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撒丁岛婴儿的新生儿黄疸与葡萄糖-6-磷酸脱氢酶缺乏症的严重程度

Neonatal jaundice and severity of glucose-6-phosphate dehydrogenase deficiency in Sardinian babies.

作者信息

Meloni T, Cutillo S, Testa U, Luzzatto L

机构信息

Department of Paediatrics, University Hospital, Sassari, Italy.

出版信息

Early Hum Dev. 1987 Nov;15(6):317-22. doi: 10.1016/0378-3782(87)90026-0.

Abstract

We have investigated the association of neonatal jaundice (NNJ) and G6PD deficiency in consecutive births in a Northern Sardinian hospital. After excluding known causes for NNJ, and after correcting for the incidence of NNJ from unknown causes, we estimated that 20% of G6PD deficient male newborns develop NNJ resulting from their enzyme deficiency. By analyzing in detail 100 G6PD deficient babies we found no differences in birth weight or haemoglobin level between those without and those with NNJ, four of whom required exchange transfusion. We further showed by an accurate quantitative method that the residual G6PD enzyme activity was not significantly lower in G6PD deficient babies with NNJ compared to G6PD deficient babies without NNJ.

摘要

我们在撒丁岛北部一家医院对连续出生的新生儿进行了新生儿黄疸(NNJ)与葡萄糖-6-磷酸脱氢酶(G6PD)缺乏症之间关联的调查。在排除NNJ的已知病因,并校正未知病因导致的NNJ发病率后,我们估计20%的G6PD缺乏男性新生儿因酶缺乏而发生NNJ。通过对100名G6PD缺乏婴儿进行详细分析,我们发现患NNJ和未患NNJ的婴儿在出生体重或血红蛋白水平上没有差异,其中4名婴儿需要进行换血治疗。我们进一步通过精确的定量方法表明,与未患NNJ的G6PD缺乏婴儿相比,患NNJ的G6PD缺乏婴儿的残余G6PD酶活性并没有显著降低。

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