Kawasaki Heart Disease

About the Auther

I. Clinical Features
II. Diagnosis
III. Causation
IV. Management
GLOSSARY
arterial dilatation enlargement or increase in luminal diameter
of the artery. myocardial infarction death of an area of heart muscle due to
blockage of a coronary artery by blood clot and atheroma;
medical term for a heart attack or coronary thrombosis. vasculitis inflammation of the walls of a blood vessel.
KAWASAKI DESCRIBED THIS ILLNESS IN Japanese children in 1967. The major cardiac lesion is an inflammatory reaction of the walls of arteries or vasculitis of the coronary arteries. A similar disease is polyarteritis nodosa. The exact cause of the disease is unknown. It is seen worldwide, however; in Japan children with a mean age of 12 months are affected and in the United States the mean age is closer to 3 years. Kawasaki heart disease is rare in children over the age of 8 with more than 85% being younger than 5 years. Children younger than 6 months or older than 8 years are rarely affected; but the older group is at increased risk of coronary-artery aneurysms. The reported annual incidence rate in Japan is approximately 140 per 100,000 children under 5 years of age versus 17 and 8 in the US and UK, respectively.

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  • I. CLINICAL FEATURES
    This disorder is virtually always accompanied by fever for more than 5 days. The fever has no identifiable cause
  • II. DIAGNOSIS
    Diagnosis is difficult and infants with three or more of the above symptoms with unexplained fever for more than 2 weeks should have an echocardiographic evaluation to exclude coronary artery aneurysms. Damage to the coronary arteries by vasculitis occurs in more than 25% of infants and in about 10% of children age 2–5. Coronary artery aneurysm and thrombosis may cause myocardial infarction and sudden death. Similar aneurysms may be found in the worst affected cases in the renal, cerebral, and abdominal arteries. Children frequently died during the acute phase of Kawasaki disease, but if they recover they may present with symptoms of aneurysms during adolescence and rupture of an aneurysm may cause sudden death. Coronary-artery aneurysms occur as a sequela of the vasculitis in 20–25% of untreated children. The syndrome may remain silent until the third or fourth decade of life, when patients are present with an acute myocardial infarction. Cardiac complications include myocarditis, pericarditis with effusion; mitral valvular lesions occur in about 1% of patients.
  • III. CAUSATION
    The cause of Kawasaki syndrome remains unknown; an infectious agent is suspected because of the following
  • IV. MANAGEMENT
    A. Intravenous Gamma Globulin Therapy
  • BIBLIOGRAPHY
    Burns, J. C. et al. Translation of Dr. Tomisaku Kawasaki’s original report