Primary Ciliary Dyskinesia: Ciliary Activity

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Abstract
In primary ciliary dyskinesia (PCD) the cilia of the respiratory tract are immotile or they show an incoordinate, abnormal beating pattern. Consequently, mucociliary clearance is lacking. Most patients with PCD have ultrastructural abnormalities in their cilia. In some patients, however, the ciliary ultrastructure is completely normal. In this study a semi-quantitative scoring method is described for studying ciliary motility in biopsies of nasal mucosa. For every cell the frequency, coordination and amplitude of the ciliary beat are scored. Thereafter, a total score of ciliary activity per cell can be calculated. This method is simple and has proved to be reproducible. By using this scoring method the presence of a PCD can be established with certainty, whether the ciliary ultrastructure is abnormal or not. In patients with a normal ciliary ultrastructure the cilia have a rather high beating frequency (vibration). By scoring the ciliary motility in mucosal biopsies, the time-consuming electronmicroscopic investigation of the cilia is no longer necessary in most patients.