Pulmonary aspects of cystic fibrosis. pp. 3-24.

Defective mucociliary transport has been implicated in the pathogenesis of the lung 
disease in cystic fibrosis, based on several observations. First, patients with 
the disease secrete more mucus than normal subjects, as indicated by 
gland hypertrophy, and frequently retain it in the lung. Second, the 
mucus is said to have altered biochemical and rheological properties; however, 
although the physiochemical properties of mucus are altered in all lung 
diseases, little histochemical or rheological difference has been found in mucus 
from patients with CF and other hypersecretory conditions such as chronic 
bronchitis and bronchiectasis. Third, a factor in the serum of CF 
patients inhibits normal ciliary activity in explain glands of ciliated epithelium; 
and infection, which damages human ciliated epithelium in culture, could further 
reduce mucociliary transport. Measurement of pulmonary function in cystic fibrosis, physical 
fitness and response to exercise in cystic fibrosis, control of breathing 
in patients with cystic fibrosis, regional lung function in cystic fibrosis, 
and therapy of cystic fibrosis are discussed. 