Journal
EUROPEAN JOURNAL OF PEDIATRICS
Volume 180, Issue 7, Pages 2019-2034Publisher
SPRINGER
DOI: 10.1007/s00431-021-03993-5
Keywords
PIMS-TS; MIS-C; COVID-19; SARS-CoV-2
Categories
Funding
- VIB Grand Challenge Programs
- Research Foundation Flanders (FWO)
- Ghent University research grant (BOF-UGent)
- VIB Grand Challenges Programs
- Jeffrey Modell Foundation
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PIMS-TS/MIS(-C) is a severe, heterogeneous disease with epidemiological enrichment for males, adolescents, and racial and ethnic minorities. Despite its severe presentation, overall short-term outcome is good. The WHO MIS definition is preferred, as it is more precise, while encompassing most cases.
An association between a novel pediatric hyperinflammatory condition and SARS-CoV-2 was recently published and termed pediatric inflammatory multisystem syndrome, temporally associated with SARS-CoV-2 (PIMS-TS) or multisystem inflammatory syndrome (in children) (MIS(-C)). We performed a systematic review and describe the epidemiological, clinical, and prognostic characteristics of 953 PIMS-TS/MIS(-C) cases in 68 records. Additionally, we studied the sensitivity of different case definitions that are currently applied. PIMS-TS/MIS(-C) presents at a median age of 8 years. Epidemiological enrichment for males (58.9%) and ethnic minorities (37.0% Black) is present. Apart from obesity (25.3%), comorbidities are rare. PIMS-TS/MIS(-C) is characterized by fever (99.4%), gastrointestinal (85.6%) and cardiocirculatory manifestations (79.3%), and increased inflammatory biomarkers. Nevertheless, 50.3% present respiratory symptoms as well. Over half of patients (56.3%) present with shock. The majority of the patients (73.3%) need intensive care treatment, including extracorporal membrane oxygenation (ECMO) in 3.8%. Despite severe disease, mortality is rather low (1.9%). Of the currently used case definitions, the WHO definition is preferred, as it is more precise, while encompassing most cases. Conclusion: PIMS-TS/MIS(-C) is a severe, heterogeneous disease with epidemiological enrichment for males, adolescents, and racial and ethnic minorities. However, mortality rate is low and short-term outcome favorable. Long-term follow-up of chronic complications and additional clinical research to elucidate the underlying pathogenesis is crucial. What is Known: A novel pediatric inflammatory syndrome with multisystem involvement has been described in association with SARS-CoV-2. To date, the scattered reporting of cases and use of different case definitions provides insufficient insight in the full clinical spectrum, epidemiological and immunological features, and prognosis. What is New: This systematic review illustrates the heterogeneous spectrum of PIMS-TS/MIS(-C) and its epidemiological enrichment for males, adolescents, and racial and ethnic minorities. Despite its severe presentation, overall short-term outcome is good. The WHO MIS definition is preferred, as it is more precise, while encompassing most cases.
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