Cystic fibrosis: a multiple exocrinopathy caused by dysfunctions in a multifunctional transport protein

EM Schwiebert, DJ Benos, CM Fuller - The American journal of …, 1998 - amjmed.com
Cystic fibrosis (CF) is a lethal, autosomal recessive genetic disease caused by mutations in
the CF gene that encodes a protein called CFTR (1, 2). According to the latest available
statistics from the CF Foundation (CFF Patient Registry Annual Data Report, August 1995),
approximately 20,000 children and young adults in North America (excluding Canada) have
been diagnosed with CF. This disease presents as a multiple exocrinopathy resulting in
abnormalities in many exocrine tissues including the airways, lung, pancreas, liver, intestine …