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Cardiology in the Young ; 32(Supplement 2):S56-S57, 2022.
Article in English | EMBASE | ID: covidwho-2062107


Background and Aim: The considerable overlap in case definition and clinical features between patients with COVID-19 associated Multisystem Inflammatory Syndrome in Children (MIS-C) and Kawasaki disease (KD) suggests shared pathogenesis. We sought to compare demographic, clinical presentation, management and outcomes of patients by COVID-19 status. Method(s): The International KD Registry (IKDR) began enrolling patients with clinical features of either acute MIS-C or KD or fever with hyperinflammation beginning in January 2020. The IKDR is unique regarding broad patient selection and includes sites from North, Central and South America, Europe, Asia and the Middle East. Patient groups stratified by COVID-19 status were compared. Result(s): As of October 6, 2021, 1330 patients were registered from 31 sites. COVID status was POSITIVE for 59% (confirmed household COVID-19 contact and/or positive SARS-CoV-2 PCR or serology), POSSIBLE for 4% (suggestive clinical features but some negative tests or absent exposure), NEGATIVE for 23%, and UNKNOWN (no known exposure and testing not com-pleted) for 14% (TABLE). Most of the UNKNOWN patients were from early in the COVID-19 pandemic before MIS-C was defined and before COVID-19 serologic testing was widely used. POSITIVE and POSSIBLE patients were older, had fewer KD clinical criteria, greater gastrointestinal symptoms, were more likely to present with shock and require ICU admission and inotropic support. POSSIBLE patients had greater days from symptom onset to first immune modulation treatment, with no differences between groups regarding days from admission to first treatment. Most patients in each group received intravenous immune globu-lin, with POSITIVE and POSSIBLE patients more likely to have received steroids and anakinra. NEGATIVE and UNKNOWN patients had higher maximal coronary artery Z scores, with a trend to having higher categories of aneurysm involvement. Conclusion(s): While there was considerable overlap in presentation, management and outcomes between COVID-19 POSITIVE/POSSIBLE (presumed MIS-C) and COVID NEGATIVE/UNKNOWN patients (presumed KD), COVID-19 POSITIVE/POSSIBLE patients had more severe presentations and required more intensive management, although coronary artery outcomes trended to be less severe. Patient recruitment con-tinues, and in-depth comparison of laboratory features and appli-cation of machine learning approaches to patient differentiation and prediction of optimal management pathways are forthcoming.

Open Forum Infectious Diseases ; 7(SUPPL 1):S338, 2020.
Article in English | EMBASE | ID: covidwho-1185903


Background: Background: Multi-system Inflammatory Syndrome of Children (MIS-C) has recently emerged internationally as a serious inflammatory complication of SARS-CoV-2 infection with significant morbidity for the pediatric population. Methods: This observational retrospective cohort study includes 33 children meeting CDC criteria for MIS-C treated between March 15 and June 17, 2020 at Children's National Hospital in Washington DC. Clinical and demographic data were extracted from medical records and are summarized. Results: Of 33 hospitalized MIS-C patients, 42% were critically ill, and 58% were non-critically ill. The median age was 8.9 years (0.7-18.7 years). More males (58 %) than females (43 %) were represented in the MIS-C cohort. The majority (75%) of children had no underlying medical condition. Criteria for incomplete or complete Kawasaki Disease (KD) were present in 39% of patients, while an additional 9% had some features of KD. However the remaining 52% of MIS-C patients presented with other sub-phenotypes including prominent severe abdominal pain and/or nonspecific multiorgan dysfunction. 30% presented with shock requiring volume and/or inotropic support. SARS-CoV-2 antibodies were present in 61% of patients. Virus was detectable by PCR in 36% of patients. At the time of initial evaluation, 39% (13/33) of children had identified cardiac abnormalities including myocardial dysfunction (5/33;15%), coronary ectasia (4/33;12%), coronary aneurysm (3/33;9%), or pericardial effusion 5/33;15%) either alone or in combination. Cytokine profiling identified elevation of several cytokines in this cohort, including IL-6. Treatment has included intravenous immunoglobulin, aspirin, anakinra and other immunomodulatory therapies, with overall rapid response to therapy. No deaths have occurred. Conclusion: The emergence of MIS-C late in the surge of SARS-CoV-2 circulation in the Washington DC metropolitan region has added to the already significant burden of hospitalized and critically ill children in our region. A significant percentage of these children present with cardiac dysfunction and abnormalities, whether or not with KD features at presentation. Detailed characterization of immune responses and long term outcome of these patients is a priority.