Pediatric Rheumatology最新文献

{"title":"The plasma metabolome of juvenile idiopathic arthritis varies according to subtype and underlying inflammatory status.","authors":"Jooa Kwon, Melanie R Neeland, Justine A Ellis, Jane Munro, Richard Saffery, Boris Novakovic, Toby Mansell","doi":"10.1186/s12969-024-01041-8","DOIUrl":"10.1186/s12969-024-01041-8","url":null,"abstract":"<p><strong>Background: </strong>Juvenile idiopathic arthritis (JIA) is challenging to classify and effectively monitor due to the lack of disease- and subtype-specific biomarkers. A robust molecular signature that tracks with specific JIA features over time is urgently required, and targeted plasma metabolomics may reveal such a signature. The primary aim of this study was to characterise the differences in the plasma metabolome between JIA patients and non-JIA controls and identify specific markers of JIA subtype. We also assessed the extent to which these signatures are due to underlying inflammation as assessed by glycoprotein acetyls (GlycA) and high-sensitivity C-Reactive Protein (hsCRP) levels.</p><p><strong>Methods: </strong>Targeted nuclear magnetic resonance (NMR) metabolomic profiles of plasma of 72 children with JIA and 18 controls were assessed cross-sectionally. Associations between 71 metabolomic biomarkers and JIA, JIA subtype, disease activity status, and inflammation markers (GlycA and hsCRP) were assessed using multivariable linear regression models.</p><p><strong>Results: </strong>JIA was associated with higher GlycA (mean difference = 0.93 standard deviations, 95% confidence interval = [0.370, 1.494], P_adj = 0.039) and docosahexaenoic acid (1.06, [0.51, 1.60], P_adj = 0.021), and lower acetate (-0.92, [-1.43, -0.41], P_adj = 0.024) relative to controls. This variation was largely driven by systemic JIA (sJIA), with 24 of 71 total biomarkers significantly different (P_adj <0.05) relative to controls. There were no specific differences identified in oligoarticular (oJIA) or polyarticular (rheumatoid factor positive or negative) JIA relative to controls. Despite being generally highly correlated with hsCRP (r > 0.70), GlycA, but not hsCRP, was positively associated with active disease in sJIA (0.22, [-0.40, -0.04], P_adj = 0.018), and 6 of 24 sJIA-associated markers were associated with GlycA levels. Only 1 sJIA-associated biomarker, histidine, was associated with hsCRP levels.</p><p><strong>Conclusion: </strong>Differences in the plasma NMR metabolomic profiles are apparent in children with sJIA, but not other JIA subtypes, relative to non-JIA controls. These findings suggest a potential utility for classifying and monitoring JIA through metabolomic profiling, with chronic inflammation, measured by GlycA, potentially playing a role in at least some of these metabolomic differences.</p>","PeriodicalId":54630,"journal":{"name":"Pediatric Rheumatology","volume":"22 1","pages":"113"},"PeriodicalIF":2.8,"publicationDate":"2024-12-30","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11686955/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142907925","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Aicardi-Goutières syndrome type 6: report of ADAR variant and clinical outcome after ruxolitinib treatment in the neonatal period.","authors":"Alba Gabaldon-Albero, Carla Martin-Grau, Miguel Marti-Masanet, Alejandro Lopez-Jimenez, Roberto Llorens, Beatriz Beseler-Soto, Sergio Martin-Zamora, Berta Lopez, Inmaculada Calvo, Sara Hernandez-Muela, Monica Rosello, Carmen Orellana, Francisco Martinez","doi":"10.1186/s12969-024-01036-5","DOIUrl":"10.1186/s12969-024-01036-5","url":null,"abstract":"<p><strong>Background: </strong>Aicardi-Goutières Syndrome is a monogenic type 1 interferonopathy with infantile onset, characterized by a variable degree of neurological damage. Approximately 7% of Aicardi-Goutières Syndrome cases are caused by pathogenic variants in the ADAR gene and are classified as Aicardi-Goutières Syndrome type 6. Here, we present a new homozygous pathogenic variant in the ADAR gene. Currently, Janus Kinase inhibitors have been proposed to treat selected interferonopathies such as Aicardi-Goutières Syndrome, although limited information is available on its use and results in the neonatal presentation of this disease.</p><p><strong>Case presentation: </strong>We present two siblings, a male neonate with congenital petechial rash, severe thrombopenia and generalized hypotonia and his deceased sister who had normal development until 5 months of age, when she suffered acute encephalopathy. We describe the clinical course, complementary examinations and follow-up with early treatment of the newborn with ruxolitinib. The homozygous variant c.2908G > A (p.Ala970Thr) in the ADAR gene was found in both siblings, parents were heterozygous carriers.