Table 1. MIS-C definition by KDCA [2], principal features of severe KD [11,15], and clinical manifestations of our patient

MIS-C (KDCA) Severe KD (e.g., KDSS or refractory KD) Our patient
Demographic criteria
 1. Children and adolescents aged <19 years Children and adolescents with KD 5-year-old boy
 2. Documented or subjective fever (≥ 38.0 ℃) for >24 hours Fever unresponsive to initial IVIG therapy Prolonged fever (for 5 days)
 3. Clinical severity requiring hospitalization or resulting in death Hospital stays longer than typical KD ICU management
Clinical and laboratory criteria (In KDSS)
 4. Systemic inflammation: CRP ≥ 3.0 mg/dL (normal < 0.5) CRP ≥ 7.0 mg/dL (92%) CRP 6.7 mg/dL
 5. Organ dysfunction: ≥ 2 of 5 categories Multi-organ dysfunction in most cases Met 3 of 5 categories
  ① Shock: clinician documentation in medical records Shock (100%) Shock with vasoactive drugs
  ② Cardiovascular: EF < 55%, CAAs, or Tn elevation EF < 50% (45%) and CAAs (73%) Myocarditis (EF 45%) and CAAs (Z score 2.3)
  ③ Hematologic: platelet count < 150,000 /μL or ALC < 1,000 /μL Thrombocytopenia (80%)
  ④ Gastrointestinal: abdominal pain, vomiting, or diarrhea Abdominal pain (43%) and vomiting (59%)
  ⑤ Mucocutaneous: KD-like features Mucocutaneous (77%) Complete KD (5 of 5 clinical features)
Microbiological criteria
 6. Evidence of SARS-CoV-2 infection within 60 days
  ① Detection of SARS-CoV-2 RNA (i.e., PCR) or specific Ag/Ab Co-existing infections in 1/3 of KD cases SARS-CoV-2 Ab 164 U/L (normal < 1.0)
  ② Close contact with a confirmed or probable COVID-19 case For example, EBV- or IFV-associated KD
 7. Absence of a more likely alternative diagnosis No significant pathogens in laboratory tests
MIS-C: multisystem inflammatory syndrome in children, KDCA: Korea Disease Control Agency, KD: Kawasaki disease, IVIG: intravenous immunoglobulin; KDSS: Kawasaki disease shock syndrome; CRP: C-reactive protein, EF: ejection fraction, CAAs: coronary artery abnormalities, Tn: troponin, ALC: absolute lymphocyte count, SARS-CoV-2: severe acute respiratory syndrome coronavirus 2, PCR: polymerase chain reaction, Ag: antigen, Ab: antibody, EBV: Epstein-Barr virus, IFV: influenza virus, –: absent or not available, COVID-19: coronavirus disease 2019.