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[心脏淀粉样变性——心力衰竭的重要鉴别诊断]

[Cardiac amyloidosis - important differential diagnosis in heart failure].

作者信息

Morbach Caroline, Steinhardt Maximilian, Störk Stefan

出版信息

Dtsch Med Wochenschr. 2022 Nov;147(23):1522-1530. doi: 10.1055/a-1810-9199. Epub 2022 Nov 16.

Abstract

The systemic disease amyloidosis is caused by deposits of misfolded proteins and can cause very different symptoms depending on the organ involvement. The two most common forms are AL amyloidosis and ATTR amyloidosis. The determination of light chains and bone scintigraphy have a high priority in the diagnostic algorithm. New, causally effective substance classes are available for the therapy of AL and ATTR amyloidosis. The classic heart failure medication is often not well tolerated by patients with cardiac amyloidosis and must be adjusted in the presence of symptoms. This article shows which findings and symptom complexes should make you think of amyloidosis and how to arrive at the correct diagnosis in a targeted manner. Causal therapy options as well as special features of heart failure therapy in this patient collective are explained.

摘要

系统性疾病淀粉样变性是由错误折叠的蛋白质沉积引起的,根据受累器官的不同可导致非常不同的症状。两种最常见的形式是AL淀粉样变性和ATTR淀粉样变性。轻链测定和骨闪烁扫描在诊断流程中具有高度优先级。有新的、具有因果效应的物质类别可用于治疗AL和ATTR淀粉样变性。经典的心力衰竭药物通常不为心脏淀粉样变性患者所耐受,出现症状时必须进行调整。本文展示了哪些检查结果和症状组合应使您想到淀粉样变性,以及如何有针对性地得出正确诊断。还解释了该患者群体的因果治疗选择以及心力衰竭治疗的特殊情况。

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