title: Gastrointestinal, Pancreatic, and Hepatobiliary Manifestations of Cystic Fibrosis.
abstract: Pulmonary disease is the primary cause of morbidity and mortality in people with cystic fibrosis (CF), but significant involvement within gastrointestinal, pancreatic, and hepatobiliary systems occurs as well. As in the airways, defects in CFTR alter epithelial surface fluid, mucus viscosity, and pH, increasing risk of stasis through the various hollow epithelial-lined structures of the gastrointestinal tract. This exerts secondary influences that are responsible for most gastrointestinal, pancreatic, and hepatobiliary manifestations of CF. Understanding these gastrointestinal morbidities of CF is essential in understanding and treating CF as a multisystem disease process and improving overall patient care.
authors.affiliation: Division of Pediatric Gastroenterology and Nutrition, Children's Health, University of Texas Southwestern, F4.06, 1935 Medical District Drive, Dallas, TX 75235, USA.
authors.forename: Meghana Nitin
authors.initials: MN
authors.lastname: Sathe
authors.affiliation: Division of Pediatric Gastroenterology, Hepatology and Nutrition, Children's Healthcare of Atlanta, Emory University, 2015 Uppergate Drive, Northeast, Atlanta, GA 30322, USA. Electronic address: afreem6@emory.edu.
authors.forename: Alvin Jay
authors.initials: AJ
authors.lastname: Freeman
mesh_terms: D003550:Cystic Fibrosis / Q000175:diagnosis* / Q000628:therapy; D019005:Cystic Fibrosis Transmembrane Conductance Regulator / Q000235:genetics; D005767:Gastrointestinal Diseases / Q000175:diagnosis* / Q000628:therapy; D006801:Humans; D008107:Liver Diseases / Q000175:diagnosis* / Q000628:therapy; D010182:Pancreatic Diseases / Q000175:diagnosis* / Q000628:therapy
chemical_list: C505032:CFTR protein, human; D019005:Cystic Fibrosis Transmembrane Conductance Regulator
keywords: CF and GI manifestations; CF pancreas; CFLD (CF liver disease); PERT (pancreatic enzyme replacement therapy)