Epidemiology of liver disease in cystic fibrosis: a longitudinal study

J Hepatol. 2004 Dec;41(6):920-5. doi: 10.1016/j.jhep.2004.08.006.


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

Methods: 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.

Results: 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.

Conclusions: 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.

MeSH terms

  • Adolescent
  • Age Distribution
  • Child
  • Child, Preschool
  • Cohort Studies
  • Cystic Fibrosis / complications*
  • Disease Progression
  • Exocrine Pancreatic Insufficiency / complications
  • Female
  • Humans
  • Ileus / complications
  • Incidence
  • Infant
  • Liver Diseases / epidemiology*
  • Liver Diseases / etiology*
  • Liver Diseases / physiopathology
  • Longitudinal Studies
  • Male
  • Meconium
  • Prevalence
  • Prognosis
  • Survival Analysis