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. 2020 Jun 8;324(3):259–269. doi: 10.1001/jama.2020.10369

Table 1. Case Definitions for Emerging Inflammatory Condition During COVID-19 Pandemic From the World Health Organization, Royal College of Paediatrics and Child Health, and Centers for Disease Control and Prevention.

World Health Organization8 Royal College of Paediatrics and Child Health (United Kingdom)7 Centers for Disease Control and Prevention (United States)9
  • Children and adolescents 0-19 y of age with fever >3 d AND 2 of the following:

    • 1. Rash or bilateral nonpurulent conjunctivitis or mucocutaneous inflammation signs (oral, hands, or feet)

    • 2. Hypotension or shock

    • 3. Features of myocardial dysfunction, pericarditis, valvulitis, or coronary abnormalities (including ECHO findings or elevated troponin/NT-proBNP)

    • 4. Evidence of coagulopathy (by PT, APTT, elevated D-dimers)

    • 5. Acute gastrointestinal problems (diarrhea, vomiting, or abdominal pain)

  • AND

  • Elevated markers of inflammation such as ESR, CRP, or procalcitonin.

  • AND

  • No other obvious microbial cause of inflammation, including bacterial sepsis, staphylococcal or streptococcal shock syndromes.

  • AND

  • Evidence of COVID-19 (RT-PCR, antigen test, or serology positive), or likely contact with patients with COVID-19

    • Consider this syndrome in children with features of typical or atypical Kawasaki disease or toxic shock syndrome

  • A child presenting with persistent fever, inflammation (neutrophilia, elevated CRP, and lymphopenia) and evidence of single or multiorgan dysfunction (shock, cardiac, respiratory, kidney, gastrointestinal, or neurological disorder) with additional features (see listed in eAppendix in Supplement 2). This may include children fulfilling full or partial criteria for Kawasaki diseasea

  • Exclusion of any other microbial cause, including bacterial sepsis, staphylococcal or streptococcal shock syndromes, infections associated with myocarditis such as enterovirus (waiting for results of these investigations should not delay seeking expert advice)

  • SARS-CoV-2 PCR test results may be positive or negative

  • An individual aged <21 y presenting with fever, laboratory evidence of inflammation, and evidence of clinically severe illness requiring hospitalization, with multisystem (>2) organ involvement (cardiac, kidney, respiratory, hematologic, gastrointestinal, dermatologic, or neurological)

    • Fever >38.0 °C for ≥24 h or report of subjective fever lasting ≥24 h

    • Laboratory evidence including, but not limited to, ≥1 of the following: an elevated CRP level, ESR, fibrinogen, procalcitonin, D-dimer, ferritin, lactic acid dehydrogenase, or IL-6; elevated neutrophils; reduced lymphocytes; and low albumin

  • AND

  • No alternative plausible diagnoses

  • AND

  • Positive for current or recent SARS-CoV-2 infection by RT-PCR, serology, or antigen test; or COVID-19 exposure within the 4 wk prior to the onset of symptoms

  • Additional comments

    • Some individuals may fulfill full or partial criteria for Kawasaki disease but should be reported if they meet the case definition for MIS-C

    • Consider MIS-C in any pediatric death with evidence of SARS-CoV-2 infection

Abbreviations: APTT, activated partial thromboplastin time; COVID-19, coronavirus disease 2019; CRP, C-reactive protein; ECHO, echocardiography; ESR, erythrocyte sedimentation rate; MIS-C, multisystem inflammatory syndrome in children; NT-proBNP, N-terminal pro–B-type natriuretic peptide; PT, prothrombin time; RT-PCR, reverse transcriptase–polymerase chain reaction; SARS-CoV-2, severe acute respiratory syndrome coronavirus 2.

a

Criteria for Kawasaki disease include persistent fever and 4 of 5 principal clinical features: erythema and cracking of lips, strawberry tongue, and/or erythema of oral and pharyngeal mucosa; bilateral bulbar conjunctival injection without exudate; rash (maculopapular, diffuse erythroderma); erythema and edema of the hands and feet and/or periungual desquamation; and cervical lymphadenopathy.