Faecal elastase-1 concentration in cystic fibrosis patients with CFTR I1234V mutation.
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.
Abdel Rahman
H
H
Department of Paediatrics, Hamad Medical Corporation, Doha, Qatar.
Abdul Wahab
A
A
Abdel Rahman
M O
MO
Mostafa
Ossama Abdel Rahman
OA
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*
D019005:Cystic Fibrosis Transmembrane Conductance Regulator; D010196:Pancreatic Elastase