文章摘要
唐发娟,陈 琳,张晓燕,肖东琼,李熙鸿.还原型谷胱甘肽治疗儿童葡萄糖-6-磷酸脱氢酶缺乏症中的临床效果研究[J].,2019,19(5):855-858
还原型谷胱甘肽治疗儿童葡萄糖-6-磷酸脱氢酶缺乏症中的临床效果研究
Clinical Study on the Efficacy of Reduced Glutathione in the Treatment of Children with Glucose-6-phosphate Dehydrogenase Deficiency
投稿时间:2018-09-18  修订日期:2018-10-13
DOI:10.13241/j.cnki.pmb.2019.05.013
中文关键词: 还原型谷胱甘肽  葡萄糖-6-磷酸脱氢酶缺乏症  急性溶血  治疗
英文关键词: Reduced glutathione  Glucose-6-phosphate dehydrogenase deficiency  Acute hemolysis  Therapy
基金项目:四川省科技厅计划项目(12C0909)
作者单位
唐发娟 四川大学华西第二医院/出生缺陷与相关妇儿疾病教育部重点实验室急诊科 四川 成都 610041 
陈 琳 四川大学华西第二医院/出生缺陷与相关妇儿疾病教育部重点实验室急诊科 四川 成都 610041 
张晓燕 四川大学华西第二医院/出生缺陷与相关妇儿疾病教育部重点实验室急诊科 四川 成都 610041 
肖东琼 四川大学华西第二医院/出生缺陷与相关妇儿疾病教育部重点实验室急诊科 四川 成都 610041 
李熙鸿 四川大学华西第二医院/出生缺陷与相关妇儿疾病教育部重点实验室急诊科 四川 成都 610041 
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中文摘要:
      摘要 目的:研究还原型谷胱甘肽治疗儿童葡萄糖-6-磷酸脱氢酶(G-6-P-D)缺乏症并发急性溶血的临床疗效,为临床治疗提供参考。方法:选取我院2015年6月-2017年6月因葡萄糖-6-磷酸脱氢酶(G-6-P-D)缺乏症并发急性溶血的患儿78例并将其随机分为两组,每组39例。对照组予以停用氧化类药物,卧床休息,水化、碱化尿液,贫血严重者输注去白红细胞治疗;观察组在对照组基础上加用还原型谷胱甘肽治疗。观察和比较两组患儿第1天、第2天、第3天小便恢复率以及平均恢复时间,血清总胆红素第3天、第5天恢复率、平均恢复时间及平均住院时间。结果:治疗后,观察组第1天、第2天、第3天小便恢复率分别为51.3%、92.3%、100%,对照组分别为25.6%、64.1%、89.7%,观察组第1天、第2天、第3天小便恢复率均显著高于对照组(P<0.05);观察组及对照组小便恢复正常平均时间分别为1.8±0.7天、2.6±0.9天,观察组明显短于对照组(P<0.05);观察组第3天、第5天血清总胆红素恢复率分别为71.8%、100%,对照组为46.2%、97.4%;观察组和对照组血清总胆红素恢复正常平均时间分别为3.6±0.9天、4.1±1.0天;平均住院时间分别为2.3±0.6天、2.8±0.6天;观察组小便及血清总胆红素平均恢复时间(P<0.05)、平均住院时间均显著短于对照组(P<0.05)。结论:在儿童葡萄糖-6-磷酸脱氢酶缺乏并发急性溶血中应用还原型谷胱甘肽可增强其治疗疗效,缩短治疗疗程。
英文摘要:
      ABSTRACT Objective: To investigate the therapeutic effect of reduced glutathione(GSH) on the children with Glucose-6-phosphate dehydrogenase deficiency(G6PD) complicated by acute hemolysis. Methods: 78 cases of children with G6PD deficiency complicated with acute hemolysis were recruited into this study over a period of two years (2015.7-2017.7). We randomly assigned the patients in a 1:1 ratio to receive routine treatment combined of reduced glutathione per day(GSH group) or routine treatment alone as the control. Rou- tine therapy included discontinued usage of oxidative drugs, bed rest, hydration, and alkalization of urine, and patients with severe anemia were given transfusion of leukocyte-depleted red blood cells. On the first day, second day, and the third day, we record the rate of patients who attain normal urine output. On the third day and fifth day, the rate of patients who attain normal serum total bilirubin(STB) level was recorded. In addition, the average time it takes for urine output and STB level returned to normal and the average duration of hospital stay was compared between the GSH group and the control. Results: In the GSH group, the urine recovery rates on the first day, the second day and the third day were 51.3%, 92.3% and 100% respectively, which were 25.6%, 64.1% and 89.7% respectively in the control group and were significantly lower than those of the GSH group (P<0.05). The average urine recovery time was 1.8±0.7 days and 2.6±0.9 days in the observation group and control group, which was significantly shorter in the observation group than that of the control group (P<0.05). The STB recovery rate of observation group was 71.8% and 100% on the third and fifth days respectively, which was 46.2% and 97.4% in the control croup and significantly lower than those of the GSH group. In the observation group, the average bilirubin re- covery time was 3.6±0.9 days, which was 4.1±1.0 days in the control group. The average duration of hospital stay was 2.3±0.6 days and 2.8±0.6 days respectively. The average recovery time of urine and serum total bilirubin and average hospitalization in the observa- tion group(P<0.05) were significantly shorter than those of the control group (P<0.05). Conclusion: Among children with G6PD compli- cated by acute hemolysis, reduced glutathione combined with routine therapy could shorten the treatment duration, and it is more effec- tive than the routine therapy alone.
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