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peds.kawasaki.core.v1PRODUCTION
peds.kawasaki.core.v1

Kawasaki disease

pediatricsacutesyndromepediatric
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Encounter flow

12/12 authored

Canonical 12-phase frame with authored status for this dossier.

Current phase

Frame

Detailed

Identify classic KD (fever ≥5d + ≥4 of 5 principal criteria) vs incomplete KD (fever ≥5d + 2-3 criteria + supportive labs/echo) per AHA 2017

Inputs
2
Actions
0
Advance rule
Set
Advance when

classic vs incomplete KD pattern identified

Patient inputs (15)

Peak incidence 6 mo-5 yr; infants <6 mo and >8 yr have higher incomplete-KD + aneurysm risk (AHA 2017 McCrindle)

Fever duration ≥5 days is core criterion (≥4 days if classic features in some 2017 AHA updates)

Principal clinical criterion (AHA 2017 McCrindle)

Strawberry tongue, cracked lips, oropharyngeal erythema — principal criterion (AHA 2017)

Principal criterion (AHA 2017)

Erythema/edema acute -> desquamation subacute — principal criterion (AHA 2017)

Unilateral cervical >=1.5 cm — principal criterion (often only one) (AHA 2017)

>=3 mg/dL supports incomplete KD (AHA 2017 algorithm)

>=40 mm/h supports incomplete KD (AHA 2017 algorithm)

Coronary z-score / aneurysm at diagnosis, 1-2 wk, 6-8 wk (AHA 2017)

IVIG 2 g/kg + ASA 30-50 mg/kg/d (or 80-100 in some protocols) are weight-based (AHA 2017)

Thrombocytosis (>450K) typically appears in subacute phase; can be normal early (AHA 2017)

<=3 g/dL is incomplete-KD supplementary criterion (AHA 2017)

Hepatitis common; supplementary criterion (AHA 2017)

Sterile pyuria supplementary criterion (AHA 2017)

* = hard-required. Engine cannot meaningfully run until these are filled.

Severity triggers (8)

8 need judgement
  • informationallife_threateningKD_shock_syndrome
    KD with hypotension, poor perfusion, ventricular dysfunction, or vasoactive requirement
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationallife_threateninggiant_aneurysm_z_ge_10
    Coronary z-score ≥10 OR absolute diameter ≥8 mm
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalsevereclassic_KD_diagnosis
    Fever ≥5 days + ≥4 of 5 principal criteria
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalsevereincomplete_KD_with_supportive_features
    Fever ≥5 days + 2–3 principal criteria + supportive labs (CRP ≥3, ESR ≥40, ≥3 supplementary)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalsevereIVIG_resistance_persistent_fever
    Persistent fever ≥36 hours after end of first IVIG
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalseverehigh_risk_kobayashi_score
    Kobayashi score ≥5 (or Egami ≥3 / Sano ≥2) at diagnosis (Asian cohort)
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalseverecoronary_aneurysm_at_presentation
    Coronary z-score ≥2.5 at first echo
    Trigger could not be auto-evaluated — needs clinician judgement.
  • informationalsevereMIS_C_overlap_post_COVID
    Suspected MIS-C with KD-like features (post-SARS-CoV-2 exposure 2–6 wk prior)
    Trigger could not be auto-evaluated — needs clinician judgement.

Workflow calculators

This dossier does not reference any calculators.

Recommended regimen

Kawasaki disease — IVIG + ASA → escalation for IVIG-resistance → long-term cardiac surveillance
axis: kawasaki_acute_5stagestep 1 - Stage 1 — IVIG + high-dose aspirin (within 10 days of fever onset, ideally days 5–10)
Selected step "Stage 1 — IVIG + high-dose aspirin (within 10 days of fever onset, ideally days 5–10)" — Classic OR incomplete KD diagnosis confirmed
  • IVIG
    first line
    immunoglobulin
    2 g/kg single dose IV over 10–12 hours • IV • single dose
    triggers: kd_diagnosis_confirmed
    AHA 2017 — primary intervention; reduces coronary aneurysm risk from ~25% to ~4% if given <10 days; still indicated after day 10 if persistent fever or evidence of inflammation
    rxcui 1426680
  • aspirin
    first line
    antiplatelet_anti_inflammatory
    High-dose 80–100 mg/kg/day PO divided q6h (US/AHA) OR 30–50 mg/kg/day (Japan/Europe) • PO • q6h
    triggers: kd_diagnosis_confirmed
    AHA 2017 — anti-inflammatory dose acutely; controversial whether high vs moderate is better; both effective when paired with IVIG
    rxcui 1191

ed playbook — drug actions (3)

  1. 1. antipyretic
    Acetaminophen 15 mg/kg PO q4-6h • PO • q4-6h
    trigger: Fever
    Symptom control (AHA 2017)
  2. 2. IV fluids if KDSS or poor PO
    Maintenance +/- 10-20 mL/kg bolus • IV • as needed
    trigger: Shock or poor intake
    KDSS in ~5% (Kanegaye Pediatrics 2009; AHA 2017)
  3. 3. admit for IVIG + ASA
    IVIG 2 g/kg over 10–12h + ASA 80–100 mg/kg/day ÷ q6h • IV + PO • IVIG single, ASA q6h
    trigger: KD diagnosis confirmed
    AHA 2017

Auto-drafted A&P note

ed

Subjective

- Possible entry pathways: Fever >=5 days in a child (AHA 2017 McCrindle); Conjunctivitis + rash + lip/oral changes + extremity changes + lymphadenopathy (AHA 2017 principal criteria); Markedly elevated CRP/ESR with normocytic anemia + thrombocytosis (AHA 2017 supplementary).

