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Cystic fibrosis: Hepatobiliary disease

Daniel H Leung, MD
Michael Narkewicz, MD
Section Editors
George B Mallory, MD
Elizabeth B Rand, MD
Deputy Editor
Alison G Hoppin, MD


Liver involvement in cystic fibrosis (CF) is common, occurring in 30 to 50 percent of individuals with CF. CF-related liver disease (CFLD) is a broad term that has been used to define a spectrum of liver involvement in CF. The recognition of CFLD is increasing due to early diagnosis of CF, improved life expectancy, and greater awareness of CFLD leading to screening and monitoring for CFLD. The most significant form of CFLD is cirrhosis with or without portal hypertension. Patients with CF cirrhosis present almost exclusively during childhood, with portal hypertension and associated complications [1]. It is unclear if milder forms of CFLD can identify individuals who will progress to advanced CFLD, and thus available screening tests have targeted nonspecific markers of liver involvement. Early identification of advanced CFLD (prior to the development of complications of portal hypertension) allows prospective monitoring for and treatment of complications, which include malnutrition, variceal bleeding due to portal hypertension, and occasionally liver failure. Other hepatobiliary complications of CF include cholelithiasis (gallstones), cholecystitis, and microgallbladder.

The clinical manifestations, diagnosis, and management of CFLD will be discussed in this topic review, and associated disorders of the gallbladder will be discussed briefly. Other topic reviews relevant to the management of a patient with CF and hepatobiliary disease include:

(See "Cystic fibrosis: Overview of gastrointestinal disease".)

(See "Cystic fibrosis-related diabetes mellitus".)

(See "Cystic fibrosis: Clinical manifestations and diagnosis".)

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Literature review current through: Nov 2017. | This topic last updated: Oct 23, 2017.
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