NASAL MUCOCILIARY TRANSPORT AND CILIARY ULTRASTRUCTURE IN CYSTIC-FIBROSIS - A COMPARATIVE-STUDY WITH HEALTHY-VOLUNTEERS

Cystic fibrosis (CF) is a deadly hereditary disease that produces an a bnormally thick, viscous and abundant secretion in the respiratory tra ct. This secretion in,turn leads to the development of recurrent respi ratory infections and irreversible lung damage. We have studied nasal mucociliary transport by means of an isotopic technique in 12 patients with CF and in 12 healthy volunteers. Nasal mucociliary transport was repeated at 12-18 months in the patients. In five randomly selected p atients ciliary ultrastructure was studied. The velocity of nasal muco ciliary transport was significantly slower than in healthy persons (P < 0.001) and no significant differences were observed in both studies (P < 0.05). No significant differences were either observed in the CF group between the homo- and heterozygotes (P < 0.5), or in those six p atients infected by Pseudomonas aeruginosa (P < 0.05). Ciliary ultrast ructure was normal in one patient. In another patient the sample showe d no cilia, while the remaining three exhibited changes similar to tho se observed in chronic respiratory infections: supernumerary periphera l tubules, ciliary disorientation and ciliary complexes. (C) 1997 Else vier Science Ireland Ltd.