Search

Primary ciliary dyskinesia is a rare inherited heterogeneous motile ciliopathy in which respiratory cilia are stationary, or beat in a slow or dyskinetic manner, leading to impaired mucociliary clearance and significant otosinopulmonary disease. Ciliary beating can be evaluated using digital high-speed videomicroscopy (DHSV). However, given the lack of standard operating procedures, and as minor variations in DHSV protocols might influence ciliary beating, varying normal reference ranges are described between centers. We aimed to evaluate whether the delay after sampling, and the temperature for conservation of respiratory ciliated samples before videomicroscopy, might modify ciliary beating.