Kawasaki disease and its cardiac sequelae.

In 1967, Tomisaku Kawasaki described a syndrome of mucocutaneous inflammation in 50 Japanese children. Seven years later, he reported his experience in English, emphasizing that 1% to 2% of affected children died of cardiac failure. Since then, Kawasaki disease has been described worldwide in children of all racial groups and has been recognized as a leading cause of acquired heart disease among children in the United States (Figure 1). The disease affects mostly toddlers; about 80% of patients are less than five years old, and only rare cases are seen in adolescents over age 15. Intravenous gamma globulin has recently been demonstrated to reduce systemic inflammation and the prevalence of coronary artery aneurysms in patients with Kawasaki disease. The treatment, however, is effective only if administered early in the illness. Hence, prompt and accurate diagnosis is essential. The following cases offer a context in which to discuss some of the clinical issues surrounding Kawasaki disease.