Hepatic Manifestations of Cystic Fibrosis.

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.