Andre confronts Kawasaki disease
When Maureen took her three-month-old boy home from a local hospital where he had been admitted for a puzzling fever, she believed there must be a better answer than the diagnosis given on discharge: "fever of unknown origin." So she brought her son, Andre, to Children's Memorial Hospital. "I knew the doctors at Children's could find the answer," she says. And they did.
"Kawasaki disease is now the leading cause of acquired heart disease in children," says Anne Rowley, MD.
The symptoms that Maureen described to the doctors at Children's Memorial — a fever, a rash, red eyes and mouth and the peeling skin — suggested Kawasaki disease, which was soon confirmed by several tests. What causes the unusual condition is unknown. It primarily strikes children under age 5 — boys more than girls. In most children, the symptoms disappear harmlessly within one to three weeks, but 25 percent develop heart disease that affects the coronary arteries , says Stanford Shulman, MD, head of the Division of Infectious Disease.
Dr. Shulman also directs the Children's Memorial Hospital's Center for Kawasaki Disease, which is part of Children's Memorial Research Center . He says that one of the young doctors in the center — Anne Rowley, MD — is currently doing some of most advanced research in the world on this condition. The heart condition caused by Kawasaki disease mimics the kind of a coronary artery disease that can lead to the heart attacks seen in adults except that blockage is not caused by a build-up of fatty deposits. Instead, says Dr. Rowley, it is caused by blood clots or scar tissue in the walls of the blood vessels that feed the heart.
By the time Andre was diagnosed, he already had severe cardiac effects, and at age 8 months, he had a coronary artery bypass at Children's Memorial Hospital by Constantine Mavroudis, MD. Andre is now doing well, however, "The one thing he can't do is sports," says his mother.
"Kawasaki disease is now the leading cause of acquired heart disease in children," says Dr. Rowley. "What begins the disease's course is an unknown common infectious agent — maybe a virus." She speculates that, perhaps for genetic reasons, a few of the many children exposed to it react with the classical heart-damaging results. Dr. Rowley is examining the immune system response to the illness, and using this information to find the agent that the body is trying to fight off. To do this, she is using advanced molecular biology. This involves taking DNA and RNA (a cell's basic genetic building blocks) from antibodies found in Kawasaki-affected heart tissue, and using electric current to force cells to take up the genetic material and produce Kawasaki disease-specific "synthetic antibodies."
These antibodies can then be used to find the agent they are targeting. Another aspect of the team's research is targeted toward understanding the processes involved in the weakening and disintegration of coronary artery walls, information that might someday also be valuable in understanding the development of aortic aneuryms in adults.
Dr. Shulman proudly points out that Dr. Rowley has two of the three existing grants from the National Institutes of Health for the study of Kawasaki disease. Heightened awareness of unusual conditions such as Kawasaki disease is typical of a first-rate pediatric teaching hospital such as Children's Memorial, where the treatment protocol is still regarded as the best available.
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