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Cystic fibrosis: Hepatobiliary disease

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


Cystic fibrosis-related liver disease (CFLD) is becoming increasingly recognized due to early diagnosis of cystic fibrosis (CF), improved life expectancy, and more vigilant screening and monitoring by CF providers. Patients with severe CFLD usually present during childhood, and the disease tends to progress rapidly. Milder forms of CFLD can be difficult to diagnose because patients may remain asymptomatic until late in the disease process, and currently available screening tests correlate poorly with disease severity. Early identification of CFLD permits anticipation and treatment of its 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: Sep 2017. | This topic last updated: Oct 12, 2015.
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