Kawasaki disease shock syndrome: Unique and severe subtype of Kawasaki disease
Kawasaki disease shock syndrome: Unique and severe subtype of Kawasaki disease
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DOI：
10.1111/ped.13614
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发表时间：
2018-09-01
影响因子：
1.4
通讯作者：
Yamazaki-Nakashimada, Marco
中科院分区：
文献类型：
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作者：
Berenise Gamez-Gonzalez, Luisa;Moribe-Quintero, Isabel;Yamazaki-Nakashimada, Marco
背景川崎病休克综合征（KDSS）是川崎病（KD）的一种罕见表现。 KDSS 与更严重的炎症、冠状动脉异常和静脉注射标志物有关。免疫球蛋白（IVIG）抵抗。方法对两家医院的 KDSS 儿童进行回顾性、描述性研究。收集有关川崎病和休克的相关文章，并在可获得时对人口统计数据、临床表现、实验室变量、超声心动图结果、治疗和特殊特征进行分析。对墨西哥两个中心诊断为 KDSS 的 12 例患者以及文献中的 91 例进行回顾性分析。结果 72 例患者表现为完全 KD (69.9%)，30.1% (31/103) 具有不寻常的 KD 表现。入院时最常见的诊断是中毒性休克综合征（TSS；n = 20）。 20 人中有 16 人患有冠状动脉异常。总体而言，65% 的患者有冠状动脉异常。死亡率为6.8%。结论冠状动脉瘤的存在与男性、IVIG抵抗、正性肌力药物治疗、心力衰竭、腹痛和神经症状呈显着正相关。 IVIG 耐药患者的中性粒细胞：淋巴细胞比率较高。几乎所有患者均出现腹部症状、低蛋白血症和C反应蛋白升高。 KDSS 中多系统参与的非典型表现很常见。一个重要的鉴别诊断是 TSS。机械通气、胃肠道和神经系统症状与 IVIG 抵抗和冠状动脉瘤的存在相关。一线治疗包括 IVIG 和脉冲皮质类固醇；在严重的情况下，英夫利昔单抗、阿那白滞素、环孢素或血浆置换是替代治疗选择。
BackgroundKawasaki disease shock syndrome (KDSS) is an uncommon presentation of Kawasaki disease (KD). KDSS has been associated with more severe markers of inflammation, coronary abnormalities and i.v. immunoglobulin (IVIG) resistance.MethodsA retrospective, descriptive study of children with KDSS in two hospitals was performed. Relevant articles about KD and shock were collected, and demographic data, clinical presentation, laboratory variables, echocardiogram findings, treatment and special features were analyzed when available. Twelve patients diagnosed with KDSS were retrospectively reviewed from two centers in Mexico, along with 91 additional cases from the literature.ResultsSeventy-two patients presented with complete KD (69.9%), and 30.1% (31/103) had unusual KD manifestations. The most frequent diagnosis at the time of admission was toxic shock syndrome (TSS; n = 20). Sixteen of the 20 had coronary artery abnormalities. Overall, abnormalities in the coronary arteries were documented in 65% of the patients. The mortality rate was 6.8%.ConclusionThe presence of coronary aneurysms was significantly and positively correlated with male gender, IVIG resistance, inotrope treatment, cardiac failure, abdominal pain and neurological symptoms. IVIG-resistant patients had higher neutrophil : lymphocyte ratio. Abdominal symptoms, hypoalbuminemia and elevated C-reactive protein were present in almost all of the patients. Multisystem involvement with atypical presentation in KDSS is frequent. An important differential diagnosis is TSS. Mechanical ventilation, gastrointestinal and neurological symptoms were associated with IVIG resistance and the presence of coronary aneurysms. The first line of treatment includes IVIG and pulse corticosteroids; in severe cases, infliximab, anakinra, cyclosporine or plasmapheresis are alternative treatment options.