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peds.kawasaki.core.v1

Kawasaki disease

pediatricsacutesyndromepediatricacuteinpatient
Start encounter →Print handoutPRODUCTION

Manifest is a batch-23 scaffold (defineBatch23ScaffoldManifest); no _design-brief.md or atoms.* files on disk for this package. Terminology codes are minimal (ICD-10 M30.3 KD; SNOMED 75053002 Kawasaki disease) — manifest itself is sparse, expand once authored. No registry calculator for KD-specific scores (Kobayashi/Egami/Sano IVIG-resistance, coronary z-score) — needed before INTEGRATED. No regimen axis for IVIG ± ASA ± infliximab ± steroid; orchestration not yet structured.

Entry points (4)

  • symptom
    Fever >=5 days in a child (AHA 2017 McCrindle)
    prolonged_fever
  • symptom
    Conjunctivitis + rash + lip/oral changes + extremity changes + lymphadenopathy (AHA 2017 principal criteria)
    mucocutaneous_findings
  • lab_abnormality
    Markedly elevated CRP/ESR with normocytic anemia + thrombocytosis (AHA 2017 supplementary)
    inflammation_pattern
  • imaging
    Echocardiographic coronary artery dilation / aneurysm (AHA 2017)
    coronary_artery_changes

Required inputs (15)

  • agerequired
    demographic • used at CONTEXT
    Peak incidence 6 mo-5 yr; infants <6 mo and >8 yr have higher incomplete-KD + aneurysm risk (AHA 2017 McCrindle)
  • weightrequired
    demographic • used at TREATMENT
    IVIG 2 g/kg + ASA 30-50 mg/kg/d (or 80-100 in some protocols) are weight-based (AHA 2017)
  • temperaturerequired
    vital • used at CONTEXT
    Fever duration ≥5 days is core criterion (≥4 days if classic features in some 2017 AHA updates)
  • conjunctivitis_bilateral_nonexudativerequired
    symptom • used at INITIAL_WORKUP
    Principal clinical criterion (AHA 2017 McCrindle)
  • oral_changesrequired
    symptom • used at INITIAL_WORKUP
    Strawberry tongue, cracked lips, oropharyngeal erythema — principal criterion (AHA 2017)
  • rash_polymorphousrequired
    symptom • used at INITIAL_WORKUP
    Principal criterion (AHA 2017)
  • extremity_changesrequired
    symptom • used at INITIAL_WORKUP
    Erythema/edema acute -> desquamation subacute — principal criterion (AHA 2017)
  • lymphadenopathy_cervicalrequired
    symptom • used at INITIAL_WORKUP
    Unilateral cervical >=1.5 cm — principal criterion (often only one) (AHA 2017)
  • crprequired
    lab • used at INITIAL_WORKUP
    >=3 mg/dL supports incomplete KD (AHA 2017 algorithm)
  • esrrequired
    lab • used at INITIAL_WORKUP
    >=40 mm/h supports incomplete KD (AHA 2017 algorithm)
  • platelets
    lab • used at INITIAL_WORKUP
    Thrombocytosis (>450K) typically appears in subacute phase; can be normal early (AHA 2017)
  • albumin
    lab • used at INITIAL_WORKUP
    <=3 g/dL is incomplete-KD supplementary criterion (AHA 2017)
  • alt
    lab • used at INITIAL_WORKUP
    Hepatitis common; supplementary criterion (AHA 2017)
  • urinalysis
    lab • used at INITIAL_WORKUP
    Sterile pyuria supplementary criterion (AHA 2017)
  • echocardiogramrequired
    imaging • used at INITIAL_WORKUP
    Coronary z-score / aneurysm at diagnosis, 1-2 wk, 6-8 wk (AHA 2017)

12-phase flow (12)

  1. 1FRAME
    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: temperature, age
    advance: classic vs incomplete KD pattern identified
  2. 2ENTRY
    Trigger captured (fever >=5d per AHA 2017, mucocutaneous constellation, MIS-C overlap concern, persistent fever in infant)
    inputs: age
    advance: demographic + entry trigger captured
  3. 3CONTEXT
    Capture vaccination status, contacts, rule out viral mimics (measles, scarlet fever, Stevens-Johnson, leptospirosis, MIS-C post-COVID), assess BCG site reactivation (KD-suggestive) per AHA 2017
    inputs: weight
    advance: mimic screen + exposure history complete
  4. 4RED_FLAGS
    Coronary aneurysm at presentation, myocarditis with shock (KDSS), macrophage activation syndrome, IVIG resistance markers (Kobayashi/Egami/Sano scores; RAISE, Kobayashi Lancet 2012)
    inputs: temperature
    advance: red flags screened; IVIG resistance risk assessed
  5. 5INITIAL_WORKUP
    CBC, CRP, ESR, CMP (albumin, ALT), urinalysis, echocardiogram (coronary z-score), ECG; cultures + viral PCR to exclude mimics; SARS-CoV-2 / antibody if MIS-C overlap
    inputs: crp, esr, platelets, albumin, alt, urinalysis, echocardiogram
    actions: panel.cbc, panel.inflammation, panel.lft, panel.ua, panel.cardiac, workup.kawasaki
    advance: criteria + echo + supportive labs returned
  6. 6BRANCHING_WORKUP
    Incomplete KD algorithm (AHA 2017) if 2-3 criteria + supportive labs; cardiac MRI if echo windows poor and coronary concern; rheumatologic workup if uveitis or arthritis prominent
    advance: incomplete-KD pathway resolved; treatment threshold met or excluded
  7. 7DIFFERENTIAL
    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
    advance: phenotype assigned
  8. 8RISK_STRATIFICATION
    Coronary z-score classification (small/medium/giant); IVIG-resistance scoring; aneurysm risk per AHA 2017
    advance: risk class + IVIG-resistance probability documented
  9. 9TREATMENT
    IVIG 2 g/kg single infusion within 10 days of fever onset (or later if persistent inflammation/coronary changes) + high-dose ASA 30-50 mg/kg/d (or 80-100 in some centers) until afebrile 48-72h then 3-5 mg/kg/d for 6-8 wk; refractory KD → second IVIG dose ± infliximab 5-10 mg/kg ± steroids; high-risk per Kobayashi → upfront steroids (RAISE)
    inputs: weight
    advance: IVIG infused + ASA dosed + refractory plan ready
  10. 10DISPOSITION
    Inpatient pediatric cardiology + ID + rheumatology coordination; PICU if KDSS or shock; transfer to KD-experienced center if available
    advance: level of care + consults secured
  11. 11MONITORING
    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
    inputs: crp, esr
    actions: panel.cardiac, panel.inflammation
    advance: fever resolved; inflammation trending down
  12. 12FOLLOWUP
    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%)
    advance: long-term cardiac plan + vaccination plan + return precautions documented