Kawasaki Disease

This puzzling disease, first described in 1967 by a Japanese pediatrician, occurs mainly in children under age five and affects boys more often than girls. The disease causes a high fever, bloodshot eyes, swelling of the lymph glands in the neck and a number of skin changes, the most striking of which are bright red lips and swelling, redness and peeling skin on the palms and soles. In about one percent of cases, serious heart abnormalities develop.

For unknown reasons, Kawasaki disease is more than five times as common among children of Asian descent than among Caucasian children. In the United States, the incidence of the disease has increased steadily since 1974, when the first cases were reported.

WHAT CAUSES KAWASAKI DISEASE?
The cause is not known, although its symptom pattern and tendency to occur in clusters within communities suggests that it may be infectious. The fact that it involves several organ systems, however, indicates that it may, instead, be an autoimmune disease—that is, a disease that occurs when the immune system produces antibodies that attack the body's healthy tissues. Environmental factors also may play a role in its development.

WHAT HAPPENS DURING KAWASAKI DISEASE?
The disease progresses through several stages. The young child or infant first experiences a high fever, irritability, eye redness, swelling of the hands, feet and lymph nodes, and a rash. This phase, which lasts a week to ten days, gives way to a second phase in which fever subsides but other symptoms—frequently, arthritis and peeling skin on the hands and feet—appear. It is during this second phase—which lasts three to four weeks—that serious heart problems may develop. In the third phase, the child generally feels much better, but laboratory tests continue to show abnormalities in the blood, and heart problems may still develop. Complete recovery may take more than twelve weeks.

The most frightening cardiac complication of Kawasaki disease is the development of aneurysms (balloonlike swellings) in the walls of the coronary arteries. A coronary aneurysm can give rise to a blood clot, which in turn may block the artery and cause a heart attack.

HOW CAN THE PEDIATRICIAN TELL IF MY CHILD HAS KAWASAKI DISEASE?
Pediatricians base the diagnosis of Kawasaki disease on the presence of several of the disease's characteristic signs and symptoms. According to established guidelines, a child almost always has five out of six major signs and symptoms of Kawasaki disease (fever; conjunctivitis; redness, swelling, and/or peeling skin on the hands and feet; a rash, mainly on the trunk; and swollen lymph nodes in the region of the neck).

WHAT TREATMENTS ARE AVAILABLE?
Kawasaki disease is treated with high-dose intravenous gamma globulin therapy, which is usually administered in the hospital. Early treatment always reduces the possibility of coronary aneurysm formation. High doses of aspirin for several weeks are often given since some evidence shows that aspirin together with gamma globulin shortens the duration of the illness. Children are generally maintained on lower doses of aspirin during the latter weeks of the disease.

GETTING HELP
Call your doctor if your child:

• Develops a sudden, high fever and any of the eye, mouth or skin changes are characteristic of Kawasaki disease

• Is recovering from Kawasaki disease and develops chest pain, pallor and difficulty breathing, which suggest serious heart problems.

Excerpt from THE DISNEY ENCYCLOPEDIA OF BABY & CHILD CARE, © 1995 DSH Communications, Inc. This material is based on current medical research and, to the best of the editors' knowledge and understanding, is accurate and valid. However the reader should not use information contained in this material to alter a medically prescribed regimen or as a form of self-treatment, without seeking the advice of a licensed physician.