Barrios Tascón Ana, Centeno Malfaz Fernando, Rojo Sombrero Henar, Fernández-Cooke Elisa, Sánchez-Manubens Judith, Pérez-Lescure Picarzo Javier
Cardiología infantil, Servicio de Pediatría, Hospital Universitario Infanta Sofía, San Sebastián de los Reyes, Madrid, España; Sociedad Española de Cardiología Pediátrica y Cardiopatías Congénitas.
Sociedad Española de Cardiología Pediátrica y Cardiopatías Congénitas; Cardiología infantil, Servicio de Pediatría, Hospital Universitario Río Hortega, Valladolid, España.
An Pediatr (Engl Ed). 2018 Sep;89(3):188.e1-188.e22. doi: 10.1016/j.anpedi.2018.04.003. Epub 2018 May 16.
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.
川崎病是一种自限性急性血管炎，可累及中小血管，是我们所处环境中儿童后天性心脏病最常见的病因。高达25%的未经治疗的患者会发生冠状动脉瘤。怀疑一种感染因子可能是该病的触发因素，但病原体仍不明确。基于先前的证据，提出了关于诊断、急性疾病治疗以及这些患者长期管理的建议，以便统一标准。诊断必须迅速，基于易于使用的算法并辅以补充检查。本文档包括可用成像技术的指征，以及基于初始受累情况的心脏检查计划。静脉注射免疫球蛋白是初始治疗的基础。皮质类固醇的作用仍存在争议，但有研究支持将其用作辅助治疗。一个多学科工作组制定了一个方案，根据诊断时的危险因素、患者的临床情况以及对先前治疗的反应制定不同的治疗指南，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，基于初始心脏受累情况及其进展的程序建议也很重要。患有冠状动脉瘤的患者需要终身持续且不间断的心脏监测。
