Ferritin was low at 9 ng/mL. She was diagnosed with iron deficiency anemia and was started on 65 mg of elemental iron by mouth three times daily. Repeat labs, one month later, showed a hemoglobin of 8.7 g/dL with an MCV of 58 fL and reticulocyte percent of 1.5. Given her lack of response while compliant with oral iron therapy, an investigation for occult chronic disease was performed and was negative. Follow up five months later revealed no change in her hemoglobin and persistently low ferritin levels with continued compliance with oral iron therapy. She subsequently failed an iron challenge performed in clinic. Since she was unable to absorb oral iron, a celiac panel was obtained and was negative. She was also referred to gastroenterology for further investigation of possible gastrointestinal malabsorption. She was seen in gastroenterology where she denied any abdominal pain or weight problems. She did report a history of choking and vomiting as a child, which improved as she aged, but continued to have trouble with pill swallowing. An esophagogastroduodenoscopy (EGD) was performed and showed an upper esophageal web. The web was disrupted with the scope and did not warrant any further intervention. Given her iron deficiency anemia, dysphagia and esophageal web, she was diagnosed with PVS. Eventually she was diagnosed with iron resistant iron deficiency anemia (IRIDA) and treated with IV iron. She has severely impaired intestinal iron uptake without evidence of a chronic inflammatory state. She is followed closely by our hematology department and has been able to increase her hemoglobin to 11.6 g/dL, MCV 71 fL, and ferritin 175 ng/mL. She will undergo yearly EGD, celiac panel, and thyroid studies, as part of her disease monitoring. Discussion While the etiology of PVS is unknown, it is important to fully investigate the cause of the underlying anemia in pediatric patients, which include chronic blood loss, including menorrhagia, inadequate iron intake, malabsorption, and inflammatory conditions. There may be an association between Plummer-Vinson and certain autoimmune diseases, especially celiac disease, which stresses the importance of monitoring for these conditions. Regular follow-up with gastroenterology, including annual EGD is recommended because of the increased risk of squamous cell carcinoma of the and esophagus in patients with PVS.

15 Collagenous Gastritis Associated with Celiac Disease in a Pediatric Patient. J. Hefner, M. Goldman, Pediatric Gastroenterology, Walter Reed National Military Medical Center, Bethesda, Maryland, UNITED STATESM. Goldman, Pediatrics, Uniformed Services University of the Health Sciences, bethesda, Maryland, UNITED STATES. Collagenous gastritis (CG) is a rare disease characterized by subepithelial collagen deposits associated with inflammation in the lamina propria. Symptoms are typically anemia, abdominal pain, vomiting, and diarrhea, with other symptoms such as failure to thrive, hemoccult positive stools, hematemesis, and chest pain also being described but less common. Adult type CG is associated with several autoimmune processes and celiac disease which is unusual in the childhood form. We present a case of a teenager with CG and celiac disease. A 13 year old female presented to the Pediatric Gastroenterology clinic for evaluation of poor weight gain. She denied nausea or vomiting, and admitted to decreased appetite when taking stimulant medications for her ADHD. Her stools were regular, soft, and non painful. She denied any bloating, distension, pain, or post-prandial symptoms. No unexplained fever, rash, or joint pain. She began having decreased height and weight gain approximately 2 years prior to her visit. Her medical history was significant for ADHD, delayed puberty, and PDD. She had no prior surgeries, and was struggling in school. Her medication regimen consisted of Clonidine, Daytrana, and Focalin. Labs obtained at a previous Pediatric Endocrinology visit, to include CBC, CMP, ESR and CRP were normal. Celiac serologies were positive for Gliadin ab IgG (106), positive Endomysial Ab IgA, and TTG ab IgA of 13. Her total IgA was normal. Endoscopic biopsies showed intraepithelial lymphocytes and villous blunting of the duodenal bulb, and collagenous bands in antral biopsies. It is interesting to note, the patient did not have the typical presentation with severe anemia and abdominal pain. She presented for evaluation of decreased weight and height velocity. Her screening labs for Celiac disease were positive warranting an EGD where she was incidentally found to have collagenous gastritis. We offer a unique case in which a pediatric patient has both Celiac disease and collagenous gastritis, an association seen mostly in the adult population.

16 Incidental Finding of Gastrinoma in an 8 year old Boy. T. Heifert, Pediatrics, Walter Reed National Military Medical Center, Bethesda, Maryland, UNITED STATESC. Chao, Pediatrics, Inova Children's Hospital, Fairfax, Virginia, UNITED STATES. Gastrinoma is a rare (incidence: 0.5-1.5 new cases per million population per year) neuroendocrine tumor (NET) in children. In children, gastrinomas most often occur sporadically with only ¼ occurring in association with Multiple Endocrine Neoplasia (MEN) Type 1. We present here the case of a child with a gastrinoma in the stomach that was found on random biopsies. An 8 year old boy with history of Down’s Syndrome, pseudo-obstruction with a gastrostomy and ileostomy presented with bright red blood coming from ileostomy and coffee-ground residuals seen in his gastrostomy tube. He had previous EGDs with severe gastritis and duodenitis in August 2011 and August 2012. He was on a proton pump inhibitor and was placed on carafate after August 2012 EGD. He had a repeat EGD in January 2013 with gross appearance of mild gastritis and questionable duodenitis. Pathologic specimen with incidental finding of a small foci of carcinoid in the gastric biopsy confirmed with immunohistochemical stains (CD56 positive, chromogranin positive, Ki-67 low, synapotphysin positive) in addition to acute and chronic gastritis with cryptitis and acute and chronic duodenitis. Gastrin stain done on the carcinoid tissue was positive for gastrin production. He had a nuclear medicine octreotide scan and abdominal MRI in March 2013. The octreotide scan was positive for left upper quadrant activity suggestive of a possible gastrinoma in the epigastric area. The MRI was negative with no focal abnormality in the stomach or pancreas to correspond with finding on octreaotide imaging. Laboratory evaluation revealed gastrin level of 234 pg/ml (normal 13-64), chromogranin A 154 ng/ml (normal 1.9-15). These labs were obtained after holding his proton pump inhibitor and other antacids for 2 weeks. Evaluation for MEN type I was negative; family history was unremarkable, serum calcium, parathyroid hormone, prolactin, insulin-like growth factor-1, serum insulin and thyroid function tests were normal. He had an endoscopic ultrasound in March 2013. The gross appearance of the stomach was normal. However endoscopic ultrasound was able to find an area with subtle focal wall thickening in the antrum from which 4 samples were taken with jumbo biopsy forceps. All had pathologic findings consistent with gastrinoma without lymph, perineural, or vascular involvement. In conclusion, neuroendocrine tumors are not only rare in children, but are not typically diagnosed until late in presentation. We describe a case where gastrinoma was incidentally found at very early stages. In this case, the patient's only symptom was severe chronic gastritis managed with long term PPI therapy. For future cases of severe unrelenting chronic gastritis, one should consider checking a gastrin level (off PPI), and if abnormal consider an octreotide scan.

17 Eosinophilic Esophagitis in 3 Young Siblings. T. Heifert, S. Min, Pediatrics, Walter Reed National Military Medical Center, Bethesda, Maryland, UNITED STATES. Eosinophilic esophagitis (EoE) now represents a commonly recognized disorder among both pediatric and adult patients throughout the world however the genetics and hereditability are not yet well understood. A genome- wide association study has indentified an EoE susceptibility locus on 5q22. In addition, there is evidence of familial association as almost 10% of EoE patients with evidence of esophageal strictures have a parent diagnosed with EoE. We present a family with 3 siblings all diagnosed early in life with eosinophilic esophagitis. Sibling A is a 7 year old female who was diagnosed with EoE at age 2 after she had an EGD for abdominal pain and diarrhea. She has a history of seasonal allergies but no asthma, eczema, or food allergies. While her symptoms initially improved after a course of swallowed fluticasone, they had clearly progressed after stopping her medication. Over the past year, she also went on to develop chronic vomiting. Her most recent endoscopy, while on a proton pump inhibitor, was notable for mucosal linear furrowing and > 50 eosinophils/high power field (hpf) in both the proximal and distal esophagus. Sibling B is a 4 year old male with no other atopic symptoms, who had a history of emesis from early infancy. Given the persistent vomiting, he was formally evaluated at 16 months of age by a pediatric gastroenterologist and was diagnosed with eosinophilic esophagitis based on EGD findings and pathology. He too was treated with a short course of swallowed fluticasone but represented for care at age 4 due to return of vomiting and increase in frequency over the preceding 6 months. His most recent EGD, while on a proton pump inhibitor, also showed linear furrowing and had up to 100 eospinophils/hpf in proximal esophagus and > 50 eosinophils/hpf in distal esophagus. Sibling C is a 19 month old male who has had a history of vomiting and reflux from birth. He continued to have intermittent vomiting well beyond one year of age. He has no other atopic history. He had an EGD at 16 months of age after being on a proton pump inhibitor for 6 weeks which showed linear furrowing, with > 25 eosinophils/hpf in the distal esophagus and > 50 eosinophils/hpf in the proximal esophagus. It is interesting to note that both parents share various degrees of atopy (father with asthma, mother with multiple seasonal and food allergies), however, none of the three siblings have much of any notable atopic history. Furthermore, while there is a paternal grandfather with a history of EoE, neither parent endorses any history of GERD, or dysphagia and has not been diagnosed with EoE. This family cohort provides interesting insight into the hereditability of EoE. While other cases linking parents and their offspring with EoE, or reports of older siblings with EoE, have been published, we offer a unique case of three young biological siblings with EoE diagnosed at an early age.

18 Massive Hematemesis as presentation of Congenital Aortic Coarctation with Mycotic Aneurysm. E.J. Hoffenberg, M. Sheiko, Pediatrics, Univeristy of Colorado School of Medicine, Aurora, Colorado, UNITED STATESJ. Bruny, Surgery, Univeristy of Colorado School of Medicine, Aurora, Colorado, UNITED STATES. Case Presentation: A 15 year old male was admitted with 3 weeks of vague upper back pain, fever, prolonged use of ibuprofen, and 1 week of emesis progressing to hematemesis. Exam was notable for blood pressure of 148/89, heart rate 126, and 2/6 systolic murmur. Abdomen was soft and there were no rashes, telangiectasias or splinter hemorrhages. Hemoglobin was 9, albumin 2.3, ESR 111, INR 1.4. After ongoing overnight hematemesis and requirement of a blood transfusion, emergent endoscopy showed only blood in the stomach which was removed, revealing no active bleeding or source. With progressive hemodynamic instability additional unrevealing interventions included repeat endoscopy with video which showed new blood but no active bleeding or source, angiography of celiac, gastroduodenal, left gastric, and SMA vessels, and CT angiogram. Finally, a repeat interventional angiography identified an aortic coarctation with multiple collaterals and extravasation into the esophagus through a very restrictive non-pulsatile aortoesophageal fistula (AEF). An aortic stent did not stop the bleeding so aortic resection with graft placement was performed. Pathology report indicated aorta with extensive acute neutrophilic inflammation in the wall, including focal disruption of the media and focal necrosis with transmural fibrin, extensive surrounding acute inflammation and granulation tissue. Esophageal tissue was not seen. There were numerous scattered Gram positive cocci and diplococci in the background. Blood cultures grew Streptococcus pneumoniae. The patient survived with 96 units of PRBC in 48 hours, a mild stroke leading to slight delay in processing speed, mediastinal infection requiring esophageal diversion and prolonged antibiotics, and severe gastroparesis due to vagus nerve injury. Review of the endoscopic video identified a non-pulsatile purple mass protruding into the esophagus, felt to be the aneurysm. Discussion: AEF with hematemesis is associated with high mortality. A congenital coarctation with mycotic aneurysm, AEF, and massive bleeding is extremely rare. We identified only 2 case reports in the literature. One is of an 11 year old male who survived and the other a 14 year old male who died, both with staphylococcal mycotic infection. At 15, our patient is older than the two reported. Early recognition is essential for survival. Recognition of hypertension, the purple mass and its significance, and the massive bleeding, may have led to earlier recognition of the large vessel source of bleeding. The mass was not pulsatile, due to a very restrictive fistula to the aneurysm. Use of a Blakemore tube may have been helpful.

19 Esophageal Foreign Body as a Cause of Chronic Vomiting and Dysphagia. N. Ibrahimi, O.F. Almadhoun, Pediatrics, Kansas University Medical Center, Kansas City, Kansas, UNITED STATES. A 13-year-old boy presented with epigastric abdominal pain, vomiting, and dysphagia for 2 years. Patient had a history of Nissen fundoplication at 5 months of age for GE reflux. Physical exam was normal. Patient had a normal Upper GI series and failed a trial of antacid. An upper endoscopy was then performed and showed a 5 cm long cord like foreign body (FB) in the distal esophagus with surrounding erosions and ulcerations. The FB was retrieved and patient had complete resolution of symptoms. Chronic esophageal FB (CEFB), defined as a FB present for over 1 week, appears to constitute about 5-8% of all esophageal FB in children. CEFB in children usually present with respiratory symptoms but upper GI symptoms including vomiting and dysphagia are also common. Esophageal abnormalities, like TE fistula repair, esophageal strictures, and eosinophilic esophagitis can predispose patients to have a retained FB. Fundoplication may also predispose to FB retention which may require a prompt endoscopic retrieval. Physicians should have high level of suspicion for CEFB in children presenting with chronic respiratory or upper GI symptoms with no other apparent causes, especially in patients with history of fundoplication or other esophageal abnormalities.

20 Real Time Use of Glucagon for Foreign Body Extraction. S. Lapsia, A. Chawla, J. Morganstern, Pediatric Gastroenterology and Nutrition, Stony Brook Long Island Children’s Hospital, Stony Brook, New York, UNITED STATES. Foreign body ingestion is a common problem in pediatrics. Among these objects are harmful and life-threatening items such as pins, button batteries, and magnets. These objects come in various shapes and sizes and can lead to mild to severe irritation of gastrointestinal mucosa, bleeding, and dreaded complications such as obstruction or perforation. Glucagon is an endogenous polypeptide that is secreted from alpha cells of the islets of Langerhans in the pancreas. Several studies have shown that glucagon may relieve esophageal foreign body impaction in one third of cases via smooth muscle relaxation of the esophageal mucosa. Here we describe a case of a child in whom intraoperative use of glucagon aided in the successful endoscopic removal of 2 adherent magnets from the mid esophagus. The patient is a 3 year old female who was brought to the ER after admitting to her mother that she had ingested two neodymium magnets in succession. An upper endoscopy revealed the objects to be adhered together in the body of the stomach, effectively creating a single object measuring 20mm x 18mm x 7mm in size. A Roth Net® standard retriever was used to capture the magnets. However on multiple attempts at retrieval there was significant resistance noted in the mid esophagus and tearing of the net occurred. After other maneuvers were unsuccessful, intravenous glucagon at a dose of 1 milligram was given over 1 minute. The two magnets were then removed using a Roth Net® approximately 2-3 minutes after glucagon administration without any resistance and with no complications. The risks associated with glucagon use are minimal, especially when weighed against potential complications that may arise from hazardous foreign bodies being present in the gastrointestinal system or the need for surgical intervention. Use of glucagon during endoscopy for esophageal relaxation to assist in foreign body removal has not been previously reported in the literature and should be considered in difficult foreign body extractions.

Hepatobiliary/Transplant 34 Hepatopulmonary syndrome associated with nodular regenerative hyperplasia after liver transplantation in a child. R. Alhosh, Y. Genyk, D. Thomas, N. Kerkar, Pediatrics GI, CHLA, Los Angeles, California, UNITED STATESS. Zhou, Pathology, CHLA, Los Angeles, California, UNITED STATES. Background: Hepatopulmonary syndrome (HPS) or (Pulmonary arterio-venous shunting) is a significant complication of portal hypertension in children with chronic liver disease and is an established indication for liver transplant (LT). It is characterized clinically by signs of hypoxia due to intrapulmonary shunting. Nodular regenerative hyperplasia (NRH) is a cause of non-cirrhotic portal hypertension. Microscopically, nodules are formed by the liver cells distorting the normal architecture, but diagnosis may be challenging by needle biopsy. Development of NRH and HPS in pediatric LT recipients has not been reported, although occasional cases have been reported in adult LT recipients. Case Description: A 3 year old male developed HPS, 2 years after LT. It manifested clinically by hypoxemia (seen on pulse oximetry and arterial PaO2) and shortness of breath. He required supplemental oxygen via nasal cannula. Pulmonary and cardiac causes for hypoxemia were ruled out by appropriate investigations. The diagnosis of HPS was confirmed via Bubble Echocardiogram that demonstrated the intrapulmonary shunting. A liver biopsy showed Nodular Regenerative Hyperplasia. The boy underwent liver re- transplantation that resulted in complete reversal of his pulmonary symptoms and normal oxygen saturations after weaning off the supplemental oxygen within 3 months after transplant. Conclusion: This is the first report of Hepatopulmonary syndrome occurring after liver transplantation in a pediatric recipient. The association with NRH is also unique. Liver re-transplantation has been curative in the subject of this report.

35 Rhabdomyosarcoma of the Biliary Tree Mimicking a Type 1 Choledochal Cyst. T.A. Altepeter, K.N. Furuya, E. Kutsch, Pediatric Gastroenterology, Nemours AI Dupont Hospital for Children, Wilmington, Delaware, UNITED STATES. A healthy 2 year old presented with diarrhea and jaundice for 2 days. Initial laboratory evaluation was notable for the following: AST 153 U/L, ALT 178 U/L, total bilirubin of 3.9mg/dL, direct bilrubin 1.4mg/dL, GGT 1037 U/L. Ultrasound demonstrated intrahepatic ductal dilatation, a markedly dilated hepatic duct (2.2cm) and common bile duct (1.7cm). MRCP showed fusiform dilatation of the common bile duct measuring up to 16 mm in diameter that was thought to represent a type 1 choledochal cyst, and a 1.3 cm nodule located in the posterior segment of the liver. There was also marked lymphadenopathy present, including a conglomerate of nodes at the head of the pancreas measuring 3.1 x 3.1 x 2.2cm. During surgical excision, yellow colored gelatinous material was found within the dilated common bile duct. The fusiform dilated portion of the duct was removed. Lymph nodes adjacent to the structure were noted to be enlarged and firm/fibrotic in consistency. Gross examination of the liver surface noted several white spots. Pathologic examination revealed botyroid embryonal rhabdomyosarcoma with anaplasia with positive resection margins. Subsequent evaluation identified metastasis into local lymph nodes. PET scan did not suggest involvement of the liver parenchyma. The patient was started on chemotherapy via COG protocol ARST0331, including actinomycin, vincristine and cyclophosphamide. She is currently being evaluated for possible proton radiation therapy. Rhabdomyosarcoma of the biliary tree is a rare childhood cancer. The biliary tree is an uncommon location for pediatric rhabdomyosarcoma. In children with suspected choledochocal cyst, it is important to consider malignancy in the differential, and for surgery to be performed by those with experience in excising biliary tract tumors, should the need arise.

36 False Negative Alpha-1 Antitrypsin Phenotype in a Patient with A1AT Deficiency with Concomitant CMV Infection Leading to Liver Failure Arias, Patricio; Kerner, John; Berquist, William; Park, KT. P. Arias, J. Kerner, W. Berquist, K.T. Park, Stanford, Palo Alto, California, UNITED STATES. This is a 4 month old female with neonatal cholestasis who presented with jaundice, ascites, coagulopathy and worsening transaminitis. At 5 weeks of age, her mother noticed yellowing of eyes and skin. Stools were initially yellow, but a few days prior to presentation, the parents noticed that stools became pale in appearance. At the time of her first hospitalization, labs were notable for conjugated hyperbilirubinemia, transaminitis, and elevated alkaline phosphatase. The patient was started on ursodiol, but her liver function tests and conjugated bilirubin remained stably elevated. A percutaneous liver biopsy was performed, with initial histopathology consistent with large duct obstruction. A subsequent intraoperative cholangiogram and ERCP were negative for . An alpha-1 antitrypsin (A1AT) phenotype was performed, and initially showed MZ heterozygosity. Quantitative A1AT levels were low at 34 (normal value is 100-250mg/dl). Based on the MZ phenotype, no clinical correlation was initially made to explain the cholestasis and liver dysfunction. After other screening labs were reassuring (including CF genotyping), patient was discharged home with close follow up with GI. In the following weeks, due to persistent cholestasis and a repeat low A1AT level, a second A1AT phenotype was sent. The repeat A1AT phenotype showed ZZ homozygosity. A second review of patient’s liver biopsy on immunohistochemistry staining showed positive intrahepatic PAS granules, resistant to diastase. During patient’s ongoing cholestasis workup as outpatient, significant elevations in CMV IgM were seen and confirmed by elevated CMV PCR of 12,800 copies. Shortly thereafter, patient presented with rapid increase in abdominal distension and ascites. Worsening cholestasis, transaminitis, and progressive coagulopathy were noted on the second hospitalization. Synthetic liver function deteriorated and daily doses of fresh frozen plasma were necessary. She was evaluated for orthotropic liver transplant and was listed with a PELD score of 30. The etiology of her acute deterioration is based on a “second hit” hypothesis, whereby the concomitant CMV infection induced rapid progression to liver failure in the context of A1AT deficiency. A1AT phenotyping is determined by electrophoresis and is not 100 % accurate . Clinically significant liver disease develops in only 10% to 15% of 1-antitrypsin (AT)–deficient children . There is no specific therapy for AT deficiency-associated liver disease and in cases of liver failure, orthotopic liver transplantation remains the only alternative with survival rates well over 90% at 1 year, and 80% at 5 years.

37 Developmental Outcomes After Orthotopic Liver Transplant for Children with Neonatal-onset Ornithine Transcarbamylase Deficiency: A single Center Experience.. R. Bader, J. Andersen, Pediatric gastroenterology , University of Texas Southwestern , Dallas , Texas, UNITED STATESR. Bader, J. Andersen, Children's Medical Center, Dallas, Texas, UNITED STATES. Although the survival of patients with Ornithine Transcarbamylase deficiency (OTCD) has improved with nutritional therapy, medications, hemodialysis and liver transplant, the neurologic outcomes are suboptimal. Children are at a high risk for cerebral damage, mental retardation and other developmental disabilities. We report a single center experience in management and transplantation of children with OTCD with particular focus on their developmental and cognitive outcomes. We retrospectively reviewed the charts of the 6 patients with neonatal- onset OTCD who underwent deceased donor orthotopic liver transplant in our center. Developmental and cognitive outcomes continue to be unfavorable. In summary, all the patients presented in the first few days of life. Despite strict dietary and pharmacological therapy, hyperammonemia control was difficult. All of them required dialysis. The age at transplant ranged between 6 weeks to 17 months. A single mortality occurred in the immediate post transplant period in the 6 week old infant. Of the 5 survived, all were left impaired neurologically with developmental delays despite complete metabolic correction. The child with the best developmental outcome was transplanted at 7 month of age. Of note, he was enrolled very early in speech, physical and occupational therapy. Summary of patient characteristics.

38 Neonatal acute liver failure as a presentation of congenital hemophagocytic lymphohistiocytosis. M.K. Boruta, C. Mack, R.J. Sokol, Digestive Health Institute, Children's Hospital Colorado, Aurora, Colorado, UNITED STATES. Hemophagocytic lymphohistiocytosis (HLH) is a hyperinflammatory syndrome due to excessive activation of T- lymphocytes and histiocytes in the setting of deficient inhibitory feedback from NK cells. The interruption in the natural termination of the inflammatory process leads to excessive production of cytokines and the characteristic symptoms of fever, pancytopenia, multi-organ injury, coagulopathy, hepatosplenomegaly, and neurological symptoms. The disease is characterized by hemophagocytosis by abnormally activated histiocytes and more commonly presents in early childhood. We report an interesting case of HLH presenting in the neonatal period as acute liver failure. A 4 day old previously healthy term male infant presented with cardiovascular collapse and respiratory arrest at home. He was noted to have hypothermia, hypoglycemia and lactic acidosis on arrival. Initial evaluation showed prominent hepatosplenomegaly and laboratory tests consistent with acute liver failure with protime 42.7 sec (13.2-16.5 nl), INR 4.48, AST 2206 U/L (30-100) and ALT 511 U/L (6-40). Initial direct bilirubin was 0.1 mg/dL (0-0.6) and total bilirubin 10.1 mg/dL (0.1-11.5), but he later developed cholestasis. Ultrasonography with Doppler flow demonstrated a non-occlusive thrombus in the main portal vein with extension into the right anterior portal vein. Initial metabolic, infectious, mitochondrial, congenital disorders of glycosylation and inborn error of bile acid synthesis screenings were negative. Serum ferritin was extremely elevated at 50,200 ng/ml (nl<400). Further evaluation for HLH showed pancytopenia, absent NK cell activity, low fibrinogen and elevated IL- 2R, meeting the diagnostic criteria for HLH. Genetic testing was negative for mutations commonly seen in familial hemophagocytic lymphohistiocytosis (FHL). The patient progressed to multi-system organ failure despite chemotherapy, but did have recovery of liver synthetic function. This case highlights a rare cause of neonatal liver failure. A diagnosis of HLH should be considered and screening ferritin and fibrinogen obtained when initial evaluation for acute liver failure etiologies is not clear.

39 A Tale of Two Wilson's. L. Chen, S. Abrams, D.H. Leung, Pediatrics, Baylor College of Medicine, Houston, Texas, UNITED STATESL. Chen, S. Abrams, D.H. Leung, Gastroenterology, Hepatology, and Nutrition, Texas Children's Hospital, Houston, Texas, UNITED STATES. We report a pair of siblings with identical mutations in ATP7B for Wilson’s disease, both with good outcomes, but with radically different clinical presentations. A 15 year old female was admitted with acute onset of scleral icterus and abdominal distension after a four day history of abdominal pain, nausea, and fatigue. She had been healthy, but took Doxycycline for acne. On exam, she was jaundiced and had hepatosplenomegaly. Ultrasound revealed liver nodularity and portal hypertension, suggestive of a chronic disease. Labs confirmed acute on chronic liver failure (see table 1), and she was listed for liver transplant with a MELD of 33 within one day. She gradually became encephalopathic with an abrupt rise in serum creatinine to 2.7 mg/dL and was started on continuous renal replacement therapy for hepatorenal syndrome. She developed a Coombs-negative intravascular hemolysis and gastrointestinal bleeding requiring daily transfusions. Her ceruloplasmin was 4.6 mg/dL (18-46), 24 hour urine copper was >250 ug/dL (0.2-8.0), and exam showed no Kayser-Fleischer rings. Given her worsening encephalopathy, coagulopathy (INR peak - 4.7), and cholestasis (conjugated bilirubin peak - 64.5 mg/dL), she received 5 days of therapeutic plasma exchange with rapid improvement in her mental status. Nevertheless, her MELD increased to 46, and was later listed status 1B. On hospital day 14, she received a liver transplantation and has done remarkably well since then. Gene sequencing showed two mutations in the ATP7B gene (one copy of c.1145_1151delCCCAACT and one copy of c.3955C>T (9.R1319X) nonsense mutation). Concern shifted to her first- degree relatives. Initial labs indicated the patient’s 10 year old brother may also be affected (see table 1). He had been completely asymptomatic. Liver biopsy was consistent with Wilson’s disease and stage III fibrosis. Trientine 250mg tid was initiated for chelation, and urine and serum unbound copper levels were followed for titration. He was later found to have the same mutations in ATP7B as his sister, but on therapy his liver enzymes normalized and he has remained healthy. This case highlights the use of plasma exchange to improve encephalopathy, the heterogeneity in phenotype of Wilson’s disease, and imparts a sobering reminder of the importance of early detection. Findings at Presentation

40 CITRULLINEMIA TYPE I, ACUTE LIVER FAILURE, NAFLD AND ALPHA-1 ANTITRYPSIN (A1AT) DEFICIENCY. M. Zaretsky, L. Turner, C. Pushpanathan, J. Critch, Memorial University, St. John's, Newfoundland, CANADA. A 13 year old female was diagnosed with citrullinemia type I (CTLN1) as a neonate, presenting with seizures and hyperammonemia. Initial therapy consisted of sodium phenylacetate, sodium benzoate and l-arginine. At 4y3m and 4y6m she presented in metabolic decompensation with hyperammonemia (peak ammonia 242, 163), elevated transaminases (peak ALT 2400, 2100) and prolonged INR (peak 4.55, 3.17) respectively. Following therapy with intravenous l-arginine, sodium phenylacetate and sodium benzoate the hyperammonemia and acute liver failure resolved. Transaminases normalized and remained normal for several years. From 11-12 years of age, persistent elevation of serum transaminases (100-300 range) was noted. Ammonia was persistently normal and she was adherent to therapy. She was neurologically normal and attending Grade 5 but showing difficulties with reading and math. No stigmata of chronic liver disease. She was overweight, BMI elevated at 27.9 (>97%ile). HOMA-IR was calculated to be 1.29. INR, IgG, IgA, ANA, anti-LKM1 Ab, TTG, CPK, ceruloplasmin, ferritin, transferrin saturation were normal. Hepatotrophic viral serologies did not reveal infection. A1AT was reduced (0.56 g/l), PiSZ. Liver u/s demonstrated increased echogenicity. Liver histology showed ballooning of hepatocytes, scattered lytic necrosis, moderate micro and macrovesicular steatosis, minimal lobular inflammation, no cholestasis and no fibrosis. PAS positive, diastase resistant globules were present. A1AT immunostain was positive. Electron microscopy showed mega mitochondria with crystalline inclusions and mildly dilated endoplasmic reticulum. DISCUSSION: Our patient with CTLN1 demonstrated hepatic involvement on several occasions. 1) On two occasions, acute liver failure occurred in association with metabolic decompensation of the CTLN1. The acute liver failure resolved following implementation of specific therapy for the underlying urea cycle defect. Liver transplantation was avoided. This presentation was described by Faghfoury et al (2011) who reported on acute liver failure in 2 cases of acute metabolic decompensation in CTLN1. The etiology for the acute hepatic dysfunction is unknown, but may relate to the accumulation of hepatotoxic urea cycle intermediates. Importantly the acute liver failure can resolve with appropriate therapy, avoiding liver transplantation. 2) Chronic elevation in liver transaminases is not typical in CTLN1. Our patient has a high BMI placing her at risk of NAFLD. Steatosis was present on liver histology, which may be secondary to NAFLD and/or CTLN1. To our knowledge, this is the first report of concurrent A1AT deficiency PiSZ and CTLN1. The A1AT deficiency may have contributed to the elevated transaminases which became apparent later in childhood. However, it is notable that prior to 11 years of age transaminase levels were normal; whereas in A1AT deficiency transaminase levels are typically elevated in early to mid childhood, often normalizing as the child becomes older. 3) Previously described liver histology in CTLN1 includes enlarged mitochondria with swollen cristae and dense matrix, similar to our patient.

41 A pediatric case series of mauriac syndrome: not yet an obsolete entity.. K. Eng, S. Baskar, K. Radhakrishnan, M. Kay, Department of Pediatric Gastroenterology and Hepatology, Cleveland Clinic Foundation, Cleveland, Ohio, UNITED STATES. Schweiger, Center for Pediatric Endocrinology, Cleveland Clinic Foundation, Cleveland, Ohio, UNITED STATES. Liu, Pathology and Laboratory Medicine Insitute, Cleveland Clinic Foundation, Cleveland, Ohio, UNITED STATES. Introduction: Mauriac syndrome is thought to be a rare condition associated with poorly controlled type I diabetes mellitus (DM). It is characterized by growth failure, hepatomegaly, as well as Cushingnoid features. Here we describe two cases of Mauriac syndrome in our pediatric population. Case #1: A 10 year old female with history of poorly controlled type I DM diagnosed at 4 years of age and a history of celiac disease was seen for routine endocrinology follow up. She was noted to have growth failure, a protuberant abdomen, and hepatomegaly on exam. Laboratory revealed a transaminitis with an AST of 763 and an ALT of 693. Further extensive laboratory work-up, including celiac titers, were negative at the time. An abdominal ultrasound (US) showed a small area of focal fatty infiltration in the liver, but was otherwise normal. A liver biopsy was ultimately performed which showed hepatic glycogenosis. Given her clinical and pathologic findings, a diagnosis of Mauriac syndrome was made. With counseling on the condition and improved adherence to her insulin regimen, her hepatomegaly improved and transaminases normalized by 1.5 years from her initial diagnosis. In addition, her growth parameters improved. Case #2: A 16 year old female with history of poorly controlled type I DM and multiple prior hospital admissions for DKA presented to the ER for evaluation of a 2 day history of abdominal pain. Physical examination revealed Cushingnoid features, growth failure, hepatomegaly, and epigastric abdominal tenderness. Her initial laboratory work-up was significant for an AST of 515 and an ALT of 146. An abdominal US showed a coarsened liver echotexture, but otherwise normal. She was admitted for further management, whereupon labs revealed a further increase of her AST to 2934 and ALT to 703 (peaking on hospital day #2), but subsequently improved. Further extensive testing, including testing for viral hepatitis, drug screen, celiac antibodies, ceruloplasmin, CK, autoimmune markers, and iron studies, to evaluate the etiology of her transaminitis was not elucidative. She therefore underwent a liver biopsy which showed diffuse hepatic glycogenosis along with centrilobular fibrosis. Her clinical and histologic features lead to a diagnosis of Mauriac syndrome, however, there was a question of a possible viral insult that may have compounded her significant transaminitis. Her transaminitis improved during her hospitalization, and the family was counseled on the importance of her insulin regimen and improved insulin control was achieved. One month after discharge her transaminitis had normalized, however she has failed to follow up in our clinic. Discussion: Mauriac syndrome was first described in 1930 and was more frequently reported during a time when only regular insulin was available. With the advent of longer acting insulin and improved glycemic control, Mauriac syndrome has become significantly less frequent. Mauriac syndrome is a preventable condition that can still occur due to poor DM compliance, and its recognition and awareness by both pediatric endocrinologists and gastroenterologists is essential to ensure these patients receive appropriate treatment.

42 Hepatic arteriovenolymphatic malformation masquerading as severe pulmonary hypertension and high output cardiac failure.. S. Fernando, C. Mack, F. Karrer, D. Dovel, S. Sundaram, Digestive Health Institute, Children's Hospital Colorado, University of Colorado Denver School of Medicine, Aurora, Colorado, UNITED STATES. Hepatic arteriovenous malformations (AVM) typically present with hepatomegaly, anemia, and congestive heart failure. Case Description: A full-term male infant with apnea and hypotonia at delivery was noted to be hypoxic with a pronounced heart murmur, soft hepatomegaly 4 cm below the right costal margin, and an audible hepatic bruit. Chest x-ray showed cardiomegaly and an echocardiogram revealed right ventricular hypertrophy and supra- systemic pulmonary artery pressures. He was coagulopathic (INR 1.65), anemic (hematocrit 33.6%), thrombocytopenic (platelets 97,000/uL), cholestatic (direct bilirubin 5.7 mg/dL), and hypoalbuminemic (albumin 2.2 g/dL). Abdominal MRI showed a large hepatic vascular malformation, most consistent with a rapidly involuting congenital hemangioma. Both intravenous steroids and three subsequent trans-arterial embolizations failed to provide clinical improvement. His clinical course deteriorated, requiring mechanical ventilation, inhaled nitric oxide, and cardiac inotropes. He then developed acute liver failure (INR 3.78; grade II hepatic encephalopathy). He received a reduced left lateral segment liver transplant at 2 months of age. His intra-operative transplant course was complicated by hemodynamic instability due to massive intra-operative blood loss (500 cc/kg) from a multitude of hepatic feeder vessels. He underwent secondary abdominal wall closure on post-operative day 3, with normalization of liver function. He subsequently developed chylous ascites and pleural effusions, with dilated mediastinal lymphatics on MRI. His explant revealed large embolized arteries and dilated, thin walled veins and lymphatic structures, consistent with an arteriovenolymphatic malformation. He suffered from ongoing pulmonary hypertension and developed significant biventricular hypertrophic cardiomyopathy with myocardial fibrosis on cardiac MRI. He was converted from tacrolimus to cyclosporine to facilitate cardiac remodeling, but due to lack of improvement tacrolimus was restarted. With judicious fluid management and continued supportive care, he was successfully extubated 10 weeks post-transplant and discharged home at 6 months of age. He is now doing well with normal graft function, stable cardiac function, and good catch-up growth and development. This case illustrates a unique presentation of a hepatic AVM. Despite undergoing successful liver transplantation, our patient’s persistent, severe cardiopulmonary disease resulted in a prolonged and complicated hospital course.

43 Autoimmune Hepatitis with recurrent Henoch-Schonlein Purpura: A Case Report. A. Ghazi-Askar, J. Grunow, University of Oklahoma Health Sciences Center, Oklahoma, Oklahoma, UNITED STATES. Introduction Autoimmune hepatitis (AIH) is an immune mediated chronic inflammatory disease characterized by elevated liver enzymes, increase in serum immunoglobulin G (IgG) and elevation of certain antibodies with histologic features of interface hepatitis on liver biopsy. Mostly found in the adolescent and adult population, AIH treatment is aimed at limiting the inflammatory response. In contrast to the rare pediatric onset of AIH, Henoch Schonlein purpura (HSP) is the most common vasculitis of childhood and is characterized by pediatric age of onset, palpable purpura, and variable bowel angina. Largely treated with supportive therapy, steroids are reserved for severe or recurrent cases. Though both are thought to be autoimmune processes, we are unaware of any reported cases of concurrent AIH and HSP. As such, we report a case of a child with recurrent HSP and persistently elevated liver enzymes, found to have autoimmune hepatitis. Case Presentation Nine year old female presented to ED with bilateral LE purpura, right knee and ankle swelling, consistent with HSP. Labs were significant for total bilirubin of 2.4 mg/dL, direct of 1.1 mg/dL, AST:412 units/L, ALT 528 units/L. PMH was significant for 3 similar prior episodes over 10 weeks, managed by rheumatology as HSP on an outpatient basis. The first 3 episodes were treated with steroids and NSAIDs. Patient was followed by rheumatology and was treated with steroids on 2 more occasions for recurrent HSP episodes over the next 5 months and eventually placed on daily colchicine. On colcichine, patient had no further episodes of purpura; however, during her months of treatment her ALT never normalized. After 11 months on colchicine, patient presented to the ED with a 2 week history of jaundice, fatigue and epistaxis. Labs significant for bilirubin 5.2 mg/dL, direct bilirubin 2.7 mg/dL, AST:1159 units/L, ALT:439 units/L, Albumin: 2.2 grams/dL, INR: 1.6. Work up for infectious, infiltrative, metabolic, and autoimmune causes of hepatitis revealed positive anti-smooth muscle antibody and an elevated serum IgG level of 4816 mg/dL. Autoimmune hepatitis was confirmed with interface hepatitis on liver biopsy. Conclusion AIH and HSP are both autoimmune diseases that are responsive to immunosuppression. As such, while treating one, signs and symptoms of the other may be masked. Our case illustrates the need to work up persistent liver enzymes elevation even when the primary disease is in remission

44 Successful Treatment of Hepatitis B Associated Nephropathy with Entecavir in a Child. S. Guimont, Pediatrics, Children's Medical Center, Dallas, Texas, UNITED STATESA. Quan, Medical City Children's Hospital, Dallas, Texas, UNITED STATESS. Cope-Yokoyama, M. Sathe, University of Texas Southwestern Medical Center, Dallas, Texas, UNITED STATES. Chronic Hepatitis B virus (HBV) infection affects 350 million people worldwide. There is pronounced morbidity particularly in the pediatric population because the risk of developing chronic infection and subsequent cirrhosis is inversely related to age of disease acquisition. In addition to end stage liver disease and hepatocellular carcinoma, nephropathy associated with chronic HBV infection has been well described in the literature. There is however, a paucity of established treatment guidelines for the pediatric population. Membranous nephropathy is the most common lesion associated with HBV associated nephrotic syndrome and is thought to be secondary to the deposition of immune complexes including the presence of HBeAg. While spontaneous regression of nephrotic syndrome has been reported, it has been in associated with seroconversion to anti-HBeAg. Accelerated clearance of the virus through the use of pharmacologic agents is thought to reduce the duration of proteinuria and improve the rate of disease morbidity. Currently, interferon (IFN) and nucleoside analogue Lamivudine are approved for treatment of chronic hepatitis in children. INF has significant clinical side effects including bone marrow suppression and Lamivudine has the potential to induce resistance. Newer nucleoside analogs such as Entecavir (ETV) have been approved in the adult population and studies have shown superior viral load suppression, normalization of transaminases and histologic endpoints with a safety profile comparable to Lamivudine. We present the case of a 10 year old Chinese female adoptee with chronic hepatitis B and nephrotic syndrome successfully treated with Entecavir for 48 weeks. The patient achieved decreased viral load, normalization of liver transaminases, resolution of hypoalbuminemia and improved level of proteinuria without significant side effects noted. Although not approved for use in young children, ETV may provide a viable treatment option for this disease entity.

45 Multiple liver lesions in an immunosuppressed patient: Is infection always the answer?. N. Hamouda, Department of Pediatric and Adolescent Medicine, Mayo Clinic, Rochester, Minnesota, UNITED STATESR.M. Grothe, S. Ibrahim, Division of Pediatric Gastroenterology and Hepatology, Mayo Clinic, Rochester, Minnesota, UNITED STATES. Pylephlebitis and fatty liver disease are both rare but known complications of inflammatory bowel disease, often associated with immunosuppressive therapy. The purpose of this case report is to relate an unusual case of focal fatty liver disease in a patient with Crohn’s disease and the diagnostic dilemma it presents. We describe the case of a 17-year-old female with Crohn’s disease who presented with multiple liver lesions, thought to be secondary to pylephlebitis. Further imaging confirmed the diagnosis of focal fatty liver disease. The atypical pattern of focal fatty infiltration in our patient has not been previously described and should be included in the differential for multiple liver lesions in a patient with inflammatory bowel disease.

46 Successful Treatment of Neonatal Hemochromatosis as Gestational Alloimmune Liver Disease with Intravenous Immunoglobulin. C. Jimenez-Rivera, M. Boland, Division of Gastroenterology, Hepatology and Nutrition, Children's Hospital of Eastern Ontario, Ottawa, Ontario, CANADAJ. Feberova, Division of Neonatology, Children's Hospital of Eastern Ontario, Ottawa, Ontario, CANADAJ. de Nanassy, Department of Pahtology, Children's Hospital of Eastern Ontario, Ottawa, Ontario, CANADA. Background: Neonatal hemochromatosis (NH) is a rare, often fatal disorder characterized by liver failure and hepatic and extra hepatic iron overload. Clinical manifestations can occur in utero or immediately after birth. Current standard therapy includes liver transplantation. With gestational alloimmune liver disease (GALD) being the mechanism of liver injury in most cases of NH, a new paradigm of treatment with intravenous immunoglobulin (IVIG) and exchange transfusion has been proposed. We describe a case of NH successfully treated with three doses of IVIG. Case: A male baby was born at 36+6 weeks gestational age to a 44 year old healthy mother G3 T2. Antenatal ultrasound at week 20 was normal. He was born by emergency caesarean section due to pre-eclampsia and fetal distress. He had hydrops fetalis and required resuscitation including endotracheal intubation and chest compressions. Acute care included high frequency oscillation, inotropics, and fluid support with glucose and electrolytes. Bilateral pleural effusions were managed with chest tube insertion. Severe ascites required peritoneal drainage and albumin infusions. Initial blood work revealed elevated transaminases with marked synthetic dysfunction: ALT 653 IU/L, AST 1996 IU/L, albumin 21 g/L (2.1 g/dL), INR 4.9. Ferritin was >10,500 μg/L (Normal range 6.5-196 μg/L). Investigations for infectious and metabolic disorders were negative. Abdominal ultrasound demonstrated coarse heterogeneous hepatic parenchyma. The presumed diagnosis was NH, but the baby was too ill for liver transplant. He was treated with three doses of IVIG 1g/kg (of estimated dry weight) at 3, 6 and 17 days of life. Ferritin decreased to 308 μg/L and his liver function and overall condition improved one week after the first dose of IVIG. Transaminases and INR normalized 2 weeks after the first dose. Buccal mucosal biopsy (minor salivary glands) at 11 days of age did not reveal iron deposits. Abdominal MRI at 37 days of age failed to show iron deposits in the liver or other organs. Percutaneous liver biopsy at 6 weeks of life revealed extensive fibrosis and slight stainable iron in hepatocytes. The infant was discharged home at 3 months of life in stable condition with G-tube feeds due to poor oral intake. Discussion: This case exemplifies successful therapy with IVIG in a neonate with severe hydrops and multiorgan failure in the presence of elevated serum ferritin highly suspicious for NH. The infant was too ill for liver transplant. Intensive care and supportive therapy played a crucial role in treatment, however IVIG appeared to halt the inflammatory cascade developed by an intrauterine alloimmune process and prevented death.

47 Eosinophilic gastrointestinal disease with extremely high IgE levels in a 2 year old male after orthotopic liver transplant. M. Kabbany, V. Hupertz, K. Radhakrishnan, N. Alkhouri, Department of Pediatric Gastroenterology, Cleveland Clinic Foundation, Cleveland, Ohio, UNITED STATES. Eosinophilic gastrointestinal disease (EGID) and De novo food sensitization has been reported in many pediatric liver transplant patients. The common driving force behind IgE-mediated EGID enhanced Th2 response to food allergens. Our patient is a 2-year-old male who is status post living-related liver transplantation for biliary atresia at the age of 7 months. 18 months after transplant patient noted to have mild eosinophilia [absolute eosinophilic count (AEC) 1.41] which progressed within 3 months to moderate eosinophilia (AEC of 3.39) with extremely high IgE levels (32,843). Patient was on Tacrolimus 0.75 mg/kg/dose BID with serum level around 4 - 6. Infectious causes were investigated including stool parasites and strongyloides serum IgG. Patient underwent bronchoscopy with negative BAL fungal culture. CT sinuses didn’t show any fungal ball and Aspergillus ab were negative. A serum triptase to rule out mastocytosis and eosinophilic leukemia mutation were also negative. Echocardiogram was negative for any cardiomyopathy caused by hypereosinophilic response. EGD/Colonoscopy showed eosinophilic enterocolitis that involved the esophagus, stomach and the colon. Patient developed an allergic reaction to peanuts 20 months post transplant (mainly facial swelling). RAST testing showed high IgE titers for peanuts but surprisingly high titers for egg white, milk, wheat, corn, soybean and tuna which he tolerated well. Prednisone was started at 0.5 mg/kg daily for 4 months. Peanut elimination diet was started. IgE and eosinophilia were followed monthly. Their values improved but didn’t completely return to normal. At 3 years of age (~ 2.5 years after transplant), patient developed decreased appetite, intermittent non bloody vomiting. CBC showed moderate eosinophilia (AEC 2.5) with strikingly elevated IgE levels 131,437. EGD/Colonoscopy showed eosinophilic enterocolitis that involved the stomach and the right colon. Skin testing was positive for egg white and cabbage. Patient also showed enhanced Th2 response with elevated IL-4. He was started on steroids with egg elimination from the diet which resulted in improvement of symptoms and improvement of eosinophilia and IgE levels. He was subsequently weaned off of steroids and his Tacrolimus levels allowed to drop to insignificant levels (<2) due to feeling that the calcineurin inhibitor was driving the enhanced Th2 response. With this low level of tacrolimus, he has not had any episodes of rejection and is thus displaying operational tolerance. Most recently eosinophil count has been normal. IgE levels continue to be in the 1000-2000 range. In conclusion, this case highlights the effect of immunosuppression post liver transplant on the natural Th1/Th2 balance. Calcineurin inhibitor is a potent inhibitor of IL-2 promoter induction implicated in skewing the immune system toward a Th2-mediated response which resulted in Th2 enhanced response. It manifested as severe eosinophilia and extremely high IgE levels with development of multiple new food allergies. As Tacrolimus was weaned further, the eosinophilia and IgE levels subsequently improved.