</p><p><strong>Conclusions: </strong>The homozygous variant c.2908G > A (p.Ala970Thr) in the ADAR gene causes Aicardi-Goutières Syndrome type 6. Intrafamilial phenotypic spectrum of the disease varies among individuals with the same pathogenic variant. Early initiation of ruxolitinib improved systemic signs but did not prevent the progression of neurological disease.</p>","PeriodicalId":54630,"journal":{"name":"Pediatric Rheumatology","volume":"22 1","pages":"110"},"PeriodicalIF":2.8,"publicationDate":"2024-12-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11682636/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142900479","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Towards a histological diagnosis of childhood small vessel CNS vasculitis.","authors":"Maryam Nabavi Nouri, Anastasia Dropol, Pascal N Tyrrell, Sheila Sheikh, Marinka Twilt, Jean Michaud, Benjamin Ellezam, Harvey B Sarnat, Christopher Dunham, Peter W Schutz, Julia Keith, David G Munoz, Harry V Vinters, Cynthia Hawkins, Susanne M Benseler","doi":"10.1186/s12969-024-01053-4","DOIUrl":"10.1186/s12969-024-01053-4","url":null,"abstract":"<p><strong>Background: </strong>Primary small vessel CNS vasculitis (sv-cPACNS) is a challenging inflammatory brain disease in children. Brain biopsy is mandatory to confirm the diagnosis. This study aims to develop and validate a histological scoring tool for diagnosing small vessel CNS vasculitis.</p><p><strong>Methods: </strong>A standardized brain biopsy scoring instrument was developed and applied to consecutive full-thickness brain biopsies of pediatric cases and controls at a single center. Stains included immunohistochemistry and Hematoxylin & Eosin. Nine North American neuropathologists, blinded to patients' presentation, diagnosis, and therapy, scored de-identified biopsies independently.</p><p><strong>Results: </strong>A total of 31 brain biopsy specimens from children with sv-cPACNS, 11 with epilepsy, and 11 with non-vasculitic inflammatory brain disease controls were included. Angiocentric inflammation in the cortex or white matter increases the likelihood of sv-cPACNS, with odds ratios (ORs) of 3.231 (95CI: 0.914-11.420, p = 0.067) and 3.923 (95CI: 1.13-13.6, p = 0.031). Moderate to severe inflammation in these regions is associated with a higher probability of sv-cPACNS, with ORs of 5.56 (95CI: 1.02-29.47, p = 0.046) in the cortex and 6.76 (95CI: 1.26-36.11, p = 0.025) in white matter. CD3, CD4, CD8, and CD20 cells predominated the inflammatory infiltrate. Reactive endothelium was strongly associated with sv-cPACNS, with an OR of 8.93 (p = 0.001). Features reported in adult sv-PACNS, including granulomas, necrosis, or fibrin deposits, were absent in all biopsies. The presence of leptomeningeal inflammation in isolation was non-diagnostic.</p><p><strong>Conclusion: </strong>Distinct histological features were identified in sv-cPACNS biopsies, including moderate to severe angiocentric inflammatory infiltrates in the cortex or white matter, consisting of CD3, CD4, CD8, and CD20 cells, alongside reactive endothelium with specificity of 95%. In the first study of its kind proposing histological criteria for evaluating brain biopsies, we aim to precisely characterize the type and severity of the inflammatory response in patients with sv-cPACNS; this can enable consolidation of this population to assess outcomes and treatment methodologies comprehensively.</p>","PeriodicalId":54630,"journal":{"name":"Pediatric Rheumatology","volume":"22 1","pages":"111"},"PeriodicalIF":2.8,"publicationDate":"2024-12-28","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11682624/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142900482","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Assessing the impact of the iPeer2Peer program for adolescents with juvenile idiopathic arthritis: a mixed-methods randomized controlled trial.","authors":"Fareha Nishat, Lauren Kelenc, Roberta Berard, Ciaran Duffy, Brian Feldman, Paula Forgeron, Adam M Huber, Nadia Luca, Heinrike Schmeling, Lynn Spiegel, Lori Tucker, Karen Watanabe-Duffy, Tieghan Killackey, Chitra Lalloo, Brittany Wiles, Anya Nair, Sofia Olaizola, Brenna McDermott, Farideh Tavangar, Sara Ahola Kohut, Jennifer N Stinson","doi":"10.1186/s12969-024-01052-5","DOIUrl":"10.1186/s12969-024-01052-5","url":null,"abstract":"<p><strong>Background: </strong>Juvenile Idiopathic Arthritis (JIA) is a chronic pediatric illness, whereby youth experience physical, emotional and psychosocial challenges that result in reduced health related quality of life (HRQL). Peer mentoring has been shown to improve disease self-management in adults with chronic conditions, with mixed results in younger populations. Building on our pilot work - which supported the feasibility and initial effectiveness of the iPeer2Peer program - the objective of this study was to assess the clinical effectiveness of the program in youth with JIA through a waitlist randomized controlled trial.