李凌云

【摘 要】目的：了解海南陵水县育龄人群G6PD缺乏症的发病情况。方法：用G6PD/6GPD定量比值法，对5340名育龄群众进行了红细胞葡萄糖-6-磷酸脱氢酶缺乏症的临床检测。结果：在5340名育龄群众中，共计检出G6PD缺乏症431例，发生率为8.1%，其中男性患者249例，发生率为9.3%，女性患者182例，发生率为6.8%，男女性间差异显著（P<0.05），男女缺乏率之比为1.4：1。另有0.96%的人员为临界值，G6PD和6PGD光密度比值在0.95～1.05之间，但无法通过本方法准确判断，这部分人员需结合其他方法检测结果或临床诊断来判断。结论：海南陵水县是G6PD缺乏症的高发区，应在本地积极普及推广G6PD的育龄人群筛查。

【关键词】育龄人群；G6PD缺乏症；发生率

【Abstract】Objective：To investigate the incidence of G6PD deficiency in Lingshui County of Hainan province. Method：Using G6PD/6GPD quantitative ratio method， 5340 people of childbearing age were in the clinical detection of erythrocyte glucose-6-phosphate dehydrogenase deficiency.Results：There were 431 cases of G6PD deficiency， 8.1% cases of male patients， 9.3% cases of male patients， 182 cases of female patients， 6.8% cases of female patients， the incidence rate was 5340， the difference between men and women was significant （P < 0.05）. The ratio of male to female deficiency rate was 1.4：1. Another 0.96% of the staff was the critical value， and the optical density ratio of G6PD and 6PGD was between 0.95 and 1.05， but it could not be judged accurately by this method. This part of the staff should be judged by combining the test results of other methods or clinical diagnosis. Conclusion： Lingshui Hainan county is a high risk area of G6PD deficiency， and it should be popularized in the local area to promote the screening of G6PD.

【Key？Words】People of reproductive age；G6PD deficiency；Incidence

紅细胞葡萄糖-6-磷酸脱氢酶（G6PD）缺乏症，俗称蚕豆病，是一种伴性不完全显性红细胞酶缺乏病[1]，其发病原因是G6PD基因突变，导致该酶活性降低，红细胞膜不能抵抗氧化损伤而遭受破坏，引起的溶血性贫血。大多数患者在日常生活中没有任何症状，甚至很多人不知道自己有G6PD 缺乏症，但有时候会对某些食物、药物和化学物质敏感，从而出现一些症状，主要包括黄疸，皮肤苍白、深色尿液、背部或腹部疼痛以及贫血等。G6PD缺乏症的发病机制尚不明确，根据相关研究资料显示，男性往往多于女，且成年人发病低于小儿，其中3岁以上儿童占70%左右，每年发生于3月～5月间蚕豆成熟的季节。临床上可按酶的缺乏程度将此病分为两类：一类平时无溶血症状，但在某些诱因作用下发生溶血性反应；另一类在无诱因情况下出现慢性溶血。我国大多数病例属前一种。此病在临床上通常表现为先天性非球形红细胞性溶血、新生儿黄疸、蚕豆病、药物性溶血、感染诱发溶血等。G6PD缺乏的新生儿，常会发生溶血性黄疸，严重时可发生新生儿核黄疸，导致患儿死亡或智力低下，给患儿家庭带来不幸，对优生优育、提高我国人口素质极为不利。……