Hepatic bilirubin-conjugating enzymes of man in the normal state and in liver disease.

Bilirubin UDP-glucuronyl transferase (BGT), bilirubin UDP-glucosyl transferase (BGLT) and bilirubin UDP-xylosyl transferase (BXT) activities were measured in wedge-biopsied liver specimens obtained from patients with various liver diseases, and compared with those in controls with normal liver histology. BGT was measured alone using needle biopsy liver specimens from the patients with Gilbert's syndrome (15 patients). Rotor's syndrome (one) and posthepatitic hyperbilirubinemia (3). BGT was decreased to about 30% of controls in Gilbert's syndrome, but showed no change in posthepatitic hyperbilirubinemia and Rotor's syndrome. About 90% decrease in BGT, and BGLT and BXT were observed in Crigler-Najjar syndrome type II (3 patients). In patients with cholelithiasis and chronic hepatitis, statistically significant changes of these three enzymes were not observed, except the statistically significant increase in BGT activity in chronic hepatitis. Slight increases in BGT and BXT activities were observed in anicteric cases with cholelithiasis. The ratio of BGT, BGLT and BXT activities in controls was 1:0.50:0.98 (expressed as "per mg protein"). Slight differences existed between the ratios of BGT, BGLT and BXT in various liver diseases, and this may suggest the separate identities of BGLT and BXT from BGT. Determination of bilirubin-conjugation is essential in the diagnosis of Gilbert's syndrome and Crigler-Najjar syndrome type II, but shows no specific change in the other chronic liver diseases.