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Kawasaki Disease

• Definitions
· Kawasaki Disease (Mucocutaneous lymph node syndrome) is a type of vasculitis (inflammatory disease of blood vessels) that occurs in children.

• Overview
· Kawasaki Disease usually occurs in children less than five years of age with the majority occurring under two years of age.

· There is currently no known cause of Kawasaki Disease, although the occurrence is likely related to:
· a genetic predisposition
· an exposure to a protein or infection that sets off the immune system to overreact and attack certain blood vessels.

• Symptoms
· Kawasaki Disease usually presents with symptoms of:
· fever
· redness and swelling of the lips and tongue and oral mucosa
· rash with scaling skin of the palms and fingers

· Kawasaki Disease is the leading cause of heart disease in children in the United States.
Since Kawasaki Disease is a vasculitis (inflammatory disease of blood vessels), children with the disease are at risk of obstruction of blood flow through arteries that are inflamed from the vascultits.
· This increases the risk of obstructed blood flow to the heart and heart attack.

• Treatment
· Kawasaki Disease may be treated with aspirin as an anti-platelet agent to prevent platelet clumping in inflamed arteries and high dose IV gamma globulin to inhibit the immune response causing the vasculitis.

References
HirataS et al: Incidence rate of recurrent Kawasaki disease and related risk factors: From the results of nationwide surveys of Kawasaki disease in Japan. Acta Paediatr 90:40, 2001 [PMID: 11227331]

GuptaM et al: Cytokine modulation with gamma-globulin in peripheral blood of normal children and its implications in Kawasaki disease treatment. J Clin Immunol 21:193, 2001 [PMID: 11403226]

Weyand CM, Goronzy JJ: Medium- and large-vessel vasculitis. N Engl J Med 349:160, 2003 [PMID: 12853590]

Fauci AS et al: the spectrum of vasculitis: Clinical, pathologic, immunologic, and therapeutic considerations. Ann Intern Med 89:660, 1978 [PMID: 31121]

Posted on January 3, 2009