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呼吸道淀粉样变性。关注肺部受累的最新综述。

Respiratory Tract Amyloidosis. State-of-the-Art Review with a Focus on Pulmonary Involvement.

机构信息

Federal University of Rio de Janeiro, Rua Thomaz Cameron, 438, Valparaiso, Petrópolis, Rio de Janeiro, CEP 25685.120, Brazil.

Federal University of Bahia, Salvador, Brazil.

出版信息

Lung. 2015 Dec;193(6):875-83. doi: 10.1007/s00408-015-9791-x. Epub 2015 Aug 27.

Abstract

Amyloidosis is a constellation of disease entities characterized by abnormal extracellular deposition and accumulation of protein and protein derivatives, which show apple-green birefringence when stained with Congo red and viewed under polarized light. Amyloid can infiltrate virtually all organ systems and can display multiple and diverse imaging findings. Pathologically, respiratory involvement occurs in 50 % of patients with amyloidosis, and its clinical signs and symptoms vary depending on whether the disease is systemic or localized. The four main patterns of respiratory tract involvement are tracheobronchial, nodular parenchymal, diffuse alveolar septal, and lymphatic. Imaging findings of amyloidosis are nonspecific and vary in each pattern; knowledge about the disease impairment type is thus very important, and amyloidosis should be considered in the differential diagnosis of other very common diseases, such as infectious diseases, neoplasms, and vasculitis. This literature review describes the main clinical and imaging manifestations of amyloidosis, focusing on respiratory tract involvement and differential diagnosis.

摘要

淀粉样变性是一组以异常细胞外沉积和蛋白质及蛋白质衍生物积累为特征的疾病实体,用刚果红染色并在偏振光下观察时,这些蛋白显示出苹果绿双折射。淀粉样物质几乎可以渗透到所有的器官系统,并能显示出多种不同的影像学表现。病理学上,50%的淀粉样变性患者存在呼吸系统受累,其临床症状和体征取决于疾病是全身性还是局限性的。呼吸道受累的四个主要模式是气管支气管、结节性实质、弥漫性肺泡间隔和淋巴。淀粉样变性的影像学表现是非特异性的,在每种模式中都有所不同;因此,了解疾病损害类型非常重要,在鉴别诊断其他非常常见的疾病(如传染病、肿瘤和血管炎)时,应考虑淀粉样变性。本文综述描述了淀粉样变性的主要临床和影像学表现,重点介绍了呼吸道受累和鉴别诊断。

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