title: Faecal elastase-1 concentration in cystic fibrosis patients with CFTR I1234V mutation.
abstract: OBJECTIVE
  To assess the exocrine pancreatic function among cystic fibrosis patients with cystic fibrosis trans-membrane conductance regulator (CFTR) I1234V mutation.
  
  
  METHODS
  Cross-sectional study of 40 cystic fibrosis patients with homozygous CFTR I1234V mutation belonging to a large Arab kindred family and 25 healthy subjects as a control group over a period of 12 mo. Assessment of their exocrine pancreatic function was performed by measuring faecal elastase-1 (FE1) concentration with a commercial ELISA kit using polyclonal antibodies (BioServ Diagnostics) in CF patients compared to healthy subjects. The results were compared with those obtained from a second laboratory using another commercial ELISA (ScheBo; Biotech, Germany) that uses two monoclonal antibodies against different specific epitopes of human pancreatic elastase.
  
  
  RESULTS
  All CF patients with CFTR I1234V mutation had normal levels of faecal elastase 1. No significant difference was found between the two methods for the CF groups or between the CF patients with and without pancreatic enzyme replacement.
  
  
  CONCLUSIONS
  Cystic fibrosis with homozygous CFTR I1234V mutation is associated with pancreatic sufficiency. Assessment of exocrine function using polyclonal antibodies does not significantly differ from that using two monoclonal antibodies against different specific epitopes of human pancreatic elastase.
authors.affiliation: Department of Paediatrics, Hamad Medical Corporation, Doha, Qatar.
authors.forename: H
authors.initials: H
authors.lastname: Abdel Rahman
authors.forename: A
authors.initials: A
authors.lastname: Abdul Wahab
authors.forename: M O
authors.initials: MO
authors.lastname: Abdel Rahman
authors.forename: Ossama Abdel Rahman
authors.initials: OA
authors.lastname: Mostafa
mesh_terms: D000293:Adolescent; D019943:Amino Acid Substitution; D016022:Case-Control Studies; D002648:Child; D002675:Child, Preschool; D003430:Cross-Sectional Studies; D003550:Cystic Fibrosis / Q000235:genetics / Q000378:metabolism*; D019005:Cystic Fibrosis Transmembrane Conductance Regulator / Q000235:genetics*; D005243:Feces / Q000201:enzymology; D005260:Female; D006801:Humans; D008297:Male; D010179:Pancreas / Q000378:metabolism; D010196:Pancreatic Elastase / Q000378:metabolism*
chemical_list: D019005:Cystic Fibrosis Transmembrane Conductance Regulator; D010196:Pancreatic Elastase