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Clinical Summary

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Kawasaki disease (KD; also known as mucocutaneous lymph node syndrome) is an immune-mediated disorder of unknown etiology that occurs in genetically predisposed children leading to endothelial cell injury and vasculitis of mainly medium-sized arteries (predilection for coronary arteries). Small arterioles, larger arteries, capillaries, and veins are affected to a lesser extent. KD occurs in all ethnic groups; however, incidence is highest in East Asia and in children of Asian descent. Clinical and epidemiologic features strongly support an infectious etiology. About 80% of patients are <5 years of age (peak: between 18 and 24 months); about 90% of patients are <8 years. There is no specific test for KD; diagnosis is based on clinical criteria summarized in Table 12.1. Other findings include extreme irritability, aseptic meningitis (50%), urethritis (sterile pyuria, 70%), hepatic dysfunction (40%), hydrops of gallbladder, diarrhea, vomiting, abdominal pain, arthritis, or arthralgia (knees, ankles, hips), uveitis, pneumonitis, testicular swelling, peripheral gangrene, erythema, or induration at bacille Calmette-Guérin inoculation site.

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Table 12.1 – Clinical Features of Kawasaki Disease
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Figure 12.1 ▪ Kawasaki Disease.
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A 3-year-old child with a history of high fever of 6 days’ duration associated with diffuse erythematous maculopapular rash, red lips, and bilateral conjunctival injection. (Photo contributor: Binita R. Shah, MD.)

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