Liver disease in cystic fibrosis

CF is a common hereditary disorder of ion transport, with increasing numbers of patients surviving beyond childhood and developing manifestations of hepatobiliary involvement. Inspissated secretions within the biliary tree result in obstruction and periductular inflammation that eventually progresses to focal and then multilobular cirrhosis. Fatty infiltration of the liver and hepatomegaly is common. Variceal hemorrhage and other findings of portal hypertension may be the initial presentation. At present, therapy with high-dose ursodeoxycholic acid should be considered standard, as it has been shown repeatedly to reduce the injurious effects of the cholestasis. Liver transplantation has been successfully performed on those with advanced disease and adequate pulmonary function. Innovative therapies for CF, including gene transfer, appear promising in preliminary studies, offering hope that earlier intervention in the course of hepatobiliary CF may soon be possible.