Joshi Manjiri, Tulloh Robert
Department of Cardiology, University of Bristol & Bristol Royal Hospital for Children, Upper Maudlin Street, Bristol BS2 8BJ, UK.
Future Cardiol. 2017 Sep;13(5):491-501. doi: 10.2217/fca-2017-0039. Epub 2017 Aug 22.
Kawasaki disease is an acute, systemic vasculitis of childhood and confers a 25% risk of developing coronary artery aneurysms. Its etiology is unknown, but the incidence is increasing rapidly with linked gene polymorphisms having been identified. A constellation of symptoms, epidemics and seasonality all implicate an unidentified infective or environmental cause. Intravenous immunoglobulin therapy, aspirin and steroids all form the mainstay of acute treatment and reduces the incidence of coronary artery aneurysms if given before 7 days. However, in some, these lesions persist and require ongoing management during follow-up during childhood and into adult life. Evidence for further investigations in order to minimize complications is presented in order to minimize the myofibroblast proliferation and stenosis in the long term.
川崎病是一种儿童期急性全身性血管炎,有25%的风险会发展为冠状动脉瘤。其病因不明,但随着已鉴定出相关基因多态性,发病率正在迅速上升。一系列症状、流行情况和季节性都暗示存在未明确的感染性或环境因素。静脉注射免疫球蛋白治疗、阿司匹林和类固醇都是急性治疗的主要手段,若在7天前给予,可降低冠状动脉瘤的发生率。然而,在一些患者中,这些病变会持续存在,在儿童期及成年后的随访期间都需要持续管理。为尽量减少并发症而进行进一步研究的证据表明,从长远来看,这样做可尽量减少肌成纤维细胞增殖和狭窄。