Treatment of Kawasaki Disease in Young Children Under 5 Years
All children diagnosed with Kawasaki disease should be treated immediately with intravenous immunoglobulin (IVIG) 2 g/kg as a single infusion plus high-dose aspirin (80-100 mg/kg/day divided into four doses), ideally within the first 10 days of fever onset. 1
Initial Treatment Protocol
Primary Therapy
- IVIG 2 g/kg should be administered as a single infusion over 10-12 hours 1
- High-dose aspirin 80-100 mg/kg/day divided into four doses for anti-inflammatory effect 1
- This combination reduces coronary artery abnormalities from 15-25% (untreated) to approximately 5% for any abnormality and 1% for giant aneurysms 1
Timing Considerations
- Treatment should be initiated as soon as the diagnosis is established, even before day 5 of fever if classic features are present 1
- Children presenting after day 10 should still receive treatment if they have ongoing systemic inflammation (ESR elevated or CRP >3.0 mg/dL) with persistent fever or coronary artery abnormalities 1
- Do not withhold treatment in children presenting late if inflammation markers remain elevated 1
Aspirin Management Algorithm
Acute Phase
- Continue high-dose aspirin (80-100 mg/kg/day) until 48-72 hours after defervescence OR until day 14 of illness with 48-72 hours afebrile 1
Convalescent Phase
- Transition to low-dose aspirin (3-5 mg/kg/day) as a single daily dose for antiplatelet effect 1
- Continue for 6-8 weeks if no coronary abnormalities are detected 1
- Continue indefinitely if coronary artery abnormalities are present 1
Critical Aspirin Precautions
- Administer annual influenza vaccination to children on long-term aspirin therapy due to Reye's syndrome risk 1
- Instruct parents to contact physician immediately if influenza or varicella symptoms develop 1
- Avoid ibuprofen as it antagonizes aspirin's antiplatelet effect 1
Treatment for Incomplete Kawasaki Disease
Children under 5 years with incomplete (atypical) Kawasaki disease face paradoxically higher rates of coronary aneurysms if untreated, making prompt recognition critical 1
Diagnostic Algorithm for Incomplete Disease
- Children with ≥5 days of fever plus 2-3 clinical criteria should undergo laboratory assessment (CRP, ESR) 1
- If CRP ≥3.0 mg/dL or ESR elevated with compatible clinical features, treat with IVIG and aspirin 1
- Perform echocardiography in all suspected cases; coronary abnormalities with ≥3 clinical features confirms diagnosis and mandates treatment 1
- Infants with ≥7 days of unexplained fever should have laboratory assessment even without KD features; perform echo if inflammation markers are elevated 1
Management of Treatment-Resistant Disease
Approximately 10-15% of patients fail to respond to initial IVIG (persistent or recurrent fever >36 hours after completion) 1, 2
Second-Line Therapy
- Administer a second dose of IVIG 2 g/kg for persistent fever beyond 36 hours after initial treatment 1, 2
- Continue high-dose aspirin throughout 1
Third-Line Options for Multiple IVIG Failures
- Intravenous methylprednisolone pulse therapy 2, 3
- Infliximab (anti-TNF therapy) 2, 3
- Cyclosporine or methotrexate may be considered for patients failing multiple IVIG doses and steroids 4, 2
Cardiac Monitoring Requirements
Acute Phase Echocardiography
- Perform baseline echocardiography at diagnosis to assess for coronary artery dilation, aneurysms, decreased ventricular function, valvular regurgitation, or pericardial effusion 1
- Obtain follow-up echo at 2 weeks and 6-8 weeks after disease onset 1
Long-Term Surveillance
- Frequency and intensity of cardiovascular monitoring depend on presence and severity of coronary abnormalities at diagnosis 5
- Children with persistent coronary abnormalities require lifelong cardiology follow-up 1
Critical Pitfalls to Avoid
- Do not delay treatment waiting for all classic criteria to appear simultaneously; features may evolve over time and careful history-taking is essential 1
- Do not dismiss incomplete presentations in infants <1 year, as they have the highest risk of coronary complications 1
- Do not mistake initial symptoms for antibiotic reactions (rash after treatment for presumed cervical lymphadenitis), partially treated UTI (sterile pyuria), or viral meningitis (CSF pleocytosis in young infants) 1
- Do not withhold IVIG based on risk scores alone; all diagnosed patients should receive treatment regardless of predicted risk 1