Physiology and pharmacology of secretion and cystic fibrosis. A review of past developments and projections into the future. pp. 311-36.

Cystic fibrosis is a recessively inherited metabolic disease which is characterized by 
a universal dysfunction in the exocrine glands of affected individuals. This 
dysfunction results in abnormalities of the composition and/or the physiochemical behavior 
of the various exocrine gland products. Although significant advances have been 
made in our understanding of exocrine gland physiology and pharmacology, many 
questions remain unanswered and significant gaps exist in our knowledge of 
exocrine gland function. Perhaps clarification of these issues may lead to 
better understanding of the pathogenesis of cystic fibrosis and to development 
of better forms of therapy for the patients. The purpose of 
this article is to review past and present developments in this 
area of biomedical research and to point out areas of exocrine 
gland physiology and pharmacology where more knowledge is needed in order 
to clarify the nature of a disease like cystic fibrosis. Composition 
of the acinar fluid, secretory mechanisms, modification of ductal fluid, ionic 
dependence and energy requirements of the secretory process, calcium metabolism in 
exocrine glands, and exocrine gland dysfunction in cystic fibrosis are discussed. 
