研究识别和处理心脏淀粉样变性的困难;获取重要知识和可靠的治疗方法。

Ghadir Mohammed Saleh Ali, William Ale Emmanuel Seme, Kiran Dudhat
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摘要

全身性淀粉样变性是一种罕见的蛋白质错误折叠和沉积病,会导致器官缓慢衰竭。全身性淀粉样变性有 15 种以上,每一种都由一种独特的前体蛋白引起,会促进淀粉样蛋白的产生和组织沉积。淀粉样变性会影响各种器官,包括心脏、肾脏、肝脏、神经、胃肠道、肺、肌肉、皮肤和软组织。淀粉样变性既可能是获得性的,也可能是遗传性的。阴险和可疑的体征往往会导致诊断的延误。近十年来,在淀粉样变性的识别、预测和处理方面取得了显著进展。基于质谱技术的射枪蛋白质组学彻底改变了淀粉样蛋白的分型,使新型淀粉样蛋白的鉴定成为可能。正确鉴定与淀粉样变性有关的前体蛋白至关重要,因为蛋白的种类会影响正确的治疗策略。心脏淀粉样变性是一种以淀粉样蛋白在心肌细胞外间隙的全身性蓄积为特征的疾病,会引起各种症状。淀粉样蛋白聚集体的堆积会导致心肌增厚和硬化,最终导致舒张功能障碍,并在适当的时候引发心力衰竭。我们在本文中研究了各种系统性淀粉样变性,为从业人员诊断和治疗这些不寻常的疾病提供了有益的工具。这篇综述全面分析了心脏淀粉样变性,并整合了当前的筛查、诊断、评估和治疗方法。
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Examining the Difficulties in Identifying and Handling Cardiac Amyloidosis; Acquiring Important Knowledge and Robust Treatment Methods.

Systemic amyloidosis is a rare protein misfolding and deposition condition that causes slow organ failure. Each of the more than 15 exclusive sorts of systemic amyloidosis, which encourage amyloid production and tissue deposition, is introduced by a unique precursor protein. Amyloidosis can affect various organs, including the heart, kidneys, liver, nerves, gastrointestinal tract, lungs, muscles, skin, and soft tissues. It can either be acquired or hereditary. Insidious and doubtful signs often cause a put-off in diagnosis. In the closing decade, noteworthy progressions have been made in the identity, prediction, and handling of amyloidosis. Shotgun proteomics based on mass spectrometry has revolutionized amyloid typing and enabled the identification of novel amyloid forms. It is critical to correctly identify the precursor protein implicated in amyloidosis because the kind of protein influences the proper treatment strategy. Cardiac amyloidosis is a disorder characterized by the systemic accumulation of amyloid protein in the myocardium's extracellular space, which causes a variety of symptoms. The buildup of amyloid aggregates precipitates myocardial thickening and stiffening, culminating in diastolic dysfunction and, in due course, heart failure. We examine every kind of systemic amyloidosis in this text to offer practitioners beneficial equipment for diagnosing and treating those unusual diseases. This review presents a comprehensive analysis of cardiac amyloidosis and consolidates current methods for screening, diagnosis, evaluation, and treatment alternatives.

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