Disturbance of fluid and electrolyte transport in cystic fibrosis epithelia.

Abstract

Studies of the disease CF suggest that the basic defect is related to impaired electrolyte movement in the epithelia of a variety of organs with exocrine function. The disturbances of electrolyte secretion in the organs classically involved in CF range from 1) a decrease in secretion‐ or uptake of chloride ions in all the organs studied; 2) an increase in sodium uptake in nasal airway epithelium and 3) a decrease in bicarbonate output of the pancreas. In this review an overview is presented of the expression of the CF defect, the abnormalities of fluid and electrolyte secretion in each CF affected organ are considered in more detail with particular emphasis on the hormonal and neuronal (dys)regulation of ion transport systems in epithelial cells.