Mucociliary function, ciliary ultrastructure, and ciliary orientation in Young's syndrome
Mucociliary clearance is impaired in patients with Young's syndrome (obstructive azoospermia with recurrent sinobronchial disease), cystic fibrosis, and primary ciliary dyskinesia. No defect of cilia or mucus has been detected in Young's syndrome. Ciliary function and ultrastructure, including ciliary orientation, were studied quantitatively in 20 patients with Young's syndrome and 20 normal subjects to determine the incidences of ciliary defects. Nasal ciliated epithelium was obtained from each subject and used for measurement of ciliary beat frequency and ultrastructural analyses. Ciliary orientation was determined by measuring ciliary deviation in electron micrographs; ciliary deviation is a measure of the relative orientation of cilia in relation to each other in which high values indicate ciliary disorientation. Ciliary beat frequency and the incidence of microtubular defects and numbers of dynein arms did not differ between patients with Young's syndrome and control subjects. In patients with Young's syndrome basal ciliary deviation (16.0 degrees) was similar to that in control subjects (14.1 degrees), but at the ciliary tip ciliary deviation (21.9 degrees) was greater than in healthy subjects (14.5 degrees). The relative disorientation of the distal ciliary axoneme in patients with Young's syndrome compared with normal subjects may be due to a structural defect but is more likely to be a consequence of abnormal mucus.