What is cystic fibrosis liver disease?

Cystic fibrosis (CF) is the most common genetic disease in Caucasians. As our understanding and management of children with CF continues to improve, complications from cystic fibrosis liver disease (CFLD) are becoming more common.

CFLD is varied and can involve either the liver or the gallbladder. When it affects the liver, problems can include mild elevation of liver enzymes, hepatic steatosis (fat in the liver), focal biliary cirrhosis and neonatal cholestasis. When the gallbladder is involved, it can cause cholelithiasis (gallstones), cholecystitis (inflammation of the gallbladder), and micro-gallbladder.

CFLD is a complication that may impact a person’s survival and quality of life.

At The Children's Hospital of Philadelphia, children with CFLD are treated by hepatologists and gastroenterologists in the Biesecker Pediatric Liver Center and the Division of Gastroenterology (GI), Hepatology and Nutrition.