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Laryngo-tracheobronchial amyloidosis: a case report and review of literature

机译:喉气管支气管淀粉样变性病:一例报道并文献复习

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摘要

Amyloidosis is a spectrum of diseases characterized by abnormal extracellular accumulation of proteinaceous material; its precise etiology still remains unclear. It may affect multiple organs, of which the commonest sites are larynx, bronchus and kidney. Laryngeal amyloidosis is usually a localized phenomenon rarely associated with systemic involvement, here we report a case of laryngeal amyloidosis with tracheobronchial involved. The patient was 31-years old. He had a history of open surgical operation for laryngeal amyloidosis in the left ventricle 4-years ago. This time he was suffered by amyloid deposition in his right ventricle, the lesion was excised on staged laryngoscopy under general anesthesia. After 20-months follow-up, the post-operative recovery was wonderful. The bronchoscopy examination and computerized tomography scan for chest demonstrated he also had right main bronchus amyloidosis. Being of no dyspnea, he was unwilling to accept an operation on bronchus. Though amyloidosis is a benign lesion, up to date, there are no curable treatments for such a disease, for severe cases, it may be fatal as a result of airway obstruction or respiratory failure. Here we review the pertinent references on this subject, and discuss the main managements for amyloidosis on larynx and bronchus.
机译:淀粉样变性病是一系列疾病,其特征在于蛋白质物质的异常细胞外蓄积。其确切病因仍不清楚。它可能会影响多个器官,其中最常见的部位是喉,支气管和肾脏。喉部淀粉样变性通常是局部现象,很少与全身性受累相关,在此我们报道一例伴有气管支气管的喉淀粉样变性。该患者为31岁。 4年前,他有左胸淀粉样变性开放手术的历史。这次他的右心室中有淀粉样蛋白沉积,在全麻下经分期喉镜切除了病变。经过20个月的随访,术后恢复良好。支气管镜检查和计算机X线断层扫描检查胸部显示他也患有右主支气管淀粉样变性。由于没有呼吸困难,他不愿接受支气管手术。尽管淀粉样变性病是一种良性病变,但迄今为止,尚无可治愈的治疗方法,对于严重的病例,由于气道阻塞或呼吸衰竭可能致命。在这里,我们审查有关此主题的相关参考文献,并讨论喉头和支气管淀粉样变性的主要治疗方法。

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