广西地区不同地中海贫血类型G6PD酶活性水平初步研究

摘要

Objective To analyse the levels of G6PD activity in different types of thalassemia in Guangxi and to study their association with thalassemia.Me thods One hundred and thirteen negative thalassemia samples and 248 different types of thalassemia samples were analyzed by gene analysis and were detected for G6PD activity.Re-sults The levels of G6PD activity was (6.76 ±2.28)U/gHb in the negative thalassemia group, (9.00 ±3.67 )U/gHb inαthalassemia group, (10.98 ±6.25) U/gHb in βthalassemia group, and (9.09 ±2.96) U/gHb in αβcomplex thalassemia group, with significantly differences compared with the negative thalassemia group ( P <0.05 ) .The G6PD activity levels ofαthalassemia in silent, trait and Hb H groups were (6.67 ±1.65)U/gHb,(8.89 ±2.12)U/gHb, (12.7 ±5.44)U/gHb respectively;of βthalassemia in trait and intermedia groups were (9.68 ±3.71)U/gHb and (18.43 ±10.71)U/gHb.There were significant differences in the levels of G6PD between the different types of αthalassemia andβthalassemia inαthalassemia and βthalassemia groups(P<0.05).Conclusion Different levels of G6PD activity go with different types of thalassemia, the more severe anemia the higher G6PD activity.%目的了解广西地区不同地中海贫血类型患者的葡萄糖－6－磷酸脱氢酶（G6PD）酶活性水平，探讨其与地中海贫血的关联性。方法对经过地中海贫血基因分析确诊阴性的113名健康人群和248例不同地中海贫血类型的患者进行G6PD 酶活性检测，并对结果进行统计学分析。结果健康人群组的G6PD 酶活性水平为（6．76±2．28）U／gHb，α地中海贫血为（9．00±3．67）U／gHb，β地中海贫血为（10．98±6．25）U／gHb，αβ复合型地中海贫血为（9．09±2．96）U／gHb，与健康人群组比较差异均有统计学意义（P ＜0．05）。α地中海贫血中静止型、轻型和中间型的G6PD 酶活性水平分别为（6．67±1．65）、（8．89±2．12）和（12．7±5．44）U／gHb，β地中海贫血中轻型和中间型／重型的G6PD 酶活性水平分别为（9．68±3．71）U／gHb 和（18．43±10．71）U／gHb，α和β地中海贫血组内不同类型之间的G6PD 酶活性水平比较差异有统计学意义（P ＜0．05）。结论不同地中海贫血类型患者的G6PD 酶活性水平不一样，贫血程度越重G6PD 酶活性越高。