</p><p><strong>Methods: </strong>Eighty-one youth (aged 12-18) were randomized to the intervention group and matched with trained peer mentors (18-25 years; successfully managing their JIA), completing of up to ten 30-min video calls over a 15-week period. Eighty-three youth in the control group received standard care. Outcome assessments occurred at enrollment, 15 weeks post randomization and 6-months post randomization. The primary outcome was self-management, measured using the TRANSITION-Q. Secondary outcomes were HRQL, pain, emotional distress, disease knowledge, self-efficacy, and perceived social support. These were assessed using linear mixed effects models. Content analysis of semi-structured interviews and focus groups was used to assess satisfaction with the program with mentors and mentees upon study completion.</p><p><strong>Results: </strong>In total, 164 youth (mean age 14.4 ± 1.9 years, 78% female) were randomized to the study. The proposed sample size was not reached due to challenges in recruitment, likely impacted by the COVID-19 pandemic. The iPeer2Peer program did not show significant improvement in self-management (p = 0.7), or any of the secondary outcomes. Three key categories emerged from content analysis: (1) Fulfillment and Support Through Shared Experience, (2) Enhancing Program Delivery and (3) Strategies to Boost Engagement. These findings highlight that mentees valued the ability to converse with mentors who empathized with their disease experience, while mentors found it fulfilling to support mentees, and noted that they could have benefited from this type of support themselves.</p><p><strong>Conclusion: </strong>While the iPeer2Peer did not result insignificant changes in clinical outcomes, both mentors and mentees were satisfied with the program and felt that mentorship provided real-world benefits for disease management and overall wellbeing.</p><p><strong>Trial registration: </strong>ClinicalTrials.gov, NCT03116763. Registered 31, March 2017, https://www.</p><p><strong>Clinicaltrials: </strong>gov/study/NCT03116763.</p>","PeriodicalId":54630,"journal":{"name":"Pediatric Rheumatology","volume":"22 1","pages":"109"},"PeriodicalIF":2.8,"publicationDate":"2024-12-27","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11674074/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142900481","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Novel multiple Z-score models for detection of coronary artery dilation: application in Kawasaki disease.","authors":"Ho-Chang Kuo, Shih-Hsin Chen, I-Fei Chen, Wen-Ing Cheng, Shih-Feng Liu, Mindy Ming-Huey Guo, Yu-Chi Lin, Yi-Hui Chen","doi":"10.1186/s12969-024-01040-9","DOIUrl":"10.1186/s12969-024-01040-9","url":null,"abstract":"<p><strong>Background: </strong>This study aims to develop Z-Score models to normalize measurements of three coronary arteries and enhance the diagnosis of Kawasaki disease (KD) in children from newborns to 10 years old. Developing a reliable Z-Score model is challenging, as some existing models fail the normality test. Overcoming these challenges is crucial for improving KD diagnosis.</p><p><strong>Method: </strong>Detailed measurements of the left main coronary artery (LCA), left anterior descending coronary artery (LAD), and right coronary artery (RCA) were collected, along with patient demographics such as age, height, weight, and body surface area (BSA). Several Z-Score models, named the Kuo Z-Score models, were proposed, with separate designs for different coronary arteries and different age groups, resulting in multiple Z-Score models. The Z-Score model for the RCA employs the Box-Cox method for data transformation. Finally, we tested various age group combinations, selecting models that passed the Anderson-Darling normality test and had higher R-square values for robustness and best data fit.</p><p><strong>Results: </strong>The study included 1180 participants free from coronary or heart diseases. The Kuo Z-Score models were optimized for LCA, LAD, and RCA across the five age groups 0-6 years, 6-7 years, 7-8 years, 8-9 years, and 9-10 years. Only the normality test for the RCA in the 7-8 year age group failed. The proposed model fitted to the normality assumption outperforming the other models.