Ahmed S G, Ibrahim U A
Department of Haematology, University of Maiduguri Teaching Hospital, PMB 1414, Maiduguri, Nigeria.
Niger Postgrad Med J. 2002 Dec;9(4):181-5.
A total of 75 (45 males, 30 females) patients with sickle cell disease (SCD) at the university of Maiduguri Teaching Hospital were screened for G-6-PD deficiency and their steady state levels of haematocrit, reticulocyte count and irreversibly sickled cells (ISC) as well as the occurrence of vaso-occlusive crises were analysed with respect to their G-6-PD status during a 3 year period (1998-2000). None of the female patients was G-6-PD deficient, while (15.6%) male patients had the enzyme deficiency. The patients with G-6-PD deficiency had significantly lower mean level of haematocrit (0.221/l) with higher level of reticulocyte count (15%) as compared to their non-G-6-PD deficient counter parts who had mean values of 0.271/l and 9% for haematocrit count respectively. However, there was no significant difference in mean levels of ISC between G-6-PD deficient (6%) and non-G-6-PD deficient (5%) patients with SCD. Patients with G-6-PD deficiency had significantly lower episodes of vaso-occlusive crises (2.1 episodes/patient). This data would suggest that co-inheritance of G-6-PD deficiency in SCD reduces frequency of crises and improves prognosis.
在迈杜古里大学教学医院，对75例镰状细胞病（SCD）患者（45例男性，30例女性）进行了葡萄糖-6-磷酸脱氢酶（G-6-PD）缺乏症筛查，并在3年期间（1998 - 2000年），根据他们的G-6-PD状态，分析了他们的血细胞比容、网织红细胞计数和不可逆镰状细胞（ISC）的稳态水平以及血管闭塞性危机的发生情况。女性患者均无G-6-PD缺乏，而男性患者中有（15.6%）存在该酶缺乏。与非G-6-PD缺乏的对应患者相比，G-6-PD缺乏的患者血细胞比容平均水平显著更低（0.221/l），网织红细胞计数水平更高（15%），非G-6-PD缺乏的对应患者血细胞比容计数平均值分别为0.271/l和9%。然而，SCD患者中G-6-PD缺乏者（6%）和非G-6-PD缺乏者（5%）的ISC平均水平无显著差异。G-6-PD缺乏的患者血管闭塞性危机发作次数显著更低（2.1次/患者）。这些数据表明，SCD中G-6-PD缺乏的共同遗传可降低危机发生频率并改善预后。
