Exocrine-gland function and the basic biochemical defect in cystic fibrosis.

A biochemical link is proposed between secretion and reabsorption in exocrine glands, in the form of a factor (kinin E) which initiates ion reabsorption. Activation of this factor involves arginine esterases, and in cystic fibrosis these may be defective, preventing the formation of kinin E. In cystic fibrosis stimulation of exocrine glands produces abnormal reabsorption of ions, leading to the physiological abnormalities and clinical manifestations of the disease.