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.
- cystic fibrosis
- pancreatic disease
- cancer syndromes
- chronic liver disease
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Contributors Following Professor Beatties kind invitation, my colleague MS and myself planned the main points of the article. MS researched new studies in pubmed. MS wrote the first draft after which we wrote the article together.
Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.
Competing interests None declared.
Patient consent for publication Not required.
Provenance and peer review Not commissioned; externally peer reviewed.
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