新生儿G-6-PD缺陷病并急性溶血(附45例临床分析)

目的：探讨新生儿葡萄糖—6—磷酸脱氢酶(G—6—PD)缺陷病并急性溶血的临床诊治特点及预后。方法：回顾性分析45例新生儿G—6—PD缺陷病并急性溶血的诱因、诊治情况，并对早产儿组与非早产儿组其胆红素脑病发生率及死亡率进行比较。结果：诱因以感染占首位(56％)，胆红素脑病发生率高(9％)，早产儿组与非早产儿组对照其胆红素脑病发生率(P＜0．05)及死亡率(P＜0．01)均有显著差异。结论：①感染是G—6—PD缺陷病并急性溶血的首位诱因。②胆红素脑病可在血清胆红素值较低(最低为302umol／L)的水平上发生，早产儿发生率高。③除常规治疗外．可换血，但要严格掌握指征。④C—6—PD缺陷病合并感染、出血、酸中毒等因素，尤其是早产儿预后极差．更应予及早重视(如防治感染、止血、纠酸等)。

Newborn G-6-PD deficiency complicating acute hemolysis

Objective:To investigate the clinical characteristic and prognosis of newborn G-6-PD deficiency complicating acute hemolysis. Methods:Clinical and prognostic data were analyzed in 45 newborns with G-6-PD deficiency complicating acute hemolysis, compared the incidence of bilirubin encephalopathy and mortality between premature group and non-premature group.Results:Infection is the major cause of all(56%).The incidence of bilirubin encephalopathy is high(9%).The incidence of bilirubin encephalopathy(P<0.05)and mortality(P<0.01) had significant differences between premature group and non-premature group.Conclusion:Infection is the major cause of newborn G-6-PD deficiency complicating acute hemolysis.Bilirubin encephalopathy may occurs in lower level of plasma bilirubin(the lowest value is 302umol/L).The incidence of premature bilirubin encephalopathy is high.Blood transfusion may put to use strictly besides routine treatment.Prognosis is worse in newborn G-6-PD deficiency complicating infection,hemorrhage,acidosis,especially premature.