Ciliary orientation in the ?immotile cilia? syndrome

Abstract

Ciliary orientation was studied in 43 patients with the "immotile cilia" syndrome. Twenty-four of these patients had total situs inversus. One mucosal specimen was taken from uterine cervical epithelium, 2 were from bronchial mucosa and 40 from nasal mucosa. The orientation of the cilia was measured from micrographs using a semiautomatic image analyzer (IBAS I). The results from patients were compared with those of 10 control subjects. The mean standard deviation and its standard deviation of the angles of ciliary orientation was 39.7 degrees +/- 9.2 degrees in 43 patients and 27.4 degrees +/- 4.3 degrees in the control group. The difference between the groups is highly significant statistically (P less than or equal to 0.001). However, there were no statistically significant differences in the standard deviations of ciliary orientation between the fields sectioned near the cell membrane or near the ciliary tip. We were also unable to find any significant differences in the standard deviations of the ciliary angles in the specimens taken from brush biopsies and excisional biopsies. There were also no statistically significant differences between the standard deviations of the ciliary angles for the groups with or without situs inversus. If 35 degrees is considered to be the limit value for the mean standard deviation between normal and pathological specimens in our total material, this would give a specificity of 0.90 and a sensitivity of 0.72.

Transmission Electron Microscopy in the Diagnosis of Primary Ciliary Dyskinesia

“…There are two tissue-obtaining techniques mainly used: nasal biopsy and bronchial biopsy. Significant differences in the SD of the ciliary angles in the specimens taken from brush biopsies and bronchial biopsies were not found [61,62]. Other factors influencing ciliary beat measurements have been reported.…”

Diagnostic approach to primary ciliary dyskinesia: a review

“…In a few cases of PCD, radial spoke defects and translocation can be found [32,33] which are ultrastructural features described as characteristic of PCD [12,24]. Random ciliary orientation changes [6,29] in the number of peripheral microtubules as well as a large number of compound cilia were found not only in patients with PCD but also in patients with chronic respiratory disease not due to PCD.…”

Cilia orientation and the fluid mechanics of development

“…: Randomization of rotational orientation of basal bodies and cilia has been observed in individual instances of primary ciliary dyskinesia (40,41). In one systematic study, ciliary rotational disorientation in PCD patients was always accompanied by immotility or dynein deficiency (42).…”