Convergence and divergence in Kawasaki disease and multisystem inflammatory syndrome in children: results from the COVASAKI survey.

OBJECTIVES: To compare Kawasaki disease (KD) and multisystem inflammatory syndrome (MIS-C) in children. METHODS: Prospective collection of demographics, clinical and treatment data. Assessment of type 1 interferon (IFN) score, CXCL9, CXCL10, Interleukin (IL)18, IFNγ, IL6, IL1b at disease onset and at recovery. RESULTS: 87 patients (43 KD, 44 MIS-C) were included. Age was higher in MIS-C compared to KD group (mean 31±23 vs. 94±50 months, p<0.001). Extremities abnormalities (p=0.027), mucosal involvement (p<0.001), irritability (p<0.001), gallbladder hydrops (p=0.01) and lymphadenopathy (p=0.07) were more often recorded in KD. Neurological findings (p=0.002), gastrointestinal symptoms (p=0.013), respiratory involvement (p=0.019) and splenomegaly (p=0.026) were more frequently observed in MIS-C. Cardiac manifestations were higher in MIS-C (p<0.001), although coronary aneurisms were more frequent in KD (p=0.012). In the MIS-C group, the multiple linear regression analysis revealed that a higher IFN score at onset was related to myocardial disfunction (p<0.001), lymphadenopathy (p=<0.001) and need of ventilation (p=0.024). Both CXCL9 and CXCL10 were related to myocardial disfunction (p<0.001 and p=0.029). IL18 was positively associated to PICU admission (0.030) and ventilation (p=004) and negatively associated to lymphadenopathy (0.004). IFNγ values were related to neurological involvement and lymphadenopathy (p<0.001), IL1b to hearth involvement (0.006). A negative correlation has been observed between IL6 values, heart involvement (p=0.013) and PICU admission (p<0.001). CONCLUSIONS: The demographic and clinical differences between KD e MIS-C cohorts confirm previous reported data. The assessment of biomarkers levels at MIS-C onset could be useful to predict a more severe disease course and the development of cardiac complications.

[Current incidence of congenital heart disease diagnosed in the first year of life: results of a 20-year registry with one-year follow-up and comparison with the literature]. / Incidenza attuale delle cardiopatie congenite diagnosticate nel primo anno di vita: risultati di un registro di 20 anni con follow-up ad un anno e confronto con la letteratura.

BACKGROUND: The epidemiological data on the incidence of congenital heart defects derive from retrospective registries based on birth discharge codes with methodological limits and different selection criteria. Our aim was to determine the actual incidence of congenital heart defects in the first year of life in a population of residents in a province of Tuscany, Italy. METHODS: This prospective study was conducted in 31 185 newborn residents in the province, enrolling a consecutive population throughout the first year of life and followed up at least for one year. The population cohort was controlled and merged with a retrospective research of the diagnostic codes derived from hospital discharge records of the region of Tuscany. RESULTS: A congenital heart disease was suspected in 10 167 newborns, 32.6% of all live births. Overall, 524 defects were diagnosed (5.2% of the exams), with an incidence at birth of 16.8/1000/year (M/F ratio 0.84). Isolated ventricular septal defects were 343 and 198 spontaneously closed within one year, therefore, the total number dropped to 326 with a one-year incidence of 10.35/1000/year. Besides ventricular septal defects, the most common defects were atrial septal defects (7.3%), followed by ductus arteriosus (4.2%), aortic coarctation (4%), pulmonary stenosis (3.3%) and tetralogy of Fallot (3.1%). CONCLUSIONS: The one-year inclusion period and follow-up allowed us to exclude those defects whose hemodynamic significance is not clear at birth, or are spontaneously reversible within the first year of life. Nevertheless, with the inclusion of defects not evident at birth, congenital heart defects are still common.