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镰状细胞贫血症红细胞渗透抵抗力的动态研究(作者译)

[Dynamic study of osmotic resistance of red cells in sickle cell anemia (author's transl)].

作者信息

Juhan I, Isnard G, Aquaron R

出版信息

Pathol Biol (Paris). 1976 Sep;24(7):451-5.

PMID:790270
Abstract

A study of the osmotic resistance of red cells from normal subjects (AA), patients with the sickle cell trait (heterozygous subjects) (AS) and homozygous subjects (SS) was carried out using a dynamic automatic micro-method: Danon's fragiligraph, permitting one to obtain simultaneous recording of cumulative and derivative curves of the hemolytic process. 15 samples of red cells of each of the three categories were studied. Fragiligrams obtained from SS red cells showed that they became hemolysed later and in a more progressive fashion than the normal red cells AA. The AS red cells occupied an intermediate position. 5 samples of each category of red cells were studied after various times of incubation in the presence of a reducing substance (sodium metabisulphite). It was shown that the red cells containing hemoglobin S were still more resistant to osmotic hemolysis when hemoglobin was in the deoxygenated form and this proportional to the quantity of deoxygenated hemoglobin, without the brutal process of sickling producing any special modification in the shape of the curve. The role of the membrane is considered.

摘要

使用动态自动微量法(达农脆性描记仪)对正常受试者(AA)、镰状细胞性状患者(杂合子受试者)(AS)和纯合子受试者(SS)的红细胞渗透抵抗力进行了研究,该方法能够同时记录溶血过程的累积曲线和导数曲线。对这三类受试者的15份红细胞样本进行了研究。从SS红细胞获得的脆性描记图显示,与正常红细胞AA相比,它们溶血较晚且过程更为渐进。AS红细胞处于中间位置。在存在还原物质(焦亚硫酸钠)的情况下孵育不同时间后,对每类红细胞的5份样本进行了研究。结果表明,当血红蛋白处于脱氧形式时,含有血红蛋白S的红细胞对渗透性溶血仍具有更强的抵抗力,且这种抵抗力与脱氧血红蛋白的量成正比,镰状化的剧烈过程并未使曲线形状产生任何特殊改变。文中还考虑了细胞膜的作用。

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