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埃及黄疸新生儿中葡萄糖-6-磷酸脱氢酶缺乏症的患病率。

Prevalence of glucose-6-phosphate dehydrogenase deficiency in jaundiced Egyptian neonates.

作者信息

M Abo El Fotoh Wafaa Moustafa, Rizk Mohammed Soliman

机构信息

a Department of Pediatrics , Menoufia University , Shebin El-Kom , Egypt and.

b Department of Biochemistry , Faculty of Medicine, Menoufia University , Shebin El-Kom , Egypt.

出版信息

J Matern Fetal Neonatal Med. 2016 Dec;29(23):3834-7. doi: 10.3109/14767058.2016.1148133. Epub 2016 Mar 3.

DOI:10.3109/14767058.2016.1148133
PMID:26866975
Abstract

BACKGROUND

The enzyme, Glucose-6-phosphate dehydrogenase (G6PD), deficiency leads to impaired production of reduced glutathione and predisposes the red cells to be damaged by oxidative metabolites, causing hemolysis. Deficient neonates may manifest clinically as hyperbilirubinemia or even kernicterus.

OBJECTIVE

This study was carried out to detect erythrocyte G6PD deficiency in neonatal hyperbilirubinemia.

METHODS AND DESIGN

To determine the frequency and effect of G6PD deficiency, this study was conducted on 202 neonates with indirect hyperbilirubinemia. All term and preterm babies up to 13 day of age admitted with clinically evident jaundice were taken for the study. G6PD activity is measured by the UV-Kinetic Method using cellular enzyme determination reagents by spectrophotometry according to manufacturer's instructions.

RESULTS

A total of 202 babies were enrolled in this study. Male babies outnumbered the female (71.3% versus 28.7%). Mean age of the study newborns was 3.75 ± 2.5 days. Eighteen neonates (8.9%) had G6PD deficiency, all are males. One case had combined G6PD deficiency and RH incompatibility. Mean serum total bilirubin was 17.2 ± 4.4 in G6PD deficient cases. There was significant positive correlation between the time of appearance of jaundice in days and G6PD levels in G6PD deficient cases.

CONCLUSION

Neonatal hyperbilirubinemia is associated with various clinical comorbidities. G6PD deficiency is found to one important cause of neonatal jaundice developing on day 2 onwards.

摘要

背景

葡萄糖-6-磷酸脱氢酶(G6PD)缺乏会导致还原型谷胱甘肽生成受损,使红细胞易受氧化代谢产物破坏,从而引发溶血。G6PD缺乏的新生儿临床上可能表现为高胆红素血症甚至核黄疸。

目的

本研究旨在检测新生儿高胆红素血症患者的红细胞G6PD缺乏情况。

方法与设计

为确定G6PD缺乏的发生率及影响,本研究对202例间接高胆红素血症新生儿进行了调查。所有足月和早产且年龄在13日龄以内、临床上有明显黄疸的婴儿均纳入研究。根据制造商说明,采用紫外动力学法,使用细胞酶测定试剂通过分光光度法测量G6PD活性。

结果

本研究共纳入202例婴儿。男婴数量多于女婴(71.3%对28.7%)。研究新生儿的平均年龄为3.75±2.5天。18例新生儿(8.9%)存在G6PD缺乏,均为男性。1例合并G6PD缺乏和RH血型不合。G6PD缺乏病例的平均血清总胆红素为17.2±4.4。G6PD缺乏病例中黄疸出现天数与G6PD水平之间存在显著正相关。

结论

新生儿高胆红素血症与多种临床合并症有关。G6PD缺乏是出生后第2天起发生新生儿黄疸的一个重要原因。

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