心脏淀粉样变性的诊断进展
首发时间:2018-06-20
摘要:淀粉样变性是由于淀粉样蛋白沉积在细胞外基质,造成沉积部位组织和器官损伤的一组疾病,出现心脏受累时即为心脏淀粉样变性,该病病情重、进展快、治疗困难、病死率高,但临床少见且识别率低。本文对该病相关概念与分类、病理生理学特点、临床表现进行介绍的同时,从心电图、无创影像学(包括超声心动图、心脏磁共振成像、核素显像)、实验室以及病理学诊断方面重点叙述了诊断手段及相关领域进展,并对该病诊断流程进行了归纳总结,以协助临床了解并正确诊断该病,使患者获得及时治疗并获益。
关键词: 心脏淀粉样变性 免疫球蛋白轻链淀粉样变性 甲状腺素运载蛋白淀粉样变性 限制性心肌病
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Progress in the Diagnosis of Cardiac Amyloidosis
Abstract:Amyloidosis is a group of diseases caused by amyloid deposition in the extracellular matrix, which is characteristic by the damage of multiple tissues and organs, and the diagnosis of cardiac amyloidosis is established when heart is involved. The aim of this article is to review relevant concepts, classification, pathophysiological features and clinical manifestations of cardiac amyloidosis, as well as all the aspects on diagnosis of this disease, in order to improve the recognition and identification of the disease.
Keywords: Cardiac amyloidosis Light chain amyloidosis Transthyretin amyloidosis Restrictive cardiomyopathy
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