48 Biliary Rhabdomyosarcoma presenting as Obstructive Jaundice: Usual Presentation of an Unusual Tumor. S. Kaur, L.M. Bass, Pediatric Gastroenterology and Hepatology, Lurie Children's Hospital of Chicago, Chicago , Illinois, UNITED STATESS. Shah , N. Navanandan, Pediatrics, Lurie Children's Hospital of Chicago, Chicago , Illinois, UNITED STATESS.R. Daram, J. Martin , Gastroenterology and Hepatology, Northwestern Memorial Hospital , Chicago, Illinois, UNITED STATESA. Popescu, Radiology, Lurie Children's Hospital of Chicago, Chicago , Illinois, UNITED STATESS. Kaur, S. Shah , S.R. Daram, J. Martin , N. Navanandan, A. Popescu, L.M. Bass, Northwestern University Feinberg School of Medicine, Chicago, Illinois, UNITED STATES. Introduction: Embryonal rhabdomyosarcoma of the biliary tree accounts for about 0.04% of all childhood neoplasms. It usually presents as obstructive jaundice with a median age of presentation of 3 years. Other diagnostic considerations include choledochal cyst, inflammatory pseudotumor and hepatoblastoma. We report the case of a 3-year-old boy who presented with obstructive jaundice. Case Report: A 3-year-old boy presented with the complaint of dark urine and pale stools for 3 days and painless jaundice for 1 day. Physical exam was significant for hepatomegaly, without any stigmata of chronic liver disease. Initial labs were remarkable for: ALT of 649 IU/L, AST of 559 IU/L, Alkaline Phosphatase of 1626 IU/L, total bilirubin of 10.9 mg/dL with a direct component of 5 mg/dL. Ultrasound demonstrated intrahepatic and extrahepatic biliary ductal dilation with some areas appearing saccular in configuration suggesting either a choledochal cyst or other obstructive etiology. A lipid panel was remarkable for total cholesterol of 746 mg/dL, LDL cholesterol of 717 mg/dL, and HDL cholesterol of 9. Alpha- fetoprotein was reported to be within normal limits. Lipoprotein X was reported to be the major component of cholesterol in the lipoprotein profile. An MRCP demonstrated diffuse nodular thickening of the extrahepatic bile duct, marked fusiform dilatation of the cystic duct with adjacent enhancing soft tissue abnormality and nodular enhancement within the CBD. These findings were suggestive of an atypical biliary ductal neoplasm. Cholangiogram at ERCP demonstrated marked CBD dilation with appearance of multiple filling defects. On biliary sphincterotomy, a soft tissue mass prolapsed into the duodenal lumen from the CBD. This mass was biopsied and a transpapillary temporary biliary stent was placed. The biopsy results demonstrated an embryonal rhabdomyosarcoma, botyroid type, testing positive for desmin and myogenin. His transaminases and bilirubin trended down post-stent placement. A CT scan and bone scintigraphy, performed to assess extent of disease, demonstrated no clear metastases. A planned pancreaticoduodenectomy was aborted intra-operatively due to concerns that resection would leave positive margins as the tumor was found to extend up to the confluence of the right and left hepatic ducts,. His disease was classified as Stage 1 Group III embryonal rhabdomyosarcoma of the biliary tree (low-risk), and he is now on the ARST0531 chemotherapy protocol. Conclusion: Elevated cholesterol and lipoprotein X are known to be associated with cholestatic liver disease. In the current case, cholestasis caused by the obstructing biliary tumor accounts for this metabolic derangement. Embryonal Rhabdomyosarcoma is perhaps the most common biliary tumor in this age group. Radiation and chemotherapy may be required prior to surgical consideration to decrease tumor burden.

49 A Serious Complication of Hepatitis A Virus in a Young Male: Case Report. M. Khatib, H. Hashmi, C. Nugent, R.D. Baker, S.S. Baker, Department of Pediatric Gastroenterology and Nutrition, University at Buffalo, New York, Women and Children’s Hospital, Buffalo, New York, UNITED STATES. A 10 year old male with no significant past medical history, recently immigrated from Nepal presented with a three week history of fever, abdominal pain and pruritis. He was treated with acetaminophen and diphenhydramine for presumed viral syndrome. His symptoms worsened and he developed jaundice. On examination he appeared ill, jaundiced and had right upper quadrant tenderness with a positive Murphy's sign. Table 1 shows the laboratory profile. The serum acetaminophen level was less than 3 mcg/mL, and infection with Cytomegalovirus, Epstein Bar virus, hepatitis B, C and HIV were excluded with serology. Serum Ceruloplasmin was slightly elevated at 40.1 mg/dL. A celiac panel was negative. Blood and urine cultures were negative and stool culture showed no pathogens. HAV IgM antibody test was positive. An abdominal ultrasound showed a thickened gall bladder wall surrounded by edema, measuring 5 mm in thickness. No duct dilatation or other abnormalities were seen. The findings were reported as consistent with Acute Acalculous Cholecystitis (AAC). He was offered supportive treatment and both his hepatitis A and AAC resolved. Discussion: AAC can be a coincidental finding in hepatitis A. AAC is a severe inflammatory disease of the gallbladder in the absence of cholelithiasis that can complicate medical or surgical conditions. AAC accounts for approximately 10% of cases of acute cholecystitis and is most frequently seen in association with severe illness. It is associated with complications such as perforation, gallbladder necrosis and mortality. AAC is associated with a high mortality (most studies, 30%; range 10%–90%). that depends how ill the patient is. AAC has a higher incidence of gangrene and perforation compared to calculous disease. The most likely cause of AAC in our patient is invasion of the gallbladder wall and the biliary tree by the HAV and cell-mediated immune response. Conclusion: Our case is unusual in that hepatitis A infection is a benign disease, yet, a potentially serious complication occurred. Fortunately, complete resolution occurred with supportive care. It is necessary to keep in mind the complications of hepatitis A infection to provide care when clinical status changes. Table1: Blood work comparison between time of diagnosis, after 2 weeks and 5 months later.

Intestinal/Colonic Disorders – Non-IBD 70 Evolution of IBD Serologies Over Time in 3 Pediatric Patients. R. Abell, J. Morganstern, Pediatric Gastroenterology, Stony Brook Long Island Children's Hospital, Stony Brook, New York, UNITED STATES. IBD serologic testing,including pANCA and ASCA is known to be helpful in diagnosing IBD,differentiating Crohn’s disease(CD)from Ulcerative Colitis(UC),and predicting which patients may require surgical intervention.There are few studies examining the use of serial IBD serologies to evaluate disease status or progression,especially in the pediatric population.We present 3 patients who had a significant change in IBD serologic markers over time.Case 1:7 year old male diagnosed with UC with a refractory disease course resulting in total colectomy.At diagnosis,he had a pANCA level of 23.4 EU/mL and absent immunofluorescence(IFA)perinuclear pattern.Other markers were negative.Repeat testing 9 years later demonstrated a pANCA of 33.1 EU/mL and was still IFA negative.A third set 7 months later revealed a pANCA of 32.4 EU/mL,and an IFA perinuclear pattern detected.Case 2: 9 year old male presented with hematochezia and was diagnosed with UC.He was treated successfully with 5-ASAs.Initial serologic testing 3 years after diagnosis revealed a pANCA of 32.8 EU/mL,absent IFA pattern and DNAse sensitivity not detected.Other markers were negative.Three years later the testing was repeated and pANCA increased significantly to 55.7 EU/mL,IFA perinuclear pattern was now detected and was found to be DNAse sensitive.Case 3:14 year old female was diagnosed with CD after presenting with abdominal pain.She was treated with mesalamine and subsequently 6-MP with good response.Her initial IBD serology at diagnosis showed ASCA IgA of 48.3 EU/mL.Other markers were negative.Repeat labs 9 years later demonstrated a markedly decreased ASCA IgA of 4.6 EU/mL with all other markers negative.These cases suggest that IBD serologic testing may not be constant over time. It is well established that serial measurements of ANCA are useful for follow-up of disease activity in adult patients with systemic vasculitidies.However,the data is less clear in IBD, particularly in pediatrics.There is inconsistent data as to whether IBD serologic markers change over time or with therapy.The current standard of practice is to check serologic markers only once at diagnosis,however serial IBD serologies may aid in optimizing patient care over time.

71 Single-Center Review of Wireless Capsule Endoscopy in Children. A. Aktay, Pediatric Gastroenterology, Rainbow Babies Children's Hospital, Cleveland, Ohio, UNITED STATESH.W. Hamandi, Pediatric, Rainbow Babies Children's Hospital, Cleveland, Ohio, UNITED STATES. Objective: The data on wireless capsule endoscopy (WCE) in children is limited. The aim of this study is to describe the demographics, indications, findings, and complications of this novel non-invasive diagnostic tool in a pediatric population. Methods: The study is an IRB approved retrospective chart review of patients <18 years of age who underwent WCE at a tertiary children’s hospital over a seven-year period. Results: 98 WCE procedures were reviewed; 7 patients were excluded due to reasons outlined below. Mean age was 14 years old; youngest patient was 4 years old; 15 patients were <10 years old; 55% were female. Prior to undergoing WCE, the most commonly reported symptoms were abdominal pain (52%), diarrhea (13%), hematochezia and melena (9%), vomiting (8%), and weight loss (5%). The indications for WCE were evaluation of suspected small bowel Crohn’s disease (62%), surveillance of established Crohn’s disease (19%), surveillance of indeterminate colitis (11%), evaluation of gastrointestinal bleeding (6%), and evaluation of suspected polyposis (2%). Overall, 29 of 91 patients (31%) had abnormal findings. The primary abnormal findings visualized on WCE were aphthous ulcers and erosions in the small bowel; these findings were evident in 25 patients (86% of all abnormal findings). Active gastrointestinal bleeding was only observed in 2 patients. One patient demonstrated findings consistent with NSAID injury, and another patient had worms in the small bowel in the context of an unremarkable travel history. Abnormal findings per indication were as follows: 14 of 56 patients with suspected small bowel Crohn’s disease had ulcers and erosions in the small bowel; 10 of 17 patients with established Crohn’s disease were found to have small bowel ulcerations; 2 of 10 patients with indeterminate colitis were diagnosed with small bowel Crohn's disease; 2 of 6 patients with gastrointestinal bleeding were found to have active bleeding in the small bowel; and none of the patients with suspected polyposis were found to have polypoid lesions. Mean small bowel transit time of capsule was 236 minutes, excluding retained capsules. The complication rate was 7%: 3 capsules showed poor visibility due to food particles, 3 capsules were retained in the stomach, and 1 failed to reach the cecum. In patients <10 years old, 40% of capsules were placed endoscopically in the duodenum without any complication. None of these capsules were retained. Body mass index was not associated with the method of placement in this subset of patients. Conclusion: The experience of WCE in children is significantly different in comparison to adults. In particular, it appears that pediatric indications are more focused on IBD versus gastrointestinal bleeding and polyps that are more consistent with adult pathology; thus, this is also reflected in the findings. As an indication, evaluating the surveillance of established Crohn’s disease demonstrated the greatest ratio of abnormal findings; although, it is yet to be determined if abnormal findings in this group of patients would result in a change in management. The complication rate is comparable to other published studies in children.

72 A Rare Presentation of Orofacial Crohn’s Disease. J. Bass, Gastroenterology, Children's Mercy Hospital, Kansas City, Missouri, UNITED STATES. A 14 year old previously healthy female presented with a three month history of significant mouth sores, swollen cheeks, and swollen lips. Trials of antibiotics, anti-fungals, and topical anesthetics by the primary physician had resulted in no improvement and possible worsening of symptoms. Otolaryngology, infectious disease, and rheumatologic evaluations were unrevealing. No anemia or inflammation was noted on laboratory evaluation. Bacterial, fungal, and viral cultures of the mouth lesions were negative. A five day course of oral corticosteroids had resulted in brief improvement in symptoms, which prompted a gastroenterology referral for evaluation of possible Crohn’s disease. No obvious gastrointestinal symptoms were noted. The patient reported daily bowel movements without blood. Growth parameters placed her at the 50th percentile. On examination, gingival edema and “knife-cut” lesions were noted in the buccal mucosa bilaterally. The lower lip and bilateral cheeks were notably swollen. Lip swelling progressed to the upper lip during the month following presentation. Upper and lower endoscopy revealed ulcerations in the duodenum and terminal ileum. The esophagus, stomach, and colon appeared normal. Biopsies from the upper endoscopy revealed chronic gastritis only. Mild focal active inflammation was noted in the terminal ileum and left colon. Right colon biopsies revealed chronic colitis with granulomas, which solidified the diagnosis of Crohn’s disease. An oral biopsy was not performed. MRE revealed bowel wall thickening in the terminal ileum and possibly jejunum, consistent with Crohn’s disease diagnosis. The patient was placed on steroids and mesalamine following endoscopy. Due to significant headaches, the mesalamine was discontinued. Oral corticosteroids did not seem to provide much benefit. Intra-lesional corticosteroid injections, performed on three occasions by a plastic surgeon, resulted in mild temporary improvement but not complete resolution of symptoms. Infliximab therapy was initiated. The patient completed the infliximab induction series and is currently on maintenance therapy. She has complete resolution of the mouth sores but does have mild residual lip and cheek swelling. Exome sequencing is in process. This case reveals a rare presentation of orofacial Crohn’s disease without obvious gastrointestinal symptoms. The response to oral and intra-lesional corticosteroids was suboptimal and relapse occurred with steroid withdrawal. There is paucity of data available regarding best treatment strategies for this rare group of patients. This case supports the consideration of infliximab as initial therapy in orofacial Crohn’s disease.

73 Complete Rectal Occlusion from Crohn’s Stricture in Very Early Onset IBD Awaiting HSCT. J.C. Burgis, K.T. Park, Pediatric GI, Stanford University, Palo Alto, California, UNITED STATESM. Bruzoni, Pediatric Surgery, Stanford University, Palo Alto , California, UNITED STATESK. Weinberg, Pediatric Hematology-Oncology, Stanford University, Palo Alto , California, UNITED STATES. 4 year old female with a strong family history of IBD initially presented with bloody stools at 4 months. Her symptom course included growth failure, oral and perineal ulcerations, skin tags, erythema nodosum and arthritis. Multiple endoscopies have shown pancolitis without terminal ileal involvement and duodenal ulcerations. Small bowel imaging is normal. Initial management included IV and PO steroids, infliximab 5mg/kg quickly escalating to 10mg/kg Q4-6 wks with adjunct methotrexate SQ weekly. After developing HACA antibodies in nine months, she started adalimumab and methotrexate SQ. Elemental gastrostomy tube feedings and TPN were concomitantly used. After exhausting anti-TNF regimens and dose escalations, tacrolimus was started with an induction goal of 10-15ng/mL and maintenance goal 5-10ng/mL. Her management has been complicated by family non-adherence and poor geographical access to healthcare. Other workup included normal QUIGS and IgGs, negative HIV, negative FOXP3, and whole exon sequencing without gene dysfunction in either IL10 or IL10 receptor. While on tacrolimus, endoscopy showed an esophageal stricture at 10cm, chronic active pancolitis with granulomas confirming Crohn’s colitis, normal terminal ileum, rectal stricture at 2-3cm form the anal verge, and a small easily visualized recto-vaginal fistula at the hymenal inlet. Plan was set to proceed with hematopoetic stem cell transplant (HSCT).[1] Three weeks prior to HSCT, while on Vancomycin and Gentamicin for gut decontamination, worsening intestinal ileus on KUB prompted a barium enema showing high grade rectal stricture. Solumedrol IV for 48 hours did not improve the stricture, and attempts at endoscopy and passage of an 8 Fr rectal tube under anesthesia were unsuccessful. In the OR under fluoroscopy, a 0.035 wire was passed and the anal canal was dilated to 20Fr using a Seldinger technique then up to 16 mm Hegar dilator. Rectal involvement is associated with poorer prognosis and higher risk for surgery in Crohn’s disease.[2] Medical options include steroid or TNF injection in addition to Hegar and balloon dilation.[3,4] Stricturoplasty and resection are surgical options but recurrence is a challenge.[5] 1. Burt, R. K. et al. Autologous nonmyeloablative hematopoietic stem cell transplantation in patients with severe anti-TNF refractory Crohn disease: long-term follow-up. Blood 116, 6123–6132 (2010). 2. Cosnes, J. et al. Factors affecting outcomes in Crohn’s disease over 15 years. Gut 61, 1140–1145 (2012). 3. Swaminath, A. & Lichtiger, S. Dilation of colonic strictures by intralesional injection of infliximab in patients with Crohn’s colitis. Inflamm. Bowel Dis. 14, 213–216 (2008). 4. Singh, V. V., Draganov, P. & Valentine, J. Efficacy and safety of endoscopic balloon dilation of symptomatic upper and lower gastrointestinal Crohn’s disease strictures. J. Clin. Gastroenterol. 39, 284–290 (2005). 5. Romeo, E. et al. Strictureplasty and intestinal resection: different options in complicated pediatric-onset Crohn disease. J. Pediatr. Surg. 47, 944–948 (2012).

74 Crohn’s disease presenting as tubo-ovarian abscess in a virginal adolescent female. G. Duh, Pediatrics, Southern California Permanente Medical Group, Downey, California, UNITED STATES. Case: An obese 15 year old virginal female with a prior history of appendectomy more than two years previously presented with a one-week history of fever, lower abdominal pain and dysuria, which did not improve with oral antibiotics for treatment of presumed urinary tract infection. Initial laboratory findings included a complete blood count that demonstrated leukocytosis (20.8 x 10^3 leukocytes per microliter of blood), an erythrocyte sedimentation rate (ESR) of 111 mm/hr, a C-reactive protein of 116.8 mg/L, and initial transabdominal ultrasound findings included incidental gallstones, mild calciectesis of the left kidney and what appeared to be an irregular, heterogeneous appearing uterus. Subsequent CT and MRI imaging of the abdomen and pelvis indicated the presence of mucosal thickening in the cecum and terminal ileum, as well as complex cystic masses bilaterally in the adnexa (6.4 x 4.7 x 5.5 cm on the left, 4.3 x 4.7 x 5.7 cm on the right), and in the presacral space (4.7 x 5.8 x 6.4 cm), suggesting a diagnosis of tubo-ovarian abscess. The patient’s external genital examination showed an intact hymen, consistent with virginal status. The patient was studied endoscopically for possible Crohn’s disease. Her esophagogastroduodenoscopy was completely normal; however, the colonoscopy showed inflammatory changes in the cecum and sigmoid colon, and a nipple-shaped lesion with extruding purulent drainage was noted in the cecum, indicating a probable fistula to a pelvic abscess. Biopsies of the affected areas revealed non-specific inflammatory changes without granulomata. The Prometheus IBD sgi Diagnostic test (Prometheus Labs, San Diego, CA) showed elevated values for ASCA IgA (12.6 EU/ml [< 8.5 EU/ml]) and anti-OmpC IgA (39.1 EU/ml [< 10.9 EU/ml], but not for pANCA, consistent with a diagnosis of Crohn’s disease. Comments: Tubo-ovarian abscess normally results from an upper genital tract infection, is a serious consequence of pelvic inflammatory disease, and is exceedingly rare in virginal teens. A review of the medical literature resulted in identification of only one other case of tubo-ovarian abscess in a virginal female secondary to Crohn’s disease.

75 Nurse Practitioner/Physician Team Care: an efficent mechanism for delivery of pediatric subspecialty care. E Gleghorn, S Moore, Children's Hospital and Research Center Oakland. S. Moore, E. Gleghorn, Pediatric GI, Children's Hospital and Research Center Oakland, Oakland, California, UNITED STATES. Pediatric subspecialty providers will be in great demand as The Affordable Care Act and similar legislation change the systems of health care delivery. Increased use of midlevel providers is one way to serve more patients within a shrinking budget. We report our experience with midlevel providers in a GI practice in a large urban pediatric hospital. Children’s Hospital and Research Center Oakland is a private not for profit 190 bed pediatric medical center with 10,000 admissions/yr and >180,000 clinic visits/yr. When our GI division was acutely stressed with the loss of half its certified subspecialists, a new framework using nurse practitioners “at the top of their license” allowed the GI Dept to maintain the service needs demanded by the community. This model of care delivery is a partnership between nurse practitioner and GI subspecialist so the patient has a dedicated NP/MD team planning care. The nurse practitioner functions mostly in information gathering, diagnosis, documentation, and teaching. The physician corroborates history and physical findings and participates in explanation of the agreed diagnosis and plan, as well as researching more complex diagnoses and coordinating care with other 'higher level' providers. This model allows the physician to increase the volume of patient flow. Nurse practitioners were also able to see patients independently as allowed by legal and payor regulations. The schedule was tailored to feature clinics in which no MD attendance was required, as well as the collaborative clinics. We will show prior and current models of care and will include productivity metrics and financial data. Mechanisms and magnitude of increased efficiency and cost saving will be illustrated. Tables below show productivity values for comparable times, showing a doubling in volume of the visits each physician was able to supervise. Staffing was changed, with a slightly larger number of Nurse Practitioners (3.4 FTE=>4.1 FTE) to extend the reach of fewer GI subspecialty MDs. Changes in cost of care due to changes in personnel will be illustrated. The new mode of practice was well-accepted by the families, who appreciated greater availability of practitioners familiar with the child’s case. MDs and NPs enjoyed professional satisfaction in the interactions of different methods and focus of each discipline as well as the opportunity to grow expertise. This is a cost-saving and labor-extending method of supplying care to a growing patient volume with ever stricter cost-containing pressures. , VISITS PER MD PER MONTH ,

76 Combined Restitutive Therapy for Treatment of Immunosuppressive Refractory Crohn’s Disease. J.R. Goldsmith, CGIBD, University of North Carolina, Chapel Hill, Durham, North Carolina, UNITED STATESM. Kelly, K. Freeman, D. Duro, Pediatrics, Division of Pediatric Gastroenterology, Hepatology and Nutrition, University of North Carolina, Chapel Hill, Chapel Hill, North Carolina, UNITED STATES. Crohn’s Disease (CD) affects over 600,000 Americans, and is believed to be the result of dys-regulated interactions between a genetically susceptible host and their commensal gut microbiota. While many patients with CD have immune systems that over-react to commensal microbiota, recent human genome screens of patients suggest that a subset of patients instead have defects in bacterial killing that results in persistent bacterial infiltration of the gut wall, leading to sustained inflammation. The existence of this patient subtype could explain why some patients with CD do not respond to traditional immunosuppressive therapies. Recent studies in humans suggest that granulocyte-macrophage colony stimulating factor (GM-CSF) can induce remission in some patients with CD. In mice, recent studies show that activation of enterocyte-specific mu-opioid signaling induces intestinal healing and prevents intestinal cell death. Additionally, a recent clinical trial has demonstrated that low-dose naltrexone can induce mucosal healing in CD patients, likely through potentiation of endogenous opioid signaling. Here, we describe a 17-yr-old female with a 3-yr history of colonic CD who presented with refractory CD, having failed 5- ASAs, 6-MP, and multiple anti-TNF agents, and who was not responding to IV methylprednisolone. The patient’s initial Crohn’s Disease Activity Index (CDAI) was 568, and the patient had ~20 bowel movements per day, with nightly wakings and intermittent hematochezia. After 2 days of failed steroid therapy, the patient was switched to 6 mcg/kg/day s.c of GM-CSF and 2 mg loperamide qhs, along with nightly mesalamine enemas for rectal urgency. The patient rapidly improved, and 8 days later was discharged with a CDAI of 205, and ~7 bowel movements per day, with less frequent bleeding. At discharge, the enema was switched to a suppository formulation. At her one and two month follow-ups, the patient reported maintained remission of her symptoms, with a final CDAI 114. This case provides support for the use of GM-CSF in patients with CD who fail to respond to immunosuppressants. Additionally, it provides for the first time clinical evidence for the use of opioid agonists in patients with CD as a therapy to induce mucosal healing and induce remission.

77 ABNORMAL BONE MARROW FINDINGS ON MRI IN A PEDIATRIC PATIENT WITH NEWLY DIAGNOSED CROHN’S DISEASE.. K. Harlow, General Pediatrics, Indiana University and Riley Hospital for Children, Indianapolis, Indiana, UNITED STATESK. Lazarus, Pediatric Hematology and Oncology, Indiana University and Riley Hospital for Children, Indianapolis, Indiana, UNITED STATESB. Karmazyn, Pediatric Radiology, Indiana University and Riley Hospital for Children, Indianapolis, Indiana, UNITED STATESB. McFerron, E. Contreras, Pediatric Gastroenterology, Indiana University and Riley Hospital for Children, Indianapolis, Indiana, UNITED STATES. Small bowel imaging is commonly done in conjunction with endoscopy at the time of IBD diagnosis. We report a case in which MRI to assess the extent of perianal disease revealed abnormalities in the bone marrow, warranting further evaluation. The patient is a 16 year old male who presented with persistent blood-streaked diarrhea for 6 weeks, progressive fatigue, pallor, 10lb weight loss, and multiple perianal abscesses requiring incision and drainage in the past year. Physical exam revealed inflamed perianal skin tags, and a single draining perianal fistula. Admission labs were notable for significant microcytic anemia requiring transfusion, thrombocytosis, and elevated inflammatory markers. Upper endoscopy revealed diffuse gastric and duodenal erythema and edema with inflammatory nodules in the pre-pyloric antrum. Colonoscopy showed patchy pancolitis and deep ulcerations in the terminal ileum. Surgical pathology confirmed the diagnosis of Crohn’s disease. The patient was then started on antibiotics and corticosteroids, with plans for infliximab therapy. MRI pelvis with fistula protocol was obtained to further evaluate his perianal disease. The hips were also included given the patient’s participation in mixed martial arts and a history of right hip pain, previously diagnosed as a hip flexor sprain. Results of the imaging demonstrated a transsphincteric anal fistula in communication with the perianal surface and a 1cm x 1cm intrasphincteric abscess. In addition, diffusely abnormal bone marrow signal concerning for an infiltrative process was noted in the pelvis, lumbar vertebrae, and shaft of bilateral femurs. Hematology performed a bone marrow aspirate and biopsy which revealed normal cellularity for age and maturing cells in all 3 cell lines, indicative of expansion of marrow secondary to an inflammatory state as opposed to an infiltrative process. This case demonstrates that normal bone marrow reaction to anemia and chronic disease can appear highly abnormal on MRI. With increasing frequency of MRI studies in IBD patients, future studies are needed to determine the prevalence of such findings and to guide when further evaluation is needed.

78 Exome sequencing identifies a novel FOXP3 mutation associated with an atypical presentation of the IPEX syndrome in a two-generational family with inflammatory bowel disease. T. Hofmekler, D. Okou, J. Waters, S. Kugathasan, Pediatrics , Emory University, Atlanta, Georgia, UNITED STATESK. Mondal, J. Mulle, D. Ramachandran, V. Patel , P. Bose, D.J. Cutler, M.E. Zwick, S. Kugathasan, Human Genetics , Emory University, Atlanta, Georgia, UNITED STATESL. Kobrynski, Pediatric Allergy and Immunology, Emory University, Atlanta, Georgia, UNITED STATESP. Sampath, Rheumatology, Emory University, Atlanta, Georgia, UNITED STATES. Introduction: Inflammatory bowel disease (IBD), compromised of Crohn’s disease (CD) and ulcerative colitis (UC), is characterized by inflammation of the intestinal tract. IBD has a wide spectrum of presentation and is mostly common in adults. However, some patients present at childhood and these children have unique phenotypes. Primary immunodeficiencies can also present like early onset IBD and the clinical, endoscopic and pathological features are indistinguishable from chronic IBD. This leads to differential diagnosis where the phenotypes do not point to an evident underlying genetic cause; therefore, single gene sequencing is not adequate. Whole exome sequencing (WES) is not hypothesis driven and has proven useful in assisting clinicians in making the proper diagnosis. Case Presentation: We present four members of a two generation Caucasian family with atypical phenotypes. WES was used to identify a novel missense variant (c.694A>C, pC232G) in the FOXP3 gene. Mutations in FOXP3 typically lead to IPEX (Immunodys-regulation, Polyendocrinopathy, Enteropathy, X-linked) syndrome characterized by enteropathy (IBD-like and celiac disease), dermatitis, and endocrinopathy (most commonly insulin-dependent diabetes mellitus). Research showed that CD4+CD25+T reg cells with expressed FOXP3 protein are important in the homeostasis of the human gut. Disrupted homeostasis of the gut may lead to IBD and IPEX. IBD and IPEX patients with atypical phenotypes may result in clinical overlap, differential diagnosis and variable outcomes. The novel FOXP3 mutation identified impairs the suppressive function of T regulatory cells and leads to a milder form of IPEX or an atypical IBD phenotype. Based on the diagnosis of atypical IPEX, a bone marrow transplant recommendation was made. Conclusion: We have demonstrated the importance of WES in the diagnosis of patients with atypical phenotypes and in whom conventional diagnostic methods were not sufficient. This underlines importance of a multidisciplinary approach in patient care and disease management.

79 Role of Colectomy to Treat Life-Threatening Thrombotic Storm in Pediatric IBD. C. Jump, K.M. Loomes, D. Piccoli, J. Kelsen, Gastroenterology, Hepatology, and Nutrition, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, UNITED STATESS. Anupindi, Radiology, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, UNITED STATEST. Blinman, General, Thoracic, and Fetal Surgery, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, UNITED STATESK. Smith-Whitley, Hematology, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, UNITED STATESJ. Rosh, Gastroenterology and Nutrition, Goryeb Children's Hospital , Morristown, New Jersey, UNITED STATES. Venous thrombosis is a known complication of inflammatory bowel disease (IBD) in both the pediatric and adult populations. Thrombotic storm is a syndrome of venous thromoboembolism characterized by the development of multifocal thromboses of various etiologies which can be triggered by active colitis. Here we present a case of thrombotic storm in a pediatric patient with Ulcerative Colitis (UC). An 8 year old female with UC, previously stable on infliximab monotherapy, presented with six days of bloody diarrhea and abdominal pain. Therapy was initiated with intravenous methylprednisolone with some improvement. On hospital day 5, she developed tachypnea and an oxygen requirement. Due to concern for pulmonary embolism, a chest CTA was obtained which demonstrated multiple pulmonary emboli in all lung lobes with extensive clot burden and concern for a small infarct in her left kidney. The patient was started on a heparin drip. On hospital day 7, Doppler ultrasound of her abdomen showed a non-occlusive thrombus of the middle hepatic vein. The next day, while on therapeutic heparin, she developed right sided weakness prompting brain MRA/MRV. These studies confirmed the diagnosis of superior sagittal sinus thrombosis. Shortly after, she developed hypertension as well as a decreased heart rate and there was concern for evolution of intracranial clots. Due to the concern for thrombotic storm secondary to active UC, the patient was treated with infliximab and a five day course of plasmapheresis. A concomitant laboratory evaluation for hypercoaguable conditions and hereditary thrombophilia was negative. Despite this therapy, follow up abdominal and extremity ultrasound showed the new findings of a non-occlusive thrombus in the left portal vein and a clot formation around her right upper extremity central line. Therefore, she proceeded to sub-total colectomy while receiving therapeutic heparin. Colectomy specimen showed severe active colitis with ulceration, pseudopolyp formation, and superficial fissuring, with a normal terminal ileum and cecum. Post- operative follow up imaging showed no further evolution of clots and resolution of intra-abdominal thrombi. She was transitioned to low molecular weight heparin therapy and discharged. Patients with IBD are at an increased risk of developing venous thromboemboli and stroke during times of high disease activity. Thrombotic storm should be recognized early and aggressively managed to avoid catastrophic outcome. Despite the risk posed by ongoing operative heparin therapy, our patient underwent successful and curative sub-total colectomy which was necessary as the definitive therapy for refractory thrombotic storm in active UC.

Intestinal/Colonic Disorders - IBD 100 Small bowel parasite diagnosed by wireless capsule endoscopy. A. Aktay, Pediatric Gastroenterology, Rainbow Babies Children's Hospital, Cleveland, Ohio, UNITED STATES. Wireless capsule endoscopy is a novel tool to diagnose small bowel Crohn's Disease and upper gastrointestinal bleeding in children. The indications for WCE has expanded further to celiac disease, polyposis syndromes, obstructive lesions and parasitic infections. Parasitic infestation of the small bowel is uncommon cause of anemia and abdominal pain in Midwest of the United States. Hookworm infestation on Wireless Capsule Endoscopy (WCE ) was reported in adult population but rarely in pediatrics. We report a hook worm infestation of small bowel found on WCE in a 15 year-old female with anemia and abdominal pain. Patient presented with abdominal pain, fatigue and anemia. She denied diarrhea, fever and rash. Laboratory findings were as follows; Hb:9.4, WBC 10.3, MCV 66 , ESR 21, Iron 19 Ferritin 7 (low) and albumin 3.3 and liver function tests were within normal limits. Patient did not have eosniophilia. IBD serology 7 was positive for Crohn's disease. Physical examination was unremarkable. Abdominal CT was wiithin normal limits. The upper and lower endoscopy were non-diagnostic. Eventually patient underwent capsule endoscopy for suspected small bowel Crohn’s disease. Capsule was placed endoscopically into the duodenum. Several dancing small hookworms were visualized in the upper small intestine hooked to the mucosa. The rest of the small bowel mucosa was appearing normal. Patient had no history of travel to endemic areas or sick contacts. The stool microscopy test for ova and parasite eggs were negative. Patient was treated with mebendazole for three days which was repeated in two weeks . Follow up labs in one month after the treatment revealed normal ESR and albumin and resolution of anemia. In this case, stool microscopy failed to identify the eggs of the parasite. This finding was also seen cases of hookworm infections diagnosed by capsule endoscopy in adults. Hookworm infections could be seen in non endemic areas in United States. It should be considered in children presenting with iron deficiency anemia and hypoalbuminemia.

101 Pediatric Gastrointestinal Sarcoidosis: Successful Treatment With Infliximab. A.A. Al-Hussaini, L. Al-Awdah, A. Nahari, Division of Pediatric Gastroenterology, King Saud bin Abdulaziz University for Health Specialities, King Fahad Medical City, Children's Hospital, Riyadh, SAUDI ARABIAD. Alshahrani, Division of infectious diseases, King Saud bin Abdulaziz University for Health Specialities, King Fahad Medical City, Children's Hospital, Riyadh, SAUDI ARABIAM. Fagih, Department of Pathology, King Saud bin Abdulaziz University for Health Specialities, King Fahad Medical City, Riyadh, SAUDI ARABIA. Background and objective: Gastrointestinal (GI) sarcoidosis is a rare disease mainly reported in the adult population with very limited data in children. Here we present two pediatric cases of GI sarcoidosis, added to the 4 pediatric cases of GI sarcoidosis already reported in pediatric literature, and report the first pediatric case of successful treatment with infliximab. Case reports: An 8-year old Saudi girl presented with continuous fever, weight loss, cough and abdominal pain that was followed in few months with hematemesis, melena, and development of hepatosplenomegaly. The other case was a 9-year old Sudanese boy who manifested with vomiting, epigastric pain, and weight loss. On upper endoscopy, both cases demonstrated severe erosive nodular gastric mucosa and erythematous granular esophageal mucosa. Colonoscopy including terminal ileum was normal. Gastric and esophageal biopsies had shown non-caseating granulomas. In addition to GI involvement, the girl had histopathological evidence of granulomatous hepatitis, and lung nodularity on CT-chest. The boy had elevated angiotensin converting enzyme level of 135 U/L (normal, 29 – 110 U/L). Because of the failure to respond to a two months course of anti-tubercolosis therapy, the systemic involvement with prominent lung findings, and negative extensive work up for infectious, infiltrative, immunodeficiency, and other inflammatory causes of granulomatous GI disease, the diagnosis of GI sarcoidosis was contemplated and corticosteroid therapy was initiated. Follow up endoscopy and biopsies confirmed endoscopic and histopathological resolution of the GI disease. The girl continues to be free of symptoms for 3 years after tapering steroid therapy, while the course of the disease in the boy was characterized by frequent relapses off steroids and complicated by development of gastric mucosal atrophy and submucosal fibrosis and pernicious anemia. The disease was brought into a remission for 1year off steroid therapy by infliximab. In conclusion: The overlap in clinical, endoscopic, and histopathological findings between GI sarcoidosis and other commoner granulomatous GI diseases is a substantial reason for delay in diagnosis and treatment. Infliximab is effective in the treatment of steroid dependent GI sarcoidosis.

102 Mesenteric Cysts Presenting as Acute Abdomen in an Infant.. Y. Sharma, O.F. Almadhoun, Pediatric , University of Kansas Medical Ctr, Kansas City, Kansas, UNITED STATES. We report a 2 month old infant who presented with one day history of acute bilious emesis, fussiness, lethargy and abdominal distension. Physical exam was remarkable for a tender, tense and distended abdomen with hypoactive bowel sounds. Patient was also intermittently tachycardic, tachypneic, and febrile. An upper GI series with small bowel follow through showed dilated loops of bowel proximally and distally with possible concern for malrotation and midgut volvulus. Due to the above findings an exploratory laparotomy was performed which revealed multiple fluid filled cysts in the mesentery of the bowel starting around the mid-jejunum and intra-peritoneal purulence. These cysts were decompressed; biopsy of cyst wall was obtained and was consistent with mesenteric-omental cyst. Mesenteric cysts are rare in the pediatric population. Presentation may vary from asymptomatic mass to acute abdomen. Ultrasound and CT imaging are usually done for detecting cyst size and position. Mesenteric cysts are a diagnostic challenge due to rarity of the entity itself and also because of such variability in presentation

103 Evolution of Adult Autoimmune Enteropathy In a 12 year Old Female. A. Al-Nimr, A. Aktay, T. Sferra, Division of Pediatric Gastroenterology & Nutrition, University Hospitals Rainbow Babies & Children's Hospital, Cleveland, Ohio, UNITED STATES. 12-year-old female with mild developmental delay presented with nausea, emesis, and abdominal pain. Six months earlier she was diagnosed with Hashimoto thyroiditis and type 2 diabetes mellitus and treatment with Metformin and Synthroid was initiated. Over 6 weeks, she required 3 hospitalizations for these complaints with spontaneous resolution by discharge. Physical exam was normal. She had normal CBC, ESR, CRP, pancreatic and liver enzymes. Fecal calprotectin was 355 ug/g. Upper GI series with small bowel follow through revealed delayed transit but no anatomic abnormalities. EGD & colonoscopy with biopsies were normal. 8 weeks after presentation, she developed watery osmotic diarrhea. CT of abdomen and pelvis revealed fluid filled small and large bowel without evidence of obstruction. Kidney biopsy for acute kidney injury revealed Acute Tubular Necrosis and Tubulointerstitial Nephritis. She was intubated due to respiratory failure with subsequent tracheostomy. Over the next 12 weeks, she had fevers, elevated inflammatory markers, luekocytosis with marked bandemia and ileus. An infectious workup was negative. Bone marrow aspirate and biopsy and Immunodeficiency workup were negative. She had no evidence of vasculitis. Genetic evaluation for Wolfram, Alstrom, APECED, Prader-Willi and Angelman syndromes was negative. 6 weeks later she developed severe secretory diarrhea. Repeat endoscopy revealed partial villous blunting of the duodenum with paucity of intraepithelial lymphocytes, lymphoplasmacytic inflammation in the lamina propria, and scattered active cryptitis. Colonic histopathology revealed focal active colitis. Indirect Immunofluorescence using her serum was performed showing positive staining of goblet cells with anti-human IgG with serum dilutions up to 1:100 and negative IgA & IgM staining. At this dilution level, the chance of artifact accounting for positive Anti-Goblet cell antibody was low. She was diagnosed with autoimmune enteropathy (AE). Methylprednisolone intravenously was initiated. She clinically improved, was weaned off the ventilator and resumed ambulation. Fecal calprotectin normalized. Intestinal biopsies 1 and 3 months after initiation of steroid therapy revealed patchy enteropathy sequentially decreasing in severity, marked esophageal & gastric improvement & a normal colon. Tacrolimus was started and the a steroid taper initiated. 12 weeks after immunosuppressive initiation, she was able to tolerate oral supplements and small bites of soft foods. This case sheds light on the relapsing and remitting initial progression of autoimmune enteropathy. This is the youngest reported case of 'adult' autoimmune enteropathy. The elevated fecal calprotectin while symptomatic with normal initial endoscopic findings, indicate the bowel is not uniformly affected at onset. Esophageal, gastric, and colonic involvement appeared after small bowel inflammation and resolved prior to small bowel healing. Disease may alternate between osmotic and secretory diarrhea and ileus. It also supports observations that AE is a systemic disease caused by an immune epiphenomena, primarily affecting small bowel that may involve other organs including bone marrow, lungs & kidneys.

104 Pseudotumor Cerebri As A Manifestation Of Celiac Disease. M.B. Beg, A. Ramaswami, SUNY Upstate Medical University, Syracuse, New York, UNITED STATEST. Dawood, Ziauddin University, Karachi, PAKISTANM. Tanveer, Jinnah Medical & Dental College, Karachi, PAKISTAN. There is a well-documented relationship between epilepsy and celiac disease, including a syndrome characterized by epilepsy, occipital calcifications, and celiac disease but literature is scant about pseudotumor cerebri and celiac disease. REVIEW SUMMARY: We report the case of a 14 year-old with headaches, blurring vision and papilledema, noted to have increased opening pressure of cerebrospinal fluid on lumber puncture, diagnosed with pseudotumor cerebri. The patient was seen at our GI clinic because of refractory constipation. Evaluation included celiac serology which revealed elevated anti-tissue transglutaminase and anti-endomysium antibodies. Upper gastrointestinal endoscopy confirmed the diagnosis of biopsy-proven celiac disease. After starting a gluten-free diet the patient showed a dramatic improvement in constipation and pseudotumor cerebri symptoms resolved. CONCLUSION: This case emphasizes the need to include celiac disease in the differential diagnosis when investigating the etiology of pseudotumor cerebri.

105 EBV Driven Smooth Muscle Tumor in the Colon. R. Bensen, J.C. Burgis, D. Bass, J. Kerner, Pediatric Gastroenterology, Hepatology and Nutrition, Stanford University, Stanford, California, UNITED STATESD. Bingham, Pathology, Stanford University, Stanford, California, UNITED STATES. A 9 year-old girl with a complex medical history including heart transplant, EBV viremia, venous thrombosis, hemorrhagic pancreatitis, protein losing enteropathy (PLE), and abdominal hernias presented with a third episode of severe peri-umbilical abdominal pain associated with emesis, distention and fever. On exam, she was febrile and tachycardic with a soft, but protuberant abdomen, stable ascites and a reducible umbilical hernia. Labs showed an elevated CRP, elevated /Cr, and low albumin. Magnetic resonance imaging of the abdomen showed an internal hernia containing small bowel versus an intramural or intraluminal mass near the proximal transverse colon. After multiple attempts, a large polypoid colonic mass was removed endoscopically. Histopathology demonstrated uniform spindled cell population with strong smooth muscle actin expression, supporting smooth muscle proliferation. In situ hybridization for EBV early RNA (EBER) was positive. Immunohistochemical stains for CD117 and myogenin were negative, excluding gastrointestinal stromal tumor or rhabdomyosarcoma. Overall, this was most consistent with an EBV driven, post-transplant smooth muscle tumor (PTSMT). Further imaging identified soft tissue masses in the paraspinal musculature, which upon biopsy, were consistent with PTSMTs. Her immunosuppression regimen was modified from cyclosporine and CellCept to cyclosporine and sirolimus, given its anti-proliferative effect. Discussion: While post-transplant lymphoproliferative disorders (PTLD) can be seen in up to 10% of transplant recipients, PTSMTs are rare. Tumors are thought to arise from smooth muscle within vascular walls and can be multifocal in distribution. Reported sites include liver, upper and lower airways, brain, and soft tissue; colonic involvement is unusual. Prognosis is mixed with treatment primarily comprised of reduction in immunosuppression and surgical excision of tumors.

106 Recurrent Lower Gastrointestinal Bleeding from Idiopathic Colonic Varices. J. Berauer, K. Gorla, Advocate Children's Hospital, Park Ridge, Illinois, UNITED STATES. A previously healthy 17-year-old male presented with acute rectal bleeding. There was no previous history of gastrointestinal bleeding or gastrointestinal symptoms. The physical examination, including orthostatic vital signs, was unremarkable. Following admission, his hemoglobin concentration decreased from 11.3g/dL to 7.7g/dL, he became hypotensive and required packed red blood cell transfusion. Basic laboratory tests including a coagulation profile, platelet count, leukocyte count, and hepatic function panel were normal. Technetium-99m pertechnetate scintigraphy, esophagogastroduodenoscopy, colonoscopy, and capsule endoscopy were nondiagnostic. He was discharged with no further bleeding and stable hemoglobin. Six days later, he was readmitted with massive hematochezia. Repeat colonoscopy revealed multiple tortuous submucosal veins throughout the entire length of the colon (Figure1) and terminal ileum (Figure2). Findings on abdominal US with Doppler imaging of the liver, spleen, and hepatic and portal veins were normal. At five-month follow-up, there were no further bleeding episodes and hemoglobin remained stable (12.4g/dL). Six weeks later, the patient was readmitted with hemodynamically significant rectal bleeding (Hgb 8.9g/dL). Superior and inferior mesenteric and celiac arteriography were undertaken. These studies showed normal arterial anatomy without intraluminal extravasation of contrast material. However, acquisition of delayed venous phase images demonstrated venous pooling originating near the ileocecal valve, extending distally from the ascending colon to the level of the hepatic flexure. Due to repeated episodes of life-threatening anemia and hematochezia, our patient underwent a laparoscopic right hemicolectomy. Colonic varices are a rare cause of lower gastrointestinal bleeding usually associated with portal hypertension or portal venous obstruction. In patients without underlying liver disease, a significant proportion of cases have shown familial aggregation, which may be related to hereditary mesenteric vascular anomalies. Direct visualization during colonoscopy is diagnostic. However, excessive air insufflation or examination during a period of hypotension, as occurred during this patient’s initial colonoscopy, may collapse the varices, obscuring visualization. The most reliable diagnostic study is selective mesenteric angiography, with identification of enlarged vessels during the venous phase. Recognition of this condition, particularly in patients with life- threatening hemorrhage or recurrent lower GI bleeding, must be emphasized. Management beyond the initial resuscitation period has not been standardized. Conservative approaches are preferable for those with less significant bleeding. Surgical resection of the involved colon is reserved for patients with life-threatening or recurrent bleeding. Other options include catheter embolization, variceal banding, sclerotherapy, and, in patients with portal hypertension, portal decompression.

107 Langerhans Cell Histiocytosis Presenting with Vomiting and Duodenal Narrowing. C. Larson-Nath, A. Martinez, J.M. Cabrera, Pediatric Gastroenterology, Medical College of Wisconsin, Milwaukee, Wisconsin, UNITED STATESM. Suchi, Pediatric Pathology, Medical College of Wisconsin, Milwaukee, Wisconsin, UNITED STATES. Introduction: Langerhans cell histiocytosis is a rare disease stemming from the proliferation of Langerhans cells leading to a diverse range of presenting symptoms. Case: A 16 month old female presented with a 4 month history of vomiting and weight loss. An upper GI series revealed a long segment of duodenal luminal narrowing; and loss of normal folding pattern of the entire duodenum and proximal jejunum. The patient was well until about 1 year of age after the introduction of cow’s milk which caused vomiting. A trial of soy caused diarrhea that resolved upon resuming cow’s milk. The emesis contained food that had been ingested up to 12 hours prior and was occasionally bilious. She had also lost 3 pounds over the previous 4 months. Her past medical history was significant for difficult to treat eczema and seborrheic dermatitis. These cutaneous lesions were present since birth but did improve with topical steroids. Laboratory evaluation demonstrated normal electrolytes, bilirubin, ESR, transaminases, alkaline phosphatase, coagulation studies, WBC count, hemoglobin, thyroid studies, tissue transglutaminase IgA, and total IgA. The platelets were elevated at 906 and the albumin was low at 2.1. Initial upper endoscopy revealed extensive stricturing ulcerations of the duodenal bulb. Colonoscopy revealed areas of diffuse nodular mucosa interspersed with areas of white patches with increased vascularity. Histopathology of the terminal ileum and colon showed an expanded lamina propria with an admixture of inflammatory cells including CD1a positive Langerhans cell histiocytes. A skin biopsy from the lower abdomen was diagnostic for Langerhans cell histiocytosis (LCH). Evaluation of other systemic findings of LCH showed lytic lesions in two fingers. Chest x- ray and head MRI were normal. This patient is being treated per protocol LCH III with vinblastine and steroids. She has started to tolerate a regular diet. Follow up colonic biopsies showed a partial response to chemotherapy. LCH is a rare disease that can affect almost any organ system. GI involvement is very rare and normally manifests as diarrhea or less often constipation. Patients with GI involvement tend to be younger and overall have worse outcomes with up to a 59% mortality rate at 18 months. We present the first documented case of LCH presenting with duodenal narrowing. It is important that clinicians are aware of LCH and GI system involvement because of the significant mortality associated with the disease.

