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Epidemiology of liver disease in cystic fibrosis

A history of meconium ileus and pancreatic insufficiency are predictive of liver disease, reports December's issue of the Journal of Hepatology, which goes on to suggest preventive treatment with ursodesoxycholic acid in patients with meconium ileus could be considered.

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Dr Lamireauac and colleagues from Canada and France designed a study in order to describe the prevalence of liver disease in a cohort of cystic fibrosis (CF) patients.

The researchers followed-up a total of 241 CF patients every 3 months with clinical and biological assessment, and then every year with ultrasonography of the liver.

The research team studied the presence of liver disease using a multivariate Cox's regression analysis including variables such as history of meconium ileus, pulmonary function, pancreatic insufficiency and CFTR gene mutations.

The researchers found that the prevalence of liver disease was 18, 29, and 41% after 2, 5 and 12 years, respectively, and did not increase thereafter.

CF related-liver disease occurs mainly in the first decade of life with a prevalence of 41% of patients at 12 years of age
Journal of Hepatology

The team found on multivariate analysis, the probability of liver disease was independently associated with history of meconium ileus and pancreatic insufficiency.

CFTR mutations and severity of pulmonary disease were not associated with liver disease.

The researchers noted that cirrhosis occurred in 7.8% of patients at a median age of 10 years, and liver transplantation was required in five patients.

Dr Lamireauac concluded, "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."

Journal of Hepatology; 2004: 41, Issue 6, Pages 920-925
06 December 2004

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