CAJ | 학술논문

目的探讨气管支气管淀粉样变(tracheobrorlchial amyloidosis,TBA)患者的临床特征和预后.方法回顾性分析1999年6月至2009年2月首都医科大学附属北京朝阳医院收治的7例TBA患者的临床资料,并结合文献进行分析.结果 7例患者中男5例,女2例;年龄33～71岁,平均年龄52.9岁.主要临床表现为反复咳嗽(6/7)、咳痰(6/7)、喘憋(5/7)、咯血(3/7)、发热(2/7)及声嘶(3/7).7例患者中5例胸片无明显异常;但7例患者胸部CT均可见受累气管支气管壁增厚、管腔不规则狭窄,2例伴管壁钙化,1例伴阻塞性肺炎和肺不张改变.7例患者均经支气管镜检查和取组织活检确诊.病理学检查7例患者支气管黏膜可见均质嗜伊红物质沉积,刚果红染色阳性.6例患者行肺功能检查,以中重度阻塞性通气功能障碍为主要表现,弥散量轻度降低或正常.目前治疗主要是减轻症状,可选用经支气管镜行介入治疗、手术或放疗等.7例患者随访时间为7个月～10年,1例老年、病变广泛且有心功能不全的患者死亡,3例病变累及气管的患者缓慢进展,3例患者稳定.结论 TBA是少见的缓慢进展的疾病,临床表现取决于气道受累的部位和程度,目前无确切治疗方法,气管受累的患者预后差.
목적 탐토기관지기관정분양변(tracheobrorlchial amyloidosis,TBA)환자적림상특정화예후.방법 회고성분석1999년6월지2009년2월수도의과대학부속북경조양의원수치적7례TBA환자적림상자료,병결합문헌진행분석.결과 7례환자중남5례,녀2례;년령33～71세,평균년령52.9세.주요림상표현위반복해수(6/7)、해담(6/7)、천별(5/7)、각혈(3/7)、발열(2/7)급성시(3/7).7례환자중5례흉편무명현이상;단7례환자흉부CT균가견수루기관지기관벽증후、관강불규칙협착,2례반관벽개화,1례반조새성폐염화폐불장개변.7례환자균경지기관경검사화취조직활검학진.병이학검사7례환자지기관점막가견균질기이홍물질침적,강과홍염색양성.6례환자행폐공능검사,이중중도조새성통기공능장애위주요표현,미산량경도강저혹정상.목전치료주요시감경증상,가선용경지기관경행개입치료、수술혹방료등.7례환자수방시간위7개월～10년,1례노년、병변엄범차유심공능불전적환자사망,3례병변루급기관적환자완만진전,3례환자은정.결론 TBA시소견적완만진전적질병,림상표현취결우기도수루적부위화정도,목전무학절치료방법,기관수루적환자예후차.
Objective To analyze the clinical features and prognosis of patients with tracheobronchial amyloidosis(TBA). Methods Clinical data of 7 TBA patients collected from June 1999 to February 2009 were reviewed and analyzed together with literature. Results There were 5 male and 2female patients, aged from 33 years to 71 years (mean age was 52. 9 years). The main clinical manifestations included chronic cough (6/7), sputum (6/7), short breath ( 5/7 ), hemoptysis ( 2/7 ), fever (2/7) and hoarseness (3/7). Chest X-rays of 5 out of 7 patients were normal. But chest CT of 7 patients showed irregular luminal narrowing and airway wall thickening, specially 2 of 7 showed calcification of airway wall,and 1 companied with obstructive pneumonia and atelectasis. All of 7 cases were diagnosed through bronchoscopes along with biopsy-proven TBA. Pathologic examination showed 7 cases had homogeneous addict hemagglutinin-esterase substance deposition in bronchial wall by confirmation of amyloid with Congo red histology. Further pulmonary function testing of 6 patients revealed obstructive ventilatory disturbance, and diffusion capacity for carbon monoxide were almost normal or reduced slightly. So far the treatments of TBA mainly alleviated the symptoms adopting interventional therapy through bronchoscopes, surgery and local radiotherapy. Follow-up ranging from 7 month to 10 years found that 1 senior patient with diffuse lesion and heart insufficiency was dead, 3 cases with lesion involving trachea had progress slowly, and the other 3 cases remained stable. Conclusions TBA was a rare and slowly progressive disease,and its clinical manifestations depended on the airway lesion of location and extent. No well-recognized therapy has been achieved yet. The cases with lesion involving trachea had poor prognosis.