Glucose-6-phosphate dehydrogenase deficiency and homozygous sickle cell disease in congo

Glucose-6-phosphate dehydrogenase deficiency and homozygous sickle cell disease in congo
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DOI:
10.1159/000022801
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发表时间:
1998-07-01
期刊:
影响因子:
1.8
通讯作者:
Galactéros, F
Galactéros, F
中科院分区:
生物学4区
文献类型:
--
作者:
Bouanga, JC;Mouélé, R;Galactéros, F

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在布拉柴维尔(刚果)对188名HbAA HbSS患者(109名女性,79名男性)和210名对照(115名女性和95名男性)进行了G6PD基因型检测。DNA样品采用聚合酶链反应(PCR)进行分析。G6PD B、A+和A-等位基因频率分别为56.9%、20.8%和22.2%,对照组为56.3、21.2和22.5%。HbSS中G6PD基因型的患病率与对照组无显著差异(p < 0.05)。当患者按年龄分层时,G6PD缺乏症的患病率没有变化,这表明G6PD缺乏症与HbSS没有关联的优势。红细胞计数、平均红细胞体积和平均红细胞血红蛋白不受G6PD基因型的影响,而具有G6PD A-的HbSS的Hb水平较低。我们的研究表明,在刚果,G6PD缺乏对镰状病人没有任何生物学上的优势。
G6PD genotypes were determined in Brazzaville (Congo) on 188 HbSS patients (109 females, 79 males) and 210 controls (115 females and 95 males) with HbAA. DNA samples were analyzed by the polymerase chain reaction (PCR). The frequencies of G6PD B, A+ and A- alleles were 56.9, 20.8 and, 22.2% in the patients versus 56.3, 21.2 and, 22.5% in the controls, respectively. The prevalence of G6PD genotypes in HbSS did not differ (p > 0.05) from that found in the controls. Prevalence of G6PD deficiency did not change when patients were stratified by age, suggesting that there is no advantage of the association of G6PD deficiency with HbSS. Red blood cell count, mean corpuscular volume and mean corpuscular hemoglobin were not modified by the G6PD genotypes, while Hb level was lower in HbSS with G6PD A-. Our study suggests that in Congo, G6PD deficiency does not offer any biological advantage to sicklers.