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目的探讨原发性支气管肺淀粉样变性临床表现、诊断、治疗及预后。方法回顾性分析10例原发性支气管肺淀粉样变性患者的临床特点。结果原发性支气管肺淀粉样变性患者临床表现为咳嗽、咳痰、咯血、气促,可经纤维支气管镜、肺穿刺或胸腔镜手术活检病理检查确诊,刚果红染色阳性为典型病理特征。结论本病临床少见,病理检查为确诊金标准。目前无疗效确切的治疗药物,气管受累及弥漫性病变者预后差。
Objective To investigate the clinical manifestations, diagnosis, treatment and prognosis of primary bronchial amyloidosis. Methods The clinical features of 10 patients with primary amyloidosis of bronchi were retrospectively analyzed. Results The clinical manifestations of patients with primary amyloidosis of bronchi were cough, expectoration, hemoptysis and shortness of breath. The results were confirmed by biopsy, lung biopsy or thoracoscopic biopsy. Congo red staining was a typical pathological feature. Conclusion The disease is rare clinical, pathological examination for the gold standard. Currently no effective treatment of therapies, tracheal involvement and diffuse lesions of poor prognosis.