URSODEOXYCHOLIC ACID EFFECTS ON CYSTIC FIBROSIS LIVER DISEASE

Cystic fibrosis (CF) is the most frequent monogenic autosomal recessive disease with lethal potential, manifested by an important clinical polymorphism. Cystic fibrosis associated liver disease (CFLD) is the second non-pulmonary cause of death in children with cystic fibrosis, being an important monitoring problem. The only existent treatment is ursodeoxicholic acid (UDCA), which has controversial efficiency. The aim of this study was to evaluate the efficiency of the treatment with UDCA on CFLD evolution. The study included 51 patients with CFLD, prospectively monitored for five years. They were routinely followed-up by clinical assessment, liver biochemical tests and ultrasound examinations. All patients with CFLD received UDCA in a dose of 15-20 mg/kg bw/day permanently. Liver tests results and ultrasound Williams score were used for CFLD monitoring as primary endpoints. After UDCA administration, transaminases values decreased significantly in one year of treatment. Important improvements in liver parenchyma were documented by the ultrasound score. UDCA showed a valuable influence on CFLD outcome and should be recommended as soon as diagnosis is made.