Abstract：
Kawasaki disease is a self-limiting acute vasculitis that affects small and medium-sized vessels, and is the most common cause of acquired heart disease in children in our environment. Up to 25% of untreated patients develop coronary aneurysms. It is suspected that an infectious agent may be the trigger of the disease, but the causative agent is still unknown. Based on the previous evidence, recommendations are proposed for the diagnosis, treatment of acute disease, and the long-term management of these patients, in order to unify criteria. The diagnosis must be quick, based on easy-to-use algorithms and with the support of complementary tests. This document includes the indication of available imaging techniques, as well as the planning of cardiological examinations based on the initial involvement. Intravenous immunoglobulin is the basis of the initial treatment. The role of corticosteroids is still controversial, but there are studies that support its use as adjuvant treatment. A multidisciplinary working group has developed a scheme with different treatment guidelines depending on the risk factors at diagnosis, the patient\'s clinical situation, and response to previous treatment, including indications for thromboprophylaxis in patients with coronary involvement. The stratification of risk for long-term treatment is essential, as well as the recommendations on the procedures based on the initial cardiological involvement and its progression. Patients with coronary aneurysms require continuous and uninterrupted cardiological monitoring for life.
摘要：
川崎病是一种影响中小型血管的自限性急性血管炎，并且是我们环境中儿童获得性心脏病的最常见原因。高达25%的未经治疗的患者发展为冠状动脉瘤。怀疑感染因子可能是疾病的触发因素，但是病原体仍然未知。根据之前的证据,提出了诊断建议，治疗急性疾病，以及对这些患者的长期管理，为了统一标准。诊断必须很快,基于易于使用的算法和互补测试的支持。本文件包括可用的成像技术的指示，以及基于初始参与的心脏病检查计划。静脉免疫球蛋白是初始治疗的基础。皮质类固醇的作用仍然存在争议，但是有研究支持将其用作辅助治疗。一个多学科工作组根据诊断时的风险因素制定了一个具有不同治疗指南的计划，患者的临床情况，以及对先前治疗的反应，包括冠状动脉受累患者的血栓预防指征。长期治疗的风险分层至关重要，以及基于初始心脏受累及其进展的程序建议。冠状动脉瘤患者需要持续和不间断的心脏监测。
