Smallman L A, Gregory J
Hum Pathol. 1986 Aug;17(8):848-55. doi: 10.1016/s0046-8177(86)80206-4.
Primary ciliary dyskinesia is thought to be caused by a primary defect of ciliary ultrastructure and function. However, atypical cilia have also been described in humans with and without acquired respiratory tract disease. With few exceptions, these abnormalities have not been quantified. Ciliary ultrastructure was therefore studied in 21 specimens of bronchial mucosa from patients with a variety of respiratory problems and in five specimens of nasal mucosa from asymptomatic nonsmokers. The incidence of microtubular abnormalities and compound cilia was generally less than 10 per cent, and there was no correlation between the incidence of these abnormalities and the presence of lung carcinoma or smoking habits. Transposition of ciliary microtubules and radial spoke defects, specific microtubular abnormalities thought to be pathognomonic for primary ciliary dyskinesia, were observed in a number of specimens, and visualization of dynein arms, particularly inner dynein arms, was extremely difficult. It is concluded that ultrastructural abnormalities of cilia should be carefully quantified in patients with primary ciliary dyskinesia and control subjects before it can be assumed they have pathologic significance.
