title: Normal levels of serum pancreatic enzymes in patients with progressive familial intrahepatic cholestasis type 2.
abstract: BACKGROUND
  High prevalence of elevated serum pancreatic enzymes in children with cholestasis with normal fecal elastase-1 concentrations has been documented. However, this state is related predominantly to biliary atresia. Therefore, we aimed to assess pancreatic damage by measuring serum pancreatic enzymes in patients with progressive familial intrahepatic cholestasis type 2 (PFIC type 2).
  
  
  METHODS
  Twenty PFIC type 2 patients with normal serum bilirubin and bile acid concentrations were included in the study. Thirty pancreatic insufficient cystic fibrosis (PI-CF) patients, thirty patients with acute pancreatitis (AP) and thirty healthy subjects (HS) served for the purpose of comparison. In all subjects, serum lipase and elastase-1 levels were measured.
  
  
  RESULTS
  In all but one PFIC type 2 patients and all HS normal lipase activities were found. Serum elastase-1 concentrations were normal in all PFIC type 2 patients and HS. The enzyme levels were very similar in both groups studied. Lipase activities in PFIC type 2 patients were significantly higher than in PI-CF patients (p < 0.00001) and lower than in patients with AP (p < 0.00001). Serum elastase-1 levels in PFIC type 2 patients were significantly lower than in patients with AP (p < 0.00001) and not different from those in PI-CF patients. In conclusion. serum pancreatic enzymes in patients with PFIC type 2 are normal. No pancreatic damage in these patients could be detected.
authors.affiliation: Poznań University of Medical Sciences, Department of Pediatric Gastroenterology and Metabolism, 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: Aleksandra
authors.initials: A
authors.lastname: Lisowska
authors.forename: Maciej
authors.initials: M
authors.lastname: Biczysko
authors.forename: Aldona
authors.initials: A
authors.lastname: Wierzbicka
authors.forename: Joanna
authors.initials: J
authors.lastname: Pawłowska
mesh_terms: D000293:Adolescent; D002648:Child; D002675:Child, Preschool; D002780:Cholestasis, Intrahepatic / Q000201:enzymology; D005260:Female; D006801:Humans; D008071:Lipoprotein Lipase / Q000097:blood*; D008297:Male; D010179:Pancreas / Q000201:enzymology*; D010196:Pancreatic Elastase / Q000097:blood*; D055815:Young Adult
chemical_list: D008071:Lipoprotein Lipase; D010196:Pancreatic Elastase