[Amyloidosis: classification, diagnosis, and treatment].

IF 0.6
Innere Medizin (Heidelberg, Germany) Pub Date : 2026-08-01 Epub Date: 2026-06-08 DOI:10.1007/s00108-026-02130-4
Kiavasch M N Farid, Stefan Schönland, Norbert Blank, Fabian Aus dem Siepen, Markus Weiler, Jörg Beimler, Ute Hegenbart
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引用次数: 0

Abstract

Amyloidoses are rare protein-misfolding disorders characterized by the deposition of insoluble fibrillar aggregates, leading to potentially life-threatening organ damage. These deposits can be systemic or localized. Systemic amyloidoses are often associated with monoclonal gammopathies, chronic inflammatory conditions, or have a hereditary origin. Treatment strategies primarily focus on reducing the production of amyloidogenic proteins-depending on the specific protein and its origin-through therapies such as chemotherapy, anti-inflammatory agents, or gene-silencing approaches. Early diagnosis is crucial to prevent progressive organ dysfunction. Over the past 25 years, significant advances in the diagnosis and therapeutic management of amyloidosis have been made.

【淀粉样变性:分类、诊断和治疗】。
淀粉样变性是一种罕见的蛋白质错误折叠疾病,其特征是不溶性纤维聚集体的沉积,导致潜在的危及生命的器官损伤。这些沉积可以是系统性的,也可以是局部性的。全身性淀粉样变性常与单克隆病变、慢性炎症或遗传有关。治疗策略主要集中于减少淀粉样蛋白的产生——这取决于特定的蛋白质及其来源——通过化疗、抗炎药或基因沉默等治疗方法。早期诊断对于预防进行性器官功能障碍至关重要。在过去的25年里,淀粉样变的诊断和治疗管理取得了重大进展。
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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