Impairment of mucociliary transport in cystic fibrosis.

Over the past few years, studies from various laboratories have suggested the 
presence of a factor(s) in cystic fibrosis serum causing widespread in 
vitro abnormalities of components of the lung defence mechanism particularly with 
respect to macrophage dysfunction, quantitative and qualitative alterations in mucus, and 
ciliary motility. The possible in vivo role of ciliary dysfunction in 
the pathogenesis of the disease is of particular clinical interest. Since 
an in vitro ciliary dyskinesia factor had been previously demonstrated and 
pulmonary complications are the hallmark of CF, yet clearance in most 
of our clinically stable CF subjects was relatively well maintained, this 
suggested to us that a mucociliary transport abnormality might be present 
intermittently. In order to explore this further, we designed nasal mucociliary 
clearance studies to determine whether CF serum might exhibit MCT inhibition 
in vivo. From these studies, we concluded that there was a 
substance(s) in CF serum which had the capacity to slow MCT. 
Whether this effect is on the cilia themselves or via overproduction 
of normal or abnormal mucus is not known. 