Wood L E, Tulloh R M R
Bristol Royal Hospital for Children, Bristol, UK.
Heart. 2009 May;95(10):787-92. doi: 10.1136/hrt.2008.143669. Epub 2008 Aug 12.
Kawasaki disease (KD) is an acute self-limiting systemic vasculitis of unknown aetiology. It is the most common cause of acquired heart disease in young children. The intense inflammatory process has a predilection for the coronary arteries, resulting in the development of aneurysmal lesions, arterial thrombotic occlusion or, potentially, sudden death. There is no specific diagnostic test; however, treatment with immunoglobulin and aspirin effectively reduces cardiac complications from 25% to 4.7% in the UK. Inflammation of the myocardium, endocardium or pericardium can occur early in the disease and endothelial dysfunction along with abnormalities of myocardial blood flow may require continuing medication, interventional catheterisation or even cardiac surgery. Several new pharmacological treatments may have important roles to play in managing KD in children and adolescents. This review discusses the history of the disease, the diagnostic challenges, epidemiology, aetiology, pathology, immunopathogenesis, treatment, genetic influences and the long-term cardiovascular sequelae.
川崎病（KD）是一种病因不明的急性自限性全身性血管炎。它是幼儿后天性心脏病的最常见病因。强烈的炎症过程易累及冠状动脉，导致动脉瘤样病变、动脉血栓性闭塞，甚至可能导致猝死。目前尚无特异性诊断检测方法；然而，在英国，使用免疫球蛋白和阿司匹林进行治疗可有效将心脏并发症的发生率从25%降至4.7%。在疾病早期可能会发生心肌、心内膜或心包的炎症，内皮功能障碍以及心肌血流异常可能需要持续药物治疗、介入导管插入术甚至心脏手术。几种新的药物治疗方法可能在儿童和青少年川崎病的管理中发挥重要作用。本综述讨论了该疾病的历史、诊断挑战、流行病学、病因、病理、免疫发病机制、治疗、遗传影响以及长期心血管后遗症。
