Cystic fibrosis and the gut

Cystic fibrosis (CF) is a recessive disease caused by mutations in the CF transmembrane conductance regulator (CFTR) gene. The gene product, CFTR protein, has important manifestations in the intestine, pancreas and hepatobiliary system. Increased survival has caused CF to be primarily an adult disease today. Physicians must be knowledgeable as to the varied phenotype in the gastrointestinal tract. This review will outline the main gastrointestinal manifestations including a section on gastrointestinal malignancy in CF. Novel treatments treating the basic effect in CF are now being introduced and their effects on the gastrointestinal tract are discussed.