Significant number of pediatric physicians fail to diagnose Kawasaki disease

Researchers at the University of California, San Diego School of Medicine report in the August 10 issue of Pediatric Infectious Disease Journal that a significant number of pediatric physicians fail to diagnose Kawasaki disease in children younger than six months and older than eight years. This childhood disease is reported in about 5,000 children a year in the United States.

First author, Pia Pannaraj, M.D., UCSD pediatric resident, said a previous study showed that delayed diagnosis of Kawasaki disease was a significant risk factor in the development of coronary abnormalities that can lead to heart muscle damage and deadly aneurysms.

“The purpose of the current study was to understand the basis of the delayed diagnosis,” she said. “We wanted to know the cause so we could help make recommendations to prevent the delay and the subsequent coronary problems that can result.”

The study’s senior author Jane Burns, M.D., Professor of Pediatrics, UCSD School of Medicine Department of Pediatrics, says the finding is significant because failure to diagnose and treat the disease at the extremes of the pediatric age range puts children at increased risk for coronary artery abnormalities and risk of heart attack later in life.

“Despite the availability of effective treatment for Kawasaki disease, children continue to needlessly suffer preventable coronary artery damage associated with the disease,” says Burns. “Numerous global studies have shown children can be at risk from as early as one month to their teens. General pediatricians and pediatric infectious disease specialists need to consider Kawasaki disease when examining all children with prolonged fever accompanied by rash or red eyes, regardless of the patient’s age.”

Tomisaku Kawasaki, M.D. of Japan, first diagnosed Kawasaki disease over 30 years ago. Kawasaki disease is characterized by inflammation of blood vessels throughout the body, and is accompanied by high fevers, rashes, bloodshot eyes, swelling of the hands and feet, redness of the mucous membranes in the mouth, throat and lips, and swollen neck lymph nodes. The disease and symptoms are treatable with gamma globulin. Full recovery can be made and heart damage prevented if treatment is begun within the first ten days. However in cases where children have the disease and do not receive treatment, up to 25 percent can develop lethal coronary artery problems.

Although researchers do not know the cause of Kawasaki disease they have discovered certain genetic backgrounds that affect KD susceptibility. The disease affects males almost twice as often as females. Kawasaki disease afflicts children of all races but physicians see it most often among children of Asian descent. The highest occurrences of the disease are reported in the winter and spring months with a second smaller peak in mid-summer.

For this study, Pannaraj and colleagues, Christena Turner, Ph.D., UCSD Department of Sociology, and John Bastian M.D., Director of Immunology at Children’s Hospital and Health Center - San Diego, sent a questionnaire to general pediatricians and pediatric infectious disease specialists listed in the American Academy of Pediatrics Membership Directory for San Diego County, the physician directories for San Diego’s five major healthcare systems and the Pediatric Infectious Disease Society Membership Directory. Of the 227 general pediatricians and 651 pediatric infectious disease physicians contacted for the survey, 58.1% of pediatricians and 53% of pediatric infectious disease physicians returned the questionnaires.

Of the general pediatricians from San Diego County who responded, 57.3% did not consider a Kawasaki disease diagnosis in children under six months of age, and 51.6% did not consider the disease in children older than eight. Of the pediatric infectious disease specialists who answered 26.5% did not consider KD in their diagnosis of children less than six months and 25% did not consider it in children over eight years of age.

According to Burns, the San Diego County Health Department Epidemiology Unit has documented 318 patients diagnosed with KD from January 1, 1998 to December 31, 2003. Of these patients, 8.3% were under 6 months of age and 18.1% were older than five years.

For more information visit the UCSD KD Research Program website at http://www-pediatrics.ucsd.edu/kawasaki or the KD Foundation website at www.kdfoundation.org.