108 Rare Presentation of Zollinger-Ellison Syndrome in a Teenage Female with Type I Neurofibromatosis. S.L. Ciciora, B.M. Boyle, Division of Gastroenterology, Nationwide Children's Hospital, Columbus, Ohio, UNITED STATES. BACKGROUND: Zollinger-Ellison syndrome (ZES) is a rare disorder with gastrin secreting tumors that can lead to a variety of GI symptoms. Neurofibromatosis type I (NF-1, OMIM 162200) is an autosomal dominant disorder that features cutaneous and osseous lesions. Patients with NF-1 have a four times risk of malignancy due to a mutation of the neurofibromin protein (NF1 gene) on chromosome 17q11.2. We present the unusual case of a young woman with known NF-1 who presented to the GI service and was found to have ZES. CASE: A 17-year-old female with NF-1 (full gene deletion) presented to the GI clinic with abdominal pain, nausea, weight loss, and diarrhea for the previous 5 months. The patient was admitted at an outside facility 2 months prior for similar symptoms and was found to be anemic and hypoalbuminemic. EGD and colonoscopy were performed with endoscopic findings concerning for celiac disease related to duodenal scalloping. However, histology and serologic testing were negative. Her symptoms improved on PPI therapy following that admission. Upon discontinuation of the PPI therapy, her symptoms recurred and she was referred to our clinic. The patient was admitted to the inpatient service due to weight loss and poor nutrition. Her evaluation included upper GI concerning for duodenitis. Subsequent EGD revealed an erythematous and ulcerated duodenum with histology notable for acute infiltration of neutrophils. A fasting serum gastrin level was obtained given the severe duodenitis and the association of gastrinoma with NF-1. The gastrin level was markedly abnormal (2380 pg/mL; normal <100). The patient had symptomatic improvement upon resumption of twice daily PPI therapy and her weight returned to normal. Subsequent evaluation included somatostatin scintigraphy that revealed two foci of abnormal uptake consistent with gastrinoma. Surgical excision via laparotomy and duodenotomy of two anti-mesenteric duodenal nodules was completed and pathology was consistent with gastrinomas. Intraoperative octreotide scan following excision showed no residual activity. The patient is well following this with normal serum gastrin levels. Further genetic testing for a deletion in the gastrin gene (17q21.2) was obtained and was normal. DISCUSSION: The association between these two uncommon disorders has been reported, but not in someone this young with multiple gastrinomas. Given the increased risk of malignancy seen in patients with NF-1 and the recognized benefit on survival of early diagnosis of gastrinomas, the constellation of abdominal pain, weight loss, and diarrhea in the setting of ulcer disease should lead to evaluation for ZES. Furthermore, though not present in this case, the recognition that the NF-1 and gastrin genes are located near one another on chromosome 17 provides a novel, theoretical explanation in regards to the recognized association between these two disorders.

109 Severe Recurrent Lower Gastrointestinal Bleeding With Profound Anemia in Teenager With Diffuse Cavernous Hemangioma of the . T. Ciecierega, Pediatrics, NYP-Weill Cornell Medical College, New York, New York, UNITED STATESM. Sultan, Pediatrics , Alquds Medical College , Jerusalem, PALESTINE, STATE OF. Background: Lower gastrointestinal (LGI) bleeding is common among pediatric patients. Some causes of LGI causing profound anemia include Inflammatory Bowel Disease, Meckel’s diverticulum, angiodysplasia and infectious colitis. Hemangiomas of the GI system are rare in children. Diffuse Cavernous Hemangioma of the Rectum (DCHR) is a rare benign vascular lesion. The rectosigmoid area is the most common site in the . We report a case of a 15 year old male with intermittent life-threatening bleedings who was diagnosed with DCHR. Case Report: A 15 year old male with recurrent episodes of large-volume hematochezia starting from 1 year of age was referred for consultation. At presentation he had severe microcytic anemia (Hgb 4, normal coagulation studies). He was admitted to PICU and hemodynamicaly stabilized with blood transfusions. He underwent an emergent endoscopic evaluation. His upper endoscopy was normal. Colonoscopy showed a DCHR starting from the dentate line and extending 11 cm proximally. The vessels were dilated having varix-like appearance. The distal part of the hemangioma was encircling the rectum circumferentially. The Cat-Scan (CT) with angiography of the abdomen and pelvis showed marked thickening of the rectal wall with intramural and perirectal dilated vessels. Patient is undergoing evaluation for surgical resection of hemangioma. Complete resection of DCHR with colo-anal anastomosis is generally regarded as the only treatment that controls bleeding effectively due to the involvement of all the layers of the rectal wall and rectal mesentery. Nonoperative techniques usually provide only temporary relief. Conclusion: DCHR is a rare benign vascular lesion in children and it is not uncommon for a delay in the diagnosis. It usually presents as chronic recurrent rectal bleeding. Management is usually surgical. Physicians should be alert to the presence of DCHR in young patients who complain of rectal bleeding.

110 Budesonide Maintenance Therapy for Triad of Autoimmune Hepatitis, Celiac Disease, and Pancreatitis in 16-year-old female. R.A. Dorner, Y. Rekhtman, Georgetown University Pediatric Gastroenterology, Georgetown University Medical Center, Washington, District of Columbia, UNITED STATES. We report on a unique regimen of steroids, gluten-free diet and oral Budesonide for treatment of autoimmune hepatitis (AIH) and pancreatitis in the setting of celiac disease (CD) in a 16-year-old female. The patient was referred to our tertiary care center after initial pancreatitis diagnosis. Upper endoscopy and percutaneous liver biopsies confirmed the pathological tissue diagnoses of concomitant CD and AIH. AIH therapy typically consists of Azathioprine (AZA) and a steroid taper, with or without Budesonide in an adjunct role. Budesonide was selected as the primary AIH maintenance therapy due to concern for pancreatitis recurrence on AZA and in light of a long- standing CD history for which Budesonide is a proven primary treatment. Improvement of her AIH was quantified by normalization of her obstructive enzyme pattern, jaundice, and aminotransferase levels. Resolution of the pancreatitis was demonstrated by normalization of amylase, lipase and pain levels. Budesonide can be a successful primary maintenance therapy for AIH in the setting of CD. This case supports future research into alternative maintenance therapies for AIH patients with coexisting diseases of immunological origin. Keywords: autoimmune hepatitis, celiac disease, Budesonide, pancreatitis

111 Diagnosis of Familial Adenomatous Polyposis in a cognitively impaired teenage male with microcytic anemia.. A. Elayappen , A. Muthukumar, pediatrics , UTMB, Galveston , Texas, UNITED STATESR.S. Radhakrishnan, Pediatric surgery, Utmb, Galveston, Texas, UNITED STATES. We report an interesting case of a cognitively impaired 15 yr old male with microcytic anemia and Familial Adenomatous Polyposis(FAP). He initially presented with a fungal infection of toe which required treatment with terbinafine; Baseline complete blood count incidentally revealed profound microcytic anemia with hemoglobin of 4.8 g/dl, MCV of 57 and iron studies were indicative of iron deficiency anemia. Review of systems was positive for intermittent, diffuse, dull, right sided abdominal discomfort without hematochezia. His family medical history revealed that his maternal uncle died at 23 years from colon cancer and maternal grandfather had unknown GI cancer at 40 years of age. Given his abdominal symptoms, Meckel scan was done which showed mildly increased uptake and was followed by diagnostic laparoscopy which eventually ruled out Meckel’s diverticulum; Colonoscopy was done as the next step to rule out GI causes for anemia which revealed numerous polyps (more than 100) extending from ileum to rectum. Biopsies of these polyps were pathologically identified as adenomas which confirmed FAP coli. Metastatic workup was negative. He underwent proctocolectomy followed by ileal pouch anal anastamosis. Post surgery his hemoglobin improved significantly along with continued oral iron supplements. Various degrees of developmental delay have been reported in patients with FAP due to APC gene deletion/duplication, but the testing was negative in our index case with cognitive impairment. He has been disease free since his surgery with no further manifestations of FAP.

112 HENOCH-SCHöNLEIN PURPURA ASSOCIATED WITH PNEUMATOSIS INTESTINALIS. A. Fatima, D. Gibson, William Beaumont Hospital, Royal Oak, Michigan, UNITED STATES. Henoch-Schönlein purpura (HSP) is the most common vasculitis in children. It is a disorder of the inflammatory cascade leading to IgA deposition and leukocytoclastic vasculitis of small vessels of skin, kidneys, joints and gastrointestinal tract. A wide variety of gastrointestinal (GI) manifestations are seen in approximately 50-75% of patients with HSP. Diffuse colicky abdominal pain is the most common gastrointestinal symptom. The small bowel is the most frequently involved GI site. Intussusception is rare, but is the most common surgical complication. We report a 2 year old girl with Henoch-Schönlein purpura who presented with abrupt onset of abdominal pain followed by a purpuric rash. Her urinalysis, CBC, and tests of renal function were normal. Plain radiographs (an acute abdominal series) were unremarkable initially, and abdominal ultrasound imaging again showed ascites and thickened bowel loops. She received one dose of prednisone, 1mg/kg. Abdominal computed tomography (CT) was done due to a bout of severe abdominal pain. CT demonstrated pneumatosis intestinalis (PI) in the descending colon. (CT scan pictures will be presented). She was started on total parenteral nutrition (TPN), bowel rest and vancomycin and piperacillin/tazobactam. Because of the pneumatosis, prednisone was discontinued and she was given 2mg/kg intravenous immunoglobulin (IVIG). Her abdominal pain improved gradually over the following week and a repeat CT scan showed significant improvement of the pneumatosis. The purpuric skin rash improved, her abdominal pain resolved. We report a case of HSP and pneumatosis intestinalis (PI), an association which, to our knowledge, has never been reported.

113 Ectopic gastric mucosa within the small intestine resulting in intestinal obstruction. A.J. Freeman, S. Kugathasan, C.G. Sauer, Pediatric Gastroenterology, Hepatology and Nutrition, Emory University School of Medicine/Children's Healthcare of Atlanta, Atlanta, Georgia, UNITED STATES. Introduction: Studies have shown that up to 5-10% of the population develop patches of ectopic gastric mucosa (EGM) that are usually discovered as an incidental finding on endoscopy. The clinical significance of these patches of EGM varies widely with physicians often relying on clinical manifestations to direct therapy. Case Report: We present a 15 year old female who presented to an outside facility with several days of sharp, periumbilical abdominal pain that occurred every 5-10 minutes and lasted only a few minutes on each occasion. She also complained of intermittent non-bloody/non-bilious emesis with decreased oral intake, but denies any other systemic symptoms. Initial evaluation included an abdominal radiograph which was normal as well as laboratory work including a CBC with differential, CMP, ESR, CRP and UA. All laboratory findings were reported as normal. She was given dicyclomine PRN but pain persisted overnight and returned the next morning to the outside facility. A CT of her abdomen was performed and was unremarkable. The patient was transferred to our institution for further care due to ongoing pain and emesis. Upon arrival, a repeat abdominal radiograph was obtained and was once again normal. An upper gastrointestinal (UGI) series was ordered showing a high-grade obstruction at the duodenal/jejunal junction. The patient underwent upper endoscopy the following day to better characterize the obstruction. A high grade obstruction was encountered along with an ulcerated lesion. Mucosal biopsies were obtained. Biopsies demonstrated heterotopic gastric tissue with parietal cells present and surrounding chronic inflammation. We felt the acid-producing parietal cells were likely the cause of the ulcerated lesion and chronic inflammation that ultimately resulted in her obstruction. The patient was started on high dose proton-pump inhibitor (PPI) therapy. Clinically the patient responded well to PPI therapy and was able to resume oral nutrition within 1 week. She was maintained on high dose PPI therapy for 6 months and was then weaned off. She has clinically continued to do well with no recurrence of her symptoms and repeat endoscopy is planned. Teaching Point: Gastric heterotopia has been reported throughout the gastrointestinal tract and often causes inflammation/ulcers due to acid secretion. Our patient developed ulcers and obstruction due to gastric heterotopia and acid secretion.

114 Recurrent lower gastrointestinal hemorrhage due to idiopathic jejunoileocolonic vascular malformations. B. Godwin, Department of Pediatrics, New York Presbyterian - Weill Cornell, New York, New York, UNITED STATESS. Kim, Division of Gastroenterology, New York Hospital Queens, Flushing, New York, UNITED STATESS. Fishman, Division of Pediatric Surgery, Boston Children's Hospital, Boston, Massachusetts, UNITED STATESV. Fox, Division of Pediatric Gastroenterology, Hepatology & Nutrition, Boston Children's Hospital, Boston, Massachusetts, UNITED STATESA. Turkish, Division of Pediatric Gastroenterology & Nutrition, New York Hospital Queens, Flushing, New York, UNITED STATES. A 21-year-old male with a history of a dilated aortic root presented with 3 days of painless hematochezia and weakness. He was afebrile and displayed a benign abdominal exam but was guaiac (+). While the hemoglobin was initially 13.3 g/dL, the remainder of his complete blood count was intact as well as chemistries, coagulation studies and liver function tests. Examination of the stool failed to reveal an infectious etiology. Following the development of profound hematochezia he underwent a colonoscopy and was found to have diffuse serpiginous varices extending from the rectum through the terminal ileum, as well as in the proximal jejunum. Capsule endoscopy, double balloon enteroscopy and finally, laparoscopic enteroscopy and exploration confirmed the diagnosis and revealed the presence of varices in the proximal jejunum as well. There was no evidence of portal hypertension and mesenteric angiography revealed dilated, ectatic veins draining the ileocolic region as well as the left and sigmoid colon. Although the bleeding initially remitted after a trial of IV octreotide and amicar, he developed massive hematochezia approximately one year later. Despite extensive sclerotherapy with 1% sodium tetradecyl sulfate, he continued to experience painless rectal bleeding with persistent requirement of blood products. A repeat bleeding scan raised concern for a subtle but active bleeding site in the right colon proximal to the hepatic flexure, and a laparoscopic right hemicolectomy with resection of 10 cm of terminal ileum was subsequently performed. Venous malformations were found throughout the resected bowel, as well as focal thrombosis within submucosal malformed veins associated with mucosal ulceration and focal transmural necrosis of the bowel wall. The patient tolerated the procedure well and has exhibited no evidence of further rectal bleeding. Idiopathic colonic varices due to an inborn vascular anomaly are an extremely uncommon cause of lower gastrointestinal bleeding. A recent review described 31 reported cases of idiopathic colonic varices, and an autopsy study in 1962 found only 2 cases of idiopathic colonic varices among 2,912 autopsies, a rate of 0.07% (Feldman 1962). Very few had varices extending into the ileum (Lopes 2006), and there have been no reported cases of idiopathic varices found in the jejunum. Interestingly, there have been ten reported cases of familial idiopathic colonic varices (Krishna 2010), suggesting an inherited abnormality, and in the case review of patients with idiopathic colonic varices, it was found that 30% had a familial history (Han 2006). There have been no reports of an association of a dilated aortic root with such vascular malformations. As such, our patient is currently undergoing an extensive genetic evaluation including exome sequencing in an attempt to illuminate the etiology of his medical history. Although conservative medical management with octreotide or clot stabilizing medications may define treatment in appropriate patients, surgical resection is frequently required to control bleeding and reduce the risk for further blood loss, as occurred in our patient. Pancreas/Cystic Fibrosis 144 Solid-pseudopapillary tumor of the pancreas in a 9-year-old boy. M. Hassan, S. Kim, D.W. Rust, S. Ali, Children's Hospital & Research Center Oakland, Oakland, California, UNITED STATES. Introduction: Solid-pseudopapillary tumor (SPT) of the pancreas is a rare pancreatic tumor, comprising 1 to 2% of exocrine pancreatic tumors. It usually develops in young women, most commonly in the head or body of the pancreas, with the majority of patients asymptomatic at diagnosis. Imaging and pathology have characteristic features that help to distinguish it from more aggressive pancreatic tumors. We present an unusual case of a young boy with SPT whose tumor was located in the pancreatic neck, allowing for surgical sparing of the pancreas. Case Presentation: A 9-year-old previously healthy boy was referred to our institution for an abdominal mass after his primary care physician screened the patient with an ultrasound due to the complaint of abdominal pain. He had endured blows to the abdomen while playing football at school, but had no abdominal trauma requiring medical attention. There was no history of jaundice, nausea, vomiting, abnormal stools, or weight loss. On physical exam, a fullness was palpated in the epigastric region, with discomfort on deep palpation. All labs were within normal limits. An abdominal ultrasound demonstrated a well-defined, nearly round, non-homogenous 3 cm cystic pancreatic mass in the neck and body of the pancreas. A follow up CT of the abdomen and pelvis showed that the lesion was unchanged in size and character. MRI was suggestive of a complex cyst or a neoplastic process with limited vascularity. Endoscopic ultrasound-guided fine-needle aspiration (FNA) biopsy yielded bland round to ovoid cells consistent with probable solid-pseudopapillary tumor of the pancreas. The patient underwent surgery, enucleating the 3.5 x 3.5 x 3.1 cm mass from the neck of the pancreas. The head and distal ¾ of the pancreas were left intact, as was the pancreatic duct. Histologic review showed bland cells arranged in sheets and in papillary- type structures containing small vessels. There were focal cystic and hemorrhagic areas. Immunohistochemistry showed strong nuclear and cytoplasmic reactivity for beta-catenin, supporting the diagnosis of solid- pseudopapillary tumor. Microscopic margins were focally positive. The patient’s post-operative course was complicated by pancreatic leak and mild pancreatitis. He was placed on bowel rest and TPN, until he was tolerating clears without abdominal pain. Conclusion: SPT should not be thought of as a tumor of only young women. Even though SPT is of low malignant potential, it can cause extensive local invasion and has a propensity to be higher risk in males. Symptoms of persistent epigastric pain, which is often attributed to gastroesophageal reflux in children, should be evaluated further if there is no response to initial treatment.

145 Calcific Pancreatitis in Children. M.G. Patel, K. Baker, U.P. Phatak, A. Alper, D.S. Pashankar, Yale University, New Haven, Connecticut, UNITED STATES. Background: Calcific pancreatitis develops in adults with chronic pancreatitis after many years and is often accompanied by pancreatic insufficiency. In children from developing tropical countries, tropical calcific pancreatitis is a well-known entity. However, calcific pancreatitis in children from the western world is extremely rare. Cases: Review of radiology database at our institution revealed 379 children diagnosed with pancreatitis on imaging studies over 22 years. Only two children had evidence of pancreatic calcifications. Patient 1 was a 16 year old female, who presented with an acute onset of abdominal pain and was diagnosed with pancreatitis based on laboratory markers. She had a history of intermittent abdominal pain for 7 years. She had a strong family history of chronic pancreatitis, and was positive for cationic trypsinogen. CT scan showed pancreatic calcifications and stones in the pancreatic duct. She underwent an ERCP which showed a dilated pancreatic duct with stones and fibrosis. Due to persistent pain, she underwent a side to side pancreatojejunostomy and Roux-en-y choledochojejunostomy. She had two subsequent episodes of pancreatitis requiring hospitalization 2 and 4 years after initial presentation. Repeat CT scans show persistent pancreatic calcifications. At her last follow-up, 12 years after initial presentation, she continues to have pancreatic insufficiency and chronic pain syndrome requiring pancreatic enzymes, insulin, and pain medications. Patient 2 was a 14 year old male, diagnosed with acute pancreatitis based on laboratory markers after a 3-year history of intermittent epigastric abdominal pain. He had recurrent attacks of pancreatitis (12 episodes over 11 years). The etiology of his pancreatitis was not clear despite extensive workup. He underwent an endoscopic ultrasound which showed features of chronic pancreatitis. ERCP showed evidence of sphincter of oddi dysfunction and he underwent a sphincterotomy and a partial pancreatic duct sphincterotomy. A CT scan 3 years after his initial presentation showed evidence of pancreatic calcifications. His subsequent CT scans have shown persistent pancreatic calcifications. During 11 years of follow up, he has not developed pancreatic insufficiency. Conclusion: We report two teenagers with calcific pancreatitis. This condition is very rare with a prevalence of 0.5% in children with pancreatitis in our hospital. It is associated with a significant morbidity of pancreatic insufficiency and multiple recurrences.

146 A rare complication of septic phlebitis of portal vein and splenic vein in a 15-year of female with acute pancreatitis. A. Aktay, Rainbow Babies Children's Hospital, Cleveland, Ohio, UNITED STATES. Septic Phlebitis of Portal Vein and its branches is an extremely rare complication of pacreatitis in pediatric population with high morbidity and mortality rate. It has been reported as a complication of inflammatory intraabdominal process such as appendicitis, cholecystitis and diverticulitis in adult population. To our knowledge it has not been reported as a complication of acute pancreatitis in children. We report a case 13-year-old female with portal and splenic vein phlebitis as a complication of acute pancreatitis. Patient presented with bilious vomiting, fever and acute abdominal pain. Laboratory tests showed elevated amylase, lipase, hypoaluminemia, prolonged PT, PTT and high inflammatory markers. An ultrasound of abdomen showed swelling and edema of the pancreas and peripancreatic tissue and phlebitis of the main portal vein extending along the main vein and proximal portal venous branches; and in the splenic vein and portal confluence. A CT scan was suspicious for necrosis in the pancreatic head. There was no thrombus in the portal vein. Patient developed septic shock . Patient was treated with broad spectrum antibiotics,NPO and TPN. The follow up US showed resolution of the phlebitis. Phlebitis of portal vein has high mortality rate without treatment, therefore early diagnosis is required for appropriate treatment. It should be considered in children presenting with acute pancreatitis.

147 A Rare Cause of Acute Pancreatitis. C. Gandhi, T. Bhatia, J. Dadhania, Saint Peter's University Hospital, New Brunswick, New Jersey, UNITED STATES. Introduction: Recent studies have reported an increasing incidence of acute pancreatitis in children over the past 2 decades. Common causes of acute pancreatitis are biliary disorders, medications and idiopathic. Lymphomas and leukemia are very rarely present with acute pancreatitis in pediatric patients. We are reporting a case of extra medullary Acute Myeloid Leukemia (AML) presenting as an acute pancreatitis. Most likely, it was caused by obstruction of pancreatobiliary ducts by masses of extramedullary AML which has never been reported in literature to date in pediatric population. Case Report: 10 year old male with h/o vitiligo presented with symptoms of acute onset abdominal pain, vomiting and lethargy. Past medical history is significant for increased TSH and positive thyroglobulin antibody and normal T4 not being on thyroid supplements. Family history is significant for parental consanguinity and Type 2 DM in parents, maternal history of papillary carcinoma of thyroid s/p resection and paternal history of Alopecia and h/o Hashimoto’s thyroiditis in elder brother. He had h/o abdominal pain, on and off for 1 month. He was seen by PMD and was diagnosed as having constipation and treated with miralax. He improved for the next 2 weeks and began having similar symptoms of intermittent vomiting and abdominal pain. 2 weeks prior to this admission, He was admitted to the hospital for severe vomiting and abdominal pain. During that hospitalization, he had elevated AST 283, ALT 244 and Lipase 604. Amylase was normal and CT scan was unremarkable. He was given iv hydration and discharged with suspected viral syndrome. According to mom, he had increased frequency of abdominal pain, mostly epigastric to periumbilical regions and nonbilious, nonbloody vomiting in past 1 week. He also lost 8 pounds in last 1 month. Initial Blood work showed normal CBC, electrolytes, amylase and elevated LFTs (Bilirubin 2.8, AST 150, ALT 194, Alk Phos 469) and Lipase 1089. Abdominal USG showed diffusely enlarged pancreas suggestive of acute pancreatitis and increased echogenicity of the liver s/o hepatitis without signs of biliary duct obstruction. Hepatitis A Ig M, HbsAg and Hepatitis C were negative. Quantitative immunoglobulins were normal including Ig G subclasses. Upper GI series, done to evaluate bilious vomiting which developed on day 2 of admission, showed numerous filling defects in second and third portion of duodenum. Endoscopic examination was done which revealed several masses protruding in duodenum in the vicinity of ampulla of duodenum. CT abdomen showed enlarged pancreas with multiple nodules in duodenum, jejunum and ileum with significant increase in the size and number of mesenteric and retroperitoneal lymph nodes. Biopsy from duodenal lesions showed mucosa with mononuclear infiltrate, mixed with eosinophils s/o myeloid sarcoma/leukemia. Subsequently, lymph node and bone marrow biopsy showed increased leukemic cells with 19 % blast cell s/o AML. After starting chemotherapy, patient improved significantly and pancreatitis resolved with normal lipase and normal pancreas on repeat CT scan. This case signifies the importance of finding the underlying etiology of acute pancreatitis and treats it to resolve pancreatitis.

148 Total Pancreatectomy with Autologous Islet Cell Transplant in a 4 year old girl for Chronic Relapsing Pancreaitits. S. Raikar, F. Del Rosario, Pediatric Gastroenterology, Hepatology and Nutrition, AI duPont Hospital for Childrens, Wilmington, Delaware, UNITED STATESS. Chinnakotla, Pediatric Solid Organ Transplant Center, University of Minnesota Amplatz Children's Hospital, Minneapolis, Minnesota, UNITED STATES. This is a case of a 4 year old female with chronic relapsing pancreatitis. She developed her first episode of pancreatitis at age 12 months. She has required hospitalizations every 3- 4 weeks for recurrent episodes of pancreatitis. Her initial workup included a normal lipid panel, ANA, IGG4 subclasses. Screening for cystic fibrosis with a sweat test was normal. Genetic panel for recurrent pancreatitis was negative for mutations for SPINK1, CFTR97, and PRSS1 at age 2 years 7 months. Imaging studies performed included abdominal ultrasounds and a MRCP. Her MRCP revealed normal appearing pancreas, gallbladder, and common bile duct. The principal pancreatic duct was normal; however there were 3 strictures in the accessory duct. Empiric sphincterotomy without stent placement was performed at 3.5 year old age. She continued to have frequent episodes of pancreatitis. She had a repeat MRCP done at 3 years and 9 months which revealed a central high grade pancreatic duct stricture. There was upstream ductal dilation and diffuse side branch dilation. 2 months later, she had an ERCP with minor papilla sphincterotomy and stent placement. She was started on Creon supplementation with low fat diet. The stent was dislodged prematurely and she required hospitalization for pancreatitis soon afterwards. During this hospitalization nasojejunal tube feeds were initiated. She underwent her third ERCP one month later and was noted to have incomplete . Dilation of minor papilla and stent placement was performed; catheter dilation of duct of Wirsung with stent placement was also performed. Due to worsening pancreatitis, the stents were removed within 2 weeks. She was discharged home on nasojejunal feedings as sole source of nutrition. We sent the Ambry genetic panel with results confirming a heterozygous SPINK1 mutation, heterozygous CTRC mutation. Since her 3rd ERCP, she has had 7 hospitalizations for pancreatitis necessitating a port placement due to poor vascular access and she required escalating doses of intravenous narcotics to manage her pain. Her overall growth and quality of life was poor. She underwent evaluation for a total pancreatectomy and autologous islet cell transplant. The procedure was performed at age 4 year 11 months. She is presently being managed by the transplant team which includes a transplant surgeon, pediatric gastroenterologist, pediatric endocrinologist, and a pain managment specialist. She has severe gastroparesis which is expected after the surgery. A full recovery is anticipated in the next 1-2 months. A gastrojejunal tube was placed and she is tolerating jejunal feeds with small amounts of food by mouth. She is currently receiving insulin replacement with the plan of starting to withdraw insulin support 3 months post procedure. Her pain is being managed with oxycodone. She will need to take pancreatic enzyme supplementation for the rest of her life. For patients with a genetic cause for pancreatitis that is severe and debilitating, total pancreatectomy with autologous islet cell transplant should be considered for improvement of quality of life.

149 Kaposiform Hemangioendothelioma of the Pancreas: A Rare Cause of Obstructive Jaundice in a Toddler. S. Sheflin-Findling, J. Chu, R. Arnon, Division of Pediatric Hepatology, Mount Sinai Medical Center, New York, New York, UNITED STATESS. Sheflin-Findling, K. Iyer, J. Chu, R. Arnon, Recanati/Miller Transplantation Institute, Mount Sinai Medical Center, New York, New York, UNITED STATESK. Iyer, Department of Surgery, Mount Sinai Medical Center, New York, New York, UNITED STATES. The most common causes of obstructive jaundice in toddlers are cholelithiasis and . Other pathologies, including extrinsic compression on the biliary tree, chronic pancreatitis, and pancreatic tumors, are relatively rare. A 3-year old girl presented with worsening abdominal distention for 2 months and abdominal pain for 1 week. On exam, the patient was afebrile with normal vital signs and was noted to be jaundiced with a palpable right upper quadrant mass. Initial laboratory evaluation revealed the following: total bilirubin 2.4 mg/dL, direct bilirubin 1.4 mg/dL, gamma-glutamyl transpeptidase 986 U/L, alanine aminotransferase 251 U/L, international normalized ratio 1.2, hemoglobin 8.2 g/dL and platelets 183,000/L. Abdominal ultrasound showed dilated intra- and extrahepatic biliary ducts and an enlarged pancreas with abnormal echogenicity. Magnetic resonance cholangiopancreatography (MRCP) confirmed these findings and suggested a possible periampullary mass. An endoscopic retrograde cholangiopancreatography (ERCP) was done shortly after and revealed a normal ampulla and a 5cm stricture of the common bile duct (CBD) with proximal dilation that required stent placement. The presumed diagnosis at that time was idiopathic fibrosing pancreatitis with obstruction of the CBD secondary to external compression by the pancreatic head. Follow-up MRCP was done two months later and again was concerning for a pancreatic mass. The patient then underwent an exploratory laporatomy. Intra-operative findings included a thickened and densely strictured CBD, a hardened pancreatic head and a fibrotic micronodular appearing liver. A cholecystectomy was performed and a hepaticojejunostomy was created. The histology was consistent with Kaposiform hemangioendothelioma (KHE) of the pancreas and biliary cirrhosis. The patient was initially treated with steroids (2mg/kg/day) for 3 months. Repeat MRCP showed a significant increase in the size of the pancreatic lesion. Steroids were resumed and Vincristine was added for a 3-month course, but subsequent imaging showed no response. The patient then underwent resection with a pancreaticohepatoduodenostomy (Whipple procedure). Laboratory and imaging follow-up 2 years later shows no further signs of obstructive jaundice or recurrence. Kaposiform hemangioendothelioma (KHE) is a rare vascular tumor that occurs most commonly in childhood. It is a locally invasive tumor and is not known to produce metastases. The skin is the most commonly affected organ, followed by soft tissue and bone involvement. Visceral involvement is very rare. Spontaneous regression is not often seen. Medical therapy includes corticosteroids, vincristine, interferon alpha, or combined chemotherapy. Surgical excision is required for refractory cases. This is the first case report of a KHE of the pancreas failing medical therapy and requiring surgical management. Although a very rare tumor, KHE should be considered in the differential diagnosis of obstructive jaundice in children.

150 Non-operative treatment in a case of traumatic pancreatic transection with pseudocyst formation. V. Badalyan, M. Khan, Gastroenterology, Hepatology, and Nutrition, Childrens National Medical Center, Washington, District of Columbia, UNITED STATES. An 8 year old healthy girl suffered non-penetrating bicycle handlebar abdominal injury. Within minutes, she had several episodes of non-bloody and non-bilious emesis, was taken to a local emergency room, and an abdominal CT scan revealed pancreatic dissection. She was admitted to our pediatric hospital and was kept NPO receiving IV fluids, and ultimately maintained with total parenteral nutrition (TPN). Serum lipase levels trended down from 3191 to 1743 U/L (normal: 146-199 U/L) within the first 3 days after trauma. MRCP revealed pancreatic inflammation with complete transection, interrupted pancreatic duct, and adjacent 7 x 7 x 4.5 cm fluid collection. Surgical interventions, including ERCP, were offered, but family and patient elected a conservative non-operative management. Naso-jejunal (NJ) elemental formula (Elecare Jr®, Abbott) tube feeds were initiated and the patient was discharged home receiving continuous NJ feeds, nineteen days after trauma. The serum lipase level at the time of the discharge was 2089 U/L. Twelve days later, the patient was re-admitted with emesis and abdominal pain as the feeding tube migratedinto the stomach. Lipase level upon readmission was 6695 U/L. A repeat MRCP showed pancreatic inflammation and pseudocyst measuring 5 x 6 x 4.7 cm. The patient was again made NPO and discharged home 9 days after the admission, receiving only TPN via a peripherally inserted central catheter. After five weeks NPO with TPN (9 weeks from initial injury), the lipase level declined to 297 U/L and an abdominal ultrasound showed a much smaller pseudocyst, measuring 1.9 cm x 2.2 cm. Based on this, her diet was gradually advanced over 4 weeks to 20 ounces of water and reduced-portion, fat free meals, while receiving TPN. She experienced no symptoms of abdominal pain, vomiting, hypoglycemia, or diarrhea, and her lipase continued to improve to a nadir of 89 U/L. Followup abdominal ultrasound then showed a reduced pseudocyst measuring 1.6 x 1.7 cm. She successfully weaned from TPN. A final abdominal ultrasound 6 months after the initial trauma showed complete resolution of the pancreatic pseudocyst. The incidence of pancreatic transection in children is rare, and occurs in approximately 0.5% cases of blunt abdominal trauma. Pancreatic transection is often complicated by post-traumatic pancreatitis, pseudocyst, intra-abdominal abscess, and/or fistula. The management of transection and its complications is controversial and may include surgery or conservative therapy with prolonged bowel rest and TPN. Our case demonstrates a successful non-operative management of pancreatic transection with major pancreatic ductal interruption and pseudocyst with complete recovery of the pancreatic function.

Nutrition/Nutrition Support 157 A Novel Case of Potential Pediatric Eosinophilic Enteritis. K.M. Barnhill, P.L. Ramirez, D. Mosher, R. Flores, L. Hewitson, The Johnson Center for Child Health and Development, Austin, Texas, UNITED STATES. Background: Eosinophilic Enteritis is a disorder that causes eosinophils to build up in the blood and gastrointestinal tract. When this occurs, symptoms can include inflammation, ulcers, intolerance to some foods, and the break down of tissue. It is an emerging diagnosis, and further diagnostic testing, evaluation, and treatment is needed. Case description: A 10-year-old female with a medical history significant for gastrointestinal pain, decreased growth trajectory, reduced appetite, and nonspecific chronic inflammation per endoscopy/colonoscopy presented for care. The family reported ongoing gastrointestinal complaints and concerns since the age of 3 years. No family history of gastrointestinal inflammatory disease or autoimmune disease was reported and the family reported no other autoimmune concerns. Two prior endoscopy and colonscopy procedures over the previous 5 years evidenced non-specific inflammation. Prior evaluation one year earlier was positive for GERD, gastric eosinophilic infiltration (less than 20HPF), and chronic lymphocytic inflammation. Current evaluation by endoscopy/colonscopy revealed resolution of GERD and the presence of 2 aphthous ulcers in the jejunum. Medical management of the initial diagnosis included various trials of pentasa, sulfasalazine, methotrexate, 6-MP, and prednisone. Despite this treatment, patient continued to report gastrointestinal pain and growth failure. Family reported significant food refusal as well as pain while eating. The patient’s health continued to decline. After the endoscopy/colonoscopy procedure at presentation, a trial of TPN was initiated. Presenting with weight of 53.5 pounds and BMI of 12.3 at initial evaluation and treatment initiation, the patient gained 14 pounds with 12 weeks of TPN and the addition of a tapered elemental diet intervention. Gastrointestinal pain completely resolved. Weight gain and pain dissolution were maintained after treatment when foods were slowly reintroduced. Discussion: This case of potential pediatric eosinophilic enteritis illustrates the benefit of utilizing TPN as part of the management of a patient presenting with a potential eosinophilic disorder. Early institution of this intervention could reduce ongoing patient pain, failure to thrive, and hospitalization. Further research on this topic is recommended.

158 Severe Infantile Vitamin B12 Deficiency Presenting with Pancytopenia and Lethargy. T.A. Altepeter, J.L. Powell, S.E. Shaffer, Pediatric Gastroenterology, Nemours AI Dupont Hospital for Children, Wilmington, Delaware, UNITED STATES. A 7 month old infant presented with 2 weeks of progressive lethargy and decreased oral intake. She was exclusively breast fed, and had been growing steadily between the 30-40% for weight and length. Physical examination was notable for lethargy, pallor, and decreased tone in the lower extremities. Labs were notable for neutropenia (ANC 450/UL) and severe macrocytic anemia (Hgb 6 g/dL, MCV 101 fl). Electrolytes included Na 139mmol/L, K 5.1mmol/L, Cl 108mmol/L, CO16mmol/L, BUN 9mg/dL, Cr 0.1mg/dL. AST was elevated at 124 U/L , ALT normal at 54U/L. A bone marrow biopsy demonstrated left shifted myelopoesis, normal erythropoeisis, and increased B cell precursors. Red cell precursors had abnormal morphology, but there were no blasts. The cause of macrocytosis was determined to be severe B12 deficiency (B12<70ng/L). RBC folate level was normal. The patient’s mother followed a general unrestricted diet, and received prenatal care throughout her pregnancy. Her blood indices were normal (hemoglobin of 12.3g/dL with MCV of 92 fl). However, further testing revealed an undetectable maternal B12 level, and she was ultimately diagnosed with pernicious anemia. Breast milk B12 level was not available. The patient was started on parenteral B12, and her level of alertness and oral intake improved within 48hours of initiation of treatment. Discussion: Severe B12 deficiency of infancy is rare in developed nations. The major risk factor is maternal vegan diet. Other less common causes may include maternal pernicious anemia, congenital intrinsic factor deficiency, malabsorption due to pancreatic insufficiency or short bowel syndrome, and congenital selective B12 intestinal malabsorption. Manifestations of B12 deficiency are broad, ranging from asymptomatic macrocytosis to severe neurologic problems including developmental regression, paresthesias, peripheral neuropathy, and posterior column demyelination. In this patient, maternal B12 deficiency was not suspected, due to normal blood indices and lack of neurologic symptoms. Folic acid supplementation in mother’s prenatal vitamins presumably led to normalization of MCV and prevention of anemia in the mother. However, maternal B12 deficiency may lead to production of breast milk deficient in vitamin B12. Many commonly used infant multivitamin drops do not contain vitamin B12. Missed B12 deficiency in an infant can result in permanent neurologic damage. However, with early recognition, neurologic symptoms may be completely reversible. In our patient, we noted rapid normalization of the mental status and resolution of hypotonia with replacement of B12.

159 Specific Carbohydrate Diet in Eleven Pediatric Patients with Crohn’s Disease. J.C. Burgis, K. Nguyen, K. Park, K. Cox, Pediatric GI, Stanford University, Palo Alto, California, UNITED STATES. Traditional treatment for Crohn’s disease (CD) involves medications which can cause many side effects. Enteral nutrition is effective for both induction and maintenance therapy for CD but adherence to a formula-based diet can be challenging. Proposed mechanisms of how enteral nutrition impacts disease activity include reduction in antigenic load, altered inflammatory cytokine profiles and modifications in intestinal microbiota.[1,2,3] We report our experience in 11 patients with CD (3 with only small bowel disease, 7 with more extensive disease involving colon) who with their original diagnosis or with a flare chose to follow the specific carbohydrate diet (SCD) for a minimum of 4 months. The SCD excludes all grains, gluten and lactose. It is restricted to simple carbohydrates found in fruit, honey, and vegetables as well as proteins from unprocessed meats, fish and lactose free dairy. Two patients used the SCD alone for both induction and maintenance therapy. Two patients used the SCD as primary therapy but were taking antibiotics (Vancomycin for Cdiff infection and Ciprofloxacin for perirectal abscess) during half of their follow-up period. Three patients started the SCD with a short steroid taper for 4-6 weeks then SCD only for maintenance. Four patients already on mercaptopurine started the SCD with the same short steroid taper. Table 1 shows mean change in BMI and laboratory values. Pre values indicate time of diagnosis and post values are at between 4-10 months after start of SCD. All 11 patients were symptom free by approximately one month and had stable or improved growth and normalization of laboratory values while on the SCD. Responses demonstrate that an effective dietary intervention may allow for medication-free remission. Another semi-vegetarian diet with high intake of fruits and vegetables, rare meat, rare bread and no sweets for adult CD patients showed similar significant prevention with longer time to relapse.[4] We are currently conducting a prospective study to evaluate potential mechanisms of the SCD by analyzing cytokine profiles and stool microbiota. 1. Gassull MA, Mañé J, Pedrosa E, Cabre E. Macronutrients and bioactive molecules: is there a specific role in the management of inflammatory bowel disease? JPEN J Parenter Enteral Nutr. 2005;29(4 Suppl):S179-182; discussion S182-183, S184- 188. 2. Lionetti P, Callegari ML, Ferrari S, et al. Enteral nutrition and microflora in pediatric Crohn’s disease. JPEN J Parenter Enteral Nutr. 2005;29(4 Suppl):S173-175; discussion S175-178, S184-188. 3. Fell JM, Paintin M, Arnaud- Battandier F, et al. Mucosal healing and a fall in mucosal pro-inflammatory cytokine mRNA induced by a specific oral polymeric diet in paediatric Crohn’s disease. Aliment. Pharmacol. Ther. 2000;14(3):281-289. 4. Chiba M, Abe T, Tsuda H, et al. Lifestyle-related disease in Crohn’s disease: relapse prevention by a semi-vegetarian diet. World J. Gastroenterol. 2010;16(20):2484-2495.

160 Cultural Influences on Infant Feeding Practices. S.B. Oliveira, J. Schwab, H. Tanuos, I. Monteiro, Pediatrics, UMDNJ-New Jersey Medical School, Newark, New Jersey, UNITED STATES. Breastfeeding is shown to have beneficial effects on common diseases like otitis media, childhood asthma, behavioral problems and brain development. The breastfeeding rates in USA are suboptimal and may have significant impact on childhood health. Inadequate transition from breast milk or infant formula to solid foods in the first months of life may also add to the problems, despite counseling from the pediatricians. Cultural background is a factor that impacts infant feeding practices. Parents coming from countries with higher breast feeding rates tend to breastfeed (BF) for a longer period of time, but this rate decreases based on how long the parents have been living in USA. A reason is the perception that if the Women Infant Child (WIC) program provides free formula, this may be a better alternative then breastfeeding. Other reasons may be the shorter maternity leave or the lack of encouragement of breastfeeding in USA. Feeding practices that can negatively influence childhood health can substantially increase the costs of health care related to care of preventable diseases and improving the health and quality of life of children will eventually lead to decrease in health care costs. The aim of this pilot study was to determine the effect of cultural influences on infant feeding practices and its impact on the infant health in an inner city in USA. Methods: We surveyed parents of 12-15 month old children, attending the Pediatric Continuity Care Practice at our Institution, about infant feeding practices and then retrospectively reviewed their charts for sick visits in the first year of life. Results: A total of 42 surveys were completed. 95% of the respondents were mothers. 49% identified themselves as African American (AA) and 41% as Hispanic. 40% of the AA children had at least 1 immigrant parent compared to 82% among the Hispanics. 75% were ever BF and of these 30% were BF for < than 1 month (m) and 33% for ¬> 6m. 14% of AA BF for ¬>6m compared to 50% Hispanics. Probability that mother will BF for ¬>6m was greater among Hispanics than AA p=0.05 (Fisher’s exact test). Families that had at least one immigrant parent were more likely to BF than when both parents were USA-born, p=0.09 (Fisher’s exact test). More immigrant families breastfed for >6m as compared to US-born (40% vs. 11%) but was not statistically significant p=0.13 (Fisher’s exact test). Time from immigration did not predict ever- breastfeeding or length of breastfeeding. No one said they did not BF because WIC provided free formula. None of those who never BF said they would BF if WIC did not provide free formula. Of those who ever BF, only 34% said they would BF longer if WIC did not provide free formula. AA were more likely than Hispanics to have visits for infectious diseases, mostly viral (70% vs. 35%) p=0.04 (Fisher’s exact test). Visits for infectious diseases or for allergic conditions (asthma, eczema) did not correlate with breastfeeding. Length of breastfeeding also did not predict visits for either condition in this small sample. Conclusions: In our sample immigrant Hispanic mothers were more likely to breast feed and for a longer period. Provision of free formula by WIC did not alter the decision to ever breast feed.

Poster Session II

Esophagus/Stomach 176 Eosinophilic Esophagitis in a Boy with Juvenile Polyposis Syndrome: Is there a link?. S. Lapsia, J. Morganstern, Pediatric Gastroenterology & Nutrition, Stony Brook Long Island Children’s Hospital, Stony Brook, New York, UNITED STATES. Colonic polyps are a common cause of painless rectal bleeding in childhood, with the vast majority being of the juvenile type. The exact etiology behind polyp formation is unknown; both genetics and lifestyle factors have been thought to play a role. Allergic disease is generally not considered to be associated with polyps or polyposis syndromes. To our knowledge, the following case is the first reported of a child with juvenile polyposis syndrome, also found to have eosinophilic esophagitis (EoE). A 15 year old male presented to us with painless rectal bleeding. Colonoscopic biopsies revealed multiple juvenile polyps. SMAD4/BMPR1A mutations were negative. Microarray testing showed a deletion within STK10 (serine/threonine kinase 10), a gene closely related to STK11, associated with Peutz-Jeghers syndrome. An upper endoscopy done to rule out upper GI polyps showed linear furrows and white plaques in the esophagus. Biopsies revealed >50 eosinophils in both the mid- and distal esophagus, confirming a diagnosis of EoE. On further questioning, a history of dysphagia with ingestion of milk products was elicited from the patient. There is growing evidence of a link between allergic diseases and polyps. A recent report from Canada showed squamous hyperplastic-inflammatory polyps in the esophagus of a patient with long-standing untreated EoE. Young males are more likely to have polyps than older females. A similar demographic is found in those diagnosed with EoE. A recent study showed that patients with juvenile polyps are more likely to have a personal and family history of allergy compared to controls. Protein tyrosine kinases such as STK10 and STK11 play an integral role in the activation of inflammatory cells, smooth muscle, and epithelial cells. Inhibitors of tyrosine kinase have been investigated to prevent airway hyperresponsiveness and eosinophilic infiltration. This case report suggests that there may be a link between patients with juvenile polyposis and eosinophilic esophagitis.

177 Asymptomatic Pneumoperitoneum Due to an Unusual Cause. B. Maksimak, M. Maksimak, Pediatric Gastroenterology and Nutrition, Janet Weis Children's Hospital - Geisinger Clinic, Danville, Pennsylvania, UNITED STATESA. Kennedy, Pediatric Surgery, Janet Weis Children's Hospital - Geisinger Clinic, Danville, Pennsylvania, UNITED STATES. Our patient, a 16 year old teenage girl, presented to her primary care provider with a history of acute onset of left sided abdominal pain in gym class that lasted 1 day. An abdominal ultrasound was scheduled and eventually performed 3 weeks later while the patient was having no symptoms. Because of concerning ultrasound findings, an abdominal CT scan was immediately performed showing significant free air in the abdomen, ascites and a possible gastric bezoar. At this time she was urgently transferred to our ER for further management. Upon arrival, she was asymptomatic, but had a mild tachycardia. An abdominal obstruction series revealed a large volume of abdominal free air of unknown etiology. Even with these concerning findings seen on imaging she remained asymptomatic, but was admitted to the Pediatric Surgery service for observation. Because she remained afebrile overnight and denied any abdominal pain, nausea, or shortness of breath, she was advanced to a regular diet, which was well tolerated. She was subsequently discharged after overnight observation. During a scheduled follow-up in the Pediatric Surgery clinic 3 weeks later, an abdominal x-ray still revealed a persistent pneumoperitoneum. At this time a request was placed with Pediatric Gastroenterology for an elective upper endoscopy. Prior to the endoscopy the patient denied any ingestion or abdominal symptoms on repeated questioning even a few minutes prior to the procedure. Physical exam revealed normal length hair with no obvious , but there was obvious tympany found on abdominal percussion. The endoscopy was completed without complication displaying a moderately large gastric trichobezoar extending into the duodenum. No attempts were made to remove the bezoar because of the risk of perforation. However on questioning immediately following the endoscopy while recovering from the sedation, the patient admitted to hair ingestion up until approximately 1 year ago. She displayed an extraordinary knowledge of the issue of trichobezoars and said that she read extensively on the subject. An elective exploratory laparotomy was performed the following week revealing free air, ascites, but no perforation was found. The gastric bezoar with Rapunzel syndrome was removed surgically via a gastrotomy. The appendix was also removed. She recovered well from the surgery and was discharged home without any issues or complications. Follow-up abdominal films 2 weeks after surgery disclosed complete resolution of the pneumoperitoneum. The poster will include a timeline of clinical presentation, the various remarkable x-ray studies, endoscopy pictures, and surgery photographs. A short discussion of pneumoperitoneum will also be presented.

