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Journals Pediatric Rheumatology Online ...

Pediatric Rheumatology Online Journal

https://read.qxmd.com/read/38773611/juvenile-dermatomyositis-with-anti-sae-antibodies-in-a-moroccan-child-associated-with-pseudo-angioedema-a-case-report
#1
JOURNAL ARTICLE
Khalila Nainia, Mohamed Amine Aouzal, Imane Ouafik, Mariyam Charhbili, Amal Bouchhab, Abdellatif Daoudi, Samira Tizki, Radia Chakiri
BACKGROUND: Juvenile Dermatomyositis (JDM) is the leading cause of non-infectious inflammatory myopathy in children. It is a heterogeneous group of autoimmune diseases characterized by a variable combination of muscular, dermatological, and visceral involvement. Myositis-specific autoantibodies help define homogeneous subgroups with common clinical characteristics and prognoses. Anti-SAE (small ubiquitin-like modifier 1 (SUMO-1) activating enzyme) antibodies are among the most recently discovered specific autoantibodies...
May 21, 2024: Pediatric Rheumatology Online Journal
https://read.qxmd.com/read/38760753/scurvy-masquerading-as-iga-vasculitis
#2
JOURNAL ARTICLE
Hanna L Kassa, S Singh, M Douglas-Jones, Gill Schermbrucker, J De Lange, Frank Phoya, Claire Butters, Carol Hlela, Ashton Coetzee, Ebrahim Banderker, Kate Webb
BACKGROUND: Vitamin C deficiency, or scurvy, is rare but poses risks for children with poor diets, limited resources, or malabsorption issues. It may also be common in children with restrictive or selective dietary habits in children with global developmental delay, autism spectrum disorder, and physical disabilities. Symptoms include fatigue, irritability, joint and muscle pain, joint swellings, edema, swollen gums, easy bruising, and delayed wound healing. Early recognition and prompt intervention are essential to prevent the progression of symptomatic vitamin C deficiency in children...
May 18, 2024: Pediatric Rheumatology Online Journal
https://read.qxmd.com/read/38760816/unravelling-the-clinical-heterogeneity-of-undefined-recurrent-fever-over-time-in-the-european-registries-on-autoinflammation
#3
JOURNAL ARTICLE
Y Vyzhga, H Wittkowski, V Hentgen, S Georgin-Lavialle, A Theodoropoulou, S Fuehner, M Jesenak, J Frenkel, E Papadopoulou-Alataki, Jordi Anton, A Nunzia Olivieri, J Brunner, J Sanchez, I Koné-Paut, S Fingerhutova, P Pillet, U Meinzer, R Khubchandani, A Jansson, J-P Haas, R Berendes, T Kallinich, G Horneff, E Lilienthal, R Papa, D Foell, E Lainka, R Caorsi, M Gattorno, M Hofer
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...
May 17, 2024: Pediatric Rheumatology Online Journal
https://read.qxmd.com/read/38750564/relationship-of-regional-ultraviolet-index-data-with-rash-and-systemic-disease-activity-in-youth-with-childhood-onset-systemic-lupus-results-from-the-childhood-arthritis-and-rheumatology-research-alliance-registry
#4
JOURNAL ARTICLE
Tamara I Tanner, Ilir Agalliu, Dawn M Wahezi, Tamar B Rubinstein
OBJECTIVE: To investigate the association between ultraviolet light index (UVI), as a marker for UV exposure, and seasonality with rash and systemic disease activity in youth with childhood-onset systemic lupus (cSLE) from the Childhood Arthritis and Rheumatology Research Alliance (CARRA) Registry. METHODS: We reviewed data on rash and disease activity from Systemic Lupus Erythematosus Disease Activity Index 2000 (SLEDAI-2 K) scores from cSLE CARRA Registry participants with visits between 2010 and 2019 and obtained zipcode level UVI data from the National Oceanic and Atmospheric Administration (NOAA)...
