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如何处理喉淀粉样变?基于 16 例的经验。

How to deal with laryngeal amyloidosis? Experience based on 16 cases.

机构信息

Department of Otolaryngology, Head and Neck Surgery, Poznań University of Medical Sciences, Poland.

出版信息

Amyloid. 2012 Dec;19(4):177-81. doi: 10.3109/13506129.2012.723073. Epub 2012 Sep 24.

Abstract

INTRODUCTION

Amyloidosis is characterized by the deposition of amorphous fibrillar protein (amyloid) in the intercellular or intracellular space. Localized amyloidosis is rare in the head and neck area. In Polish literature of the last decade it has been the subject of case studies.

AIM

The goal of the study was to carry out a retrospective analysis of patients treated for amyloidosis in the Department of Otolaryngology and Head and Neck Surgery of the Poznań University of Medical Sciences in the period from 2000 to 2009. Based on the collected clinical material, the authors made an attempt to determine the most frequent anatomical location of amyloid deposits and the most frequently affected organs, the type and duration of symptoms, the therapeutic approach, further diagnostic measures taken and the final outcome of the treatment. The next goal was to assess the number of microlaryngoscopies performed in patients with amyloidosis affecting the larynx as compared to the overall number of such procedures.

MATERIAL

The detailed analysis included 16 patients with laryngeal amyloidosis.

RESULTS

Amyloidosis of the larynx was found in only 0.52% of patients undergoing microlaryngoscopies in the period from 2000 to 2009. In the vast majority of patients (11 of 16), there was no suspicion of amyloidosis in the initial diagnosis. Only the histopathological assessment definitely confirmed the nature of pathological changes in the larynx. Glottis was the most common location of amyloid deposits in the study group. In all cases, additional studies and long-term monitoring of the postoperative course excluded generalized amyloidosis.

CONCLUSIONS

Laryngeal amyloidosis is very rare, however it should be considered in the differential diagnosis in patients with laryngeal dysfunction. Surgery remains the treatment of choice in most patients, although the attempts of radiotherapy are undertaken. Preserving the normal function of the organ remains the priority.

摘要

简介

淀粉样变性的特征是无定形纤维蛋白(淀粉样蛋白)在细胞间或细胞内沉积。局限性淀粉样变性在头颈部较为罕见。在过去十年的波兰文献中,它一直是病例研究的主题。

目的

本研究的目的是对 2000 年至 2009 年期间在波兹南医科大学耳鼻喉科和头颈部外科接受淀粉样变性治疗的患者进行回顾性分析。基于收集的临床资料,作者试图确定淀粉样沉积物最常见的解剖部位和最常受累的器官、症状的类型和持续时间、治疗方法、采取的进一步诊断措施以及治疗的最终结果。下一步的目标是评估患有喉淀粉样变性的患者进行的喉显微镜检查的数量与这些手术的总数相比。

材料

详细分析包括 16 例喉淀粉样变性患者。

结果

在 2000 年至 2009 年期间,喉显微镜检查中仅发现 0.52%的患者患有喉淀粉样变性。在绝大多数患者(16 例中的 11 例),在最初诊断时没有怀疑淀粉样变性。只有组织病理学评估明确证实了喉部病理变化的性质。声门是研究组中淀粉样沉积物最常见的部位。在所有情况下,进一步的研究和长期监测术后过程排除了全身性淀粉样变性。

结论

喉淀粉样变性非常罕见,但在有喉功能障碍的患者中应考虑进行鉴别诊断。手术仍然是大多数患者的治疗选择,尽管也尝试了放疗。保持器官的正常功能仍然是首要任务。

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