178 Importance of pre-operative contrast assessment of the esophageal lumen in children with EoE and recurrent symptoms. C. Menard-Katcher, R. Kramer, G. Furuta, Section of Pediatric Gastroenterology, Hepatology and Nutrition, Gastrointestinal Eosinophilic Diseases Program, Children’s Hospital Colorado, Aurora, Colorado, UNITED STATESC. Menard-Katcher, R. Kramer, G. Furuta, Department of Pediatrics, University of Colorado School of Medicine, Aurora, Colorado, UNITED STATESL. Fenton, M. Swerdlow, Department of Radiology, University of Colorado School of Medicine, Aurora, Colorado, UNITED STATES. Introduction: Radiologic contrast studies are the best tool to assess esophageal strictures. The exact incidence of eosinophilic esophagitis (EoE) associated esophageal narrowing in children is unknown. Specific symptom complexes associated with esophageal strictures and narrowing are non-specific and include feeding dysfunction, dysphagia and food impaction. Because of the commonality of these symptoms, children with EoE may not undergo full assessment for esophageal strictures. We present two cases that emphasize the importance of obtaining radiologic contrast studies in patients with EoE prior to endoscopic assessment. Case 1: A 17 year old female with EoE presented for evaluation of dysphagia. Past evaluations included 11 esophagogastroduodenoscopies (EGD), the most recent of which was 6 months before this consultation. No dilation or radiographic assessment of the esophagus had been performed previously. At the time of her consultation, an upper gastrointestinal contrast series (UGI) detected proximal esophageal narrowing. During subsequent EGD, no discrete stricture was identified. Esophageal dilation was performed using Maloney dilators, starting at 30 French and proceeding up to 50 French. Following dilation, an esophageal rent was identified in the proximal and mid portion of the esophagus. Case 2: A 6 year old female with EoE, dysphagia and recurrent history of food impactions presented for evaluation. Past evaluations included 4 EGDs, two of which were performed for food impaction removal, which did not detect esophageal stricture or narrowing. The most recent mucosal biopsy revealed improvement in eosinophilia. An UGI was performed for persistent symptoms and detected circumferential narrowing of the middle esophagus with proximal dilatation. A Boston Scientific CRE TTS balloon dilator was used to perform esophageal dilation from 12 to 18 cm under fluoroscopic guidance. Following dilation, a rent in the mucosa was identified in the mid portion of the esophagus. Discussion: It is suggested that EGD is not sensitive in detecting the strictures and esophagheal narrowing observed in EoE. These cases emphasize the importance of pre-operative contrast evaluation of the esophageal lumen in patients with EoE and recurrent symptoms.

179 Upper Gastrointestinal Bleeding (UGIB) in a Full Term Neonate. A.N. Maxwell, S.B. Oliveira, M.R. Jean, I. Monteiro, Pediatrics, UMDNJ-New Jersey Medical School, Newark, New Jersey, UNITED STATES. UGIB is uncommon in healthy full-term neonates, although seen frequently in critically ill preterms. In some, there is an obvious cause whereas the etiology remains unclear in others. We report a case of a healthy, full-term neonate who presented with UGIB on the first day of life with favorable outcome after conservative management. 37-week male was born via uneventful, scheduled C-section. Vitamin K was given after birth. Family history was negative for bleeding disorders. Breastfeeding was started after birth and at 18 hours of life, blood was noticed coming out of his mouth. A nasogastric tube was placed and yielded fresh blood. Apt test was negative. Physical exam was unremarkable with stable vital signs. He was given intravenous fluids, gastric lavages, made NPO and started on a proton pump inhibitor (PPI). Abdominal X-ray showed soft tissue density occupying the entire gastric lumen suggestive of intraluminal blood. Initial labs showed Hemoglobin (Hb) 14.9, platelets 314,000, PT 18.5 and PTT 31.3. He passed several bloody stools. Repeat Hb 6 hours later decreased to 7.9. He received PRBCs twice, vitamin K, FFP, cryoprecipitate and Factor VII. The bleeding ameliorated after 24 hours. Initial Esophagogastroduodenoscopy (EGD) performed on day of life (DOL) 3 was significant for superficial gastric ulcers, numerous blood clots in the body of the stomach and normal esophageal/duodenal mucosa. Biopsy revealed acute erosive gastritis, no H. pylori was seen. Repeat EGD on DOL 10 was normal. Oral feeds were restarted on DOL 14 and no further bleeding occurred. Discussion: It is known that perinatal stress factors can lead to stress induced gastritis. However, it remains unclear why healthy full-term neonates would develop severe bleeding in the first days of life. It has been suggested that a hyperpepsinogenic state may represent a significant risk factor. In our case, pepsinogen levels were not measured. The role of H. pylori in UGIB at this age is not known. Conservative management includes fluid resuscitation, blood products as needed and the use of an anti-secretory agent. Conclusion: For neonates with UGIB, the long-term prognosis is favorable with no recurrence of symptoms.

180 A pretty penny, a potential gastric ulcer.. L.E. Mullinax, Department of Medical Education, Miami Children's Hospital, Miami, Florida, UNITED STATESR. Arboleda, E. Hernandez, J. Reeves-Garcia, W. Muinos, R. Gomara, Department of Pediatric Gastroenterology, Miami Children's Hospital, Miami, Florida, UNITED STATES. Introduction: The composition of the penny was changed in 1982 to a zinc core (97.5% volume) with a thin copper coating (2.5% volume). While the post-1982 penny is seen well on radiographs, zinc is reactive with gastric acid and the increased zinc content may cause local and fairly rapid corrosion and ulceration in the stomach. Objective: To recognize the importance and urgency of the removal of ingested pennies via esophagogastroduodenoscopy (EGD) due to the potential corrosive effects of pennies minted after 1982. Clinical Presentation: We report 2 patients that developed rapid gastritis from post-1982 pennies that were lodged in their stomachs for < 1 week in comparison to a patient that developed mild gastritis from a pre- 1982 penny that was lodged in her stomach for > 2 weeks. Patient A, a 2 year old female with emesis and abdominal pain after ingestion of a 2012 penny 4 days prior, was found to have gastritis on EGD. Patient B, a 6 year old male with emesis and abdominal pain after ingestion of a 1988 penny 6 days prior, was found to have gastritis and a small linear ulcer on EGD. Patient C, a 3 year old female was found to have mild gastritis on EGD once abdominal pain developed after ingestion of a 1968 penny 2 weeks prior. Conclusion: Ingested coins are often observed periodically through radiographic images in asymptomatic patients and are likely to pass through the GI tract due to their small size. However, ingested post-1982 pennies propose a more serious risk for ulceration than pre-1982 pennies due to their higher concentration of zinc. This problem may be seen with increasing frequency due to the increasing amount of circulating post-1982 pennies. Thus awareness should be raised in the importance of identification of ingested coin type by modalities such as coin size (penny size = 19.05 mm) and abdominal X-ray (AP and Lateral views). Findings indicating an ingested penny should prompt urgency of coin removal, given the probability of variable corrosive capabilities.

181 Plummer-Vinson syndrome (esophageal web, severe iron deficiency anemia and dysphagia) associated with Helicobacter pylori infection in a 19 year old female.. L.E. Mullinax, H.E. Hagerott, L.I. Castillo, Department of Medical Education, Miami Children's Hospital, Miami, Florida, UNITED STATESJ. Reeves-Garcia, E. Hernandez, R. Arboleda, W. Muinos, R. Gomara, Department of Pediatric Gastroenterology, Miami Children's Hospital, Miami, Florida, UNITED STATES. Objective: To recognize the role of esophagogastroduodenoscopy (EGD) with biopsy in pediatric patients who present with refractory iron deficiency anemia (IDA) and dysphagia. When esophageal webs were found associated with dysphagia, the diagnosis of Plummer-Vinson syndrome was established. Gastric biopsies confirmed severe H.pylori infection, the cause of the IDA. Clinical presentation: We report a 19 year old female with 1 year history of severe IDA (hgb 3.6 gm/dL); treated with multiple transfusions and iron treatment as outpatient. Hematological workup was negative except for IDA. Due to dysphagia, she refused to take iron tablets and had an unintentional 10 lb weight loss in 6 months. On admission, vital signs were stable, physical exam was remarkable for low BMI, pallor, stomatitis, depapillated and . Work up for celiac disease, collagen vascular diseases and malignancy were negative. EGD was attempted but not completed due to inability to cannulate the esophagus with a regular endoscope. A second EGD was repeated with the assisted of an otorhinolaryngologist. Images of a web in the upper esophagus were taken but a regular pediatric endoscope could not be advanced beyond the web. Then a 5mm fiberoptic endoscope was advanced past the web into the distal esophagus, stomach and duodenum. Biopsies, video and images were taken of the lesions seen. EGD findings: 1. Webs in cervical esophagus, 2. Candida esophagitis in distal esophagus, 3. Diffuse cobblestone gastritis, no ulcers seen. Gastric biopsies and urease test of the tissue confirmed gastritis and presence of H.pylori. The patient was treated with a proton pump inhibitor, antibiotics for H.pylori gastritis, antifungal treatment for Candida esophagitis and blood transfusion with iron supplements for IDA. After treatment, the patient was lost to follow up. Conclusion: We present the findings of Plummer-Vinson syndrome (esophageal Webs, clinical signs of IDA and dysphagia) and a possible cause associated with H.pylori gastritis. To our knowledge no cases of Plummer-Vinson syndrome have been reported in the pediatric literature associated with or secondary to H.pylori gastritis as documented in our patient. EGD and biopsy findings were instrumental in diagnosing esophageal webs as well as H.pylori gastritis in our patient.

182 Squamous cell papilloma of the esophagus in children. G. Naguib, S. Blanchard, Pediatric gastroenterology, University of Maryland, Baltimore, Maryland, UNITED STATESW. Twaddell, Pathology, University of Maryland, Baltimore, Maryland, UNITED STATES. Squamous cell papilloma of the esophagus is a benign infrequent finding occurring in an estimated 0.01-0.04 percent of all upper gastrointestinal endoscopies. They usually are asymptomatic solitary lesions and are often detected as incidental findings on endoscopy. They are rarely linked with Human papilloma virus (HPV). Due to the rarity of the condition in children, we report two cases of squamous papillomatosis of the esophagus.The first patient was a 19 year old female who had endoscopy for reflux and abdominal pain. The second patient was a 15 year old female who had screening endoscopy for juvenile polyposis coli. Both patients had one small polypoid lesion in the mid esophagus and they were removed with cold biopsy forceps. An immunostain for HPV was negative on both patients. The etiology of squamous cell papilloma can be due to irritation of the mucosa with acid or trauma or due to HPV infection. HPV infection has been associated with an increased risk for in adults. However most of the papillomas in literature are negative for HPV stain and they have benign outcome. As pediatric gastroenterologists, we should recognize these rare lesions and remember to obtain immunostains for HPV so that we can guide our patients accordingly. There are no standard therapeutic or surveillance guidelines due to the scarcity of reported cases especially in pediatric patients.

183 Fighting Fire with Fire: Novel Technique for Endoscopic Removal of Ingested Neodymium Magnets In Pediatrics.. S. Nanton, J. Roskens, S. Matchan, Gastroenterolgy, Avera Childrens, Sioux Falls , South Dakota, UNITED STATESD. Strand, Avera McKennan Hospital, Sioux Falls , South Dakota, UNITED STATES. Ingested Neodymium magnets are up to ten times as powerful as traditional magnets and have emerged as a serious health hazard in children leading to bowel perforation, volvulus and death. We described a novel use of the force of Neodymium magnets in the endoscopic removal of multiple ingested magnets in a two year old child who presented with a one month history of recurrent non-bloody, non-bilious vomiting. Abdominal x-ray revealed bead like foreign bodies in stomach . EGD demonstrated multiple Neodymium magnets in the lesser curvature of the stomach and gastric antrum. It was noted that the walls of the lesser curvature and antrum were attached due to the attractive forces. Several magnets had eroded through the gastric wall creating gastric fistulae between the lesser curvature of the stomach and the gastric anthrum. A net retrieval device successfully removed some of the magnets; however, this device was unable to remove the embedded magnets. A multiprong forcep also failed to remove the embedded magnets. We therefore devised a novel technique to use the power of Neodymium magnets to remove the magnets that were embedded in the gastric wall. We used one of the magnets which was previously retrieved from the stomach and front loaded it onto the video gastroscope using a net retrieval device (Roth net). The front loaded endoscope was then advanced under direct visualization to the magnets which were embedded in the gastric wall. All of the embedded magnets were then attracted by the magnetic force of the front-loaded magnet and removed en bloc. The 25 neodymium magnets which had been ingested were thus removed using only three passes of the endoscope. The fistulae were then closed endoscopically via application of endo-clips. The child had an uneventful recovery and was discharged home the next day. Further studies are needed to determine the optimal methods for endoscopic removal ingested neodymium magnets in pediatric patients. Our method of front-loading a neodymium magnet to the video endoscope proved to be optimal in this patient.

184 Management of the Elusive H-type Tracheoesophageal Fistula: NOTES (Natural Orifice Transluminal Endoscopic Surgery) for the Future?. A.K. Pai, D. Thomas, Department of Gastroenterology and Nutrition, Children's Hospital Los Angeles, Los Angeles, California, UNITED STATES. Case: A 2-week old baby girl born to consanguineous parents at 39 weeks was evaluated for feeding difficulty after receiving antibiotics for a right upper lobe pulmonary infiltrate. She was found to cough and experienced a desaturation event while feeding. Although an initial Modified Barium Swallow Study (MBSS) revealed no abnormalities, the baby was subsequently diagnosed with an isolated (H-type) tracheoesophageal fistula on repeat MBSS and esophagram. She was positioned with head elevated and with a Replogle tube to low-intermittent suction until she underwent a successful operative repair via a cervical approach. Discussion: The incidence of all tracheoesophageal fistulae is about 1/3000 to 1/4000 live births, but the H-type TE fistulae are exceptionally rare, accounting for only 4% of this group. In patients with H-type TE fistulae, the classic triad of presenting symptoms is coughing/choking during feeding sessions, gaseous distension of the gastrointestinal tract, and pneumonitis from aspiration of gastric contents. Many patients with H-type TE fistulae are unrecognized until late childhood or even adulthood, presenting with chronic cough or recurrent pneumonias. This case reviews the challenges of making this diagnosis radiographically and highlights the importance of keeping this in our diagnostic arsenal to prevent sequelae such as chronic lung disease. Although the standard of treatment is open surgical correction, endoscopic modalities have also been attempted utilizing tissue adhesives, Nd-YAG lasers, sclerosants, and electrocautery. This is an area where further investigation is warranted as most case series feature a small patient sample, only have short-term follow-up, and report variable success rates. In recent years, NOTES has become an emerging field, whereby operative tools and a camera are delivered through the patient’s natural orifices to eliminate incisions mostly in adult patients. This clinical challenge of isolated TEF repair is a new frontier for research collaboration between pediatric surgeons and gastroenterologists to design hybrid procedures to minimize pain, infection, and recovery time.

185 Esophageal Stricture formation after Transesophageal Pacing. J. Panganiban, B. Barth, L. Chan, Pediatric Gastroeneterology, UTSW, Dallas, Texas, UNITED STATESI. Zeltser, Pediatric Cardiology and Electrophysiology, UTSW, Dallas, Texas, UNITED STATES. Background: A benign esophageal stricture is a narrowing or tightening of the esophagus. This can develop in infants as a primary constriction, secondary to a surgically repaired esophageal atresia, complication following esophageal surgery or as a result of chemical injury after caustic ingestion. Transesophageal pacing can be used to diagnose and treat arrhythmias in children and serve as a temporary lifesaving measure to reestablish circulatory integrity and flow. This procedure has been reported to cause mild erosive esophagitis and esophageal burns without stricture formation. Case: Patient is an ex 36 week old male with complex congenital heart disease consisting of Tetralogy of Fallot with pulmonary atresia and multiple aorta-pulmonary collaterals who was born with junctional ectopic tachycardia (JET). He required medical management with amiodarone via nasogastric tube (NGT) and transesophageal atrial pacing to maintain adequate cardiac output. A 5 French (Fr) temporary pacing electrode catheter (Tapcath 205, Cardiocommand) was inserted transnasally and advanced 12 cm in the esophagus, positioned behind the left atrium. He was paced at energy of 40mA, pulse width of 10 sec at a rate of 170bpm. Esophageal pacing was continued for 44 hrs until he was hemodynamically stable for surgical placement of temporary epicardial pacing wires. Patient stabilized and was tolerating enteral feeds via NGT. On day 29 of life, NGT was dislodged and could not be reinserted under fluoroscopy by interventional radiology. There appeared to be a blind-ending mid thoracic esophagus. Endoscopy was performed which revealed a 1mm mid esophageal stricture seen at 12 cm from the gum line. The stricture was dilated using a 4 Fr Soehendra biliary dilation catheter over a guide wire and a 5 Fr NGT was placed for medication and feeding. Patient underwent a total of 9 dilatations that occurred at a frequency of 1-2 weeks to the latest diameter of 8mm and a surgical gastrostomy tube was placed for feeding. Discussion: To our knowledge, this is the first case report of esophageal stricture formation after transesophageal pacing. The distal esophagus lies in direct contact with the atrium thus is a site used for pacing. Due to the electrical current delivered to pace the heart, this same current can cause esophageal damage as seen in animal studies and one case report (1, 2). Both articles describe esophageal injury but no stricture formation. In animal studies, pacing for 24 hrs at output of 20mA with a rate of 140bpm resulted in only mild esophagitis with no permanent changes (1). In one case report, a 13 year old patient developed severe esophageal burns with no stricture formation after pacing for 18 hours at an output of 20mA with a rate of 320bpm.(2) Conclusion: Our patient is the first reported case of severe esophageal mucosal injury and subsequent stricture formation after prolonged high voltage transesophageal pacing. References: 1. Green HW 3rd, Sanders RA, et al. Safety of transesophageal pacing for 24 hours in a canine model. Pacing Clin Electrophysiol 2009; 32(7):888-93. 2. Köhler H, Zink S, Scharf J, Koch A. Severe esophageal burn after Transesophageal pacing. Endoscopy. 2007; 39:E300.

Hepatobiiary/Transplant 209 Exertional Heat Stroke in a Young Athlete resulting in Acute Liver Failure. J. Kurowski, H. Lin, S. Mohammad, E.M. Alonso, Pediatric Gastroenterology, Hepatology, and Nutrition, Ann and Robert H. Lurie Children's Hospital of Chicago, Chicago, Illinois, UNITED STATES. Heat stroke is a well-described medical emergency that can result in multi-organ dysfunction including acute liver failure, neurologic dysfunction, rhabdomyolysis, and death. Heat stroke from exertion occurs in the setting of decreased dispersion of heat and decreased thermoregulation resulting in core body temperature ≥40.5°C. Young athletes are particularly at risk for heat stroke. We report a 13 year old male wrestler taking an over the counter supplement containing synephrine, caffeine, and salicin who presented with heat stroke and subsequently developed acute liver failure (ALF), rhabdomyolysis, and kidney failure. The patient collapsed during a rigorous day of exercise in an effort to lose weight and was found to have a temperature of 40.5°C. Initial management focused on airway protection, fluid resuscitation, and neuroprotective measures. Preliminary labs included sodium 150mEq/L, bicarbonate 15mEq/L, anion gap 27, blood urea nitrogen 35mg/dL, and creatinine 2.4mg/dL. Liver function was initially normal but severe liver injury with ALF was evident by day 3 (Table 1). The patient had neurologic dysfunction on presentation that was attributed to heat stroke and then later compounded by ALF and uremia. Other causes of ALF were excluded including acute acetaminophen toxicity, autoimmune hepatitis, viral hepatitis, Wilson’s Disease, and hemophagocytic lymphohistiocytosis. While awaiting liver transplantation (LT) and receiving supportive care which included hemodialysis, his encephalopathy and liver function improved and he was removed from the LT list on day 9. He went on to have full recovery of his neurologic, liver, and kidney function. Reported mortality rates are as high as 85% in patients with multi-organ dysfunction due to heat stroke. Only four patients undergoing LT in the setting of exertional heat stroke have been reported, three of whom died in the early post-transplant period. This case is unique in that it demonstrates spontaneous recovery of organ function in a teenager with heat stroke induced ALF where the post-transplant mortality has been historically high. Table 1: Laboratory values by hospital day n/a: not available

210 A Challenging Diagnosis of a Forme Fruste Choledochal Cyst complicated by Mirizzi Syndrome in a Pediatric Patient. J. Kurowski, J.R. Brown, Pediatric Gastroenterology, Hepatology, and Nutrition, Ann and Robert H. Lurie Children's Hospital of Chicago, Chicago, Illinois, UNITED STATESH. Melin-Aldana, Pathology, Ann and Robert H. Lurie Children's Hospital of Chicago, Chicago, Illinois, UNITED STATESC. Hunter, Pediatric Surgery, Ann and Robert H. Lurie Children's Hospital of Chicago, Chicago, Illinois, UNITED STATESS. Komanduri, Gastroenterology and Hepatology, Feinberg School of Medicine, Northwestern University, Chicago, Illinois, UNITED STATES. Choledochal cysts are rare malformations of the biliary tree and typically classified by dilatation of the intra- or extrahepatic ducts. A forme fruste choledochal cyst (FFCC) has been defined by a common bile duct (CBD) diameter less than 10mm and Mirizzi Syndrome is a compression of the extrahepatic bile duct by the adjacent lithiasic gallbladder or cystic duct. We report a pediatric patient with an FFCC complicated by Mirizzi Syndrome. The patient is a 3 year old female who presented with three months of colicky abdominal pain and anorexia followed by acute onset jaundice. Initial evaluation revealed significant elevation of direct bilirubin, transaminases, and lipase concerning for gallstone pancreatitis (Table 1). Coagulation studies were also elevated. Ultrasound demonstrated a dilated CBD that was confirmed by MRCP. ERCP was performed demonstrating a large 14mm stone and diffusely dilated extrahepatic bile duct to 15mm. A biliary sphincterotomy was performed and the stone was extracted successfully with a balloon catheter. A long common channel suggestive of anomalous pancreaticobiliary junction (APBJ) was noted. Surgery was consulted and she was scheduled for elective cholecystectomy pending normalization of her coagulation profile. Prior to surgery she presented with fever, pancreatitis and subsequent Klebsiella pneumoniae bactermia for which she completed seven days of parenteral ceftriaxone. Emergent ERCP was performed for presumed biliary obstruction causing cholangitis. The prior sphincterotomy appeared open; however, the CBD remained dilated measuring 9mm in diameter. The cystic duct was found to have multiple filling defects and was dilated at 15mm. Compression of the extrahepatic CBD by the cystic duct consistent with type I Mirizzi Syndrome was demonstrated and a 10Fr 10cm plastic biliary stent was deployed. An APBJ was again noted. After discussion with pediatric surgery, the persistent biliary dilatation despite stenting of the CBD in conjunction with APBJ raised a high index of suspicion for an FFCC. She then underwent laparoscopic converted to open excision of the FFCC with Roux-en-Y biliary reconstruction. Histologic evaluation confirmed the diagnosis of a choledochal cyst with erosion and fibrosis of the wall, which was lined with biliary epithelium in the excised cyst. The challenging aspect of this patient was distinguishing between isolated Mirizzi Syndrome in which a cholecystectomy would be sufficient versus a choledochal cyst requiring excision. To our knowledge this is the first pediatric patient diagnosed with forme fruste choledochal cyst complicated by Mirizzi Syndrome. Table 1: Laboratory values prior to and after Endoscopic Retrograde Cholangiopancreatography

211 Abernethy Malformation in a 12 year old with Dyspnea. G.D. Laroche, W. Karnsakul, S. Mitchell, H. Lau, Pediatric Gastroenterology and Nutrition, Johns Hopkins Hospital, Baltimore, Maryland, UNITED STATES. Abernethy malformation is a rare congenital vascular malformation resulting in an extrahepatic portosystemic shunt. While Type I is defined by complete absence of an extrahepatic portal vein (PV) with portal blood into the inferior vena cava (IVC), Type II is a partial shunt, characterized by a hypoplastic PV that minimally supplies the liver with a side-to-side anastomosis which empties into the IVC. Case: 12 year-old with multiple year history of dyspnea and exercise intolerance. A CXR showed mild diffuse increase in the interstitial pattern bilaterally. Large interatrial shunting was noted during echocardiogram. A chest CT demonstrated one small left lower lobe AVM. CT and MRI of abdomen/pelvis showed an 8x9 cm mass in the right hepatic lobe, along with a small PV with a large dilated inferior mesenteric vein communicating with the left common iliac vein (LCIV). A six-minute walk test had an initial heart rate 92 bpm with oxygen saturation 93% on room air, but final HR was 135 bpm with oxygen saturation 84%. Due to the abnormal vasculature noted on MRI she underwent portal venogram, which confirmed the direct communication between the portosystemic shunt and PV consistent with type II Abernethy malformation. A test occlusion of the left iliac collateral caused the portal venous pressures to rise 2 mmHg from baseline. Biopsy of liver nodule showed findings consistent with hyperplastic nodule/adenoma. The large shunt connecting the IMV and the LCIV was then ligated by surgery. No complications were noted during this surgery. Patient currently has less dyspnea.

212 Challenging Case of Extrahepatic Bile Duct Involvement in . H.C. Lin, K.M. Loomes, Division of Gastroenterology, Hepatology and Nutrition, The Children’s Hospital of Philadelphia, Philadelphia, Pennsylvania, UNITED STATESB. Zoll, College of Arts and Sciences, University of Pennsylvania, Philadelphia, Pennsylvania, UNITED STATESP. Russo, N.B. Spinner, Pathology and Laboratory Medicine, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania, UNITED STATESH.C. Lin, P. Russo, N.B. Spinner, K.M. Loomes, Pediatrics, Perelman School of Medicine at the University of Pennsylvania, Philadelphia, Pennsylvania, UNITED STATES. We report a unique case of Alagille syndrome (ALGS) characterized by extreme extrahepatic ductal hypoplasia that highlights the diagnostic challenges in cholestatic liver disease of infancy. A full-term boy with Tetralogy of Fallot, pulmonary atresia, and aortopulmonary collaterals presented with cholestasis at 2.5 months of age. Abdominal ultrasound demonstrated a normal gallbladder and liver without intra- or extra-hepatic biliary ductal dilation, and increased kidney echogenicity. The combination of cholestasis, congenital heart disease, and renal disease was suggestive of ALGS and genetic testing was sent. Liver biopsy showed ductal obstruction with portal expansion, ductular proliferation, and bile plugs, raising concern for biliary atresia (BA). Intraoperative cholangiogram showed no filling of the intrahepatic biliary tree and his biliary anatomy was most consistent with large duct obstruction although clinical suspicion for ALGS remained high. Following interdisciplinary discussion between surgical, medical, and pathology teams, a Kasai hepatoportoenterostomy was performed. Examination of the fibrous plate and biliary remnant showed an atretic extrahepatic biliary tree indistinguishable from BA. Post-Kasai, bilirubin levels continued to increase. Genetic testing revealed a missense mutation in JAG1: c.551G>A (p.R184H) confirming the diagnosis of ALGS with the combination of genetics, liver disease, congenital heart disease, posterior embryotoxon, and medical renal disease. With worsening synthetic liver function, he was listed for a liver transplant at 6 months. While awaiting transplant, the patient died at 7 months from complications related to heart and liver disease. This case reflects the spectrum of extrahepatic biliary involvement in ALGS and emphasizes the diagnostic and management challenges of biliary obstruction. Large duct obstruction raised concern for BA for which the success of therapy is correlated with timing of surgery, but poor outcomes have been reported with performing a Kasai in ALGS. Histopathology guides initial diagnostic work-up and intraoperative cholangiogram can help differentiate intra- and extrahepatic ductal involvement. However, there is significant overlap between BA and ALGS, and intraoperative cholangiogram is sometimes unable to distinguish these two entities. Extrahepatic biliary involvement in ALGS is variable and the presence of ductal hypoplasia presents surgical management challenges, specifically with performing a Kasai in ALGS. Accurate diagnosis is integral as there are differing management strategies and responses to treatment specifically with the performance of a Kasai, as this surgery did not improve the ultimate prognosis of liver disease. Trend of Liver Funciton Tests

213 Forme fruste choledochal cyst causing recurrent pancreatitis. S. Liu, GI Care For Kids, Atlanta, Georgia, UNITED STATES. A previously healthy 20-month-old male presented with acute onset of vomiting, diarrhea, and jaundice. Laboratory studies showed a direct hyperbilirubinemia, elevated transaminases, and elevated lipase. An abdominal ultrasound was significant for a mildly prominent common bile duct without choledocholithiasis and some gallbladder wall thickening. The patient’s laboratory studies normalized/improved with bowel rest, and the patient was discharged after a 3-day hospitalization. Five months later, the patient presented with acute onset of vomiting, jaundice, and clay-colored diarrhea. Again, his labwork was indicative of cholestasis, hepatitis, and pancreatitis. An ultrasound showed intrahepatic biliary dilatation and a dilated common bile duct to a maximum of 8 mm, but no choledocholithiasis. Magnetic resonance cholangiopancreatography (MRCP) demonstrated intrahepatic and extrahepatic biliary dilatation, dilatation of the pancreatic duct, and a focal area of decreased intensity in the distal common bile duct that possibly represented a stone but was thought to have the intensity of a soft tissue mass. A percutaneous transhepatic cholangiogram (PTC) visualized an obstruction at the distal common bile duct that could not be relieved. The patient was scheduled for a laparoscopic cholecystectomy with a common bile duct exploration by a pediatric surgeon, with concern of a malignancy causing a biliary obstruction. A transhepatic choledochoscopy was performed intraoperatively, but a mass could not be visualized in the region of the obstruction. However, a 3 cm firm mass was noted in the head of the pancreas. The pancreatic mass was biopsied with the results showing pancreatic fibrosis with pancreatitis, but no malignancy. Upon further review of the radiographic studies, it was noted that the patient had an anomalous pancreatobiliary junction (APBJ), with the pancreatic duct entering the common bile duct 13 mm proximal to where the common bile duct reached the ampulla of Vater, leading to a long “common channel.” The maximum diameter of his dilated extrahepatic bile duct was only 8 mm, so it was suspected that he had a forme fruste choledochal cyst (FFCC), which is associated with a high incidence of protein plugs or debris in the common channel. The patient underwent a cholecystectomy, resection of the common bile duct, and hepatico-duodenostomy. Pathology of the common bile duct was compatible with the suspected diagnosis of FFCC. The patient was discharged 4 days post-operatively, and he has had no further bouts of pancreatitis or cholestasis. This case report highlights the importance of including a forme fruste choledochal cyst in the differential diagnosis of a pediatric patient with recurrent pancreatitis and/or cholestasis. This sometimes unrecognized condition typically coexists with an anomalous pancreatobiliary junction, which is a finding that can sometimes be overlooked when evaluating radiographic studies of the pancreatobiliary system. Children with a forme fruste choledochal cyst may be at high risk for carcinogenesis, so the treatment is typically resection of the common bile duct followed by Roux-en-Y hepatico- jejunostomy or hepatico-duodenostomy.

214 Vertically Acquired HCV Infection Leading to Liver Transplant and Incidental Finding of Hepatocelluar Carcinoma in an Adolescent Female. M.S. Loayza, A. Delgado-Borrego, Pediatrics , University of Miami Miller School of Medicine , Miami, Florida, UNITED STATESM.S. Loayza, A. Delgado-Borrego, Jackson Memorial Hospital / Holtz Children's Hospital, Miami, Florida, UNITED STATES. Hepatitis C virus (HCV) infection continues to pose a major health concern. In the U.S., there are approximately 200,000 infected children (HepatitisC. Wkly Epidemiol Rec, 2000). Children tend to have different disease outcomes since 25 to 40% show spontaneous resolution by 24 months, 6 to 12% will have signs of resolution by early childhood, and the rest develop the chronic disease (Mack, L.C, et al. J Pediatr Gastroenterol Nutr 2012). Only a small percentage of chronically infected children will require liver transplantation. Hepatocellular Carcinoma (HCC) is uncommon in chronically infected children, and there have been fewer than 5 reported cases all of whom acquired HCV via blood transfusion. H.S. presented at 17 years of age to Holtz Children's Hospital complaining of generalized weakness, noticeable ascites, minimal jaundice, and abnormally elevated liver enzymes along with abnormal PT/INR. She was found to have positive HCV antibodies, and infection was confirmed by PCR (initial viral load was 9 million IU/ml, genotype IA). She underwent a liver biopsy which revealed cirrhosis. An upper endoscopy showed an esophageal varyx. Family history revealed that her mother died of complications of HCV infection when H.S. was 10 years old, and her father also had chronic HCV infection with symptoms of liver failure. H.S. was started on medical management to control her ascites and esophageal varyx. Interferon therapy was contraindicated given H.S.'s decompensated cirrhosis. Upon presentation, AFP was mildly elevated (194 ng/ml) and this elevation persisted, but CT and MRI scans did not reveal abnormal masses. 13 months following initial presentation, CT scan revealed a 2.3 x 2.3 cm mass with in the second segment of her liver. H.S. underwent liver transplantation on 10/25/12 with removal of tumor. Pathologic evaluation confirmed the diagnosis of HCC and her staging was AJJ PT1 N0 M n/a. During her post-transplant care, her immunosuppressant regimen has been limited as per protocol to prevent HCV recurrence. Her most recent viral load is less than 1 million IU/ml. INF therapy continues to be held. H.S.'s liver enzymes and other liver function test are slowly normalizing after her transplant. She continues to remain optimistic about her care and wellbeing. By presenting this case, we hope to demonstrate that otherwise healthy children may develop advanced liver disease including cirrhosis. Hepatocellular carcinoma as a result of HCV infection can occur in the first 20 years of life in an otherwise healthy individual. From a public health standpoint, this case demonstrates failure of the health care system to diagnose and treat a patient whose parents were chronically infected with HCV and whose mother had died of the disease. H.S.'s outcomes were likely preventable given the high rates of virologic response associated with HCV treatment. Lack of diagnosis and linkage to care in this case resulted in a significant outcome including a life threatening condition, development of cancer, and was associated with a high cost as the patient ultimately required liver transplantation and lifelong medical management.

215 Granulomatous Hepatitis in a 14 years old child due to Phenytoin. A.M. McClain, Y. Smadi, C. Bessett, D. Mehta, Center for Pediatric Digestive Health and Nutrition, Orlando Health, Orlando, Florida, UNITED STATES. Numerous diseases can lead to granulomatous hepatitis including infections, cancers, autoimmune disorders and drugs. We report a case of granulomatous hepatitis due to phenytoin use in a 14 year-old Haitian female. The patient was a previously healthy teenager who started having confusion, poor memory, headaches and generalized weakness. She was admitted to an outside facility and was found to have a cystic brain lesion and an abnormal EEG. Due to those findings, she underwent a lumbar puncture and was started empirically on treatment for cysticercosis and seizures with albendazole, dexamethasone, phenytoin and levetiracetam. ELISA testing for cysticercosis was negative and the patient had otherwise normal labs including liver function tests. The patient was stabilized and discharged home on the antiepileptic medications. She continued to have confusion, fatigue, with new onset of generalized abdominal pain and vomiting, but no rash or lymphadenopathy. On initial presentation to our hospital, her labs revealed abnormal liver function tests with AST of 164, ALT of 190, AP of 276, Total bilirubin (TB)of 2.1 and INR of 1.4. A liver ultrasound revealed thickened and dilated gallbladder along with mild hepatomegaly. An autoimmune workup and an extensive infectious disease workup was negative. Malignancy was ruled out with imaging and normal flow cytometry. Her liver function rapidly worsened throughout the hospital course with AST up to 614, ALT up to 367, AP up to 631, TB of 5.1, INR of 1.6 and ammonia of 99. These levels caused concern for hepatic failure with CNS involvement. A liver biopsy was then performed and pathology of the biopsy revealed granulomatous hepatitis [see photo]. Repeated 24-hour EEG revealed no seizure activity and antiepileptic drugs were stopped. With the possibility of potential fulminant course, she was treated with IV methylprednisolone that resulted in symptomatic improvement and normalization of liver function. Repeated brain MRI revealed unchanged cystic lesion. Phenytoin has been reported since the 1980s as a cause for granulomatous hepatitis in adult literature, but to our knowledge this is the first reported case in children. Most causes of granulomatous hepatitis in children have been reported as cat-scratch disease or idiopathic. Infectious, malignancy and autoimmune are common causes of granulomatous hepatitis, which were all ruled out in this patient. Our patient did not recently take any of the other drugs that commonly cause granulomatous hepatitis, such as: antibiotics, allopurinol, anti-hypertensive, aspirin, diazepam or antiarrhythmic. She was also not using any illicit substances. Although her case was complicated by preceding neurological findings, after the cessation of the phenytoin, in combination with appropriate steroid treatment, she has returned to her normal mental state and continued to improve.

216 Spontaneous resolution of Type 2 Abernethy malformation in an infant with Noonan Syndrome. P. Mohanty, V. Sood, M. Brown, Pediatric Gastroenterology, University of Rochester Medical Center, Rochester, New York, UNITED STATESY. Lee, Pediatric Surgery, University of Rochester Medical Center, Rochester, New York, UNITED STATES. Background- Congenital Extrahepatic Portosystemic Shunt (CEPS) or Abernethy malformation is a congenital anomaly in which splanchnic blood drains directly into the inferior vena cava. The diversion of splanchnic blood into the inferior vena cava may be complete in the form of an end-to side portocaval anastomosis with deficient intrahepatic portal veins (Abernethy type 1) or partial, via a side-to-side portocaval union (Abernethy type 2). Type 1 is usually associated with malformations such as heterotaxy with polyspenia and congenital heart defects. Associated anomalies are less frequent in Type 2. Only 40 cases of Type 1 and 22 cases of Type 2 CEPS have been described in literature. We present a case of an asymptomatic child with Noonan Syndrome and Type 2 Abernethy malformation that resolved spontaneously. Case presentation- A male infant born at 36 weeks of gestation was noted to have multiple congenital anomalies on the prenatal ultrasound (US) which included polyhydramnios, fetal macrosomia, thickened nuchal folds, large abdominal circumference, bilateral hydronephrosis, undescended testis, patent ductus arteriosus and a portosystemic shunt. Genetic testing revealed a mutation in the SOS1 gene, consistent with the diagnosis of Noonan’s syndrome. Postnatal abdominal Doppler US showed flow through the left portal vein, but reversed flow in the right portal vein. Follow up US at 12 days showed similar findings and presence of additional vessels which were not clearly identified. There were no signs of splenomegaly. At 5 weeks, CT showed patent portal veins and a large vein extending from the confluence of the portal vein to the IVC at the level of the aortic bifurcation, concerning for Type 2 Abernethy malformation. Follow up US at 3 months showed occlusion of the portosystemic shunt. He never had any signs of liver dysfunction. His transaminases were normal. He initially had elevated ammonia which normalized over time. Discussion- The patient had a prenatal diagnosis of portosystemic shunt that was later confirmed to be a Type 2 Abernethy malformation. Determining the type of shunt is important in formulating a treatment plan. In Type 1 Abernethy malformation, occlusion of the shun is not possible and liver transplantation may be the only option, whereas in Type 2, banding or embolization of the shunt can be done. Patients with Abernethy malformation may be asymptomatic or may develop mild liver dysfunction with transaminitis. This patient had normal transaminases, but had hyperbilirubinemia and hyperammonemia which have normalized. Since the risks of regenerative nodules and hepatic neoplasms have been reported in 40% of published cases, long term follow up is recommended even in case of asymptomatic shunts. No intervention is usually done before 2 years since the shunt rarely causes any issues before this age and may close spontaneously.

217 Acute Liver Failure Induced by Anakinra. S. Taylor, M. Martinez, S. Lobritto, J.M. Vittorio, N. Ovchinsky, Pediatrics, Columbia University, Morgan Stanley Chilren's Hospital Of New York-Presbeterian, New York, New York, UNITED STATESK. Fester, Pharmacy at NewYork-Presbyterian Hospital , Columbia University Medical Center, New York, New York, UNITED STATES. Anakinra, an Interleukin-1 (IL-1) receptor antagonist that prevents the binding of IL-1α and IL-1β, has become a more common therapy for Rheumatoid arthritis (RA) and other inflammatory processes. With the increased used of anakinra, more adverse events are being recognized. Previous cases of acute liver failure in the setting of initiation of anakinra have not been reported. Our case describes a 16-year-old male presenting with a history of significant weight loss, arthritis, and elevated inflammatory markers. On exam he was found to be tachypneic, tachycardic and had an audible pericardial rub. He was admitted for a pericardiocentesis in the setting of significant pericardial and pleural effusions and was ultimately diagnosed with Adult-onset Still’s Disease. Initial therapy consisted of naproxen and steroids. Anakinra was added shortly thereafter. Within five days of the initiation of anakinra our patient’s hepatic aminotransferases increased significantly (aspartate aminotransferase to 2271 U/L and alanine transaminase to 2002 U/L). This was followed by a rise in bilirubins (peak direct bilirubin level of 1.7 mg/dL), prothrombin time (peak value of 20.4 seconds), and international normalized ratio (INR) (peak value of 1.71). He developed altered mental status in a pattern consistent with stage I encephalopathy and was admitted to the intensive care unit for management of acute liver failure. Anakinra was held at this time due to the concern of drug induced liver injury. A thorough work-up was done to exclude alternative potential etiologies of liver injury including autoimmune hepatitis, viral etiologies, and macrophage activation syndrome. A liver biopsy revealed marked lobular necroinflammatory activity, apoptosis, and central perivenulitis suggestive of acute hepatitis in the setting of drug-induced liver injury. The patient’s medication regimen was augmented to steroids and cyclosporine, and his hepatic aminotransferases continued to improve with normalization a month later. The patient remains stable with normal liver function tests at one-year follow-up. This is the first reported case of acute liver failure in the setting of treatment with anakinra with rapid resolution of symptoms and laboratory parameters upon discontinuation of therapy. Anakinra’s use to treat various inflammatory conditions continues to expand, and clinicians should be aware of this potentially serious complication.

218 A child with unresectable biliary tract rhabdomyosarcoma: 48-month disease-free survival after liver transplantation. M. Paganelli, F. Alvarez, Gastroenterology, Hepatology and Nutrition, Ste-Justine Hospital, Montreal, Quebec, CANADAM. Beaunoyer, M. Lallier, Surgery, Ste-Justine Hospital, Montreal, Quebec, CANADA. Liver transplantation is an effective treatment for children with unresectable hepatoblastoma. In contrast, the results of liver transplantation for other malignant tumors remain poor because of vascular invasion and extrahepatic spread at presentation. We describe here a 2-year old boy with embryonal rhabdomyosarcoma of the biliary tract successfully treated by chemotherapy and liver transplantation. The child presented with obstructive jaundice at the age 20 months. At admission he had elevated total serum bilirubin, γ-glutamiltranspeptidase and alanine aminotransferase, with preserved liver function and normal α–fetoprotein. A mildly vascularized, non- calcified and partially cystic lesion of 72x80x82 mm, with irregular borders, was visualized in the left hepatic lobe (segments III, IV and VIII) at ultrasound, and confirmed at CT scan. Solid infiltration of the common bile duct and of both left and right hepatic ducts was suspected, with intrahepatic bile ducts dilatation in the right lobe. Liver biopsy suggested an embryonal rhabdomyosarcoma originating from the biliary tract. Extrahepatic spread of the tumor was excluded by CT scan, bone scintigraphy and bone marrow biopsy. The lesion was judged unresectable (TNM stage 3b and or PRETEXT III). An internal-external percutaneous transhepatic biliary drain was applied and neoadjuvant chemotherapy was started. After 4 cycles of VAC protocol (vincristine, D-actinomycin and cyclophosphamide) the mass volume had reduced to 42x50x45 mm, but was still unresectable (centrally located PRETEXT II). Liver transplantation with a reduced segment II/III graft was performed 4 months after diagnosis. Pathologic analysis of the explanted liver confirmed the diagnosis of embryonal rhabdomyosarcoma of the left lobe originated from the left hepatic bile duct, with multicystic proliferation of intrahepatic biliary tract, acute and chronic cholangitis of the left liver lobe and fibrosis of the choledocal and right hepatic duct walls. Hilar lymph nodes were described tumor-free (COG-STS group I). Antirejection treatment with tacrolimus, basiliximab and corticosteroids was administered. Post-operatory course was complicated by spontaneously resolving chylous ascites, arterial hypertension and CMV reactivation (successfully treated by valganciclovir). The patient underwent 6 cycles of adjuvant chemotherapy (vincristine, etoposide and ifofosfamide) and at 48 months post-transplantation he is alive and recurrence-free. To our knowledge, this is the first case of biliary tract rhabdomyosarcoma ever treated by liver transplantation. The 4-year disease-free survival of this patient proves that, when coupled with effective chemotherapy, transplantation has to be considered a potential treatment for unresectable forms of this rare malignant tumor.

219 Liver transplantation for acute infantile liver failure secondary to TRMU gene mutation. M. Paganelli, F. Alvarez, Gastroenterology, Hepatology and Nutrition, Ste-Justine Hospital, Montreal, Quebec, CANADAM. Beaunoyer, M. Lallier, Surgery, Ste-Justine Hospital, Montreal, Quebec, CANADA. TRMU nuclear gene encodes a mitochondrial-specific tRNA-modifying enzyme. TRMU mutation has been described in 18 infants with mitochondrial liver disease and normal mtDNA content. All patients presented with acute liver failure and lactic acidosis, 5 died while the survivors recovered and never had a recurrence. We describe here the first patient ever treated with liver transplantation (LT) for TRMU gene mutation. A 10- week old boy was admitted to the hospital for acute-onset jaundice, acholic stools, vomiting and irritability. Fasting hypoglycemia and lactic acidemia were discovered, and mtDNA content was normal. He was referred to our institution at 4 months of age because of acute liver failure. At admission he presented jaundice, portal hypertension, elevated liver enzymes, severe coagulopathy and lactic acidemia. Liver biopsy showed cirrhosis with bile duct proliferation and hepatocyte degeneration (giant cell transformation, steatotic change and mild intrahepatocellular cholestasis). TRMU mutation was confirmed and LT with an extended right graft was performed. Immunosuppression was induced with steroids, basiliximab and tacrolimus. Portal thrombosis occurred at day 1 post-transplant, rapidly entailing diffuse hepatic necrosis and severe liver failure. The post- operatory course was further complicated by hyperammonemia and lactic acidosis, anoxic brain lesions leading to neurologic deterioration and seizures, CMV reactivation, ascites with pleural effusion, biliary anastomotic stricture and mild tubulopathy. Moreover, diffuse cardiac calcifications were discovered 3 months post-transplant. Such a finding has never been described in patients with TRMU mutation, and was attributed to tacrolimus toxicity. The child was therefore switched to cyclosporine. Both liver function and neurological status of the patient gradually improved over several months, despite an extensive necrosis of the biliary tract. The child was retransplanted after 8 months with an extended right graft. The post-operative course was characterized by a bacterial peritonitis and the development of arterial hypertension. Acute rejection developed one month post- transplant and was treated with intravenous corticosteroids. Mycophenolate mofetil (MMF) was added because of the lack of response and the evidence of chronic rejection at liver biopsy. The child is presently alive and well on cyclosporine and MMF 5 months after LT. His liver enzymes almost normalized and hypertension improved along with steroid tapering. A significant neurodevelopmental progress was noted. secondary to cyclosporine is the only complain. TRMU protein requires cysteine for its activity. Endogenous synthesis of cysteine in the neonatal period is limited and increases after the first months of life. This explains the increased risk of liver failure in 2-4-month old infants with reduced TRMU protein, as well as the lack of recurrence in those surviving the neonatal episode. This case shows that LT could represent an effective rescue treatment for these infants. Nevertheless, alternative strategies aimed at assuring survival during the few critical months of cysteine deficiency should be evaluated.

220 Green Tea Extract: A Potential Cause of Acute Impending Liver Failure. S. Patel, S. Silver, D.L. Kearney , G. Phillips, B.A. Carter, Department of Pediatrics , Baylor College of Medicine, Houston, Texas, UNITED STATES. Green tea is consumed worldwide and is found in more than 100 over-the-counter, unregulated supplements. Although it has many touted benefits, there is increasing concern regarding its hepatotoxic potential. We report a case of an obese 16 year-old Hispanic male who presented with new onset jaundice. He denied abdominal pain, changes in his stool, fever, changes in mental status, alcohol consumption or recent travel. He was taking several dietary supplements as part of an unsupervised weight-loss plan: Applied Nutrition® Green Tea Fat Burner, Whey protein, GNC Mega Men® Sport, and Nopal® (Cactus), losing 56 pounds in the 2 months prior. Initial labs included: AST 2106 U/L, ALT 2984 U/L, GGT 78 U/L, conjugated bilirubin (CB) 12.9 MG/DL, albumin 4 G/DL and INR 1.3. During his hospitalization, peak INR and conjugated bilirubin (CB) were 1.5 and 17.5 MG/DL, respectively. Albumin and Factor 7 levels reached a nadir at 2.5 G/DL and 39% (normal 58-150%), respectively, which was concerning for impending liver failure. Serological markers of autoimmune hepatitis, infectious hepatitis A, B, and C, Wilson’s disease, alpha-1-antitrypsin deficiency, CMV, EBV and Adenovirus PCR were negative. Liver biopsy showed pan- lobular injury with portal and lobular mixed inflammatory cell infiltrates, acute and chronic inflammation that included scattered eosinophils and interface hepatitis, hepatocyte unrest, ballooning degeneration, and multifocal individual hepatocyte necrosis and cholestasis, consistent with published histological descriptions of green tea extract-induced hepatoxicity (Mazzanti G et al Eur J Clin Pharmacol 2009). It was not until hospital day 24 that liver indices began to improve and not until 5 weeks after discharge that liver function recovered. The Food and Drug Administration (FDA) was contacted and conducted an independent investigation on the green tea extract containing supplement. Yet, we highlight the general lack of oversight regarding the safety and efficacy of herbal supplements. It is imperative that physicians monitor the use of green tea, recognize that it may be contained in a variety of products, and be cognizant of its hepatotoxic potential.

