Clinical Vignette Abstracts Thursday October 21, 2010 1 ESOPHAGEAL

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Clinical Vignette Abstracts Thursday October 21, 2010 1 ESOPHAGEAL Clinical Vignette Abstracts Thursday October 21, 2010 1 ESOPHAGEAL INFLAMMATION WITH EOSINOPHILIC INFILTRATION IN CHRONIC GRANULOMATOUS DISEASE B. Kessler, Pediatrics, Good Samaritan Hospital Medical Center, West Islip, NY; S. Schuval, Pediatrics, Schneider Children's Hospital, New Hyde Park, NY Chronic granulomatous disease(CGD)is a primary immunodeficiency disorder of phagocytic oxidative metabolism. Gastrointestinal involvement is common in CGD especially if the inheritance is x‐linked. Colonic disease is the most common area of involvement. Esophageal disease including stricture formation and dymotility, while reported is less common. Few studies have described the histopathology in CGD‐associated esophageal disease but nonspecific inflammation or granuloma formation has beem seen. We report a 16 month old male diagnosed with X‐linked CGD at age 1 month who presented at 16 months with rectal bleeding, abdominal pain and anemia. There was no previous history of vomiting,regurgitation or feeding difficulties. Past history was significant for rectal bleeding at age 10 days while receiving cow's milk‐ based formula and breast milk. The bleeding resolved with substitution of a hydrolyzed cow's milk‐based formula, although breastfeeding without maternal milk avoidance continued. Colonoscopy and upper endoscopy were performed at 16 months. Biopsies of the colon revealed inflammation only of the right side i.e. acute cryptitis and colitis without granuloma. Biopsies of the duodenum and stomach were normal. Esophageal biopsies revealed active esophagitis with 20 eosinophils per HPF and neutrophilic infiltrate without granuloma. GMS and PAS stains were negative for bacteria or fungi. Endoscopic appearance of the distal esophagus demonstrated scattered white plaques. Testing for serum specific IgE antibodies only demonstrated a class II response to beta lactoglobulin. A CBC did not reveal peripheral eosinophilia. Prick puncture skin tests to milk proteins were negative. This patient was not on acid suppression at any time prior to the endoscopic procedures. In conclusion, we report the first case of esophagitis with eosinophilic infiltration in a child with CGD. Whether the findings were specific to esophageal involvement secondary to CGD, gastroesophageal reflux disease or eosinophilic esophagitis is open to speculation. 2 EOSINOPHILIC ESOPHAGITIS (EE) IN A PEDIATRIC PATIENT WITH HERPES SIMPLEX VIRUS ESOPHAGITIS (HSVE). A CAUSE OR A CONSEQUENCE? THE DILEMMA CONTINUES. M. Mavers, A. Shah, S. Kumar, B.A. Becker, A.K. Jain, , Saint Louis University, St Louis, MO; B.A. Becker, A.K. Jain, Pediatrics, SSM Cardinal Glennon Children's Medical Center, St Louis, MO; Background: HSV is a rare, but well described cause of esophagitis. Its association with EE is not clear. We present a patient with HSVE who was found to have EE. Case report: A 12 year‐old previously healthy male presented with fever, emesis, dysphagia, abdominal and ‘burning’ chest pain. Physical exam was benign, except for epigastric tenderness. He had a high sedimentation rate (44 mm/hour) and mild anemia (Hgb 11.5 gm/dL). Endoscopy showed ulceration, erythema and friability of the esophagus with inflammation and basal cell hyperplasia on histology and a normal stomach and duodenum. Immunohistochemical staining and culture showed HSV in proximal and distal esophagus. Immunological workup revealed an elevated white cell count (19.2K/mm3), normal total and subtype immunoglobulin titers, complement activity, NK cell function and no peripheral eosinophilia. Infectious and parasitic workup including ELISA for HIV was negative. IgE immunocap assay was negative for an extensive panel of food allergens. Flow cytometry showed CD4 lymphocytes at 33%, a CD4 count of 786/mm3 with a CD4/CD8 ratio at 2.36. Adequate antibody titers to diphtheria, tetanus and Hib were documented with a poor IgG response to 4 of 7 Prevnar‐7 serotypes. Lymphocyte proliferation to phytohemagglutinin, concanavalin A and pokeweed was decreased but normal for candida and tetanus. He was treated with esomeprazole, sucralfate and acyclovir. Patient had recurring emesis and a repeat endoscopy two months later revealed esophageal furrowing and erythema with greater than 40 eosinophils/HPF on histology. The patient was started on oral fluticasone with an improvement in symptoms. Conclusion: The development of EE following HSVE was unusual. Whether this patient had a primary immunodeficiency or immunomodulation secondary to HSV is debatable. One could envision viral infection(s) predisposing to EE. Physicians should have a high level of suspicion for EE in HSVE patients with persisting symptoms. 