Abstract :
[en] Cilia are hair-like organelles, distributed widely throughout the human body, and may be either motile or sensory. Primary ciliary dyskinesia is an inherited motile ciliopathy, in which respiratory cilia are stationary, or beat in a slow or dyskinetic manner. This leads to impaired mucociliary clearance and significant sinopulmonary disease.
The diagnosis of primary ciliary dyskinesia is challenging, and a panel of different tests are required. Digital high speed videomicroscopy is one technique which allows real time analysis of complete ciliary function, including ciliary beat frequency and beat pattern, and allows beating cilia to be visualized in three different planes. Despite it being highly sensitive and specific test for diagnosing primary ciliary dyskinesia, digital high speed videomicroscopy lacks standardization.
A review of the current state of the art concerning digital high speed videomicroscopy ciliary functional assessment was conducted, and the methodology between different laboratories compared. The data was used to generate recommendations concerning digital high speed videomicrosopy methodology, evaluation, and interpretation.
The variability of ciliary beat pattern within healthy respiratory ciliated epithelium, and reproducibility in manual beat pattern analysis, was evaluated using digital high speed videomicroscopy. The results highlighted issues with single and limited manual beat pattern analysis. Compared to the sideways profile, in healthy subjects, cilia beating toward the observer and from above profile were found to be less sensitive in detecting ciliary dyskinesia. However, these beating profiles may be used to calculate ciliary beat frequency. In addition, the evaluation of five beat cycles to measure ciliary beat frequency is as accurate as ten beat cycles.
Summary
Standardized protocols for ciliary functional analysis using digital high speed videomicroscopy need to be established and applied in different laboratories with appropriate reference ranges. Ciliary beat pattern is not uniform within healthy respiratory ciliated epithelium, emphasizing the risk of limited beat pattern analysis. Further work is needed to evaluate the use of the different beat profiles for evaluation of ciliary function in patients with primary ciliary dyskinesia.
