伊朗南部新生儿筛查项目对降低 G6PD 缺乏症患者住院率的效果。

The efficacy of a neonatal screening programme in decreasing the hospitalization rate of patients with G6PD deficiency in southern Iran.

机构信息

Hematology Research Center, Shiraz University of Medical Sciences, Shiraz, Iran.

出版信息

J Med Screen. 2010;17(2):66-7. doi: 10.1258/jms.2010.009105.

DOI:10.1258/jms.2010.009105

PMID:20660433

Abstract

OBJECTIVE

To investigate whether a neonatal screening programme for G6PD deficiency has decreased hospitalization for acute haemolytic attack in the Fars province of southern Iran.

METHODS

A total of 850 patients registered with G6PD deficiency were included in the study. Variables including age, sex, time and cause of hospitalization, cause of haemolytic crisis, positive history of blood transfusion, G6PD enzyme deficiency, blood urea nitrogen (BUN) and creatinine were recorded based on a standard questionnaire. All patients were analysed for G6PD enzyme level based on a quantitative test.

RESULTS

Five hundred and fifty-three patients were hospitalized before the introduction of the neonatal screening programme (2001-2004) and 297 afterwards (2005-2008). Of those patients hospitalized after the introduction of the screening programme, 237 were wrongly classified as normal and 60 were recorded as having G6PD enzyme deficiency by the neonatal screening programme. The main causes of haemolytic crisis in G6PD-deficient patients were fava bean consumption (88.2%), underlying infection (10.9%) and drugs (0.8%).

CONCLUSION

Our study showed the effectiveness of the neonatal screening programme in decreasing the hospitalization rate.

摘要

目的

研究新生儿葡萄糖-6-磷酸脱氢酶（G6PD）缺乏症筛查项目是否降低了伊朗法尔斯省急性溶血性贫血发作的住院率。

方法

共纳入 850 例 G6PD 缺乏症患者。根据标准问卷记录变量，包括年龄、性别、住院时间和原因、溶血性危象的原因、输血阳性史、G6PD 酶缺乏、血尿素氮（BUN）和肌酐。所有患者均根据定量试验分析 G6PD 酶水平。

结果

553 例患者在新生儿筛查项目实施前（2001-2004 年）住院，297 例患者在新生儿筛查项目实施后（2005-2008 年）住院。在筛查项目实施后住院的患者中，237 例被错误分类为正常，60 例被新生儿筛查项目记录为 G6PD 酶缺乏。G6PD 缺乏症患者溶血性危象的主要原因是食用蚕豆（88.2%）、潜在感染（10.9%）和药物（0.8%）。