3 FATAL ATTRACTION: AWARENESS OF THE MANAGEMENT OF INGESTED MULTIPLE MAGNETS OR SINGLE MAGNET AND A METALLIC OBJECT. D. Mirchandani, A. Aybar, S. Blanchard, A. Malkani, Pediatric Gastroenterology, University of Maryland, Baltimore, MD; Most ingested gastric foreign bodies will pass spontaneously from the stomach. However, if these objects are more than 5 cm long, corrosive in nature, sharp or magnetic they should be removed immediately. The purpose of this case report is to alert clinical providers to the detrimental effects on the gastrointestinal tract when more than one ingested magnet or magnetic object is not removed urgently. If not removed, pressure necrosis resulting from attraction between the magnetic objects across two bowel loops leads to perforation, hemorrhage, volvulus or even death. We describe 3 asymptomatic pediatric patients with known ingestion of multiple magnets. All had successful endoscopic retrieval avoiding possible complications. Case 1: 3 year old swallowed 16 plastic coated cylindrical magnets (resembling mint candy), part of a magnetic building set. Radiograph revealed 14 magnets arranged in a circle in the stomach and 2 in the small bowel. The gastric magnets were removed endoscopically with a snare. Other 2 magnets passed uneventfully. Case 2: 3 year old swallowed 2 magnetic toy pieces. X‐ray confirmed gastric and proximal duodenal location. Successful retrieval of both was accomplished with a Roth Net. Case 3: 9 year old swallowed 2 dumbbell toy magnets. X‐ray revealed gastric location. At endoscopy, the magnets were separated from retained food as they attracted to the biopsy forceps, and removed with a Roth Net. Conclusions: Public and physicians must be made aware of the urgency of managing patients who have ingested multiple magnets or a single magnet and magnetic object to prevent significant morbidity and mortality. In unknown ingestions, when the radiologist determines that more than one metallic object is present radiographically, the clinician should be contacted to verify if one of them is magnetic. If there is a suspicion of magnets accessible with an endoscope, they should be removed urgently, even if asymptomatic. If inaccessible endoscopically, the patient must be observed vigilantly for possible complications. 4 A RARE CASE OF CONCURRENT EOSINOPHILIC ESOPHAGITIS (EOE) AND EOSINOPHILIC GASTRITIS (EOG). M. Le‐Carlson, J.A. Kerner, Pediatric Gastroenterology, Lucile Packard Children's Hospital at Stanford University, Palo Alto, CA; R. Pai, Pathology, Stanford Medical Center, Palo Alto, CA; BACKGROUND: Eosinophilic gastrointestinal diseases are characterized by the infiltration of eosinophils into the gastrointestinal tract mucosa. EoE is the most common form with EoG being much rarer. In general, EoE and EoG are distinct entities that typically are not associated. CASE: A 13 year‐old male with asthma and multiple food allergies presented in 1978 with poor growth, hypoproteinemia and anemia. Small bowel biopsy (Crosby capsule) was negative with no esophageal samples collected at that time. Chromium‐labeled albumin was intravenously injected into the patient and levels measured in the stool. Labeled albumin levels were normal when the patient was not eating but significantly elevated when patient was placed on a normal diet. Patient initially improved with diet restriction, hypoallergenic formula and cromolyn. At 23 years of age, the patient developed dysphagia and a recurrence of hypoproteinemia. Repeat endoscopy was significant for an esophageal ulcer with otherwise normal esophageal and jejunal biopsies. At 43 years of age, the patient underwent a third endoscopy due to ongoing dyspaghia. Endoscopy findings included an esophageal stricture, narrowed antrum and severe eosinophilic infiltration of both the esophagus and stomach consistent with EoE and EoG. Patient was initially treated with oral prednisone alone. Shortly thereafter patient underwent a final endoscopy for a flare and was found to have a feline esophagus and partial gastric outlet obstruction. Patient was then treated with prednisone and swallowed fluticasone with an improvement in symptoms. CONCLUSION: Presented here is an exceedingly rare case of concurrent EoE and EoG. It is important for the clinician to be aware that although typically distinct entities, in rare instances they can occur concurrently and that multiple biopsies may be needed to make the correct diagnosis. 5 PERCUTANEOUS GASTROJEJUNOSTOMY (PGJ) TUBE FEEDINGS IN INFANTS WITH PRUNE BELLY SYNDROME (PBS) V.M. Pineiro‐Carrero, R. Shonce, R. Caicedo, J. Dranove, V. Gopalareddy, Pediatrics, Levine Children's Hospital, Charlotte, NC; Infants with chronic renal disease (CRD) are at risk for malnutrition. These infants often require peritoneal dialysis (PD) and need enteral feedings to achieve their growth potential. We report our experience of PGJ
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