2983例新生儿α地中海贫血的筛查及监测

Screening and monitoring of alpha-Thalassemia in 2983 newborns

目的:探讨在我国农村地区(县、乡)应用血红蛋白电泳技术测定新生儿脐血Bart含量以筛查、监测α地中海贫血的可行性。方法:采用国产SH-2040型全自动琼脂糖凝胶电泳系统对2 983例新生儿脐血进行血红蛋白电泳、测定Bart含量,并对α地中海贫血新生儿开展监测工作。结果:检测出α地中海贫血228例,其中Bart胎儿水肿综合征3例、血红蛋白H病7例、静止型(α地贫2)87例、标准型(α地贫1)131例;3例Bart胎儿水肿综合征死亡,对存活的225例全部建立档案,告知家长,发给监测卡和防治材料,进行防治宣教。结论:应用新生儿脐血血红蛋白电泳测定Bart含量筛查新生儿α地中海贫血,并进行监测,是我国农村地区(县、乡)防治α地中海贫血的可行途径之一。

Objective： To explore the feasibility of Hb Bart＇s content testing in neonatal umbilical cord blood measured by hemoglobin electrophoresis in screening and monitoring α-thalassemia in the rural areas（counties,townships） of P.R.China.Methods： Automatic agarose gel electrophoresis system（Domestic SH-2040） was used for hemoglobin electrophoresis to determine Hb Bart＇s content of 2 983 neonatal umbilical cord blood,and newborns with α-thalassemia were screened and monitored.Results： It was demonstrated that 228 cases with α-thalassemia were detected in 2 983 newborns,of which 3 cases with Bart hydrops fetalis syndrome,7 cases with hemoglobin H disease,87 cases with static type（α-thalassemia 2）,131 cases with standard（α-thalassemia 1）.All of 3 cases with Bart hydrops fetalis syndrome were in death.Medical files of the 225 survival cases were set up and informed to their parents.Moreover,the monitoring cards and control materials about α-thalassemia were established,and prevention and cure mission concerned with the disease were undertaken for these newborns.Conclusion：It is suggested that screening and monitoring newborns α-thalassemia by applying hemoglobin electrophoresis to detect Hb Bart＇s levels of neonatal cord blood is one useful way which favours controlling α-thalassemia in rural areas（county,township） in P.R.China.