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Recurrent Kawasaki Disease: A Case Report of Three Separate Episodes at 4-Year Intervals

机译:复发性川崎病:每隔4年间隔发生3次独立发作的病例报告

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摘要

Kawasaki disease (KD) is a self-limited systemic vasculitis, most often occurring in children 1–5 years old. It has a 2% recurrence rate and is associated with coronary aneurysms (CA), which can develop within two weeks of onset. A 25% increased risk is noted in patients who are recalcitrant to treatment. We describe a patient with recurrence of KD three times, approximately four years apart. A 10-year-old female with two previous episodes of KD, at 11 months and five years of age), in which she met five out of five criteria for KD and had no coronary involvement, presented with 15 days of fever, conjunctivitis and mucocutaneous changes. Infectious work-up was negative, and she was diagnosed with incomplete KD meeting three out of five criteria. An echocardiogram (ECHO) on day 12 revealed dilation of the right coronary artery (RCA) and left coronary artery (LCA). Treatment with intravenous immunoglobulin (IVIG) and high-dose aspirin was started at an outside hospital. After transfer, serial ECHOs showed evolving coronary aneurysms, left anterior descending (LAD) z-score + 8.2 and RCA z-score + 4.0. She received 10 mg/kg infliximab (day 18) and began clopidogrel. A cardiac MRI (day 20) demonstrated progression of the LAD aneurysm, with a z-score + 13, and warfarin was started. To our knowledge, this is the first report of recurrent KD occurring three times at ~5 year intervals.
机译:川崎病(KD)是一种自限性系统性血管炎,最常见于1-5岁的儿童中。它具有2%的复发率,并与冠状动脉瘤(CA)相关,后者可在发病后两周内发展。顽固治疗的患者风险增加了25%。我们描述了3次KD复发的患者,大约间隔4年。一位10岁的女性,在11个月大和5岁时有两次KD发作,其中她满足了五分之五的KD标准,并且没有冠状动脉受累,伴有15天发烧,结膜炎和皮肤粘膜变化。传染病检查结果为阴性,她被诊断出不符合五分之三的KD。第12天的超声心动图(ECHO)显示右冠状动脉(RCA)和左冠状动脉(LCA)扩张。静脉注射免疫球蛋白(IVIG)和大剂量阿司匹林的治疗始于一家外部医院。转移后,连续的ECHO显示出正在发展的冠状动脉瘤,左前降支(LAD)z评分+ 8.2,RCA z评分+ 4.0。她接受了10 mg / kg英夫利昔单抗(第18天),开始服用氯吡格雷。心脏MRI(第20天)显示LAD动脉瘤进展,z评分+ 13,并开始使用华法林。据我们所知,这是每5年间隔出现3次复发性KD的第一份报告。

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