Calcinosis, a common complication in juvenile dermatomyositis (JDM), affects up to 40% of patients and can be associated with uncontrolled disease activity and morbidity.A 9-year-old female with a 17-month history of JDM presented with worsened bilateral knee pain.
View Article and Find Full Text PDFObjectives: The aim of the study was to establish an international multicenter registry to collect data on patients with Multisystem Inflammatory Syndrome in Children (MIS-C), in order to highlight a relationship between clinical presentation, age of onset and geographical distribution on the clinical outcome.
Study Design: Multicenter retrospective study involving different international societies for rare immunological disorders.1009 patients diagnosed with MIS-C between March and September 2022, from 48 centers and 22 countries were collected.
Background: Systemic autoinflammatory disorders (SAIDs) represent a growing spectrum of diseases characterized by dysregulation of the innate immune system. The most common pediatric autoinflammatory fever syndrome, Periodic Fever, Aphthous Stomatitis, Pharyngitis, Adenitis (PFAPA), has well defined clinical diagnostic criteria, but there is a subset of patients who do not meet these criteria and are classified as undefined autoinflammatory diseases (uAID). This project, endorsed by PRES, supported by the EMERGE fellowship program, aimed to analyze the evolution of symptoms in recurrent fevers without molecular diagnosis in the context of undifferentiated AIDs, focusing on PFAPA and syndrome of undifferentiated recurrent fever (SURF), using data from European AID registries.
View Article and Find Full Text PDFOrphanet J Rare Dis
July 2023
Background: Among the various numbers of different autoinflammatory diseases (AIDs), the absolute majority of them remains rare, with a single representative in large populations. This project, endorsed by PRES, supported by the EMERGE fellowship program, and performed in line with the Metadata registry for the ERN RITA (MeRITA), has the objective of performing a data synchronization attempt of the most relevant research questions regarding clinical features, diagnostic strategies, and optimal management of autoinflammatory diseases.
Results: An analysis of three large European registries: Eurofever, JIR-cohort and AID-Net, with a total coverage of 7825 patients from 278 participating centers from different countries, was performed in the context of epidemiological and clinical data merging.
Background: Tofacitinib is an oral Janus kinase inhibitor. This trial assessed the efficacy and safety of tofacitinib versus placebo in patients with polyarticular course juvenile idiopathic arthritis (JIA).
Methods: This double-blind, withdrawal phase 3 trial enrolled patients with polyarticular course JIA (extended oligoarthritis, rheumatoid factor-positive or rheumatoid factor-negative polyarthritis, or systemic JIA without active systemic features) aged 2 years to younger than 18 years, and was done at 64 centres of the Paediatric Rheumatology International Trials Organisation and Pediatric Rheumatology Collaborative Study Group networks in 14 countries.
Objective: The aim: To improve efficacy of the right ventricle functional condition evaluation in children with tetralogy of Fallot after surgical correction by estimation of instrumental markers of myocardial dysfunction.
Patients And Methods: Materials and methods: We completely examined 35 children with tetralogy of Fallot after their surgical correction at the age of 3 - 17 years. For all the patients was presented tissue doppler.
Objective: Introduction: Speed myocardial indicators and estimation of galectin-3 presented for estimation of myocardial dysfunction in children with congenital heart defects (CHD). The aim: Evaluate myocardial function while using tissue Doppler imaging (TDI) and galectin-3 in children with CHD after surgical correction.
Patients And Methods: Materials and methods: We examined 184 children.
Goal of the study - estimation of the transforming growth factor ß1 and monocyte chemoattractant protein-1 (MCP-1) in infants with pyelonephritis. It was found that pyelonephritis against the background of vesicoureteral reflux is accompanied by the high activity of the inflammatory process and increase of serum monocyte chemoattractant protein-1 levels (2.5 times higher than in children with primary pyelonephritis).
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