Drugs Effective Against Children’s Kawasaki Syndrome : New Treatment Urged for Disease

Large doses of gamma globulin combined with aspirin dramatically decrease life-threatening coronary abnormalities in youngsters suffering from Kawasaki syndrome, a disease of mysterious origin with a high incidence among children of Japanese and Korean descent, researchers reported Wednesday.

“We recommend that this therapy be adopted for the treatment of children in the early stage of this disease as soon as possible after diagnosis,” said Dr. Fred Rosen of the department of pediatrics at Children’s Hospital in Boston. “It is the only therapy which has been proven effective. . . .”

Scientists at eight institutions, including USC and the University of California, San Diego, studied two groups of children. In one group, 75 children were given gamma globulin--a mixture of antibodies derived from human blood--and aspirin; in the other, 78 children were given aspirin alone, the conventional treatment.

Groups Showed Differences

After two weeks, coronary abnormalities were seen in 23% of the children in the aspirin group, contrasted with 8% in the gamma globulin group. After seven weeks, abnormalities were present in 18% of the aspirin group, contrasted with 4% of the gamma globulin group.

Researchers said that children given the gamma globulin with aspirin “were one-fifth as likely to have coronary abnormalities as those treated with aspirin alone.”

The findings were reported in this week’s New England Journal of Medicine and released at simultaneous press conferences here and in Los Angeles.

An accompanying editorial in the Journal suggested that additional studies be conducted before the treatment becomes routine. Noting that gamma globulin costs $1,500 to $3,000 per patient, the editorial recommended that physicians try to identify those at highest risk of heart damage and administer the treatment only to them.

But Dr. Jane W. Newburger, assistant professor of pediatrics at Harvard University and one of the lead researchers, said that giving gamma globulin to every victim of Kawasaki syndrome would be less expensive than treating those who suffer heart damage.

Origin of Disease Unknown

Kawasaki syndrome, the single major cause of acquired heart disease in children in the United States, is believed to be caused by an unknown microbial agent--an as-yet-unidentified bacteria, virus or parasite.

Researchers, noting that reporting of the syndrome is sporadic in this country, said that an estimated 2,000 to 5,000 new cases occur in the United States each year. Dr. Alan Rauch, a medical epidemiologist with the federal Centers for Disease Control, said that since July, 1976--when reporting of Kawasaki syndrome began--more than 2,000 cases have been reported. But “they represent a fraction of the total cases that occur,” he said.

In the United States, the syndrome has occurred in localized outbreaks, most recently in Los Angeles from October through February, when about 40 children were afflicted. Other cities with recent outbreaks include Honolulu, Boston, Denver and Chicago.

Because of the large Asian population in Southern California, Childrens Hospital of Los Angeles was a key center in the national study. “It has become a significant illness here in the last 10 years,” said Dr. Wilbert H. Mason, an infectious disease expert.

70,000 Cases in Japan

While most of the children affected in this country are white, the incidence of disease is highest among children of Japanese and Korean ancestry. Blacks, Latinos and other Asians have an “intermediate” incidence, researchers said, while whites are “the least susceptible.”

In Japan, more than 70,000 cases have been reported, the researchers said.

The illness, first described in 1967 by Dr. Tomisaku Kawasaki of the Japan Red Cross Hospital, usually affects children under age 5 and begins with a high fever.

Other signs include red, cracked lips, a “strawberry” tongue, redness of the throat, swelling in the hands and feet, red-purple discoloration of the palms and soles, peeling of the fingertips and toes, and a deep red rash that can resemble either hives, measles or scarlet fever.

Permanent Complications

“Of all the complications of Kawasaki syndrome, only the cardiac ones can become permanent,” said Dr. Marian E. Melish, professor of pediatrics at the University of Hawaii, who first identified the syndrome in the United States. The heart complications include heart valve damage, aneurysms--or swelling--of the coronary arteries, and fatal or non-fatal coronary artery thrombosis, leading to heart attacks.

About 30% of those stricken with Kawasaki syndrome will suffer heart disease; 20% of those will develop coronary aneurysms. A small proportion of the group with aneurysms, Melish said, “are subject to heart attacks,” which “are usually fatal.”

Times medical writer Harry Nelson in Los Angeles also contributed to this story.