May 15, 2024: Pediatric Rheumatology Online Journal
https://read.qxmd.com/read/38730442/increasing-the-etanercept-dose-in-a-treat-to-target-approach-in-juvenile-idiopathic-arthritis-does-it-help-to-reach-the-target-a-post-hoc-analysis-of-the-best-for-kids-randomised-clinical-trial
#5
RANDOMIZED CONTROLLED TRIAL
Bastiaan T van Dijk, Sytske Anne Bergstra, J Merlijn van den Berg, Dieneke Schonenberg-Meinema, Lisette W A van Suijlekom-Smit, Marion A J van Rossum, Yvonne Koopman-Keemink, Rebecca Ten Cate, Cornelia F Allaart, Daniëlle M C Brinkman, Petra C E Hissink Muller
BACKGROUND: Etanercept has been studied in doses up to 0.8 mg/kg/week (max 50 mg/week) in juvenile idiopathic arthritis (JIA) patients. In clinical practice higher doses are used off-label, but evidence regarding the relation with outcomes is lacking. We describe the clinical course of JIA-patients receiving high-dose etanercept (1.6 mg/kg/week; max 50 mg/week) in the BeSt for Kids trial. METHODS: 92 patients with oligoarticular JIA, RF-negative polyarticular JIA or juvenile psoriatic arthritis were randomised across three treat-to-target arms: (1) sequential DMARD-monotherapy (sulfasalazine or methotrexate (MTX)), (2) combination-therapy MTX + 6 weeks prednisolone and (3) combination therapy MTX + etanercept...
May 10, 2024: Pediatric Rheumatology Online Journal
https://read.qxmd.com/read/38724970/causal-relationship-between-serum-metabolites-and-juvenile-idiopathic-arthritis-a-mendelian-randomization-study
#6
JOURNAL ARTICLE
Han Zhang, Xiao Ma, Wanlu Liu, Ze Wang, Zian Zhang, GuanHong Chen, Yingze Zhang, Tianrui Wang, Tengbo Yu, Yongtao Zhang
BACKGROUND: Juvenile Idiopathic Arthritis (JIA) is a condition that occurs when individuals under the age of 16 develop arthritis that lasts for more than six weeks, and the cause is unknown. The development of JIA may be linked to serum metabolites. Nevertheless, the association between JIA pathogenesis and serum metabolites is unclear, and there are discrepancies in the findings across studies. METHODS: In this research, the association between JIA in humans and 486 serum metabolites was assessed using genetic variation data and genome-wide association study...
May 9, 2024: Pediatric Rheumatology Online Journal
https://read.qxmd.com/read/38715014/adherence-to-low-dose-methotrexate-in-children-with-juvenile-idiopathic-arthritis-using-a-sensitive-methotrexate-assay
#7
JOURNAL ARTICLE
Julia E Möhlmann, Sytze de Roock, Annelies C Egas, Evelien Ter Weijden, Martijn J H Doeleman, Alwin D R Huitema, Matthijs van Luin, Joost F Swart
BACKGROUND: Low-dose weekly methotrexate (MTX) is the mainstay of treatment in juvenile idiopathic arthritis. Unfortunately, a substantial part of patients has insufficient efficacy of MTX. A potential cause of this inadequate response is suboptimal drug adherence. The aim of this study was to assess MTX adherence in juvenile idiopathic arthritis patients by quantification of MTX concentrations in plasma. Secondly, the association between MTX concentrations and either self-reported adherence issues, or concomitant use of biologics was examined...
May 7, 2024: Pediatric Rheumatology Online Journal
https://read.qxmd.com/read/38693550/relationship-between-ocular-manifestations-laboratory-findings-echocardiographic-findings-and-intravenous-immunoglobulin-resistance-in-kawasaki-disease
#8
JOURNAL ARTICLE
Mohsen Jari, Hajar Esmaeili
BACKGROUND: This study investigates the incidence of ocular involvement in Kawasaki disease (KD) and evaluates the relationship between ocular manifestations, laboratory findings, echocardiographic findings, and intravenous immunoglobulin (IVIG) resistance. METHODS: We conducted a cross-sectional study with 58 KD patients from June 2021 to March 2023. For all patients, a complete ophthalmologic examination and echocardiography were performed in the acute phase before starting the treatment...
