title: Biliary bile acid composition in Wilson's disease.
abstract: Biliary bile acid composition and pattern of bile acid conjugation with glycine or taurine were found to be within normal limits in six patients with documented Wilson's disease. Four patients had previous biopsy evidence of cirrhosis (three with active hepatitis), but most conventional liver function tests gave normal results at the time of the study. Serum levels of conjugates of cholic acid, measured by radioimmunoassay, were not increased. However, plasma disappearance if intravenously injected glycine conjugate of cholic acid was significantly delayed in all subjects, suggesting that this is a more sensitive test of hepatic excretory function and may be of value for assessing hepatic function in patients with this rare genetic disorder. No evidence of a primary disturbance in bile acid metabolism was found in these patients.
mesh_terms: D000293:Adolescent; D000328:Adult; D001646:Bile / Q000032:analysis* / Q000378:metabolism; D001647:Bile Acids and Salts / Q000032:analysis* / Q000378:metabolism; D002250:Carbon Radioisotopes; D002793:Cholic Acids / Q000627:therapeutic use; D002849:Chromatography, Gas; D002855:Chromatography, Thin Layer; D003300:Copper / Q000378:metabolism; D005260:Female; D005998:Glycine / Q000627:therapeutic use; D006527:Hepatolenticular Degeneration / Q000097:blood / Q000378:metabolism*; D006801:Humans; D008111:Liver Function Tests; D008297:Male; D011868:Radioisotopes; D013654:Taurine / Q000627:therapeutic use
chemical_list: D001647:Bile Acids and Salts; D002250:Carbon Radioisotopes; D002793:Cholic Acids; D011868:Radioisotopes; D013654:Taurine; D003300:Copper; D005998:Glycine