- 期刊
鼻及鼻竇腔之局限型類澱粉沈著症 — 病例報告
Amyloidosis Localized in the Nasosinal Cavity - Case Report
摘要
類澱粉沈著症是一群未知病因的疾病，定義為身體的一處、或多處有蛋白質纖維絲（protein fibrils），以β摺片（β-pleated sheet）的結構，堆積於細胞外。臨床上分為局限型及全身型。局限型只有局部器官受侵犯，或無嚴重臨床症狀，預後不錯；至於全身型，可侵犯任何器官，造成嚴重的後遺症，預後相當不好。本科於1999年7月，經歷一78歲男性病人，主訴右側鼻塞已有3至4年。檢查發現右側鼻腔內，有一不規則腫塊，對去充血劑無反應。電腦斷層掃瞄發現，右側副鼻竇內亦有腫塊。經切片後，病理報告為類澱粉沈著症。乃安排內視鏡鼻竇手術將之完全清除。而全身性檢查，包括血清及尿液蛋白質電泳分析，均為正常。且病人並無慢性副鼻竇炎，或鼻部創傷的病史。乃診斷為鼻部及副鼻竇的局限型類澱粉沈著症。
並列摘要
Amyloidosis describes a group of diseases of unknown aetiology that is defined by the extracellular deposition of protein fibrils in a β-pleated sheet configuration in one or more sites of the body. The clinical classifications of amyloidosis are: 1) systemic disease involving any organ system, with serious clinical sequelae and a grave prognosis, 2) local disease involving only one organ, with only local or no clinical consequences. In July 1999, we treated a 78-year-old man who presented with three to four years history of right sided nasal obstruction. On physical examination, an irregular, mucosa-covered mass was identified in the right nasal cavity which did not respond to decongestants. A subsequent CT scan identified a non-enhancing soft tissue opacity in the right nasal cavity and maxillary sinus. A biopsy was taken and tissue pathology showed amyloid deposits within normal mucosa. Systemic amyloidosis was ruled out by further work-ups, including urine and serum protein electrophoresis and bone marrow aspiration, all of which were within the normal range. Accordingly, a diagnosis of localized amyloidosis of the nose and paranasal sinuses was made. Endoscopic sinus surgery was selected for treatment, and symptoms were completely eliminated after total extirpation. There has been no evidence of recurrence over a follow-up period of 10 months. An otolaryngologist is often the first physician to evaluate such patients and should therefore be mindful of the distinction between local and systemic disease as the prognosis differs markedly. This case is presented owing to its extreme rarity.