PDF(Pubmed)
Abstract：
Kawasaki disease is a systemic vasculitis with a risk of developing coronary artery lesions if left untreated. Kawasaki disease can be diagnosed clinically with classical symptoms (conjunctivitis, rash, lymphadenopathy, mucositis, edema of hands and feet), but predicting the risk of developing coronary artery aneurysm remains challenging. The coronary sequelae of Kawasaki disease have significant morbidity and mortality and are the second most common cause of acquired cardiac disease in children. Several genetic and immune factors are involved in the inflammation of coronary artery lesions in Kawasaki disease. Inositol trisphosphate 3-Kinase (ITPKC), Foxp3+, circular RNAs, mannose-binding lectin 2 (MBL2), complement factor H (CFH), kininogen 1 (KNG1), serpin family C member 1 (SERPINC1) and fibronectin 1 (FN1) are the essential genes identified in the pathogenesis of coronary artery lesions in Kawasaki disease. The addition of methylprednisolone to a combination of aspirin and intravenous immunoglobulins and biological agents like anakinra, etanercept, infliximab, and immunosuppressants like cyclosporine prevents the occurrence of coronary artery aneurysms in Kawasaki disease. Since the coronary artery lesions form the second most common cause of acquired cardiac disease in children and the incidence of myocardial infarction is a late complication, the risk stratification for coronary artery aneurysms and follow-up protocols for the prevention of cardiac thrombosis were proposed by the American Heart Association in 2017.
摘要：
川崎病是一种全身性血管炎，如果不及时治疗，有发生冠状动脉病变的风险。川崎病可以在临床上诊断为经典症状（结膜炎，皮疹,淋巴结病，粘膜炎,手和脚水肿)，但预测发生冠状动脉瘤的风险仍然具有挑战性.川崎病的冠状动脉后遗症具有显着的发病率和死亡率，是儿童获得性心脏病的第二常见原因。多种遗传和免疫因素参与了川崎病冠状动脉病变的炎症反应。三磷酸肌醇3-激酶（ITPKC），Foxp3+,环状RNA，甘露糖结合凝集素2（MBL2），补体因子H（CFH），激肽原1（KNG1），serpin家族C成员1（SERPINC1）和纤连蛋白1（FN1）是川崎病冠状动脉病变发病机制中鉴定的重要基因。将甲基强的松龙添加到阿司匹林和静脉免疫球蛋白以及诸如anakinra之类的生物制剂的组合中，依那西普,英夫利昔单抗,环孢素等免疫抑制剂可预防川崎病冠状动脉瘤的发生。由于冠状动脉病变是儿童获得性心脏病的第二常见原因，而心肌梗死的发生率是晚期并发症，美国心脏协会于2017年提出了冠状动脉瘤的危险分层和预防心脏血栓形成的随访方案.
