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镰状细胞病中葡萄糖-6-磷酸脱氢酶缺乏症的发生率。沙特阿拉伯的一项研究。

Frequency of glucose-6-phosphate dehydrogenase deficiency in sickle-cell disease. A study in Saudi Arabia.

作者信息

Warsy A S

出版信息

Hum Hered. 1985;35(3):143-7. doi: 10.1159/000153534.

DOI:10.1159/000153534
PMID:3997159
Abstract

The frequency of glucose-6-phosphate dehydrogenase (G-6-PD) deficiency in 50 Hb S homozygotes (SS) and 98 Hb S heterozygotes (AS) was determined and compared with the frequency obtained in individuals with normal haemoglobin (AA). The observed number of SS patients with G-6-PD deficiency was significantly greater than the expected value (p less than 0.05). The frequency of G-6-PD deficiency in AA, AS and SS was found to be 0.172, 0.214 and 0.420, respectively. A statistically significant increase of G-6-PD deficiency was apparent in the Saudi sicklers. The possibility that G-6-PD deficiency and Hb S gene interact, influencing the survival of the carriers of these genetic abnormalities, is discussed.

摘要

测定了50名血红蛋白S纯合子(SS)和98名血红蛋白S杂合子(AS)中葡萄糖-6-磷酸脱氢酶(G-6-PD)缺乏症的发生率,并与正常血红蛋白个体(AA)的发生率进行了比较。观察到的G-6-PD缺乏症SS患者数量显著高于预期值(p小于0.05)。发现AA、AS和SS中G-6-PD缺乏症的发生率分别为0.172、0.214和0.420。沙特镰状细胞贫血患者中G-6-PD缺乏症有统计学意义的增加。讨论了G-6-PD缺乏症与血红蛋白S基因相互作用影响这些遗传异常携带者生存的可能性。

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