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喀麦隆镰状细胞性状与葡萄糖-6-磷酸脱氢酶缺乏症的相互作用。

Interaction of sickle cell trait and glucose-6-phosphate dehydrogenase deficiency in Cameroon.

作者信息

Bernstein S C, Bowman J E, Noche L K

出版信息

Hum Hered. 1980;30(1):7-11. doi: 10.1159/000153080.

Abstract

The prevalence of glucose-6-phosphate dehydrogenase (G6PD) deficiency and sickle cell trait was determined in 371 Cameroonian males and 668 male blood donors in Chicago. The number of males with both sickle cell trait and G6PD deficiency was significantly greater than expected (p less than 0.05) in Cameroon. The number of males with both sickle cell trait and G6PD deficiency in the Chicago population also exceeded the exptected number, although this was not statistically significant (p greater than 0.30). A young red cell population associated with the sickle cell gene leading to elevated G6PD levels in G6PD-deficient males suggests that sickle hemoglobin may exert a beneficial effect on G6PD deficiency, rather than the opposite, as had previously been proposed. These red cells may be better able to deal with oxidative stress, which can precipitate severe hemolytic disease in G6PD deficiency.

摘要

在喀麦隆的371名男性以及芝加哥的668名男性献血者中,对葡萄糖-6-磷酸脱氢酶(G6PD)缺乏症和镰状细胞性状的患病率进行了测定。在喀麦隆,同时患有镰状细胞性状和G6PD缺乏症的男性数量显著高于预期(p小于0.05)。芝加哥人群中同时患有镰状细胞性状和G6PD缺乏症的男性数量也超过了预期数量,尽管这在统计学上并不显著(p大于0.30)。与镰状细胞基因相关的年轻红细胞群体导致G6PD缺乏男性的G6PD水平升高,这表明镰状血红蛋白可能对G6PD缺乏症产生有益影响,而不是像之前所认为的那样产生相反影响。这些红细胞可能更有能力应对氧化应激,氧化应激会在G6PD缺乏症中引发严重溶血性疾病。

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