Kawasaki Disease (KD) is an idiopathic rash/fever illness of early childhood. Coronary artery aneurysms (CAA) may develop in up to 25% of untreated children. Although KD has been diagnosed in children on every continent, Japan remains the country with the highest annual incidence rate at 130-140/100,000 children < 5 years of age. Rates for the continental U.S. have varied between 9 and 20/100,000 children < 5 years of age. The syndrome is named after the Japanese pediatrician Tomisaku Kawasaki who first described it in 1967. After more than 40 years of investigation, the etiology of KD remains elusive.

The morning session was held at Egleston Children’s Hospital on the Emory Campus, where Bastian, Burns, and Kushner presented a talk on the need for a new KD clinical case definition. Because the etiologic agent remains unknown, diagnosis of KD relies on observation and recognition of the clinical signs that comprise the KD case definition criteria. This approach has been successful in identifying and treating many children at risk for CAA. However, it also has delayed the effective treatment of children who failed to meet the KD case definition criteria, but who, nevertheless, developed CAA. The original case definition was developed prior to the general acceptance of CAA as sequelae of KD, the availability of the echocardiogram, and effective treatment with intravenous immunoglobulin (IVIG). Despite an evolution in awareness, detection, and treatment of possible CAA sequela, the case definition has not been altered so as to incorporate this knowledge. Burns, Bastian, and Kushner’s talk explored the transformation of the case definition from an epidemiological instrument to a diagnostic tool. They urged the construction of a more sensitive KD case definition that includes signs and laboratory findings associated with CAA.

Attendees at session “Looking for the Etiology of Kawasaki Disease” held in Emory’s Rollins School of Public Health.

The afternoon session, “Looking for the Etiology of KD,” was held at the Rollins School of Public Health. In 1998 the UCSD KD Center initiated an historical investigation of Kawasaki Disease in the hope that it would yield clues to its etiology. A team consisting of pediatricians, a pediatric immunologist, an epidemiologist, a medical historian, an anthropologist, and a film-maker was constituted. Three of the team members, Kushner, Burns, and Bastian, explored the unique interdisciplinary approach that developed and how it has resulted in some surprising preliminary results. The discussion also examined the utility of “applied history of medicine.”

More than 50 people across Emory and from the CDC attended the symposium One result of the meeting was the constitution of an interdisciplinary effort at Emory’s School of Medicine to parallel and cooperate with the UCSD effort. Led by Dr. Stephen Nesheim of Emory’s Department of Pediatrics Infectious Diseases, this group includes researchers from the Departments of Cardiology, Pediatrics Infectious Diseases, Pediatrics, Microbiology, and the Rollins School of Public Health.

Workshop speakers included Dr. John F. Bastion, UCSD, Dr. Jane C. Burns, UCSD and Dr. Howard Kushner, Emory University.