Progressive familial intrahepatic cholestasis (PFIC) is a rare inherited condition in which children are unable to drain bile from the liver even though the large bile ducts are open (cholestasis).
This usually begins in infants less than 6 months of age and may get worse very quickly. However, some children initially show signs later, even as late as the teen years, and the condition progresses more slowly. Nearly all children with progressive familial intrahepatic cholestasis will require treatment before age 30.
In many cases, progressive familial intrahepatic cholestasis leads to cirrhosis (irreversible scarring of the liver) and liver failure within the first 10 years of life. A liver transplant may be necessary for survival.