What you need to know about: Kawasaki Syndrome

What is Kawasaki syndrome?

Kawasaki syndrome is an inflammatory illness that can potentially affect the heart and its larger arteries. It primarily affects infants and young children under the age of five. First studied in Japan in 1967, it was later recognized worldwide in children of every racial group.

What are the symptoms of Kawasaki syndrome ?

Kawasaki syndrome has a sudden onset, with a fever as high as 104°F (40°C) that does not respond to antibiotics and a rash that spreads over the patient's chest and genitals. The fever is followed by a characteristic peeling of the skin beginning at the fingertips and toenails. The patient's lips become very red, with the tongue developing a "strawberry" appearance. The palms, soles, and inner portion of the eyelids become purplish-red and swollen. The lymph nodes in the patient's neck may also become swollen. These symptoms may last from two weeks to three months, with relapses in some patients.

What does Kawasaki syndrome cause?

Kawasaki syndrome progresses through three different stages:

Acute phase : This is when symptoms are most severe. It usually lasts 1-2 weeks.

Subacute phase : At this stage, the child's fever, rash, and swollen lymph nodes go away. However, the child still feels irritable, has a poor appetite and slight eye redness, and may develop peeling skin on the fingers and toes. This stage usually ends 3-4 weeks after the fever began.

Convalescent stage : This stage begins when all clinical symptoms are gone, but the results of a blood test indicate that there is still inflammation in the body. The convalescent stage ends when the test results return to normal, usually 6-8 weeks after the fever began.

About 20% of the cases have complications involving the cardiovascular system. These complications include myocarditis (inflammation of heart muscle), pericarditis (inflammation of membranes covering the heart) or valvulitis (inflammation of the heart valves). However, the most important problem related to Kawasaki syndrome is the threat of vasculitis (blood-vessel inflammation). This can be especially dangerous when it damages the heart's coronary arteries, producing an abnormal widening or bulging (aneurysm) in these vessels.

A third of patients will have joint symptoms, which generally last about two weeks, but may persist for as long as three months.

How common is Kawasaki syndrome?

For children in the U.S. and other industrialized nations, Kawasaki syndrome is now the most common cause of heart disease that is not present at birth. According to U.S. government statistics, a total of 1,262 cases of Kawasaki syndrome were reported between 1994 and 1997 .

Who is likely to get Kawasaki syndrome?

Males are 1.5 times as likely as females to get the illness. Asians and blacks have a higher incidence rate of Kawasaki syndrome than do whites.

How do people get Kawasaki syndrome?

The specific cause of Kawasaki syndrome is unknown, although the disease resembles infectious illnesses in many ways. It has been suggested that Kawasaki syndrome represents an allergic reaction or another unusual response to certain types of infectious agents.

Although the disease usually appears in individuals, it sometimes affects several members of the same family and occasionally occurs in small epidemics.

How do I protect myself from Kawasaki syndrome?

There is no evidence that Kawasaki syndrome is contagious.

How do I protect others from Kawasaki syndrome?

Since the cause of the illness is not known, there are currently no vaccines available. Since it is probably not spread from person-to-person, no disease control measures are necessary when a child in a day care center is diagnosed with this condition.

What do I do if I think my child has Kawasaki syndrome?

Consult a physician if the child has a persistent fever.

How is Kawasaki syndrome diagnosed?

Because Kawasaki syndrome is rare in the U.S., doctors will want to rule out other illnesses that are more common and have similar symptoms. The doctors may want to know about any drug reactions, exposure to someone with strep throat or measles, and recent tick bites.

Since the cause of Kawasaki syndrome is unknown, there are no laboratory tests that can confirm the diagnosis.

How is Kawasaki syndrome treated?

Kawasaki syndrome is usually treated with a combination of aspirin, to control the patient's fever and inflammation, and high doses of intravenous immune globulin to reduce the possibility of coronary artery complications. Other treatments are given depending on the symptoms manifested.

For the more severe cases, follow-up monitoring and long-term treatment may be indicated.

Should I worry about Kawasaki syndrome when I travel out of the country?

No precautions are needed because the illness is not believed to be contagious.