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G-6-PD基因位于Xq28,在第Ⅷ因子及红绿色盲基因之间,全长20114个bp,由13个外显子和12个内含子组成,编码515个氨基酸。葡萄糖-6-磷酸脱氢酶(G-6-PD)缺乏症为X连锁不完全显性遗传病,溶血反应可由伯氨喹,阿司匹林等药物诱发,也可由蚕豆、金银花等诱发,是人类最常见的遗传性红细胞酶病。现将我院儿科门诊接治的因蚕豆诱发溶血的患儿情况报告如下。
The G-6-PD gene is located on chromosome Xq28 and consists of 20114 bp between the Ⅷ factor and the red-green blind gene. It consists of 13 exons and 12 introns and encodes 515 amino acids. Glucose-6-phosphate dehydrogenase (G-6-PD) deficiency is X-linked incomplete dominant genetic disease, hemolytic reaction may be induced by primaquine, aspirin and other drugs, but also by fava beans, honeysuckle and other induced, is the most human Common hereditary erythrocytic enzyme disease. Now in our hospital Pediatric outpatient treatment due to Vicia faba induced hemolytic children are as follows.