Immotile cilia syndrome: radial spokes deficiency in a patient with Kartagener's triad

Acta Paediatr Scand. 1981 Jul;70(4):571-3. doi: 10.1111/j.1651-2227.1981.tb05742.x.

Abstract

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".

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adolescent
  • Cilia / physiology*
  • Cilia / ultrastructure
  • Humans
  • Kartagener Syndrome / physiopathology*
  • Male
  • Mucus / physiology
  • Nasal Mucosa / ultrastructure
  • Syndrome