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镰状细胞贫血中胎儿血红蛋白的波动

Fluctuation of fetal hemoglobin in sickle-cell anemia.

作者信息

Morrison J C, Whybrew W D, Bucovaz E T, Wiser W L

出版信息

Am J Obstet Gynecol. 1976 Aug 15;125(8):1085-8. doi: 10.1016/0002-9378(76)90812-7.

DOI:10.1016/0002-9378(76)90812-7
PMID:952305
Abstract

A reduction of morbidity and mortality rates in homozygous sickle-cell patients was found in those with high fetal hemoglobin (HbF) levels. This factor would lead one to believe that an adequate amount of this substance would be protective to a patient with this hemoglobinopathy. This study utilizing pregnant and nongravid females, as well as males, followed for long periods of time indicates that the HbF level fluctuates with crisis. Some patients had low HbF levels with many crises and others had high amounts of HbF with no crises. However, many patients with high levels on one occasion demonstrated a decrease in HbF levels when crisis occurred. More importantly, no patient had high levels of HbF during a crisis although the amount was elevated before and after the episodes. The possible explanation and ramifications of this finding are discussed.

摘要

在胎儿血红蛋白(HbF)水平较高的纯合子镰状细胞病患者中,发现其发病率和死亡率有所降低。这一因素会让人认为,足量的这种物质对患有这种血红蛋白病的患者具有保护作用。这项针对孕妇、非孕妇以及男性进行长期跟踪研究表明,HbF水平会随病情危象而波动。一些患者HbF水平低且病情危象频繁,而另一些患者HbF水平高且无病情危象。然而,许多曾有高水平HbF的患者在病情危象发生时,HbF水平会下降。更重要的是,尽管在病情发作前后HbF量有所升高,但没有患者在病情危象期间HbF水平处于高位。本文讨论了这一发现的可能解释及其影响。

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Fluctuation of fetal hemoglobin in sickle-cell anemia.镰状细胞贫血中胎儿血红蛋白的波动
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