Hepatic Manifestations of Cystic Fibrosis.

ABSTRACT: Cystic fibrosis liver disease (CFLD) remains the third leading cause of death in patients with cystic fibrosis. Although most patients with CFLD present in childhood, recent studies suggest a second wave of liver disease in adulthood. There are no clear guidelines for diagnosing CFLD. Treatment options for CFLD remain limited, and while UDCA is widely used, its long-term benefit is unclear. Those who develop hepatic decompensation or uncontrolled variceal bleeding may benefit from liver transplant, either alone, or in combination with lung transplant.

SUBMITTER: Sakiani S 

PROVIDER: S-EPMC6452885 | biostudies-literature | 2019 May

REPOSITORIES: biostudies-literature