Kawasaki disease (KD) is a systemic vasculitis of infants and children that may present initially with fever and unilateral cervical adenopathy as the only clinical features. Early diagnosis and administration of high-dose intravenous gamma-globulin (IVIG) can prevent coronary artery aneurysms, which occur in up to 25% of untreated children. The otolaryngo-logist may see these patients in referral and must learn to recognize the principal diagnostic criteria that constitute the clinical case definition.

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