<原著>びまん性汎細気管支炎様所見を呈した Primary Ciliary Dyskinesia の 1 例

Abstract

33歳, 女性, 保母。13歳頃より咳嗽, 多量の膿性痰を生ずる。慢性副鼻腔炎, 滲出性中耳炎, 不妊症を合併し, 上下気道の線毛の検索の結果, 気管線毛の運動能の欠除, 線毛正切横断面での内側dynein armの著しい欠損, 耳管粘膜移送能の著明な低下をみとめ, primary ciliary dyskinesiaと診断した。臨床症状, 胸部X-P所見, 肺機能検査所見はびまん性汎細気管支炎(DPB)に合致しており, primary ciliary dyskinesiaがDPBの病因の一つである可能性を示唆する症例である。

A case of primary ciliary dyskinesia in which clinical, laboratory and radiological findings were compatible with diffuse panbronchiolitis (DPB) was reported. The patient was 33 yearold female. She had complained of a quantity of viscid purulent sputum and mild exertional dyspnea for 20 years. She had also suffered from chronic sinusitis and otitis media exudativa since her childhood as well as infertility. Chest X-ray film demonstrated overinflation and diffuse micronodular shadows in all lung fields and lung function tests revealed moderate obstructive impairment. Cilia from her tracheal mucosa were immotile and ultrastructural examination by transmission electron microscopy revealed the lack of inner dynein arms of cilia which was one of the specific findings to primary ciliary dyskinesia. Since this case satisfied the so-called clinical diagnostic criteria for DPB, the chronic inflammation in both upper and lower respiratory tracts was supposedly caused by primary ciliary dyskinesia and this case suggests that primary ciliary dyskinesia is one of the etiologic factors which can cause DPB.