Cystic fibrosis compared with the immotile-cilia syndrome. A study of mucociliary clearance, ciliary ultrastructure, clinical picture and ventilatory function.

Patients with cystic fibrosis (CF) were investigated for mucociliary clearance (with and 
without stimulation by terbutaline), clinical picture, ventilatory function and ultrastructure of 
cilia. The results were compared with those of patients with congenitally 
immotile cilia (immotile-cilia syndrome). Mucociliary clearance could be demonstrated in all 
the seven CF patients who succeeded in inhaling the test aerosol. 
Ciliary ultrastructure from a deceased CF patient was normal. Patients with 
the immotile cilia syndrome had no substantial clearance and defective cilia. 
The CF patients coughed more during the clearance measurements than any 
other group studied earlier, and their coughing was effective. One patient 
succeeded in avoiding coughing in both measurements and had faster clearance 
when he got terbutaline than when he got the vehicle. Although 
younger, the CF patients tended to be more obstructed in their 
lungs and more handicapped than the patients suffering from the immotile-cilia 
syndrome. The latter patients had more discomfort from rhinitis, sinusitis and 
otitis than had the CF patients. An impairment of the mucociliary 
transport rate is hence unlikely to be a primary pathogenic factor 
for the respiratory tract disease in CF patients. 