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Kawasaki disease (KD), an acute febrile illness of childhood, was described in Japan in 1967 by Dr. Tomasaku Kawasaki as the mucocutaneous lymph node syndrome.1 A vasculitis of medium-sized vessels, KD has a predilection for the coronary arteries, and 20% to 25% of untreated children develop coronary artery aneurysms. Fortunately, a regimen of intravenous immunoglobulin and aspirin reduces the incidence of coronary artery abnormalities to less than 5%.2 Nonetheless, KD is a leading cause of acquired heart disease in children in North America and Japan. Furthermore, the etiology of KD remains elusive, impeding the development of targeted therapy and diagnostic testing.

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Epidemiology and Specific Populations at Risk

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The Japanese Ministry of Health has collected epidemiologic data on Kawasaki disease (KD) since 1970 with nationwide surveys every 2 years. In total, over 225,000 cases of KD were reported in Japan between 1970 and 2006.3-5 In addition to endemic disease, 3 epidemics of KD occurred in Japan in 1979, 1982, and 1986, with an incidence of 196.1 KD cases per 100,000 children less than 5 years of age in 1982, the most severe of the epidemics. Interestingly, there have been no recent epidemics, but recent surveys have revealed an increase in the incidence of KD.4,5 The incidence of KD in Japan from 2005 to 2006 was 184.6 per 100,000 in children younger than 5 years of age.3 The highest incidence of KD was in children younger than 1 year of age, with a peak incidence of 6 to 8 months, and cardiac abnormalities occurred most commonly in infants and in children older than 4 years.3

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In the United States, Holman et al6 reported a hospitalization rate for Kawasaki disease (KD) in the United States in 2000 as 17.1 per 100,000 children less than 5 years of age, with a median age at admission of 2 years. Children of Asian and Pacific Islander heritage had the highest hospitalization rate at 39 per 100,000 children. A subsequent study of KD in Hawaiian children revealed an incidence higher than the national findings, with an incidence of 45.2 per 100,000 children less than 5 years of age from 1996 to 2001.7 Furthermore, that study revealed a striking discrepancy amongst ethnicities in patients less than 5 years of age, with an incidence of 35.3 per 100,000 white children and 70.9 per 100,000 Asian and Pacific Islander children residing in Hawaii. The incidence of KD in Japanese Americans living in Hawaii was 197.7 per 100,000 children, higher than the incidence in Japan during the last epidemic. Siblings of patients with KD may be at higher risk for developing the disease,8 and parents of affected children in Japan are twice as likely to have a history of KD themselves.9 These findings are consistent with the hypothesis that KD results from unidentified environmental triggers in genetically susceptible hosts.

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Risk factors for poor ...

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