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Kawasaki disease (KD) is an acute, self-limited vasculitis of infants and children characterized by prolonged fever and a constellation of nonspecific clinical findings: polymorphous skin eruption; erythema of lips, tongue, and oral mucosa; erythema of the palms, soles, or both; bilateral conjunctival injection; and cervical lymphadenopathy. Coronary artery aneurysms develop in 15% to 25% of untreated patients, leading to ischemic heart disease, myocardial infarction, and sudden death. KD is the leading cause of acquired pediatric heart disease in industrialized countries. IVIG treatment reduces the aneurysm rate to less than 5%, but 10% to 20% of patients fail to respond to IVIG and have a high risk for developing coronary artery disease.
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