ENFERMEDAD DE KAWASAKI: CURSO CLÍNICO EN PACIENTES PEDIÁTRICOS TRATADOS EN EL HOSPITAL MATERNO INFANTIL DE ISSEMYM EN EL PERIODO DE 1 DE ENERO DE 2004 A 30 DE OCTUBRE DE 2012

La enfermedad de Kawasaki (EK) es una vasculitis multisist̩mica de etiologíadesconocida que se presenta con mayor frecuencia en niños menores de 5 años y afecta en forma predominante a las arterias de mediano calibre repercutiendo en diferentes órganos y tejidos

Childhood-onset of primary Sjogren's syndrome:phenotypic characterization at diagnosis of 158 children

Objectives. To characterize the phenotypic presentation at diagnosis of childhood-onset primary SS.Methods. The Big Data Sjogren Project Consortium is an international, multicentre registry using worldwide data-sharing cooperative merging of pre-existing clinical SS databases from the five continents. For this study, we selected those patients in whom the disease was diagnosed below the age of 19years according to the fulfilment of the 2002/2016 classification criteria.Results. Among the 12083 patients included in the Sjogren Big Data Registry, 158 (1.3%) patients had a childhood-onset diagnosis (136 girls, mean age of 14.2years): 126 (80%) reported dry mouth, 111 (70%) dry eyes, 52 (33%) parotid enlargement, 118/122 (97%) positive minor salivary gland biopsy and 60/64 (94%) abnormal salivary US study, 140/155 (90%) positive ANA, 138/156 (89%) anti-Ro/La antibodies and 86/142 (68%) positive RF. The systemic EULAR Sjogren's syndrome disease activity index (ESSDAI) domains containing the highest frequencies of active patients included the glandular (47%), articular (26%) and lymphadenopathy (25%) domains. Patients with childhood-onset primary SS showed the highest mean ESSDAI score and the highest frequencies of systemic disease in 5 (constitutional, lymphadenopathy, glandular, cutaneous and haematological) of the 12 ESSDAI domains, and the lowest frequencies in 4 (articular, pulmonary, peripheral nerve and CNS) in comparison with patients with adult-onset disease.Conclusions. Childhood-onset primary SS involves around 1% of patients with primary SS, with a clinical phenotype dominated by sicca features, parotid enlargement and systemic disease. Age at diagnosis plays a key role in modulating the phenotypic expression of the disease.</p