G-6-PD缺陷与新生儿高胆、婴幼儿贫血及婴儿肝炎综合征关系的调查

广西是我国Ｇ－６－ＰＤ缺陷高发区。Ｇ－６－ＰＤ缺陷发病的主要表现为急慢性溶血性贫血和由此而产生的高间胆红素血症及胆汁郁积。近十年我院门诊及病房４４０例患儿：２３７例新生儿高间胆包括核黄症后遗症患儿。Ｇ－６－ＰＤ缺陷检出率达６９．６％，其中显著缺陷４３．９％（１０４／２３７），中间值２５．７％（６１／２３７）；１０３例３个月以下的婴儿贫血原因待查患儿，Ｇ－６－ＰＤ缺陷检出率达５９．２２％（３６／１０３）；１００例婴儿肝炎综合征患儿，Ｇ－６－ＰＤ缺陷检出率达１８％（１８／１００）。Ｇ－６－ＰＤ缺陷男女经例为２．３７：１。６７例进行家系调查，２８例Ｇ－６－ＰＤ缺陷男婴，母亲检出率为６７．８％（１８／２８）；１４例Ｇ－６－ＰＤ缺陷女婴，父亲检出率４３％（６／１４），母亲检出率５０％（７／１４）。说明了在我区Ｇ－６－ＰＤ缺陷是引起新生儿高间胆以及核黄痊后遗症、婴幼儿贫血较常见而且重要原因之一。

A SURVEY ON THE RELATIONS BETWEEN G-6-PD DEFICIENCY AND NEONATAL INDIRECIED-HYPERBILIRUB INEMIA,ANEMIA AND HEPATITIS SYNDROME IN INFANTS

Abstract: Guangxi is one of the prevalent area of G-6-PD deficiency in China. In order to find outthe relations between G-6-PD deficiency and neonatal md indirected-hyperbilirubinemia, anemia and hepatitis syndrome in infants, the authors have.conducted a survey on 440 cases of outand in-patents of infants suffered from the above mentioned disorders in the period from 1983to 1993. Among the 237 cases of neonatal indirected-hyperbilirubinemia (including severalcases of sequence of kernicterus), the positive incidence rate in G-6-PD deficiency was 69.6%. 59. 2% and 18% among the 103 cases of infantile anemia and 100 of infantile hepatitissyndrome, respectively. The results of this survey suggested that G-6-PD deficiency is verycommon in Guangxi Zhuang Minority Region, and G-6-PD deficiency would be one of themost important etiology of neonatial indirected hyperbilirubinemia, anemia and hepatitis syndrome in infants.