The Harada score in the US population of children with Kawasaki disease.

OBJECTIVE: To describe and quantify the presentations of Kawasaki disease (KD) in a children's hospital over 10 years to assess the Harada score in a US population.

METHODS: A retrospective chart review from 2001 to 2011 of children discharged from Cleveland Clinic with the diagnosis of KD. Demographic and clinical data were collected and Harada scores were derived to evaluate efficacy in predicting risk for coronary artery aneurysms (CAAs).

RESULTS: A total of 105 children met diagnostic criteria for KD, and 97 of 105 had long-term follow-up. Full criteria for KD were found in 67 of 105 (64%); 38 had incomplete presentations. CAA developed in 10 children, 5 during follow-up despite treatment with intravenous immunoglobulin (IVIG.) Children with incomplete presentations had a higher risk of developing CAA (20% vs 5%, P = .03) and a delayed diagnosis (median days from fever to diagnosis 8.0 vs 5.0 days, P < .001). Of children who developed CAA, 9 of 10 had a positive Harada score (sensitivity of 90%). All children who developed CAA after IVIG were in the high-risk group, but 1 child with an incomplete presentation who had a CAA at presentation was missed by the score. Overall, the negative predictive value was 98%.

CONCLUSIONS: As in Japanese studies, a positive Harada score in a US population could be used to identify a high-risk population for CAA development. All children who developed CAA after treatment with IVIG would have been assigned to a high-risk category. Though not specific enough to select initial therapy, the score might be useful in identifying high-risk children for evaluation of new therapies and more frequent follow-up.