Effects of glucose-6-phosphate dehydrogenase deficiency upon sickle cell anemia.
Effects of glucose-6-phosphate dehydrogenase deficiency upon sickle cell anemia.
复制标题
葡萄糖-6-磷酸脱氢酶缺乏症对镰状细胞性贫血的影响。
作者：
Martin;H.;Steinberg;M.;Stewart;West;Dianne;Gallagher;William;Mentzer
我们研究了葡萄糖-6-磷酸脱氢酶（G6 PD）缺乏和镰状细胞贫血（HbSS）之间的相互作用，以了解G6 PD缺乏是否影响HbSS的实验室和临床特征。共有801名2岁以上的男性患者在醋酸纤维素膜上进行了G6 PD电泳。然后对具有正常野生型（GdB）以外的电泳模式的所有样品进行G6 PD活性和己糖激酶活性的测定。临床数据的收集使用标准化方案。使用聚类分析，我们将10.4%的男性归类为G6 PD缺乏，而18.4%的GdA+酶功能正常。当年龄按十年分层时，G6 PD缺乏症的患病率没有显著变化，这表明G6 PD缺乏症和HbSS联合治疗的生存优势或劣势很小。与非G6 PD缺乏的患者相比，G6 PD缺乏的HbSS患者的血红蛋白浓度、平均红细胞体积、网织红细胞计数、胆红素或SGOT水平无显著差异。两组中疼痛发作、脓毒症或急性贫血发作的发生率相似。我们的结果与最近针对少量患者的研究一致，这些研究发现G6 PD缺乏对HbSS的影响很小。具体而言，我们没有发现G6 PD增加HbSS溶血严重程度或增加急性贫血发作或脓毒症发生率的证据。
We studied the interactions of the A- variety of glucose-6-phosphate dehydrogenase (G6PD) deficiency and sickle cell anemia (HbSS) to see if G6PD deficiency influenced laboratory and clinical features of HbSS. A total of 801 male patients over age 2 had G6PD electrophoresis on cellulose acetate membranes. Assays of both G6PD activity and hexokinase activity were then done on all samples that had an electrophoretic pattern other than the normal wild type (GdB). The collection of clinical data used a standardized protocol. Using cluster analyses we classified 10.4% males to be G6PD deficient, while 18.4% had the functionally normal GdA+ enzyme. The prevalence of G6PD deficiency did not change significantly when age was stratified by decade, suggesting little survival advantage or disadvantage of the combination of G6PD deficiency and HbSS. Compared to patients who were not G6PD deficient, there were no significant differences in the hemoglobin concentration, mean corpuscular volume, reticulocyte count, bilirubin, or SGOT level in patients with HbSS who had G6PD deficiency. The incidence of painful episodes, sepsis, or acute anemic episodes was similar in both groups. Our results are consistent with recent studies of smaller numbers of patients that have found little influence of G6PD deficiency upon HbSS. Specifically, we found no evidence that G6PD enhanced the severity of hemolysis or increased the incidence of acute anemic episodes or sepsis in HbSS.