新生儿溶血病和葡萄糖-6-磷酸脱氢酶缺乏症致重度高胆红素血症的临床特点分析  

Clinical analysis of severe hyperbilirubinemia caused by hemolytic disease of the newborn and G6PD deficiency

作　　者：郭琼[1] 张蜜[1] 胡月圆[1] 林艳萍 李贵南[1] Guo Qiong;Zhang Mi;Hu Yueyuan;Lin Yanping;Li Guinan(Department of Neonatology,Hunan Children's Hospital,Changsha 410007,China)

机构地区：[1]湖南省儿童医院新生儿科,长沙410007

出　　处：《中华新生儿科杂志（中英文）》2023年第2期65-69,共5页Chinese Journal of Neonatology

基　　金：湖南省卫建委科研项目(20200797)。

摘　　要：目的探讨新生儿溶血病(hemolytic disease of newborn,HDN)和葡萄糖-6-磷酸脱氢酶(glucose-6-phosphate dehydrogenase,G6PD)缺乏症所致重度高胆红素血症的临床特征及差异。方法选取2014年1月至2021年12月湖南省儿童医院新生儿科收治的胎龄≥35周、日龄≤28 d确诊为HDN或G6PD缺乏症的重度高胆红素血症新生儿进行回顾性分析,根据黄疸病因分为HDN组和G6PD缺乏症组,应用SPSS 26.0统计软件比较两组患儿一般情况、临床表现、实验室检查结果、治疗及预后等临床资料。结果HDN组532例,G6PD缺乏症组413例。HDN组黄疸峰值日龄早于G6PD缺乏症组[3(2,5)d比6(4,8)d],血清总胆红素峰值和贫血发生率低于G6PD缺乏症组[379.5(345.6,426.7)μmol/L比486.4(413.5,577.4)μmol/L,37.4%(199/532)比55.0%(227/413)],贫血伴网织红细胞比值增高比例高于G6PD缺乏症组[66.3%(132/199)比5.7%(13/227)],换血治疗率、重复(≥2次)换血治疗率、急性胆红素脑病发生率、放弃治疗死亡率均低于G6PD缺乏症组,差异有统计学意义(P<0.05)。结论HDN所致新生儿重度高胆红素血症的黄疸出现早;G6PD缺乏症所致新生儿重度高胆红素血症的贫血发生率高、黄疸程度重、急性胆红素脑病发生率更高,但多数不伴网织红细胞比值增高。Objective To study the clinical characteristics and differences of severe hyperbilirubinemia caused by hemolytic disease of the newborn(HDN)and glucose-6-phosphate dehydrogenase(G6PD)deficiency.Methods From January 2014 to December 2021,newborns(gestational age≥35 weeks and postnatal age≤28 d)admitted to the Department of Neonatology of Hunan Children's Hospital with severe hyperbilirubinemia caused by HDN or G6PD deficiency were retrospectively analyzed.According to the etiology of hyperbilirubinemia,they were assigned into HDN group and G6PD deficiency group.The general conditions,clinical manifestations,laboratory results,treatment and prognosis of the two groups were compared using SPSS 26.0 software.Results A total of 532 cases were in the HDN group and 413 cases in the G6PD deficiency group.The HDN group reached peak hyperbilirubinemia earlier than the G6PD deficiency group[3(2,5)d vs.6(4,8)d,P<0.05].The HDN group had lower peak value of total serum bilirubin[379.5(345.6,426.7)μmol/L vs.486.4(413.5,577.4)μmol/L]and lower incidence of anemia[37.4%(199/532)vs.55.0%(227/413)]than the G6PD deficiency group.The incidence of anemia with elevated reticulocyte percent(Ret%)in the HDN group was higher than the G6PD deficiency group[66.3%(132/199)vs.5.7%(13/227),P<0.05].Compared with the G6PD deficiency group,the incidences of exchange transfusion and repeated(≥2 times)exchange transfusion,acute bilirubin encephalopathy(ABE)and the mortality rate after withdrawal of treatment in the HDN group were significantly lower(P<0.05).Conclusions Neonatal severe hyperbilirubinemia caused by HDN has early onset.G6PD deficiency caused hyperbilirubinemia has higher incidences of anemia,more severe jaundice and ABE,without increased Ret%.

关 键 词：新生儿溶血病 葡萄糖-6-磷酸脱氢酶缺乏症 新生儿重度高胆红素血症 

分 类 号：R722.18[医药卫生—儿科]