異なる部位に肺炎を反復した原発性びまん性気管気管支アミロイドーシスの1例  [in Japanese] Diffuse Tracheobronchial Amyloidosis with Airflow Limitation and Repeated Pneumonias  [in Japanese]

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Abstract

症例は67歳の男性で湿性咳嗽で入院し, 右S<sup>6</sup>, 左舌区の肺炎と呼気時の連続性ラ音, 閉塞性換気障害を認めた. 胸部CT検査で中枢気管支はびまん性に壁の肥厚と石灰化を呈していた. 気管支鏡検査では気管下部から気管支全般にわたり, 多発性の黄色プラークとそれに伴うびまん性の内腔狭窄がみられた. 気管支粘膜生検を行い, 均質な好酸性物質の粘膜下沈着が認められ, Congo-red 染色によりアミロイドであることが証明された. 肺炎は抗生剤投与により改善したが, 1年後には右下葉に肺炎を反復した. 以上よりびまん性気管気管支アミロイドーシスと診断した. 閉塞性換気障害をもとに肺炎を反復する場合, 鑑別診断のひとつとして本疾患の可能性を念頭に置く必要がある.

A 67-year-old man was first admitted to our hospital complaining of a productive cough, and repeated episodes of pneumonia in different sites. Physical examination revealed expiratory wheezing and airflow limitation wlas documented with lung function tests. A chest X-ray film showed patchy pneumonic infiltrates. Chest computed tomography revealed a marked thickening of the tracheal and bronchial wall with linear calcification. Fiberoptic bronchoscopy revealed a diffuse infiltrative process in the tracheobronchial tree, which uniformly reduced the bronchial lumen. Bronchial biopsy specimens showed amyloid deposits with focal calcification, which was confirmed by Congo red and Dylon staining. Extensive examinations including rectal biopsy were negatie for systemic amyloidosis. We suggest that diffuse tracheobronchial amyloidosis should be included in the differential diagnosis of repeated pulmonary infections with airflow obstruction.

Journal

  • The Japanese journal of thoracic diseases

    The Japanese journal of thoracic diseases 34(1), 90-95, 1996-01-25

    The Japanese Respiratory Society

References:  18

Codes

  • NII Article ID (NAID)
    10008109289
  • NII NACSIS-CAT ID (NCID)
    AN00187758
  • Text Lang
    JPN
  • Article Type
    NOT
  • ISSN
    03011542
  • Data Source
    CJP  J-STAGE 
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