221 Abernathy Type Ib Associated to Turner Syndrome in a Patient with Hypoglycemia and Dumping Syndrome. C. Pichardo, N. Cohen, Department of Medical Education, Miami Children's Hospital, Miami, Florida, UNITED STATESR. Arboleda, E. Hernandez, Department of Gastroenterology, Miami Children's Hospital, Miami, Florida, UNITED STATESA. Aldousany, Department of Cardiology, Miami Children's Hospital, Miami, Florida, UNITED STATESR. Restrepo, Department of Radiology, Miami Children's Hospital, Miami, Florida, UNITED STATESA. Diaz, Department of Endocrinology, Miami Children's Hospital, Miami, Florida, UNITED STATES. We present a case of a 5-year-old female with Turner’s syndrome, repaired transverse arch hypoplasia, severe coarctation and partially anomalous pulmonary venous return and Nissen fundoplication who presented with three years of hypoglycemia accompanied by postprandial hyperglycemia that started after three months of growth hormone therapy. During admission for hypoglycemia work up, the patient’s hepatic enzymes and ammonia level were found to be elevated ALT 51, AST 101, ammonia level 89. A liver ultrasound showed absent portal vein with hypertrophied hepatic artery and cavernous transformation of the portal vein. An MRA showed congenital extrahepatic porto-systemic shunt and congenital absence of the intrahepatic branches of the portal vein consistent with an Abernathy Type 1b malformation. A glucose tolerance test was done in which the patient’s glucose levels at 30 and 60 minutes were well over 200 mg/dL and at 90 minutes it dropped to 39 mg/dL thus suggesting the diagnosis of Dumping Syndrome secondary to the fundoplication and was discharged home on acarbose, hypoglycemia resolved. Congenital absence of the portal vein (CAPV) is a very rare congenital vascular malformation that carries risks of severe complications in children which include but are not limited to encephalopathy, pulmonary hypertension, hepatopulmonary syndrome, hypoglycemia, focal nodular hyperplasia and hepatocellular carcinoma. To the best of our knowledge the association of Turner syndrome and Abernathy malformation is infrequent.

222 Portal Vein Thrombosis (PVT) in Association with Celiac Disease and Crohn’s Disease: Two Pediatric Case Presentations. A. Pierog, S.S. Lusman, P. Kazlow, N. Ovchinsky, Columbia University, New York, New York, UNITED STATES. PVT in children without pre-existing liver disease is rare and the etiology is often unknown. It has been reported that inflammatory bowel disorders, such as celiac and Crohn’s diseases, may predispose patients to hypercoagulable states. There have been reports of hospitalized children and adults with PVTs in association with these conditions, but we report two cases of PVT in children that presented from home. Case 1: A 10-year-old female with splenomegaly and abdominal pain was referred to our center. Her evaluation revealed neutropenia, thrombocytopenia, and positive celiac titers. Her abdominal ultrasound demonstrated cavernous transformation of the portal vein. An upper endoscopy revealed esophageal varices and biopsies consistent with celiac disease, MARSH-3b. Her hypercoagulability evaluation revealed low proteins C and S. Case 2: A 9-year-old male with history of Crohn’s disease presented with persistent abdominal pain that was unresponsive to infliximab, as well as weight loss and fever. His spleen was palpable 3 cm below the costal margin. Abdominal ultrasound revealed cavernous transformation of the portal vein. Endoscopy revealed mild disease in the terminal ileum and descending colon and no varices. He initially had a positive anti-phospholipid antibody, but it was negative when repeated. To our knowledge, these are the only pediatric cases of PVT in ambulatory patients in potential association with two inflammatory conditions of the bowel: celiac disease and Crohn’s disease. The possible association could be attributed to nutritional deficiencies, as low levels of proteins C and S may be related to vitamin K deficiency. Protein losing enteropathy, often found in these conditions, can result in a loss of coagulation factors. Development of antiphospholipid and anticardiolipid antibodies may occur in autoimmune conditions and contribute to hypercoagulability. Intestinal inflammation surrounding the portal vein may also play a role in developing PVT. These cases demonstrate the importance of screening for and better awareness of hypercoagulable states contributing to PVT in patients with inflammatory conditions of the small bowel.

223 Portal Vein Thrombosis (PVT) in Association with Celiac Disease and Crohn’s Disease: Two Pediatric Case Presentations. A. Pierog, S.S. Lusman, P. Kazlow, N. Ovchinsky, Columbia University, New York, New York, UNITED STATES. PVTs in children without pre-existing liver disease is rare and the etiology is often unknown. It has been reported that inflammatory bowel disorders, such as celiac and Crohn’s diseases, can predispose patients to hypercoagulable states. There have been reports of hospitalized children and adults with PVTs in association with these conditions, but we report two cases of PVT in children that presented outpatient. Case 1: A 10-year-old female with thrombocytopenia, splenomegaly, and abdominal pain was referred to our center. She was found to have a large spleen and her evaluation revealed neutropenia, thrombocytopenia, and positive celiac serologies. Her abdominal ultrasound demonstrated cavernous transformation of the portal vein. An upper endoscopy revealed esophageal varices and biopsies consistent with celiac disease, MARSH-3b. Her hypercoagulable work-up revealed low protein C and S. Case 2: A 9-year-old male with history of Crohn’s disease presented with persistent abdominal pain that was unresponsive to infliximab, as well as weight loss and fever. His spleen was palpable 3 cm below the costal margin. Abdominal ultrasound revealed cavernous transformation of the portal vein. Endoscopy revealed persistence of his disease in the terminal ileum and descending colon and no varices. He initially had a positive anti-phospholipid antibody, but it was negative when repeated. To our knowledge, these are the only outpatient pediatric cases of PVT in potential association with two inflammatory conditions of the bowel: celiac disease and Crohn’s disease. The possible association could be attributed to nutritional deficiencies, as protein C and S can be low in vitamin K deficiency and PLE can also cause a loss of coagulation factors. Other autoimmune conditions such as the development of antiphospholipid and anticardiolipid antibodies may also contribute to hypercoagulability. Another possible mechanism for PVT may be intestinal inflammation surrounding the portal vein. These cases demonstrate the importance of screening for and better awareness of hypercoagulable states contributing to PVT in patients with inflammatory conditions of the small bowel.

224 Elevated gamma glutamyl transferase in an adolescent with systemic lupus erythematous. A.E. Pope, T. Miloh, Gastroenterology and Hepatology, Phoenix Children's Hospital, Phoenix, Arizona, UNITED STATESK. Ede, Rheumatology, Phoenix Children's Hospital, Phoenix, Arizona, UNITED STATES. A 17-year-old female presented with a four month history of myalgias, arthralgias and fevers for several weeks with pancytopenias (WBC 3.8 K/mL, hemoglobin 8.8 gm/dL, and PLT 137 K/mL), ALT 153 U/L, AST 630 U/L, alkaline phosphatase 326 U/L, GGT 1302 U/L, bilirubin 0.6 mg/dL, INR 1.1, creatine kinase 900 U/L, ESR 59 mm/hr, ANA 1:640, low C3 and C4, 17 mg/dL and 2.8 mg/dL, respectively, and proteinuria. Physical exam was significant for extremity weakness, diffuse pain with movement, and absence of jaundice. She was diagnosed with new-onset SLE, supported by positive ANA and dsDNA, and kidney biopsy revealed WHO Class II lupus nephritis. Following initiation of methylprednisone and IVIG, myalgias, cell lines, and creatine kinase normalized, ALT/AST improved to 109/180 U/L and GGT decreased to 859 U/L. MRCP was normal. A liver biopsy revealed preserved histoarchitecture, scattered necrotic hepatocytes, mild steatosis, no inflammation, mild ductular proliferation and minimal cholestasis. The biopsy was not consistent with autoimmune hepatitis. GGT remained elevated for two months and normalized to 19 U/L with improved control of SLE on prednisone, hydroxychloroquine, and azathioprine. Discussion: This is the first case report of a juvenile SLE patient presenting with elevated GGT levels in the absence of intrinsic hepatic pathology, with normalization following treatment of lupus. This association has been previously reported in adults specifically with SLE and not in other autoimmune disease, although GGT elevation did not correlate with SLE disease activity. Further studies are required to determine whether GGT can be used as a diagnostic tool or biomarker of disease activity in pediatric lupus.

225 Congenital Portosystemic Shunts: A Cause of Hepatopulmonary Syndrome. K. Queliza, L. Chan, UT Southwestern Medical Center, Dallas, Texas, UNITED STATES. Introduction: Congenital portosystemic shunts are presumed to be products of abnormal angiogenesis, particularly involving maldevelopment of the vitelline veins and ductus venosus. They are best classified as either intrahepatic or extrahepatic. The latter form is further subdivided into two types based on the presence or absence of the portal vein. Congenital portosystemic shunts are rare entities and can also cause hepatopulmonary syndrome (HPS), which classically involves liver dysfunction, arterial hypoxemia, and widespread pulmonary vascular dilations. Case: Our case describes an 11-year-old female who presented to the emergency room with exercise-induced chest pain and hypoxia. Significant chronicity was suggested by prominent digital clubbing and right ventricular hypertrophy on echocardiogram. Since echocardiogram failed to demonstrate intracardiac shunting, pulmonary source of ventilation-perfusion defect was considered. Further work-up, including ventilation/perfusion scan and bubble study, yielded evidence of pulmonary arteriovenous malformations. These findings, in combination with a history of infantile hemangioma and recurrent epistaxis, provoked consideration for hereditary hemorrhagic telangiectasia. In search for associated vascular anomalies, abdominal CT was only notable for cirrhotic changes, which then made hepatopulmonary syndrome (HPS) a concern. However, subsequent liver biopsy was not consistent with cirrhosis. Further evaluation for vascular abnormalities with abdominal MRA revealed multiple extrahepatic portosystemic shunts including splenorenal, splenic vein- hemiazygos vein and portocaval connections. Thereafter, the patient underwent exploratory laparotomy for shunt ligation and measurement of hepatic venous pressure gradient of 4mmHg. After the ligation, her exercise tolerance and oxygen requirements had improved. Repeat MRA of the abdomen one month later demonstrated absence of previously identified portosystemic collateral vessels and dilated coronary veins. Discussion: This is a report of congenital portosystemic shunts causing HPS. The pathogenesis of HPS caused by congenital portosystemic shunts remains to be clarified especially in the setting of preserved liver function and normal portal pressure. The patient required extensive work-up as confounding factors complicated reaching the root cause of her symptoms. This case highlights the complexity in which congenital portosystemic shunts can present and should be considered in the evaluation of HPS.

Intestinal/Colonic Disorders - IBD 246 Medical Management of Crohn’s Appendicitis.. V. Kasper, J. Shapiro, C. Cerezo, N. LeLeiko, Pediatric GI, Brown University, Providence, Rhode Island, UNITED STATESM. Wallach, Radiology, Brown University, Providence, Rhode Island, UNITED STATES. Appendicitis is a known complication of Crohn’s disease. The majority of literature on management of this entity is limited to surgical interventions with reports of long term post-surgical outcomes. There are no reports of medical management of Crohn’s appendicitis. We present a case of a 16 year old female with a 6 month history of non- bloody diarrhea without abdominal pain. Laboratory tests were significant for an elevated ESR 38 mm/h and CRP 19.85 mg/L. Endoscopy demonstrated friable and erythematous mucosa throughout the colon with similar patchy findings in the cecum and terminal ileum. Pathology was consistent with a diagnosis of Crohn’s Disease. Specifically, multiple granulomas were detected in the sigmoid colon in addition to pancolitis and ileitis. Immediately following endoscopy the patient was started on a 5-ASA compound. At two week follow up she reported complete resolution of her diarrhea and labs showed normalization of the CRP and improvement of the ESR. Three weeks following endoscopy, an MRE was obtained for routine surveillance of small bowel disease. In addition to disease in the proximal and sigmoid colon, significant enlargement of the appendix with surrounding inflammatory change and diffuse enhancement of the appendiceal wall was noted. The patient remained asymptomatic and abdominal examination was normal. After consultation with surgery, we elected to treat her with oral budesonide 9 mg daily given the disease distribution and the fact that she was otherwise clinically well. In addition, she was started on 75 mg of 6-mercaptopurine (6MP) one week later. Repeat MRE obtained after 5 weeks of budesonide and 4 weeks of 6MP showed complete resolution of the appendiceal inflammation. Five month follow up reveals that she remains in clinical remission on 6MP and 5-ASA. This case demonstrates that Crohn’s appendicitis may be safely and effectively managed medically in select cases.

247 Bilateral Central Retinal Vein Occlusion in a patient with Ulcerative Colitis and Antiphospholipid Antibody Syndrome. S. Lapsia, K. Usmani, G. Gathungu, Pediatric Gastroenterology and Nutrition, Stony Brook Long Island Children’s Hospital, Stony Brook, New York, UNITED STATESR. Meyer, Pediatric Hematology/Oncology, Stony Brook Long Island Children’s Hospital, Stony Brook, New York, UNITED STATES. Inflammatory bowel disease (IBD) is a recognized cause of hypercoagulability, and vascular thrombotic events are a significant cause of morbidity and mortality in patients with this disease. Coagulation abnormalities in IBD include elevated levels of Factor V and Factor VIII, or decreased concentrations of antithrombin III. However, patients with IBD have presented with deep vein thrombosis and pulmonary emboli, despite normal coagulation laboratory values. Common ocular manifestations of IBD include episcleritis, uveitis, and keratitis; however retinal vascular diseases such as unilateral central retinal vein occlusion (CRVO), branch retinal artery occlusion, and obliterative retinal arteritis have also been reported. Only 10% of patients with CRVO are younger than 40 years. Here were present a unique case of a young adult with IBD found to have bilateral CRVO. The patient is a 19 year male diagnosed 2 years prior with ulcerative colitis. He had moderate to severe disease activity despite treatment with steroids, aminosalicylates, immunomodulators, and anti-TNF therapy. He began to have blurry vision in his right eye for 2 weeks and was noted to have bilateral Grade III disc edema and widespread retinal hemorrhages. He was subsequently seen by a retina specialist and found to have bilateral CRVO with 3/200 vision in his right eye and remarkably 20/20 vision in his left eye. A hypercoagulable workup revealed that the patient had antiphospholipid antibody syndrome based on increased Anti-β2 glycoprotein I IgG, Anti-cardiolipin IgG, and Phosphotidylserine IgG levels. The patient was started on enoxaparin and low-dose aspirin due to presence of both IBD and antiphospholipid antibodies placing the patient at risk for further thrombotic events and to prevent further extension of the current thrombi. This case emphasizes two key considerations: a) Patients with IBD with complaints of blurry vision need immediate evaluation; b) Although having underlying IBD can place patients at risk for thrombotic events, a wider differential diagnosis should be considered.

248 Anti-tumor necrosis factor (TNF)-α therapy did not induce regulatory T lymphocytes in a child with severe Crohn's disease. C. Lin, G. Bultron, D. Thomas, Pediatric GI, Hepatology & Nutrition, Children Hospital Los Angeles, Los Angeles, California, UNITED STATESR. Parkman, Research Immunology, Children Hospital Los Angeles, Los Angeles, California, UNITED STATES. Background: Crohn's disease (CD) is a chronic inflammatory disorder of the gastrointestinal tract. The T lymphocytes of CD patients are associated with increased Th1/Th17 cytokines. The immune system down- regulates unwanted inflammatory responses through specialized subpopulations of T lymphocytes, namely T- regulatory lymphocytes (Treg; CD127-, CD25+). The Treg lymphocytes can be subdivided into resting Treg, activated Treg and activated conventional T lymphocytes. Only resting and activated Treg (rTreg and aTreg) lymphocytes have suppressive activity. Anti-TNF- α agents have been shown to be effective for the treatment of CD. The effect of anti-TNF-α on Treg (rTreg and aTreg) is not clear. Objective: To conduct an evaluation of a child with CD to 1) assess the frequency of rTreg and aTreg lymphocytes, and 2) identify CCR7 (C-C chemokine receptor type 7) expressing, CD45RA+ effector memory (EMRA) T lymphocytes as candidate disease initiating T lymphocytes. Design/Methods: A 15 year old child with severe CD was evaluated. Normal controls were derived from the mean of 10 normal pediatric bone marrow transplant donors. Blood samples were collected before the initiation of Anti-TNF-α (Infliximab) and after a standard 3 doses of induction treatment of Infliximab. The disease activities were divided into mild and severe based on the PCDAI scores. Treg lymphocytes were immunophenotypically assessed using the Miyara classification by 6-color immunofluorescence on a CANTO FACS machine. A sequential gating strategy was used. Results are presented as a percentage of total Treg lymphocytes. The identification of candidate disease initiating T lymphocytes included identifying EMRA T lymphocytes since the disease initiating T lymphocytes are hypothesized to be present in the memory T lymphocyte subpopulations. Results: The patient had severe disease activity before Infliximab treatment. The rTreg lymphocytes were suppressed when compared to normal control (frequency of 7.2 compared to those of normal individuals of 16.9). aTreg lymphocytes are similar to controls. However, EMRA T lymphocytes (CD45RA+, CD62L-) were increased 19 times of normal (frequency of 66 compared to those of normal individuals of 3.4). After Infliximab treatment, the patient had mild disease activity and the rTreg lymphocytes remained suppressed (frequency of 5.4 compared to controls of 16.9). However, EMRA T lymphocytes were significantly decreased (from 66 to 0.7). Conclusions: Our findings demonstrated that a child with severe CD had suppressed rTreg lymphocytes. The rTreg remained suppressed after Infliximab treatment. Our data also showed highly increased EMRA (candidate disease initiating T lymphocytes) in a child with severe CD which was significantly reduced after Infliximab treatment. Our findings of Treg dysfunction in severe CD may provide an alternative treatment approach of pediatric CD (ie in vivo boosting of Treg cell number/function). Our data also demonstrated that anti-TNF-α (Infliximab) treatment did not restore rTreg.

249 Very Early Onset Inflammatory Bowel Disease in a 20 month-old female. A.M. McClain, Y. Smadi, R. Figueroa-Colon, K. Horvath, Center for Pediatric Digestive Health and Nutrition, Orlando Health, Orlando, Florida, UNITED STATES. Inflammatory Bowel Disease (IBD) has a peak incidence in the 15-25 year age group and is less frequent in younger age groups. We describe an unusual presentation of IBD in a 20 month-old female. The child was previously healthy and was admitted for severe dehydration and 3 weeks history of non-bloody diarrhea, weight loss, fatigue and vomiting. Her initial workup was significant for several electrolyte abnormalities and hypoalbuminemia [<1] due to protein losing enteropathy. Over the course of her admission, she continued having diarrhea, up to 15 stools a day, and eventually required TPN. All infectious etiologies of her diarrhea were ruled out. On further work up, she had a normal vasoactive peptide level, normal IL-10, negative anti-enterocyte antibodies and a negative HLA-B51 [found in 60% of Behchet's patients]. The child did have slightly decreased IgG, requiring a dose of IVIG. Her EGD was normal and her flexible sigmoidoscopy revealed active colitis with inclusion bodies in the mucosa that raised suspicion of CMV colitis. Although CMV was not confirmed with immunostaining, she was treated empirically for CMV colitis, but with no change in her symptoms. She subsequently started passing sloughed intestinal mucosa, multiple times, that were 1 to 4 inches long [see photo]. A repeat flexible sigmoidoscopy revealed an ulcer in the sigmoid colon, but full colonoscopy was not performed due to the risk of perforation at that time. The child’s course of diarrhea exceeded 8 weeks and her protein losing enteropathy slowly improved. She developed mouth ulcers later in the course and her evaluation for retinitis was negative. She was started on steroids and her symptoms and labs gradually improved. Trying to wean her off steriods led to relapses in symptoms. Seven months after her initial presentation, she was able to have a full colonoscopy that revealed chronic active colitis compatible with IBD. In conclusion, her initial presentation was concerning for infectious or autoimmune process but a very extensive work-up ruled them out. Her initial presentation and symptoms at the age of 20 months is an unusual manifestation of inflammatory bowel disease

250 Ewing Sarcoma (ES) In A Child With Ulcerative Colitis (UC).. L. Mehyar, W. Attia, I. Monteiro, Pediatrics, UMDNJ-New Jersey Medical School, Newark, New Jersey, UNITED STATESJ. Menell, Pediatrics, St. Joseph's Regional Medical Center, Paterson, New Jersey, UNITED STATES. Colorectal cancer (CRC) risk is increased in UC. There are conflicting reports of risk for extraintestinal cancer (Ca) in UC. We present a child with UC and Ewing sarcoma. Case report: 9 y female with UC, diagnosed 3y prior, initially treated with steroids and 5-aminosalicylic acid (5-ASA) and 6-mercaptopurine (6MP). Received steroids at 8y and switched to Sulfasalazine and continued on 6MP. Her colitis was in remission, when she developed cough, chest pain and fever. No family history of Ca. Physical exam significant for a bulge on the right posterior chest. CT scan revealed: pleural effusion with an irregularly enhancing lobulated mass arising from the right lateral pleura, extending through rib spaces into subcutaneous fascia; no enlarged lymph nodes nor lung nodules. Biopsy revealed small round blue cell tumor consistent with ES. Bone marrow biopsies: no metastasis. Bone scan: positive in the region of the right 8th rib. She was started on chemotherapy(chemo), 6MP was held and sulfasalazine was continued. She had some non-bloody diarrhea with 1st chemo cycle and received metronidazole. Her mass decreased after 4 cycles and she had resection of the mass and several ribs with chest wall reconstruction. She had chemo for a total of 42weeks and received radiation to the chest wall. She is off therapy almost 7 y with no evidence of recurrence. Her UC is in remission. Discussion: Management options of UC include 5-ASA, steroids, immunomodulators, biologicals and surgery. Thiopurines are used for maintaining remission in children with 5-ASA intolerance, steroid dependence or frequent relapsers, as well as a bridging therapy after the start of cyclosporine or tacrolimus. UC itself has increased risk for CRC, which may decrease with thiopurines because of their role as antineoplastic agents. However, thiopurines have been linked with many cancers, like non-Hodgkin lymphoma, hepatobiliary carcinoma, hepatosplenic T-cell lymphoma but not ES. ES occurs in bone, and less frequent in soft tissues. Our child with UC on 6MP had pleural ES. Conclusion: Though UC is associated with increased risk of CRC and thiopurines with different cancers, this is the first association of ES in a child with UC and thiopurines.

251 Esophageal ulcer in children with ulcerative colitis.. R. Nambu, S. Hagiwara, M. Kubota, S. Kagimoto, Saitama Children's Medical Center, Saitama,Saitama, JAPAN. Objectives: Esophageal ulcer is rare condition in children having ulcerative colitis (UC). Recently, vigorous upper endoscopy has shown UC could involve upper gastrointestinal tracts, even without symptoms. Although some reported that children with UC have gastritis and gastric ulcer, it is rare that they have esophageal ulcer. The aim of the study is to elucidate the frequency and clinical features of esophageal ulcer with pediatric UC. Methods: 7 pediatric UC patients who had undergone upper gastrointestinal endoscopy from April 2010 to March 2013 in Saitama Children’s Medical Center were retrospectively analyzed. Results: Esophageal ulcer with UC was found in 4 (57 percent) of 7 patients. 2 of the four children with esophageal ulcer were male. All 4 patients were diagnosed between 10-13years-old. None had gastro esophageal reflux, and were negative for cytomegalovirus infection. 2 ulcers were confirmed of esophageal ulcer in initial diagnosis of UC, the other were found at the time of recurrence. Among 4 children having ulcer, 2 experienced nausea and epigastric pain, and the other were asymptomatic. Esophageal ulcers of all 4patients recovered following improvement of PUCAI (Pediatric Ulcerative Colitis Activity Index). 2 symptomatic patients become pain-free two days after initial therapy: 5-ASA (5- aminosalicylic acid), prednisone and histamine type 2 receptor antagonists. There were no specific findings pathologically: 2 cases were nonspecific inflammatory mucosa, and the others were normal-findings. Conclusions: Although it is thought to be rare that UC involves esophageal ulcer, we found unexpectedly that 57% children with UC had esophageal ulcer. In children with UC, the incidence of esophageal ulcer may be higher than we think. Now we don’t know so far about its clinical significance, it will indicate the unknown feature about UC. Upper gastrointestinal endoscopy has been proved useful in the diagnosis of children suspected of having UC. Further study should be needed.

252 Sensitivity to 5-ASA in Pediatric Inflammatory Bowel Disease. S. Lee, A. Noble, C. Justinich, Queen's university, Kingston , Ontario, CANADA. OBJECTIVES: Mesalamine or 5-aminosalicylic acid (5-ASA), is commonly used to treat inflammatory bowel disease (IBD). 5-ASA sensitivity has many manifestations including a paradoxical exacerbation of gastrointestinal (GI) symptoms. Although reported to be relatively rare, we have observed this adverse event in increasing numbers of patients. This study was conducted to determine the prevalence and nature of 5-ASA sensitivity in a pediatric ulcerative colitis population and its correlation with atopic disease and eosinophilia. METHODS: This is a retrospective review of pediatric ulcerative colitis cases from 01-2003 to 06-2011 . Demographic information, disease location, atopic history, eosinophil counts, and details of 5-ASA sensitivity were collected. Patients were considered sensitive to 5-ASA if they experienced an adverse reaction requiring withdrawal of the medication that was reproduced on re-challenge. RESULTS: 44 children with ulcerative colitis were identified. The median age was 13 years (range 2 – 17 years) and 55% were male. The location of disease: 3 proctitis, 10 left-sided and 31 pancolitis. 5-ASA re-challenge was not possible in three patients secondary to early colectomy. Of the remaining 41 patients, 5-ASA sensitivity was seen in 11 (27%). Reproducible worsening of GI symptoms was identified in 10 and rash/Stevens-Johnson syndrome in the 11th. The presence of atopic disease and peripheral blood eosinophilia were not significantly different between patients sensitive and not sensitive to 5-ASA. CONCLUSIONS: The prevalence of 5-ASA sensitivity among children with ulcerative colitis is significantly higher in our IBD population than previously reported. This may indicate previous under-recognition or an increasing prevalence of 5-ASA sensitivity in children. Atopy and peripheral blood eosinophilia do not correlate with 5-ASA sensitivity.

253 Gingival Hyperplasia as a Presentation of Crohn’s Disease. C.A. Nugent, D. Gelfond, M. Khatib, S.S. Baker, Digestive Disease Nutrition Center , Childrens Hospital of Buffalo and State University of New York at Buffalo, Buffalo, New York, UNITED STATESA.R. Khan, Department of Pathology and Laboratory Medicine, Women & Children’s Hospital of Buffalo, State University of New York at Buffalo, Buffalo, New York, UNITED STATES. Oral manifestations of Crohn’s Disease (CD) are widely recognized features and vary from the common aphthous ulcer to the rare buccal abscess. We describe a patient who presented with a nine-month history of worsening gingival hyperplasia, buccal and facial edema, intermittent facial swelling and chelitis. Dental biopsies of oral lesions were significant for granuloma formation. The clinical history was suggestive of inflammatory bowel disease and Crohn’s Disease was confirmed by histological examination of colonic biopsies. The patient was treated with mesalamine and prednisone initially, followed by tapering and cessation of the prednisone. Maintenance was achieved with 6-mercaptopurine. The oral lesions resolved. This patient’s presentation highlights the importance of recognizing oral lesions as a first presentation of Crohn’s Disease. It focuses attention on an interesting and unusual manifestation with gingival hyperplasia as the presenting symptom. This finding highlights the importance of including CD in the differential diagnosis of oral lesions. After histopathological confirmation of a suspicious oral lesion, careful follow-up is needed with attention to the entire gastrointestinal tract. Strong collaboration with dental care providers is critical in facilitating prompt evaluation and therapy for pediatric patients with Crohn’s disease. IBD care models should include dental practitioners.

254 A Case of Fecal Microbial Transplant as Adjunctive Therapy for Crohn’s Disease. S.L. Page, Gastroenterology, Children's Mercy Hospitals & Clinics, Kansas City, Missouri, UNITED STATES. A 16 month old boy presented with fulminant colitis and was diagnosed with Inflammatory Bowel Disease, likely ulcerative colitis. Frequent hematochezia with blood transfusions and a lack of response to intravenous steroids required the initiation of infliximab therapy. Improvement was noted in frequency of stools and decreasing hematochezia; however, symptoms recurred quickly, requiring further blood transfusions. Repeat endoscopy revealed inflammation in the small intestine as well and a surgical consultation was obtained. Colectomy was avoided after infliximab therapy was increased to maximum dosing. The patient’s parents sought the opinion of a naturopathic doctor who was utilizing fecal microbial transplants (FMT). FMT was initiated with a parent as a stool donor with resolution of hematochezia. However, when the parent developed a sinus infection and FMT was discontinued secondary to antibiotic use, his hematochezia returned. Infliximab was continued with the addition of a small dose of oral steroids. FMT was reinitiated when the patient failed a trial wean off prednisone; FMT was provided through an enema nightly for 3 weeks and then weaned to every 3rd night for 2 weeks. He was able to wean off prednisone therapy quickly. Parents also instituted a completely vegan diet. Since his FMT therapy, we have been able to maintain his hemoglobin and ESR at normal values with every 6 week infliximab therapy for the last 10 months. He is growing and thriving and developing well. This case demonstrates that FMT may be a viable adjunctive therapy in the treatment of IBD.

255 Pulmonary nodules in a teenager with Ulcerative Colitis. A. Alper, U.P. Phatak, M.G. Patel, D.S. Pashankar, Yale University, New Haven, Connecticut, UNITED STATES. Background : Respiratory problems in association with ulcerative colitis or its therapy are rare. We report a case of a 14 year old boy with ulcerative colitis and pulmonary nodules. To the best of our knowledge, there are no similar reports in the pediatric literature. Case Report : A 14 year old boy with mild ulcerative colitis presented with fever and cough. Three years prior to admission he was diagnosed with ulcerative colitis and was treated with mesalamine. Since his diagnosis he had two relapses. His last relapse was 5 months prior to admission, and since then he has not been on steroids. He presented with a one month history of cough that progressed to chest pain, worsened by deep inspiration, and associated with spikes of fever. He denied any gastrointestinal symptoms or weight loss. His physical exam was unremarkable. Blood tests were remarkable for elevated inflammatory marker (CRP - 84 mg/L). Chest x-ray revealed a peripheral right upper lobe consolidation. A course of antibiotics lead to no improvement. CT of the chest revealed multiple bilateral pulmonary nodules. Bronchoscopy showed non specific diffuse exudative infiltrates. All broncho-alveolar lavage studies were negative. CT guided percutaneous lung biopsy revealed an organizing pneumonitis with marked neutrophil exudates and a focal peripheral granulomatous rim. Stain for fungi, pneumocystis carini and mycobacteria was negative. The possibility of drug-induced pneumonitis was considered, and mesalamine was discontinued. Over the next two weeks his respiratory symptoms significantly improved and the CRP trended down. Steroid therapy was not used. Repeat CT 6 weeks after revealed near complete resolution of the pulmonary nodules. At his last followup 8 months after his presentation, he continues to do well without any respiratory symptoms and is not on mesalamine. Conclusion : We report a very rare case of mesalamine induced pneumonitis, with a radiographic pattern of pulmonary nodules, in a teenager with ulcerative colitis. His symptoms and lesions resolved with discontinuation of mesalamine without any therapy. This case underscores the importance of considering the side effects of medical therapy in a patient with respiratory symptoms and inflammatory bowel disease.

Intestinal/Colonic Disorders – Non-IBD 271 Holding the Record for Retention of an Endoscopy Capsule…. M.L. Herrera, H. Awai, M. Hilfiker, J. Huang, Pediatrics, University of California, San Diego, La Jolla, California, UNITED STATESM.L. Herrera, H. Awai, M. Hilfiker, J. Huang, Gastroenterology, Rady Children's Hospital, San Diego, California, UNITED STATES. Wireless capsule endoscopy (WCE) is an important tool for direct visualization of the small bowel without associated risks of radiation or sedation in youth who are able to swallow the capsule. FDA approval for WCE in children 10-18 years was granted in 2003 and in 2009 for children 2 years and older for evaluation of gastrointestinal bleeding and small bowel disease. While noninvasive, WCE carries certain risks, particularly capsule retention. Case: A 10 year-old well appearing male presented to his primary care pediatrician for evaluation of abdominal pain, which subsequently resolved over a 2-day period. At the height of the patient’s symptoms, his pediatrician performed an abdominal X-ray and found a foreign body consistent with a wireless endoscopy capsule localized to the right abdomen. Further interview revealed that the boy had a perinatal history of congenital and that had been surgically repaired with subsequent development of adhesions, which were lysed. When the patient was 2.5 years old, he was admitted for two months at an out-of-state institution for evaluation of gastrointestinal bleeding. WCE evaluation during that admission revealed multiple arteriovenous malformations, which were managed conservatively. The patient’s family reported multiple changes in attending physicians over the course of their hospital stay. While their son was hospitalized, his parents report initial diaper checking for passage of the capsule; however, ultimately the patient was discharged without the capsule passing. No subsequent follow-up for capsule passage was performed. The patient subsequently remained asymptomatic outside of the interim abdominal pain when he was referred to the Pediatric Gastroenterology clinic for intestinal foreign body evaluation at age 10 years. At the GI clinic visit, the patient was asymptomatic. Physical exam was unremarkable. Stool guaiac testing was positive. Hemoglobin was 13.3 g/dL with normal red blood cell indices. A computed tomography revealed a radio-dense body consistent with the endoscopy capsule in the proximal small bowel and distention of small bowel loops consistent with partial obstruction from adhesions. The patient is scheduled to undergo surgical removal of the retained capsule. Surgical findings will be presented. Discussion: Capsule retention requiring surgical removal is a known complication of WCE. This case highlights the role for patency capsule use prior to WCE in patients with prior surgery, abdominal adhesions, and/or potential obstruction and the importance of adequate patient follow-up to ensure WCE passage. The case also underscores the need for appropriate sign-out and follow-up across providers and between providers and patients during transitions of care.

272 MANAGEMENT OF A CHILD WITH A SIMULTANEOUS MULTIPLE MAGNET INGESTION: A CASE REPORT.. J.W. Ibrahim, E. Wan, F. Daum, Department of Pediatrics, Winthrop University Hospital, Mineola, New York, UNITED STATESD. Barlev, Department of Radiology, Winthrop University Hospital, Mineola, New York, UNITED STATES. Case presentation: A 3.5 year old, swallowed eight 0.5 cm magnets. She was asymptomatic at presentation. Initial abdominal X-ray revealed the magnets to be stuck together in a ringlet formation in the stomach. Attempted endoscopic removal was aborted, as the magnets had passed beyond the stomach. The patient was admitted for observation and maintained NPO in anticipation of possible colonoscopy/surgery. Serial abdominal examinations and X-rays were performed. On day 2, radiographs showed progression of the magnets into the ascending colon. Six capfuls of polyethylene glycol PO Bid were given. On day four, without incident, the magnets passed spontaneously. Background: Magnet ingestion has become increasingly common in the pediatric population. Management differs according to the number of magnets ingested, the timing of ingestion (separate or simultaneous ingestion), the initial presenting symptoms and the location of the magnets. Discussion: Based on our management of this patient, we propose a conceptual modification to the NASPGHAN (North American Society for Pediatric Gastroenterology, Hepatology and Nutrition) algorithm, 2012, JPGN 55 (3): 239–242.Patients who ingest multiple magnets can suffer complications including intestinal obstruction, perforation or fistula formation. According to the NASPGHAN algorithm, the primary management of asymptomatic patients with simultaneous multiple magnet ingestion, or co-ingestion of a magnet with a metallic object is enteroscopy or colonoscopy with consultation by surgery. This case highlights the possibility of a successful and safe approach using observation and PEG laxative obviating the need for enteroscopy/ surgery. Conclusion: Our conceptual modification is using observation both clinically and radiographically as the primary approach in management of asymptomatic cases of multiple magnet ingestions with direct adherence of the ingested magnets to each other or to a metallic object.

273 Colonic Polyposis: an unusual presentation of pseudomembranous colitis. T. Imam, K. Gorla, K. Hayani, Pediatrics, Children's Hospital University of Illinois, Chicago, Illinois, UNITED STATES. Introduction A rare case of pseudomembranous colitis due to Clostridium difficile (C. diff) infection is described. Pseudomembranous colitis is a presentation of C. diff infection that typically presents with bloody diarrhea and exudative white-yellow plaques on the surface of the affected colon. We describe a 19-year-old perinatally HIV- infected, severely immunocompromised male with C. diff-positive pseudomembranous colitis and diffuse polyposis found on colonoscopy. Case The patient had a history of poorly controlled HIV (CD4= 68 cells/µL; HIV RNA PCR= 97,219 copies/mL), end-stage renal disease requiring hemodialysis, hepatitis B infection, cardiomyopathy, and recurrent C. diff-positive diarrhea. He was admitted to the intensive care unit with marked abdominal distention, pain, and severe bloody diarrhea. He was found to be C. diff positive and was treated with various combinations of vancomycin, metronidazole, rifaximin, and fidaxomicin. Despite aggressive and prolonged therapy, he exhibited minimal clinical improvement. Colonoscopy demonstrated multiple pedunculated yellow polyps of varying sizes throughout the recto-sigmoid colon. Histologic findings indicated pseudomembranous colitis: colonic mucosa with mild chronic inflammation in the lamina propria and an exudate composed of neutrophils admixed with fibrin and superficial gram-positive cocci in chains on the surface of the fragments of colonic mucosa. AFB stains and immunohistochemical stains for CMV were negative. GMS and PAS stains were positive for fungal elements in the exudate but negative in the fragments of colonic mucosa. Discussion Diffuse polyposis can be seen in malignancy, familial adenomatous polyposis, Gardner’s syndrome, Peutz-Jeghers syndrome, or chronic non-steroidal anti- inflammatory use. A review of the literature revealed only one similar case reported in a 53-year-old HIV-positive male. Conclusion This case illustrates a rare endoscopic finding of pseudomembranous colitis with colonic polyposis in an HIV-positive patient with Clostridium difficile infection.

274 Survival of a 2 Month Old Infant with Junctional (JEB) and Fulminating Amoebic Colitis after Conservative Treatment. S.S. Khorsheed, B. Mansouri, A. Bali, Department of Pediatrics, King Fahad Armed Forces Hospital, Jeddah, Makkah Region, SAUDI ARABIAS. Al Zeair, Department of Radiology, King Fahad Armed Forces Hospital, Jeddah, Makkah Region, SAUDI ARABIA. Acute fulminating colitis is a very rare complication of amoebiasis occurring in less than 0.5 percent of all cases of amoebiasis and is associated with a mortality rate of more than 75 percent. On the other hand, Junctional Epidermolysis Bullosa (JEB) is a blistering skin disease which may involve the gastrointestinal tract. We are reporting the survival of a 2 month old infant with JEB and fulminating amoebic colitis that was managed conservatively. Up to our knowledge there is no reported case of surviving infant with this combination in the English literature. In this report, we are discussing the spectrum of clinical presentations of amoebic colitis in infants, the gastrointestinal manifestations of JEB which further complicated the clinical picture, and the treatment modalities of amoebic colitis.

275 Does Milk Protein-induced Colitis in Infants Resolve Prior to One Year of Age?. F. Lazare, T. Marciano, F. Daum, Pediatric Gastroenterology, Hepatology and Nutrition, Winthrop University Hospital, Mineola, New York, UNITED STATES. Milk protein intolerance affects approximately 10% of term infants. Infants present with bright red blood per rectum (BRBPR) and/or guaiac positive stools consistent with colitis. Babies with milk protein intolerance are given a hypoallergenic formula usually until one year of age. HYPOTHESIS: Infants may become tolerant to the offending agent by 6 months of age. METHODS: Patients with BRBPR and/or guaiac positive stools were prescribed either a casein hydrolysate or amino acid based formula depending on the original offending formula. Stool guaiac cards were checked weekly for 3 weeks and then monthly for 2 months. At 6 months of age, guaiac negative infants were rechallenged with the offending agent. CBC with differential and serum IgE to milk and soy were drawn at the time of diagnosis. RESULTS: Sixteen infants were enrolled, and all 16 completed the study. All enrolled patients were switched from a casein hydrolysate formula to an amino acid based formula. Fourteen were successfully weaned off their new amino acid based formula to the original offending agent by 6 months of age. The other 2 were successfully weaned off by 8-9 months of age. IgE to milk and soy were negative, no subject had peripheral eosinophilia, only 1 had iron deficiency anemia. CONCLUSION: Infants with hematochezia and milk protein intolerance become tolerant to their original offending formula usually by 6 months of age helping families and insurance companies avoid an additional, unnecessary expense for an amino acid based formula for several unwarranted months

276 Baby Bottle Nipple Causing Small Bowel Obstruction. N. Malhotra, C. Haller, T. Weinstein, Pediatric Gastroenterology and Nutrition, Cohen Children's Medical Center - Hofstra Northshore-LIJ School of Medicine, New Hyde Park, New York, UNITED STATESS. Soffer, A. Lipskar, Pediatric Surgery, Cohen Children's Medical Center - Hofstra Northshore-LIJ School of Medicine, New Hyde Park, New York, UNITED STATES. Introduction: More than 100,000 cases of foreign body ingestion are reported annually in the United States, with the majority occurring in the pediatric population. Most foreign bodies that reach the gastrointestinal tract pass spontaneously. Approximately 10-20% of foreign bodies ingested require endoscopic removal, and less than 1% require surgical intervention. Unobserved foreign body ingestions in young children and children with developmental delay often present as a diagnostic dilemma. We report an unusual case of small bowel obstruction due to a baby bottle nipple ingestion. Case Report: A 13 year-old male with a history of cerebral palsy, seizure disorder and blindness presented to our facility with multiple episodes of non-bilious, non-bloody emesis and increased seizure activity. Physical exam revealed an afebrile male with developmental delay. Abdominal exam was significant for diffuse tenderness. Emesis persisted and eventually became bloody and bilious. An abdominal radiograph revealed a distended air fluid filled stomach. An UGI series revealed a filling defect consistent with a baby bottle nipple at the third portion of the duodenum. An emergent upper endoscopy and removal of foreign body was attempted. However, due to the suction effect of the nipple on the bowel wall, the foreign body was irretrievable, but manipulated to reduce the obstruction. A repeat UGI revealed the foreign body was still in the third portion of the duodenum and again caused complete obstruction. A repeat upper endoscopy with foreign body removal was performed with surgery present in preparation for possible laparotomy. With rat tooth forceps and persistent slow, gentle traction, the nipple was dislodged and the suction effect was relieved. At the site of obstruction, there was evidence of mucosal damage including ulcerations, erosions, and erythema. There was no perforation. The patient was started on a PPI, feedings were resumed, and the patient was discharged home without difficulty. Upon further questioning, it was revealed that the child received a nightly bedtime bottle. The parents had not realized that a bottle nipple was missing. It is unclear how long the foreign body was present in his gastrointestinal tract prior to presentation. Discussion: There have been no known documented cases of baby bottle nipple foreign body in the duodenum, and no known cases of small bowel obstruction due to a bottle nipple. This case highlights the importance of looking for a non-opaque foreign body in children who present with evidence of small bowel obstruction. Additionally in the management of obstructive duodenal foreign bodies, a persistent attempt at endoscopic removal to dislodge the suction effect with slow traction may help facilitate a successful endoscopic removal and avoid major surgery.

277 Endoscopic Repair of Neodymium Magnet Perforation. S. Malowitz, R.A. Noel, Pediatric Gastroenterology, LSUHSC-New Orleans, New Orleans, Louisiana, UNITED STATES. BACKGROUND: Complications from magnet ingestions are common due to the availability of rare-earth magnets, whose attraction is powerful enough to perforate the intestine. Multiple magnet ingestion carries a higher risk of surgical intervention and morbidity than ingestion of most other foreign bodies. Endoscopic clipping has been used as a minimally-invasive method of treating appropriate bowel perforations. Most cases of perforation of the gastrointestinal tract necessitate surgical intervention. We describe a case of a gastric and duodenal perforation that was treated using an endoscopic method of perforation closure. CASE HISTORY: A healthy asymptomatic 3 year old boy presented to the emergency room because he swallowed magnets at the end of two toy darts. An X- ray performed 6 hours later confirmed a cylindrical foreign body in the mid-abdomen. PROCEDURE: On endoscopy, a disc magnet was found embedded in the antral mucosa. Removal using a net retrieval catheter, several different grasping types of endoscopic forceps, and rare earth magnets contained in a retrieval net was unsuccessful from the deep pressure lesion in the antrum. Inspection of the duodenum revealed 2 visible disc magnets embedded in the mucosa of the third portion of the duodenum. Using an endoscopic snare, three disc magnets were retrieved from the duodenum linked into a cylinder, and gastric and duodenal perforations were noted. Using endoscopic clips (3 in the stomach and 5 in the duodenum) the perforation was closed and nasogastric and nasojejunal tubes were placed endoscopically over guidewires for decompression. The patient was treated with broad-spectrum antibiotics for 7 days. RESULT: An upper GI contrast study on postoperative day 1 showed no extravasation of contrast into the peritoneum. Three days following endoscopy the patient began a liquid diet, and he was discharged on a regular diet on day 7. The patient returned to the emergency room on day 11 with vomiting, diarrhea, and abdominal pain. A repeat abdominal X-ray showed that two endoscopic clips were still in place in the duodenum while the rest had migrated through the intestine; there were no free air or intraluminal air/fluid levels. The patient ate without pain or vomiting and was discharged. In clinic 2 weeks later he was asymptomatic on a regular diet. CONCLUSION: To our knowledge there have been no reported repairs of magnet perforation using endoscopic clips. This successful case offers an alternative method of repair in perforations caused by rare earth magnets. This endoscopic method may save appropriate patients the morbidity of an open surgical procedure. This case also highlights the importance of a high index of suspicion of intestinal injury associated with magnet ingestion.

278 Recurrent Ileitis as a manifestation of Familial Mediterranean Fever. J. Merves, L. Albenberg, A. Maqbool, Gastroenterology, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, UNITED STATESP.F. Weiss, Rheumatology, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, UNITED STATES. M.B. presented with 10 episodes of recurrent abdominal pain and fever prior to his diagnosis of Familial Mediterranean Fever (FMF). He was a healthy male who moved to the U.S. from Eritrea 1 year prior. He presented initially with fever and RLQ abdominal pain concerning for appendicitis. However, imaging revealed terminal ileum (TI) inflammation and labs suggested normocytic anemia, CRP 8.9 and ESR 65. Infectious workup was negative. The symptoms resolved spontaneously. M.B. had two subsequent episodes of self-resolving (after 3-4 days) fever (Tmax 103 degrees) and RLQ abdominal pain separated by 2-4 weeks. An additional work-up included UGISBFT which suggested ileitis with cobblestoning features of the TI. EGD and Colonoscopy was performed following the 3rd episode and was notable for mild antritis and reactive lamina propria lymphoid follicles in the TI, but was otherwise normal. M.B. had several subsequent, identical episodes approximately monthly. Some episodes were associated with chest and back pain. Additional work-up during episodes included CT and MRE with TI thickening. EGD and colonoscopy were repeated with concern for Inflammatory Bowel Disease (IBD). Pathology suggested mild chronic antral gastritis, mild acute ileitis with focal cryptitis and patchy, mild acute colitis with focal cryptitis. He was started on an oral 5-ASA, but episodes of fever and abdominal pain continued with the same frequency. In addition to IBD, the differential included infection, immunodeficiency and a rheumatologic process. Rheumatology was consulted and became concerned for an auto-inflammatory syndrome. M.B. tested positive for the MEFV mutation associated with FMF. He was started on colchicine and has been well without further episodes for 6 months. FMF is an auto-inflammatory disorder that results in recurrent episodes of fever and serositis and can mimic IBD. It is the most common periodic fever syndrome and occurs mostly in individuals of Mediterranean and Middle Eastern descent. It often presents between the ages of 5-15 years of age. FMF results from an autosomal recessive mutation most commonly in the MEFV gene resulting in disruption of pyrin function and uncontrolled inflammation. The serositis associated with FMF usually results in abdominal pain, but can also cause pleuritic chest pain, synovitis and/or rash. FMF can also be associated with amyloidosis as a late finding in Type I FMF and as a presenting feature of Type II FMF. Symptoms are episodic occurring anywhere from days to years apart and children are healthy in between. There are case reports of children diagnosed with IBD who did not respond to therapy and who were later diagnosed with FMF with excellent response to colchicine (Egritas, et al). Diagnosis can be made clinically using the Tel Hasomer Criteria and with laboratory testing including a recurrent fever panel and MEFV gene mutation testing. Colchicine is usually effective as treatment and to prevent further episodes. Steroids and other immune modulators can also be used during acute episodes. FMF should be considered on the differential diagnosis of fever and abdominal pain including in patients who are being evaluated for IBD.

