Publications by authors named "Ummusen Kaya Akca"
A rare disease with many faces: A multicenter registry of IgG4-related disease in children.
Rheumatology (Oxford)
September 2024
Article Synopsis
- - The study aimed to describe pediatric IgG4-related disease (IgG4-RD), analyze disease patterns, and assess the effectiveness of new diagnostic criteria across multiple centers. - Researchers collected data from 35 children diagnosed with IgG4-RD, finding that eye issues were the most common symptoms, and identified three distinct disease clusters among the patients. - The findings revealed that the 2020 revised comprehensive diagnostic criteria were significantly more effective than the 2019 ACR/EULAR classification criteria for diagnosing this disease in children.
Objective: Systemic lupus erythematosus (SLE) constitutes an autoimmune disorder with potential involvement of the gastrointestinal system (GIS). Our objective was to assess the gastrointestinal (GI) manifestations in patients diagnosed with childhood onset SLE.
Methods: The study cohort consisted of 123 patients with childhood onset-SLE and GIS involvement from 16 referral departments of pediatric rheumatology.
Objectives: The transition of adolescents and young adults (AYAs) from pediatric to adult-oriented healthcare may be affected by many factors, including the personal and cultural settings. We aimed to analyze the transition readiness and the factors affecting the transition success in rheumatology.
Methods: Patients older than 12 years were included in this prospective study.
Article Synopsis
- Drug-induced lupus erythematosus (DILE) is when kids develop lupus-like symptoms after taking certain medications, but it's not very common in children.
- In this study, four kids (three girls and one boy) were found to have DILE due to taking different drugs, and when the drugs were stopped, their symptoms went away completely.
- Researchers also looked at other cases and found that common signs of DILE include fever, joint pain, and rashes, and they discovered that many patients improved after stopping the medicine that caused the problem.
Article Synopsis
- This study compares Kawasaki disease (KD) and multisystem inflammatory syndrome in children (MIS-C) during the pandemic, focusing on how to distinguish between them.
- Findings show that KD patients are generally younger and exhibit specific symptoms like rashes and oral changes, whereas MIS-C patients tend to experience hypotension and gastrointestinal issues.
- Laboratory tests reveal that MIS-C patients have distinct blood count abnormalities and elevated inflammatory markers, leading to different treatment approaches, with biologic drugs used more frequently in MIS-C cases.*
Objective: In this study, we aimed to evaluate the characteristics of pediatric rhupus patients including all the related series in the literature.
Methods: Thirty pediatric patients with rhupus syndrome from 12 different centers in Turkey were included in this study. The literature was also reviewed for pediatric patients with rhupus syndrome.
Article Synopsis
- The study evaluated the effectiveness of two sets of classification criteria for Granulomatosis with Polyangiitis (GPA) in pediatric patients, comparing the ACR/EULAR criteria with the Ankara 2008 criteria.
- Data from 77 pediatric patients with GPA were analyzed, revealing high sensitivity (94.8%) and specificity (95.3%) for the Ankara criteria, while the ACR/EULAR criteria showed similar rates (89.6% sensitivity and 96.3% specificity).
- The results indicate that both classification criteria are reliable and perform similarly in diagnosing GPA in children.
Article Synopsis
- The study investigated the plasma levels and cell surface expression of TIM-3 and PD-1 checkpoint molecules in pediatric patients with chronic non-bacterial osteomyelitis (CNO).
- Researchers collected plasma samples from CNO patients during diagnosis and treatment and measured TIM-3 and PD-1 levels using specific assay techniques.
- Results showed lower plasma PD-1 levels in CNO patients compared to healthy controls, but no significant differences in cellular expression of TIM-3 and PD-1, suggesting T cell exhaustion may not be a significant factor in CNO and pointing to a need for further research.
Article Synopsis
- The study assesses the effectiveness and safety of biological drugs for treating systemic juvenile idiopathic arthritis (SJIA) to help enhance patient outcomes.
- A total of 147 SJIA patients were evaluated, with the majority receiving interleukin (IL)-1 blockers, and findings indicated that anakinra led to better disease management scores within three months.
- The research highlights that biological treatments, especially anti-IL-1 and anti-IL-6 drugs, significantly improve disease activity and reduce the occurrence of macrophage activation syndrome (MAS).
Objective: Prospective comparative effectiveness research (CER) in chronic nonbacterial osteomyelitis (CNO) is lacking. Our objectives were to (1) determine the use and safety of each consensus treatment plan (CTP) regimen for CNO, (2) assess the feasibility of using the Chronic Nonbacterial Osteomyelitis International Registry (CHOIR) data for CER, and (3) develop and validate a CNO clinical disease activity score (CDAS) using CHOIR.