May 1, 2024: Pediatric Rheumatology Online Journal
https://read.qxmd.com/read/38685034/checkpoint-inhibitor-immunotherapy-induced-inflammatory-arthritis-secondary-to-nivolumab-and-ipilimumab-a-pediatric-first
#9
JOURNAL ARTICLE
John A Storwick, Herman Tam, Daniel G Rosenbaum, Kristin Houghton
BACKGROUND: Immune checkpoint inhibitors (ICIs) have expanded the arsenal of cancer therapeutics over the last decade but are associated with a spectrum of immune-related adverse events (irAEs), including inflammatory arthritis. While these complications are increasingly recognized in the adult population, no cases of inflammatory arthritis irAEs have been reported in the pediatric literature. CASE PRESENTATION: A 14-year-old female with metastatic epithelioid mesothelioma was referred to the pediatric rheumatology clinic after developing progressive inflammatory joint pain in her bilateral shoulders, hips, and small joints of hands following the second cycle of Nivolumab and Ipilimumab...
April 29, 2024: Pediatric Rheumatology Online Journal
https://read.qxmd.com/read/38678299/long-noncoding-rnas-ctc-471j1-2-nest-as-epigenetic-risk-factors-of-active-juvenile-lupus-nephritis-a-case-control-study
#10
JOURNAL ARTICLE
Mohamed M Zedan, Ali Sobh, Alshimaa Magdy, Mai S Korkor, Zeinab R Attia, Nada Khaled, Yousra Sadeq, Ahmed Hazem El-Nagdy, Ahmed E Taha, Mohamed Ahmed Noureldin, Mohamed Taman, Doaa Mosad Mosa, Marwa H Elnagdy
BACKGROUND: Measurement of the circulating levels of long-non-coding RNAs (lncRNAs) in lupus nephritis (LN) patients could dramatically explore more insights about the disease pathogenesis. Hence, we aimed to quantify the level of expression of CTC-471J1.2 and NeST in LN patients and to correlate it with the disease activity. METHOD: This case-control study was conducted on a group of children with juvenile LN attending to Mansoura University Children's Hospital (MUCH)...
April 27, 2024: Pediatric Rheumatology Online Journal
https://read.qxmd.com/read/38671480/the-clinical-relevance-of-different-antiphospholipid-antibody-profiles-in-pediatric-rheumatology-patients
#11
JOURNAL ARTICLE
Jheel Pandya, Karen Onel, Doruk Erkan
BACKGROUND: The clinical relevance of different antiphospholipid antibody (aPL) profiles, including low level anticardiolipin (aCL) and anti-β2 -glycoprotein-I (aβ2 GPI) antibodies, is ill-defined in the pediatric population. Our purpose is to describe the demographic, clinical, and laboratory characteristics of aPL positive pediatric patients based on different aPL profiles. FINDINGS: In this single center retrospective cohort study, based on the screening of our pediatric (age ≤ 18) rheumatology electronic medical records (2016-2022), we identified patients who had at least one "positive" aPL (lupus anticoagulant [LA], aCL IgG/M, or aβ2 GPI IgG/M) result...
April 26, 2024: Pediatric Rheumatology Online Journal
https://read.qxmd.com/read/38671467/blood-brain-barrier-permeability-and-astrocyte-derived-extracellular-vesicles-in-children-with-juvenile-idiopathic-arthritis-a-cross-sectional-study
#12
JOURNAL ARTICLE
Lillemor Berntson, Andreas Elfving, Alice Gabrielsson Samuelsson, Anders Öman, Fariborz Mobarrez
BACKGROUND: Juvenile idiopathic arthritis (JIA) is the most prevalent rheumatic disease in children, and the inflammatory process is widely studied, primarily characterized by its impact on joint health. Emerging evidence suggests that JIA may also affect the central nervous system (CNS). This study investigates the potential CNS involvement in JIA by analyzing the presence of astrocyte-derived extracellular vesicles (EVs) and the S100B protein in plasma, both of which are indicative of astrocyte activity and blood-brain barrier (BBB) integrity...
