Bottini E
J Med Genet. 1973 Jun;10(2):154-7. doi: 10.1136/jmg.10.2.154.
Haemolytic favism is a severe, acute anaemia which occurs in glucose-6-phosphate dehydrogenase deficient individuals, usually following the ingestion of seeds. Current interest is focused on the nature of the active substances of and on the causes of the varying susceptibility among G6PD-deficient individuals to episodes of severe haemolysis. The results of experiments favour the hypothesis that contains several active substances which may act in a synergistic way. Red cell acid phosphatase and thalassaemia genes appear to play a remarkable role in conditioning the susceptibility to severe haemolysis in G6PD-deficient subjects. In addition to erythrocyte enzymes and to enzymes which intervene in the absorption and metabolism of the active substances of another field for future investigations may be that of plasma factors which influence the stability of reduced glutathione in the red cells.
蚕豆病是一种严重的急性贫血症,发生于葡萄糖-6-磷酸脱氢酶缺乏的个体,通常在摄入蚕豆种子后发作。目前的研究兴趣集中在蚕豆的活性物质的性质以及葡萄糖-6-磷酸脱氢酶缺乏个体对严重溶血发作易感性不同的原因。实验结果支持这样的假说,即蚕豆含有几种可能以协同方式起作用的活性物质。红细胞酸性磷酸酶和地中海贫血基因在调节葡萄糖-6-磷酸脱氢酶缺乏受试者对严重溶血的易感性方面似乎起着显著作用。除了红细胞酶以及参与蚕豆活性物质吸收和代谢的酶之外,另一个未来研究的领域可能是影响红细胞中还原型谷胱甘肽稳定性的血浆因子。