Kawasaki disease in Sicily: a 7 year survey

Background Scant reports concerning Kawasaki Disease (KD) in Sicily have been published. Aim: to collect data from a 7-year survey. Methods: the charts of children discharged with the diagnosis of KD since January 2000 to December 2007, from the paediatric Units of 5 tertiary level Hospitals in Sicily were retrospectively reviewed. Data were collected through a questionnaire; gender, age at onset, ancestry, seasonality, ESR, CRP, PTL count, clinical manifestations, medication and cardiac abnormalities during the acute phase and up to 48 month follow-u were recorded. Results: 98 Caucasian children (55 M, 43 F, mean age at onset 36 mths), were diagnosed; 88/98 fulfilled the criteria while 10/98 had the incomplete form. The M: F ratio was 1.3: 1. 85% were children aged 36–40 months and 15% infants. Most cases occurred in August. The typical fever was present in 100%, conjunctivitis and exanthema in 98%, mucositis and extremity changes in 89%, and cervical lymphoadenopathy in 79% of patients. 87/98 pts had received timely IVIG; 4 patients required a second infusion. Cardiac abnormalities developed in 10 pts (6 ectasia and 4 aneurysms) all in the group with delayed therapy; 3/4 were giant aneurysms, all in infants. Three patients in addition to CAA displayed peripheral artery involvement. At 4-yr follow-up all CAA normalized except for 3 giant CAA that regressed to dilatations.