April 26, 2024: Pediatric Rheumatology Online Journal
https://read.qxmd.com/read/38641611/bone-health-and-physical-activity-in-adolescents-with-juvenile-idiopathic-arthritis-a-cross-sectional-case-control-study
#13
JOURNAL ARTICLE
Egi Vasil, Colleen M Nesbitt, Clodagh Toomey, Gregor Kuntze, Shane Esau, Carolyn A Emery, Leigh Gabel
BACKGROUND: Adolescents with juvenile idiopathic arthritis (JIA) tend to engage in less physical activity than their typically developing peers. Physical activity is essential for bone development and reduced physical activity may detrimentally effect bone health. Thus, we examined differences in total body bone mineral content (BMC) and areal bone mineral density (aBMD) between adolescents with JIA and adolescent controls without JIA. We also examined associations between moderate-to-vigorous physical activity (MVPA), lean mass, and bone outcomes...
April 19, 2024: Pediatric Rheumatology Online Journal
https://read.qxmd.com/read/38637849/risk-perception-well-being-depression-and-anxiety-in-children-and-adolescents-with-rheumatic-diseases-during-the-covid-19-pandemic-results-from-the-prospective-multicenter-kick-covid-study-in-germany
#14
JOURNAL ARTICLE
Claudia Sengler, Jens Klotsche, Malthe Jessen Pedersen, Martina Niewerth, Julia Göldel, Daniel Windschall, Johannes-Peter Haas, Frank Dressler, Ralf Trauzeddel, Anton Hospach, Frank Weller-Heinemann, Stefanie Lanzinger, Clemens Kamrath, Reinhard W Holl, Petra Warschburger, Kirsten Minden
OBJECTIVE: To investigate the psychosocial burden in children and adolescents with juvenile rheumatic diseases during the COVID-19 pandemic. METHODS: As part of the multicentre observational KICK-COVID study linked to the National Pediatric Rheumatology Database, adolescents < 21 years and parents of children < 12 years with rheumatic diseases answered questions on perceptions of health risk (PHR) due to SARS-CoV2, stress, well-being (WHO-5) and symptoms of depression (PHQ-9) and anxiety (GAD-7)...
April 18, 2024: Pediatric Rheumatology Online Journal
https://read.qxmd.com/read/38616268/kawasaki-disease-in-kenya-and-review-of-the-african-literature
#15
REVIEW
A Migowa, C M Njeru, E Were, T Ngwiri, I Colmegna, C Hitchon, R Scuccimarri
BACKGROUND: Kawasaki disease has been described across the globe, although publications from Africa are limited. To our knowledge, there are no publications on Kawasaki disease from Kenya, which triggered this report. METHODS: A retrospective cross-sectional study was undertaken to identify in-patients with a discharge diagnosis of Kawasaki disease, over 2 different 5-year periods, at two pediatric hospitals in Nairobi, Kenya. We reviewed the medical records of all patients and report their clinical findings, diagnostic workup and treatment...
April 14, 2024: Pediatric Rheumatology Online Journal
https://read.qxmd.com/read/38610057/c-reactive-protein-to-albumin-ratio-as-a-prognostic-tool-for-predicting-intravenous-immunoglobulin-resistance-in-children-with-kawasaki-disease-a-systematic-review-of-cohort-studies
#16
REVIEW
Jue Liu, Xingguang Chen, Minling Yang, Fangfang Shen, Feng Zhu, Jian Jin, Yiqun Teng
BACKGROUND: Intravenous immunoglobulin (IVIG) is the primary treatment for Kawasaki disease (KD). However, 10-20% of KD patients show no response to IVIG treatment, making the early prediction of IVIG resistance a key focus of KD research. Our aim is to explore the application of the C-reactive protein to albumin ratio (CAR) for predicting IVIG resistance in children with KD through meta-analysis. METHODS: Cochrane Library, PubMed, MEDLINE, EMbase, CNKI, WanFang, the Chinese Biomedical Database, and CQVIP were searched up to November 2023 for cohort studies on predicting IVIG-resistant KD using the CAR...