</p><p><strong>Conclusion: </strong>The Kuo Z-Score models, applicable across a broad age range, provides robust identification of coronary artery dilatation and aneurysm in KD. The models' capability to normalize diverse data sets marks a significant advancement in KD diagnostic sensitivity, aiding in better clinical decision-making and potentially improving patient outcomes.</p>","PeriodicalId":54630,"journal":{"name":"Pediatric Rheumatology","volume":"22 1","pages":"108"},"PeriodicalIF":2.8,"publicationDate":"2024-12-21","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11662764/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142873526","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Thrombotic thrombocytopenic purpura with juvenile systemic lupus erythematosus: successful treatment with caplacizumab and rituximab.","authors":"Tadafumi Yokoyama, Takuya Mimura, Hiroki Tanaka, Ria Kasahara, Masaaki Usami, Yusuke Matsuda, Toshihiro Fujiki, Taizo Wada","doi":"10.1186/s12969-024-01049-0","DOIUrl":"10.1186/s12969-024-01049-0","url":null,"abstract":"","PeriodicalId":54630,"journal":{"name":"Pediatric Rheumatology","volume":"22 1","pages":"106"},"PeriodicalIF":2.8,"publicationDate":"2024-12-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11654370/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142856946","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Influenza knowledge and barriers to vaccination in immunosuppressed patients in the pediatric rheumatology clinic.","authors":"Julia G Harris, Leslie Favier, Jordan T Jones, Maria Ibarra, Michael J Holland, Emily Fox, Kelly Jensen, Ashley K Sherman, Ashley M Cooper","doi":"10.1186/s12969-024-01048-1","DOIUrl":"10.1186/s12969-024-01048-1","url":null,"abstract":"<p><strong>Background: </strong>Most patients with a pediatric rheumatic disease are at increased risk of influenza due to immunosuppressive medication use. Despite initial quality improvement efforts, our influenza vaccination rate plateaued at 72%, which prompted a survey of patients and families to assess provider vaccine recommendations, influenza knowledge, and barriers to influenza vaccination.</p><p><strong>Methods: </strong>Patients on immunosuppressive medication or their parent were eligible to complete a survey between July 2019 and January 2020. Survey questions assessed demographics, rheumatology diagnosis, immunosuppressive medication(s), influenza vaccination recommendation, patient/parent influenza knowledge, and barriers to influenza vaccination. Influenza vaccination rates for immunosuppressed patients were acquired each influenza season from 2015-2020 and tracked on a control chart.</p><p><strong>Results: </strong>Of the 226 completed surveys, 145 (64.2%) were completed by parents and 81 (35.8%) by patients. The majority (85%) reported the influenza vaccine was recommended. The most common reasons for not receiving the influenza vaccine included: worry about disease flare (25.6%), concern the vaccine will cause influenza (25.6%), and lack of vaccine effectiveness (20.5%). Parents (40.9%) were more worried about disease flare compared to patients (17%; p = 0.024). Most respondents were able to correctly answer fever, cough and/or congestion as the most common symptoms of influenza; however, 23% answered gastrointestinal symptoms and 10.2% joint swelling. Most respondents (95.1%) were aware that immunosuppressive medication increases risk of infection. The average weekly influenza vaccination rate for the 2019-2020 flu season was 85.5%, which increased from 72.0% the previous year. Parents with higher education status were more likely to have their child receive the influenza vaccine compared to parents with less education.</p><p><strong>Conclusions: </strong>This survey indicates that respondents understand the potential severity of influenza and the increased risk of infection due to immunosuppressive medication use; however, many inaccurately identified the most common symptoms of influenza and also reported misconceptions of influenza vaccine risks. The barriers identified in this survey will help drive future improvement efforts to increase influenza vaccination rates in this high-risk population.