279 Primary Intestinal Lymphangiectasia and Hennekam Syndrome. J. Panganiban, I. Rojas, Pediatric Gastroeneterology, UTSW, Dallas, Texas, UNITED STATES. Background: Primary Intestinal lymphangiectasia (PIL) is a disorder of the lymphatic system characterized by dilated and tortuous intestinal lymphatics resulting from impaired lymphatic drainage. This leads to leakage of protein-rich chyle into the intestinal lumen causing protein-losing enteropathy, hypoalbuminemia, and peripheral or mucosal edema. Hennekam syndrome is one of the few syndromes associated with PIL. This is a rare autosomal recessive disease that is associated with intestinal lymphangiectasia, severe lymphedema, facial dysmorphism and mental retardation. Case: A 15 year old female, presented at 12 years of age with on and off swelling of her right leg for 3 years, intermittent cramping of her hands and tongue, weight loss and 3 weeks of watery diarrhea. On physical exam patient was developmentally appropriate with no facial dysmorphisms. Facial and right lower extremity swelling was observed as well as multiple hypopigmented patches on the face and legs. Labs showed significant hypoalbuminemia at 1.1mg/dl, multiple electrolyte disturbances, vitamin deficiencies and liver dysfunction. Alpha 1 antitrypsin in the stool was positive. Imaging included a normal liver ultrasound and MRI of the right lower extremity revealed no lymphatic obstruction. CT of the abdomen showed thickening of the duodenum. On upper endoscopy the mucosa of the stomach was grossly nodular, histology was positive for H. Pylori and colonoscopy was normal. Patient underwent capsule endoscopy which revealed significant evidence of lymphangiectasia in the jejunum with mild involvement of the duodenum. Patient was initially placed on TPN and was transitioned to a 20 gram fat restricted, high protein and low salt diet with MCT and vitamin supplementation. Patient had showed initial improvement yet was non compliant to the prescribed diet. One year later, she presented with intermittent groin swelling and a persistent rash seen on her genital area thus was evaluated by dermatology. She was diagnosed with lymphangioma circumscriptum. MRI of the pelvis showed lymphedema with mild ascites. Given the history of PIL and lymphedema patient was referred to genetics due to concerns for a mosaic of Hennekam syndrome. Genetic testing for mutation in the CCBE1 gene was negative. Discussion: PIL primarily affects children and can present with edema, diarrhea and ascites. 22% of cases can be associated with peripheral lymphedema. Upper endoscopy is the main method of diagnosis but since involvement may occur at the jejunoileum diagnosis must be confirmed by capsule endoscopy. Hennekem syndrome is known to be autosomal recessive. Only a quarter of cases have been associated with homozygous or compound heterozygous mutations in the CCBE1 gene. Since this condition is likely polygenic, the absence of a mutation does not imply the condition is not present and could represent a mosaic form. Conclusion: Intestinal lymphangiectasia is a rare disease, capsule endoscopy should be performed to evaluate the small bowel and diet is the cornerstone of therapy. Lymphedema may be associated with PIL but Hennekam syndrome, as a mosaic form cannot be ruled out.

280 Benign Nodular Lymphoid Polyposis in Childhood. M. Patel, A. Ghazi-Askar, A. Bitar, Z. Yu, M. Altaf, C. Marshall, University of Oklahoma Health Sciences Center, Oklahoma City, Oklahoma, UNITED STATES. Introduction: Benign Nodular Lymphoid polyposis (NLP) is a rare, self-limiting, disorder in children. It is characterized by clusters of multiple, small (3-9 mm) sessile polyps in the rectosigmoid colon and ileum. Little is known about the etiology, natural history, complications, diagnostic methods, and treatment. Our practice has encountered 3 such cases of NLP. Case presentations: We present 3 cases with NLP; two of whom are twins. The most recent case is a 4 year old healthy male, who presented with recurrent rectal prolapse. Endoscopic evaluation revealed several polyps in the recto-sigmoid area. Computed tomography revealed multiple abnormal polyps in the duodenum, sigmoid colon, and rectum. Histologic evaluation showed florid atypical lymphoid proliferation, and cytology revealed heavy chain rearrangement and polyclonality with positive staining for CD3, CD5, and Cyclin D1, consistent with a reactive lymphoid process such as benign lymphoid hyperplasia. The twins presented at age 6 with rectal bleeding. Colonoscopic examination revealed multiple polypoid lesions. Immunohistologic stains of tissue for cluster of differentiation (CD) revealed lymphoid tissue with follicles made up of polyclonal population of B-cells with heavy chain rearrangement consistent with polyclonal lymphoid proliferation. Tissue in both twins was negative for APC gene. Discussion: NLP is a lymphoproliferative disorder of unknown etiology. In children, the rectosigmoid colon and ileum are most often involved. The polyps and polypoid lesions are formed by lymphoid nodules with reactive germinal centers. NLP should not be confused with malignant lymphoma. Diagnosis is usually made via histological and immunochemistry analysis. Based on literature review, this appears to be the first case of NLP involving the duodenum. In most reported cases in pediatrics, age of presentation was less than 10 years with spontaneous regression reported. Radiotherapy and chemotherapy have been used in the past without significant reduction in polyposis. Surgical interventions may become necessary if patients develop complications, which include intussusception, intestinal obstruction, recurrent abdominal pain, and chronic gastrointestinal bleeding.

281 Attraction of foreign bodies in children: A magnetic force. S.A. Patel, V. Hupertz, O.S. Soldes, J. Moses, Cleveland Clinic Children's, Cleveland, Ohio, UNITED STATES. Introduction: Ingestion of foreign bodies is a common pediatric problems; magnet ingestion is increasingly becoming a common occurrence in this population. Multiple magnets that progress past the stomach attract one another across the bowel well, and often result in severe damage including obstruction, fistula formation, ulceration, perforation, and volvulus of the small and large intestine. Case report: A 7 year old boy with past medical history of attention-deficit disorder and oppositional defiant disorder presented with abdominal pain and vomiting. The patient had been seen originally by his pediatrician 8 months earlier with the above symptoms, which continued for 3 months. He was then seen by a pediatric gastroenterologist and placed on ranitidine for acid suppression and advised to have an upper gastrointestinal series (UGI) and an abdominal ultrasound (US). However, the patient improved on the ranitidine and was lost to follow-up. Four months later, his symptoms worsened with development of abdominal distension and weight loss, and he was brought in for the previously recommended imaging. A scout abdominal x-ray before the UGI revealed three oval shaped, radio-opaque objects in the right lower quadrant. The patient underwent an emergent laparoscopy, during which multiple old and recent injuries to the bowel from the magnets were found. A total of six enterotomies and a jejunogastric and jejunojejunal fistula were noted. Post-operatively, he developed an intra-abdominal abscess, requiring central access and long-term intravenous antibiotics. The magnets were discovered to be a Christmas gift given to his brother 8 months prior to the operation. Discussion: Multiple magnet ingestion can lead to many complications. Compressive necrosis caused by multiple magnets can lead to fistula formation, as well as volvulus, perforation, and sepsis. It is likely that the patient initially had a fistula formation without abscess, and eventually developed obstruction, leading to weight loss. Conclusion: Multiple magnets are dangerous when ingested. Due to greater awareness of their danger, many companies have recalled these products. Physicians and parents need to be well- educated on the dangers of letting children play with magnets.

282 Cecal Burkitt’s Lymphoma Presenting with Rectal Bleeding, Anemia and Subsequent Intussussception. Susan Peck, MSN, CRNP and Asim Maqbool, MD Division of Gastroenterology, Hepatology and Nutrition The Children’s Hospital of Philadelphia, Philadelphia, PA. S. Peck, A. Maqbool, Gastroenterology, Hepatology and Nutrition, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, UNITED STATES. BC is a 3 year old healthy male who presented with a 3 week history of abdominal pain, and preceding history of episodic abdominal pain. The pain was initially described as an ache and progressively intensified with him bending over and complaining of a crampy pain. He had decreased appetite and early satiety, and passage of hard stool daily to every other day with straining for some time, and had initiated Miralax for constipation within the recent few weeks. He had then had been on antibiotics for otitis media and was having 3 loose stools per day, which had become bloody. BC was ill ~ 5 months prior to initial GI clinic visit , had been hospitalized and had received IV antibiotics. On examination he appeared pale, thin and tired, height 61st %ile for age and BMI at 18th %ile for age. He was not tachycardic or tachypneic. His abdominal examination did not suggest tenderness, guarding, or masses; anorectal examination was notable for the absence of skin tags, fissures or tears; he had heme+ testing. An abdominal flat plate 2 days prior showed moderate rectal fecal material. His HGB/HCT was 8.8/26.0, decreased from 11.2/ 32 in January, 2013. The initial differential diagnosis included infections and IBD. Stool studies for bacterial and parasitic infections were positive for C. Difficile. He was started on metronidazole. UGI with SBFT was normal. An EGD and colonoscopy were to follow treatment and clearance of the detected colitis. He then presented to the ED~ 9 days later with RLQ pain and a palpable mass. U/S suggested ileocolonic intussusception (non-reducible radiologically), for which he had an exploratory laparotomy and had an ileocecectomy and appendectomy for a cecal mass and intussusception. Grossly, a cecal white fleshy mass and some ulceration were identified. Histology was consistent with colonic Burkitt’s lymphoma; specimen margins were viable and negative for lymphoma. 5 mesenteric lymph nodes with reactive follicular hyperplasia were documented. Burkitt’s lymphoma is relatively rare outside of Africa. In the U.S. and Western Europe, it accounts for up to 40% of pediatric lymphoma cases. Intussception as the presenting feature is much less common than abdominal pain, nausea or vomiting. In 1 series, intussusception as the presenting feature occurred in 17.5% of patients and was felt to be associated with early stage disease requiring less intensive therapy. BC has undergone a PET scan, CT of the neck as well as bone marrow aspirate, lumbar puncture and port placement for chemotherapy. The tumor was initially staged as IIB, and chemotherapy is being initiated.

283 Perforated diverticulitis – a rare cause of acute abdominal pain in a teenager.. A. Alper, M.G. Patel, D. Pashankar, U.P. Phatak, Yale University, New Haven, Connecticut, UNITED STATES. Introduction: Diverticulitis is typically seen in the elderly population and is reported in adult patients with chronic steroid use. We report a rare case of a 19 year-old boy who presented with acute abdominal pain due to perforated colonic diverticulitis. To the best of our knowledge, there are no previous pediatric cases in the absence of connective tissue disorders, which have been reported in the literature. Case Report: A 19 year old boy with a history of juvenile idiopathic arthritis and fifteen years of immunosuppression including prednisone, presented with a one day history of severe left lower quadrant abdominal pain, nausea and fever. Two months prior to admission, he had similar complaints, and was diagnosed with mild diverticulitis of the descending colon by CT scan. He was treated with oral antibiotics for 2 weeks, after which his symptoms gradually resolved. He was doing relatively well, with only intermittent mild left lower quadrant pain that was thought to be secondary to constipation. The patient had a past medical history of borderline diabetes mellitus, nephrolithiasis and severe systemic and polyarticular juvenile idiopathic arthritis with joint erosive disease, which was diagnosed at the age of six years. In addition to long-term steroids, medical therapy included adalimumab, methotrexate and naproxen. At the time of admission, abdominal examination was remarkable for left lower quadrant tenderness and guarding. Blood tests were remarkable for an elevated white blood cell count (28.3 X1000/uL) and inflammatory marker (C- reactive protein 119 mg/L). CT scan of the abdomen showed diverticulitis in the left colon and extensive left retroperitoneal air, suggestive of perforation. This prompted an exploratory laparotomy, which showed an inflamed left colon with perforation. The descending colon was resected and a transverse colostomy and a Hartmann pouch were performed. His immediate post-operative course was uneventful with satisfactory ostomy function. He underwent stoma closure two months later and has recovered well. Conclusion: Diverticular disease of the colon is very rare in patients below the age of 40 years. In children, reports are limited to selected connective tissue disorders. Certain conditions predispose patients to an early development of diverticulitis. We believe that our patient developed diverticular disease secondary to long-term immunosuppression, especially steroid use. This rare presentation of acute abdominal pain in a pediatric patient highlights the importance of considering this life-threatening complication in any patient with long term steroid use, regardless of age.

284 Lane Hamilton syndrome: Pulmonary hemosiderosis associated to celiac disease.. C. Pichardo, A. Luqman, E. Malvica, Department of Medical Education, Miami Children's Hospital, Miami, Florida, UNITED STATESC. Brathwaite, Department of Pathology, Miami Children's Hospital, Miami, Florida, UNITED STATESM. Franco, Department of Pulmonology, Miami Children's Hospital, Miami, Florida, UNITED STATESR. Restrepo, Department of Radiology, Miami Children's Hospital, Miami, Florida, UNITED STATESE. Hernandez, Department of Gastroenterology, Miami Children's Hospital, Miami, Florida, UNITED STATES. We report a case of a 6 year-old boy presenting with iron deficiency anemia unresponsive to ferrous sulfate, and hemoptysis. Physical examination revealed a well-nourished child with pallor. Investigation showed severe microcytic anemia with hemoglobin of 4.5 gm/dL. Chest X ray showing confluent infiltrates with associated small right pleural effusion. Computerized tomography of the chest depicted ground glass appearance compromising the entire right lung due to interstitial and mainly alveolar infiltrates with focal area of ground glass seen in the left lung. Bronchoalveolar lavage showed approximately 60% hemosiderin-laden macrophages which is consistent with the diagnosis of idiopathic pulmonary hemosiderosis (IPH). Occult blood in stool was positive. Since severe anemia was disproportionately to the amount of hemoptysis as well as unresponsive to prolonged ferrous sulfate treatment, searching for celiac disease was done showing elevated transglutaminase IgA was >128, antiendomysial IgA positive with absence of gastrointestinal complaints. Esophagogastroduodenoscopy revealed duodenitis with biopsy showing mild to moderate duodenitis with attenuation and broadening of the villi with intraepithelial lymphocytes. Patient was diagnosed with Lane Hamilton syndrome which is the simultaneous occurrence of celiac disease accompanied by IPH. The importance of diagnosing this association is that significant improvement is obtained with a gluten-free diet both for the intestinal and pulmonary symptoms.

285 A RARE CASE OF COMPLICATED PAN-COLONIC DIVERTICULOSIS IN A HEALTHY TEENAGER. P. Porayette, G. Subbarao, S. Waseem, Riley Hospital for Children, Indiana University, Indianapolis, Indiana, UNITED STATES. Diverticulosis is a disease of old age. It is usually found incidentally during colonoscopy, predominantly involving the left colon. Only three percent of cases occur in patients less than forty years of age. Its noted to occur rarely in the pediatric population and is usually associated with connective tissue disorders like Williams Syndrome, Marfans syndrome, and Ehler- Danlos syndrome. Moreover, children are more prone to developing complications of diverticulosis compared to adults. These include diverticular bleeding, diverticulitis, perforation and abscess formation. We report a 15 year old, otherwise healthy female, who presented with a one week history of severe left sided abdominal pain with nausea and vomiting. She was found to have diverticulitis in the sigmoid flexure of colon on CT imaging. Subsequent colonoscopy revealed multiple diverticulae throughout the colon most concentrated in the transverse colon but also had involvement of left and right colon including the cecum. She responded well to the first episode of diverticulitis with oral antibiotics and dietary modification with high fiber foods. However, she had recurrence of her abdominal pain in 3 months for which she was treated with outpatient oral antibiotic therapy but had recurrence of symptoms shortly after completion of treatment. A repeat CT scan of abdomen revealed diverticulitis flare extending into the cecum. She required inpatient admission for intravenous antibiotics with metronidazole and ceftriaxone, initial gut rest and then dietary modification with a low residue diet. The extent of disease and quick recurrence of complications with poor response to medical therapy is warranting a surgical evaluation as recommended by ACG committee. This case presents insight into the unique challenges in pediatric patients with diverticular disease who may require total colectomy at an early age.

Nutrition/Nutrition Support 318 Gastrointestinal Manifestations in Diploid Triploid Mixoploidy (DTM). M.B. Beg, G. Elsner, R.R. Lebel, SUNY Upstate Medical University, Syracuse, New York, UNITED STATESF. Bhamani, Aga Khan University, Karachi, PAKISTAN. REVIEW SUMMARY: A female infant was delivered by cesarean section at 32 weeks of gestation due to growth arrest and poor movement patterns. The infant had feeding problems which were based on gastroesophageal reflux, laryngomalacia, and decreased gut motility. Hypotonia was notable from the outset and she eventually displayed significant delays in both motor and cognitive milestones. Meanwhile, lymphocytes had yielded a normal karyotype (46,XX) but at two years of age she underwent a skin biopsy and mosaicism for a 68,XX cell line was discovered in fibroblasts. At six years and four months of age, the patient is very short of stature but has a weight at the 24th percentile and head circumference at the 50th. Other phenotypic features include: low set ears, piebald irides and scalp hair, eyelid ptosis, strabismus, broad nasal bridge, anteverted nares, upswept , hypoplastic teeth, pectus excavatum, hypoplastic labia, scoliosis, 3-4 finger syndactyly, 2-3 toe syndactyly. We present this case with a review of the literature for mixoploidy (the rare event of mosaicism for diploid and triploid cell lines). We add to the existing data on the clinical features of diploid/triploid mixoploidy. The complexities of the gastrointestinal problems make this case unusual.

319 Shiitake Mushroom mediated Enterocolitis in an Infant. A. Erakat, S. Lapsia, A. Chawla, Pediatric Gastroenterology and Nutrition, Stony Brook Long Island Children’s Hospital, Stony Brook, New York, UNITED STATES. Cow’s milk allergy is an immunologically mediated reaction to cow’s milk protein. It affects 2-3% of infants under 2 years and is the most common cause of food allergy in this age group. Avoidance of cow’s milk by an exclusively breastfeeding mother is an accepted approach to managing this allergy. Infants may react to dietary proteins during breastfeeding with clinical signs of atopic dermatitis, reflux, colic, enterocolitis, and constipation. Soy, eggs, peanuts, fish, and wheat may also be involved and their elimination from the mother’s diet has been recommended when symptoms persist. However, infants can react to other food proteins. Here we describe a unique case of a baby found to have colitis secondary to maternal ingestion of Shiitake mushrooms. A 7 week ex- full term exclusively breastfed male presented with 4 week history of bloody diarrhea, regurgitation, and irritability. Physical exam was significant for mild eczema and a positive stool guaiac. A plan to systematically eliminate common allergens from mother’s diet was implemented and included elimination of cow’s milk followed by soy, nuts, wheat and fish if clinical response was not seen. Despite these dietary modifications, his symptoms persisted and stool occult blood remained positive. A colonoscopy performed revealed nodularity with biopsies significant for few eosinophils noted throughout the colon. Subsequent skin allergy testing showed reactivity to poultry and eggs, both of which had already been eliminated from the mother’s diet. As a consequence of her limited diet, the mother increased her consumption of raw shiitake mushrooms and noticed an increase in hematochezia and irritability in the infant. With strict elimination of mushrooms, clinical improvement with decreased frequency of bowel movements was seen within 2 days and complete resolution of hematochezia and reflux occurred within 9 days. Reintroduction of some of the previously eliminated foods has not resulted in recurrence of symptoms. This case stresses the importance of taking a comprehensive diet history beyond the conventional six offending allergens from the breastfeeding mother and performing a sequential elimination diet to determine offending protein allergens.

320 Significant Obesity in the Setting of Malabsorptive Conditions In Two Children with Prader-willi Syndrome. S.B. Honigbaum, Z. Turner, A. Scheimann, Pediatric Gastroenterology and Nutrition, Johns Hopkins Hospital, Baltimore, Maryland, UNITED STATESN. Terrazas, S. Silver, A. Scheimann, Pediatric Gastroenterology and Nutrition, Texas Children's Hospital, Houston , Texas, UNITED STATES. Prader willi syndrome (PWS) is a complex inheritable syndrome caused by the lack of expression of paternal genes on the long arm of chromosome 15. The hallmarks of this disease involve neonatal hypotonia, short stature, developmental delay, early onset hyperphagia, and the development of morbid obesity. Obesity typically becomes problematic after one year of age secondary to hyperphagia and lack of satiety. In efforts to prevent the development of obesity and obesity-related co-morbidities, calorie restricted diets with strict limitation to food access is typically recommended for children. In contrast to PWS, certain diseases often make it difficult for children to properly gain weight. Both Cystic Fibrosis (CF) and Short Bowel Syndrome (SBS) are two examples of clinical scenarios in which patients often require excessive calories to achieve adequate growth. Though the causes of poor nutrition in these two diseases may be multifactorial, malabsorption is a common theme in both. In CF, estimated enteral needs are often as high as 150-200% of the recommended daily allowance. Children with SBS frequently require additional parenteral nutrition to achieve their energy needs. In this report, we present two children with significant nutritional challenges because of their dual diagnoses being associated with contrasting caloric needs. Our first case is a nine year old obese female with PWS and SBS secondary to congenital volvulus. Her diet is entirely oral and she suffers from chronic diarrhea and malabsorption. Despite this, her current BMI is 27 (>95th%). Our second case is a full term female infant with both PWS and CF with pancreatic insufficiency who had initial failure to thrive requiring transient gastrostomy tube supplementation. At the age of three and a half years old, she also had progressed to obesity with a BMI greater than the 95th percentile. There is a lack of literature discussing the management of such contrasting nutritional diseases. We present two cases of obese children with Prader Willi Syndrome despite their concomitant malabsorptive conditions of Cystic Fibrosis with pancreatic insufficiency and Short Bowel Syndrome.

321 Nutritional Adequacy of The Specific Carbohydrate Diet. N. Gregory, H. Vendettuoli, D. Suskind, Seattle Children's Hospital, Seattle, Washington, UNITED STATES. Background The Specific Carbohydrate Diet (SCD) is a diet gaining in popularity among individuals with Inflammatory Bowel Disease (IBD). The diet excludes all grains, starchy vegetables, most dairy, and only allows honey and saccharin for sweetening. The diet theorizes decreased intestinal inflammation by restricting the energy source for intestinal microbes; improving the bacterial balance of the gastrointestinal tract. Currently the SCD is supported only by anecdotal reports. No research studies have been published on the efficacy of it’s use. This however does not detract individuals from trying the diet to see if they get improvement and/or relief of their symptoms. This study was undertaken to evaluate the nutrient content of several patients who follow the SCD. Methods Food records and recipes were obtained from 4 patients following the SCD. 4-5 days of food records were analyzed for each patient using Food Processor SQL. Average nutrient intake was compared to the established Recommended Dietary Allowances (RDA) and Adequate Intakes (AI) by age for 20 nutrients. Results 3 of the 4 patients were following the SCD for treatment of Inflammatory Bowel Disease. The one nutrient that none of the 4 diets analyzed met recommendations for was vitamin D. Intake ranged from 22-30% of the RDA. Half of the diets analyzed also demonstrated low intake of <80% of the RDA/AI for thiamin, vitamin E, folate, calcium, phosphorus, and zinc. Conclusion This small project demonstrates that there is variability in the nutrient adequacy of the SCD. The one nutrient deficiency that all the diets shared, was inadequate intake of vitamin D. Vitamin D deficiency is common in IBD, yet has a known role in bone health for these patients and may play a greater role in their disease activity. As vitamin D intake appears low for children on the SCD, special focus on vitamin D intake as well as supplementation should be made for these children. % of RDA/AI for Children on the SCD *for ease of presentation, all levels of intake >100% of the RDA/AI were rounded to 100%. No intake exceeded Tolerable Upper Intake Levels.

322 Severe Persistent Unremitting Dermatitis, Chronic Diarrhea and Hypoalbuminemia in a Child; Hartnup Disease in Setting of Celiac Disease. M. Sultan, Pediatrics , Makassed Hospital - Alquds Medical College, Jerusalem, PALESTINE, STATE OFT. Ciecierega, Pediatrics, NYP-Weill Cornell Medical College, New York, New York, UNITED STATES. Background: While Celiac disease (CD) is a common disorder in children, Hartnup disease is not. Exfoliative erythema can be found among celiac patients due to malabsorption of many microelements. Hartnup disease is an autosomal recessive disorder caused by increased urinary excretion of neutral amino acids. Clinical abnormalities are heterogeneous ranging from asymptomatic to pellagra-like. Co-occurrence of Hartnup disease and CD is extremely rare with only few reported cases. Case Report: A 3-year old female with persistent chronic diarrhea and hypoalbuminemia since 1 year of age was referred for evaluation. Additional symptoms included severe scaly skin rash involving the upper body, photosensitivity, hyperpigmentation, painful recurring oral ulcers and low energy. Physical exam showed abnormally low growth parameters, generalized pitting edema and desquamated hyperpigmented skin rash. Based on abnormal screening labs and endoscopic evaluation (Marsh 3b) she was diagnosed with Celiac Disease. Patient was started on gluten-free diet and supplemented with total parental nutrition, intravenous zinc sulphate and multivitamins. She had only mild clinical and labolatory improvement after 2 weeks of treatment. A short-course of steroids also did not improve her clinical status. Because of persistent symptoms and nonimproving clinical course, she was suspected to have a niacin deficiency (The Pellagra) based on rash distribution, chronic diarrhea and low mood. Diagnosis of Hartnup disease was confirmed by presence of neutral aminoaciduria. High dose oral niacin was started followed shortly by a dramatic improvement and complete resolution of her symptoms. Conclusion: Presence of Celiac and Hartnup disease in single individual is very rare. It appears that CD with its severe enteropathy have uncovered the Hartnup disease and magnified symptoms of niacin deficiency. Hartnup should be considered in all celiac patients not responding to gluten free diet. Poster Session III

Esophagus/Stomach 350 A budding sword swallower: serial escalation of multiple foreign body ingestions in a pediatric patient. N. Patel, M. Kay, K. Eng, V. Okwu, S. Ali, K. Radhakrishnan, V. Hupertz, Pediatric Gastroenterology, Cleveland Clinic, Cleveland, Ohio, UNITED STATES. Introduction: Over 100,000 cases of foreign body ingestions are reported each year in the United States. Approximately 80% occur in children between the ages of 6 months and 3 years and 98% of foreign body ingestions in children are accidental. Serial intentional foreign body ingestions are more prevalent in adults due to underlying psychiatric disorders, mental retardation, alcohol ingestion, or in prisoners seeking secondary gain (i.e. transfer to medical facility). We present a case of a 16 year old female who started serial intentional foreign body ingestions at the age of 10. We describe the nature of progressive escalation and innovation of foreign bodies ingested. Case: The patient is a 16 year old female with a past medical history of major depression, bipolor disorder, and post-traumatic stress disorder who presented with recurrent self mutilation behaviors and foreign body ingestions since 10 years of age for attempted suicide. Despite being in a highly monitored inpatient psychiatric facility, she has continued to ingest foreign bodies intentionally for secondary gain. The patient self reports her ingestions so she can be removed from the psychiatric facility and be transferred to a “safer” hospital. She initially started swallowing smaller, blunt objects such as coins, EKG leads and AA batteries from clocks and remotes. Some of these objects were allowed to pass spontaneously and required no intervention. In order to meet urgent endoscopic criteria for foreign body removal, she quickly learned to start swallowing a variety of innovative long, sharp and large foreign bodies such as sharpened coloring pencils (10 cm), pencils (6-10 cm), sharp blades (3 cm), broken CD pieces, sharp metal nails taken off walls, plastic spoons (6 cm), broken toothbrush to create sharp edges (6 cm). Recently, she has learned to break and ingest sharp and long pieces of wood from a bookshelf. She has undergone approximately 15 endoscopies in the past 4 months for foreign body retrieval. Thus far, patient has not required surgical intervention and survived with minimal morbidity. (Images to be included on poster presentation). Discussion: Despite having complete supervision in an inpatient psychiatric facility, our 16 year old enterprising pediatric patient started escalating foreign body ingestion patterns starting at 10 years of age and identified new techniques for ingesting longer, larger and sharper foreign bodies, which are associated with higher complications rates. Although, ingestions are usually accidental in the pediatric population, we describe a child who started ingesting foreign bodies intentionally similar to adult patients. Pediatric gastroenterologists caring for pediatric patients need to be aware of this behavior, as well as the endoscopic techniques for removal of intentionally ingested sharp foreign bodies.

351 Panayiotopoulos Syndrome: Kids That Go Vomit In the Night. S.A. Patel, R. Steffen, L. Mahajan, Cleveland Clinic Children's, Cleveland, Ohio, UNITED STATES. Recurrent episodic emesis is a common cause for referral to pediatric gastroenterology. There are many causes of recurrent emesis including reflux, peptic ulcer disease, pancreatitis, cyclic vomiting syndrome, etc. We present a case of recurrent emesis that highlights a cause that may not be familiar to pediatric gastroenterologists. Case Report: A 7 year old female presented to GI clinic with complaints of episodic nocturnal emesis and daytime nausea that began at age 5 years. Mother also noted occasional loose stools, abdominal pain, and fatigue. For several weeks at a time, emesis would occur between 3 and 4am, and would not awaken her from sleep. She would then have several weeks free of emesis. She had an extensive workup, including normal brain MRI, negative upper endoscopy, and negative upper gastrointestinal series. She was initially diagnosed with atypical cyclic vomiting syndrome and was started on cypropheptadine with no improvement of her symptoms. She subsequently developed daily nocturnal urinary and fecal incontinence, prompting neurology referral. An EEG demonstrated focal epileptic discharges arising from right centro-temporal regions, consistent with Panayiotopoulos Syndrome (PS). She was started on oxcarbazepine without improvement and subsequently transitioned to levetiracetam with resolution of recurrent emesis and incontinence. Discussion: PS is a benign focal seizure disorder that occurs in children with onset most commonly between 1 and 8 years of age. Approximately 67% of seizures in PS occur during sleep with the majority of patients experiencing vomiting or other autonomic symptoms. An electroencephalogram is the only definitive diagnostic test. Prognosis is good and there is no known association with adult seizure syndromes later in life. Conclusion: Patients who present with nocturnal emesis, fecal and/or urinary incontinence, and other autonomic symptoms should be further evaluated and screened for Panayiotopoulos syndrome. The pediatric gastroenterologist should maintain a high index of suspicion as early recognition of PS may prevent invasive and expensive evaluation.

352 Human Papilloma Virus (HPV) and Epithelial Hyperplasia in Esophagus: Bystander versus Culprit. E.M. Reyes-Santiago, B.J. Infantino, F. Zapata, Pediatric Gastroenterology, University of Nebraska Medical Center, Omaha, Nebraska, UNITED STATESE.M. Reyes-Santiago, B.J. Infantino, D. Perry, J. Bedrnicek, F. Zapata, Pediatric Gastroenterology, Children's Hospital and Medical Center, Omaha, Nebraska, UNITED STATES. Background: Human papillomavirus (HPV) esophagitis is a rare entity in adults and children. It is grossly characterized by polypoid, smooth, whitish-pink lesions, and histologic findings consistent with superficial acanthotic epithelial hyperplasia and cellular koilocytosis.4 HPV infections have been linked to squamous cell carcinoma (SCC).3 Several studies have looked for HPV as the etiologic factor for esophageal squamous papillomas (ESP), which are uncommon, asymptomatic lesions usually discovered during endoscopy (small, single, round and elevated sessile lesions with smooth or rough surfaces).4 In children, recurrent respiratory papillomatosis (RRP) has also been identified as a primarily laryngeal disease caused by HPV. The potential for malignancy related to HPV infection, as seen in other tissues, is a concern, especially in children. Case: A 9 y/o ex-28 week premature Hispanic female with Dandy-Walker Syndrome, a VP shunt, seizure disorder, developmental delay, short stature, tracheostomy, dysphagia, gastrostomy tube dependence, and fundoplication was evaluated for gastroesophageal reflux. Her physical examination showed dysmorphic features, but was otherwise unremarkable, with a normal BMI. Initial endoscopy was normal. Repeat endoscopy due to airway concerns showed an edematous, irregular, whitish appearance throughout the esophagus. Histology showed epithelial hyperplasia and positive HPV staining with no fungus or eosinophils. Follow-up endoscopy demonstrated similar findings. Discussion: Esophageal HPV is a rare entity. ESP and RRP are infectious diseases that appear to be driven by HPV, although the exact potential for malignancy is uncertain. HPV should be considered in any patient with esophageal epithelial hyperplasia. Due to the known risk of malignancy in other tissues infected by HPV, regular surveillance biopsies may be advisable.

353 Lymphocytic esophagitis and celiac disease presenting after coin impaction: An incidental, exclusive or concomitant disease?. J. Rodrigues, K. Sycamore, A. Knutsen, A.K. Jain, Saint Louis University, St Louis, Missouri, UNITED STATES. Background: Lymphocytic Esophagitis (LE) is a rare and recently described disease characterized by the presence of dense intraepithelial lymphocytes (IELs) in the esophageal mucosa with the absence or rare presence of intraepithelial granulocytes. It remains unclear if LE is an independent pathological entity with any specific clinical or long term implications. We present the first known case of LE discovered after foreign body impaction. Case Report: A 4 year old female presented to the emergency room 5 hours after witnessed coin ingestion complaining of chest pain. Physical examination revealed a petite patient with mid sternal discomfort. X-ray confirmed a foreign body in the mid-thoracic esophagus. An upper endoscopy was performed and a nickel (5 cents coin) was removed from the mid-esophagus. The esophagus, stomach and duodenum looked normal on visual endoscopic examination. Pathology revealed a moderate to severe increase in IELs and rare plasma cells in the esophagus, with normal gastric and duodenal biopsy. The patient was discharged home on a proton pump inhibitor and then subsequently brought back for a repeat endoscopic evaluation two months later. On visual endoscopic examination the esophagus, stomach and duodenum appeared normal, however increased IELs, with CD3 positive T lymphocytes in the surface epithelium, villous atrophy and crypt hyperplasia was noted in the duodenum. The esophageal biopsy showed mild esophagitis with occasional IELs. The stomach biopsy was normal. A tissue transglutaminase IgA (tTG IgA) antibody was found to be very high (71 units/ml) and the patient was placed on a gluten-free diet. At a 5 month follow up the patient was asymptomatic and the tTG IgA normalized. Discussion: Lymphocytic esophagitis was first described by Rubio et al in 2006. The lymphocytes were noted to express CD3, CD4 and CD8 markers with a patchy distribution. Dysphagia, chest or abdominal pain, heartburn and nausea have been described as clinical presentations. For our patient, it is possible that the nickel in the coin caused an allergic contact dermatitis and local topical damage causing the lymphocytosis, but definite evidence is lacking and the short duration of exposure makes this unlikely. Other than low anthropometric percentiles, our patient was healthy and presented with foreign body impaction and celiac disease; the mechanistic basis for such association remains elusive. It is plausible that an altered esophageal motility secondary to LE could have resulted in the impaction; however such an association to LE has not been previously described in literature. Conclusion: With its true clinical implications remaining elusive, further large multicenter, prospective and case-control studies are required to determine etiology, characterize natural history, define diagnostic criteria and establish treatment strategies for lymphocytic esophagitis. Physicians should remain vigilant with regard to this entity and maintain a low threshold for diagnostic testing.

354 Lithium Battery ingestion: Aggressive or Conservative Management?. S. Rohatgi, C. Larson-Nath, G. Chelimsky, S. Werlin, Pediatric Gastroenterology, Medical College of Wisconsin, Milwaukee, Wisconsin, UNITED STATES. Background: For the past several years lithium battery ingestion has been a growing problem. The esophagus has been the organ most associated with severe damage by these batteries and there have been only a few cases reported describing damage to the gastric mucosa associated with batteries in the stomach. We present two consecutive cases of lithium battery ingestion associated with gastric ulcerations that occurred within a few hours of ingestion. Case 1: A 7 year old white male ingested a 20 mm lithium battery. Upper endoscopy was performed because of abdominal discomfort and failure of the battery to move from its position in the stomach over 12 hours. At endoscopy, 18 hours following ingestion, diffuse ulcerations and erosions were found in the fundus, body and antrum. Case 2: A 17 month old infant ingested a 10 mm lithium button battery. Upper endoscopy with battery removal was performed because of the age of the child. Ulcerations and erosion were present throughout the body of the stomach. Discussion: The current suggested guidelines by Litovitz and Brumbaugh in 2010 and 2011 respectively, and followed by the National Capital Poison Center recommend emergent removal of impacted button batteries from the esophagus but management of those that reach the stomach is conservative. Since only a few of these patients undergo endoscopy the frequency and degree of gastric damage is unknown. Hence, in light of our cases we propose that the guidelines be modified and that gastric button batteries be removed instead of managing them conservatively or waiting for 4 days before further investigation per the present guidelines. Conclusion: Within a few hours of ingestion lithium batteries retained in the stomach cause gastric erosions and ulcerations. Hence, early removal of ingested button batteries from the stomach should be considered.

355 Tale of toothbrush ingestion in two adolescent girls with bulimia in two weeks. R. Vithana, T. Sebastian, R. Hofer, S. Khan, Childrens National Medical Center, Washington, District of Columbia, UNITED STATES. BACKGROUND: Foreign body (FB) ingestions are common in pediatrics (more than 100,000/year), and often pass uneventfully through the gastrointestinal tract. However, toothbrush ingestions are rare with approximately 40 reported cases. We report our unusual experience with successful endoscopic intervention in 2 adolescent females with toothbrush ingestion presenting within a 2 week period. CASE 1: A 16 year old female with bulimia presented within 24 hr of accidental ingestion of a toothbrush handle after inducing emesis. She denied any symptoms. An ovoid 3.8cm long gastric FB was noted on abdominal x-ray. An esophagogastroduodenoscopy (EGD) under general anesthesia completed within 24hrs of the ingestion allowed successful retrieval of the 14 cm toothbrush portion by use of a snare. Mild esophageal erythema and superficial gastric ulceration were noted. She tolerated the procedure well and was discharged home the same day. Psychiatry was consulted regarding her bulimia and outpatient follow up was arranged. CASE 2: A 17 year old female, with bulimia and depression, presented with accidental ingestion of her intact battery powered toothbrush after inducing emesis. An endoscopic attempt to retrieve the toothbrush in the first 24 hr at another institution was unsuccessful after which she was transferred to us. She reported mild nausea. An abdominal x-ray showed a radiopaque FB, measuring 22 cm long with the bristles of the toothbrush extending via pylorus into the duodenal bulb .Repeat EGD was done within 48hrs of ingestion and the toothbrush was successfully removed with a snare through an over tube. A Magill forceps was used to retrieve the object once it was pulled up to the cricopharyngeus. Abrasions were appreciated in the duodenal bulb. She tolerated the procedure well and was discharged the following day. Psychiatry was actively involved during the hospital stay DISCUSSION: Endoscopic removal should be the preferred intervention in cases of toothbrush ingestion, and performed at the earliest possible opportunity due to the extremely low likelihood of spontaneous and uneventful passage through the GI tract. Management of toothbrush ingestion is particularly challenging because of the long, rigid shape, high probability of gastric retention and consequent complications over time. Radiographs may underestimate the dimensions due to partial radiolucency. Our experience emphasizes endoscopic retrieval as safe and successful with the anesthesia and surgical teams available for further assistance. The use of an over tube is also recommended to minimize esophageal trauma, and supported by our 2nd case. Of note, the toothbrush in case 2 is the longest one to be retrieved to our knowledge. Concurrent psychiatric disorders must be appropriately addressed to prevent recurrent events.

356 A Series of Detergent Pod Ingestions in Toddlers. T. Sebastian, K. Shirron, L.S. Conklin, Childrens National Medical Center, Washington, District of Columbia, UNITED STATES. Laundry detergent pods are brightly colored capsules comprised of a liquid detergent within a water-soluble membrane. The detergent is composed of sulfated and ethoxylated alcohols, with a pH of 6.8-7.4. Most of the reported cases involving laundry detergent pod exposure have been among children less than 5 years of age. A significant number of cases have been associated with vomiting and respiratory side effects, as well as mental status changes. In this case series, we describe five single-center cases of detergent pod ingestion, all Tide Pod™ brand, in children under 4 years of age, who presented and were admitted to Children’s National Medical Center over an 8 month period. Vomiting occurred in each of the 5 cases, and 3 of them also presented with respiratory symptoms. Three cases presented with respiratory symptoms, one with respiratory failure requiring mechanical ventilation likely due to aspiration pneumonitis. Two out of the 5 patients underwent upper endoscopy. One of these patients had grade 1 esophageal injury on endoscopy. One of the 5 patients also had associated mental status changes. Case Descriptions: 1: A 3 year- old who presented with emesis after ingestion. She had normal exam findings her diet was advanced and an EGD was not done. 2: A 21-month old who presented with stridor and drooling. EGD was normal. 3. A 14 month-old who presented with lethargy and respiratory distress requiring mechanical ventilation. Chest radiograph was concerning for aspiration pneumonia. Upper endoscopy revealed grade 1 esophageal injury. 4. An 11 month-old boy who presented with moderate respiratory distress, requiring oxygen via nasal cannula. Chest radiograph was normal. The patient improved within 24 hours and EGD was not done. 5. A 13 month-old who presented with emesis after ingestion. Had a normal physical exam and chest radiograph, hence was discharged within 24 hours without endoscopic evaluation. Laundry detergent pods have been a commercial success since their introduction in the US in 2010. Our case series highlights potentially serious respiratory sequelae, though esophageal and gastric mucosal injuries were not significant. Complications associated with detergent pod exposure reported in literature are eye injuries, respiratory failure, vomiting, and mental status changes. Vomiting is common and dysphagia has been reported following ingestion. Associated mental status changes may lead to a higher risk aspiration. Injury to the gastrointestinal tract has not been highlighted as a complication in reported cases. Since pH is neutral, endoscopy may not be necessary in all children with detergent pod ingestion, particularly in those with no dysphagia or respiratory symptoms. Physicians and parents should be aware of the potential multisystem effects of detergent pod ingestion in young children.

357 Detergent pod ingestion causing airway and gastrointestinal injury: Case report and literature review. J. Silverman, J. Butzner, University of Calgary , Calgary, Alberta, CANADAJ. Silverman, J. Butzner, Alberta Children's Hospital, Calgary, Alberta, CANADA. Laundry detergent “pods” have recently become popular in North America. Their small size and bright colors have contributed to cases of toddler ingestion resulting in gastrointestinal and respiratory complications. Here we present a recent case and review published reports. A 21 month-old presented to our center after ingesting the contents of a detergent pod. She developed emesis followed by refusal to drink and drooling. She was brought to our ER where she developed progressive stridor and respiratory distress leading to the need for non-invasive respiratory support including Heliox. Laryngoscopy revealed mild burn-like injury and associated edema of the epiglottis and both aryepiglottic folds. Upper endoscopy showed erythema and mild edema of the upper and lower esophagus as well as at the pylorus, also in keeping with mild burn injury. Our local poison control centre was involved and we reviewed the product’s safety sheets. The pH of the product is listed at 7.6. The pattern of injury was very consistent with contact irritation without deep caustic injury seen in caustic household product ingestions. She remained intubated post-procedure for approximately 24 hours to allow for resolution of airway edema and was observed on the ward for a further 24 hours. On day 2 she developed a right upper lobe infiltrate on xray suggestive of aspiration pneumonia or chemical pneumonitis. She was treated with oral antibiotics and a proton pump inhibitor. At the time of discharge she was well and eating and drinking normally. Reports of laundry pod ingestions in the literature are quite limited. The majority of reported ingestions have occurred in children less than 5 years of age. Case reports indicate that respiratory distress and airway swelling requiring intubation or non-invasive support are the most commonly reported severe complications. Significant gastrointestinal complications including abdominal pain and feeding and swallowing difficulties requiring nasogastric feeding have also been reported. Laundry pods represent an emerging ingestion for risk in young children and may result in significant gastrointestinal and respiratory complications despite their neutral pH. Reports of these ingestions should be made to the manufacturer, the US Consumer Product Safety Commission or Health Canada- Consumer Product Safety. Increased awareness and vigilance of laundry pod ingestions may lead to product changes to reduce this risk.

358 Delayed gastric emptying in an adolescent female with ectopic pancreas. K. Sturgeon, W. San Pablo, S. Atkin, C. Snyder, Children's Mercy Hospital, Kansas City, Missouri, UNITED STATES. Ectopic pancreatic tissue, frequently referred to as a pancreatic rest, is a relatively common finding seen during EGD and reported in autopsy series to occur at an average frequency of 1-2% . The majority of ectopic pancreatic tissue is found in the foregut. Pancreatic rests can be found in a variety of locations including ileum, gallbladder, spleen and lung. Seventy five percent are found in the stomach; commonly the prepyloric gastric antrum. Most pancreatic rests are found incidentally. The majority are asymptomatic. Occasionally, etopic pancreatic tissue can be associated with symptoms of abdominal pain, dyspepsia and gastrointestinal bleeding. The tissue may also cause biliary or gastric outlet obstruction. We describe a previously healthy, active teenage female with abdominal pain found to have gastric outlet obstruction secondary to the presence of intramural pancreatic tissue within the pylorus. A 17 year old healthy, active female presented to her primary care physician with acute onset abdominal pain while playing softball. The pain was localized to the upper and mid abdomen, worsened by solid food intake and associated with occasional vomiting. Initially, the patient was counseled that symptoms were consistent with a viral illness. As symptoms persisted, she had an abdominal ultrasound which was normal. She underwent upper endoscopy where she was found to have a 2-3 cm polyp obstructing the gastric outlet as well as a strictured pylorus. The polyp was biopsied and balloon dilation of the pylorus was performed. When symptoms persisted, she was admitted for further evaluation. Repeat EGD was performed, and more than 50% of the polyp was removed. The strictured pylorus was again observed. The endoscope could not be advanced past the pylorus. No colonic polyps were observed. The patient was discharged home after endoscopy and her symptoms persisted. This was felt to be due to gastric outlet obstruction from the strictured pylorus. CT scan was unrevealing and patient was taken to surgery where, ultimately, pyloric resection with Billroth-I anastomosis was performed. Pathology revealed ectopic pancreatic tissue, and patient’s symptoms have markedly improved. This highlights a rare case of ectopic pancreatic tissue causing pyloric stricture and gastric outlet obstruction. This should be considered when the etiology of pyloric stricture is unclear.

359 Acquired Gastric Diverticulum in a Teenage Boy. J.T. Stutts, Department of Pediatrics, Division of Pediatric Gastroenterology, University of Louisville, Louisville, Kentucky, UNITED STATES. A 14-year-old boy presented with a 6 month history of epigastric abdominal pain, vomiting and weight loss. He had been placed on a daily proton pump inhibitor 4 months prior to presentation, with resultant improvement but not resolution of his vomiting. His abdominal pain had not improved with acid suppression. He had decreased his oral intake due to pain associated with eating. A 13 pound weight loss resulted. His physical examination was normal. His previous laboratory evaluation revealed a normal complete blood count with differential, comprehensive metabolic panel, urinalysis, amylase and lipase. A previous ultrasound was normal, but was limited to the liver and kidneys. Endoscopy revealed a blind pouch near the pylorus on the greater curvature of the stomach. The opening of the diverticulum could not be intubated. Duodenal, gastric and esophageal biopsies were normal. A subsequent upper gastrointestinal (UGI) contrast study was performed to better evaluate the depth of the diverticulum, but failed to confirm the defect. Double contrast UGI with air also failed to reveal the diverticulum. His proton pump inhibitor dose was increased with subsequent resolution of abdominal pain, vomiting and weight loss. The pediatric literature suggests the limitation of upper endoscopy and promotion of UGI contrast study as the optimal modality for diagnosis.(1) The literature further suggests the use of air contrast as an added technique for increasing the diagnostic capability of the UGI.(2) Despite these claims, our case underscores the limitation of UGI in consistently diagnosing gastric diverticula. Our case further supports upper endoscopy as having a continued important role in the diagnosis of these extremely rare diverticula in children. There is not a consensus on management. Due to the low complication rate, acid suppression has been advocated.(3) However, surgical intervention has been recommended to differentiate gastric diverticula from peptic ulcer disease or malignancy, prevent future complications or when acid suppression fails to resolve symptoms.(1) Due to the low gastric malignancy rate in children, as well as the resolution of symptoms in our patient, we have elected to continue acid suppression with close follow-up. 1. Rodeberg DA, et al. Gastric Diverticulum: a series of four pediatric patients. J Ped Gastr Nutr 2002;34:564-567. 2. Dickinson RJ, Freeman AH. Partial gastric diverticula: radiological and endoscopic features in six patients. Gut 1986;27:954-957. 3. Palmer ED. Gastric diverticulosis. Am Fam Physician 1973;7:114-117.

