title: The cystic fibrosis intestine.
abstract: The clinical manifestations of cystic fibrosis (CF) result from dysfunction of the cystic fibrosis transmembrane regulator protein (CFTR). The majority of people with CF have a limited life span as a consequence of CFTR dysfunction in the respiratory tract. However, CFTR dysfunction in the gastrointestinal (GI) tract occurs earlier in ontogeny and is present in all patients, regardless of genotype. The same pathophysiologic triad of obstruction, infection, and inflammation that causes disease in the airways also causes disease in the intestines. This article describes the effects of CFTR dysfunction on the intestinal tissues and the intraluminal environment. Mouse models of CF have greatly advanced our understanding of the GI manifestations of CF, which can be directly applied to understanding CF disease in humans.
authors.affiliation: Anatomy and Cell Biology, University of Kansas School of Medicine, Kansas City, Kansas 66160.
authors.forename: Robert C
authors.initials: RC
authors.lastname: De Lisle
authors.forename: Drucy
authors.initials: D
authors.lastname: Borowitz
mesh_terms: D000818:Animals; D001639:Bicarbonates / Q000378:metabolism; D003550:Cystic Fibrosis / Q000150:complications*; D019005:Cystic Fibrosis Transmembrane Conductance Regulator / Q000235:genetics / Q000378:metabolism*; D004195:Disease Models, Animal; D064806:Dysbiosis / Q000209:etiology*; D005183:Failure to Thrive / Q000209:etiology; D005759:Gastroenteritis / Q000209:etiology*; D006801:Humans; D006863:Hydrogen-Ion Concentration; D007413:Intestinal Mucosa / Q000378:metabolism; D007415:Intestinal Obstruction / Q000209:etiology*; D044342:Malnutrition / Q000209:etiology; D051379:Mice; D012333:RNA, Messenger / Q000378:metabolism
chemical_list: D001639:Bicarbonates; D012333:RNA, Messenger; D019005:Cystic Fibrosis Transmembrane Conductance Regulator