Objective

- No vitals, labs, or imaging entered for this encounter.

Assessment

**Kawasaki disease** (peds.kawasaki.core.v1).
Phenotype framing: Phenotype: classic KD, incomplete KD, KD shock syndrome (KDSS), IVIG-refractory, MIS-C overlap per AHA 2017; rule out viral exanthem, drug reaction, scarlet fever, JIA-systemic
Scope: Identify classic KD (fever ≥5d + ≥4 of 5 principal criteria) vs incomplete KD (fever ≥5d + 2-3 criteria + supportive labs/echo) per AHA 2017

No severity triggers fired against current inputs.

Plan

Regimen axis: **Kawasaki disease — IVIG + ASA → escalation for IVIG-resistance → long-term cardiac surveillance** — step "Stage 1 — IVIG + high-dose aspirin (within 10 days of fever onset, ideally days 5–10)".
1. IVIG 2 g/kg single dose IV over 10–12 hours IV single dose (immunoglobulin, first line) — AHA 2017 — primary intervention; reduces coronary aneurysm risk from ~25% to ~4% if given <10 days; still indicated after day 10 if persistent fever or evidence of inflammation
2. aspirin High-dose 80–100 mg/kg/day PO divided q6h (US/AHA) OR 30–50 mg/kg/day (Japan/Europe) PO q6h (antiplatelet_anti_inflammatory, first line) — AHA 2017 — anti-inflammatory dose acutely; controversial whether high vs moderate is better; both effective when paired with IVIG

Setting playbook (ed) — Recognize KD pattern (classic vs incomplete), expedite admission, initiate IVIG + ASA promptly
3. antipyretic Acetaminophen 15 mg/kg PO q4-6h PO q4-6h — Fever (Symptom control (AHA 2017))
4. IV fluids if KDSS or poor PO Maintenance +/- 10-20 mL/kg bolus IV as needed — Shock or poor intake (KDSS in ~5% (Kanegaye Pediatrics 2009; AHA 2017))
5. admit for IVIG + ASA IVIG 2 g/kg over 10–12h + ASA 80–100 mg/kg/day ÷ q6h IV + PO IVIG single, ASA q6h — KD diagnosis confirmed (AHA 2017)

Non-pharmacologic actions:
- Echo before discharge (AHA 2017)
- Pediatric cardiology consult day 1 (AHA 2017)
- ID + rheumatology if MIS-C overlap (AAP 2024)
- PICU consult if KDSS / shock / myocarditis (AHA 2017)

AVOID / contraindication checks:
- Delay live vaccines 11 months after IVIG (AAP 2024)
- ASA Reye risk during VZV or influenza exposure (AHA 2017 McCrindle)
- Check IVIG infusion reactions premedicate diphenhydramine (AHA 2017)
- Steroid taper no abrupt stop (RAISE Lancet 2012)
- Warfarin INR 2 3 for giant aneurysm (AHA 2017)
- Dual antiplatelet for medium aneurysms (AHA 2017)

Monitoring

Regimen monitoring:
- temperature q4h until afebrile 48-72h (AHA 2017)
- CRP ESR q1-2 days until normal (AHA 2017)
- CBC q1-2 days during acute (AHA 2017)
- echocardiogram at diagnosis then 1-2w then 6-8w (AHA 2017)
- ECG at diagnosis and pre-discharge (AHA 2017)
- IVIG response assessment at 36h (AHA 2017)
- long-term cardiology follow-up lifetime if aneurysm (AHA 2017)
- live vaccine schedule adjusted for IVIG (AAP 2024)

Setting (ed) monitoring:
- Vitals q1h initially (AHA 2017)
- Continuous SpO2 (AHA 2017)
- Cardiac monitor if KDSS (AHA 2017)

Follow-up plan: Pediatric cardiology follow-up at 6-8 wk + lifetime if aneurysm per AHA 2017; influenza/VZV vaccination timing relative to IVIG (delay live vaccines 11 months per AAP 2024); discontinue ASA at 6-8 wk if no aneurysm; family counseling on recurrence (~2%)
- Close-out criterion: long-term cardiac plan + vaccination plan + return precautions documented

