Newburger Jane W
Department of Cardiology, Boston Children's Hospital, Department of Pediatrics, Harvard Medical School, Boston, Massachusetts.
Congenit Heart Dis. 2017 Sep;12(5):633-635. doi: 10.1111/chd.12498. Epub 2017 Jun 5.
Kawasaki disease is an acute febrile arteritis of childhood that can result in coronary artery aneurysms if untreated in the first 10 and ideally 7 days of illness. Kawasaki disease begins as a necrotizing arteritis with neutrophilic infiltrate, followed by subacute/chronic changes and luminal myofibroblastic proliferation that can cause coronary artery stenosis. Manifestations include the presence of ≥5 days of fever, together with clinical criteria of extremity changes, rash, conjunctivitis, oral changes, and unilateral cervical lymphadenopathy. Echocardiography should be performed at the time of diagnosis, then 1-2 weeks and 4-6 weeks later, with more frequent studies in individuals with coronary artery dilation or persistent fever. Coronary artery dimensions are characterized both as z-scores and absolute measurements, and coronary architecture evolves over time in children who have aneurysms in the first weeks of illness. Systematic follow-up and therapies are tailored to the degree of coronary disease and to coronary ischemia.
川崎病是一种儿童急性发热性动脉炎，如果在发病的前10天（理想情况下是7天）内未得到治疗，可能会导致冠状动脉瘤。川崎病最初表现为伴有中性粒细胞浸润的坏死性动脉炎，随后出现亚急性/慢性改变以及管腔内肌成纤维细胞增殖，这可能导致冠状动脉狭窄。其表现包括发热≥5天，同时伴有肢体变化、皮疹、结膜炎、口腔改变和单侧颈淋巴结肿大等临床标准。诊断时应进行超声心动图检查，然后在1 - 2周和4 - 6周后复查，对于有冠状动脉扩张或持续发热的患者应更频繁地进行检查。冠状动脉尺寸通过z评分和绝对测量值来表征，在发病最初几周出现动脉瘤的儿童中，冠状动脉结构会随时间演变。系统的随访和治疗方案是根据冠状动脉疾病的程度以及冠状动脉缺血情况量身定制的。