360 Eosinophilic Esophagitis and Achalasia a Pediatric Case. C.S. Welty, J. Fuentabella, Children's Hospital and Research Center Oakland, Oakland, California, UNITED STATES. A previously healthy 6-year-old male presented with a 3-month history of dysphagia. The patient was evaluated in the ED and had a CXR which showed some anterior bowing of the trachea. Based on this XRAY there was a significant concern for gastroesophageal outlet obstruction. It was decided to complete a barium swallow study to further evaluate. His barium swallow study was notable for distension of the esophagus with essentially complete obstruction at the gastroesophageal junction, findings concerning for achalasia. The patient was admitted to the hospital for NG feeds and upper endoscopy with biopsy. His upper endoscopy was notable for a tight LES and a patulous esophagus. A food bolus was noted in the distal esophagus and was removed with a Roth net. His esophageal biopsy showed up to 150 eosinophils per high power field, in the distal esophagus. Based primarily on histology the patient was given the diagnosis of Eosinophilic Esophagitis (EoE). He was started on nasogastric feeds with a hypoallergenic formula and treated with oral Budesonide and a proton pump inhibitor. He was referred to an allergist for patch testing, which showed marked reactivity to multiple foods. His symptoms gradually improved and repeat endoscopy done 2 months later showed complete resolution of his esophageal eosinophilia. He was then sent for manometry study to evaluate for esophageal dysmotility and achalasia. Manometry was completed and demonstrated simultaneous nonpropagating contractions with swallows in the esophagus and absent relaxation of the lower esophageal sphincter, consistent with the diagnosis of achalasia. In the adult literature many esophageal dysmotility disorders have been described in conjunction with EoE, including achalasia. However, in pediatrics only ineffective and low amplitude esophageal contractions have been described. This case report demonstrates EoE and achalasia as comorbid conditions in the pediatric population. This further raises the question of whether EoE can predispose to achalasia, which has been suggested in the adult literature.

Hepatobiiary/Transplant 368 Two cases of papillary carcinoma of the thyroid gland after treatment with peginterferon and ribavirin for chronic HCV infection.. R. Raza, M.M. Jonas, Medicine-GI, Hepatology & Nutrition, Boston Children's Hospital, Boston, Massachusetts, UNITED STATES. Chronic HCV infection has been associated with a multitude of extra-hepatic manifestations including papillary thyroid carcinoma; however this has not been reported in children or young adults with HCV infection. We encountered two cases of papillary carcinoma of the thyroid post peginterferon and ribavirin therapy for HCV infection. A 17 year old boy with severe hemophilia A acquired chronic hepatitis C (genotype 1b) during early childhood from therapeutic blood or Factor VIII infusions. There was no other significant past medical or family history of thyroid disease. He was treated with peginterferon alfa-2a and ribavirin (PEG/RV) for 48 weeks and achieved early virologic response (EVR), end of treatment response (ETR) and sustained virologic response (SVR); no dose reduction was required during the course of the therapy. He had no thyroid dysfunction before initiation of HCV treatment. At week 36 of treatment, he developed elevated TSH (58 µU/ml) without clinical hypothyroidism and, hence, was started on levothyroxine 50 mcg daily at week 40 of treatment. The TSH was back to normal (4.26 µU/ml) by week 48 and levothyroxine was stopped 3 months post completion of HCV therapy. TSH was only minimally elevated to 5.65 µU/ml 6 months after completing HCV therapy. He presented with a thyroid mass 26 months after cessation of therapy, and underwent total thyroidectomy. Pathology showed papillary carcinoma of thyroid. He subsequently received radioactive iodine and, at this writing, was doing well. A 22 year old man who had a history of bone marrow transplant at the age of 3 for neuroblastoma had multiple blood transfusions early in life and acquired HCV infection (genotype 1). His oncologic treatment included total body irradiation. He was later found to have growth hormone deficiency, bilateral subcapsular cataracts, significant bilateral hearing loss and a vestibular Schwannoma. He presented with thyroid nodules at age 21. Biopsies of the thyroid nodules at that time were considered inconclusive and he was treated with thyroxine due to clinical hypothyroidism. There was no family history of thyroid disease. He subsequently received peginterferon alfa-2a and RV for 36 weeks. During the course of the therapy, dose for PEG/RV had to be reduced multiple times by 50% due to hematological complications. At week 16, the treatment was stopped for a week because his hematocrit dropped to 19.6% from 39.8% and the platelet count dropped to 61,000/mm3 from 195,000/mm3. He was given two units of packed red blood cells and erythropoetin alfa. Treatment was eventually stopped at week 36 due to ongoing hematological complications. He achieved EVR, ETR and SVR. A total thyroidectomy was performed 7 months after cessation of therapy. The thyroidectomy specimen was notable for papillary thyroid carcinoma. He subsequently received radioactive iodine and, at this writing, was doing well. Cases presented above document the rare occurrence of papillary carcinoma of thyroid in chronic HCV infected patients who presented at Boston Children’s Hospital. Thyroid monitoring should be done carefully in the follow up of these patients.

369 Massive Intrahepatic Fluid Collection Secondary to Umbilical Venous Catheter. J. Yeh, L. Wozniak, J. Vargas, Pediatric Gastroenterology, Hepatology, and Nutrition, UCLA, Los Angeles, California, UNITED STATESI. Boechat, Pediatric Radiology, UCLA, Los Angeles, California, UNITED STATESM. Touma, Neonatology and Developmental Biology, UCLA, Los Angeles, California, UNITED STATES. Background: Umbilical venous catheter (UVC) placement is a common procedure in neonatology. Although infrequent, hepatic complications including portal vein thrombosis, biliary-venous fistula, liver laceration, abscess, necrosis, and hematoma have been reported with significant risk for long term morbidity. Case Presentation: A 36 week female infant with hypoxic ischemic encephalopathy was admitted to the NICU. A UVC was placed, and x- ray showed malposition of the catheter in the left branch of the portal vein. After repositioning, the tip appeared to terminate at T9 but below the diaphragm. Total parental nutrition (TPN) and intralipid were initiated via the UVC. At 10 days of age the infant developed abdominal distention. A chest x-ray revealed a pleural effusion while abdominal ultrasound revealed ascites. Pleural and peritoneal drains were placed, and analysis revealed high triglycerides. The UVC was promptly removed. Abdominal imaging including ultrasound, CT, and MRI ultimately revealed a 72 x 80 x 38 mm complex cystic lesion containing a pocket of air and heterogeneous debris. Laboratory evaluation revealed hepatocellular injury and cholestasis. A conservative approach was adopted without intervention. Hepatic function tests and serial ultrasounds were monitored. The patient was discharged home at 48 days and follow up ultrasound at 8 months showed the lesion had decreased in size to 40 x 30 mm with a patent portal vein. Discussion: TPN extravasation and hepatic injury are rare complications of UVC placement. In this patient, the UVC was not placed above the diaphragm. The subsequent ascites and pleural effusion as well as the massive liver lesion were likely the result of extravasation of TPN within the liver due to UVC malpositioning. Hyperosmolar solutions such as TPN may produce both vascular and hepatocellular injury. Potential long term complications include portal hypertension. Correct placement of UVC, ideally above the diaphragm at the right atrial and inferior vena cava junction, prior to its use is essential in preventing serious complications. Awareness of potential hepatic complications involving use of hyperosmolar fluids is paramount.

370 Hepatic Sarcoidosis Presenting with Hematemesis and Anemia in a Young Adult Patient. C.A. Reynolds, R.T. Fischer, Gastroenterology, Hepatology and Transplant Medicine, Children's Mercy Hospital, Kansas City, Missouri, UNITED STATES. Sarcoidosis is a multisystem, granulomatous disease which most frequently presents with hilar lymphadenopathy, pulmonary infiltration, and ocular and cutaneous lesions. The disease most frequently affects the lungs, but any organ system can be involved. Rarely, hepatic sarcoidosis results in symptomatic liver disease and portal hypertension. Hepatic granulomatous disease leading to portal hypertension is seen in less than 1% of sarcoidosis patients in the adult population), but has been known to lead to bleeding varices or even liver transplant. Although pediatric sarcoidosis results in hepatosplenomegaly in 43% of patients, clinical hepatic manifestations are not as apparent and hepatic granulomas were not seen in a retrospective pediatric sarcoidosis population study. Herein, we present a case of a 22-year-old male with history significant for DiGeorge syndrome, hypoparathyoidism, 3 failed thymic transplants, and known pulmonary, lymphatic and splenic granulomas since the age of 6. He presented to a pediatric emergency center with symptoms of syncope, coffee-ground emesis and hemoglobin of 7.7 gm/dL. Endoscopy demonstrated one mild and two moderate esophageal varices with portal hypertensive gastropathy and duodenitis. He underwent band ligation to control the bleeding with success. Hepatic ultrasound with Doppler demonstrated normal portal flow. Liver core biopsy showed chronic hepatitis with mild activity and multiple non-necrotizing granulomata, consistent with sarcoidosis. He is now on chronic steroid therapy for management of his sarcoidosis. Conclusion: Variceal bleeding is a potentially deadly complication of portal hypertension in children and adults. Pediatric and young adult patients with sarcoidosis should be considered for screening for portal hypertension on a regular basis to prevent catastrophic consequences.

371 Serum Sickness Following Rabbit Anti-Thymoglobulin (ATG) Induction For Liver Transplant. A. Rodriguez, W. Berquist, Pediatric Gastroenterology, Hepatology and Nutrition, Stanford University, Palo Alto, California, UNITED STATESJ. Mombourquette, J. Frankovich, Pediatric Rheumatology, Stanford University, Palo Alto, California, UNITED STATES. Serum sickness is a type III immune complex hypersensitivity reaction that results from exposure to foreign proteins. The most common causes are drugs such as antibiotics, vaccines and antitoxins. Polyclonal and monoclonal antibodies prepared from horse, rabbit or mouse serum are also associated with serum sickness. This association has been reported in 7-27% kidney transplant patients in whom ATG was used as induction therapy. In liver transplants this finding is more rare, with no reported cases in children. Here we present a case of serum sickness in a pediatric liver transplant patient following ATG induction therapy The patient is a 15 y/o female who initially presented to our hospital in acute liver failure. She was previously healthy with no significant medical history. On presentation she was coagulopathic (INR 3.0) and encephalopathic with elevated ammonia levels (197). Despite a thorough workup there was no clear etiology for her liver failure except for a mildly elevated ANA which was concerning for a possible immune-mediated hepatitis. Over the next few days her clinical course worsened so she underwent an orthotopic liver transplant 7 days after initial presentation. For induction therapy after transplant she received 4 doses of ATG for a total of 4.5mg/kg. She was started on Prograf and Cellcept in addition to prednisone due to the immune mediated etiology. After transplant her coagulopathy and encephalopathy improved and on POD 10 she was discharged home. Eight days after discharge, she was readmitted due to high fevers for 1 day with diffuse joint pain including severe jaw pain. On initial workup her labs were within normal limits except for elevated ESR (>140) and CRP (61) and a low magnesium level of 1.9. Her pain continued despite magnesium repletion. Over the next 2 days her pain worsened, and she developed swelling and an erythematous rash around her DIP and PIP joints on both her hands and feet. In collaboration with rheumatology she underwent an extensive workup including complement levels, immune complex markers and rheumatoid factor that were all within normal limits. Due to the clinical symptoms and laboratory findings a diagnosis of serum sickness was established and her prednisone was increased to 60mg daily. Over the course of 1 week her symptoms resolved and by HD 19 she was discharged home with a steroid taper. This case illustrates the potential for serum sickness in pediatric liver transplant patients following ATG for induction therapy. Given the increasing frequency of ATG use after liver transplant, serum sickness should be considered in any of these patients who presents with arthalgias, especially jaw pain, and fever. This patient also had exposure to rabbits prior to her transplant which is a known risk factor for serum sickness after receiving ATG. However, given the rarity of serum sickness the avoidance of ATG among those with a history of exposure is not warranted.

372 Fibrinogen Storage Disease Presenting in Infancy Associated with Hypofibrinogenemia and Typical EM Findings. M. Rodriguez, L.M. Bass, Department of Gastroenterology, Hepatology, and Nutrition, Ann & Robert H. Lurie Children's Hospital of Chicago/Northwestern University Feinberg School of Medicine, Chicago, Illinois, UNITED STATESH. Melin-Aldana, Department of Pathology, Ann & Robert H. Lurie Children's Hospital of Chicago/Northwestern University Feinberg School of Medicine, Chicago, Illinois, UNITED STATESK. Bove, Department of Pediatric Pathology, Cincinnati Children’s Hospital, Cincinnati, Ohio, UNITED STATES. Fibrinogen storage disease (FSD) is a rare storage disease that can cause significant liver disease in adults due to accumulated mutated protein in the endoplasmic reticulum (ER). FSD has been described in asymptomatic pediatric patients associated with and without hypofibrinogenemia. Electron microscopy (EM) findings in these patients have been classified into 3 different categories. Type I inclusions are described as tubular structures arranged in curvilinear bundles that resemble a fingerprint like pattern within dilated cisternae of the rough ER (RER). Type II inclusions appear as granular fibrillar material within dilated cisternae of the RER. Type III inclusions demonstrate tubular structures similar to Type I in the center while there is material similar to Type II in the periphery. Here we present 2 pediatric cases associated with hypofibrinogenemia and EM findings. Cases Patient 1 presented at 8 months of age with a 2 month history of elevated AST and ALT. Physical exam was normal; laboratory investigation revealed mildly prolonged PT and INR. Screening for coagulation disorders, thyroid disease, viral hepatitis, and inborn errors of organic acid and amino acid metabolism was negative. Percutaneous liver biopsy showed mild periportal fibrosis and mild lymphocytic inflammation. EM revealed dilated smooth and rough ER. Larger cisternae within the RER contained moderately electron-dense material with a plate like crystalline morphology that resembled fingerprints, consistent with Type I inclusions. Plasma fibrinogen was 60 mg/dL, normal 150-400 mg/dL; it has remained at 60 mg/dL over 2 years of follow up. Patient 2 presented at 2 years of age with an 11 month history of elevated AST, ALT, ALP, and GGT. Physical exam was normal; laboratory investigation revealed prolonged PT and INR. Screening for viral hepatitis, Wilson’s disease, A1AT deficiency, autoimmune hepatitis, and celiac disease was negative. Serum bile acids were normal. Percutaneous liver biopsy showed mild periportal fibrosis, focal bridging, and mild chronic inflammation. EM revealed prominently dilated cisternae mainly of the smooth ER in all hepatocytes. All cisternae contained moderately dense finely granular crystallized proteinaceous material. Plasma fibrinogen was 60 mg/dL, normal 180-410 mg/dL. Discussion We present 2 cases of pediatric patients with elevated aminotransferases, hypofibrinogenemia, and EM findings consistent with FSD. The first case is representative of the previously described Type I inclusions associated with hypofibrinogenemia, mild transaminitis, and prolonged PT without clinical signs of coagulation defect. However, the second case involved mostly the smooth ER, which differs from previously reported cases. Fibrinogen storage disease should be considered in a clinically well pediatric patient who presents with elevated aminotransferases and prolonged PT. Patients with FSD should be monitored for the development of clinically significant liver disease as they grow older. Fibrinogen levels and electron microscopy may be helpful in making this diagnosis. Cryoprecipitate infusion should be utilized for a major bleeding episode.

373 Hepatitis C Virus-Autoimmune Hepatitis Overlap Syndrome in an Adolescent. D. Rosen, J. Chu, S. Rose, R. Arnon, Division of Pediatric Hepatology, Mount Sinai Medical Center, New York, New York, UNITED STATESD. Rosen, Department of Pediatric Gastroenterology, Mount Sinai Medical Center, New York, New York, UNITED STATESJ. Chu, S. Rose, R. Arnon, Recanati/Miller Transplantation Institute, Mount Sinai Medical Center, New York, New York, UNITED STATESS. Lee, Division of Hospital Medicine, Connecticut Children's Medical Center, Hartford, Connecticut, UNITED STATES. Hepatitis C virus-autoimmune hepatitis overlap syndrome (HCV-AIH) is characterized by clinical, immunologic and histologic features of both diseases, not simply by the presence of autoantibodies as often seen with HCV infection alone. Treatment of HCV-AIH presents a clinical dilemma due to the contradictory treatment approaches for each disease; administration of IFN can exacerbate underlying AIH, while corticosteroids have been shown to increase HCV viremia and precipitate fulminant hepatic failure. There are no published cases on the treatment approach to the pediatric patient with HCV-AIH. An 11 year-old female was referred for elevated transaminases with positive autoimmune markers and HCV genotype 1b. Initial labs were: HCV viral load 36,100 IU/ml, ALT 262 IU/ml, AST 141 IU/ml, albumin 4.5 gm/dL, direct bilirubin 0.1 mg/dL, INR 1, ANA negative, anti-smooth muscle Ab positive (1:320), anti-LKM Ab positive and quantitative IgG 1.7 gm/dL. Liver biopsy showed chronic inflammation in portal tracts and mild interface hepatitis consistent with chronic HCV infection with features of AIH. A diagnosis of HCV-AIH type 2 was made. She was first treated for HCV with ribavirin 15mg/kg/day and weekly subcutaneous Pegylated Interferon-2b 60 mcg/M2 and was a rapid responder after 4 weeks. At 8 weeks serum HCV RNA remained negative but she had elevation of liver enzymes (ALT 369 IU/mL, AST 401 IU/mL) and was started on prednisone 20 mg daily. Liver enzymes normalized and steroids were tapered. She completed 48 weeks of antiviral treatment and achieved sustained virological response. She was started on azathioprine 1mg/kg/day with prednisone to control her AIH, in part due to non-compliance. She is now 36 months post-HCV treatment with normal liver enzymes, ASMA positive and anti-LKM Ab positive on azathioprine and prednisone 5mg daily. Serum HCV RNA remains negative. The adult literature favors initiating treatment with corticosteroids to treat the AIH followed by treatment for HCV. Here we present the first pediatric case report of a child with HCV-AIH overlap syndrome that successfully responded to initial antiviral treatment of HCV followed by prednisone to control AIH.

374 BRTO (Balloon-Occluded Retrograde Transvenous Obliteration) for Recurrent Gastric Variceal Bleeding in an Adolescent. D. Rosen, J. Chu, R. Arnon, Division of Pediatric Hepatology, Mount Sinai Medical Center, New York, New York, UNITED STATESD. Rosen, Department of Pediatric Gastroenterology, Mount Sinai Medical Center, New York, New York, UNITED STATESJ. Chu, K. Iyer, J. Moon, R. Arnon, Recanati/Miller Transplantation Institute, Mount Sinai Medical Center, New York, New York, UNITED STATESR. Patel, Division of Interventional Radiology, Mount Sinai Medical Center, New York, New York, UNITED STATES. Gastric variceal bleeding is associated with high morbidity and mortality. Balloon-occluded retrograde transvenous obliteration (BRTO) is a new and effective treatment to control bleeding gastric varices that has not yet been reported in pediatrics. A 14 year old female with no prior medical history presented with large volume hematemesis. Laboratory results revealed anemia with Hgb 8.9 g/dL, AST 15 IU/L, ALT 19 IU/L, INR 1.3 and albumin 2.7 gm/dL. Following fluid resuscitation and blood transfusion, esophagogastroduodenoscopy showed grade 2 esophageal varices and large fundal gastric varices along with 1500 ml of blood in the stomach, but no active bleeding. The patient was started on an octreotide drip. Abdominal ultrasound and CT scan were consistent with cirrhosis. Investigation was negative for autoimmune hepatitis, viral hepatitis, and Wilson disease. Octreotide was weaned off but resumed on hospital day 3 following a large episode of hematemesis. Over the next few days, hematemesis persisted and was refractory to endoscopic injection of cyanoacrylate (0.6cc total) into a single large fundal gastric varix. Due to inadequate size of the hepatic veins she was not a candidate for TIPS and underwent a successful BRTO procedure on hospital day 12. In brief, a balloon catheter was inflated within a spontaneous left gastrorenal (portosystemic) shunt and 70ml of foam sclerosant was administered into the gastric varices. The balloon catheter was left in place overnight and was deflated and removed the following day without complications. Octreotide drip was discontinued following the procedure and she was discharged in stable condition. The patient had no further episodes of hematemesis. Repeat EGD one month later showed multiple grade 2 esophageal varices and few small fundal varices that were decreased in size from prior EGD. One week later she underwent an orthotopic liver transplant from a deceased donor. The explanted liver pathology confirmed cirrhosis of unknown etiology. This is the first report of BRTO in a pediatric patient that demonstrates BRTO is a viable treatment option for intractable gastric variceal bleeding.

375 Spontaneous Bile Duct Perforation Presenting as Acute Abdomen. D.S. Say, M. Sivagnanam, Pediatrics, University of California, San Diego, San Diego, California, UNITED STATES. A 6-month-old previously healthy male infant with a history of prematurity was hospitalized for evaluation of progressive abdominal distention, respiratory distress, and jaundice, initially suspicious for a surgical abdomen with acute hepatic dysfunction. Laboratory studies revealed normal liver synthetic function while an initial CT scan of the abdomen demonstrated massive ascites with evidence of multiple gallstones, both within the gallbladder and in the common bile duct. A therapeutic paracentesis yielded copious amounts of bilious drainage, suggesting a biliary leak that was later confirmed both radiographically and during an exploratory laparotomy. The infant was ultimately diagnosed with ascites and peritonitis secondary to spontaneous perforation of the common bile duct. He underwent successful surgical repair of the duct with T-tube drainage and placement of a cholecystotomy tube. While spontaneous bile duct perforation is an uncommon condition in the pediatric population, it remains one of the most common surgical causes of jaundice in infancy. This diagnosis should be suspected in any infant with insidious onset of jaundice and abdominal distention, as prompt recognition and surgical intervention can be lifesaving.

376 Acalculous Choleycystitis Complicating a Primary Ebstein-Barr Virus Infection in a Female Adolescent. N. Davis, E.A. Schaefer, Pediatrics, Peyton Manning Children's Hospital, Indianapolis, Indiana, UNITED STATESE.A. Schaefer, Pediatric Gastroenterology, Peyton Manning Children's Hospital at St. Vincent, Indianapolis, Indiana, UNITED STATES. Background: Acute acalculous cholecystitis (AAC) is rarely seen in childhood. It is often associated with complex medical or surgical conditions, but has been reported in association with viral infections, including Epstein-Barr virus (EBV). Ultrasound is the most reliable imaging study for diagnosis and can also be used to follow the course of the disease. Therapeutic management ranges from expectant to surgical intervention and is specific to each patient's disease course. Case Report: We report a previously healthy 14-year-old female who was admitted with a two-week history of right upper quadrant pain, sore throat, and fatigue. Physical exam revealed scleral icterus, obesity, and right upper quadrant tenderness. Bloodwork revealed elevated liver enzymes, gamma-glutamyl transpeptidase and bilirubin. Abdominal ultrasound revealed a markedly thickened gall bladder wall (>1cm) without sludge or gallstones consistent with acute acalculous cholecystitis. She was treated with expectant management, demonstrated clinical improvement, and was discharged home within four days of admission. Serologic testing confirmed a primary EBV infection. Liver function tests, bilirubin, and imaging all returned to baseline within a month of discharge. Conclusion: To our knowledge, this patient marks the 13th reported case of AAC during a primary EBV infection, and only the 3rd reported case within the United States. Currently, there are no consistent guidelines on the diagnosis, monitoring, and management of AAC secondary to primary EBV. By raising awareness of gallbladder involvement during EBV infection, we hope to avoid unnecessary surgical procedures and/or overuse of antibiotics.

377 Our experience of ABO-Incompatible Living Related Liver Transplantation in Children.. M.A. Shagrani, M. Burdelski, T. Al goufi, F. Zahr Eldeen, H. Al Bahili , M. Al Sebayel , D. Broering , Department of Liver and Small bowel Transplantation and Hepatobiliary –Pancreatic Surgery, King Faisal Specialty Hospital , Riyadh, SAUDI ARABIA. INTRODUCTION: The no. of children waiting for liver transplantation without having a suitable living donor is increasing .Spilt liver transplantation from deceased donors is very limited due to shortage of such high quality donor organs.A Second option would be to perform ABO-Incompatible (ABO-I) transplantation in children where only an ABO-I liver donor is available. METHODS: Blood type and cross-match with an irregular antibody screen were performed prior to transplantation.Isohemagglutinins titers were drawn pretransplantation.Isohemagglutinins titers against ABO antigen were monitored post transplantation according to our protocol. We defined indications for plasmapheresis as increased isohemagglutinin titers associated with allograft dysfunction ,picture of hemolysis and histological evidence of rejection . POSTTRANSPLANTATION IMMUNOSUPPRESSION PROTOCOL: Consisted of two doses of basiliximab day 1 and day 4. I.V.Methylprednisolne was given during anhepatic phase at the time of biliary anastomosis, followed by prednisolone tapered slowly. Mycophenolate mofetil was started at 10-20 mg/kg/day on postoperative day 1and increased to reach 600mg/m2. Oral Tacrolimus began on postoperative day7 aiming for a trough level 7-9 ng/ml at the first 3 months.In the absence of rejection, steroids were planned to discontinue within 12 months after transplantation. As A protocol blood bank supply our patients if needed packed red blood cells from O positive donors and blood products from AB donors to avoid any increment in the ABO Isotitre. RESULT: Posttransplantation surgical complications were nil either vascular or biliary complications. No acute cellular or antibody mediated rejection episodes were identified, The 1-yr actuarial patient and graft survival for ABO-I were 100 %, no patients required any plasmapheresis either pre- or Posttransplantation or extra medical therapy i.e. (I.V. Rituximab or immunoglobulins). All patients continued to have very low ABO-Isotitre for the median follow up period of 147.5 days. DISCUSSION: Historically, ABO-I grafts have been limited to emergency situations and have resulted in inferior patient and graft survival. We are reporting here the same experience as in Japan and Atlanta(USA) plus that no one of our patients require plasmapheresis, IV immunoglobulin or Rituximab,. As the firs series to be done in the Middle East even though with the small no. but our conclusion that pediatric recipients patients with ABO-I grafts have the same outcomes comparable or superior with ABO-compatible graft without any difference in rejection or in vascular or biliary complications. Crossing blood groups will play significant role in avoiding pediatric waiting mortality in emergent patients .We do believe in role of HLA matching in our cases . Demographic data of the ABO-I cases

378 An Unusual Case of Ascites After Kasai Procedure. L. Sifuentes-Dominguez, N. Rodriguez-Baez, Pediatric gastroenterology, UT Southwestern, Dallas, Texas, UNITED STATES. Background Biliary leaks are uncommon complications after biliary surgery. We present a case of biliary leak after Kasai portoenterostomy (KP) leading to ascites. Case Report A 16 week old female infant was diagnosed with biliary atresia at 2 months of age after presenting with cholestasis and elevated aminotransferases. She underwent Kasai procedure at day of life 65. Surgery was uneventful. She was discharged home 9 days after surgery. One month after surgery, she developed abdominal distension, feeding intolerance and acholic stools. Bilirubin levels normalized 46 days after the KP. Physical examination showed a non-jaundiced female with significant abdominal distention and mild hepatosplenomegaly. Her weight was in the <3rd percentile. Laboratory tests revealed moderate elevation of the aminotransferases, elevated gamma-glutamyl transpeptidase, but normal albumin, bilirubin and INR. Abdominal ultrasound revealed a large complex appearing fluid collection extending from the inferior surface of the liver inferiorly to the pelvis. A 99mTc-HIDA scan showed gradual accumulation of activity over the peritoneal region consistent with bile extravasation into the abdomen causing ascites. Patient was diagnosed with a biliary leak. She was managed conservatively with bowel rest and parenteral nutrition. She underwent paracentesis that showed the presence of Candida albicans, Klebsiella oxytoca and Stenotrophomonas maltophila. She was placed on intravenous antibiotics. A paracentesis catheter was placed to help with her abdominal distension with daily output of 30 - 45mL of ascitic fluid. Output significantly decreased 10 days after she was placed on bowel rest. Catheter was removed 28 days after diagnosis of the biliary leak. Patient was discharged home. She was seen as outpatient 1 week after removal of the catheter. Patient has no evidence of reaccumulation of ascites. Discussion We present a case of ascites after KP. Biliary leaks are uncommon complications of biliary surgery. The case is unusual as it occurred 1 month after KP. Medical literature regarding KP anastomotic leaks is scarce and most management guidelines stem from bile leaks from other conditions, particularly post-liver transplant patients. All KP anastomotic leaks in published clinical series required KP revision. This patient was treated successfully with conservative management with external drainage and bowel rest.

379 Wilson Disease: A Novel Mutation in the ATP7B gene. L. Sifuentes-Dominguez, N. Rodriguez-Baez, J. Andersen, Pediatric gastroenterology, UT Southwestern, Dallas, Texas, UNITED STATESD. Desai, Pediatric Surgery, UT Southwestern, Dallas, Texas, UNITED STATES. Background Wilson disease (WD), a disorder of copper metabolism, is caused by mutations in the ATP7B gene, a copper transporting ATPase. We describe a novel mutation in exon 11 of the ATP7B gene in an Asian family associated with a more aggressive course of the disease. Case Report A 15 year old Burmese refugee female, with no significant medical history, was admitted for a 4-day history of jaundice, right upper quadrant abdominal pain and non-bloody, non-bilious emesis. Physical examination was significant for scleral icterus, hepatomegaly, generalized jaundice and an intact neurological exam with no Kayser-Fleisher rings. Admission studies showed mild elevation of the aminotransferases, cholestasis, and elevated INR. Studies were negative for Hepatitis A, B, C, CMV, EBV, HHV6, VZV, HSV, and Enterovirus. Alpha-1-antitrypsin phenotype was normal. Autoimmune hepatitis markers were negative. She had low ceruloplasmin levels and elevated copper in a 24-hour urine collection. Liver biopsy showed chronic active hepatitis with portal and lobular inflammation, bridging portal fibrosis, steatosis, ballooning degeneration, and hepatocellular and intracanalicular cholestasis. Hepatic copper content was elevated at 1361 ug/g. Findings were consistent with Wilson disease. Genetic analysis showed a novel mutation in the ATP7B gene (nonsense homozygous mutation to exon 11 of ATP7B gene with a premature stop codon, characterized by nucleotide change c2692 C>T, amino acid change p.Q898X). Her family history was significant for a brother with similar complaints who died at age 14 of liver disease. She was placed on chelation therapy. However, she developed gastrointestinal bleeding secondary to trientine and she was transitioned to penicillamine. She continued with progressive coagulopathy and worsening liver disease. She underwent orthotopic liver transplantation 30 days after admission. Her explanted liver showed cirrhosis. Her post-transplant course was uncomplicated and she was discharged on immune suppression with tacrolimus. Discussion This case describes a novel mutation which appears to be associated with predominant hepatic features, aggressive liver disease and poor response to conventional chelation therapy. Studies have shown that nonsense mutations are associated with a more aggressive disease course. The most common mutation for Wilson disease seen in Chinese and ethnically-related populations is an amino acid substitution in exon 8. Our patient’s novel mutation differs in location from the previously reported ones, and may represent a prevalent mutation in non-Chinese Southeast Asian populations.

380 Variability of Hepatitis E serologic assays in a pediatric liver transplant recipient: Challenges in the diagnosis of Hepatitis E infection among at risk individuals. P.K. Sue, K. Schwarz, W. Karnsakul, Pediatrics, Johns Hopkins University School of Medicine, Baltimore, Maryland, UNITED STATESN. Pisanic, C. Heaney, Environmental Health Sciences, Johns Hopkins Bloomberg School of Public Health, Baltimore, Maryland, UNITED STATESM. Forman, Pathology and Medical Microbiology, Johns Hopkins University School of Medicine, Baltimore, Maryland, UNITED STATESK. Nelson, Epidemiology, Johns Hopkins Bloomberg School of Public Health, Baltimore, Maryland, UNITED STATES. Background: In recent years, there has been growing recognition of the significance of autochthonous Hepatitis E infection in the industrialized world. Reports from Europe describe the role of this disease in immuno-suppressed populations, particularly solid organ transplant (SOT) recipients. Yet the diagnosis of Hepatitis E Virus (HEV) infection in the United States remains challenging, due to variable sensitivity and specificity of commercially available assays. We report a case of multiple contradicting results of HEV serologic tests in a 4 year old solid organ transplant recipient with idiopathic hepatitis. Case Description: A four year old female with a history of orthotopic liver transplant presented with a 1 week history of elevated liver enzymes of uncertain etiology. She had a prior history of hepatic vein stricture requiring recent stent placement, but no history of graft rejection. Initial testing revealed a ten-fold rise in ALT over the course of 7 days, with a peak of 540 IU/L. Imaging revealed no evidence of hepatic obstruction, and a liver biopsy showed evidence of patchy inflammation suspicious for viral hepatitis, though stains for CMV and EBV were negative. Testing for Hepatitis A, B, and C, CMV and EBV revealed an unchanged baseline EBV viremia of 2000 copies per milliliter, but no evidence of acute infection. The patient was started on prednisolone for presumed graft rejection, and a HEV ELISA was sent to a commercial laboratory on day 7. Initial HEV testing returned positive for both HEV IgG and IgM. Liver enzymes improved on prednisolone but did not return to baseline. Repeat testing on day 14 returned positive for HEV IgM alone, and due to discordant results, serum and stool samples were sent for additional ELISA testing and PCR analysis. Results: Serum samples from days 7, 28, 39,and 46 were retested for HEV antibody utilizing an ORF-2 based assay and were negative for both HEV IgG and IgM. The performance of the assay was confirmed using the WHO 95/584 anti-HEV reference reagent (anti-HEV IgG and IgM positive) and the WHO HEV nucleic acid standard 6329/10 (anti-HEV negative). Corresponding serum and stool samples were also tested via real time PCR, and sent to the CDC for confirmatory HEV antibody and PCR testing. All PCR and confirmatory antibody tests were also negative. Discussion: Autochthonous HEV is an emerging disease among immuno-suppressed patients in industrialized countries. In the absence of a federally approved standard, the diagnosis of HEV infection in the US remains challenging due to variable characteristics of commercially available assays. Landry et al recently reported a case of missed HEV infection in a SOT adult due to poor sensitivity of a commercial HEV assay. In our case, poor specificity of a recombinant ORF 2/3 antigen based ELISA led to multiple false positive test results over a span of two weeks, and additional subsequent tests to ultimately rule out the diagnosis. In the absence of an FDA approved assay, we would like to draw attention to the need for confirmatory testing in cases of suspected HEV infection.

381 A Teenager with Abdominal Pain. S. Syed, M. Vos, S. Karpen, Pediatric Gastroenterology, Hepatology and Nutrition, Emory University, Atlanta, Georgia, UNITED STATES. We report a 16 year old African American female who presented with acute right upper quadrant abdominal pain, fever, weight loss and fatigue. She had hepatomegaly on exam and elevated transaminases (AST 75 U/L, ALT 77 U/L) with a total bilirubin of 1.6 mg/dL on her initial evaluation. The patient was seen at an outside hospital and underwent a laparoscopic cholecystectomy, where the appearance of her liver was noted by the surgeon to be abnormal and a liver biopsy was obtained. Pathological review revealed non-caseating granulomas with epithelioid histiocytes and a few multinucleated giant cells, bile ducts showed ductulitis, there was no portal/periportal fibrosis. Her gall bladder contained no gall stones, the GB wall was 0.3 cm without any histopathological abnormality. There was no significant change noted in her LFTs after cholecystectomy. After referral to our institution, her autoimmune workup was unrevealing, including normal or mildly elevated lysozyme and ACE levels, along with normal/negative profiles for anti-mitochondrial ab, anti-MPO ab, ANCA, serine protease 3, complement profile, Sjogren’s, liver-kidney microsomal panel, anti-smooth muscle antibodies, ANA and uric acid. She did have elevated IgE at 128 kU/L. The patient had a normal bone marrow biopsy and an eye exam negative for uveitis. Upper and lower endoscopies were unremarkable with biopsy findings of mild chronic gastritis and mild cecal mucosal eosinophilia with very focal acute inflammation. Fecal calprotectin levels were normal. Her abdominal MR imaging showed a slightly enlarged liver and spleen size of 10.2 cm with a normal biliary tract. CT imaging of her chest was negative for any pulmonary nodules, focal parenchymal consolidations and mediastinal or hilar lymphadenopathy. Infectious Diseases work-up included unremarkable investigations for Lymes’s disease, Bartonella, Toxoplasma, Histoplasmosis, syphilis, TB, HIV, CMV, EBV (had evidence of past infection), Hepatitis A, B and C, Toxocara canis, or any stool pathogens on O&P. No fungi or mycobacterium were identified by GMS, PAS-F and AFB stains on the liver tissue. Repeat liver biopsy was obtained which revealed multiple non-caseating granulomas and treatment with steroids led to resolution of fevers and diminution of abdominal pains. Her hepatic enzymes trended down (AST 26 U/L, ALT 34 U/L, GGT 85 U/L) as did the total bilirubin (TB 0.4 mg/dL). This case highlights that granulomatous hepatitis can be present in the setting of mildly elevated liver enzymes. Approximately 35 percent of granulomatous hepatitis is found to be secondary to sarcoid, although at this time for this patient, this diagnosis is not confirmed. In about 10 to 36 percent of patients with hepatic granulomas, the underlying disease process remains unidentified despite extensive investigations. The recommended treatment course for idiopathic granulomatous hepatitis involves immunosuppression using steroids or steroid-sparing therapy such as with Methotrexate.

382 Successful Outcome of Hepatic Angiosarcoma with Liver Transplant and Sirolimus. M. Xue, Baylor College of Medicine, Houston, Texas, UNITED STATESD. Leung, Department of Pediatrics, Baylor College of Medicine, Houston, Texas, UNITED STATESD. Leung, Division of Gastroenterology, Hepatology, and Nutrition, Texas Children's Hospital, Houston, Texas, UNITED STATES. A previously healthy 5 year-old female presented with a distended abdomen, early satiety and shortness of breath that increased in severity over a four-week period. An abdominal ultrasound revealed a large hepatic mass. MRI further characterized a large heterogeneous mass involving all 4 lobes of the liver that displaced the upper abdominal contents to the left. Liver biopsy revealed an infantile hemangioendothelioma that was not amenable to surgical resection. During her admission, she developed intermittent right shoulder pain, constipation and anorexia. Physical exam revealed a pale, well-nourished female with a largely distended abdomen. The liver was large, firm and non-nodular. She was found to be hypothyroid (TSH 81. 5, T3 81, free T4 0.6). Her AFP was 1.6 ng/ml and INR was 1.3. Serum ammonia peaked at 104 umol/L. As a result of rapid tumor growth, the patient soon developed respiratory competition, ascites and grade 3 encephalopathy, prompting urgent liver transplant (LT) listing and ultimately LT on hospital day #10. Following transplant, the patient’s explant revealed a diffuse angiosarcoma arising from an infantile hemangioendothelioma. The family declined chemotherapy as an adjuvant treatment option due to its toxicities. She was immediately placed on Sirolimus for its immunosuppressive and putative anti-tumor properties. 21 months later, the patient shows no evidence of disease recurrence or metastasis. Malignant liver tumors represent approximately 1% of malignancies in children. Hepatic epithelioid hemangioendothelioma (HEHE) is a rare vascular tumor of the liver that has a variable clinical course. It is histologically characterized by spindle-shaped tumor cells infiltrating sinusoids and blood vessels. HEHE is a transplantable condition due to its high survival and recurrence-free rates. Hepatic angiosarcoma (HA) of the liver is a high-grade tumor of endothelial cells that is even more rare, with less than 50 cases published worldwide. There are no clear radiographic findings that differentiate benign from malignant vascular tumors in the liver, and histologically differentiating HEHE from HA can be challenging. HA of the liver has a limited response to chemotherapy, radiation and resection with universal tumor recurrence with LT and nearly 100% mortality by 18 months. This is the first reported successful case of hepatic angiosarcoma treated by LT in combination with Sirolimus. Sirolimus antagonizes the mTor pathway which regulates cell proliferation, differentiation, and migration and is being studied as an anti-neoplastic agent for solid tumors. Its dual use in cases such as this needs further study. This case also highlights the difficulty in differentiating HEHE from angiosarcoma and raises the ethical concerns surrounding liver transplantation in pediatric patients with angiosarcoma.

383 Early rituximab use in giant cell hepatitis with autoimmune anemia resulting in rapid clinical improvement. C.H. Yang, S. Kim, Pediatrics, Children's Hospital Los Angeles, Los Angeles, California, UNITED STATESD. Thomas, N. Kerkar, Pediatric Gastroenterology and Nutrition, Children's Hospital Los Angeles, Los Angeles, California, UNITED STATES. A 5 month old female with a history of neonatal jaundice presented with scleral icterus. Exam revealed a fussy, consolable child with scleral icterus and jaundice to the lower extremities, and liver edge 3 centimeters below the costal margin; no splenomegaly. Initial labs showed conjugated hyperbilirubinemia (total 21.9, direct 16.0), transaminitis (AST 2285, ALT 3226, alkaline phosphatase 521), macrocytic anemia with Hb/Hct 7.2/22.6, and reticulocytosis >25%; PT, PTT, and INR grossly normal, and viral etiologies negative. She was transfused with pRBCs without improvement, and was found to be warm auto-antibody positive. Abdominal ultrasound showed liver size at the upper limit of normal with parenchymal disease. Liver biopsy showed hepatocellular cholestasis with canalicular bile plugs and giant cell transformation. She was started on high-dose methylprednisolone 2mg/kg for giant cell hepatitis with autoimmune hemolytic anemia. When she showed minimal improvement after a week, rituximab 375mg/m2 was started, with hyperbilirubinemia, transaminitis, and anemia improving 6 days after the 1st dose. She received 4 doses total, each 1 week apart. Methylprednisolone wean was initiated after 3 weeks, and azathioprine 2mg/kg started as maintenance. At discharge, total bilirubin was 4.5 (direct 0.8), AST 164, ALT 339, alkaline phosphatase 149, Hb/Hct 11.3/35.2, reticulocyte 3.2%. She was sent home on azathioprine and a steroid wean. At follow-up, she continued to do well, and labs continued to normalize. The combination of giant cell hepatitis with autoimmune hemolytic anemia is an extremely rare entity with poor prognosis. Immunosuppressive drugs, IVIG, rituximab, and liver transplantation have been used as treatment. Rituximab has been used only in refractory cases, with significant response seen months after 4 doses. Ours is the first case of rituximab use early in the disease course, with rapid clinical improvement in anemia and liver failure. While prednisone and azathioprine are considered first line treatment for this disease, our case illustrates the benefit to early utilization of rituximab, particularly in cases which are unresponsive to initial therapy.

384 Valganciclovir Prophylaxis in Pediatric Liver Transplantation: Is a Longer Course More Protective?. S. Young, S. Sengupta, P. Boone, R. Azzam, Pediatric Gastroenterology and Hepatology, University of Chicago, Chicago, Illinois, UNITED STATES. Cytomegalovirus (CMV) infection causes significant morbidity and mortality among solid organ transplant recipients. Valganciclovir (VGCV) prophylaxis against CMV in orthotopic liver transplant recipients is not approved by the Food and Drug Administration, but many centers still use it. In pediatric liver transplant patients, the average length of prophylaxis with VGCV is 120 days, but there is a lack of data examining its use and duration. We performed a retrospective study of 33 patients who underwent a pediatric liver transplant between the years 2006-2012 and received CMV prophylaxis with VGCV at 15 mg/kg/day following a 14-day course of IV ganciclovir. All patients received immunosuppression per protocol with a calcineurin-inhibitor, specifically tacrolimus unless not tolerated, and low dose Prednisone for 2 months post-transplant. Patients were followed for two years post- transplant or until time of death. Nine patients (27%) developed CMV infection, defined as a positive CMV PCR. One patient (3%) developed CMV disease, defined as a positive CMV PCR plus biopsy-confirmed tissue-invasive disease. Of the 10 patients with either CMV infection or disease, the donor/recipient (D/R) CMV status pre- transplant was: 3 D+/R+ (30%), 3 D+/R- (30%), 1 D-/R+ (10%), 1 D-/R- (10%), and 1 D+/R unknown (10%). The average time to virus acquisition was 169 days, with a range of 33-329 days. Of the 9 patients who had evidence of CMV infection, 7 (78%) developed CMV while still on VGCV prophylaxis or after completing more than standard 120-day therapy. 3 of these 7 patients (43%) had early-onset CMV, defined as infection occurring within 120 days of liver transplant. 4/7 (57%) developed late-onset CMV, defined as infection occurring at >120 days. Two patients with late onset CMV infection had anti-viral prophylaxis discontinued at around 90 days, while the rest of late infections were in patients who had received VCGC for an average of about 200 days, with range of 126-314 days. The single patient with documented CMV disease had VGCV required discontinuation of VGCV at 97 days due to leukopenia, and was diagnosed with CMV disease at 166 days post-transplant. Of the remaining 23 patients who did not develop CMV, 15 were treated for longer than 120 days, and 8 were treated less than or equal to 120 days. Our data showed a nearly equal risk of developing CMV infection or disease while on prophylaxis for 120 days or an extended course of therapy greater than 120 days. Based on this observation, we feel that longer prophylaxis with VGCV is not superior to the more commonly used 120-day course. However, as 57% of CMV positive patients had late-onset of infection, we recommend continuing to periodically check CMV PCR after standard prophylaxis for 120 days is discontinued based on clinical correlation. Subsequently, our institution has developed a protocol to stop VGCV prophylaxis after 120 days. The limited volume of patients included poses a limitation in terms of drawing major conclusions. A multi-center prospective study is recommended to further establish the optimal dose and duration of VGCV prophylaxis in pediatric liver transplant patients.

Intestine/Colonic Disorders - IBD 400 Reducing Medication Errors: An Improve Care Now Quality Improvement Project. M. Patel, A. Bitar, A. Ghazi-Askar, C. Fraga-Lovejoy, M. Osman, C. Pant, C. Marshall, University of Oklahoma Health Sciences Center, Oklahoma City, Oklahoma, UNITED STATES. Background: In our practice caring for patients with Inflammatory Bowel Disease (IBD) as part of the Improve Care Now collaborative network, we have often found inconsistencies in the medications patients report taking, versus what has been documented in the medical record. Patients also often seem unable to identify their IBD medications. We believed this could be an important quality improvement project since adherence to therapy is expected to produce greater remission rates in IBD. Objective: Improve patients’ understanding of their IBD medications and ensure accurate charting of their medicine in the medial record. Design and Methods: Several interventions have been undertaken in an effort to improve patients’ understanding and recording of their IBD medications. The first was the use of the Clinic Visit Summary form, which is given at the end of each visit listing all current medications and any changes to their medicines. We performed a chart review of active IBD patients in our practice to determine if there was congruency or non-congruency with regards to what medications (if any) they listed on a patient intake form, compared with what was recorded in the medical record. We then used this information in Pre-Visit Planning to let practitioners know of discrepancies and to identify patients who may require further education. We compiled a list of commonly used IBD medicines and designed a laminated card with these listed, as well as common dosage forms. We asked our medical assistants and PSR’s to encourage patients to complete the list of medications on our patient intake form, using the laminated medicine card list as a reference. After initiating these practice changes, we performed a subsequent chart review to see if the congruency rates had increased. Results: Preliminary results of a subset of patients followed by one provider show a marked improvement in congruency rates with all visits showing congruent med lists after initiation of the project (16 visits). This compared with 38 visits which were reviewed before project initiation, of which 18 (47%) were congruent, 3 (8%) were noncongruent, 2 (5%) were incomplete, and 15 (39%) failed to list any meds. Discussion: With better education and more accurate charting, we expect to improve adherence to therapy and remission rates in patients with IBD.

401 Development of Hemophagocytic Lymphohistiocytosis associated with Human Granulocytic Anaplasmosis in a patient with Crohn’s disease on Infliximab. S. Raikar, Z. MolleRios, Pediatric Gastroenterology, Hepatology and Nutrition, AI duPont Hospital for Childrens, Wilmington, Delaware, UNITED STATES. We report a case of a 14 year old male with ileocolonic Crohn's disease. He was ongoing maintenance therapy of mesalamine and infliximab for the past 1.5 years. He presented with a biphasic fever, headache, and pneumonia. His laboratory workup was remarkable for significant thrombocytopenia (34), anemia, elevated LFTs and elevated inflammatory markers. An extensive infectious workup was ensued which was negative for adenovirus, parvovirus, enterovirus, influenza, RSV, legionella, Rocky Mountain spotted fever, tuberculosis, leptospirosis, CMV and EBV. He was treated with Cefepime. Levofloxacin was added due to persistent fever. His clinical picture was suspicious for hemophagocytic lymphohistiocytosis (HLH). Screening tests revealed an elevated ferritin at 4317 and triglycerides at 306. Additional testing revealed low natural killer (NK) cell function and elevated soluble IL2 receptor alpha which supported the diagnosis of HLH. There was dramatic clinical improvement with levofloxacin and he was discharged home to complete a 10 day course. Subsequently, his human granulocytic anaplasmosis (HGA) DNA PCR resulted positive. The genetic markers for HLH were negative. This case has several unique aspects. We speculate that the HGA infection lead to development of HLH in this immunocompromised patient. There are no known reported cases of such an association. Anaplasma phagocytophilum is the cause of human granulocytic anaplasmosis. The clinical presentation of HGA typically includes fever, headache, leucopenia, thrombocytopenia and elevated liver enzymes. Infection with HGA results in significant disruption of neutrophil function, production of inflammatory cytokines and macrophage activation. HLH is a rare but potentially fatal hyper inflammatory condition caused by a highly stimulated but ineffective immune response. HLH can be primary, with a genetic etiology. Secondary causes are associated with malignancies, autoimmune diseases or infections such as brucellosis, rickettsial disease, visceral leishmaniasis, histoplasmosis, mycobacterium tuberculosis, CMV and EBV. There seems to be an increased risk for HLH in pediatric patients with Crohn’s disease who have been on thiopurines and developed primary EBV infection. In our patient’s case he improved with appropriate antibiotic treatment. His HLH markers subsequently normalized except for a persistent low NK cell function. He has done remarkably well and he continues on infliximab therapy for Crohn’s disease. Patients with HLH have high levels of various cytokines including TNF alpha. Anti-TNF therapy increases susceptibility to serious infections that can lead to HLH. However, infliximab has been used as treatment in refractory HLH. It is possible that infliximab actually blunted the severity of HLH in this case.

