Cystic fibrosis associated with liver disease

In cystic fibrosis (CF), approximately 5-10% of the patients develop multi lobular cirrhosis during the first decade of life. Most of these will develop signs of portal hypertension with complications, mainly variceal bleeding during the second decade, while liver failure is usually kite, after the pediatric age. Annual screening for liver disease is recommended in order to initiate ursodeoxycholic acid, that may halt the progression of liver disease. Liver disease should be considered if at least two of the following variables are present: hepatomegaly and/or splenomegaly; persistent abnormalities of liver enzymes, and pathological ultrasonography of the liver. A liver biopsy is indicated if there is diagnostic doubt. All CF patients with liver disease need annual follow-up in order to evaluate the progression to cirrhosis, and screen for portal hypertension and liver failure. Management should focus on nutrition and prevention of variceal bleeding. The decision for a liver transplant is evaluated with the transplant team, taking into account the liver function and complications of portal hypertension as well as CF related extrahepatic involvement. (C) 2012 Elsevier Masson SAS. All rights reserved.