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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

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