Definition:

Familial cirrhosis is a condition that is associated with the presence of liver disease with genetic linkage among multiple family members in a generation or in multiple generations. The well-documented causes of familial cirrhosis are mutations of keratin 8 and 18 gene. They are the cytoskeletal intermediate filament proteins of hepatocytes, and protect the liver from various forms of injury. It has been shown that KRT8 or KRT18 mutations predispose the liver to acute or subacute injury and promote apoptosis and fibrosis.