Ｉｍｍｏｔｉｌｅ ｃｉｌｉａ ｓｙｎｄｒｏｍｅの臨床的・電顕的観察

Abstract

Recently immotile cilia syndrome has gained the interest of a number of investigators from the aspects of physiology and pathology of the ciliary movement . This is because microstructual 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 addition, the author carried out an animal experiment as to whether or not morphological abnormalities of the cilia can be caused by external factors, namely, by sulfur dioxide (SO2) gas inhalation.In order to detect patients with immotile cilia syndrome, 62 male patients with one or more conditions such as paranasal sinusitis, bronchiectasis, situs inversus and sterility were examined using a ciliary function test and electron microscopic observation of the nasal cilia and sperm tails. 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 and sperm tails, 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 microscopic findings of this syndrome, Afzelius has reported defects of the dynein arms, spoke head and central sheath. Beyond these microstructural abnormalities, the author identified abnormal attachment of the dynein arms as a new parameter in the electron microscopic diagnosis of immotile cilia syndrome.This syndrome is hereditary in origin, since external stimulation by both chronic inflammation and SO2 gas has not produced the ultrastructural changes of the cilia characteristic of this syndrome.