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. Valinomycin 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.
Zusammenfassung
Das mittlere Zellvolumen (MCV) und der Volumen-Variationskoeffizient von Erythrocyten wurde mit Hilfe eines elektronischen Partikel-Volumen-Analysators (Zentralstrahlcapillare) bei Patienten mit Mucoviscidose (cystische Pankreasfibrose (C.F.)) und gesunden Kontrollen gemessen. Da die Meßwerte bei C.F. nicht signifikant von den Normalwerten abweichen, wird ein ausgeprägter Defekt des Salztransportes bei C. F.-Erythrocyten als unwahrscheinlich angesehen. Nach Zusatz von Valinomycin zu Normalerythrocyten wird eine Volumenabnahme (durch Steigerung der K+-Permeabilität), nach Zusatz von Gramicidin D eine Volumenzunahme (durch Steigerung der Na+-Permeabilität) beobachtet. Zugabe von C.F.-Schweiß zu Normalerythrocyten bewirkt keine Volumenänderung. Hieraus wird geschlossen, daß der „C.F.-Faktor” keinen Einfluß auf die passive Ionenpermeabilität der Erythrocytenmembran hat.
This is a preview of subscription content, log in via an institution to check access.
Similar content being viewed by others
References
Balfe, J. W., Cole, C., Welt, L. G.: Red-cell transport defect in patients with cystic fibrosis and their parents. Science 162, 689–690 (1968)
Cole, C. H., Dirks, J. H.: Changes in erythrocyte membrane ATP'ase in patients with cystic fibrosis of the pancreas. Pediat. Res. 6, 616–621 (1972)
Emrich, H. M., Junge, W., Witt, H. T.: Further evidence for an optical response of chloroplast bulk pigments to a light induced electrical field in photosynthesis. Z. Naturforsch. 24b, 1144–1146 (1969)
Emrich, H. M., Stoll, E., Friolet, B., Colombo, J.-P., Richterich, R., Rossi, E.: Sweat composition in relation to rate of sweating in patients with cystic fibrosis of the pancreas. Pediat. Res. 2, 464–478 (1968)
Feig, S. A., Segel, G. B., Kern, K. A.: Intrinsic transport function in cystic fibrosis erythrocytes. Clin. Res. 22, 234A (1974a)
Feig, S. A., Segel, G. B., Kern, K. A., Osher, A. B., Schwartz, R. H.: Erythrocyte transport function in cystic fibrosis. Pediat. Res. 8, 594–597 (1974b)
Horton, C. R., Cole, W. Q., Bader, H.: Decreased Ca transport ATP'ase in cystic fibrosis erythrocytes. Biochem. biophys. Res. Commun. 40, 505–509 (1970)
Kaiser, D., Drack, E., Rossi, E.: Inhibition of net sodium transport in single sweat glands by sweat of patients with cystic fibrosis of the pancreas. Pediat. Res. 5, 167–172 (1971)
Mangos, J. A., McSherry, N. R.: Sodium transport inhibitory factor in sweat of patients with cystic fibrosis. Science 158, 135–136 (1967)
Pressman, B. C., Harris, E. J., Jagger, W. S., Johnson, J. H.: Antibiotic-mediated transport of alkali ions across lipid barriers. Proc. nat. Acad. Sci. (Wash.) 58, 1949–1956 (1967)
Sant'Agnese Di, P. A., Darling, R. C., Perera, G. A., Shea, E.: Abnormal electrolyte composition of sweat in cystic fibrosis of the pancreas. Pediatrics 12, 549–563 (1953)
Thom, R.: Vergleichende Untersuchungen zur elektronischen Zellvolumen-Analyse. Telefunken-Hochfrequenz-Technik (1972)
Author information
Authors and Affiliations
Rights and permissions
About this article
Cite this article
Emrich, H.M., Heitz, J., Katz, S. et al. Cystic fibrosis: Normal volumes of red cells. Eur J Pediatr 122, 293–296 (1976). https://doi.org/10.1007/BF00481510
Received:
Issue Date:
DOI: https://doi.org/10.1007/BF00481510