Mucociliary clearance from central airways in patients with excessive sputum production with and without primary ciliary dyskinesia
Studies of mucociliary clearance were made on 17 patients with bronchiectasis and excessive sputum production. Tracheal mucus velocity was measured. Five patients with primary ciliary dysfunction and 12 who had no clinical evidence of this defect were studied. The mean (+/- SD) tracheal mucus velocity of the five patients with proved or presumptive primary ciliary dysfunction was 2.9 +/- 0.2 mm per minute and for the 12 patients without ciliary dysfunction, 7.9 +/- 1.4 mm per minute. (The previously determined rate for normals was 4.7 +/- 1.3 mm per minute.) In four of five patients with ciliary dysfunction, CBF was below 7.5 Hz while in the one patient of the 12 without ciliary dysfunction CBF was at the lower limit of normal values. Thus, mucociliary clearance responds to load, depends partly on CBF, and has a built-in control system and the means for compensating for ciliary dysfunction.