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成人慢性非溶血性非结合胆红素血症中肝脏胆红素UDP-葡萄糖醛酸基转移酶活性

Liver bilirubin UDP-glucuronosyltransferase activity in chronic nonhemolytic unconjugated hyperbilirubinemia of adults.

作者信息

Watanabe A, Wakabayashi H, Kuwabara Y, Yamamoto H, Hattori S, Tsuji T

机构信息

Third Department of Internal Medicine, Faculty of Medicine, Toyama Medical and Pharmaceutical University, Japan.

出版信息

Res Exp Med (Berl). 1998 Apr;197(6):329-36. doi: 10.1007/s004330050081.

Abstract

Ten adult patients with chronic nonhemolytic unconjugated (indirect) hyperbilirubinemia were analyzed by determining bilirubin uridine diphosphate-glucuronosyltransferase activity according to a more physiological and sensitive method (9 control cases, 0.457 +/- 0.163 nmole/mg protein/min). There was no overlap of the enzyme activities of 2 cases with Crigler-Najjar syndrome (type II) (0.006 nmole/mg protein/min on average) and 6 cases with Gilbert's syndrome (0.051 +/- 0.016 nmole/mg protein/min). The enzyme activities in 2 patients with post-hepatitic hyperbilirubinemia were within the normal range. A new classification of nonhemolytic unconjugated hyperbilirubinemia in adults is proposed according to the results of this enzyme activity and the recent data on the gene mutation of this enzyme.

摘要

通过一种更符合生理且更敏感的方法测定胆红素尿苷二磷酸 - 葡萄糖醛酸基转移酶活性,对10例患有慢性非溶血性非结合(间接)高胆红素血症的成年患者进行了分析(9例对照病例,0.457±0.163纳摩尔/毫克蛋白质/分钟)。2例克里格勒 - 纳贾尔综合征(II型)患者(平均0.006纳摩尔/毫克蛋白质/分钟)和6例吉尔伯特综合征患者(0.051±0.016纳摩尔/毫克蛋白质/分钟)的酶活性没有重叠。2例肝炎后高胆红素血症患者的酶活性在正常范围内。根据该酶活性结果和该酶基因突变的最新数据,提出了成人非溶血性非结合高胆红素血症的新分类。

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