Lamireau T et al. (2005) Epidemiology of liver disease in cystic fibrosis: a longitudinal study. J Hepatol 41: 920–925

Patients with cystic fibrosis are at increased risk of liver injury. This is caused by the obstruction of intrahepatic bile ducts by inspissated bile or abnormal mucoid secretions, among other mechanisms. The problem appears to be increasing as survival rates improve, but reports of the exact prevalence of liver disease in these patients vary widely. Lamireau and colleagues have addressed this question in their recent longitudinal study.

The authors reviewed the records of 241 patients diagnosed with cystic fibrosis at a single center in Canada. All underwent clinical and biological assessment every 3 months, and an ultrasound examination of the liver was performed annually. The median follow-up period was 9.8 years.

Liver disease was recorded in 85 patients (35%) during the study, and the median age at diagnosis was 3 years. Nineteen patients (8%) developed cirrhosis and five of these children required a liver transplant. The disease developed mainly in the first decade of life; Kaplan-Meier analysis showed a prevalence of 18%, 29% and 41% at age 2, 5 and 12 years, respectively, and no increase from then on. Pancreatic insufficiency and a history of meconium ileus were independently associated with an increased prevalence of liver disease, whereas age at diagnosis of cystic fibrosis, gender, severity of pulmonary disease and genotype for cystic-fibrosis transmembrane conductance regulator were not.

Although half of the patients with liver disease were treated with ursodeoxycholic acid, this therapy was not randomly administered and so conclusions could not be drawn about its effectiveness. The authors propose that ursodeoxycholic acid treatment might be appropriate for the prevention of liver disease in patients with meconium ileus, and they call for long-term studies in this area.