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OBJECTIVES: This literature review describes the epidemiology, pathophysiology, diagnosis, and management of multisystem inflammatory syndrome in children (MIS-C), assesses economic impacts, and differentiates MIS-C from Kawasaki disease and toxic shock syndrome. METHODS: It was performed in two stages: a search for articles on PubMed followed by a search for documents from government agencies. Ten articles containing information on the epidemiology, pathophysiology, signs and symptoms, complementary evaluations, diagnostic criteria, clinical management and/or outcomes of MIS-C were selected. Duplicate articles, papers focusing on adult patients, publications that did not include description of changes and approach to MIS-C were excluded. RESULTS: Patient mean age ranged from 8.5 to 12 years; male gender was the most affected; most of the sample had positive serology for COVID-19; males accounted for the majority of the cases; most patients tested positive for COVID-19; most patients were admitted to an intensive care unit; mortality was low. Pathophysiology of the disease has not been clearly defined yet. The main symptoms were fever, gastrointestinal symptoms, skin rash, and conjunctivitis. The predominant dysfunctions were shock and respiratory failure. Laboratory tests showed increases in inflammatory markers, procalcitonin, IL-6, troponin, and pro-BNP. The most used treatments were ventilatory support, vasoactive drugs, immunoglobulin, and corticosteroids. CONCLUSIONS: This article allowed a better understanding of MIS-C, despite the lack of studies covering the subject. It described an epidemiological, clinical, workup, and diagnostic profile of the condition. The treatment of the disease still lacks a definitive protocol, although therapies described in most studies were similar. Therefore, further studies and protocols are needed to better understand and treat MIS-C.