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[纯合子镰状细胞病中葡萄糖-6-磷酸脱氢酶缺乏症的患病率及发病率]

[Prevalence and morbidity of G6PD deficiency in sickle cell disease in the homozygote].

作者信息

Diop S, Sene A, Cisse M, Toure A O, Sow O, Thiam D, Diakhate L

机构信息

Travail du service d'Hématologie CHU Dakar.

出版信息

Dakar Med. 2005;50(2):56-60.

Abstract

Sickle cell disease and G6PD deficiency have similar prevalence of 8 to 10% in Senegalese population. Our objectives were to determine the prevalence of G6PD deficiency in Hb S carriers and normal subjects, and to assess the interaction of G6PD deficiency on clinical severity of sickle cell disease. G6PD activity was measured in 319 sickle cell patients and in 318 subjects without HbS. Clinical severity was compared in male homozygous sickle cell patients (11 with G6PD deficiency and 19 without deficit). In homozygous sickle cell patients, the G6PD status was assessed after correction of reticulocyte count following the micro-centrifugation method of Herz. We found that prevalence of G6PD deficiency was higher in sickle cell disease patients (21.6 %) than in normal subjects (12.3 %) (p = 0.001). No difference was found in the two groups of male sickle cell disease patients concerning number of vaso-occlusive crisis, number of transfusion, frequency of infectious episodes, number of chronic complications, disturbances on patient's activity and total index severity.

摘要

镰状细胞病和葡萄糖-6-磷酸脱氢酶(G6PD)缺乏症在塞内加尔人群中的患病率相似,均为8%至10%。我们的目标是确定Hb S携带者和正常受试者中G6PD缺乏症的患病率,并评估G6PD缺乏症对镰状细胞病临床严重程度的影响。对319例镰状细胞病患者和318例无Hb S的受试者进行了G6PD活性检测。比较了男性纯合镰状细胞病患者(11例G6PD缺乏和19例无缺乏)的临床严重程度。在纯合镰状细胞病患者中,采用赫兹微量离心法校正网织红细胞计数后评估G6PD状态。我们发现,镰状细胞病患者中G6PD缺乏症的患病率(21.6%)高于正常受试者(12.3%)(p = 0.001)。在两组男性镰状细胞病患者中,关于血管闭塞性危象的次数、输血次数、感染发作频率、慢性并发症的数量、对患者活动的干扰以及总指数严重程度方面未发现差异。

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