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囊性纤维化:红细胞体积正常。关于该疾病发病机制的模型实验。

Cystic fibrosis: normal volumes of red cells. Model experiments on the pathomechanism of the disease.

作者信息

Emrich H M, Heitz J, Katz S, Thom R

出版信息

Eur J Pediatr. 1976 Jul 12;122(4):293-6. doi: 10.1007/BF00481510.

Abstract

Normal values of mean cell volume (M.C.V.) and of distribution of single cell volumes (S.C.V.) have been observed in erythrocytes of patients with cystic fibrosis using an electronical particle-volume analyzer (sheath flow detector). From these results a strong defect in red cell salt transport seems improbable. Valinomucin induces shrinking (by increase of K+ permeability) and gramicidin D induces swelling (by increase of Na+ permeability) of normal erythrocytes. Addition of C.F. sweat to normal erythrocytes induces no volume change. From this result no influence of the "C.F. factor" on passive ion permeability is concluded.

摘要

使用电子颗粒体积分析仪(鞘流检测器)观察了囊性纤维化患者红细胞的平均细胞体积(M.C.V.)和单细胞体积分布(S.C.V.)的正常值。从这些结果来看,红细胞盐转运存在严重缺陷的可能性不大。缬氨霉素可诱导正常红细胞收缩(通过增加钾离子通透性),短杆菌肽D可诱导正常红细胞肿胀(通过增加钠离子通透性)。向正常红细胞中添加囊性纤维化患者的汗液不会引起体积变化。由此结果得出“囊性纤维化因子”对被动离子通透性没有影响的结论。

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