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正常婴儿和杂合子β地中海贫血婴儿出生后头两年血红蛋白F水平的发育变化。

Developmental changes in hemoglobin F levels during the first two years of life in normal and heterozygous beta-thalassemia infants.

作者信息

Metaxotou-Mavromati A D, Antonopoulou H K, Laskari S S, Tsiarta H K, Ladis V A, Kattamis C A

出版信息

Pediatrics. 1982 Jun;69(6):734-8.

PMID:6176937
Abstract

To study the developmental pattern of hemoglobin F (HbF) during the first two years of life, levels of HbF were estimated in two groups of infants: 117 normal infants and 98 heterozygotes for beta-thalassemia, all aged between 1 and 24 months. The results may be summarized as follows: (1) Levels of HbF in beta-thalassemia heterozygotes were significantly higher than those of normal infants of the same age (P less than .01). (2) A reference curve for the decline of HbF in infants with beta-thalassemia trait was established to facilitate the diagnosis of heterozygotes during this period of life. (3) Hemoglobin A2 (HbA2) was also higher in beta-thalassemia heterozygotes than in normal infants of the same age. HbA2 increases with increasing age, reaching normal adult values at age 5 to 6 months. It is postulated that the higher level of HbF in heterozygous infants during the first two years of life is associated with the presence of the beta-thalassemia gene, which influences the increased synthesis of HbF in red cell.

摘要

为研究出生后头两年血红蛋白F(HbF)的发育模式,对两组婴儿的HbF水平进行了评估:117名正常婴儿和98名β地中海贫血杂合子婴儿,年龄均在1至24个月之间。结果总结如下:(1)β地中海贫血杂合子婴儿的HbF水平显著高于同龄正常婴儿(P<0.01)。(2)建立了β地中海贫血特征婴儿HbF下降的参考曲线,以利于在此生命阶段对杂合子进行诊断。(3)β地中海贫血杂合子婴儿的血红蛋白A2(HbA2)也高于同龄正常婴儿。HbA2随年龄增长而增加,在5至6个月时达到正常成人水平。据推测,杂合子婴儿在出生后头两年HbF水平较高与β地中海贫血基因的存在有关,该基因影响红细胞中HbF合成增加。

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