April 12, 2024: Pediatric Rheumatology Online Journal
https://read.qxmd.com/read/38589909/provider-assessment-of-the-temporomandibular-joint-in-juvenile-idiopathic-arthritis-a-retrospective-analysis-from-the-carra-database
#17
JOURNAL ARTICLE
Anna Costello, Marinka Twilt, Melissa A Lerman
BACKGROUND: Temporomandibular joint (TMJ) involvement is an often underrecognized complication of juvenile idiopathic arthritis (JIA) that can cause decreased mandibular growth, altered facial morphology, and orofacial pain. It is estimated that the TMJ is affected in 30-45% of children with JIA. Standardized physical examination and imaging evaluations are important in accurately assessing active TMJ arthritis and sequalae. Little is known about the rate at which providers evaluate TMJ involvement in their clinical practice...
April 8, 2024: Pediatric Rheumatology Online Journal
https://read.qxmd.com/read/38515165/exploring-paediatric-rheumatology-care-a-ten-year-retrospective-analysis-of-the-patient-population-in-ghana
#18
JOURNAL ARTICLE
Dzifa Dey, Bright Katso, Afia Baah, Saudatu Isaaka, Emmanuella Amoako
BACKGROUND: Rheumatic diseases can seriously impact children's general health, development, and growth. However, due to a lack of resources, paediatric rheumatology is a largely underdeveloped speciality in many African nations. Children with rheumatic disorders face obstacles in accessing specialized medical care, including lack of specialists, care centres, medication access, and limited research and education to increase understanding of paediatric rheumatic disease among healthcare practitioners...
March 21, 2024: Pediatric Rheumatology Online Journal
https://read.qxmd.com/read/38509613/level-and-correlates-of-physical-activity-among-children-and-adolescents-with-juvenile-idiopathic-arthritis-compared-to-controls-results-from-a-german-nationwide-prospective-observational-cohort-study
#19
JOURNAL ARTICLE
Florian Milatz, Sandra Hansmann, Jens Klotsche, Martina Niewerth, Tilmann Kallinich, Frank Dressler, Johannes-Peter Haas, Rainer Berendes, Gerd Horneff, Markus Hufnagel, Frank Weller-Heinemann, Daniel Windschall, Ralf Trauzeddel, Moritz Klaas, Hermann Girschick, Prasad T Oommen, Ivan Foeldvari, Serdar Mustafa Cantez, Annette F Jansson, Matthias Hartmann, Joachim Peitz-Kornbrust, Kirsten Minden
BACKGROUND: Physical active lifestyles are essential throughout growth and maturation and may offer potential preventive and therapeutic benefit in patients with juvenile idiopathic arthritis (JIA). Insufficient physical activity (PA), in contrast, can lead to aggravation of disease-related symptoms. This study aimed to i) examine PA levels in children and adolescents with JIA compared to general population controls and ii) investigate correlates of pronounced physical inactivity in order to identify risk groups for sedentary behaviour...
March 20, 2024: Pediatric Rheumatology Online Journal
https://read.qxmd.com/read/38504360/efficacy-and-safety-of-canakinumab-in-systemic-juvenile-idiopathic-arthritis-the-first-chinese-experience
#20
JOURNAL ARTICLE
Lingzhi Qiu, Le Ma, Yifan Xie, Jing Jin, Yuting Pan, Shumin Li, Zhidan Fan, Haiguo Yu
BACKGROUND: Systemic juvenile idiopathic arthritis (sJIA) is a severe form of juvenile arthritis that is characterized by chronic joint inflammation and systemic symptoms such as fever, rash, and organ involvement. Anti-IL-6 receptor monoclonal antibody tocilizumab is an effective treatment. However, some patients still experience persisting or recurrent symptoms and the real-world effectiveness of canakinumab in Chinese patients with sJIA has never been reported. Therefore, this study aimed to assess the efficacy and safety of canakinumab in Chinese patients with sJIA using real-world data...
March 19, 2024: Pediatric Rheumatology Online Journal
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