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Objective: to evaluate the incidence of coronary anomalies (CAA) and risk factors for coronary involvement in children with KD from 2000 through 2013 in Emilia-Romagna. Design: an 11-centers retrospective study was conducted using data from 132 patients diagnosed with KD. Results: during the acute phase, CAA developed in 13/132 pts (9.8%): 2 pts had isolated ectasia of right coronary artery (RCA), 10 pts had ectasia of left anterior descending CA (LAD), 1 pt had aneurysm of LAD, 1 pt had ectasia of circonflex CA (CX). During the subacute phase 14/117 pts (11.9%) presented CAA: 3 had aneurysm of LAD, 1 had aneurysm of LAD and RCA, 1 had isolated ectasia of RCA, 9 had ectasia of LAD. During the convalescent phase CAA were detected in 18/83 pts (21%): 3 had aneurysm of LAD and RCA, 2 isolated aneurysm of LAD, 1 had aneurysm of RCA, 12 ectasia of LAD. 3 out of 6 aneurysms detected in theconvalescent phase developed in the subacute stage. Univariate analyses identified WBC and RBC in the subacute phase, and PLT in chronic phase as predictors of chronic CAA (respectively p 0.04, 0.013 and 0.002). A multivariate logistic regression analysis revealed that only PLT of the chronic stage was predictor for chronic CAA. ROC curve for PLT identified a value superior to 652000/mm3 to be the cut off value for CAA, with an 72% sensitvity and 87% specificity. Incomplete or atipical clinical presentation was correlated with CAA (p 0.02). Younger age (< 1 yr) at diagnosis, seasonal distribution, IVIG responsivness, race, elevation of ALT, percentage of neutrophil, hemoglobin, CRP and ESR, albumin and sodium were not correlated with risk for CAL. Conclusions: atipical/incomplete clinical presentation, higher WBC and RBC in the subacute phase and PLT of the convalescent phase were predictors of CAA in our region, while blood exams of the acute stage, IVIG responsivness, sex and younger age were not.

We updated our regional database for KD patients from 2011 to 2014 to investigate differences in epidemiological, clinical and outcome data between 2011-2014 pts and those diagnosed between 2000-2010 in Emilia Romagna. We had 77 KD patients during last 4 years ( vs 81 pts during 2000-2010), mean age at diagnosis 40 months+- 36,4 (vs 31,2 mths ), 10% were younger than 6 months at diagnosis (vs 14%), male were 60 % (vs 59%). Seasonality was as follows: spring 27 (% vs 33%), winter 25% (vs 28%), autumn 25% (vs 26%), summer 23% (vs 14%). Clinical presentation was complete in 69 % (vs vs 74%), incomplete in 30% (vs 26%), atipical 1 % (vs 0%). IVIG responder were 77% (vs 67%), non responders 16 % (vs 12%), late treatment 2 % (vs 12% P 0.002), not treated with IVIG 5 % (vs 9%). 18/77 pts ( vs 7 of 2000-2010,P 0.004 ) had alteration of ALT ( v> 45 U/) and AST ( v > 55 U/l) and among those with transminases alteration 8 ( vs 4 pts) had cholestasis. Coronary arteries anomalies (CAA) were present in 15% ( 9 % ectasia and 6% (vs 2 %) aneurysms ( mean age 12,6 months, 2 pts non responders to standard therapy, 1 pts with late treatment, 1 pts not treated, and 1 pts well treated. The 2 patients who developed giant aneurysms were both non responder to standard therapy, 1 pts had associated cholestasis. Conclusion: during the last four years we observed an higher number of diagnosis of KD than 2000-2010; incomplete and atipical cases, and non-responder pts were more frequent but we noted a decreased of pts not treated or delayed treated; more cases of cholestasis associated to KD and an increased number of patients with aneurysms.