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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.

PMID:5563348
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC1931133/
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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1
Thalassemia and G-6-PD deficiency in Chinese-Canadians: admission screening of a hospital population.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
Can Med Assoc J. 1971 Aug 7;105(3):283-6.
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Prevalence of hemoglobin E, alpha-thalassemia and glucose-6-phosphate dehydrogenase deficiency in 1,000 cord bloods studied in Bangkok.在曼谷对1000份脐带血进行研究中血红蛋白E、α地中海贫血和葡萄糖-6-磷酸脱氢酶缺乏症的患病率。
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引用本文的文献

1
Thalassemic hydrops fetalis in two Chinese-Canadian families.两个华裔加拿大家庭中的地中海贫血胎儿水肿综合征
Can Med Assoc J. 1972 Dec 23;107(12):1186-90.
2
Hemoglobinopathies in a hospital population in Vancouver.温哥华医院人群中的血红蛋白病
Can Med Assoc J. 1978 Oct 7;119(7):701-4.

本文引用的文献

1
Types of thalassaemia-trait carriers as revealed by a study of their incidence in Greece.通过对希腊地中海贫血特征携带者发病率的研究揭示的携带者类型。
Br J Haematol. 1962 Jan;8:5-14. doi: 10.1111/j.1365-2141.1962.tb06489.x.
2
THE AMINO ACID COMPOSITION OF HEMOGLOBIN. V. THE PREPARATION OF PURIFIED HEMOGLOBIN FRACTIONS BY CHROMATOGRAPHY ON CELLULOSE EXCHANGERS AND THEIR IDENTIFICATION BY STARCH GEL ELECTROPHORESIS USING TRIS-BORATE-EDTA BUFFER.血红蛋白的氨基酸组成。V. 通过在纤维素交换剂上进行色谱法制备纯化的血红蛋白组分,并使用三羟甲基氨基甲烷-硼酸盐-乙二胺四乙酸缓冲液通过淀粉凝胶电泳对其进行鉴定。
Blood. 1965 May;25:646-61.
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METHOD FOR THE AUTOMATIC DETERMINATION OF SERUM IRON.血清铁自动测定方法
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NOTES ON THE ACID ELUTION TEST FOR DETECTING FOETAL ERYTHROCYTES.检测胎儿红细胞的酸洗脱试验注释
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A METHOD FOR THE QUANTITATIVE DETERMINATION OF HGB A2.
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The methemoglobin reduction test for primaquine-type sensitivity of erythrocytes. A simplified procedure for detecting a specific hypersusceptibility to drug hemolysis.红细胞对伯氨喹型敏感性的高铁血红蛋白还原试验。一种检测对药物溶血的特定超敏反应的简化程序。
JAMA. 1962 May 5;180:386-8. doi: 10.1001/jama.1962.03050180032008.
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Haemoglobin A2 estimations by starch block electrophoresis.通过淀粉块电泳法测定血红蛋白A2
Med J Malaya. 1959 Sep;14:31-5.
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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.