HEPATOBILTARY SCANNING IN PATIENTS WITH CYSTIC FIBROSIS AND LIVER DISEASE BEFORE AND AFTER URSODEOXYCHOLIC ACID (UDCA) THERAPY

It has recently been indicated that 96% of patients with liver disease secondary to Cystic Fibrosis (CF) showed evidence of common bile duct stenosis on hepatobiliary scanning (Gaskin et Al:N.Eng.J.Med.318:340, 1938). We performed IDA scan pre and 10-12 months post administration of the hydrophilic and choleretic UDCA (15mg/Kg/day) in 8 children with CF related liver disease. Marphological pattern as well as functional parameters(T½ of hepatic wash-out, time of visualizationof the intestine) were evaluated. At baseline, evidence of severe biliary obstruction was found in 2 cases, striking visualization of secondary and tertiary bile ducts in 5 and dilatation of cammon bile duct in 4; an enlarged gallbladder with delayed emptying was present in 2 patients and in 3 the gallbladder could not be visualized. After UDCA therapy, morphologic appearance at IDA scan improved markedly in all patients: none showed evidence of biliary obstruction, dilatation of common bile duct and intrahepatic ducts was substantially reduced and in all cases the gallbladder was visualized and appeared to empty normally. Mean T½ of hepatic wash-out decreased from 50 ± 21 min.at baseline to 36.0 ± 18 min.after therapy and mean time of visualization of the intestine decreased from 46.8 ± 40.8 min.to 17.5 ± 9.3 min.after UDCA. In all patients on UDCA liver function tests improved significantly and enrichment of the biliary bile acid pool with UDCA (from 4.8 ± 2.2 % to 28.5 ± 9.6 %)occurred.