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小儿气道多灶性淀粉样变

Multifocal amyloidosis of the pediatric airway.

作者信息

Clevens R A, Wiatrak B J, Myers M W

机构信息

Department of Otolaryngology-Head and Neck Surgery, University of Michigan Hospitals, Ann Arbor.

出版信息

Arch Otolaryngol Head Neck Surg. 1995 Feb;121(2):229-32. doi: 10.1001/archotol.1995.01890020091017.

Abstract

Amyloidosis of the upper aerodigestive tract is rare in the pediatric age group. We present the first reported case (to our knowledge) of multifocal primary upper airway amyloidosis in a child. An otherwise-healthy 15-year-old girl presented with hoarseness, nasal congestion, and odynophagia. Diagnostic evaluation included flexible nasopharyngoscopy, rigid nasal endoscopy, direct laryngoscopy, bronchoscopy, and biopsy. The results of Congo red staining of the specimen were characteristic of amyloid. The findings of an extensive immunologic and systemic evaluation were normal. We discuss the treatment and postoperative course of this patient, as well as the clinical and pathologic characteristics of amyloidosis, with particular reference to the otolaryngologic manifestations of amyloidosis.

摘要

上消化道气道的淀粉样变性在儿童年龄组中较为罕见。我们报告了(据我们所知)首例儿童多灶性原发性上呼吸道淀粉样变性病例。一名原本健康的15岁女孩出现声音嘶哑、鼻塞和吞咽痛。诊断评估包括纤维鼻咽喉镜检查、硬性鼻内镜检查、直接喉镜检查、支气管镜检查和活检。标本刚果红染色结果具有淀粉样蛋白的特征。广泛的免疫和全身评估结果均正常。我们讨论了该患者的治疗及术后过程,以及淀粉样变性的临床和病理特征,特别提及了淀粉样变性的耳鼻咽喉科表现。

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