Watanabe A, Wakabayashi H, Kuwabara Y, Yamamoto H, Hattori S, Tsuji T
Third Department of Internal Medicine, Faculty of Medicine, Toyama Medical and Pharmaceutical University, Japan.
Res Exp Med (Berl). 1998 Apr;197(6):329-36. doi: 10.1007/s004330050081.
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.
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。
