Kawasaki disease is an acute multisystem vasculitic syndrome of unknown etiology occurring mostly in infants and children younger than 5 years of age especial in Asia countries. In developed countries, Kawasaki disease is currently the leading cause of acquired heart diseases in children. However, it is still a mysterious disease since the first report of Dr. Kawasaki in 1967. In this chapter, we review and summarize the history, epidemiology, infection, genetics, and immune response of this disease.
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