Abstract
Although developed as an epidemiological tool, the Kawasaki Disease (KD) case definition has become a clinical tool used to detect and treat children with KD. Based on our experience treating 103 cases at Children's Hospital, San Diego from January 1998 through December 2000, we found that the Center for Disease Control (CDC) KD case definition results in failure to detect children with coronary artery abnormalities (CAA). Using published nomograms for coronary artery diameters based on body surface area, we determined the prevalence of CAA in all children receiving IVIG treatment for Kawasaki disease at Children's Hospital, San Diego. There were 102 patients with one recurrence. 86/103 cases met the Center for Disease Control case definition (typical KD) and 17/103 did not (atypical KD). CAA occurred in 42/86(49%) typical KD cases and 10/17 atypical KD cases (59%). These data demonstrate that 50%(52/103) of KD patients have detectable CAA. Five patients developed coronary aneurysms- 2 in the atypical group (12%) and 3 in the typical group (3.5%). The sensitivity of the KD case definition for detecting children with CAA was only 0.81(42/42+10). With this level of sensitivity for CAA detection, reliance on the CDC case definition as the sole clinical tool for diagnosis results in physician failure to detect children with CAA. Because there is an effective treatment, clinicians need a case definition that is highly sensitive. As the treatment is relatively safe, specificity of the case definition is less important. Efforts to revise the clinical case definition in order to improve its sensitivity for CAA are warranted. New criteria may include clinical laboratory findings such as leukocyte count or ESR. Revisions may eliminate current criteria such as cervical lymphadenopathy. Candidate clinical case definitions should undergo prospective testing with CAA detection as the primary outcome.
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Bastian, J., Kushner, H., Miller, E. et al. Sensitivity of the Kawasaki Case Definition for Detecting Coronary Artery Abnormalities. Pediatr Res 53, 163 (2003). https://doi.org/10.1203/00006450-200301000-00060
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DOI: https://doi.org/10.1203/00006450-200301000-00060