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红细胞6-磷酸葡萄糖脱氢酶(G-6-PD)缺乏是一种遗传性缺陷的红细胞酶病,是新生婴儿遗传性溶血性贫血的最常见原因。已有许多调查表明,广东为红细胞G-6-PD缺乏的高发地区,其人群G-6-PD缺乏的发生率为8.65％。但国内过去均采用高铁血红蛋白(MetHb)还原试验难予正确反映红细胞G-6-PD真正活性。已有发现某些G-6-PD活性正常的其他疾病患者,其MetHb还原率减低显示假阳性。因此推测以往检出的G-6-PD缺乏者中,有否包括了假阳性。为了弄清广东地区红细胞G-6-PD缺乏的真实发生率及基因频率,我们采用了Chapman-Dern紫外分光光度法直接测定G-6-PD活性,以提供广东地区红细胞
The lack of erythrocyte glucose-6-phosphate dehydrogenase (G-6-PD) is a hereditary deficient erythrocytosis that is the most common cause of hereditary hemolytic anemia in newborn infants. There have been many surveys that show that Guangdong has a high incidence of G-6-PD deficiency in the area where the incidence of G-6-PD deficiency is 8.65%. However, the past use of methemoglobin (MetHb) reduction test is difficult to correctly reflect the true activity of erythrocyte G-6-PD. It has been found that some patients with other diseases with normal G-6-PD activity have a reduced false-positive MetHb reduction rate. Therefore, it is speculated that the false positives were not included in the G-6-PD deficiency detected in the past. To clarify the true incidence and frequency of G-6-PD deficiency in RBCs in Guangdong, we used Chapman-Dern UV spectrophotometry to directly measure G-6-PD activity to provide Guangdong red blood cells