滲出性中耳炎と慢性副鼻腔炎を合併したImmotile cilia症候群の1例

A Case of Immotile Cilia Syndrome with Secretory Otitis Media and Chronic Sinusitis

　はじめに

　気道粘膜には多数の線毛細胞が存在し，上皮表面の線毛運動により異物や細菌を運搬除去して生体の防御機構の一端を担っている。Immotile cilia syndromeはこれらの線毛の先天的異常のため線毛運動不全を起こし，上，下気道に感染を反復したり，精子鞭毛運動不全による男子不妊などさまざまな症状を呈する疾患である^1,2）。この度，長期にわたって肺炎を反復し，臨床的に慢性副鼻腔炎と滲出性中耳炎を合併したImmotile cilia syndromeの1例を経験したので報告する。

The immotile cilia syndrome (ICS) is charac-terized by chronic respiratory tract infectons and male sterility caused by congenital ultrastructual ciliary abnormalities.

A 27-year-old male was complaining of produc-tive cough and remittent fever. Electron micro-scopic observation of cilia of the nasal and bron-chial mucosa and sperm tails revealed defects of both dynein arms and subfibers, compatible to findings of ICS.

The patient had a long history of chronic sinu-sitis and secretory otitis media. It is emphasized that the management of ENT symptoms should be conservative but early and active treatment will be required to prevent further pulmonary damages and development of bronchiectasis.