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换血治疗后溶血病新生儿的珠蛋白链合成失衡。

Imbalance of globin chain synthesis in newborn infants with hemolytic disease after exchange transfusion.

作者信息

Gahr M, Jentsch E, Schföter W

出版信息

Pediatr Res. 1977 Jan;11(1 Pt 1):9-13.

PMID:556652
Abstract

The relative rates of beta-, gamma-, and alpha-globin chain formation in reticulocytes of six newborn infants ,ith hemolytic disease were determined before and in weekly intervals after exchange transfusion. Directly after birth (before exchange transfusion) the beta/alpha, gamma/alpha, and (beta + gamma)/alpha ratios in newborn infants with hemolytic disease (0.35 +/- 0.20, 0.67 +/- 0.12, and 1.06 +/- 0.29) were similar to those of normal newborn infants (0.33 +/- 0.07, 0.65 +/- 0.06, and 0.98 +/- 0.06). The gradual increase in the beta/alpha ratio seen during the postnatal period of eight normal newborn infants was also observed in newborn infants with hemolytic disease after exchange transfusion. In contrast, the gamma/alpha ratio was found to be increased (1.89 +/- 0.25) in all infants with hemolytic disease in the second week after exchange transfusion. The corresponding value for normal newborn infants was 0.48 +/- 0.25. After exchange transfusion the number of hemoglobin F-containing erythrocytes increased. Possible reasohs for this imbalance of globin chain formation after exchange transfusion may be the decreased erythropoiesis and/or the abnormal high hemoglobin A concentration after exchange transfusion or the introduction of an unknown, gamma chain formation-stimulating agent by exchange transfusion. Speculation In newborn infants with hemolytic disease the repression of gamma-globin chain formation normally seen during the postnatal period is replaced by a stimulated gamma-globin chain production after exchange transfusion.

摘要

测定了6例患有溶血性疾病的新生儿网织红细胞中β-、γ-和α-珠蛋白链形成的相对速率,分别在换血前及换血后每周进行一次测定。出生后立即(换血前),患有溶血性疾病的新生儿的β/α、γ/α和(β+γ)/α比值(分别为0.35±0.20、0.67±0.12和1.06±0.29)与正常新生儿(分别为0.33±0.07、0.65±0.06和0.98±0.06)相似。在8例正常新生儿出生后的时期内β/α比值逐渐增加的情况,在换血后的患有溶血性疾病的新生儿中也观察到了。相反,在换血后第二周,所有患有溶血性疾病的婴儿的γ/α比值均升高(1.89±0.25)。正常新生儿的相应值为0.48±0.25。换血后含血红蛋白F的红细胞数量增加。换血后珠蛋白链形成失衡的可能原因可能是换血后红细胞生成减少和/或血红蛋白A浓度异常升高,或者是换血引入了一种未知的、刺激γ链形成的物质。推测在患有溶血性疾病的新生儿中,出生后通常可见的γ-珠蛋白链形成的抑制被换血后受刺激的γ-珠蛋白链产生所取代。

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Imbalance of globin chain synthesis in newborn infants with hemolytic disease after exchange transfusion.换血治疗后溶血病新生儿的珠蛋白链合成失衡。
Pediatr Res. 1977 Jan;11(1 Pt 1):9-13.
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[Our experience with exchange transfusion of newborn infants with hemolytic disease].[我们对溶血性疾病新生儿进行换血治疗的经验]
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Hemoglobin biosynthesis in juvenile erythroleukemia: evidence of imbalanced globin chain synthesis.青少年型红白血病中的血红蛋白生物合成:珠蛋白链合成失衡的证据。
Blut. 1982 Jan;44(1):7-13. doi: 10.1007/BF00320681.