</p>","PeriodicalId":54630,"journal":{"name":"Pediatric Rheumatology","volume":"22 1","pages":"104"},"PeriodicalIF":2.8,"publicationDate":"2024-12-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11654255/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142856938","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"A Chinese child with both systemic lupus erythematosus coexisting with neuromyelitis optica spectrum disorder: a case report.","authors":"Rong-Xuan Hu, Yao Yao, Dan-Dan Xu, Yue-Qi Bao, Xun-Wei Liu, Guo-Qin Zhu, Guo-Min Li","doi":"10.1186/s12969-024-01045-4","DOIUrl":"10.1186/s12969-024-01045-4","url":null,"abstract":"","PeriodicalId":54630,"journal":{"name":"Pediatric Rheumatology","volume":"22 1","pages":"107"},"PeriodicalIF":2.8,"publicationDate":"2024-12-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11654328/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142856849","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Consensus on transition care for juvenile idiopathic arthritis: a Delphi study with youth, caregivers, and health professionals.","authors":"Nihal Şahin, Gülcan Özomay Baykal, Ceyhun Açarı, Pinar Özge Avar Aydın, Özge Baba, Esra Bağlan, Sevcan Bakkaloğlu, Sibel Bakırcı, Yelda Bilginer, Burcu Yücel Bozkaya, Şengül Çağlayan, Mustafa Çakan, Figen Çakmak, Taner Coşkuner, Ferhat Demir, Fatma Gül Demirkan, Şeyda Doğantan, Hatice Adıgüzel Dündar, Emine Duygu Ersözlü, Sercan Gücenmez, Oğuz Gürler, Rana İşgüder, Adem Küçük, Mukaddes Kalyoncu, Levent Kılıç, Sara Şebnem Kılıç, Hakan Kısaoğlu, Ayşenur Paç Kısaarslan, Zehra Kızıldağ, Duygu Kurtuluş, Semanur Özdel, Kübra Öztürk, Pelin Şenol, Ayşe Tanatar, Sema Nur Taşkın, Fatma Tuncer Kuru, Serkan Türkuçar, Kadir Ulu, Erbil Ünsal, Ayten Yazıcı, Ayşe Cefle, Deniz Gezgin Yıldırım, Selçuk Yüksel, Özgür Kasapçopur, Seza Özen, Nuray Aktay Ayaz, Hafize Emine Sönmez, Betül Sözeri","doi":"10.1186/s12969-024-01047-2","DOIUrl":"10.1186/s12969-024-01047-2","url":null,"abstract":"<p><strong>Background: </strong>The field of transitional care for chronic conditions in adolescents, notably juvenile idiopathic arthritis (JIA), is rapidly growing. Transitioning these patients to adult healthcare systems presents significant challenges in practical implementation. Consequently, it would be appropriate for each country to develop a transition program tailored to its specific infrastructure. To pursue this goal, a Delphi study was conducted to identify the key components of transitional care in JIA.</p><p><strong>Methods: </strong>Three panels and two rounds were held consisting of adolescents and young adults, parents, and clinicians (pediatric or adult rheumatologists). As a result, feedback on acceptance of the key statements of transitional care was obtained using the Delphi method.</p><p><strong>Results: </strong>Out of 102 contacted, 88 (86.3%) participants responded to the Round 1 survey, which included 48 clinicians, 20 youths, and 20 parents. In Round 2, the number of clinicians dropped to 29, while the number of youths and parents remained constant. Based on expert opinions, 29 statements were selected for the first round. Statements that received ≥ 70% approval in the first round advanced to the next round. Sixteen statements did not achieve ≥ 70% approval. Of the remaining, 12 were reviewed in the second round, while four were excluded.</p><p><strong>Conclusion: </strong>Although consensus has been reached on the basic transitional care issues for JIA patients, several issues still need to be agreed upon. Acceptance and applicability of the final 20-item checklist in clinical practice are critical for advancing JIA transition care in Turkey.</p>","PeriodicalId":54630,"journal":{"name":"Pediatric Rheumatology","volume":"22 1","pages":"105"},"PeriodicalIF":2.8,"publicationDate":"2024-12-18","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11654283/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142856901","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}

{"title":"Mere coincidence or an association? Case of juvenile idiopathic arthritis in a patient with Klinefelter syndrome.","authors":"Aditi Shaily, Michael Ryan, Mileka Gilbert","doi":"10.1186/s12969-024-01042-7","DOIUrl":"10.1186/s12969-024-01042-7","url":null,"abstract":"","PeriodicalId":54630,"journal":{"name":"Pediatric Rheumatology","volume":"22 1","pages":"103"},"PeriodicalIF":2.8,"publicationDate":"2024-12-05","publicationTypes":"Journal Article","fieldsOfStudy":null,"isOpenAccess":false,"openAccessPdf":"https://www.ncbi.nlm.nih.gov/pmc/articles/PMC11619603/pdf/","citationCount":null,"resultStr":null,"platform":"Semanticscholar","paperid":"142781679","PeriodicalName":null,"FirstCategoryId":null,"ListUrlMain":null,"RegionNum":3,"RegionCategory":"医学","ArticlePicture":[],"TitleCN":null,"AbstractTextCN":null,"PMCID":"OA","EPubDate":null,"PubModel":null,"JCR":null,"JCRName":null,"Score":null,"Total":0}