Middle-ear osteolytic transthyretin amyloidosis: A CARE case report.

IF 16.4 1区 化学 Q1 CHEMISTRY, MULTIDISCIPLINARY
C Maquet, A Willemet, A Francois, F Crampon, S Deneuve
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引用次数: 0

Abstract

Introduction: Wild-type transthyretin amyloidosis (ATTRwt) is a rare but serious disease that is underestimated due to asymptomatic progression. Cardiac deposits worsen prognosis, highlighting the importance of early detection for preventive treatment.

Case report: An elderly patient presented with an osteolytic lesion of the middle ear. Pathology diagnosed amyloid transthyretin deposits associated with cholesteatoma.

Discussion: Identifying reliable markers to screen for risk of cardiac amyloidosis is important, due to poor prognosis. Recent studies found higher prevalence of hearing loss in ATTRwt than in the general population. The present case identified the middle ear as a target of ATTR, which could improve our understanding of the pathophysiology.

中耳溶骨性转甲状腺素淀粉样变性病:CARE病例报告。
简介野生型转甲状腺素淀粉样变性(ATTRwt)是一种罕见但严重的疾病,由于无症状进展而被低估。心脏沉积物会使预后恶化,这突出了早期检测对预防性治疗的重要性:病例报告:一名老年患者出现中耳溶骨性病变。病理诊断为胆脂瘤相关的淀粉样转甲状腺素沉积:讨论:由于心脏淀粉样变性的预后较差,因此确定可靠的标志物来筛查心脏淀粉样变性的风险非常重要。最近的研究发现,ATTRwt 患者听力损失的发生率高于普通人群。本病例确定了中耳是 ATTR 的靶点,这有助于加深我们对病理生理学的理解。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
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来源期刊
Accounts of Chemical Research
Accounts of Chemical Research 化学-化学综合
CiteScore
31.40
自引率
1.10%
发文量
312
审稿时长
2 months
期刊介绍: Accounts of Chemical Research presents short, concise and critical articles offering easy-to-read overviews of basic research and applications in all areas of chemistry and biochemistry. These short reviews focus on research from the author’s own laboratory and are designed to teach the reader about a research project. In addition, Accounts of Chemical Research publishes commentaries that give an informed opinion on a current research problem. Special Issues online are devoted to a single topic of unusual activity and significance. Accounts of Chemical Research replaces the traditional article abstract with an article "Conspectus." These entries synopsize the research affording the reader a closer look at the content and significance of an article. Through this provision of a more detailed description of the article contents, the Conspectus enhances the article's discoverability by search engines and the exposure for the research.
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