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J Hepatol. 2004 Dec;41(6):920-5.

Epidemiology of liver disease in cystic fibrosis: a longitudinal study.

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Division of Pediatric Gastroenterology, Hospital Sainte-Justine, 3175 chemin de la Côte Sainte-Catherine, Montreal, Canada H3T 1C5.



To describe the prevalence of liver disease in a cohort of 241 cystic fibrosis (CF) patients.


241 CF patients were followed-up every 3 months with clinical and biological assessment, and every year with ultrasonography of the liver. The presence of liver disease was studied using a multivariate Cox's regression analysis including variables such as history of meconium ileus, pulmonary function, pancreatic insufficiency and CFTR gene mutations.


The prevalence of liver disease was 18, 29, and 41% after 2, 5 and 12 years, respectively, and did not increase thereafter. In multivariate analysis, the probability of liver disease was independently associated with history of meconium ileus (P = 0.001) and pancreatic insufficiency (P = 0.004). CFTR mutations and severity of pulmonary disease were not associated with liver disease. Cirrhosis occurred in 19 (7.8%) patients at a median age of 10 years, and liver transplantation was required in five patients.


This study shows that CF related-liver disease occurs mainly in the first decade of life with a prevalence of 41% of patients at 12 years of age. A history of meconium ileus and pancreatic insufficiency are predictive of liver disease. Preventive treatment with ursodesoxycholic acid could be considered in patients with meconium ileus.

[Indexed for MEDLINE]

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