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目的:分析原发性呼吸系统淀粉样变的临床特点,诊断和治疗,并结合文献复习,以提高对该病的诊治水平。方法:回顾性分析我院1998年11月到2008年10月34例原发性呼吸系统淀粉样变性患者,其中发生于咽的淀粉样变性有2例,喉的有27例,气管支气管的有5例。结果:24例行全麻下CO2激光手术治疗,其中3例由于严重的呼吸困难先行支气管切开后再行手术治疗,2例行纤维支气管镜下单纯肿物切除术,余8例由于无法手术而给予对症治疗。结论:呼吸系统淀粉样变性的临床表现缺乏特异性,确诊主要靠病理活检和刚果红染色。目前尚无特异的治疗方法。
Objective: To analyze the clinical features, diagnosis and treatment of amyloidosis in primary respiratory system and to review the literature to improve the diagnosis and treatment of the disease. Methods: A retrospective analysis of 34 patients with primary respiratory amyloidosis from November 1998 to October 2008 in our hospital, including 2 cases of pharyngeal amyloidosis, 27 cases of larynx and tracheobronchial 5 cases. Results: Twenty-four patients underwent CO2 laser surgery under general anesthesia. Three patients underwent primary bronchotomy due to severe dyspnea and then underwent surgical resection. Two patients underwent simple resection of the tumor under fiberoptic bronchoscopy, And give symptomatic treatment. Conclusion: The clinical manifestations of amyloidosis in respiratory system are lack of specificity. The diagnosis mainly depends on biopsy and congo red staining. There is no specific treatment.