Exocrine pancreatic function in children with progressive familial intrahepatic cholestasis type 2

Journal of Pediatric Gastroenterology and Nutrition
Jaroslaw WalkowiakJerzy Socha

Abstract

In progressive familial intrahepatic cholestasis type 2 (PFIC-2), severe steatorrhea is often documented. However, pancreatic exocrine secretion has not yet been studied. In 14 children with PFIC-2, pancreatic function was assessed using standard fecal tests. Normal fecal lipase concentrations excluded isolated lipase deficiency. No differences in fecal elastase-1 concentrations and chymotrypsin activities were detected between PFIC-2 patients with or without steatorrhea, nor between these patients and healthy subjects. In conclusion, pancreatic exocrine function in patients with PFIC-2 is normal. Steatorrhea observed in those patients is not related to pancreatic insufficiency.

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Citations

Mar 17, 2010·BMC Gastroenterology·Lee M BassPeter F Whitington
Jul 17, 2012·Clinics and Research in Hepatology and Gastroenterology·Ralf KubitzDieter Häussinger
Apr 9, 2008·Gastroenterology·Sandra S StrautnieksRichard J Thompson
Dec 23, 2008·Hepatology : Official Journal of the American Association for the Study of Liver Diseases·Jane A ByrneRichard J Thompson
Mar 1, 2012·Hepatology Research : the Official Journal of the Japan Society of Hepatology·Isabella GiovannoniFilippo M Santorelli
Oct 23, 2009·Liver International : Official Journal of the International Association for the Study of the Liver·Li-Yan LiuJian-She Wang
May 8, 2010·Journal of Hepatology·Ludmila PawlikowskaLaura N Bull
Dec 18, 2013·Molecular Aspects of Medicine·Anna BaghdasaryanMichael Trauner
Oct 18, 2016·Scientific Reports·Dorota GliwiczJarosław Walkowiak
Nov 21, 2020·Pediatric Gastroenterology, Hepatology & Nutrition·Wen ZhangLi Liu

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