Abstract
IMPORTANCE: Kawasaki disease shock syndrome (KDSS) is a rare Kawasaki disease (KD) manifestation. The pediatricians are not aware of the full range of clinical characteristics of KDSS. OBJECTIVE: We aimed to investigate the clinical features, diagnosis and treatment of KDSS in two patients and we included a literature review. METHODS: We collected and analyzed the clinical data for two patients with KDSS. Additionally, using "Kawasaki diseases shock syndrome" as a key phrase, we searched PubMed, Biotechnology Information and Wanfang Data Knowledge Service Platform databases for any similar reports between January 2009 and March 2017. RESULTS: Both of our patients diagnosed with KD developed sustained hypotension during the course of intravenous immunoglobulin treatment, as well as hypoalbuminemia, and increased C-reactive protein and brain natriuretic peptide levels during hypotension. Both patients responded well to fluid resuscitation and inotropic support. No aneurysms formed in either patient during follow-up. We reviewed two related studies in Chinese and 11 studies in English. INTERPRETATION: KD may present with severe shock, and requires proper diagnosis and rapid treatment. The prognosis for most patients with KDSS is excellent.