LYCOS RETRIEVER 
Kawasaki Disease: Cases
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Kawasaki disease causes inflammation of the blood vessels; in some cases this includes the arteries of the heart (the coronary arteries). This inflammation weakens the walls of the blood vessels. In most cases the blood vessels return to normal after several months, but in some cases they remain weakened and may even balloon out, causing aneurysms (blood-filled swellings of the blood vessels).
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Up to 15 to 20% of children with untreated Kawasaki disease suffer damage to the coronary arteries, making the disease a leading cause of acquired heart disease in children. This complication is commoner in younger infants, and in boys. About 2% of cases are fatal and aneurysms usually recover spontaneously but slowly.
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This disease, first described by Dr. Tomisaku Kawasaki in 1967, usually affects children between the ages of 6 months and 4 years, with most cases occurring between the ages of 18 and 24 months. Its causes are unknown.
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Carditis is present to some degree in all cases of Kawasaki disease. The pericardium, myocardium and endocardium can be affected. Pericardial effusion is present in 20 to 40% of cases. Inflammation of the mitral or aortic valves can lead to valvular regurgitation. Physical examination is often normal but gallops or muffled heart sounds may be heard. Congestive heart failure occurs in about 5% of cases.
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Some children with Kawasaki disease do not have the symptoms in the definition. The younger they are, the less likely they are to have a classic case--particularly if they are less than a year old.
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Even though the initiating event has not yet been identified, the immune system is known to be involved during the acute stage of Kawasaki disease. In response to an unknown triggering process, marked immunoregulatory abnormalities are observed. It is postulated that the various secreted cytokines target vascular endothelial cells, producing cell-surface neoantigens. Antibodies produced against these antigens may then target the vascular endothelium, resulting in a cascade of events leading to vascular damage.
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