Servicio de Inmunología Clínica, Instituto Nacional de Pediatría, Insurgentes Sur 3700-C Col. Insurgentes Cuicuilco, Mexico D.F., Mexico.
Eur J Pediatr. 2013 Mar;172(3):337-42. doi: 10.1007/s00431-012-1879-1. Epub 2012 Nov 15.
Recently, there have been increasing reports of severe forms of Kawasaki disease (KD) associated with shock that have been managed in pediatric intensive care units. It has been suggested that KD is more severe in the Hispanic population. We conducted a study to determine the frequency of Kawasaki disease shock syndrome (KDSS) in our population and compared characteristics between patients with KD without shock and patients with KDSS. Data from 214 patients with KD treated in a tertiary pediatric hospital were collected during a 12-year period. We compared clinical and laboratory features of KD patients without shock and KDSS patients. Of 214 consecutive patients with KD, 11 (5 %) met the definition for KDSS. All of these patients received fluid resuscitation, seven (64 %) required inotropic treatment, and six (54 %), ventilatory support. On admission, seven of these patients (64 %) had an incomplete presentation of the disease, whereas in the group of patients without shock, the relative frequency of an incomplete presentation was 29 %. Twenty percent (3/11) of patients with KDSS presented giant coronary aneurysms versus none of 203 KD patients without shock (p = 0.001); myocardial infarction, 27 % (3/11), versus 1 % (2/203) (p = 0.001); and intravenous immunoglobulin (IVIG) resistance, 60 % (6/11), versus 12 % (24/203). Gastrointestinal manifestations in the acute phase occurred in 91 % of KDSS patients versus 30 % patients without shock (p = 0.001).
Patients with KD presenting in shock seem to have an increase in gastrointestinal manifestations, incomplete presentation, IVIG resistance, and worse cardiac outcomes. Larger, prospective, multicentre studies should be carried out to corroborate these findings.
最近,有越来越多的关于与休克相关的严重川崎病(KD)的报告,这些病例都在儿科重症监护病房中进行管理。有人认为,西班牙裔人群中的 KD 更为严重。我们进行了一项研究,以确定我们人群中川崎病休克综合征(KDSS)的频率,并比较了无休克的 KD 患者和 KDSS 患者的特征。在 12 年期间,我们从一家三级儿科医院治疗的 214 例 KD 患者中收集了数据。我们比较了无休克的 KD 患者和 KDSS 患者的临床和实验室特征。在 214 例连续 KD 患者中,有 11 例(5%)符合 KDSS 的定义。所有这些患者均接受了液体复苏,7 例(64%)需要使用正性肌力药物治疗,6 例(54%)需要呼吸机支持。在入院时,这些患者中有 7 例(64%)存在疾病的不完全表现,而在无休克组中,不完全表现的相对频率为 29%。KDSS 组中有 20%(3/11)的患者出现巨大冠状动脉瘤,而无休克的 203 例 KD 患者中无一例出现(p=0.001);心肌梗死,27%(3/11),而无休克的 203 例 KD 患者中为 1%(2/203)(p=0.001);静脉注射免疫球蛋白(IVIG)抵抗,60%(6/11),而无休克的 203 例 KD 患者中为 12%(24/203)。KDSS 患者在急性期有 91%出现胃肠道表现,而无休克的患者为 30%(p=0.001)。
出现休克的 KD 患者似乎有更多的胃肠道表现、不完全表现、IVIG 抵抗和更差的心脏结局。应该进行更大规模的、前瞻性的、多中心研究来证实这些发现。