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心肌病的分子机制与病理生理学:来自与SARS-CoV-2相关的儿童炎症性多系统综合征(PIMS)的见解

Molecular Mechanisms and Pathophysiology of Myocardial Disease: Insights from Pediatric Inflammatory Multisystem Syndrome (PIMS) Associated with SARS-CoV-2.

作者信息

Viadero María Teresa, Caldeiro María Jesús, Fernández-Suarez Natalia, Garde Jesús, Cabero María Jesús, González-Lamuño Domingo

机构信息

Pediatric Cardiology, Division of Pediatrics, University Hospital "Marqués de Valdecilla", University of Cantabria, Avda. Valdecilla s/n, 39008 Santander, Spain.

Division of Pediatrics, University Hospital "Marqués de Valdecilla", Avda. Valdecilla s/n, 39008 Santander, Spain.

出版信息

Int J Mol Sci. 2025 Apr 10;26(8):3580. doi: 10.3390/ijms26083580.

Abstract

Multisystem inflammatory syndrome in children (MIS-C), also known as pediatric inflammatory multisystem syndrome (PIMS), presents significant challenges in pediatric cardiology, due to its complex molecular pathophysiology. In this retrospective analysis of 15 cases that were managed at a single tertiary care center, we investigated the molecular contributors to myocardial dysfunction, including cytokine storms, hyperinflammation markers, and hypercoagulable states. Transient myocardial involvement was identified in 46.6% of patients, with complete recovery achieved within 2-4 weeks following treatment. Ferritin, NT-ProBNP, and troponin levels were significantly elevated in patients with ventricular dysfunction compared to those without. The neutrophil-to-lymphocyte ratio (NLR), which was previously identified as a severity marker in acute COVID-19, was also significantly higher in patients with ventricular dysfunction, suggesting its potential as a prognostic indicator in MIS-C. Notably, no coronary artery aneurysms were detected in the cohort. These findings underscore the importance of early, standardized therapeutic interventions in mitigating severe outcomes, and they provide valuable insights into the molecular mechanisms driving myocardial dysfunction in MIS-C. Incorporating NLR and ferritin into the initial diagnostic workup may improve the early triage and identification of high-risk MIS-C patients.

摘要

儿童多系统炎症综合征(MIS-C),也称为儿童炎症性多系统综合征(PIMS),由于其复杂的分子病理生理学,给儿科心脏病学带来了重大挑战。在对一家三级医疗中心收治的15例病例进行的回顾性分析中,我们研究了导致心肌功能障碍的分子因素,包括细胞因子风暴、高炎症标志物和高凝状态。46.6%的患者出现短暂性心肌受累,治疗后2至4周内完全恢复。与无心室功能障碍的患者相比,心室功能障碍患者的铁蛋白、N末端脑钠肽前体(NT-ProBNP)和肌钙蛋白水平显著升高。中性粒细胞与淋巴细胞比值(NLR)先前被确定为急性冠状病毒病(COVID-19)的严重程度标志物,在心室功能障碍患者中也显著更高,表明其在MIS-C中作为预后指标的潜力。值得注意的是,该队列中未检测到冠状动脉瘤。这些发现强调了早期标准化治疗干预在减轻严重后果方面的重要性,并为MIS-C中心肌功能障碍的分子机制提供了有价值的见解。将NLR和铁蛋白纳入初始诊断检查可能会改善对高危MIS-C患者的早期分诊和识别。

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