AHA president Dr. Elliott Antman and Dr. Tomisaku Kawasaki

AHA president Dr. Elliott Antman and Dr. Tomisaku Kawasaki

Dr. Tomisaku Kawasaki first described the mysterious disease in 1967. For a decade, he called it GOK, for “God only knows.”

The cause remains unknown today, but researchers believe the disease – now known as Kawasaki Disease — may be an immune response to an acute infectious illness based in part on genetic susceptibilities.

Kawasaki, who turns 90 years old this week, welcomed about 400 doctors, nurses and scientists from around the world to Honolulu this week, where they shared information and heard the latest research about Kawasaki Disease, which targets infants and young children and is characterized by acute inflammation of blood vessels.

It is one of the leading cause of acquired heart disease in children.

Nearly 100 patients and families affected by KD also are registered for the 11th International Kawasaki Disease Symposium, held every three years since 1984 in Japan, the United States or Taiwan.

“Certainly as clinicians we are beholden to the patients and the families,” said Dr. Brian McCrindle, one of two program chairs for the symposium and a cardiologist at The Hospital for Sick Children in Toronto.0204-Feature-Kawaski_Blog 01

“When we think about this disease, we always have to keep them in the back of our head, and they are an important part of the program.”

The four-day conference featured discussions and presentations on the influence of genetics on the outcomes of the disease; novel therapies to prevent artery damage; the use of serum biomarkers for diagnosis and prognosis; and the use of newer imaging techniques to gauge abnormalities in coronary arteries and heart muscle.

Japan has more incidents of KD than any other countries, but about 2,000 to 4,000 cases are reported annually in the United States, according to the Centers for Disease Control and Prevention. Symptoms include a red-colored tongue; swollen, cracked lips; conjunctivitis in the eyes without discharge; and a high, prolonged fever.

 

It can be treated with an injection of immunoglobulin, a blood product containing antibodies. But without treatment, up to one in five children infected with Kawasaki disease will develop arterial aneurysms, a bulge in the wall of the artery that can weaken it, and permanent heart disease. Infants younger than 1 year old are usually the most seriously ill and are at greatest risk for heart involvement. The acute phase of Kawasaki disease commonly lasts 10 to 14 days or more. Rarely, complications can include heart attacks which can be fatal.

A writing group is currently working on updating the AHS’s Scientific Statement about Kawasaki Disease. It’s been 10 years since the last statement, guidelines for diagnosis and management of the illness, was issued.

In his opening remarks at the symposium, American Heart Association President Dr. Elliott Antman recalled the strides made during the AHA’s last 90 years and its work in not only reducing heart disease but in creating a culture of health.

“So as we unravel the mysteries of Kawasaki disease and refine our strategies for preventing non-communicable diseases, it’s important to remember that the success of associations such as the AHA is proof that there is hope,” he said, “despite concerns over what we don’t know or obstacles we may face.”