Kawasaki syndrome

Kawasaki Syndrome is the kind of exotic sounding illness that one expects to see on TV medical dramas but never to experience first hand. It is common enough that, in our moderate sized community hospital, we see four to six cases per year of the actual syndrome. Much more often it is included in a differential diagnosis of a sick child.

This condition was first described in 1967 by Dr. Tamisaku Kawasaki who noted a constellation of symptoms in several children of his Tokyo practice. These children fit into no other previously described diagnostic category and all had similar clinical characteristics.

First, they had high fever (102 [degrees] F to 104 [degrees] F) lasting for at least five days with no other explanation. Almost all of them experienced inflamed conjunctivae (red eyes) without drainage. He noticed changes around the mouth which always included either red, cracked lips, a bright red ("strawberry") tongue or a very red throat. Most of these children also experienced a bright red rash over most of their bodies, more accentuated on the trunk and extremities. Later he noticed changes in the hands and feet consisting of swelling, redness or peeling of the skin of the palms and soles. Most of these children had swelling of the glands of the neck, and many had GI (digestive system) complaints.

At first Dr. Kawasaki thought this condition belonged in the category of one of the benign childhood diseases associated with rashes and fever, until he noticed a curious complication. Many of the children who had experienced the signs and symptoms described above later went on to develop heart disease. Some of these children died from heart attacks, and when examined at autopsy, were found to have ruptured coronary aneurysms -- a quite unexpected finding in otherwise healthy children.

Because of the possibility of lethal complications to what had previously appeared to be a benign childhood illness, researchers worldwide attempted to understand more about this mysterious disease.

Although no one has yet determined the underlying cause, most agree that it falls into the category of a vasculitis, or inflammation of the small blood vessels of the body. In other words, some event -- a preceding viral infection, for example -- sets in motion an inflammatory reaction that, at its extreme, can result in inflammation of these tiny blood vessels. The places we most notice this effect is along the mucus membranes of the mouth and lips, the tiny vessels of the eye and sometimes, the coronary arteries.

Because of the potential cardiac complications, aggressive research has gone into their prevention. After trying various treatments, it appears that combinations of aspirin and immune globulins have had the best result in preventing the formation of coronary aneurysms in children with Kawasaki Syndrome.

So, for a child under 5 (80% of kids with Kawasaki are under four) who presents to his doctor with a history of five days of unexplained fever along with any combination of a strawberry tongue, swollen glands, cracked lips, rash, swollen hands and feet and GI complaints, Kawasaki Syndrome may well be considered as a possible diagnosis.

Experienced parents will notice that these signs and symptoms can be seen in many other pediatric illnesses other then Kawasaki's. Thus, the diagnosis is sometimes tricky. There are some supportive laboratory findings, but by and large this is a clinical diagnosis based on history, signs and symptoms.

When Kawasaki is seriously considered, hospital admission is required for further diagnostic work-up and treatment. Many times aspirin and immune globulin therapy will be initiated, echocardiogram is performed and cardiac function closely monitored. Once the diagnosis is certain, pediatric cardiology follow up is indicated.

The overall incidence of this syndrome in this country is anywhere between 1 and 10 per 100,000 children under 5, making it common enough to be carefully considered in a child with unexplained high fever. The treatment, aimed at preventing cardiac complications, can be life saving.

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