上呼吸消化道之類澱粉沉積症

Amylodiosis in the Upper Aerodigestive Tract

林余安(Yu-An Lin) ； 許振益(Chen-Yi Hsu) ； 詹以吉(Yee-Jee Jan)

《台灣耳鼻喉頭頸外科雜誌》 38卷2期 (2003/04) Pp. 49-55

https://doi.org/10.6286/2003.38.2.49

摘要

背景：類澱粉沉積症是一群疾病的總稱，可能只侵犯單一解剖位置或也可能同時侵犯多個器官，所以可以造成相當多不同的症狀。本篇主要是要了解此病在上呼吸消化道的表現特性，處理方法和癒後等。
方法：本文回顧1986年5月至2000年7月間，至本院耳鼻喉科接受檢查，並經病理切片診斷為上呼吸消化道類澱粉沉積症之病人，共有11例。我們根據其病歷記載做一回溯性之研究。
結果：總共統計11例病患中，平均年齡為50.6歲（9歲至70歲）。有6例屬於局限性類澱粉沉積症，其中單純發生於喉部的為編號1-4例，其中第2例為相當罕見之小兒喉部類澱粉沉積症，第5例同時發生在喉部，口咽腔後壁及鼻咽腔。第6例為單側顎扁桃線類澱粉沉積症。喉部類澱粉沉積症病患多主訴聲音嘶啞，治療以二氧化碳雷射切除為主，術後病患主觀感覺聲音嘶啞皆有改善，但是容易有局部復發之現象。有5例屬於系統性類澱粉沉積症，皆有不同程度之巨舌症，病患主要接受內科治療，癒後比局限性類澱粉沉積症差。
結論：類澱粉沉積症為上呼吸消化道罕見之疾病，它可以沈積在不同的地方產生許多不同的臨床症狀，身為耳鼻喉科醫師應對此疾病有所了解，在正確診斷後，應給予適當的處理與治療，並且排除系統性類澱粉沉積症的可能性。

關鍵字

類澱粉沉積症 ； 上呼吸消化道 ； 喉 ； 巨舌症

並列摘要

BACKGROUND: Amyloidosis is a group of diseases that can exhibit diverse symptoms since they arise from amyloid infiltration of a single anatomical location or of multiple organs simultaneously. This article retrospectively reviews the presentation, management, and prognosis of amyloidosis in the upper aerodigestive tract.
METHODS: Eleven patients were diagnosed with amyloidosis in the upper aerodigestive tract between May 1986 and July 2000 in our ENT department. Medical charts were collected for retrospective review.
RESULTS: The average age of the 11 patients was 50.9 years (range: 9 to 70). Six cases were diagnosed with localized amyloidosis. In these six cases, four had laryngeal involvement alone, and one of the four was a rare pediatric patient. One case had unusual involvement of the nasopharynx, oropharynx, and larynx. The last case involved right sided palatine tonsillar amyloidosis. The chief complaint of laryngeal amyloidosis was hoarseness and the main treatment was endolaryngeal microsurgery with a CO2 Laser. The prognosis for the voice after surgery was good although local recurrence was common. Five patients with symptoms of macroglossia were diagnosed with systemic amyloidosis and were treated mainly with medications. They had a poorer prognosis and shorter life expectancy than the patients with localized amyloidosis.
CONCLUSION: Amyloidosis is rare in the upper aerodigestive tract. It can cause various clinical symptoms in multiple locations. As an otolaryngologist, a good understanding of amyloidosis and its management is crucial.

並列關鍵字

amyloidosis ； upper aerodigestive tract ； larynx ； macroglossia