婴儿葡萄糖-6-磷酸脱氢酶缺陷病的特点与预防

A study of the characteristics and prevention of glucose-6-phosphate dehydrogenase (G-6-PD) deficiency in infants

目的 探讨婴儿红细胞葡萄糖 - 6 -磷酸脱氢酶 (G - 6 -PD)缺陷病的特点与预防措施。方法 回顾分析 1995～ 2 0 0 0年儿内科住院的 1周～ 2个月 (不含 2个月 )的婴儿 310 4例 ,其中G - 6 -PD缺陷病患儿 2 39例。结果 G - 6 -PD缺陷病占同期住院患儿的 7 70 % ,占间接高胆红素血症患儿的 17 5 9% ,病死率为 5 0 2 % ,与肺炎的3 5 7%相近 (χ2 =0 88,P >0 0 5 )。合并高胆红素血症的占 10 0 0 0 % ,合并感染性疾病的占 38 4 9%、继发胆红素脑病的占 13 81%。结论 婴儿中G - 6 -PD缺陷病很常见 ,是高胆红素血症和胆红素脑病的首要病因 ,病死率也很高 ,提议将其列入本地区儿童疾病综合管理的范围 ,制定并推广预防的常规措施。

Objective To explore the features and preventive measures of G-6-PD deficiency in infants.Methods From 1995～2000,3104 cases of infants,1 week～2 months (except for 2 months) old,were hospitalized in pediatrics.The case reports were analyzed retrospectively.Results 239 cases suffered from G-6-PD deficiency,which took 7.70% in that group of infants and 17.59% in hyper-bilirubinemia infants.The death rate of G-6-PD deficant infants was 5.02%,which was higher than 3.57% of the pneumonia(χ 2=0.88,P>0.05).The G-6-PD deficient infants with complicated hyper-bilirubinemia,infection and bilirubin encephalopathy accounted for 100.00%,38.49% and 13.81% respectively.Conclusion G-6-PD deficiency was the primary pathogenesis of hyper-bilirubinemia and bilirubin encephalopathy in infants.The morbidity and mortality of G-6-PD deficient infants were higher.We should put it into IMCI and to make and enlarge some routine measures to prevent that disease.