Lie-Injo L E, Virik H K, Lim P W, Lie A K, Ganesan J
Acta Haematol. 1977;58(3):152-60. doi: 10.1159/000207822.
A study was carried out of 332 babies suffering from severe neonatal jaundice who were admitted to the General Hospital, Kuala Lumpar, Malaysia. Of the 332 neonates, 51 were premature and 281 were full-term babies, 178 (110 Chinese, 58 Malay, 9 Indian and 1 European-Pakistani) had bilirubin levels of 20 mg% or higher, requiring exchange blood transfusion. Of the Chinese neonates, 23 (20.9%) had G6PD deficiency, 9 (8.2%) had Hb Bart's and 2 (1.8%) had an abnormal haemoglobin, one Hb Q and one fetal variant. Among the Malay infants, 10 (17.2%) had G6PD deficiency, 7 (12.1%) had Hb Bart's and 10 (17.2%) had abnormal haemoglobins (four had Hb E trait, one had Hb K and Bart's in addition to Hb E, three had Hb CoSp with Hb Bart's, one had Hb Q and one Hb Tak). One of the nine Indian neonates had G6PD deficiency and one had Hb S trait. The one European-Pakistani baby was a carrier of Hb D Punjab. In addition to G6PD deficiency, abnormal haemoglobins seem to have contributed to the high incidence of severe neonatal jaundice in Malaysia. The mean activities of GP, GR and GR after stimulation with FAD were higher, while the mean activity of PK and mean level of reduced glutathione were lower than in normal cord bloods. The percent increase of GR after FAD stimulation was significantly lower; fewer in this group had increases above 20% than in normal cord blood. The possible significance of the findings is discussed.
对马来西亚吉隆坡总医院收治的332例患有严重新生儿黄疸的婴儿进行了一项研究。在这332例新生儿中,51例为早产儿,281例为足月儿,178例(110例华裔、58例马来裔、9例印度裔和1例欧洲 - 巴基斯坦裔)胆红素水平达到或高于20mg%,需要进行换血治疗。在华裔新生儿中,23例(20.9%)患有葡萄糖 - 6 - 磷酸脱氢酶(G6PD)缺乏症,9例(8.2%)患有血红蛋白Bart's,2例(1.8%)有异常血红蛋白,1例血红蛋白Q和1例胎儿变异型。在马来裔婴儿中,10例(17.2%)患有G6PD缺乏症,7例(12.1%)患有血红蛋白Bart's,10例(17.2%)有异常血红蛋白(4例有血红蛋白E性状,1例除血红蛋白E外还有血红蛋白K和Bart's,3例有血红蛋白CoSp和血红蛋白Bart's,1例有血红蛋白Q,1例有血红蛋白Tak)。9例印度裔新生儿中有1例患有G6PD缺乏症,1例有血红蛋白S性状。1例欧洲 - 巴基斯坦裔婴儿是血红蛋白D旁遮普的携带者。除了G6PD缺乏症外,异常血红蛋白似乎也导致了马来西亚严重新生儿黄疸的高发病率。与正常脐带血相比,用黄素腺嘌呤二核苷酸(FAD)刺激后葡萄糖磷酸变位酶(GP)、谷胱甘肽还原酶(GR)和刺激后的GR平均活性较高,而丙酮酸激酶(PK)平均活性和还原型谷胱甘肽平均水平较低。FAD刺激后GR的百分比增加明显较低;该组中增加超过20%的人数比正常脐带血少。讨论了这些发现的可能意义。