Dietz Sanne M, Kuipers Irene M, Tacke Carline E A, Koole Jeffrey C D, Hutten Barbara A, Kuijpers Taco W
Department of Pediatric Hematology, Immunology and Infectious Diseases, Emma Children's Hospital, Academic Medical Centre, Amsterdam, The Netherlands.
Department of Pediatric Cardiology, Emma Children's Hospital, Academic Medical Centre, Amsterdam, The Netherlands.
J Cardiol. 2017 Oct;70(4):359-365. doi: 10.1016/j.jjcc.2016.12.014. Epub 2017 Mar 18.
Kawasaki disease (KD) is a pediatric vasculitis of unknown origin. Its main complication is the development of coronary artery aneurysms (CAA) with giant CAA at the end of the spectrum.
In this cohort study, we evaluated the association between patient characteristics and the development of giant CAA based on z-scores. Multivariable, multinomial logistic regression analysis was used to identify variables associated with giant CAA.
A total of 301 KD patients, comprising 216 patients without enlargement, 45 with small-sized, 19 with medium-sized, and 21 with giant CAA with all echocardiographies at our center were retrospectively included. Remarkably, 95% of patients with giant CAA were boys. In addition to 'no/late intravenous immunoglobulin (IVIG) treatment', 'male gender' (OR 16.23, 95% CI 1.88-140.13), 'age<1 year' (OR 7.49, 95% CI 2.29-24.46), and 'IVIG re-treatment (9.79, 95% CI 2.79-34.37)' were significantly associated with an increased risk of giant CAA, with patients without enlargement as reference. Compared to patients with medium-sized CAA, 'IVIG re-treatment' was significantly associated with giant CAA. The majority of giant CAA continued to increase in size during the first 40 days.
We identified risk factors associated with an increased risk of giant CAA. The difference in variables between the giant CAA group and the other CAA subgroups suggests a separation between patients with the treatment-resistant giant CAA and the other IVIG-responsive patients, in which gender may be factored as a most relevant genetic trait. The increase in size during the first 2 months indicates the need for repeated echocardiography.
川崎病（KD）是一种病因不明的儿童血管炎。其主要并发症是冠状动脉瘤（CAA）的形成，在疾病谱的末端为巨大冠状动脉瘤。
在这项队列研究中，我们基于z评分评估了患者特征与巨大冠状动脉瘤发生之间的关联。采用多变量多项逻辑回归分析来确定与巨大冠状动脉瘤相关的变量。
我们回顾性纳入了本中心301例川崎病患者，其中216例无冠状动脉扩张，45例有小型冠状动脉瘤，19例有中型冠状动脉瘤，21例有巨大冠状动脉瘤，所有患者均进行了超声心动图检查。值得注意的是，95%的巨大冠状动脉瘤患者为男性。除了“未进行/延迟静脉注射免疫球蛋白（IVIG）治疗”外，“男性”（比值比16.23，95%置信区间1.88 - 140.13）、“年龄<1岁”（比值比7.49，95%置信区间2.29 - 24.46）和“IVIG再次治疗”（9.79，95%置信区间2.79 - 34.37）与巨大冠状动脉瘤风险增加显著相关，以无冠状动脉扩张的患者作为对照。与中型冠状动脉瘤患者相比，“IVIG再次治疗”与巨大冠状动脉瘤显著相关。大多数巨大冠状动脉瘤在最初40天内继续增大。
我们确定了与巨大冠状动脉瘤风险增加相关的危险因素。巨大冠状动脉瘤组与其他冠状动脉瘤亚组之间变量的差异表明，治疗抵抗性巨大冠状动脉瘤患者与其他IVIG反应性患者之间存在区别，其中性别可能是最相关的遗传特征因素。最初2个月内的增大表明需要重复进行超声心动图检查。
