原发性呼吸系统淀粉样变性及文献复习

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。

Primary Respiratory System Amyloidosis and Review of the Literature

Objective： To study the clinical features, diagnosis and treatments of primary respiratory system amyloidosis, and review the literature to improve diagnosis and treatments of the disease. Methods：A retrospective analysis of 34 cases is made, which were diagnosed and treated in our hospital between November of nineteen ninety-nine and October of two thousand and eight, among which 2 cases were pharyngeal amyloidosis, 27 cases were laryngeal amyloidosis and 5 eases were trachebronchial amyloidosis. Results：24 cases were treated with CO2 laser lesion, and 3 patients required tracheotomy beacause of severe dyspea,2 cases were treated with tumor lesion endobronchialscopieally,the other 8 cases treated with conservative measures. Conclusion： The clinical features of primary respiratory amyloidosis are non-specific,their diagnosis rely on pathological biopsy and Congo red stain. There are no specific treatments for primary respiratory system amyloidosis.