一例独特的AH显性型结节性肺淀粉样变性,表现为自发性肺气肿:病例报告和文献复习。

IF 2.3 4区 医学 Q3 ONCOLOGY Pathology & Oncology Research Pub Date : 2023-09-22 eCollection Date: 2023-01-01 DOI:10.3389/pore.2023.1611390
Valeria Skopelidou, Pavel Hurník, Lubomír Tulinský, Vladimir Židlík, Jiří Lenz, Patricie Delongová, Helena Hornychová, Patrik Flodr, Tomáš Jelínek, Ludmila Muroňová, Dušan Holub, Petr Džubák, Marián Hajdúch
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引用次数: 0

摘要

淀粉样变性是一种罕见的代谢紊乱,主要由自体蛋白质的错误折叠引起,导致其以异常的原纤维形式局部或全身沉积。肺组织很少受到淀粉样变性的影响;在涉及肺组织的三种形式中,结节性肺淀粉样变性最为罕见。结节性肺淀粉样变性很少引起临床症状,大多数情况下,它是在尸检或成像技术中意外发现的。文献中仅发现一例结节性肺淀粉样变性,表现为自发性肺气肿。就更精确的分型而言,结节性淀粉样变性通常为AL或混合型AL/AH。在文献中没有发现关于AH显性型结节性淀粉样变性的出版物。我们报告一例81岁男性结节性肺AH显性型淀粉样变性,并发自发性肺气肿。为了更深入地了解这一主题,本研究还回顾了结节性肺淀粉样变性病例与淀粉样纤维精确分型的关系。由于这通常是一个困难的过程,准确的淀粉样蛋白类型识别很少实现。然而,这些信息对于识别潜在的疾病过程(如果有的话)和概述随后的诊断和治疗步骤非常有帮助。即便如此,在对肺部病变进行鉴别诊断时,了解这个单元并确保将其考虑在内也是至关重要的。
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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A unique case of AH-dominant type nodular pulmonary amyloidosis presenting as a spontaneous pneumothorax: a case report and review of the literature.

Amyloidosis is a rare metabolic disorder primarily brought on by misfolding of an autologous protein, which causes its local or systemic deposition in an aberrant fibrillar form. It is quite rare for pulmonary tissue to be impacted by amyloidosis; of the three forms it can take when involving pulmonary tissue, nodular pulmonary amyloidosis is the most uncommon. Nodular pulmonary amyloidosis rarely induces clinical symptoms, and most often, it is discovered accidentally during an autopsy or via imaging techniques. Only one case of nodular pulmonary amyloidosis, which manifested as a spontaneous pneumothorax, was found in the literature. In terms of more precise subtyping, nodular amyloidosis is typically AL or mixed AL/AH type. No publications on AH-dominant type of nodular amyloidosis were found in the literature. We present a case of an 81 years-old male with nodular pulmonary AH-dominant type amyloidosis who presented with spontaneous pneumothorax. For a deeper understanding of the subject, this study also provides a review of the literature on cases with nodular pulmonary amyloidosis in relation to precise amyloid fibril subtyping. Since it is often a difficult process, accurate amyloid type identification is rarely accomplished. However, this information is very helpful for identifying the underlying disease process (if any) and outlining the subsequent diagnostic and treatment steps. Even so, it is crucial to be aware of this unit and make sure it is taken into consideration when making a differential diagnosis of pulmonary lesions.

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来源期刊
CiteScore
6.30
自引率
0.00%
发文量
134
审稿时长
4-8 weeks
期刊介绍: Pathology & Oncology Research (POR) is an interdisciplinary Journal at the interface of pathology and oncology including the preclinical and translational research, diagnostics and therapy. Furthermore, POR is an international forum for the rapid communication of reviews, original research, critical and topical reports with excellence and novelty. Published quarterly, POR is dedicated to keeping scientists informed of developments on the selected biomedical fields bridging the gap between basic research and clinical medicine. It is a special aim for POR to promote pathological and oncological publishing activity of colleagues in the Central and East European region. The journal will be of interest to pathologists, and a broad range of experimental and clinical oncologists, and related experts. POR is supported by an acknowledged international advisory board and the Arányi Fundation for modern pathology.
期刊最新文献
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