title: Exocrine pancreatic function in children with progressive familial intrahepatic cholestasis type 2.
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.
authors.affiliation: Department of Gastroenterology and Metabolism, Poznan University of Medical Sciences, Poznan, Poland. jarwalk@am.poznan.pl
authors.forename: Jaroslaw
authors.initials: J
authors.lastname: Walkowiak
authors.forename: Irena
authors.initials: I
authors.lastname: Jankowska
authors.forename: Joanna
authors.initials: J
authors.lastname: Pawlowska
authors.forename: Sandra
authors.initials: S
authors.lastname: Strautnieks
authors.forename: Laura
authors.initials: L
authors.lastname: Bull
authors.forename: Richard
authors.initials: R
authors.lastname: Thompson
authors.forename: Karl-Heinz
authors.initials: KH
authors.lastname: Herzig
authors.forename: Jerzy
authors.initials: J
authors.lastname: Socha
mesh_terms: D000293:Adolescent; D015415:Biomarkers / Q000032:analysis; D002648:Child; D002675:Child, Preschool; D002780:Cholestasis, Intrahepatic / Q000201:enzymology / Q000235:genetics / Q000503:physiopathology*; D002918:Chymotrypsin / Q000032:analysis; D005243:Feces / Q000201:enzymology*; D005260:Female; D006801:Humans; D007223:Infant; D008049:Lipase / Q000032:analysis; D008297:Male; D046790:Pancreas, Exocrine / Q000502:physiology*; D010196:Pancreatic Elastase / Q000032:analysis; D045602:Steatorrhea / Q000209:etiology*
chemical_list: D015415:Biomarkers; D008049:Lipase; D002918:Chymotrypsin; D010196:Pancreatic Elastase