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

Research paper by Jaroslaw J Walkowiak, Irena I Jankowska, Joanna J Pawlowska, Sandra S Strautnieks, Laura L Bull, Richard R Thompson, Karl-Heinz KH Herzig, Jerzy J Socha

Indexed on: 28 Apr '06Published on: 28 Apr '06Published in: Journal of pediatric gastroenterology and nutrition


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.