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内皮功能障碍:川崎病的分子机制与治疗策略

Endothelial Dysfunction: Molecular Mechanisms and Therapeutic Strategies in Kawasaki Disease.

作者信息

Paolini Lucia, Guida Fiorentina, Calvaruso Antonino, Andreozzi Laura, Pierantoni Luca, Lanari Marcello, Fabi Marianna

机构信息

Specialty School of Paediatrics, Alma Mater Studiorum, University of Bologna, 40139 Bologna, Italy.

Pediatric Emergency Unit, IRCCS Azienda Ospedaliero-Universitaria di Bologna, 40139 Bologna, Italy.

出版信息

Int J Mol Sci. 2024 Dec 12;25(24):13322. doi: 10.3390/ijms252413322.

DOI:10.3390/ijms252413322
PMID:39769085
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11676170/
Abstract

The endothelium plays a key role in regulating vascular homeostasis by responding to a large spectrum of chemical and physical stimuli. Vasculitis is a group of inflammatory conditions affecting the vascular bed, and it is known that they are strongly linked to endothelial dysfunction (ED). Kawasaki disease (KD) is one childhood systemic vasculitis, and it represents the leading cause of acquired cardiac disease in children due to coronary damage and subsequent cardiovascular (CV) morbidity and mortality. We aimed to focus on the actual knowledge of ED in the pathogenesis of KD and its practical implications on therapeutical strategies to limit cardiovascular complications. Understanding ED in KD provides insight into the underlying mechanisms and identifies potential therapeutic targets to mitigate vascular damage, ultimately improving cardiovascular outcomes in both the acute and chronic stages of the disease. However, research gaps remain, particularly in translating findings from animal models into clinical applications for cardiovascular lesions and related morbidity in KD patients.

摘要

内皮细胞通过对多种化学和物理刺激作出反应,在调节血管稳态中发挥关键作用。血管炎是一组影响血管床的炎症性疾病,已知它们与内皮功能障碍(ED)密切相关。川崎病(KD)是一种儿童系统性血管炎,由于冠状动脉损伤以及随后的心血管(CV)发病率和死亡率,它是儿童后天性心脏病的主要原因。我们旨在关注KD发病机制中ED的实际知识及其对限制心血管并发症治疗策略的实际影响。了解KD中的ED有助于深入了解潜在机制,并确定减轻血管损伤的潜在治疗靶点,最终改善该疾病急性和慢性阶段的心血管结局。然而,研究空白仍然存在,特别是在将动物模型的研究结果转化为KD患者心血管病变及相关发病率的临床应用方面。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/06a4/11676170/4bea22a91cc8/ijms-25-13322-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/06a4/11676170/4bea22a91cc8/ijms-25-13322-g001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/06a4/11676170/4bea22a91cc8/ijms-25-13322-g001.jpg

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polymorphisms are associated with susceptibility to Kawasaki disease but do not predict intravenous immunoglobulin resistance and coronary artery aneurysms.多态性与川崎病的易感性相关,但不能预测静脉注射免疫球蛋白抵抗和冠状动脉瘤。
Front Immunol. 2024 Sep 18;15:1323171. doi: 10.3389/fimmu.2024.1323171. eCollection 2024.
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Whole-exome sequencing reveals Kawasaki disease susceptibility genes and their association with coronary artery lesion.全外显子组测序揭示川崎病易感基因及其与冠状动脉病变的关联。
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