Evidence for ciliary dyskinesia in cystic fibrosis

Abstract

Background: Mucociliary clearance (MCC) results in an effective interaction between the mucus layer and the normal coordinated ciliary beating. In cystic fibrosis (CF), MCC is impaired due to dehydrated mucus, but recent data suggest that an abnormal ciliary beating may contribute to poor MCC in CF patients. However, results are controversial: in CF patients, ciliary beat frequency (CBF) might be either decreased, or increased, and ciliary dyskinesia might be increased, compared with healthy subjects.

Aims: To compare ciliary function in CF patients and in healthy subjects.

Method: Ciliated epithelial samples were obtained by nasal brushing from 4 non-transplanted patients with CF (range age: 4-23). Beating cilia were recorded using digital high-speed videomicroscopy at 37°C within 9 hours after brushing. Ciliary functional analysis (CFA) is assessed by CBF and the percentage of dyskinetic ciliary beat pattern (CBP) and is compared with our laboratory normal values, calculated on 14 healthy subjects (range age: 22-54).

Results: There is no difference in CBF between CF patients and healthy subjects. However, ciliary dyskinesia is significantly higher in CF patients.

Conclusion: This pilot study suggests that, unlike previous studies, CBF is normal in CF patients. However, our results suggest that an abnormal ciliary function might contribute to impaired MCC in CF patients, as shown by the higher dyskinetic CBP compared with healthy subjects. To evaluate if ciliary dyskinesia is primary or secondary to chronic inflammation, CFA will be repeated after air-liquid interface cell culture.