Cystic fibrosis. A dilemma in the metabolic pathogenesis of genetic disease.

The fundamental pathological observations in cystic fibrosis (CF) were made over three 
decades ago. More recently, physiological findings have directed investigation toward a 
plethora of humoral substances and their possible role in disturbances of 
the secretory processes in CF. The application of the techniques of 
molecular biology and of tissue culture is beginning to increase our 
knowledge about the so-called CF factors and their diverse effects on 
epithelial and ciliated surfaces. Emphasis in current research is on the 
chemical characterization of the CF factors, the metabolism of cells cultured 
from patients with CF, and the biochemical events controlling the processes 
of secretion. Some current theories as to the metabolic pathogenesis of 
CF are considered in the context of existing findings. 