Watanabe Y, Okuda M
Rhinology. 1984 Sep;22(3):193-9.
Recently immotile cilia syndrome has gained the interest of a number of investigators from the aspects of the physiology and pathology of the ciliary movement. This is because microstructural abnormalities of the dynein arms in the cilia of the respiratory mucosal epithelium and in the flagella of sperm tails have been identified in this syndrome. The present study was designed to find a simple clinical method for detecting patients having this syndrome, and was conducted to elucidate the clinical significance and etiology of this syndrome. In order to detect patients with immotile cilia syndrome, 72 patients with one of more conditions such as sinusitis, bronchiectasis, situs inversus and sterility were examined using a ciliary function test and electron microscope observation of the nasal cilia. Seven of the examined patients were diagnosed as having immotile cilia syndrome on the basis of the presence of the characteristic ultrastructural patterns of the nasal cilia, i.e., disorders of the dynein arms. The possibility of positive test results increases greatly as the degree of the complications increases, especially in the case of a combination of chronic inflammation of the respiratory tract with situs inversus and sterility. As the electron microscope findings of this syndrome, Afzelius (1979) has reported defects of the dynein arms, spoke head and central sheath. Beyond these microstructural abnormalities, I have identified abnormal attachment of the dynein arms as a new parameter in the electron microscopic diagnosis of immotile cilia syndrome.
近来，不动纤毛综合征已从纤毛运动的生理学和病理学方面引起了许多研究者的关注。这是因为在该综合征中已发现呼吸道黏膜上皮纤毛和精子尾鞭毛中的动力蛋白臂存在微观结构异常。本研究旨在寻找一种简单的临床方法来检测患有该综合征的患者，并阐明该综合征的临床意义和病因。为了检测不动纤毛综合征患者，对72例患有鼻窦炎、支气管扩张、内脏转位和不育等一种或多种病症的患者进行了纤毛功能测试和鼻纤毛的电子显微镜观察。根据鼻纤毛特征性超微结构模式的存在，即动力蛋白臂异常，在所检查的患者中有7例被诊断为患有不动纤毛综合征。随着并发症程度的增加，检测结果呈阳性的可能性会大大增加，尤其是在呼吸道慢性炎症合并内脏转位和不育的情况下。作为该综合征的电子显微镜检查结果，阿费利乌斯（1979年）报告了动力蛋白臂、辐条头部和中央鞘的缺陷。除了这些微观结构异常外，我还发现动力蛋白臂的异常附着是不动纤毛综合征电子显微镜诊断中的一个新参数。