Monitoring phase: Daily fever curve, CRP/ESR trend; serial echo at diagnosis, 1-2 wk, 6-8 wk (longer if aneurysm) per AHA 2017; IVIG infusion reactions; ASA Reye risk during VZV/influenza exposure

Disposition

Current setting: ed — Recognize KD pattern (classic vs incomplete), expedite admission, initiate IVIG + ASA promptly

Disposition criteria:
- Inpatient pediatrics for all KD (AHA 2017)
- PICU for KDSS / myocarditis / large aneurysms (AHA 2017)

Escalation triggers (move to higher acuity):
- KD shock syndrome -> PICU + pressors (AHA 2017)
- Coronary aneurysm at presentation -> tertiary KD center (AHA 2017)
- MIS-C overlap -> infection control + ID + rheumatology (AAP 2024)

Patient Action Plan

**Kawasaki disease post-discharge action plan — coronary surveillance + medication adherence**
Personalised values: coronary_z_score_at_discharge, aneurysm_size_classification, medication_regimen, age_for_dosing.

**Recovering well — no aneurysm or small aneurysm regressing** (green):
Triggers:
- Afebrile x 1 week (AHA 2017)
- Normal/mild coronary changes (z-score <2.5) (AHA 2017)
- Normal CBC + CRP/ESR trending normal (AHA 2017)
- Tolerating low-dose ASA (AHA 2017)
- No new symptoms (AHA 2017)
Actions:
- Continue low-dose ASA 3-5 mg/kg/day until 6-8 weeks afebrile + normal echo (AHA 2017)
- Cardiology follow-up at 1-2 weeks + 6-8 weeks (AHA 2017)
- Avoid live vaccines for 11 months after IVIG (consult vaccine schedule; AAP 2024)
- Avoid contact with VZV/influenza while on ASA (Reye risk) — switch to clopidogrel if exposed (AHA 2017)
- Daily activity as tolerated (AHA 2017)
- Inform pediatrician + cardiologist of any new symptoms (AHA 2017)

**Caution — coronary aneurysm OR fever recurrence OR medication reaction** (yellow):
Triggers:
- New coronary aneurysm (z-score 2.5-10) (AHA 2017)
- Recurrent fever after discharge (AHA 2017)
- Persistent inflammation (CRP/ESR not normalizing) (AHA 2017)
- Medication side effects (GI bleed on ASA, easy bruising) (AHA 2017)
- Suspected adverse reaction to IVIG (delayed hemolysis) (AHA 2017)
Actions:
- Contact pediatric cardiology within 24h (AHA 2017)
- May need additional anti-thrombotic (clopidogrel if medium aneurysm) (AHA 2017)
- Recurrent fever then re-evaluate for IVIG resistance OR alternative diagnosis (AHA 2017)
- Adverse reaction then contact provider for medication change (AHA 2017)
- Bring child to ED if signs of bleeding or coronary ischemia (chest pain, syncope) (AHA 2017)
Contact provider when:
- Any aneurysm progression or new aneurysm (AHA 2017)
- Fever recurrence (AHA 2017)
- Medication side effects (AHA 2017)

**Emergency — coronary thrombosis, MI, severe bleeding, KDSS recurrence** (red):
Triggers:
- Chest pain in child with prior KD (AHA 2017)
- Syncope, sudden collapse (AHA 2017)
- Severe bleeding on antiplatelet/anticoagulant (AHA 2017)
- New shock syndrome with rash + perfusion changes (AHA 2017)
- Stroke symptoms (focal neuro deficit) (AHA 2017)
Actions:
- Call 911 immediately (AHA 2017)
- Bring all medications + KD records (AHA 2017)
- Identify as Kawasaki disease patient with coronary aneurysm if applicable (AHA 2017)
- Pediatric cardiac center transfer if available (AHA 2017)
Contact provider when:
- Any red zone trigger — go to ED, do not wait (AHA 2017)

Earlier-Return Triggers

Return-precaution thresholds (watch for):
- [LIFE_THREATENING] KD with hypotension, poor perfusion, ventricular dysfunction, or vasoactive requirement
- [LIFE_THREATENING] Coronary z-score ≥10 OR absolute diameter ≥8 mm
- [SEVERE] Fever ≥5 days + ≥4 of 5 principal criteria

Citations

- 2017 AHA Scientific Statement on Diagnosis, Treatment, and Long-term Management of Kawasaki Disease + RAISE trial (steroid for high-risk) + Egami/Kobayashi/Sano IVIG-resistance scores [PMID:28356445](https://pubmed.ncbi.nlm.nih.gov/28356445/)
- Cited evidence (PMID 30337183) [PMID:30337183](https://pubmed.ncbi.nlm.nih.gov/30337183/)

Last reconciled with current guidelines: 2026-04-27.
References
  • 2017 AHA Scientific Statement on Diagnosis, Treatment, and Long-term Management of Kawasaki Disease + RAISE trial (steroid for high-risk) + Egami/Kobayashi/Sano IVIG-resistance scores — PMID:28356445
  • Cited evidence (PMID 30337183) — PMID:30337183