Kawasaki Disease (6 mo - 18 years)



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Kawasaki Disease (6 mo - 18 years)

Other obvious source of fever?

Treat appropriately

Persistant fever?

Note: Positive respiratory viral PCR results should NOT influence the diagnosis and management of KD

Retreat with IVIG (2 gm/kg)

Intravenous Immune Globulin (IVIG) infusion

CRP ≥ 3 mg/DL OR ESR ≥ 40 mm/hr?

Inclusion Criteria
  • 5 days of fever > 38.0 C (100.4 F)
  • No identifiable source of fever
Exclusion Criteria
  • Immunosuppressed, cancer
  • History of rheumatologic disease

Adjunct therapies for KD

  • Glucocorticoids
  • Not recommended for routine use in KD, but may be useful in refractory KD.
  • Anti-TNF-alpha agents (infliximab, pentoxifylline, etanercept)
  • May be useful in refractory KD
  • Immunosuppressive agents (cyclophosphamide, cyclosporine, etc.)
  • May be useful in refractory KD
  • Plasmapheresis
  • May be useful in refractory KD

Repeat echocardiogram, consult KD expert (pediatrics, rheumatology, infectious disease)

Assess supplemental laboratory criteria:

  • Albumin ≤3.0 g/dL
  • Anemia for age
  • Elevation of alanine aminotransferase (ALT)
  • Platelets after 7 days ≥ 450 000/mm3
  • White blood cell count ≥ 15 000/mm3
  • Urinalysis ≥10 WBC/hpf

  • Repeat CBCD, CRP, ESR with PMD in one week
  • Repeat echocardiogram at 6 weeks (or per cardiology consult)
  • Aspirin 5 mg/kg PO daily till markers of inflammation normalize (usually 8 weeks depending on if CA abnormalities exist)
  • If febrile within 2 weeks of IVIG consult rheumatology/ID & readmit

Other clinical characteristics consistent with KD

  • Cardiovascular
  • CHF, myocarditis, pericarditis, valvular regurgitation
  • Coronary artery abnormalities
  • Aneurysms of medium-sized noncoronary arteries
  • Raynaud's phenomenon
  • Peripheral gangrene
  • Musculoskeletal
  • Arthritis, arthralgia
  • Gastrointestinal
  • Diarrhea, vomiting, abdominal pain
  • Hepatic dysfunction
  • Hydrops of the gallbladder
  • Neurologic
  • Extreme irritability
  • Aseptic meningitis
  • Sensorineural hearing loss
  • Genitourinary
  • Urethritis/meatitis
  • Other
  • Erythema/induration at BCG inoculation site
  • Anterior uveitis (mild)
  • Desquamating rash in groin
  • Macrophage activation syndrome

Persistant fever?

Discharge, daily PCP visit

Fever resolved?

Consider other etiology

Peeling beginning under nail bed of fingers then toes?

Consider other etiology

Consider other etiology

Clinical characteristics consistent with KD?

Aspirin 20 mg/kg PO Q6hrs (high-dose) until afebrile for 48 hours then 5 mg/kg per day (low-dose), Cardiology consult if Echo positive

Aspirin tabs

Persistant fever for 2 or more days?

Fever more than 36 hours after IVIG?

Meets discharge criteria?

Quantify number of primary criteria present:

  1. Oral mucous membrane changes (injected/cracked lips, injected pharynx, strawberry tongue)
  2. Polymorphous rash
  3. Peripheral extremity changes (palmar erythema, hand or feet edema, periungual desquamation)
  4. Cervical lymphadenopathy (one lymph node >1.5 cm in diameter)
  5. Bilateral bulbar conjunctival injection without exudate

Persistant fever?

Admit to Floor
Complete Blood Count, Serum
Complete Metabolic Panel, Serum
C-Reactive Protein (CRP), Serum
Erythrocyte Sedimentation Rate (ESR), Serum
Urinalysis with micro, Urine
Intravenous Immune Globulin (IVIG) infusion

Discharge criteria

  • Afebrile
  • Reassuring vital signs
  • Tolerating PO
  • Well-appearing
  • No social/family concerns
  • Reliable follow up in 24 hours
  • Parents comfortable and understand discharge plan
  • Provider comfortable with outpatient therapy

Consult KD expert (pediatrics, rheumatology, infectious disease)

Note: Children < 6 months with greater than 5 days of fever with no other source and even with negative supplemental laboratory and clinical findings may still be at high risk for KD and benefit from treatment.

Echocardiogram

Echocardiogram

Contact Kawasaki disease expert (rheumatology or infectious disease)

Complete Blood Count, Serum
Complete Metabolic Panel, Serum
C-Reactive Protein (CRP), Serum
Erythrocyte Sedimentation Rate (ESR), Serum
Urinalysis with micro, Urine



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