Transposition of Ciliary Microtubules

Abstract

SPECIFIC defects of cilia^{1 , 2} in the respiratory mucosa have been implicated in the pathogenesis of Kartagener's syndrome, a hereditary disease characterized by situs inversus, bronchiectasis, and sinusitis³ and occurring in siblings with sinobronchial disease but without transposition of the viscera. The term "immotile cilia syndrome"⁴ has been proposed for the hereditary disease characterized by ultrastructural abnormalities in the dynein arms^{1 , 5} and the radial-spoke linkages² of respiratory cilia and sperm. As part of a survey of children with respiratory disease, we have investigated two siblings with a chronic sinobronchial syndrome. We observed a new and distinctive structural defect in the cilia . . .