Ｉｍｍｏｔｉｌｅ Ｃｉｌｉａ症候群

Abstract

In a recent year congenital anomalies of the cilia of the respiratory mucosal epithelium have been thought of as a cause of chronic airway disease, and electron microscopic observations on cilia in patients with airway disease have revealed that axonemes containing ATP-ase called dynein arms are missing in those cilia. This was first reported in 1977 by Eliasson as immotile cilia syndrome.In order to detect the patients with immotile cilia syndrome, 103 patients with one or more involved 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 (male only). Ten of the examined patients were diagnosed as having the 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.As the election microscopic findings of this syndrome, Afzelius has reported defects of the dynein arms, spoke head and central sheath. In the present study, besides these abnormalities, we have revealed new findings such as abnormal attachment of the dynein arms and twist of the peripheral microtubules near the distal end of central microtubules.