EFFECT OF A MEDIUM DOSE OF URSODEOXYCHOLIC ACID WITH OR WITHOUT TAURINE SUPPLEMENTATION ON THE NUTRITIONAL-STATUS OF PATIENTS WITH CYSTIC-FIBROSIS - A RANDOMIZED, PLACEBO-CONTROLLED, CROSSOVER TRIAL

Ursodeoxycholic acid administration has been reported to improve chole stasis and inflammatory activity in primary biliary cirrhosis and, in an uncontrolled study, also in young adults with cystic fibrosis (CF) and chronic cholestasis. As an improvement in nutritional status was a lso observed in these young adult patients, we investigated whether th e administration of a medium dose of ursodeoxycholic acid ameliorates the nutritional status of malnourished young adult CF patients with ch ronic liver disease. The study included 51 patients (27 male patients and 24 female patients; age range, 8-32 years; median, 14) with body m ass percentiles <90%. Patients were randomly assigned to receive eithe r ursodeoxycholic acid (10-12 mg/kg/day) alone or with taurine (18-22 mg/kg/day). Patients were followed in a crossover fashion within each group; 6 months of treatment was randomly alternated with 6 months of placebo. Nine patients dropped out before concluding the study. Liver function tests, nutritional status, and coefficients of fat absorption were determined at entry and after each 6 months of placebo or treatm ent. Nutritional status and fat absorption were not significantly modi fied by either treatment. Liver function tests improved after ursodeox ycholic acid administration only in patients with concomitant chronic liver disease. Our findings indicate that 6 months of therapy with a m edium dose of ursodeoxycholic acid, either alone or with taurine, does not improve the nutritional status of young malnourished CF patients. Higher given for longer periods might be worth investigating.