Studies on the destruction of red blood cells. X. The biophysics and biology of sickle-cell disease.

Studies on the destruction of red blood cells. X. The biophysics and biology of sickle-cell disease.
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红细胞破坏的研究。

DOI:
10.1001/archinte.1956.00250200021002
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发表时间:
1956
期刊:
A.M.A. archives of internal medicine
影响因子:
--
通讯作者:
W. Castle
W. Castle
中科院分区:
--
文献类型:
--
作者:
J. Harris;H. Brewster;T. H. Ham;W. Castle

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1927年,Hahn和Gillespie(19)在一篇经典论文中明确地将镰状细胞异常物理行为的基础定义为其血红蛋白在脱氧状态下的异常,并指出受影响的细胞是导致特征性溶血性贫血的主要原因。根据对悬浮在毒气室中的血滴的显微观察,以及从一个患有镰状贫血的儿童身上切除一个大脾脏的结果,他们得出如下结论:镰状细胞的形成是一种可逆的现象,取决于易感小体的血红蛋白的自由或结合状态。当血红蛋白处于结合状态时,盘状形态稳定;当处于非组合状态时,扭曲形式是稳定的。因此,易感小体对氧窒息的行为构成了与等高线相关的假设的特殊应用
In 1927, in a classic paper, Hahn and Gillespie 19 clearly defined the basis of the anomalous physical behavior of the sickle cell as an abnormality of its hemoglobin when in the deoxygenated state and pointed to the affected cells as the primary cause of the characteristic hemolytic anemia. From microscopic observations of drops of blood suspended in a gas chamber and from the results of the removal of a large spleen from a child with sicklemia, they were led to make the following statement: Sickle cell formation is a reversible phenomenon depending on the free or combined state of the hemoglobin of the susceptible corpuscles. When the hemoglobin is in the combined state, the discoid form is stable; when in the uncombined state, the distorted form is stable. The behavior of the susceptible corpuscles toward oxygen asphyxia thus constitutes a special application of a hypothesis correlating contour with