葡萄糖-6-磷酸脱氢酶缺乏与镰状细胞病合并症对红细胞指标有显著影响。

IF 2.2 Q3 HEMATOLOGY Anemia Pub Date : 2019-03-19 eCollection Date: 2019-01-01 DOI:10.1155/2019/3179173
Samuel Antwi-Baffour, Jonathan Kofi Adjei, Peter Owadee Forson, Stephen Akakpo, Ransford Kyeremeh, Mahmood Abdulai Seidu
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引用次数: 18

摘要

背景:葡萄糖-6-磷酸脱氢酶(G6PD)通过磷酸戊糖途径将葡萄糖-6-磷酸转化为6-磷酸葡萄糖酸盐,并保护红细胞免受氧化损伤。因此,它们的缺乏使红细胞容易溶血。另一方面,镰状细胞病(SCD)是一种遗传性血液疾病,其中氨基酸6的密码子中存在单个核苷酸替换,用缬氨酸取代谷氨酸。由于红细胞的形状,SCD患者容易发生溶血,如果他们缺乏G6PD,溶血可能会加剧。已报道的研究表明,SCD患者中G6PD缺乏症的患病率存在差异,因此需要进一步的工作。因此,本研究的目的是估计SCD患者中G-6-PD缺乏症的发生率,并确定其对红细胞参数的影响,以衡量贫血的发生率。方法:将120例临床诊断为HbSS和HbSC基因型的SCD患者纳入研究。通过静脉穿刺从每位患者采集约5ml血液,用于G6PD、全血细胞计数和血红蛋白(Hb)电泳检测。数据采用SPSS version 20和Graphpad prism进行分析。结果:G6PD缺乏症43例(35.83%),其中男性16例(13.33%),女性27例(22.50%),部分缺乏症17例(14.17%),完全缺乏症10例(8.33%)。G6PD缺乏症患者与G6PD正常活动者的Hb浓度分别有p=0.036和p=0.038的统计学差异。结论:本研究结果提示G6PD缺乏可能会加重SCD患者贫血的严重程度。因此,有必要对所有SCD患者进行G6PD缺乏症筛查,以确保他们的病情在治疗期间不会恶化。
本文章由计算机程序翻译,如有差异,请以英文原文为准。

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Comorbidity of Glucose-6-Phosphate Dehydrogenase Deficiency and Sickle Cell Disease Exert Significant Effect on RBC Indices.

Background: Glucose-6-phosphate dehydrogenase (G6PD) converts glucose-6-phosphate into 6-phosphogluconate in the pentose phosphate pathway and protects red blood cells (RBCs) from oxidative damage. Their deficiency therefore makes RBCs prone to haemolysis. Sickle cell disease (SCD) on the other hand is a hereditary blood disorder in which there is a single nucleotide substitution in the codon for amino acid 6 substituting glutamic acid with valine. SCD patients are prone to haemolysis due to the shape of their red blood cells and if they are deficient in G6PD, the haemolysis may escalate. Reported studies have indicated variations in the prevalence of G6PD deficiency in SCD patients and as such further work is required. The aim of this study was therefore to estimate the incidence of G-6-PD deficiency among SCD patients and to determine its impact on their RBC parameters as a measure of incidence of anaemia.

Methods: A total of 120 clinically diagnosed SCD patients of genotypes HbSS and HbSC were recruited into the study. About 5ml of blood was collected via venipuncture from each patient and used to run G6PD, full blood count, and haemoglobin (Hb) electrophoresis tests. The data were analyzed using SPSS version 20 and Graphpad prism.

Result: G6PD deficiency was detected in 43 (35.83%) of the participants made up of 16 (13.33%) males and 27 (22.50%) females of whom 17 (14.17%) had partial deficiency and 10 (8.33%) full deficiency. Statiscally significant differences p=0.036 and p=0.038 were established between the Hb concentration of the participants having a G6PD deficiency and those with normal G6PD activity for males and females, respectively.

Conclusion: From the results obtained, it implies that G6PD deficiency may increase the severity of anaemia in SCD patients. There is therefore the need to screen all SCD patients for G6PD deficiency to ensure that their condition is not exacerbated during treatment.

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来源期刊
Anemia
Anemia HEMATOLOGY-
CiteScore
4.80
自引率
3.40%
发文量
11
审稿时长
18 weeks
期刊介绍: Anemia is a peer-reviewed, Open Access journal that publishes original research articles, review articles, and clinical studies on all types of anemia. Articles focusing on patient care, health systems, epidemiology, and animal models will be considered, among other relevant topics. Affecting roughly one third of the world’s population, anemia is a major public health concern. The journal aims to facilitate the exchange of research addressing global health and mortality relating to anemia and associated diseases.
期刊最新文献
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