The gastrointestinal tract is a significant source of morbidity in adults with cystic fibrosis (CF). It is also associated with a wide range of complications, few of which are specific to the underlying disease.

Abnormal cystic fibrosis transmembrane conductance regulator (CFTR) function, with decreased bicarbonate and other ion transport levels through the apical surface of epithelial cells, have the ability to influence the intestinal tract including the pancreas and the liver. Gastrointestinal CFTR dysfunction, comparable to observations in the respiratory tract causes interrelated features including mucus accumulation, dysmotility, small bowel bacterial overgrowth and inflammation with alteration of innate immune responses. Approximately, half of adult patients with CF in developed countries are followed in multidisciplinary CF care centres by pneumologists who often have to manage gastrointestinal complications.

Therefore, it is essential for adult gastroenterologists to develop the expertise required for managing gastrointestinal complications associated with CF in partnership with multidisciplinary CF care centre teams to enhance the quality of life of adult patients with CF.

Source: Munck A, Languepin J, Debray D, Lamireau T, Abely M, Huet F, Maudinas R, Michaud L, Mas E. Management of pancreatic, gastrointestinal and liver complications in adult cystic fibrosis. Rev Mal Respir. 2015 Jun;32(6):566-85.