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也门塔伊兹地区镰状细胞病患者中的地中海贫血症和葡萄糖-6-磷酸脱氢酶缺乏症。

Thalassaemia and glucose-6-phosphate dehydrogenase deficiency in sickle-cell disorder patients in Taiz, Yemen.

机构信息

Department of Haematology, Faculty of Medicine and Health Sciences, Sana'a University, Sana'a, Yemen.

出版信息

East Mediterr Health J. 2011 May;17(5):404-8.

Abstract

A pilot study was conducted to determine the prevalence and haematological characteristics of the interaction between thalassaemia or/and glucose-6-phosphate dehydrogenase (G6PD) deficiency in patients with sickle-cell disorder (SCD) in Taiz city, Yemen, where the prevalence of sickle-cell trait (HbAS) is 8.2%. Blood samples were collected from 31 SCD patients. Complete blood count and haemoglobin electrophoresis, G6PD activity and serum ferritin were determined. Thalassaemia was found in 6 patients (19.4%) and G6PD deficiency (6 mild and 1 severe) was detected in 7 patients (22.6%). The frequency ofthalassaemia and/or G6PD deficiency with SCD was high and this may have an effect on the severity of the clinical course of SCD in Taiz. The study should be repeated with DNA analysis to define the nature of the globin gene defect and to clarify its role in the severity of SCD

摘要

一项初步研究在也门塔伊兹市进行,旨在确定镰状细胞病(SCD)患者中海贫血症和/或葡萄糖-6-磷酸脱氢酶(G6PD)缺乏症相互作用的流行率和血液学特征。该地区镰状细胞特征(HbAS)的流行率为 8.2%。研究采集了 31 名 SCD 患者的血液样本。检测了全血细胞计数和血红蛋白电泳、G6PD 活性和血清铁蛋白。发现 6 名患者(19.4%)患有地中海贫血症,7 名患者(22.6%)患有 G6PD 缺乏症(6 例轻度,1 例重度)。SCD 中海贫血症和/或 G6PD 缺乏症的发生率较高,这可能会影响 SCD 在塔伊兹的临床病程严重程度。应通过 DNA 分析重复该研究,以确定珠蛋白基因缺陷的性质,并阐明其在 SCD 严重程度中的作用。

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