Kawasaki disease shock syndrome: Unique and severe subtype of Kawasaki disease

作者：Luisa Berenise Gámez‐González, Isabel Moribe‐Quintero, Martı́n Cisneros-Castólo, Javier Varela Ortiz, Mireya Cleotilde Muñoz-Ramírez, Martin Garrido‐García, Marco Antonio Yamazaki‐Nakashimada · 发表于：Pediatrics International · 年份：2018 · DOI：10.1111/ped.13614 · 被引用次数：130 · 研究领域：Kawasaki Disease and Coronary Complications、Coronary Artery Anomalies、Autoimmune and Inflammatory Disorders Research

BACKGROUND: Kawasaki 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. METHODS: A 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. RESULTS: Seventy-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%. CONCLUSION: The 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...