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

IF 1.9 4区 医学 Q2 OTORHINOLARYNGOLOGY European Annals of Otorhinolaryngology-Head and Neck Diseases Pub Date : 2024-09-17 DOI:10.1016/j.anorl.2024.09.002
C Maquet, A Willemet, A Francois, F Crampon, S Deneuve
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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.

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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。
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来源期刊
CiteScore
3.70
自引率
28.00%
发文量
97
审稿时长
12 days
期刊介绍: European Annals of Oto-rhino-laryngology, Head and Neck diseases heir of one of the oldest otorhinolaryngology journals in Europe is the official organ of the French Society of Otorhinolaryngology (SFORL) and the the International Francophone Society of Otorhinolaryngology (SIFORL). Today six annual issues provide original peer reviewed clinical and research articles, epidemiological studies, new methodological clinical approaches and review articles giving most up-to-date insights in all areas of otology, laryngology rhinology, head and neck surgery. The European Annals also publish the SFORL guidelines and recommendations.The journal is a unique two-armed publication: the European Annals (ANORL) is an English language well referenced online journal (e-only) whereas the Annales Françaises d’ORL (AFORL), mail-order paper and online edition in French language are aimed at the French-speaking community. French language teams must submit their articles in French to the AFORL site. Federating journal in its field, the European Annals has an Editorial board of experts with international reputation that allow to make an important contribution to communication on new research data and clinical practice by publishing high-quality articles.
期刊最新文献
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