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肺淀粉样变性:病例系列

Pulmonary amyloidosis: A case series.

作者信息

Gandham Anil Kumar, Gayathri A R, Sundararajan L

机构信息

Department of Respiratory Medicine, Apollo Hospital, Chennai, Tamil Nadu, India.

出版信息

Lung India. 2019 May-Jun;36(3):229-232. doi: 10.4103/lungindia.lungindia_205_18.

Abstract

Amyloidosis is a spectrum of diseases, in which various proteins which are usual components of plasma are deposited as insoluble beta-pleated sheets extracellularly, disrupting function of various organs. Amyloid light-chain amyloidosis occurs due to the deposition of proteins, derived from immunoglobulin light chains, routinely manifesting with multisystem involvement. Pulmonary involvement is seen in about 50% of cases. Three common patterns of pulmonary amyloidosis on computed tomography (CT) chest are tracheobronchial, nodular parenchymal, and diffuse alveolar septal variety. We hereby report two cases of pulmonary amyloidosis, one being a case of diffuse alveolar septal pulmonary amyloidosis, which is an extremely rare pattern of involvement, with a very poor prognosis, and the other one being tracheobronchial pattern of involvement, which usually results due to the localized deposition of amyloid in the tracheobronchial tree. Knowledge about pulmonary amyloidosis is important due to its poor prognosis and nonspecific findings in CT chest.

摘要

淀粉样变性是一类疾病,其中作为血浆常见成分的各种蛋白质以不溶性β折叠片层的形式在细胞外沉积,破坏各个器官的功能。淀粉样轻链淀粉样变性是由于源自免疫球蛋白轻链的蛋白质沉积所致,通常表现为多系统受累。约50%的病例可见肺部受累。胸部计算机断层扫描(CT)上肺部淀粉样变性的三种常见模式为气管支气管型、结节性实质型和弥漫性肺泡间隔型。我们在此报告两例肺部淀粉样变性病例,一例为弥漫性肺泡间隔型肺部淀粉样变性,这是一种极其罕见的受累模式,预后很差,另一例为气管支气管型受累,通常是由于淀粉样蛋白在气管支气管树中的局部沉积所致。鉴于其预后不良以及胸部CT表现不具特异性,了解肺部淀粉样变性很重要。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/148b/6503711/12deb135e1f6/LI-36-229-g001.jpg

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