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镰状细胞和β地中海贫血基因与塞拉利昂儿童及非妊娠妇女血红蛋白浓度和贫血之间的关联:塞拉利昂2013年全国微量营养素调查数据的辅助分析

Association between sickle cell and β-thalassemia genes and hemoglobin concentration and anemia in children and non-pregnant women in Sierra Leone: ancillary analysis of data from Sierra Leone's 2013 National Micronutrient Survey.

作者信息

Wirth James P, Ansumana Rashid, Woodruff Bradley A, Koroma Aminata S, Hodges Mary H

机构信息

GroundWork, Fläsch, Switzerland.

Mercy Hospital Research Laboratory, Kulanda Town, Bo, Sierra Leone.

出版信息

BMC Res Notes. 2018 Jan 17;11(1):43. doi: 10.1186/s13104-018-3143-x.

Abstract

OBJECTIVE

By measuring the associations between the presence of sickle cell and β-thalassemia genes, we assessed the extent to which these hemoglobinopathies contribute to the high prevalence of anemia observed in preschool-aged children and women of reproductive age in Sierra Leone.

RESULTS

The prevalence of anemia was statistically significantly higher in children with homozygous sickle cell genes (HbSS) than in children with normal hemoglobin genes (HbAA or HbAC), but there was no difference in anemia prevalence in those with heterozygous sickle cell trait (HbAS or HbSC) compared with those with normal hemoglobin genes. In women, there was no difference in anemia prevalence by sickle cell status. In both children and women, there was no difference in the anemia prevalence for individuals with or without the β-thalassemia gene. For both sickle cell and β-thalassemia, there was no significant difference in hemoglobin concentrations by sickle cell or β-thalassemia status. Anemia prevalence was higher in children and women with homozygous sickle cell (HbSS). However, as the prevalence of HbSS children (5.4%) and women (1.6%) was quite small, it is unlikely that these hemoglobinopathies substantially contributed to the high anemia prevalence found in the 2013 national micronutrient survey.

摘要

目的

通过测量镰状细胞基因和β地中海贫血基因的存在之间的关联,我们评估了这些血红蛋白病在多大程度上导致了在塞拉利昂学龄前儿童和育龄妇女中观察到的贫血高患病率。

结果

纯合镰状细胞基因(HbSS)儿童的贫血患病率在统计学上显著高于正常血红蛋白基因(HbAA或HbAC)儿童,但杂合镰状细胞性状(HbAS或HbSC)儿童与正常血红蛋白基因儿童相比,贫血患病率没有差异。在女性中,贫血患病率按镰状细胞状态没有差异。在儿童和女性中,有无β地中海贫血基因的个体贫血患病率没有差异。对于镰状细胞病和β地中海贫血,血红蛋白浓度按镰状细胞或β地中海贫血状态没有显著差异。纯合镰状细胞(HbSS)的儿童和女性贫血患病率较高。然而,由于HbSS儿童(5.4%)和女性(1.6%)的患病率相当低,这些血红蛋白病不太可能对2013年全国微量营养素调查中发现的高贫血患病率有实质性贡献。

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Sickle cell anaemia and malaria.镰状细胞贫血与疟疾。
Mediterr J Hematol Infect Dis. 2012;4(1):e2012065. doi: 10.4084/MJHID.2012.065. Epub 2012 Oct 3.
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Beta-thalassemia.β-地中海贫血。
Genet Med. 2010 Feb;12(2):61-76. doi: 10.1097/GIM.0b013e3181cd68ed.
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Alpha and beta thalassemia.α和β地中海贫血
Am Fam Physician. 2009 Aug 15;80(4):339-44.

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