Journal of Pediatric Gastroenterology and NutritionVolume 39, Issue S1 p. S296-S296 ABSTRACTS: Poster Session Abstracts P0629 CARCINOID TUMOR COMPLICATING CROHN’S DISEASE IN A CHILD WITH INTESTINAL OBSTRUCTION G. Tomer, G. Tomer Division of Pediatric Gastroenterology, Maimonides Medical Center, Brooklyn, United StatesSearch for more papers by this authorS. Narwal, S. Narwal Division of Pediatric Gastroenterology, Maimonides Medical Center, Brooklyn, United StatesSearch for more papers by this authorG. Bultron, G. Bultron Department of Pediatrics, Maimonides Medical Center, Brooklyn, United StatesSearch for more papers by this authorG. Wetzler, G. Wetzler Division of Pediatric Gastroenterology, Maimonides Medical Center, Brooklyn, United StatesSearch for more papers by this author G. Tomer, G. Tomer Division of Pediatric Gastroenterology, Maimonides Medical Center, Brooklyn, United StatesSearch for more papers by this authorS. Narwal, S. Narwal Division of Pediatric Gastroenterology, Maimonides Medical Center, Brooklyn, United StatesSearch for more papers by this authorG. Bultron, G. Bultron Department of Pediatrics, Maimonides Medical Center, Brooklyn, United StatesSearch for more papers by this authorG. Wetzler, G. Wetzler Division of Pediatric Gastroenterology, Maimonides Medical Center, Brooklyn, United StatesSearch for more papers by this author First published: 01 June 2004 https://doi.org/10.1002/j.1536-4801.2004.tb13059.x Submitted by: [email protected] Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onEmailFacebookTwitterLinkedInRedditWechat No abstract is available for this article. Volume39, IssueS1June 2004Pages S296-S296 RelatedInformation

Introduction: Nonalcoholic fatty infiltration of the liver is a known sequel of obesity in adults. It has previously been reported that fatty infiltration of the liver affects 2.6% of children and 10–25% adolescents with obesity. Serum levels of alanine (ALT) and aspartate aminotransferase (AST) have been proposed as surrogate markers of hepatic fat accumulation. We describe the incidence of abnormal liver enzymes in obese children involved in Kids–Weight Down Program and attempt to seek correlations between other variables associated with insulin resistance. Methods: One hundred fifty six (ages 5 to 20 years) obese children were enrolled. Liver enzymes, fasting lipid profile, thyroid function tests, glucose and insulin levels were obtained and screened. Results: ALT and AST were elevated in 30/156 (19.2 %) children with obesity and normal glucose tolerance. In the group with elevated liver enzymes, HDL was significantly lower and triglycerides (TG) were significantly higher (P <0.001). TG level correlated positively with ALT level (r=0.39, P< 0.001). While HDL negatively correlated with ALT level (r= − 0.29, p <0.001). Ratio TG/HDL correlated with ALT (r= 0.37, p <0.001) and AST (r=0.27, p< 0.001). Frequency of elevated liver transaminases increases with age: from 15% at 5–10 years of age, to 18 % at 11 to 15 years and 31 % at 16-to 20 years of age. There were no differences in insulin resistance index (QUICKI), BMI or age between groups. Conclusion: Elevated liver transaminases are a frequent complication of obesity in up to 19 % of our patients. Frequency of such complications increases with age. Elevated TG/HDL index can be a marker of abnormal liver enzymes, an important surrogate marker of fatty infiltration of the liver.

Cholestasis occurs in ≈25% of low birthweight infants receiving parenteral nutrition via central venous catheter (TPN). Although progressive liver injury may evolve, other etiologies should be considered in infants with TPN-related hepatic dysfunction. We studied African-American male, dizygotic twins (26 wk gestation; twins A/B = 745/800 g), presenting with direct hyperbilirubinemia after 1 m of TPN and partial enteral feedings (using a disaccharide-free formula containing medium-chain triglycerides). In twin B only, “TPN-cholestasis” was complicated by hypoalbuminemia, and a coagulopathy developed at 4 m. Physical examination demonstrated firm, nodular hepatomegaly, splenomegaly and ascites. Further studies included: AST/ALT/γGTP = 134/34/127 U/L (nl <40); α1-antitrypsin = Pi MM; Hepatitis A-C serologies = negative. Metabolic evaluation demonstrated: Table Findings were consistent with a diagnosis of Tyrosinemia type I. Therapy with 2-(nitro-4-trifluoromethylbenzoyl)-1,3-cyclohexanedione (NTBC) was attempted; however, the infant expired prior to starting treatment. Postmortem, skin fibroblast culture generated normal fumarylacetoacetase activity. Twin A's studies were normal, and cholestasis resolved without complications. CONCLUSIONS: 1. Our patient represents the earliest reported case of Tyrosinemia type I with apparent mosaicism (J Clin Invest 1994;94;1657-61); 2. These data emphasize the importance of early, thorough metabolic evaluations in infants with presumed TPN-associated hepatic dysfunction.