不动纤毛综合征：卡塔格内三联征患者的放射辐条缺陷

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

作者信息

Antonelli M, Modesti A, De Angelis M, Marcolini P, Lucarelli N, Crifo S

出版信息

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

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".

摘要

对一名患有卡塔格内综合征的13岁男孩的鼻纤毛黏液纤毛运输和超微结构进行了研究。黏液纤毛运输明显延迟（超过30分钟）。电子显微镜显示纤毛缺乏辐条、偏心中央微管以及一个外双联管错位。存在动力蛋白臂。我们认为辐条缺陷是一种独特的先天性异常，它促成了“不动纤毛综合征”的发病机制。