FLUCTUATION OF FETAL HEMOGLOBIN IN SICKLE-CELL ANEMIA

FLUCTUATION OF FETAL HEMOGLOBIN IN SICKLE-CELL ANEMIA
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DOI:
10.1016/0002-9378(76)90812-7
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发表时间:
1976-01-01
影响因子:
9.8
通讯作者:
WISER, WL
WISER, WL
中科院分区:
医学1区
文献类型:
--
作者:
MORRISON, JC;WHYBREW, WD;WISER, WL

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高胎儿血红蛋白(HbF)水平的纯合子镰状细胞患者的发病率和死亡率降低。这一因素将使人们相信,足够量的这种物质将对这种血红蛋白病的患者起到保护作用。这项研究利用怀孕和未怀孕的女性,以及男性,随访了很长一段时间,表明HbF水平波动与危机。一些患者的HbF水平低,有许多危象,而另一些患者的HbF水平高,没有危象。许多高水平的患者在危机发生时表现出HbF水平下降。更重要的是,没有患者在危机期间具有高水平的HbF,尽管在发作之前和之后量升高。讨论了这一发现的可能解释和后果。
A reduction of morbidity and mortality rates in homozygous sickle-cell patients was found in those with high fetal Hb (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, indicated that the HbF level fluctuated with crisis. Some patients had low HbF levels with many crises and others had high amounts of HbF with no crisis. 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 were discussed.