Kuptamethi S, Tantiniti P, Wanachiwanawin W, Fucharoen S, Thareeruchata S, Choopanya K
Department of Clinical Microscopy, Faculty of Medical Technology, Mahidol University, Bangkok, Thailand.
Southeast Asian J Trop Med Public Health. 1992 Mar;23(1):64-70.
Erythrocyte glucose-6-phosphate dehydrogenase (G6PD) and pyruvate kinase (PK) activities were studied in hemoglobin H (HbH) patients by spectrophotometric method, cytochemical method and the methemoglobin reduction (MR) test for the detection of heterozygous G6PD deficiency. G6PD deficiency was found in 7 of 64 cases (10.9%), including 3 cases of genotype alpha 1/alpha 2 and 4 cases of genotype alpha 1/CS. None of the HbH patients was found to be PK-deficient. Spectrophotometrically determined G6PD and PK activities were significantly higher in HbH patients than in normals (p less than 0.001), whereas the MR test yielded a significantly lower percentage of residual methemoglobin in HbH patients than in normals (p less than 0.05). All three methods were efficient in the detection of hemizygous G6PD deficiency in HbH patients, but not in G6PD-deficient females.
采用分光光度法、细胞化学法和高铁血红蛋白还原（MR）试验，对血红蛋白H（HbH）患者的红细胞葡萄糖-6-磷酸脱氢酶（G6PD）和丙酮酸激酶（PK）活性进行了研究，以检测杂合子G6PD缺乏症。64例患者中有7例（10.9%）发现G6PD缺乏，其中3例为α1/α2基因型，4例为α1/CS基因型。未发现HbH患者存在PK缺乏。分光光度法测定的HbH患者G6PD和PK活性显著高于正常人（p<0.001），而MR试验显示HbH患者高铁血红蛋白残留百分比显著低于正常人（p<0.05）。这三种方法在检测HbH患者的半合子G6PD缺乏症方面均有效，但对G6PD缺乏的女性无效。
