Publications by authors named "Ozen S"

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.

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Background/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.

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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.

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Article Synopsis
  • - The study focuses on improving transitional care for adolescents with juvenile idiopathic arthritis (JIA) as they move to adult healthcare systems, suggesting the need for tailored programs in each country.
  • - A Delphi study involving adolescents, parents, and clinicians was conducted to gather expert opinions and identify key components of transitional care, with significant participation in the initial surveys.
  • - While a consensus was reached on essential transitional care issues for JIA patients, further agreement is needed on practical applications of a final checklist to enhance care in Turkey.
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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.

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Objective: 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.

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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.

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  • - This study investigates the safety and effectiveness of the MiniMed™ 780G insulin pump in children under seven years old with type 1 diabetes, an age group where clinical results are less understood compared to older children.
  • - Researchers compared children using the MiniMed™ 780G with those using the MiniMed™ 640G and multiple-dose insulin therapies, measuring factors like daily insulin dose, HbA1c levels, and glucose metrics over time.
  • - Findings showed that the MiniMed™ 780G group had significantly improved glucose control, with lower Time Above Range and higher Time in Range metrics, and no severe hypoglycemic events, suggesting it is a safe and effective treatment for young children.
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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.

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  • The study aimed to explore gender differences in the phenotypical expression of Behçet's disease (BD) using data from the International AIDA Network Registry, focusing on damage index, disease manifestations, and cardiovascular risk.
  • A total of 1024 patients (567 males and 457 females) were examined, revealing that males had a significantly higher overall damage index and more frequent occurrences of uveitis and vascular involvement, while females showed higher instances of arthralgia, arthritis, and CNS involvement.
  • Key factors associated with major organ involvement included male gender, treatment with biologic agents, origin from endemic regions, and longer disease duration, indicating a more severe course of BD in males compared to females.
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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.

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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.

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Article Synopsis
  • A EULAR task force developed guidelines for educating young patients with juvenile-onset rheumatic and musculoskeletal diseases about managing physical activity and pain during their transition from pediatric to adult healthcare.
  • They formulated two overarching principles highlighting the need for personalized care and seven points to consider, emphasizing the importance of patient education for a smoother transition.
  • The guidelines are backed by moderate to weak levels of evidence and are intended to standardize care in Europe while encouraging further research and policy development in this area.
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Systemic 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.

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  • The study tested the effectiveness of a Turkish version of a virtual pediatric gait examination (v-pGALS) in a large group of children aged 4-18 during May and June 2022.
  • It compared the findings of v-pGALS with traditional hands-on physical exams, finding that v-pGALS had high sensitivity (92.6%) and perfect specificity (100%) for identifying musculoskeletal issues.
  • Parents and patients reported little to no discomfort and found the duration of the v-pGALS examination acceptable, indicating it is a practical tool for assessing children's musculoskeletal problems.
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Phantoms 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.

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Objectives: 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.

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  • Some experts and patients worked together to create a set of important data to help study childhood lupus, a disease that affects children.
  • They made two datasets: a Core Dataset with 46 necessary items and an Expanded Dataset with 26 extra items, to help gather more information.
  • This new information will help researchers around the world study childhood lupus better by using the same important facts and details.
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  • - The study focuses on Familial Mediterranean Fever (FMF), an autoinflammatory disease characterized by the activation of the pyrin inflammasome, potentially leading to a specific type of cell death called pyroptosis.
  • - Researchers analyzed plasma samples from 25 FMF patients, alongside 10 patients with PFAPA and 10 healthy controls, to investigate the cell-death mechanisms during FMF attacks and the attack-free period.
  • - Findings revealed that levels of specific proteins (sFasL and granzyme A) were significantly elevated during FMF attacks but returned to normal when the patient was attack-free, indicating a distinct inflammatory response not seen in the PFAPA group.
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  • NIFTP is a recently identified thyroid condition that is considered benign and has different clinical characteristics compared to invasive fvPTC.
  • A study comparing 20 patients (10 with NIFTP and 10 with fvPTC) found no significant differences in demographics or preoperative nodule sizes; however, the NIFTP group received no radioactive iodine treatment post-surgery, unlike the fvPTC group.
  • The results suggest that NIFTP can be managed effectively with observation and lobectomy alone, as it shows low risk for recurrence, indicating that more aggressive treatments like radioactive iodine are unnecessary.
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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).

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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.

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