Moussa Taha, Wagner-Weiner Linda
Pediatr Ann. 2019 Oct 1;48(10):e400-e405. doi: 10.3928/19382359-20190919-01.
Kawasaki disease (KD) is a vasculitis syndrome of unknown etiology, affecting medium-sized vessels and occurring primarily in young children. It is a self-limited illness classically presenting with fever, conjunctivitis, mucocutaneous lesions, and cervical adenopathy. However, vasculitis of coronary arteries with aneurysm formation may occur, leading to morbidity and rarely mortality. Prompt diagnosis of KD is essential as early treatment with intravenous immune globulin (IVIG) decreases the occurrence and severity of coronary vasculitis. This article reviews the clinical characteristics of KD, laboratory assessment, echocardiogram findings, and recommended initial medical therapy. Complications of KD are discussed, including KD refractory to IVIG, the presence of shock or macrophage activation syndrome (MAS), and short/long-term cardiac sequelae. The medical management of these complications is reviewed. Although improved treatment of KD has resulted in a decrease of coronary artery vasculitis over the past 3 decades, KD remains the most common cause of acquired heart disease in children. [Pediatr Ann. 2019;48(10):e400-e405.].
川崎病（KD）是一种病因不明的血管炎综合征，主要累及中等大小血管，好发于幼儿。它是一种自限性疾病，典型表现为发热、结膜炎、皮肤黏膜病变和颈部淋巴结肿大。然而，可能会发生伴有动脉瘤形成的冠状动脉血管炎，导致发病，甚至罕见情况下会导致死亡。及时诊断KD至关重要，因为早期静脉注射免疫球蛋白（IVIG）治疗可降低冠状动脉血管炎的发生率和严重程度。本文综述了KD的临床特征、实验室评估、超声心动图表现以及推荐的初始药物治疗。还讨论了KD的并发症，包括对IVIG难治的KD、休克或巨噬细胞活化综合征（MAS）的存在以及短期/长期心脏后遗症。并对这些并发症的药物治疗进行了综述。尽管在过去30年中，KD治疗的改善使冠状动脉血管炎有所减少，但KD仍然是儿童后天性心脏病的最常见原因。[《儿科年鉴》。2019年；48(10):e400 - e405。]
