Kawasaki disease, also called Kawasaki syndrome, is the leading cause of acquired heart disease in children in the developed world. It usually appears in children under 5 years old. If left untreated, it can result in the formation of a coronary artery aneurysm in up to 30% of cases. With treatment, this complication rate is reduced to about 2%.
Kawasaki disease was first diagnosed in Japan in the 1960s. It can occur in people of any race or ethnic origin, although it is more common in people of Asian descent. It affects mostly children 5 years old and under, although children of any age can get it. Boys are twice as likely to get the disease as girls.
In Canada, about 30 in every 100,000 children under the age of 5 years are diagnosed with Kawasaki disease every year.
Doctors don’t know what causes the disease. They believe a virus or some other kind of infection may be responsible for causing the body to mount an "overzealous" immune response, leading to the inflammation of a child’s blood vessels (vasculitis) and causing the signs and symptoms of Kawasaki disease.
If Kawasaki disease is treated early, there’s less of a chance of coronary artery damage. Children with the condition get high doses of intravenous immunoglobulin as soon as the diagnosis is made. They’re also given very high doses of acetylsalicylic acid* (ASA). After their fever has gone down, children with Kawasaki disease are given a lower dose of ASA for a few months. This treatment prevents the blood from clotting in case there is some damage to the coronary arteries. Some children may be given steroids if they have more resistance to immunoglobulin. Live-virus vaccines, such as vaccines for measles, mumps, rubella and chickenpox, should be delayed by about 1 year for children given immunoglobulin since the vaccines may not work as well.
Small coronary artery aneurysms resulting from Kawasaki disease are treated with ASA. For large aneurysms, other blood thinning agents (e.g., warfarin, clopidogrel, or heparin) may be added to the ASA treatment. Small aneurysms go away within a year, but often leave weakened arteries that can cause heart problems in the future. For example, children with Kawasaki disease can have heart attacks 10 years after first being diagnosed with the condition.
If their coronary arteries haven’t been damaged, children with the condition can make a complete recovery. Kawasaki disease is fatal in about 1% of cases, where most of the fatalities occur within 6 months of developing heart complications.
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