Background: Japanese nationwide survey reported that Down syndrome (DS) is less-frequently occurring comorbidity in Kawasaki disease (KD). Thus, no studies have focused treatment response and risk for coronary artery abnormalities (CAAs) in KD with DS. The aim of this study was to evaluate clinical manifestations, treatment response and incidence of CAAs in KD with DS.
Methods: We retrospectively reviewed the medical records of KD with DS from 2005 through 2012. Data were collected according to survey questionnaires from 16 hospitals.
Results: The response rate was 80% and the survey questionnaires from 16 KD patients (11 boys and 5 girls) with DS were collected. Age at diagnosis was 3 years (8 months to 12 years). Eight children (50%) were diagnosed incomplete KD. Five children had previous history of repaired congenital heart disease (AVSD 2, VSD+ASD 1, ASD 2, PDA 1). Of all, 8 children were classified as high risk group based on Kobayashi score. Twelve children received IVIG and 3 children were treated with only high dose aspirin. All 15 children were responded to initial treatment. In the remaining one girl with incomplete KD, the clinical symptoms spontaneously resolved. CAAs were not detected by echocardiography during follow-up.
Conclusions: All DS children with KD were responded to initial IVIG or aspirin therapy despite the high risk of IVIG resistance and none of children had CAAs. Therefore, our finding suggested DS is not a risk factor for IVIG resistance and developing CAAs in KD.