CAJ | 학술논문

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。
목적：분석홍세포포도당-6-린산탈경매(홍세포G-6-PD매)결핍증적림상특점、치료과정급림상예후정황，제고림상적진치수평。방법선취45례홍세포G-6-PD매결핍적녀환인，관찰환인치료전후상관지표변화。결과환인치료후빈혈급황달증상명현감경，구진급피부안색호전，치료3 d후화치료5 d후기혈청총담홍소급혈홍단백명현우우치료전(P<0.05)。결론홍세포G-6-PD매결핍증다여식용잠두급기제품유관，림상상발병다이급성혈관내용혈위주요적림상표현，환혈치료시일충행지유효적치료방법。
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.