Abstract
A randomized cross over study was conducted to determine the efficacy of ursodeoxycholic aced (UDCA) in the treatment of possible candidates for liver transplantation in children with cholestatic disease. Fourteen patients (Biliary atresia 6, Alagille syndrome 6, alphal-antitrypsin deficiency 2, hypoplastic bile duct syndrome 1) were submitted to this study. The median age was 4.2 years with a range of 0.6-14 years. UDCA was administered orally (median dose 19.5mg/kg/day, range 12.5-24.4mg/kg/day). Assessments, which consisted of pruritus score, physical measurement, total body potassium (TBK), standard liver function tests, and monoethylglycinexylidide (MEGX) formation test, were carried out just before UDCA or placebo was commenced and 8 weeks after that. Each group was crossed over. Ten children completed the whole study. UDCA resulted in no significant improvement either in standard liver function tests nor in nutritional status. Only MEGX at 30 minutes showed significant improvement by UDCA (UDCA vs. Placedo ; +17.1±25.3 vs. -5.3±13.4, P < 0.05). It could be worthwhile to use this drug for longer period in the management of cholestatic children.