402 Pneumatosis Intestinalis secondary to steroid therapy during acute exacerbation of Crohn disease. V. Raju, Z. Khaled, E. Bugaieski, Pediatrics, University of Illinois College of Medicine at Peoria, Peoria, Illinois, UNITED STATESZ. Khaled, Pediatrics, Methodist Medical Center, Peoria, Illinois, UNITED STATESS. Theivanayagam, Gastroenterology, University Of Missouri hospitals and clinics, Columbia, Missouri, UNITED STATES. Case report Our patient is a 9 year old Caucasian female who after been newly diagnosed with crohn disease was treated with prednisone and mesalamine. She had good clinical response with resolution of her symptoms. So prednisone was stopped and was continued on mesalamine to maintain remission. After four months she had an acute exacerbation. So prednisone was restarted. However her symptoms got worse. Physical examination revealed distended abdomen with diffuse tenderness. Her abdominal Xray showed unusual gas pattern in the right upper quadrant. To evaluate further she had abdominal CT which revealed extensive Pneumatosis Intestinalis involving the ascending and transverse colon. Prednisone was immediately stopped and she was admitted to the hospital. She was treated with antibiotics ciprofloxacin, flagyl and parenteral nutrition with complete bowel rest. She was also continued on mesalamine. She had significant improvement with the above regime. Her antibiotics were stopped after ten days and she was put back on enteral feeds. She was started on 6 mercaptopurine along with mesalamine to maintain remission. The repeat CT abdomen done after 3 weeks showed near complete resolution of Pneumatosis Intestinalis. She continued to do well at 2 months follow up. Discussion Crohn is an immune mediated inflammatory disease affecting any part of the GI tract. The most common symptoms are diarrhea, blood in stool, abdominal pain, weight loss, low grade fever and fatigue. It manifest in childhood or adolescence. Various pharmacological agents are used to reduce inflammation and to maintain remission. They include corticosteroids, aminosalicylates, antibiotics, immunosuppressive agents like thiopurine, methotrexate and biological agents like Infliximab, Adalimumab. Short term corticosteroids have traditionally been the mainstay of treatment to induce remission in the event of an acute flare up. Their potential side effects are cushingoid changes, acne, weight gain, muscle weakness, insomnia, depression, abdominal pain, osteoporosis, blurred vision, and avascular necrosis of the hip joint. Pneumatosis intestinalis (PI) is reported as a rare side effect of steroid therapy among transplant recipients. PI has also been reported as a rare clinical presentation during an acute exacerbation of inflammatory bowel diseases. But there are no reports of steroid induced PI in children with inflammatory bowel disease. PI can be diagnosed with plain abdominal radiograph. Other studies like barium studies, sonography, computed tomography, MRI are also helpful. Conservative management which includes bowel rest and antibiotic therapy is the usual treatment of choice. They rarely need surgical intervention especially if they develop intestinal perforation. Conclusion In our patient, symptoms got worse and patient developed PI after starting steroids. But the clinical symptoms and radiological abnormalities of PI resolved after stopping steroids. So we postulate that PI developed and got worse secondary to steroid treatment. This scenario also emphasis the significance of reevaluation and considering iatrogenic causes if the expected clinical outcome is not met.

403 Hodgkin’s Lymphoma Following Adalimumab For The Treatment of Crohn’s Disease In An Adolescent. A. Rodriguez, J. Kerner, Pediatric Gastroenterology and Hepatology, Stanford University, Palo Alto, California, UNITED STATESG. Berry, Pathology, Stanford University, Palo Alto, California, UNITED STATESS. Luna-Fineman, Pediatric Hematology-Oncology, Stanford University, Palo Alto, California, UNITED STATES. An 11 y/o girl was initially referred to our practice due to persistent fever, abdominal pain and bloody stools. Her health prior to this period was normal except for a history of constipation. On initial presentation her exam was normal. Her labs were significant for mild anemia (hemoglobin 10.7), elevated ESR (46) and positive EBV IgM. She was diagnosed with infectious mononucleosis and treated supportively. She was seen back 1 month later with continuation of symptoms. Her physical exam was unchanged and she continued to have anemia with an increase in her ESR (55) and decrease in albumin (2.3). Given these findings there was concern for possible IBD so she underwent an EGD and colonoscopy. On histology there was evidence of acute ileitis with granulomas and ulcerations as well as antral granulomas consistent with Crohn’s Disease. She was started on prednisone and mesalamine. Subsequently, she was started on 6-MP. She was later started on allopurinol (100 mg daily) due to persistently elevated 6-MMP levels to achieve therapeutic 6-TGN levels with normal 6-MMP levels. Even with good 6-TGN levels, she continued to have multiple flares requiring steroids over the next 2 years. Due to her poor disease course and steroid dependence she was switched to adalimumab monotherapy. Shortly after starting adalimumab she was no longer having any GI symptoms, and by 5 months after switching her anemia resolved, albumin (4.0), ESR (8) and CRP (0.4) all normalized. Two years later she began developing a persistent, nonproductive cough with pruritus. She had a chest x-ray which showed evidence of a large bulky lobulated anterior and middle mediastinal mass. A chest CT was then performed which also showed evidence of the large (15X9X9cm) mediastinal mass. The mass was biopsied and confirmed the diagnosis of classical Hodgkin disease, stage IIA, with bulky disease (intermediate risk). Adalimumab was stopped. She received 3 months of chemotherapy followed by radiation therapy which put her disease into remission. One year after her tumor treatment, mesalamine was restarted and omega-3 fatty acids orally were added. Since restarting mesalamine her Crohn’s disease has been well controlled and she has not had a flare for more than 2 years. Her malignancy remains in remission. This is the first case of a malignancy developing after treatment with a biologic for Crohn’s disease in the history of our practice at Stanford. Although such a lymphoma is a rare side effect of such treatment, full disclosure of all potential risks should be reviewed with parents and patients prior to the initiation of biologics in IBD.

404 Pancreatitis as an initial presentation of Crohn’s disease. S. Syed, B. Schoen, Pediatric Gastroenterology, Hepatology and Nutrition, Emory University, Atlanta, Georgia, UNITED STATESJ. Quiros , Pediatric Gastroenterology, Medical University of South Carolina, Charleston, South Carolina, UNITED STATES. Acute pancreatitis can be associated with Crohn’s disease. The estimated risk has been reported to be 4.3 times greater than that of the general population. In adult Crohn’s patients, pancreatitis may be caused by gallstones or alcohol, less frequently by duodenitis or medications such as Azathioprine. We report a 13 year old male who presented with acute tail pancreatitis with no history of trauma, which later advanced to involve the pancreatic head and body. Work-up for was negative for any mutations in the CFTR, PRSS1 and SPINK1 genes. Autoimmune investigations including Anti-Nuclear (ANA) antibody IgG, Anti-Neutrophil Cytoplasmic Antibodies (ANCA) and immunoglobulin G 4 subclass profile were within normal limits. Serum triglycerides were normal. He underwent four ERCPs with sphincterotomy and stent placements, however remained symptomatic with persistent intermittent abdominal pain despite eventual normalization of his pancreatic enzymes. After biopsies obtained during the fifth ERCP revealed granulomatous gastritis, he underwent an EGD and colonoscopy. The EGD was notable for diffuse gastric edema, inflammation and ulceration with duodenal loss of villous surface. Gastric biopsies showed antral mucosa with moderate chronic active gastritis with no H. Pylori-like organisms identified by Diff-Quik stain. Duodenal biopsies showed moderate-severe chronic active duodenitis with marked villous blunting. His colonoscopy was grossly normal but cecal biopsies revealed focal eosinophilic colitis. His esophagus, terminal ileum, remaining colon and rectum were all normal on luminal imaging and biopsy. No ileal or colonic granulomas were identified. Based on his endoscopic and histologic findings, he was diagnosed with Crohn’s disease and has initiated corticosteroid therapy. From review of the literature, acute pancreatitis may precede the clinical onset of Crohn’s disease but the initial demonstration of isolated tail pancreatitis in our patient was an unusual presentation.

405 Too much of a good thing? Remission of ileal Crohn’s disease with reduced infliximab dosing. A. Turkish, Pediatrics, New York Hospital Queens, Flushing, New York, UNITED STATES. A 20 year-old-female with a 3 year history of long-segment distal ileal Crohn’s disease presented to our institution with persistent right-lower-quadrant pain, anorexia, weight loss, diarrhea with occasional blood, anemia, mild hypoalbuminemia and mildly elevated serum inflammatory markers non-responsive to Lialda 1.2g twice a day. A CT scan of her abdomen confirmed a 30cm long segment of distal ileum that was diffusely thickened, and a colonoscopy revealed diffuse ileitis, consistent with her initial examination 3 years ago that exhibited diffuse ileal ulceration and histologic evidence of granulomas and marked inflammation in the terminal ileum. She was placed on metronidazole, 6-mercaptopurine (6MP) at 1mg/kg/day and induced with infliximab (IFX) at 5mg/kg/dose. 2 weeks after the 2nd dose of IFX she developed several days of vague fleeting sharp pains and paresthesias radiating from her arms and legs distally that was attributed to metronidazole, which was discontinued. 2 weeks after the 3rd dose of IFX, however, she developed several weeks of daily sharp pain around her neck as well as a recurrence of shooting pain radiating down her arms and legs distally. Neurological consultation confirmed there was no evidence of a radiculopathy or motor/sensory deficits, and opined that her symptoms were arthralgias, likely resulting from infliximab. An EMG was normal and these symptoms gradually resolved. A mutual decision was made to discontinue IFX for the time being, and despite complete non-compliance with 6MP, she remained in clinical remission for 16 weeks, after which she re-developed frequent diarrhea, abdominal pain, malaise and weight loss. Prior to restarting IFX, an antibody to infliximab was excluded and interestingly, she exhibited a serum IFX level of 9 mcg/mL (Anser-IFX, Prometheus). IFX infusions at 2.5mg/kg every 8 weeks for several infusions maintained her in remission without recurrence of the neck and extremity pain. Her serum IFX level at trough was 7.2 mcg/mL, and was 10.2 mcg/mL 9 weeks after a slight dose increase to 3.5mg/kg. She is currently in remission on this dose of IFX every 8 weeks. This case demonstrates that there is likely significant variability of IFX metabolism within the inflammatory bowel disease population, with extent and localization of disease a major contributing factor. The availability to check serum IFX levels may be beneficial, in this regard, in order to tailor an individual’s IFX dose or infusion frequency. We did not obtain a “peak” IFX level after the 3rd infusion, but the adverse reactions she experienced that did not recur with lower dosing (and less frequent infusions) along with very “therapeutic” serum IFX levels at trough and even 16 weeks following induction may indicate a need for future studies into the potential toxic effects of very high serum levels of infliximab.

406 Neurologic Changes in a Patient with Inflammatory Bowel Disease. D. Wendel, O. Waisbourd-Zinman, E. Goldberg, C. Liacouras, Gastroenterology, Hepatology, and Nutrition, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, UNITED STATES. An 11 year-old previously healthy boy was diagnosed with Crohn’s disease after presenting with severe perianal disease, a perianal abscess and CT with thickening of the terminal ileum and transverse colon. He was started on IV antibiotics, underwent EGD, colonoscopy, and exam under anesthesia. The results showed areas of ulceration and inflammation in the colon with granulomas on the pathology and an exam showing perianal ulcerations and a fistula for which a seton was placed. With these findings he was started on IV steroids and Pentasa with improvement of his symptoms. He was discharged on prednisone, Pentasa, and oral ciprofloxacin/metronidazole. After he completed the ciprofloxacin and weaned off of the prednisone he was continued on the metronidazole and Pentasa. Clinically, his GI symptoms resolved at a visit 6 weeks later. On follow up in clinic 2 months later, the family noted that he had changes in his speech for the last few weeks. The changes were subtle and improving so they planned for evaluation with neurology as an outpatient. Over the next few days his speech became progressively more slurred and he developed a wide based gait with instability and he was admitted to the hospital. He had no fever, emesis, change in stool pattern, or abdominal pain. He had no other neurologic changes other than previously mentioned. His labs were normal including CBC, CMP, CK, LDH, ESR, CRP, B12, and Vitamin E. Lumbar puncture was revealed normal cell counts with negative gram stain and culture. The CSF was also negative for enterovirus and parechovirus. There were no oligoclonal bands in the CSF or serum. A head MRI was done that showed multiple bilateral symmetric T2 hyperintense lesions involving the brainstem, a distribution consistent with metronidazole-induced encephalopathy. The metronidazole was discontinued and his symptoms gradually improved without further intervention. Metronidazole is a commonly used medication for various gastrointestinal illnesses and although rare, should be considered as a cause of encephalopathy or neurologic changes. Head MRIs are valuable in determining metronidazole encephalopathy.

407 Oral vancomycin as adjunct maintenance therapy for ulcerative colitis. A. Yeh, K. Cox, Pediatric Gastroenterology, Stanford University, Palo Alto, California, UNITED STATES. Oral vancomycin (OV) is an antibiotic against gram-positive bacteria that has been shown to be an effective treatment for concomitant primary sclerosing cholangitis and inflammatory bowel disease due to immune modulation of tumor necrosis factor-alpha pathways and on induction of regulatory T cells. We present two patients with ulcerative colitis without primary sclerosing cholangitis that responded to oral vancomcyin as an adjunct maintenance therapy for ulcerative colitis treatment. Case 1: A five year old male with ulcerative colitis (UC) with pancolitis and chronic cryptitis and crypt abscesses on colonoscopy began treatment with sulfasalazine without significant improvement. He was subsequently started on 6-mercaptopurine (6-MP) and a prednisolone burst. Upon attempting to wean off prednisone, he was found to be steroid dependent and required a repeat burst of steroids. 6-MP metabolites were within therapeutic range. Clostridium difficile toxin and stool cultures were repeatedly negative. Subsequently, patient was trialed on a course of metronidazole as an adjunct treatment for 2 weeks with marked improvement. However, upon weaning the metronidazole dose his IBD symptoms flared. Patient was then restarted on metronidazole and weaned off of prednisolone. Given concern of toxicity with chronic use of metronidazole, he was transitioned to oral vancomycin (10mg/kg/dose TID). He has been maintained on 6-MP and oral vancomycin without flare of UC symptoms for 1 year. Fecal calprotectin values have trended down from 590 mcg/g to 139mcg/g. Inflammatory markers have remained normal with an ESR of 10 mm/hr, CRP of <0.2mg/dL and he is tracking on his growth curves. Case 2: A fourteen year old male with ulcerative colitis and type I diabetes mellitus was initially started on Asacol with improvement. One year after diagnosis he had a clostridium difficile infection and was started on metronidazole without improvement, but improved on oral vancomycin 250mg QID for 10 days and prednisone taper. Patient then presented with recurrent c. difficile and diarrhea and hematochezia. Oral vancomycin was restarted at 500mg TID. After 2 weeks of oral vancomycin, c. difficile toxin was negative but discontinuation of vancomycin caused worsened hematochezia. Therefore, oral vancomycin was restarted at 500mg TID for 30 days with a slow taper of decreasing his dosing regimen from three times a day to twice a day to daily to off every week. On maintenance asacol he has been asymptomatic for approximately 6 months with an ESR of 6mm/hr and CRP of 1.1mg/dL . Discussion: These two cases illustrate the novel use of oral vancomycin as an adjunct treatment for ulcerative colitis maintenance therapy. Further research with a larger patient population is needed to better elucidate the efficacy of treatment, immunomodulating effects, and changes in fecal microbiota with oral vancomycin in inflammatory bowel disease.

421 Routine Ileoscopy Complicated by Intestinal Perforation in a Small Bowel Transplant Patient with a Parastomal Hernia. J. Yeh, S. Yusung, R. Venick, L. Wozniak, Department of Pedidatric Gastroenterology, Hepatology, and Nutrition, UCLA, Los Angeles, California, UNITED STATESV. Agopian, D. Farmer, Department of Surgery, Liver Transplantation, UCLA, Los Angeles, California, UNITED STATES. Background: Ileoscopy with mucosal biopsy is utilized often after intestinal transplantation for surveillance of the allograft. Complications from endoscopy are rare but include perforation and bleeding. The risks of ileoscopy in the presence of a parastomal hernia are not described in the literature. Case Presentation: Our case is a five year old girl with a history of and intestinal failure associated liver disease status post liver and small bowel transplant in August 2008. She underwent ileostomy takedown in May 2010 but due to multiple complications required placement of an end ileostomy in April 2011. In December 2012, she presented for gastrojejunostomy feeding tube exchange with concurrent surveillance enteroscopy and ileoscopy. She had chronic ileostomy prolapse that had been noted since May 2011 likely due to the extent of bowel dilation at the time of stoma creation. Digital stoma exam was performed prior to ileoscopy and was normal. The endoscopists found it difficult to advance the scope and only reached a distance of 20 cm. The mucosa was noted to be friable. There were no anesthesia complications and her abdomen remained soft, but the ostomy appeared more edematous at the conclusion of the procedure. In recovery, the patient had progressive retching and abdominal discomfort. Plain radiographs revealed free air. She was admitted to the PICU and started on broad spectrum antibiotics. CT of the abdomen with both oral and ostomy contrast did not reveal the site of perforation. The patient underwent exploratory laparotomy and a perforation was found in association with a parastomal hernia. Discussion: Parastomal hernia occurs at the site of or adjacent to the stoma and can be identified by the presence of a bulge at the stoma site, with or without pain. Incidence ranges from 0 to 22%. There is only one study of endoscopy in small bowel transplant patients which examined a total of 1273 endoscopies (760 ileoscopies). With only one perforation, endoscopy, in general, was concluded as safe in this population. However, there is no literature on the risks of ileoscopy in the presence parastomal hernias. Given anatomy and likely presence of adhesions, there is a high risk of perforation in ileoscopies involving parastomal hernias.

Intestinal/Colonic Disorders – Non IBD 423 Protein-losing enteropathy secondary to chronic intususception.. V. Raju, Z. Khaled, Pediatrics, University of Illinois College of Medicine at Peoria, Peoria, Illinois, UNITED STATESZ. Khaled, Pediatrics, Methodist Medical Center, Peoria, Illinois, UNITED STATESS. Theivanayagam, Gastroenterology, University Of Missouri Hospitals and Clinics, Columbia, Missouri, UNITED STATES. Our patient is a 9 month old Caucasian girl presenting with diarrhea, intermittent vomiting and fussiness. Physical examination revealed mildly distended abdomen with discomfortness upon deep palpation. Initial workup including blood counts, metabolic profile, urine analysis, stool cultures was negative. So diagnosed as feeding intolerance and changed to soy based formula. Over the course of next four weeks her symptoms continued and she started losing weight. Further stool studies included fat analysis, WBC lactoferrin and alpha-1-antitrypsin. Her alpha-1-antitrypsin was significantly elevated at 215 mg/dl. Since the initial presentation, her total protein dropped for 5.7 gm/dl to 2.7 gm/dl with albumin trending down from 3.5 gm/dl to 1.2 gm/dl. The above results indicated protein-losing enteropathy. For further evaluation she had EGD with biopsies and small bowel follow through, which were unremarkable. However, Barium enema was consistent with Ileocolic intussusception which subsequently got reduced. With the continuation of the symptoms she had CT of her abdomen which revealed ileoileal intussusception. She underwent surgical correction. Following which her symptoms improved. She was not losing protein anymore and showed consistent gain in her weight. Discussion Intussusception occurs when the proximal portion of bowel invaginates into the distal portion, dragging the mesentery along with. It presents with acute abdominal pain, vomiting without diarrhea and heme positive stool. Our patient presented with protein- losing enteropathy, an unknown complication of intussusception. Protein-losing enteropathy (PLE) is characterized by an excessive loss of protein into the gastrointestinal tract. The common clinical presentation consists of edema, ascites, pleural and pericardial effusion. Fat malabsorption can lead to deficiencies of fat soluble vitamins. The gastrointestinal tract lose protein secondary to abnormalities of the lymphatic system resulting in leakage of protein-rich lymph and mucosal injury leading to increased mucosal permeability. The common causes of protein-losing enteropathy secondary to lymphatic abnormalities include intestinal lymphangiectasia either primary or secondary to congestive heart failure, Fontan procedure, sarcoidosis, lymphoma and Crohns. Those that cause mucosal damage include inflammatory bowel disease, infections, malignancies and vasculitic disorders. Protein-losing enteropathy can be detected by increased concentration of alpha-1-antitrypsin in the stool specimen. Functional imaging with radiopharmaceuticals give more detailed assessment. After diagnosing PLE further studies like imaging, endoscopy are needed to search for underlying causes. Treatment for PLE should consist of maintaining adequate nutritional status and treatment of underlying disease. PLE secondary to Fontan has a high mortality of 46%. Otherwise the mortality and morbidity depend on the underlying cause and the treatment strategies. Conclusion Since our patient clinically improved soon after correcting intussusception, we report that as the possible cause for protein-losing enteropathy which has not been reported in the pediatric literature.

424 Infantile Juvenile Polyposis Syndrome (IJPS), Case Report of a Rare Pediatric Condition. E.M. Reyes- Santiago, R.E. Quiros-Tejeira, K. Azarow, A. Langnas, W.E. Grant, D. Mercer, L. Vargas, P.J. Palomo, Pediatric Gastroenterology, Hepatology and Nutrition, University of Nebraska Medical Center, Omaha, Nebraska, UNITED STATESE.M. Reyes-Santiago, R.E. Quiros-Tejeira, D. Perry, K. Azarow, P.J. Palomo, Pediatric Gastroenterology, Hepatology and Nutrition, Children's Hospital and Medical Center, Omaha, Nebraska, UNITED STATES. Background: Juvenile Polyposis Syndrome (JPS) is an autosomal dominant, pre-cancerous condition characterized by multiple hamartomatous polyps in the gastrointestinal tract. Family history is found in 20-50% of patients. Its incidence is 1/100,000. Reported germ line mutations: SMAD4, BMPR1A. These account for about 60% of JPS cases (1). JPS includes three subtypes: juvenile polyposis coli, generalized juvenile polyposis and IJPS (2). Patients usually have a 10q23 microdeletion in the BMPR1A and PTEN genes (1) (3). The case: A 14 months old female, that at 11 months old presented diarrhea and rectal prolapse. Patient had a colonic resection due to colo-colonic intussusception, secondary to a large polyp. Patient persisted with melena, requiring multiple blood transfusions. Three endoscopies (EGD/Colon) confirmed the presence of multiple polyps in the GI tract. The patient developed intestinal failure requiring Total Parenteral Nutrition (TPN). She continued with protein loosing enteropathy (PLE). A repeated the EGD/Colon showed multiple polyps in the stomach, duodenum and throughout the rectum and colon. Capsule endoscopy showed multiple large polyps through the entire small bowel. Genetic testing showed a heterozygous deletion in the entire BMPR1A gene. Discussion: IJPS is a rare condition. Few case reports are available. These patients may develop significant anemia and PLE. Our patient’s nutrition is being managed with TPN and a clinical decision was made to perform a total colectomy with ileostomy. She is being managed conservatively through the intestinal rehabilitation program. Small bowel transplant may be an option if persistence of anemia, PLE, loss of vascular access or TPN complications. Yearly cancer surveillance will be required with small bowel endoscopies, alpha feto protein, abdominal ultrasound and thyroid cancer screening.

425 Eosinophilic Esophagitis in a child with Juvenile Polyposis associated with SMAD4 mutation.. J. Rodrigues, .R. Braddock, A.K. Jain, Saint Louis University, St Louis, Missouri, UNITED STATES. Background: Juvenile polyposis syndrome (JPS) is a rare syndrome characterized by 5 or more juvenile polyps (JP) in the colorectum, JP throughout the gastrointestinal tract or any number of JP with a family history of JPS. It is autosomal dominant and carries a high lifetime risk of colorectal cancer. We describe a case of JPS with SMAD4 gene mutation and coexisting eosinophilic esophagitis (EoE). Case Description: A 12 year old boy with a history of attention deficit hyperactivity disorder, developmental delay, petit mal seizures and asthma was evaluated for blood in stools. He had a colonoscopy which showed many ‘carpet-like’, flat, pedunculated, sessile, non-bleeding polyps throughout his colon, some of which were biopsied. Pathology revealed juvenile polyps. Low grade dysplastic and tubulovillous adenomatous polyps were also noted in the descending colon. History revealed a paternal family history of polyposis, frequent epistaxis and intracranial aneurysms. He continued to have intermittent blood in stools and was referred to our hospital a few months later. A subsequent endoscopic evaluation continued to show numerous sessile and pedunculated polyps throughout the colon. Esophageal biopsies revealed > 30 eosinophils/high power field in the esophagus, the stomach and duodenum were normal. Genetic testing for mutations in APC, BMPR1A, CDH1, CHEK2, EPCAM, MLH1, MSH2, MSH6, MUTYH, PNS2, PTEN, STK11 and TP53 was negative. He was however, noted to have a c.1351_1375del25 mutation in the SMAD4 gene. Patient was evaluated by surgery and in light of the high risk for progression to malignancy he underwent a prophylactic colectomy. Discussion: JPS was first described by Hertz in 1914. The genetic link of JPS to a 4 base pair deletion in exon 9 of SMAD4 was described by Howe et al. in an Iowa kindred in 1998. It is now estimated that approximately 40-50% of patients with JPS have germline mutations in either SMAD4 or BMPR1A gene, both of which play a role in the BMP/TGF β signaling pathway. Hereditary Hemorrhagic Telangiectasia (HHT) has been described to coexist in patients with JPS and SMAD4 mutation, which can result in life-threatening arterio-venous malformations. Many extra-colonic anomalies have been described to coexist with JPS including extracolonic cancers, hydrocephaly, amyotonia congenita, polydactyl, gut malrotation, hypertelorism, undescended testes, mesenteric lymphangioma and acute porphyria. However, an association of EoE with JPS has not been previously described. Since alterations in the TGF β signaling have also been noted in EoE, the existence of a common pathway is plausible. Our patient continues further evaluation. Since most patients with diffuse polyposis undergo prophylactic colectomy, EoE would potentially interfere with nutrition in the post-operative period and as such affect recovery and prognosis. There obviously is a need for further research and vigilance to determine if there truly is an association between JPS and EoE.

426 Eosinophilic Gastroenteritis Masquerading as Partial Duodenal Obstruction. K.D. Bhosrekar, Pediatrics, Peyton Manning Children's Hospital at St. Vincent, Indianapolis, Indiana, UNITED STATESE.A. Schaefer, Pediatric Gastroenterology, Peyton Manning Children's Hospital at St. Vincent, Indianapolis, Indiana, UNITED STATES. Background: Eosinophilic gastroenteritis (EGE) is an uncommon condition characterized by eosinophilic infiltration of gastrointestinal tissues and manifested by symptoms of varying quality and severity. We present an unusual case of a toddler with antral and duodenal wall thickening and partial duodenal obstruction related to EGE. Case Report: A 2 ½ year old girl presented with progressively worsening abdominal pain, vomiting, and dehydration. Past history was notable for milder abdominal pain four months prior with endoscopy at that time revealing duodenal erosions and mild esophageal eosinophilia treated with lansoprazole. Multiple food allergens had been identified and she had adopted an elimination diet with variable compliance. Her symptoms had been quiescent until days leading to her hospital admission. Testing revealed a normal CBC, CMP, and lipase. Stool was hemoccult positive. Abdominal ultrasound showed marked antral thickening and irregularity of the duodenal bulb and proximal duodenum. A contrast study showed similar features along with marked narrowing of the proximal duodenum although barium was able to pass through. Due to concern for a possible mass lesion, a CT scan of the abdomen was performed showing thickening of the gastric antrum, duodenal bulb and the proximal duodenal wall. Upper gastrointestinal endoscopy confirmed a deformed appearance of the antrum, pylorus and duodenal bulb, focal narrowing in the second portion of the duodenum, and vertical lines of the esophageal mucosa. Gastric biopsies demonstrated dense eosinophilia in the antrum (up to 50 eosinophils per HPF) and esophagus (100 eosinophils per HPF with submucosal fibrosis). Multiple duodenal and colonic biopsies were obtained but were unrevealing. A diagnosis of EGE was made with esophageal and gastric mucosal involvement and duodenal narrowing suggesting muscularis involvement. Rapid clinical improvement was noted with amino acid based formula and corticosteroid therapy. After several weeks of elemental formula and steroid taper, the patient remained symptom free. Endoscopic and ultrasound improvement were confirmed (pre- and post- treatment images will be presented). Conclusion: EGE is an uncommon and under-recognized disorder in children. Diagnosis hinges on tissue eosinophilia, however, the diagnosis may be obscured by the patchy nature of the disease and muscular EGE subtypes. This case highlights the diagnostic dilemma of a child presenting with acute obstructive features, a suspected mass lesion and tissue samples that may underestimate the depth of involvement. Recognizing the potential for EGE is critical in cases that may have otherwise lead to surgical exploration. Non- invasive monitoring by ultrasound was helpful in assessing improvement on therapy. The short and long term management of pediatric EGE is not well established and there is a need for double-blinded, controlled trials to examine efficacy of nutritional treatment and/or corticosteroids for induction and maintenance.

427 No Pain, No Diagnosis? An unusual case of duodenal duplication cyst with intussusception and electrolyte disturbance in an adolescent girl. V. Shakhnovich, S. St. Peter, J. Fraser, B. Reading, J. Colombo, Children's Mercy Hospital, Kansas City , Missouri, UNITED STATES. Duodenal duplication cysts are rare congenital malformations of the digestive tract, most commonly diagnosed in childhood, with an estimated incidence of 1 per 100,000 live births (1, 2). Abdominal pain and pancreatitis are the most common and consistent presenting symptoms and findings at diagnosis (2). Other than elevated pancreatic enzymes, most commonly secondary to the compression of the ampulla of Vater or the pancreatic duct, laboratory abnormalities are not a typical feature of duodenal duplication (3); however, in theory, electrolyte disturbances could occur secondary to vomiting or duodenal obstruction. We describe the case of a previously healthy 14- year-old African American female, with a family history of Crohn’s Disease, who presented with a one-month history of intermittent, non-bloody, non-bilious emesis and a 10-lb weight loss, in the complete absence of abdominal pain. Review of systems was negative for headache, diarrhea, blood in the stool, toxic ingestion, somnolence, malaise, fever or chills. A detailed history revealed absence of daily vomiting and no history of emesis 24 hours prior to, or subsequent to, hospital admission; however, the patient was found to have profound hypokalemic, hypochloremic, metabolic alkalosis that was resistant to correction with IV fluid or electrolyte repletion. She was also noted to have elevations in amylase and lipase, without abdominal pain. An ultrasound of the abdomen was performed and a possible intussusception, with an ill-defined lead point, was identified. This finding was corroborated by CT of the abdomen, which confirmed a duodeno-duodenal intussusception with a duodenal duplication cyst and malrotation. She was taken to the operating room for successful Ladd procedure and laparoscopic reduction of duodenal intussusception with duodenal duplication cyst resection and primary duodeno-duodenal anastamosis. The rise in the patient’s pancreatic enzymes and her electrolyte disturbance resolved after the procedure. Our case is unique in its unusual presentation of marked electrolyte disturbance (profound hypokalemic, hypochloremic, metabolic alkalosis resistant to correction) and the complete absence of abdominal pain. To our knowledge, only two cases of duodenal duplication cyst with intussusception have been described in the literature (2). In both cases, abdominal pain was a prominent symptom. Furthermore, although pancreatitis, which occurs in greater than 50% of cases of duodenal duplication (1, 2), can be seen in the setting of duodenal duplication, electrolyte abnormalities are not a common feature. 1. Katzka DA & Jaffe DL, Eds. Clinical challenges and images in GI: a rare cause of acute pancreatitis in an adolescent. Gastroenterology 2011; 140:783- 784 2. Chen JJ, Lee HC, Yeung CY, et al. Meta-analysis: the clinical features of the duodenal duplication cyst. J Pediatr Surg 2010; 45:1598-1606 3. Lim JR, Fitzgerald JF, Dewitt, JM. Clinical Quiz. J Pediatr Gastroenterol Nutr 2004; 38:26

428 Rectal bleeding in a patient with portal hypertension and unusual findings on colonoscopy. M. Shields, L. Saubermann, Division of Pediatric Gastroenterology, University of Rochester Medcial Center, Rochester, New York, UNITED STATES. Portal hypertension can result in the formation of intestinal abnormalities, including varices, portal hypertensive colopathy, and possibly polyps, that are at risk for bleeding. Certain conditions, for example, hypertension, might cause these abnormalities to form at an accelerated rate or bleed more frequently. We present a case report of a 13-year old female with a history of portal vein thrombus now status post meso-rex shunt (a shunt connecting her superior mesenteric vein to the left portal vein) and portal hypertension who has had recurrent bright red blood per rectum whenever she receives a dose of her levalbuterol, a beta-agonist that results in increased blood pressure. Recent colonoscopy revealed the presence of submucosal rectal varices and multiple polyps throughout the colon, both of which could be the cause of her bleeding. Recently, she decreased the use of her levalbuterol which resulted in improvement of her rectal bleeding. While beta-agonists are important in the management of asthma and other respiratory disorders, physicians should remind patients with portal hypertension that beta- agonists increase blood pressure and consequently may increase their risk of gastrointestinal bleeding.

429 Fecal Calprotectin and Pediatric Juvenile Polyps. J. Vanderhoof, Boston Children's Hospital, Boston, Massachusetts, UNITED STATESR. Pauley-Hunter, S. Kunnath, K. Wolff, J. Vanderhoof, Boys Town National Research Hospital, Boys Town , Nebraska, UNITED STATES. Calprotectin is a protein found in neutrophils. Its presence in the feces is a useful marker for intestinal inflammation. It is often utilized to screen for disease activity in inflammatory bowel disease. Juvenile polyps are inflammatory lesions containing large quantities of neutrophils and often present with rectal bleeding which can be confused with inflammatory bowel disease (IBD). We have recently observed 4 patients with juvenile polyps who had very high levels of fecal calprotectin which normalized immediately after polypectomy. The cases included a 29 month old female with bloody, mucousy, loose stools for one month, an 8 year old male with intermittent blood in the stools for one year, a 5 ½ year old male with an intermittent history of blood in his stools since age 2 thought to be related to constipation, and a17 year old male with persistent blood in the stools for 6 months. All had elevated fecal calprotectin levels prior to colonoscopy, and were considered possible IBD cases for that reason. In both of the younger children the initial calprotectin levels were greater than 2500 µg/ml, the 17 year old and 8 year old had levels of 534 and 1100 µg/ml respectively. No pathogens including clostridium difficile were identified. None of the children had anemia, an elevated sedimentation rate or C-reactive protein. Family history for gastrointestinal disease was positive only in the youngest male who had a maternal grandmother with a history of a sigmoid colon resection for diverticulitis. The youngest child had 3 pedunculated polyps removed, two from the descending colon and one from the ascending colon. The other children had solitary polyps located in the sigmoid, descending and transverse colon. All polyps were removed with snare cautery without incident. Histologically, all demonstrated ulcerated surfaces with acute and chronic inflammatory changes characteristic of benign juvenile polyps. Repeat fecal calprotectin levels within 2-3 weeks post procedure were normal in all cases. Fecal calprotectin levels may be elevated in children with juvenile polyps. Juvenile polyps should be considered in the differential diagnosis of an elevated fecal calprotectin and rectal bleeding. Fecal calprotectin cannot be used to differentiate between juvenile polyps and inflammatory bowel disease or as a highly specific marker of IBD.

430 Congenital fallopian tube bands causing large bowel obstruction.. S.I. Wadhwani, Y. Ahmedi, E. Gleghorn, Children's Hospital & Research Center Oakland, Oakland, California, UNITED STATES. Large bowel obstruction is most commonly caused by functional constipation but there are other rare and more dangerous causes. We report a 16 year old female with a history of intermittent constipation who presents with 6 days of abdominal pain after eating fast food. She was originally diagnosed and treated for a urinary tract infection but her symptoms failed to improve. She then started to develop yellow colored emesis and inability to tolerate anything by mouth. She was found to have copious stool on initial abdominal radiograph and underwent an enema, however, her symptoms continued to worsen with persistent emesis, weakness and stool incontinence. A computerized tomography scan demonstrated significant ascending, transverse, descending and proximal sigmoid colon dilatation. There was bowel wall edema with copious retained stool. There was some caliber change at the mid sigmoid area. On admission, the patient started to become febrile, tachycardic, hypotensive and have delayed capillary refill. The patient was immediately transferred to the intensive care unit and subsequently taken to the operating room. Initial colonoscopy showed unremarkable colon mucosa in the distal colon, however, the scope could not be advanced past 90 centimeters. The surgeon proceeded with exploratory laparoscopy. The patient was found to have a band from her right fallopian tube to the omentum that was obstructing the sigmoid colon. Additionally, there was a band from the left fallopian tube that was tethering the sigmoid colon providing further obstruction. The patient was found to have a stool impaction proximal to the obstruction and no other anatomical abnormalities. It is likely that these bands were intermittently obstructing the patient’s large bowel causing her to slowly develop an impaction. We describe a rare cause of large bowel obstruction from congenital fallopian tube bands.

431 Severe Bannayan-Riley-Ruvalcaba Syndrome caused by a deletion encompassing both the BMPR1A and PTEN genes. O. Waisbourd-Zinman, P. Mamula, J.R. Friedman, D.A. Piccoli, Gastroenterology, Hepatology and Nutrition, Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, UNITED STATES. Bannayan-Riley-Ruvalcaba syndrome (BRRS) is a rare autosomal dominant disorder linked to germline PTEN mutations characterized by macrocephaly, penile lentigines, gastrointestinal polyps, thyroid disease (Hashimoto thyroiditis and thyroid cancer), high palate, proximal muscle myopathy and developmental delay. We report a 10 year old male who presented at the age of 15 months with macrocephaly, muscle weakness, developmental delay and chronic diarrhea. Before the age of two years he was found to have numerous juvenile polyps with gastrointestinal bleeding, protein losing enteropathy and hypoalbuminemia. At the age of two years, he had intussusception with subsequent sub-total colectomy. He was later diagnosed with superior vena cava syndrome and needed a balloon angioplasty. At the age of 8 years he was diagnosed with thyroid cancer and had a thyroidectomy with subsequent radioactive iodine treatment after a recurrence. He continued to have recurrent ileo-ileal intussusceptions, abdominal pain, diarrhea and hypoalbuminemia. He was total parenteral nutrition dependent and had colonoscopies every few weeks with numerous polypectomies. Double balloon ileoscopy as well as MRE demonstrated hundreds of polyps in the stomach, small bowel and remaining colon. PTEN is a tumor suppressor gene located on chromosome 10q23.3. Germline mutations in the PTEN gene have been identified in Cowden syndrome, BRRS, and Proteus-like syndrome. At the time our patient was diagnosed with BRRS (age two years), he had PTEN sequencing that did not reveal any mutations. At the age of 8 years, it was repeated and he was found to be heterozygous for a deletion of the entire PTEN gene. Due to the severity of his polyposis, a subsequent genome wide array analysis was performed, which revealed a 3.05Mb deletion of chromosome 10q23.2 to q23.31. This deletion encompassed more than 30 genes including BMPR1A (Juvenile Polyposis Syndrome), PTEN, ACTA2, and FAS. Deletions at chromosome 10q involving both BMPR1A and PTEN cause even more complex disorders. The number of reported cases with 10q deletion is not negligible, but only a few of them address the genetic status precisely. Due to the severity of our patient's small bowel disease with multiple polyps, (also having potential malignancy risk) severe chronic pain, and recurrent intussusception with severe protein losing enteropathy we recommended small bowel transplant evaluation. This case demonstrates the need to understand the limitations of various genetic testing platforms, and to consider additional testing modalities, such as gene specific deletion/duplication analysis or genome wide oligo or SNP array analysis to ensure accurate assessment of all mutational possibilities in disease associated genes. As in the child reported here, accurate identification of the underlying genetic etiology enables the clinician to provide a more accurate prognosis for the patient and helps in guiding appropriate therapeutics as well as meaningful counseling to the family about recurrence risk and risk to other family members.

452 Methylnaltrexone use in a patient with mitochondrial disorder dysmotility and opioid-induced bowel dysfunction - Case report and Review of Literature. S.B. Oliveira, Pediatrics, University of Medicine and Dentistry of New Jersey, Newark , New Jersey, UNITED STATESS.B. Oliveira, J. Beversdorf, Pediatrics, Hackensack University Medical Center - The Joseph M. Sanzari Children's Hospital, Hackensack, New Jersey, UNITED STATES. Although there are four major types of opioid-binding receptors, analgesia is primarily mediated through the µ (mu) receptors. Presence of µ receptors is within the central and peripheral nervous system and gastrointestinal tract. µ receptor activation in the gastrointestinal tract results in peristalsis inhibition and increased GI secretion, leading to opioid-induced bowel dysfunction presenting as constipation. Methylnaltrexone bromide (MB) is a peripherally acting µ receptor antagonist, recently FDA approved, which does not cross the blood-brain barrier. When used, MB does not interfere with analgesia nor precipitate withdrawal symptoms. We report a case of MB use in a patient with mitochondrial disorder and opioid-induced constipation. Case Report: 20 year-old female with mitochondrial disorder and secondary chronic pain, hospitalized due to increased pain and dehydration. The gastrointestinal dysmotility related to her mitochondrial disease combined with chronic opioid use, resulted in severe constipation with poor response, despite maximal use of different laxatives. 8mg of subcutaneous MB was given, and the patient had rapid laxative response, with substantial relief of the discomfort. She continued having formed stools for the next two days, without additional MB. She had no adverse effects; in fact, the resolution of constipation was crucial for her overall improvement. Discussion: The safety and efficacy of MB has been demonstrated in randomized, double-blind, placebo-controlled studies conducted in adults with advanced illness. The safety and efficacy of MB has not been studied in the pediatric population. Recent case reports indicate good outcomes in pediatric patients. Lee, J.M. (2012), reported a 4 year-old with epidermolysis bullosa that experienced significant improvement of opioid-induced constipation with enteral MB. Kissling, K.T (2012) described an 8 year-old with relapsed neuroblastoma, who had resolution of opioid induced constipation with MB, after other traditional laxatives were unsuccessful. Potential clinical benefits for pediatric patients with opioid- based analgesia regimens for pain (chronic malignant, non-malignant, acute, and other) include: improvement/resolution of constipation, continuation of a opioid-based analgesia, no need for opioid rotation/changes because of gastrointestinal side effects, potential decrease in length of stay for patients with pain that may remain hospitalized due to constipation. In our case, the use of MB for opioid-induced constipation was beneficial and safe in a patient with gastrointestinal dysmotility related to mitochondrial disorder.

Motility/Functional Gastrointestinal Disorders 453 A case of caudal regression syndrome detected in a 3-year-old boy presenting with chronic constipation. J. Hong, Department of Pediatrics, Kangwon National University Hospital , Chuncheon-si, Gangwon, KOREA, REPUBLIC OF. INTRODUCTION: Caudal regression syndrome (CRS) is a rare neural tube defect affecting terminal spinal segment. It manifests as total neurological deficit in the lower limbs and loss of bladder and bowel control. It may be associated with other structural anomalies so that patients are more likely to present first to the pediatric surgeon, the urologist, the orthopedic surgeon or neurosurgeon, than to the pediatric gastroenterologist. Delayed diagnosis increases the risk of renal impairment, recurrent urinary tract infections, and fecal incontinence. Moreover, it can be a progressive neurological disorder due to in association with a tethered cord. CASE REPORT: A 27 month-old boy was referred to our pediatric GI department for a several-month history of constipation. He was born to a mother with type 2 diabetes and had a history of hypocalcemic seizure at 10 days of age, when initial workup included PTH, TFT and CATCH 22 FISH was unremarkable. He was also diagnosed with congenital hearing impairment when newborn hearing screening test was performed. The mother of the boy have noticed that his constipation had started since infancy. She also complained that it was hard to control his diarrhea when she small amounts of laxative. His voiding stream was not considered to be weak and dribbling of urine was sometimes noticed. On examination, he had low set ears with hearing aids and small buttocks with dimples at the lateral aspects of the upper buttocks. However, he was active and had passed normal mental milestones except a delay of language development. He showed no motor deficit of his lower limbs and had sensation in the lower limbs and perineum without any gross deformities. X-ray of the abdomen revealed absence of the distal sacrum and the coccyx. A voiding cystourethrogram showed poor bladder filling with no reflux. MRI of the spine identified abrupt termination of the conus medullaris at the level of T12~L1 and thickened filum terminale. Associated anomalies reported in CRS were not detected by abdomen and kidney ultrasonography, echocardiography and Brain MRI. Conclusion: Although CRS has a number of associated anomalies, this is a unique case report there was a congenital sensory-neural hearing loss along with the CRS. We present this case to highlight the fact that spinal dysraphism should be kept in the differential diagnosis when confronting the patient with chronic constipation and congenital anomalies even though showing no neurologic deficit.

454 Mitochondrial Neurogastrointestinal Encephalomyopathy (MNGIE) Complicated by Superior Mesenteric Artery (SMA) Syndrome: Pediatric Case Presentation. S. Kinberg, A.A. Mencin, Pediatric Gastroenterology, Columbia University Medical Center, New York, New York, UNITED STATES. MNGIE is a rare multisystemic autosomal recessive disorder with only 200 cases reported worldwide. Presentation is typically in childhood. However, diagnosis is often delayed by years, and patients are often misdiagnosed. The broad spectrum of clinical presentations of MNGIE and its low prevalence are largely responsible for a delayed diagnosis. Gastrointestinal (GI) dysmotility is the most common presenting feature and has been misattributed to anorexia nervosa, inflammatory bowel disease, celiac disease and SMA syndrome. We present a case of MNGIE syndrome complicated by SMA syndrome. Case: A 19-year-old male with abdominal pain, nausea, vomiting and weight loss was transferred to our center. He reported a history of recurrent abdominal pain and vomiting since age 3 with poor weight gain since age 10. He had bilateral hearing loss and mild speech delay. One month prior to presentation, he had an exacerbation of symptoms and unintentional weight loss of 11 pounds. He was cachectic (weight 35 kg, height 159 cm, BMI 13.9 kg/m2). He had bilateral ptosis, ophthalmoplegia and hyporeflexia of the bilateral lower extremities. Esophagogastroduodenoscopy revealed a dilated stomach with 600 cc of bilious fluid. Biopsies showed reflux esophagitis and mild gastritis. Colonoscopy was normal. Gastric Emptying Scan showed delayed gastric emptying. Magnetic Resonance (MR) Enterography and Upper GI Series with Small Bowel Follow Through were suggestive of SMA syndrome, which was confirmed by MR Angiography (MRA) of the abdomen. Brain MR Imaging revealed diffuse white matter abnormality. Electromyography showed a demyelinating sensorimotor peripheral neuropathy. Bilateral high frequency sensorineural hearing loss was found on audiometry. MNGIE disorder was suspected because of the phenotypic manifestations and radiological findings. Thymidine Phosphorylase (TP) activity was decreased with elevated plasma thymidine, consistent with a diagnosis of MNGIE. Central venous catheter was placed for hyperalimentation treatment with resultant weight gain and improvement in GI symptoms. To our knowledge, this is the first case report to demonstrate SMA syndrome via MRA as a complication of MNGIE. MNGIE is characterized by GI dysmotility, cachexia, ptosis, peripheral neuropathy and leukoencephalopathy. The disease is caused by mutations in the gene encoding TP, resulting in mitochondrial dysfunction. It is a progressive and fatal disease with an average age-at-death of 37-years-old. Treatment options are limited. Nutritional support can relieve some of the GI symptoms of MNGIE and can prevent the development of SMA syndrome. Enzyme replacement by allogeneic hematopoietic stem cell transplantation (HSCT) has been explored with varying success, and our patient is being evaluated for HSCT. Pediatric gastroenterologists should be aware of the possible diagnosis of MNGIE in patients with coexistent GI dysmotility, cachexia, ocular and neurologic manifestations. Recognition of this disorder is critical as early diagnosis can facilitate directed symptomatic relief and genetic counseling and may retard the progression of MNGIE.