Tuberculosis and Respiratory Diseases

The Korean Academy of Tuberculosis and Respiratory Diseases (대한결핵및호흡기학회)
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A Case of Primary Localized Tracheobronchial Amyloidosis

원발성 국한성 기관기관지형 유전분증 1예

  • Kwak, Yee-Gyung (Department of Internal Medicine, College of Medicine, Inha University) ;
  • Kim, Hyun-Jeong (Department of Internal Medicine, College of Medicine, Inha University) ;
  • Lee, Chung-Hwon (Department of Internal Medicine, College of Medicine, Inha University) ;
  • Kim, Sung-Yeon (Department of Internal Medicine, College of Medicine, Inha University) ;
  • Cho, Jae-Hwa (Department of Internal Medicine, College of Medicine, Inha University) ;
  • Kwak, Sung-Min (Department of Internal Medicine, College of Medicine, Inha University) ;
  • Lee, Hong-Lyeol (Department of Internal Medicine, College of Medicine, Inha University) ;
  • Kim, Joon-Mee (Department of Anatomical Pathology, College of Medicine, Inha University) ;
  • Han, Hye-Seung (Department of Anatomical Pathology, College of Medicine, Inha University) ;
  • Ryu, Jeong-Seon (Department of Internal Medicine, College of Medicine, Inha University)
  • 곽이경 (인하대학교 의과대학 내과학교실) ;
  • 김현정 (인하대학교 의과대학 내과학교실) ;
  • 이충훤 (인하대학교 의과대학 내과학교실) ;
  • 김성연 (인하대학교 의과대학 내과학교실) ;
  • 조재화 (인하대학교 의과대학 내과학교실) ;
  • 곽승민 (인하대학교 의과대학 내과학교실) ;
  • 이홍렬 (인하대학교 의과대학 내과학교실) ;
  • 김준미 (인하대학교 의과대학 해부병리학교실) ;
  • 한혜승 (인하대학교 의과대학 해부병리학교실) ;
  • 류정선 (인하대학교 의과대학 내과학교실)
  • Published : 2002.02.28
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Abstract

Primary pulmonary amyloidosis is a rare condition that can be classified into the tracheobronchial, diffuse alveolar septal, and nodular parenchymal type. Tracheobronchial amyloidosis is characterized by deposition of fibrilar proteins in the tracheobronchial tree, and it can be subdivided into diffuse and focal varieties. In this report, a case of diffuse tracheobronchial amyloidosis confirmed by flexible fiberoptic bronchoscopic biopsy is presented. The patient was a 43-year old male with a chief complaint of cough and sputum for 20 days and dyspnea for one day. The chest CT scan showed diffusely thickened walls of both the main and lobar bronchi with calcification. The bronchoscopic findings showed nodular lesions of the trachea, a diffuse bronchial stenosis of both the main bronchi and a pinpoint narrowing of the left upper and right middle lobar bronchus. The biopsy showed submucosal deposits of homogenous eosinophilic amyloid materials and an apple-green birefringence under polarizing microscopy following the Congo-red stain.

기침 및 호흡곤란 주소로 내원한 43세 남자 환자에서 흉부 전산화 단층촬영 및 기관지경하 생검으로 다른 장기의 침범 없이 폐에만 국한된 미만성 기관기관지형 유전분증 1예를 경험하였기에 문헌고찰과 함께 보고하는 바이다.

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