원발성 국한성 후두 기관기관지형 유전분증 1예
A Case of Primary Localized Laryngo-tracheobronchial Amyloidosis
리원연 (연세대학교)
정순희 (연세원주의대)
권우철 (연세원주의대)
이선녕 (연세대학교 원주의과대학 내과학교실)
이남석 (연세대학교 원주의과대학 내과학교실)
김상하 (연세대학교)
신계철 (연세대학교 원주의과대학 내과학교실)
용석중 (연세대학교 원주의과대학 내과학교실)
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초록
저자들은 가래를 동반한 기침과 쉰 목소리로 내원한 68세 남자에게 흉부 전산화 단층촬영으로 우중엽 무기폐를 확인하고 기관지경 및 생검을 시행하여 후두와 기관기관지에 국한된 유전분증 1예를 경험하였기에 문헌고찰과 함께 보고한다.
- keywords
- Tracheobronchial amyloidosis, Laryngeal amyloidosis, Right middle lobe syndrome, Hoarseness
Abstract
We report on a case of a patient with laryngo-tracheobronchial amyloidosis who complained of cough, sputum,and hoarseness. A chest X-ray showed consolidation in the right middle lobe. A chest CT scan showed diffuse,irregular narrowing of the tracheobronchial tree and atelectasis of the right middle lobe, with calcification of bronchial wall. Bronchoscopic findings were multinodular submucosal thickening of the right vocal cord, and yellowish multinodular submucosal thickening from the lower trachea through both main bronchi, as well as the lingular division of the left upper lobe, the right middle lobe, and the right lower lobe. The right middle lobe bronchus was nearly obstructed. The diagnosis of amyloidosis was made by multiple bronchoscopic biopsies on the right vocal cord and both bronchi. Pathologic findings were characteristic apple-green birefringence under polarized microscopy with Congo-red stain. The patient had no evidence of systemic amyloidosis. The patient is under conservative symptomatic treatment
- keywords
- Tracheobronchial amyloidosis, Laryngeal amyloidosis, Right middle lobe syndrome, Hoarseness
참고문헌
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김미옥 ; 손장원 ; 양석철 ; 윤호주 ; 신동호 ; 박성수 ; 박문향, (2003) 레이저 소작술을 시행한 미만성 기관기관지 유전분증 1예, 대한내과학회지
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