Kawasaki syndrome can have serious effects

The Baltimore Sun

After the death of John Travolta's 16-year-old son this month, speculation about possible causes blossomed in the print and broadcast media and blogosphere, including discussions of Kawasaki syndrome. This disease - an inflammatory, autoimmune disorder - typically affects young children and, in some cases, can cause serious heart damage, says Stacy Fisher, a cardiologist at MidAtlantic Cardiovascular Associates.

Before last week, little was written in the media about Kawasaki syndrome, which is also called mucocutaneous lymph node syndrome. Could you further describe it?

Kawasaki is a syndrome - basically an illness of infants and small children usually between the ages of 1 and 5 years old. It is uncommon in children over the age of 8 years and unheard of in infants under the age of 3 months.

What are the symptoms of Kawasaki disease?

The symptoms can include high-grade fever (usually above 104 degrees F) that last 10-12 days; a rash spreading from the palms and soles of feet to the torso; swelling and redness of the hands and feet; irritated, red eyes; and swollen lymph glands. There can also be what's called 'strawberry tongue' (the tongue has a bright red appearance with white patches).

According to the American Heart Association, Kawasaki disease and rheumatic fever are leading causes of acquired heart disease in children in the United States. How does Kawasaki disease affect the heart?

The coronary arteries can be weakened and an aneurysm can form. The disease can also cause inflammation of the heart muscle and arrhythmias [irregular heartbeats]. The heart valves also can be affected.

How is this disease diagnosed?

There is no specific test, but diagnosis is made if you have persistent high fever and four of the [other] symptoms.

What causes Kawasaki disease?

It is not clear what causes it, and there are a lot of theories. Most people who deal with Kawasaki think that it is from a ubiquitous exposure - to a bacteria or similar organism to which everyone is exposed, but some people have an autoimmune reaction to it. But this is a theory.

How is it treated?

Usually we hospitalize patients ... and the main treatment is aspirin [which reduces fever, treats pain and helps prevent blood clots from forming]. We also treat with an IVIG [intravenous gamma globulin, which reduces the coronary artery and general heart muscle involvement, and is given for one or two days depending upon the weight of the patient].

If you are treated early with aspirin and IVIG, there is a 1 percent incidence of these complications. If not treated early, there is a 15 percent to 25 percent incidence of long-term effects involving heart muscle dysfunction or coronary aneurysm. Patients who develop these complications are treated long-term with aspirin and, in general, also do well.

We continue to follow those patients who have coronary artery or heart muscle involvement throughout young adulthood.

How prevalent is it?

That is hard to answer. I know that at Sinai [Hospital], about eight to 10 people are hospitalized each winter and spring.

The disease does have a seasonal prevalence: It is more common in the winter and early spring. It also is much more common in Japan, where it was discovered, and is more common in males than in females. But it is found in all socioeconomic categories and all races.

Is there anything else parents should know about this disease?

If your child has a persistent high-grade fever, you should seek medical counsel. You shouldn't just treat with aspirin. Aspirin can cause something called Reyes Syndrome, so you really do want a professional diagnosis. Pediatricians are very aware of and are concerned about this diagnosis, so seek counsel early and do not resist hospital admission if this is the suspected diagnosis.

Also, it is good to know that there is no real evidence that Kawasaki disease is contagious and that, historically, 1 percent of patients die from complications, which often happen in the second phase. On the other hand, in the spectrum of things, it is a fairly rare childhood illness and most people who have the disease go on to lead healthy, unrestricted lives.

Holly Selby is a former reporter for The Baltimore Sun.

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