Study of the ciliary function in adult and pediatric patients with cystic fibrosis

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 ciliary function is poorly studied. Recent data suggested that abnormal ciliary beating may contribute to poor MCC in CF patients. However, results are controversial: ciliary beat frequency(CBF) might be either decreased or increased, compared with healthy subjects. We aimed to evaluate if ciliary function may have a role in impaired mucociliary clearance in CF patients and if this ciliary dyskinesia was present in childhood.
Methodology: Ciliated epithelial samples were obtained by nasal brushing from 22 pediatric (0-17 years) and 15 adult CF patients (18-78 years). Beating cilia were recorded using digital high-speed videomicroscopy at 37°C. Ciliary functional analysis(CFA) was assessed by CBF and the percentage of abnormal ciliary beat pattern(CBP) and was compared with 14 healthy subjects(HS) (22-54 years).
Results: There was a significant difference in CBF only between adult CF patients and HS. Abnormal CBP was significantly higher in CF patients, regardless of the age of patients.
Conclusion: This study suggested that an abnormal ciliary function, present in childhood, might contribute to impaired MCC in CF patients as shown by lower CBF or higher dyskinetic CBP compared with HS. To evaluate if ciliary dyskinesia is primary or secondary to chronic inflammation, CFA should be repeated after air-liquid interface cell culture.