Bronchial mucus secretion in cystic fibrosis.

Mucus is the primary aetiological factor in the development of pulmonary disease 
in cystic fibrosis. Biochemical studies on bronchial secretions have not yet 
confirmed any qualitative difference in composition or in the nature of 
the glycoproteins, the major component of secretion. With the lack of 
any clear evidence of a basic abnormality in the nature of 
mucus, attention has been directed to the possible defect in the 
control of mucus secretion. This encompasses alteration in the normal regulation 
of the autonomic nervous system or to its stimulation either at 
the cellular or intracellular control level. In recent years, attention has 
focused on the presence of ciliotoxic factors in serum of children 
with cystic fibrosis, and their possible role in the pathophysiology of 
cystic fibrosis. Control of mucus secretion, submucosal glands, goblet cells, experimental 
models of lung disease in cystic fibrosis, the role of serum 
factors in mucus secretion, and the mucus blanket in chronic lung 
disease are discussed. 