Cystic fibrosis: An inborn error of cellular electrolyte transport?
- 1 June 1990
- journal article
- review article
- Published by Wiley in Journal of Paediatrics and Child Health
- Vol. 26 (3) , 126-131
- https://doi.org/10.1111/j.1440-1754.1990.tb02408.x
Abstract
Abnormal epithelial transport in cystic fibrosis (CF) appears to provide a unifying hypothesis to explain the varying clinical manifestations of CF. The major abnormality is the cell regulation of epithelial Cl- secretion; however, a number of other abnormalities of electrolyte transport has been observed. A description of the normal physiology of secretion and the current state of our knowledge of the abnormalities of epithelial secretion is discussed.Keywords
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