Tanphaichitr V S, Mahasandana C, Suvatte V, Yodthong S, Pung-amritt P, Seeloem J
Department of Pediatrics, Faculty of Medicine, Siriraj Hospital, Mahidol University, Bangkok, Thailand.
Southeast Asian J Trop Med Public Health. 1995;26 Suppl 1:271-4.
Thalassemia hemoglobinopathies and glucose-6-phosphate dehydrogenase (G-6-PD) deficiency are prevalent in Thailand. We studied the prevalence of these disorders from 1,000 cord bloods collected during 14 months period, using EDTA as anticoagulant. Red blood cell G-6-PD quantitative assay was performed in all male subjects. Nine hundred and eighty five specimens were available for hemoglobin (Hb) typing by starch gel electrophoresis. Further evaluation by cellulose acetate electrophoresis and follow up were made in the cases who had Hb E and/or high level of Hb Bart's. It was found that out of 505 males, 61 cases (12.08%) had G-6-PD deficiency. Among 985 cases studied for Hb typing, 61.92% revealed normal Hb type AF while Hb E was present in 18.68% and Hb Bart's designated alpha-thalassemias were present in 25.18% respectively. Of these 985 cases, 18.78% had low Hb Bart's level ie detectable to 8.2% consistent with alpha-thal2, Hb Constant Spring (CS) or alpha-thal1 trait. Ten cases (1.02%) had high levels of Hb Bart's ranging from 16.1-35% without or with Hb CS and E, and further follow-up revealed homozygous Hb CS, Hb A-E-Bart's, Hb H and Hb H with Hb CS disease. The other 53 cases (5.38%) had low level of Hb Bart's with Hb E consistent with alpha-thalassemia trait with Hb E trait. There were 127 cases (12.89%) who had only Hb E trait and 3 cases (0.3%) who had Hb F and E without Hb A initially.
地中海贫血血红蛋白病和葡萄糖-6-磷酸脱氢酶(G-6-PD)缺乏症在泰国很普遍。我们使用乙二胺四乙酸(EDTA)作为抗凝剂,对14个月期间采集的1000份脐带血进行了这些疾病的患病率研究。对所有男性受试者进行了红细胞G-6-PD定量检测。985份标本可用于通过淀粉凝胶电泳进行血红蛋白(Hb)分型。对患有Hb E和/或高水平Hb Bart's的病例进行了醋酸纤维素电泳进一步评估和随访。结果发现,在505名男性中,61例(12.08%)患有G-6-PD缺乏症。在985例进行Hb分型研究的病例中,61.92%显示正常Hb型AF,而Hb E占18.68%,指定为α地中海贫血的Hb Bart's占25.18%。在这985例病例中,18.78%的Hb Bart's水平较低,即与α-thal2、血红蛋白Constant Spring(CS)或α-thal1特征一致,可检测到8.2%。10例(1.02%)的Hb Bart's水平较高,范围为16.1%-35%,伴有或不伴有Hb CS和E,进一步随访发现为纯合子Hb CS、Hb A-E-Bart's、Hb H和伴有Hb CS病的Hb H。另外53例(5.38%)的Hb Bart's水平较低,伴有Hb E,与伴有Hb E特征的α地中海贫血特征一致。有127例(12.89%)仅具有Hb E特征,3例(0.3%)最初具有Hb F和E但无Hb A。