The changing face of the exocrine pancreas in cystic fibrosis: pancreatic sufficiency, pancreatitis and genotype

J Walkowiak, A Lisowska… - European journal of …, 2008 - journals.lww.com
Cystic fibrosis (CF) is the most frequent cause of exocrine pancreatic insufficiency in
childhood. The cystic fibrosis transmembrane conductance regulator (CFTR) gene encodes
CFTR protein that functions as cyclic AMP-dependent chloride channel allowing the
passage of anions and secondarily water into the lumen of pancreatic ducts. Luminal
chlorides are exchanged for bicarbonates. The lack of CFTR channel or its disrupted
function (being the consequence of CFTR gene mutations) results in reduced volume of …