KAWASAKI DISEASE IN SARDINIA: A SINGLE-CENTER EXPERIENCE

Background. Kawasaki disease (KD) is an acute systemic vasculitis affecting young children, potentially fatal if not promptly diagnosed and treated. No study exists on KD in the island of Sardinia. Objective. To investigate the incidence and characteristics of KD in North Sardinia. Methods. We performed a 6-year (2009-2014) cross-sectional analysis of KD records at the Pediatric Clinic, University of Sassari, Sassari, Italy. Results. Seventeen children (mean age= 28 ± 26 months; M=9) were identified, among whom 13 (76%) were aged below 5 years and the youngest was a 5-month-old baby. The overall incidence was 10/100.000, similarly to that reported in western countries, with no seasonality. The clinical records were available for 9 out of the 17 patients. Five of nine (56%) children (mean age 4.5 years; range 16 months-6 years) had complete KD at presentation, 3/9 (33%) had incomplete KD (23, 46 and 48 months; mean age 3.5 years) and only a 33-month-old boy had atypical KD (11%). Intravenous immunoglobulin (IVIG) therapy was given to all but one, because of the delayed diagnosis. A high risk for coronary aneurisms was detected in 3/9 children (33%), two of them with complete and one with atypical KD; all responded to IVIG. Conclusion. This is the first study on KD in a cohort of Sardinian children. An increased awareness among pediatricians that incomplete and atypical forms represent almost half of all forms of KD is essential to establish an early diagnosis and to prevent the worsening and cardiac complications of the disease.