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亚兹德新生儿病理性高胆红素血症中的葡萄糖-6-磷酸脱氢酶(G6PD)酶缺乏症

G6PD Enzyme Deficiency in Neonatal Pathologic Hyperbilirubinemia in Yazd.

作者信息

Pahlavanzadeh M, Hekmatimoghaddam S, Teremahi Ardestani M, Ghafoorzadeh M, Aminorraaya Mm

机构信息

Doctor of Laboratory Sciences, School of Paramedicine, Shahid Sadoughi University of Medical Sciences and Health Services, Yazd, Iran.

Assistant Professor of Pathology, School of Paramedicine, Shahid Sadoughi University of Medical Sciences and Health Services, Yazd, Iran.

出版信息

Iran J Ped Hematol Oncol. 2013;3(2):69-72. Epub 2013 Apr 22.

Abstract

BACKGROUND

About 7.5% of the world population carries one or two deficient copy of glucose-6-phosphate dehydrogenase (G6PD) genes. According to WHO, its prevalence in Iran is 10 to 14.9%. This study aimed on determination of frequency of G6PD deficiency in neonates with jaundice who were hospitalized during 6 months (September 2008 to February 2009) in the city of Yazd, Iran.

MATERIALS AND METHODS

In this study, 105 icteric neonates in the hospitals of Yazd were evaluated. Data was collected from hospital records, and the G6PD activity was measured by photometric biochemical assay. Statistical analysis of data was performed by the SPSS-16 software, using Student's t-test and Pearson's chi-squared test.

RESULTS

Between all of studied neonates, 19 (18.1%) had G6PD deficiency, and consisted of 15 boys (29.4% of boys) and 4 girls (7.4% of girls). In 100% of cases, the jaundice began in the first week after birth. The average total serum bilirubin at hospitalization was 17.22 mg/dL. In 31.5% of the G6PD-defficient neonates, exchange transfusion became necessary, which is significantly more than the rate in G6PD-sufficient (4.6%) neonates (P-value<0.05).

CONCLUSION

In general, the frequency of G6PD deficiency in this study seems quite high. Regarding its severity and frequent need for exchange transfusion, we recommend that all of the icteric neonates should be evaluated for G6PD activity. Also, it is better to test for G6PD deficiency in all of the neonates, to detect its presence and to prevent its complications such as favism and oxidant drug-induced hemolysis, since the test has a low cost.

摘要

背景

世界上约7.5%的人口携带一个或两个葡萄糖-6-磷酸脱氢酶(G6PD)基因缺陷拷贝。据世界卫生组织称,其在伊朗的患病率为10%至14.9%。本研究旨在确定2008年9月至2009年2月这6个月期间在伊朗亚兹德市住院的黄疸新生儿中G6PD缺乏症的发生率。

材料与方法

本研究对亚兹德市医院的105例黄疸新生儿进行了评估。从医院记录中收集数据,并通过光度生化测定法测量G6PD活性。使用SPSS - 16软件对数据进行统计分析,采用学生t检验和Pearson卡方检验。

结果

在所有研究的新生儿中,19例(18.1%)患有G6PD缺乏症,其中包括15名男孩(占男孩的29.4%)和4名女孩(占女孩的7.4%)。100%的病例中,黄疸在出生后第一周开始出现。住院时血清总胆红素的平均值为17.22mg/dL。在31.5%的G6PD缺乏新生儿中,需要进行换血治疗,这一比例显著高于G6PD正常(4.6%)的新生儿(P值<0.05)。

结论

总体而言,本研究中G6PD缺乏症的发生率似乎相当高。鉴于其严重性以及频繁需要进行换血治疗,我们建议对所有黄疸新生儿进行G6PD活性评估。此外最好对所有新生儿进行G6PD缺乏症检测,以发现其存在并预防其并发症,如蚕豆病和氧化药物诱导的溶血,因为该检测成本较低。

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