Methods: Consenting children or young adults with CNO were enrolled into CHOIR.
Multisystem inflammatory syndrome in children (MIS-C) is a serious condition characterized by excessive inflammation that can arise as a complication of SARS-CoV-2 infection in children. While our understanding of COVID-19 and MIS-C has been advancing, there is still uncertainty regarding the optimal treatment for MIS-C. In this study, we aimed to compare the clinical and laboratory outcomes of MIS-C patients treated with IVIG plus corticosteroids (CS) to those treated with IVIG plus CS and an additional biologic drug.
View Article and Find Full Text PDFObjective: Children with suspicious complaints of rheumatic diseases are generally referred to a pediatric rheumatologist. We aimed to evaluate the profile of patients referred to the pediatric rheumatology unit and were not diagnosed with a rheumatic disease and to assess the impact of the coronavirus disease-2019 pandemic on referral complaints.
Materials And Methods: All new outpatients who applied to the pediatric rheumatology department between March 2019 and February 2021 and were not diagnosed with rheumatic disease were included.
Article Synopsis
- Cogan's syndrome (CS) is a rare disease that causes eye problems and hearing issues, often getting worse over time.
- Two kids with CS were treated and one improved with a cochlear implant, while the other felt better after treatment.
- A study found that most kids with CS had sudden hearing loss and eye symptoms, and doctors from different specialties should work together to help these patients.
Objective: COVID-19-associated pediatric vasculitis, other than Kawasaki disease (KD)-like vasculitis in multisystem inflammatory syndrome in children (MIS-C), is very rare. This study sought to analyze the characteristics, treatment, and outcomes in patients with COVID-19-associated pediatric vasculitis (excluding KD-like vasculitis in MIS-C).
Methods: The inclusion criteria were as follows: 1) age <18 years at vasculitis onset; 2) evidence of vasculitis; 3) evidence of SARS-CoV-2 exposure; and 4) ≤3 months between SARS-CoV-2 exposure and vasculitis onset.
Objective: The aim of the study was to evaluate the allergic rhinitis severity, to identify risk fac- tors associated with asthma, and to determine the frequency of comorbid conditions in allergic rhinitis patients with positive skin prick test.
Materials And Methods: Clinical characteristics of pediatric patients with allergic rhinitis were investigated. The frequency of comorbidities and risk factors for asthma development were investigated.
Pediatric primary antiphospholipid syndrome (APS) is a very rare disease with significant distinctions from the APS in adults. Herein, we present our experience in the diagnosis and treatment of six pediatric primary APS patients, who met the updated Sapporo criteria for the APS diagnosis. One of them was also diagnosed as having probable catastrophic APS (CAPS) due to the involvement of three different organ systems simultaneously.
View Article and Find Full Text PDFObjectives: The lower extremity venous wall thickness (VWT) of Behçet's disease (BD) patients was reported to be significantly increased in adults, suggesting its use for the support of BD diagnosis. This prospective study aimed to investigate the lower extremity VWT in childhood-onset definite and incomplete BD patients and compare it to healthy age-matched controls.
Methods: Paediatric patients classified with BD according to the 2015 international paediatric BD criteria in our centre were included in the study.
Background: Coronavirus disease 19 (COVID-19) may have a severe course in children. Multisystem inflammatory syndrome in children (MIS-C) is the post-COVID complication characterized by an exaggerated inflammation, observed in children. However, data on the underlying pathophysiology are sparse.
View Article and Find Full Text PDFArticle Synopsis
- A study examined the experiences of seven children with Takayasu arteritis (TA) who were treated with biologics such as adalimumab or tocilizumab.
- The patients, mostly young females, initially received steroids or combined treatments, but switched to biologics when their conditions improved.
- Both biologics showed effectiveness in managing TA, but further research through controlled trials is necessary to compare their efficacy.
Periodic fever, aphthous stomatitis, pharyngitis, and adenitis (PFAPA) syndrome is an autoinflammatory recurrent fever syndrome that mainly affects children. Probiotics are currently used to prevent upper respiratory tract infections and flares of diseases associated with immune dysregulation. We aimed to evaluate the response to probiotic treatment in PFAPA patients.
View Article and Find Full Text PDFObjective: To evaluate the course of coronavirus-19 (COVID-19) infection in paediatric familial Mediterranean fever (FMF) patients and to investigate the risk factors for COVID-19 infection.
Methods: Medical records of 100 consecutive paediatric FMF patients and their COVID-19 infection status were evaluated. Age- and gender-matched control group consisted of 51 patients with positive results for severe acute respiratory syndrome coronavirus 2.