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来自不同国家的新生儿以及患有某些严重血红蛋白病的婴儿的成人血红蛋白水平。

Adult hemoglobin levels in newborn babies from different countries and in babies with some significant hemoglobinopathies.

作者信息

Kutlar F, Kutlar A, Gu Y C, Huisman T H

机构信息

Department of Cell and Molecular Biology, Medical College of Georgia, Augusta.

出版信息

Acta Haematol. 1987;78(1):28-32. doi: 10.1159/000205831.

Abstract

The level of adult hemoglobin (Hb A, Hb S, Hb C, Hb E) was determined in cord blood samples of 66 Black babies (41 with four alpha-globin genes; 25 with two alpha-globin genes) with Hb A, of 51 SS, 7 CC, and 6 EE babies, of 359 babies from Mediterranean countries, and 197 babies from Japan and China. Methodology involved high-performance liquid chromatographic procedures, which are considered most accurate because of a complete separation of Hb F (or gamma chains) and the adult hemoglobins (or beta A or beta X chains). The presence of an alpha 2-thalassemia homozygosity (-alpha/ -alpha versus alpha alpha/alpha alpha) did not affect the average Hb A level. The levels of Hb S and Hb E in homozygous Hb S or Hb E babies were about one-third lower than the Hb A of the normal baby, while that of Hb C in homozygous Hb C babies was not decreased. The average level of Hb A in Chinese (50 babies) and Japanese (147 babies) was about 16.0%, which was significantly lower than the average level of 19.0% in newborns of Italian (221 babies), Yugoslavian (68 babies), and Turkish (70 babies) origin.

摘要

测定了66名携带成人血红蛋白(Hb A、Hb S、Hb C、Hb E)的黑人婴儿(41名有4个α珠蛋白基因;25名有2个α珠蛋白基因)、51名患镰状细胞贫血(SS)婴儿、7名患血红蛋白C病(CC)婴儿、6名患血红蛋白E病(EE)婴儿、359名来自地中海国家婴儿以及197名来自日本和中国婴儿的脐血样本中的成人血红蛋白水平。所采用的方法涉及高效液相色谱程序,由于该程序能将胎儿血红蛋白(Hb F,即γ链)与成人血红蛋白(即βA或βX链)完全分离,因而被认为是最准确的。α2地中海贫血纯合子(-α/-α与αα/αα相比)的存在并不影响平均Hb A水平。纯合子Hb S或Hb E婴儿的Hb S和Hb E水平比正常婴儿的Hb A水平低约三分之一,而纯合子Hb C婴儿的Hb C水平并未降低。中国婴儿(50名)和日本婴儿(147名)的Hb A平均水平约为16.0%,显著低于意大利(221名婴儿)、南斯拉夫(68名婴儿)和土耳其(70名婴儿)新生儿19.0%的平均水平。

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