Objectives: Systemic Lupus Erythematosus (SLE) is an autoimmune disease characterized by multisystem, including neuropsychiatric, involvement. The nervous system is affected in 20-27 % of patients within approximately two years after diagnosis. This study aimed to examine neurocognitive impairment in childhood-onset SLE (cSLE) patients before the development of any neurological, psychiatric, or cognitive manifestations.
View Article and Find Full Text PDFBackground/aim: The transition from pediatric to adult-oriented care for individuals with juvenile-onset systemic lupus erythematosus (SLE) poses significant challenges. This study aimed to assess the outcomes of transitioning patients with juvenile-onset SLE from pediatric to adult-oriented care.
Materials And Methods: Patients with juvenile-onset SLE were included in the study.
Background: This study aimed to evaluate the effects of hydroxychloroquine on cardiac functions and left ventricular mass in patients with childhood-onset systemic lupus erythematosus (cSLE).
Research Design And Methods: Fifty patients with cSLE undergoing treatment with hydroxychloroquine underwent echocardiographic evaluation. All patients exhibited negative disease activity markers and were clinically in remission.
This study examines the role of waste pickers in individual waste separation behavior. Given the environmental and economic importance of waste separation, this behavior has been extensively studied, but the influence of waste pickers-who perform waste separation as a livelihood, potentially substituting for the voluntary efforts expected from individuals-has been overlooked. Using stratified random sampling in İzmir, Turkey, we collected data from 670 individuals to test our hypotheses via the factor score regression method.
View Article and Find Full Text PDFObjective: Antinuclear antibodies (ANA) staining patterns can provide useful information in systemic lupus erythematosus (SLE). In our study, we examined the frequency of ANA staining patterns in disease-related features in childhood-onset SLE patients.
Methods: ANA and its staining patterns were assessed in childhood-onset SLE patients.
Mevalonate kinase deficiency (MKD), a rare auto-inflammatory disorder, arises from mutations in the gene, disrupting isoprenoid biosynthesis, and affecting cellular processes. This comprehensive review provides an updated perspective on MKD, including its aetiology, pathogenesis, diagnostic modalities, and therapeutic strategies. Based on recent research and clinical advances, our objective is to bridge the knowledge gaps in the 2015 SHARE guidelines.
View Article and Find Full Text PDFJ Clin Res Pediatr Endocrinol
November 2024
Background/purpose: Our objective was to investigate real-world outcomes and treatment strategies in individuals affected by DADA2 using over 10-year period real-life experience.
Methods: This descriptive analysis encompassed all adult/pediatric patients with DADA2 from our Vasculitis Research Center prospective database. Patients on anti-TNF therapy have been specifically examined, analyzing the treatment's duration, indications, and outcomes.
Objective: We aimed to (1) evaluate the cardiac involvement, with a focus on myocarditis, in patients with Still disease included in the multicenter Autoinflammatory Disease Alliance (AIDA) Network Still disease registry; and (2) assess the predictive factors for myocarditis by deriving a clinical risk patient profile for this severe manifestation.
Methods: A multicenter observational study was established, in which consecutive patients with Still disease in the AIDA Network Still disease registry were characterized by cardiac involvement. Cardiac involvement was defined according to the presence of pericarditis, tamponade, myocarditis, and/or aseptic endocarditis.
Vasculitis in children represents a diverse group of diseases characterized by inflammation of blood vessels, which can lead to significant morbidity if not promptly recognized and managed. This review explores the epidemiology, pathophysiology, classification, and management of key pediatric vasculitides. Classification based on vessel size aids in diagnosis and treatment.
View Article and Find Full Text PDFSystemic autoinflammatory diseases are a group of disorders characterized by sterile episodes of inflammation resulting from defects in the innate immune system. In contrast to classical autoimmune diseases, where circulating autoantibodies and the adaptive immune system are involved, these conditions involve excessive presence of proinflammatory cytokines leading to inflammatory attacks. Excessive cytokine production, functional mutations in regulatory pathways, excessive interferon production, defects in the nuclear factor-kappa B signaling pathway, abnorARCHmal protein folding, and complement activation are the mechanisms leading to autoinflammatory diseases.
View Article and Find Full Text PDFPhantoms representing anatomical deformations are necessary to investigate and improve dynamic treatments. In this study, we aimed to produce a deformable liver phantom by simulating respiratory motion. The dynamically DEformable Liver Phantom (DELP) is designed to create a human-specific respiratory model and to produce synchronised, repeatable motion with this model.
View Article and Find Full Text PDFObjectives: Existing literature lacks data on a subgroup exhibiting psychiatric symptoms below the DSM-5 diagnostic threshold within differences of sexual development (DSD) cases. Our study aims to assess parental knowledge, attitudes toward DSD, and parental perceptions of emotional and behavioral states through a transdiagnostic perspective.
Methods: The study was conducted with a total of 35 parents of children with DSD.
Unlabelled: Systemic juvenile idiopathic arthritis (sJIA) and adult-onset Still's disease (AOSD) are considered the same disease, but a common approach for diagnosis and management is still missing.
Methods: In May 2022, EULAR and PReS endorsed a proposal for a joint task force (TF) to develop recommendations for the diagnosis and management of sJIA and AOSD. The TF agreed during a first meeting to address four topics: similarity between sJIA and AOSD, diagnostic biomarkers, therapeutic targets and strategies and complications including macrophage activation syndrome (MAS).
Rheumatology (Oxford)
September 2024
Objectives: We aimed to report the characteristics of pediatric IgG4-related disease (IgG4-RD) through a multicentre registry, to assess disease clusters, and to evaluate the performances of the 2019 American College of Rheumatology and European League Against Rheumatism (ACR/EULAR) classification criteria and the 2020 revised comprehensive diagnostic (RCD) criteria in this cohort.
Methods: Data of IgG4-RD patients in 13 pediatric rheumatology centers were recorded to a web-based registration system. The diagnosis of IgG4-RD was made according to the 2011 comprehensive diagnostic criteria.