Cystic fibrosis: an inborn error of cellular electrolyte transport?

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.