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Abstract：
Kawasaki disease is an acute self-limiting systemic vasculitis in childhood, resulting in arterial swelling or inflammation and eventually leading to cardiovascular problems, such as coronary artery aneurysms. Based on previous studies, serum sodium ≤133 mmol/L, albumin ≤3.2 g/dL, alanine transaminase ≥80 U/L, and neutrophil percentage ≥80% at diagnosis are risk factors for intravenous immunoglobulin (IVIg). However, the prevalence of resistance to Ig among children with Kawasaki disease varies among different countries due to diversity in evaluation, treatment, and diagnosis. Approximately, 10% to 20% of patients have IVIg-resistant Kawasaki disease. As the probability of coronary artery damage associated with IVIg-resistant Kawasaki disease is higher than that with IVIg-sensitive Kawasaki disease, the early detection and appropriate treatment of IVIg-resistant Kawasaki disease can decrease the probability of damage to coronary arteries and hospital lengths of stay and cost. Kawasaki disease in early infancy is uncommon, and sometimes it occurs with thrombosis and peripheral gangrene. A positive genetic background may play a role in susceptibility to thrombosis. We herein describe a patient suffering from an IVIg-resistant Kawasaki disease with severe coronary artery thrombosis and positive genetic mutation. Medical treatment resolved the thrombosis, but the coronary arteries remained dilated.
摘要：
川崎病是一种儿童期急性自限性系统性血管炎,导致动脉肿胀或炎症，最终导致心血管问题，如冠状动脉瘤。根据以前的研究，血清钠≤133mmol/L，白蛋白≤3.2g/dL，丙氨酸转氨酶≥80U/L,诊断时中性粒细胞百分比≥80%是静脉注射免疫球蛋白(IVIg)的危险因素.然而,由于评估的多样性，川崎病儿童对Ig的耐药性在不同国家之间有所不同，治疗,和诊断。大约,10％至20％的患者患有IVIg耐药的川崎病。由于与IVIg抗性川崎病相关的冠状动脉损伤的概率高于IVIg敏感性川崎病,早期发现IVIg抵抗性川崎病并给予适当治疗可降低冠状动脉损伤的概率,降低住院时间和费用.婴儿早期的川崎病并不常见，有时发生血栓形成和周围坏疽。积极的遗传背景可能在血栓形成的易感性中起作用。我们在此描述了患有IVIg抗性川崎病并伴有严重冠状动脉血栓形成和阳性基因突变的患者。药物治疗解决了血栓形成，但冠状动脉仍然扩张.
