葡萄糖-6-磷酸脱氢酶缺乏症溶血的病理生理学

来源 :国外医学.输血及血液学分册 | 被引量 : 0次 | 上传用户:xiomantou_3737
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据估计,红细胞在其生存期中,行程约175英里,共经历约17万个循环周期,其间,要经受湍流、挤压及高渗的影响,并受到潜在杀伤细胞的威胁.同时,红细胞的内环境也由于其高氧载及内源性氧化物的产生而受到威胁.为对抗各种外在和内在危险因素.红细胞具有一种简单而有效的代谢途径以维护其功能.葡萄糖-6-磷酸脱氢酶(G6PD)为维持红细胞的抗氧化系统、保证红细胞的稳定性和活性起着极其主要的作用.本章着重讨论G6PD缺乏症患者摄入蚕豆后发生溶血所出现的一系列病理生理学改变,以阐明其发病机制.摄入蚕豆诱发的溶血蚕豆病的发生率及分布:蚕豆病主要发生于地中海沿岸和中东地区,如巴尔干半岛、希腊、塞浦路斯、土尔其、黎巴嫩、以色列、埃及、北非、伊朗、伊拉克等.在远东主要发生于台湾省和中国南部.其发病 Erythrocytes are estimated to travel approximately 175 miles in their lifetime, experiencing approximately 170,000 cycles, subject to the effects of turbulence, compression and hypertonicity, and are at risk of potentially killing the cells. In the meantime, The environment is also threatened by its high oxygen load and the production of endogenous oxides.To combat a variety of extrinsic and intrinsic risk factors, erythrocytes have a simple and effective metabolic pathway to maintain their function.Glucose-6-phosphate Dehydrogenase (G6PD) plays an important role in maintaining erythrocyte anti-oxidant system and ensuring the stability and activity of erythrocytes.This chapter focuses on a series of pathophysiological changes that occur after hemolysis in G6PD deficiency patients, In order to elucidate the pathogenesis of Vicia faba induced hemolysis Vicia faba incidence and distribution: Vicia faba occurred mainly in the Mediterranean coast and the Middle East, such as the Balkans, Greece, Cyprus, Turkey, Lebanon, Israel, Egypt, North Africa , Iran, Iraq, etc. In the Far East, the disease mainly occurs in Taiwan Province and southern China
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