Parker G S, Mehlum D L, Bacher-Wetmore B
Laryngoscope. 1983 May;93(5):573-7. doi: 10.1002/lary.1983.93.5.573.
In the past 5 years much has been learned about the syndrome of ciliary dyskinesia, commonly referred to as immotile cilia syndrome. This syndrome appears to be a congenital defect in the ultrastructure of the cilia, which results in one of three basic defects; lack of dynein arms, absence of radial spokes, or transposition of microtubules. Three cases are presented with electron micrographs; they illustrate the diverse clinical presentations of this disease entity as well as some of the structural abnormalities. The normal and abnormal anatomy of the cilia is discussed and some explanation is offered as to why these structural abnormalities present with such a variety of clinical expressions.
在过去5年里，人们对纤毛运动障碍综合征（通常称为不动纤毛综合征）有了很多了解。该综合征似乎是纤毛超微结构的先天性缺陷，会导致三种基本缺陷之一：动力蛋白臂缺失、放射辐条缺失或微管移位。本文展示了3例伴有电子显微镜照片的病例；它们说明了这种疾病实体的不同临床表现以及一些结构异常。文中讨论了纤毛的正常和异常解剖结构，并对这些结构异常为何会呈现出如此多样的临床症状提供了一些解释。
