Puzzling Peril for the Young

A mystery malady of many symptoms is spreading steadily

Jeffrey Brown, 11, came home from a Cub Scout meeting in Dedham, Mass., one day last spring feeling sick. He had vomited, and by next morning was lethargic and complaining that his neck hurt. Jeffrey seemed to be coming down with a sore throat, but soon his temperature reached 106DEG F (41DEG C). A lymph gland in his neck swelled to golf-ball size, his lips and tongue turned strawberry, and scarlet blotches appeared on his chest and back. Jeffrey's illness: a perplexing and long unrecognized childhood malady called Kawasaki disease.

In 1961 a Tokyo pediatrician named Tomisaku Kawasaki, now 55, was struck by something unusual. Several of his patients at the Japan Red Cross medical center had the symptoms of scarlet fever, yet did not respond to penicillin. In the next years Kawasaki spotted similar cases. By 1967 he was convinced that he was seeing a new illness, one that mostly struck children under five, and could only be diagnosed by a combination of distinct symptoms. Among them: high fever persisting for five or more days, congested blood vessels in the eye, skin rashes, enlarged lymph nodes in the neck, peeling of the skin from fingers and toes.

Since Kawasaki first described the disease, more than 20,000 cases have been diagnosed in Japan alone. It has also been observed in other countries, though with a much lower incidence. In the U.S., the first cases were detected during the mid-'70s; so far only about 650 cases have been reported to the Center for Disease Control in Atlanta. But C.D.C. Epidemiologist David Bell points out that many doctors may still be unfamiliar with the disease.

Its symptoms can be confused not only with those of scarlet fever but also with those of juvenile rheumatoid arthritis and other ailments. While the illness is still considered rare, Bell says: "There's no question that 650 cases is an undercount."

The origin of the disease remains unknown. Since brothers and sisters of the victims almost never become ill, the ailment does not appear to be contagious. Says Kawasaki: "It could be a virus or some other microorganism. Because of the preponderance of cases among Japanese, it may have some ethnic and hereditary cause triggered by some new factor in the environment."

The disease has no known cure. Aspirin is the standard treatment, because it lowers fever, reduces inflammation and helps keep the blood from clotting. Most afflicted children eventually recover fully, as Jeffrey did, but up to one-third develop problems--especially aneurysms in the coronary arteries, weak patches in the walls of the arteries supplying blood to the heart. Complications from the condition, which may include abnormalities in the heart rhythm, heart attacks, or even a rare rupture of the coronary artery, kill about two in 100 patients.

At the New England Medical Center Hospital's pediatric unit in Boston, where Jeffrey was treated, and elsewhere, doctors are trying to find the cause of the disease, and its cure, by taking blood, spinal fluid and skin samples. Some are examined immediately, others frozen for future study. For now, says Jeffrey's physician, Richard Meade III, doctors confronted by a youngster with odd symptoms might "think of Kawasaki." -

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