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一名携带新型葡萄糖-6-磷酸脱氢酶(G6PD)变体的患者出现病毒性肝炎,伴有极高胆红素血症、大量溶血和脑病。

Viral hepatitis with extreme hyperbilirubinemia, massive hemolysis and encephalopathy in a patient with a new G6PD variant.

作者信息

Sidi Y, Aderka D, Brok-Simoni F, Benjamin D, Ramot B, Pinkhas J

出版信息

Isr J Med Sci. 1980 Feb;16(2):130-3.

PMID:7364565
Abstract

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.

摘要

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。

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