红细胞葡萄糖-6-磷酸脱氢酶缺乏症45例临床分析

目的 分析红细胞葡萄糖-6-磷酸脱氢酶（红细胞G-6-PD酶）缺乏症的临床特点、治疗过程及临床预后情况,提高临床的诊治水平。方法 选取45例红细胞G-6-PD酶缺乏的女患儿,观察患儿治疗前后相关指标变化。结果 患儿治疗后贫血及黄疸症状明显减轻,口唇及皮肤颜色好转,治疗3 d后和治疗5 d后其血清总胆红素及血红蛋白明显优于治疗前（P〈0.05）。结论 红细胞G-6-PD酶缺乏症多与食用蚕豆及其制品有关,临床上发病多以急性血管内溶血为主要的临床表现,换血治疗是一种行之有效的治疗方法。

Clinical analysis of erythrocyte glucose-6-phosphatase dehydrogenase enzyme deficiency disease

Objective To analyze clinical features, treatment and prognosis of erythrocyte glucose- 6-phosphatase dehydrogenase（G-6-PD）enzyme deficiency disease to improve level of clinical diagnosis and treatment. Methods Selected 45 cases of female children with erythrocyte G-6-PD enzyme deficiency in our hospital,related indicators before and after treatment were observed. Results After treatment, symptoms of anemia and jaundice in children were significantly reduced, lips and skin color improved after treatment, and after 5 d and 3 d treatment, its serum total bilirubin and hemoglobin index was better than before treatment（P〈0.05）. Conclusion Erythrocyte G-6-PD deficiency is ralated to edible beans and its products, acute intravascular hemolysis as its major clinical manifestations, blood transfusion therapy is an effective treatment.