Immotile cilia syndrome: radial spokes deficiency in a patient with Kartagener's triad.
Mucociliary transport and ultrastructure of nasal cilia in a 13 year old boy with Kartagener's triad, were investigated. Mucociliary transport was significantly delayed (greater than 30 minutes). Electron microscopy showed cilia lacking radial spokes, eccentric central tubules, and a dislocation of one the outer doublets. Dynein arms were present. We consider the radial spoke defect as a distinct congenital anomaly which contributes to the pathogenesis of the "immotile cilia syndrome".
D000293:Adolescent; D002923:Cilia / Q000502:physiology* / Q000648:ultrastructure; D006801:Humans; D007619:Kartagener Syndrome / Q000503:physiopathology*; D008297:Male; D009093:Mucus / Q000502:physiology; D009297:Nasal Mucosa / Q000648:ultrastructure; D013577:Syndrome