Sidi Y, Aderka D, Brok-Simoni F, Benjamin D, Ramot B, Pinkhas J
Isr J Med Sci. 1980 Feb;16(2):130-3.
Extreme hyperbilirubinemia, massive hemolysis and hepatic encephalopathy were reported in a G6PD-deficient patient with viral hepatitis. Investigation of the G6PD properties revealed that this patient's enzyme represented a hitherto undescribed variant (Bnei Brak), characterized by heat stability and a unique triphasic pH-related enzyme activity curve. It is possible that the marked hemolytic process in this patient was enhanced by a further decrease in the activity of the already deficient enzyme during acidosis.
一名患有病毒性肝炎的葡萄糖-6-磷酸脱氢酶(G6PD)缺乏症患者出现了极重度高胆红素血症、大量溶血和肝性脑病。对G6PD特性的研究表明,该患者的酶代表了一种迄今未描述的变异型(内布拉卡型),其特点是热稳定性以及独特的三相pH相关酶活性曲线。酸中毒期间,该患者体内本就缺乏的酶活性进一步降低,有可能加剧了显著的溶血过程。