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。
Citation：
LI Xiaoliang,HUANG Na,LIU Dan,et al. Primary Respiratory System Amyloidosis and Review of the Literature. West China Medical Journal, 2009, 24(7): 1813-1815. doi:
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Copyright © the editorial department of West China Medical Journal of West China Medical Publisher. All rights reserved
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- 2. MERLINNI G, BELLOTTI V. Molecular mechanisms of amyloidosis[J]. N Engl J Med,2003,349:583-596..
- 3. BATIAKAPLAN, BRIANM.MARTIN, OLGA BOYKOV, et al.Codeposition of amyloidogenic immunoglobulin light and heavy chains in localized pulmonary amyloidosis[J]. Virchows Arch,2005,447:756-761..
- 4. ANTHONY C B. AYLWIN,MBCHB,MRCS. Imaging Appearance of Thoracic Amyloidosis[J].Thorac Imaging,2005,20:41-46..
- 5. SHAHPL, GILLMORE J D, COPLEYSJ, et al.The importance of complet Screening for amyloid fibril type and systemic disease in patients with Amyloidosis in the respiratory tract[J]. Sarcoidosis Vasc Diffuse Lung Dis,2002,19:134-142..
- 6. KENNEDY T L, PATEL N M. Surgical management of localized amyloidosis[J]. Laryngoscope,2000,110: 918-923..
- 7. MICHELLEA NEBENWITTICH M D, ROBERTL FOOTE M D, ANDSANJAYKALRA M D. External Beam Radiation Therapy for Tracheobronchial Amyloidosis[J].Chest,2007,132:262-267..
- 8. PEPYS M B, HERBERT J, HUTCHINSON WL, et al.Targeted pharmacological depletion of serum amyloid P component for treatment of human amyloidsis[J].Nature,2002,417:254-259..
- 9. PACCALIN M, HACHULLA E, CAZALETC, et al. Localized amyloidosis:a survey of 35 French cases[J].Amyloid,2005,12(4):239-245.
- 10. UTZ J P, SWENSEN S, GERTZ M. Pulmonary amyloidosis.The Mayo Clinic Experience from 1980 to1993[J]. Ann Intern Med,1996,124:407-413..
