Clinica Pediatrica, IRCCS “Burlo Garofolo”, Università di Trieste
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Key words: Kawasaki disease, Atypical and incomplete forms, Cardiac complication, Retrospective study
Aims: Kawasaki disease (KD), a systemic vasculitis of the childhood, represents the leading cause of acquired heart disease in developed countries. This study sought to assess clinical presentation, course, treatment options and prognosis of KD patients referred to Institute of Child Health, IRCCS “Burlo Garofolo”, Trieste, Italy.
Methods: This is a retrospective chart review study. All patients affected by KD, and referred to our hospital from 1988 to 2009, were enrolled. Medical records from onset to the end of follow-up were reviewed.
Results: Our population consisted in 45 patients (29 males, 16 females). The median age at diagnosis was 32 months (range 18-53) and 78% of patients presented within the first 5 years. Atypical and incomplete forms represented 14% and 18% respectively. A typical presentation was associated with older median age (75.8 months, SD=45.2; p=0.014) and diagnostic delay. 24% of patients developed cardiac complications, with high frequency of valvular involvement (18%). Coronary aneurism developed in 1 patient (2%). Older age, higher CRP levels, lack of response to the first IVIg infusion and IVIg dosage < 2 g/kg were associated with tendency to develop cardiac involvement, while no difference was found between classical and non classical forms.
Conclusions: A typical and incomplete forms represent one third of all KD forms; they are characterized by an older age at onset and diagnostic delay. Although the low incidence of coronary aneurisms, we report an uncommon high incidence of transient valvular involvement.
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