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加拿大华裔人群中的地中海贫血和葡萄糖-6-磷酸脱氢酶缺乏症:医院人群的入院筛查

Thalassemia and G-6-PD deficiency in Chinese-Canadians: admission screening of a hospital population.

作者信息

Gray G R, Marion R B

出版信息

Can Med Assoc J. 1971 Aug 7;105(3):283-6.

Abstract

Admission screening was performed on 684 Chinese-Canadian patients for thalassemia, abnormal hemoglobins and glucose-6-phosphate dehydrogenase (G-6-PD) deficiency. Thirty-six healthy Chinese adults were also studied. The incidence of beta-thalassemia minor (hemoglobin A(2) greater than 3.5%) was 3.8%. Presumptive alpha-thalassemia minor (demonstration of occasional red cells containing hemoglobin H inclusion bodies) was found in 6.7%. Two patients had findings consistent with alpha-beta-thalassemia. The incidence of G-6-PD deficiency (abnormal methemoglobin reduction test) in adult males was 4.7%. In a parallel study the incidence of hemoglobin Bart's in 310 Chinese newborns was 6.8%. Two mutant hemoglobins were found - hemoglobin E and hemoglobin J (Bangkok).

摘要

对684名华裔加拿大患者进行了地中海贫血、异常血红蛋白和葡萄糖-6-磷酸脱氢酶(G-6-PD)缺乏症的入院筛查。还对36名健康的中国成年人进行了研究。轻度β地中海贫血(血红蛋白A2大于3.5%)的发病率为3.8%。推测的轻度α地中海贫血(偶尔发现含有血红蛋白H包涵体的红细胞)的发生率为6.7%。两名患者的检查结果与α-β地中海贫血一致。成年男性中G-6-PD缺乏症(高铁血红蛋白还原试验异常)的发生率为4.7%。在一项平行研究中,310名中国新生儿中血红蛋白Bart's的发生率为6.8%。发现了两种突变血红蛋白——血红蛋白E和血红蛋白J(曼谷型)。

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本文引用的文献

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METHOD FOR THE AUTOMATIC DETERMINATION OF SERUM IRON.血清铁自动测定方法
J Clin Pathol. 1965 Jan;18(1):98-102. doi: 10.1136/jcp.18.1.98.
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GLUCOSE-6-PHOSPHATE-DEHYDROGENASE DEFICIENCY AND NEONATAL JAUNDICE IN CHINESE MALE INFANTS IN HONG KONG.
Lancet. 1965 Feb 13;1(7381):350-1. doi: 10.1016/s0140-6736(65)91780-0.
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Ann N Y Acad Sci. 1964 Oct 7;119:557-60. doi: 10.1111/j.1749-6632.1965.tb54056.x.
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Estimation of small percentages of foetal haemoglobin.微量胎儿血红蛋白的测定。
Nature. 1959 Dec 12;184(Suppl 24):1877-8. doi: 10.1038/1841877a0.

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