【摘要】 本文报告以高铁血红蛋白(Met-Hb)微量组织化学洗脱法筛选了上海地区1,000例新生儿脐血标本。阳性者并以分光光度法定量测定葡萄糖-6-磷酸脱氢酶(简称G-6-PD)活性为对照。1,000例新生儿红细胞G-6-PD缺陷发病率为0.2％(男、女各1例).该2例临床均无黄疸。家系调查发现,男性患儿之兄亦为G-6-PD缺陷者,目前有核黄疸后遗症。我室亦曾检查87例新生儿高胆红素血症,其中2例为G-6-PD缺陷引起.因此,我们认为上海地区新生儿高胆红素血症病人中,G-6-PD缺陷并非重要原因.更多还原

【Abstract】 1,000 neonates, male 538, female 462, in Shanghai were screened for erythrocyte glucose-6-phosphate dehydrogenase (G-6-PD) by the cytochemical method. The results showed that two cases, 1 male and 1 female, were found to be erythrocyte G-6-PD deficient. The ghost cells in normal newborns range from 0-1%. The incidence of erythrocyte G-6-PD deficiency in 1,000 neonates was 0.19% in male and 0.22% in female (mean 0.2%). No jaundice was present in both cases. The family of the male G-6-PD deficient baby was studied. His mother was a heterozygote and his brother was also a G-6-PD deficiency, but his brother presented hyperbilirubinemia when he was 6 days old (total serum bilirubin 27 mg% with 1 minute bilirubin 0.2mg%). A diagnosis of septicemia, hyperbilirubinemia, kernicterus and omphalitis was made, since then he suffered from sequalae of kernicterus. The results of this investigation indicated that the incidence of the erythrocyte G-6-PD deficiency in the population of Shanghai was low. In addition,87 cases of neonatal hyperbilirubinemia were investigated for G-6-PD deficiency and only two were found to be G-6-PD deficient. This also showed that the G-6-PD deficiency was not an important cause of neonatal hyperbilirubinemia in Shanghai.更多还原