Mucociliary transport in trachea of patients with cystic fibrosis.

Mucociliary tracheal transport rates were measured in 20 patients with cystic fibrosis, 
in whom these rates ranged from 0 to 12.8 mm/min. The 
patients were divided into 3 roughly equal groups on the basis 
of their transport rates. (1) Those in whom no abnormality in 
mucociliary transport was detected in the trachea; (2) those in whom 
normal transport rates were measured but in whom abnormalities such as 
cessation, or reversal of bolus movement were observed; (3) those in 
whom no normal transport rates were observed. In the first group 
the rates were similar to those observed in a population of 
healthy adults. These normal rates were observed in some patients who 
had a productive cough. The mean mucociliary tracheal transport rate increased 
with increasing maximum midexpiratory flow. Those patients with a low Shwachman 
score and poor arterial oxygen tension tended to fall into groups 
2 and 3. In the ciliary dyskinesia assay in rabbit trachea 
the serum from the patients with the higher transport rates tended 
to initiate more rapid discharge of material from the epithelium and 
ciliary dyskinesia. 