Gastrointestinal Disease and Nutrition in Cystic Fibrosis

Cystic fibrosis (CF) is characterized by an abnormal cystic fibrosis transmembrane conductance regulator (CFTR) gene which causes abnormal transport of chloride. The mutation in this gene creates tenacious secretions in the lung, pancreas, intestinal tract, and hepatobiliary systems. The focus in this chapter will be pancreatic insufficiency, intestinal diseases, hepatobiliary disease, and nutritional factors (Sabharwal S, Schwarzenberg SJ. Cystic fibrosis: overview of gastrointestinal disease. Up to Date. 2019. https://www.uptodate.com/contents/cystic-fibrosis-overview-of-gastrointestinal-disease. Accessed March 2019).