Best practice guidance for the diagnosis and management of cystic fibrosis-associated liver disease

Dominique Debray, Deirdre Kelly, Roderick Houwen, Birgitta Strandvik, Carla Colombo

Research output: Contribution to journalArticlepeer-review


Approximately 5-10% of cystic fibrosis (CF) patients develop multilobular cirrhosis during the first decade of life. Most CF patients later develop signs of portal hypertension with complications, mainly variceal bleeding. Liver failure usually occurs later, after the paediatric age. Annual screening for liver disease is recommended to detect pre-symptomatic signs and initiate ursodeoxycholic acid therapy, which might halt disease progression. Liver disease should be considered if at least two of the following variables are present: abnormal physical examination, persistently abnormal liver function tests and pathological ultrasonography. If there is diagnostic doubt, a liver biopsy is indicated. All CF patients with liver disease need annual follow-up to evaluate the development of cirrhosis, portal hypertension or liver failure. Management should focus on nutrition, prevention of bleeding and variceal decompression. Deterioration of pulmonary function is an important consideration for liver transplantation, particularly in children with hepatic dysfunction or advanced portal hypertension.

Original languageEnglish
JournalJournal of Cystic Fibrosis
Issue numberSUPPL. 2
Publication statusPublished - Jul 2011


  • Cystic fibrosis
  • Esophageal varices
  • Liver biopsy
  • Liver disease
  • Ultrasonography
  • Ursodeoxycholic acid

ASJC Scopus subject areas

  • Pulmonary and Respiratory Medicine
  • Pediatrics, Perinatology, and Child Health


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