川崎病进展课件.ppt

Kawasaki Disease:An Update Bevin Weeks, MD Yale University School of Medicine Objectives Review the historical clinical features of a “common” but poorly understood childhood disease Explore the differential diagnosis of this illness Consider the most important sequelae of this disease Discuss treatment long-term management recommendations Kawasaki Disease:An Update History of Kawasaki disease Epidemiology and etiology Presentation and diagnosis Treatment Chronic cardiovascular manifestations Follow up of patients Questions in the chronic management Kawasaki Disease:Mucocutaneous Lymph Node Syndrome “A self-limited vasculitis of unknown etiology that predominantly affects children younger than 5 years. It is now the most common cause of acquired heart disease in children in the United States and Japan.” Jane Burns, MD* *Burns, J. Adv. Pediatr. 48:157. 2001. History of Kawasaki Disease Original case observed by Kawasaki January 1961 4 y.o. boy, “diagnosis unknown” CA thrombosis 1st recognized 1965 on autopsy of child prev. dx’d w/MCOS First Japanese report of 50 cases, 1967 First English language report from Dr. Kawasaki 1974, simultaneously recognized in Hawaii What is Kawasaki Disease? Idiopathic multisystem disease characterized by vasculitis of small medium blood vessels, including coronary arteries Epidemiology Median age of affected children = 2.3 years 80% of cases in children 4 yrs, 5% of cases in children 10 yrs Males:females = 1.5-1.7:1 Recurs in 3% Positive family history in 1% but 13% risk of occurrence in twins Overall U.S. in-hospital mortality ≈ 0.17% Epidemiology Annual incidence of 4-15/100,000 children under 5 years of age Over-represented in Asian-Americans, African-Americans next most prevalent Seasonal variation More cases in winter and spring but occurs throughout the year The Biggest Question:What is the Etiology of Kawasaki Disease? Etiology Infectious agent most likely Age-restricted susceptible population Seasonal variati