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Systemic juvenile idiopathic arthritis

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https://www.readbyqxmd.com/read/28923429/frequency-of-auditory-involvement-and-of-associated-factors-in-patients-with-juvenile-idiopathic-arthritis
#1
Adriana Ivonne Céspedes Cruz, Myriam Méndez Núñez, Eunice Solís Vallejo, Maritza Zeferino Cruz, Alfonso Ragnar Torres Jiménez, Verónica Ocampo Sánchez, Beatriz Flores Meza, Norma Quintana Ruiz
INTRODUCTION: Juvenile idiopathic arthritis (JIA) is a chronic autoimmune disease characterized by the presence of arthritis in children under 16 years of age for more than 6 weeks in the absence of any other known cause. The extra-articular manifestations, especially in the audiovestibular system, are related to the involvement of the joints of the ossicular chain as a result of the inflammatory process in the synovium. Previous clinical studies in pediatric patients have shown conductive or sensorineural hearing loss...
September 8, 2017: Reumatología Clinica
https://www.readbyqxmd.com/read/28917219/reuma-pt-contribution-to-the-knowledge-of-immune-mediated-systemic-rheumatic-diseases
#2
Maria José Santos, Helena Canhão, Ana Filipa Mourão, Filipa Oliveira Ramos, Cristina Ponte, Cátia Duarte, Anabela Barcelos, Fernando Martins, José António Melo Gomes
Patient registries are key instruments aimed at a better understanding of the natural history of diseases, at assessing the effectiveness of therapeutic interventions, as well as identifying rare events or outcomes that are not captured in clinical trials. However, the potential of registries goes far beyond these aspects. For example, registries promote the standardization of clinical practice, can also provide information on domains that are not routinely collected in clinical practice and can support decision-making...
September 14, 2017: Acta Reumatológica Portuguesa
https://www.readbyqxmd.com/read/28916545/serologic-evidence-of-gut-driven-systemic-inflammation-in-juvenile-idiopathic-arthritis
#3
Lampros Fotis, Nurmohammad Shaikh, Kevin W Baszis, Charles M Samson, Raffi Lev-Tzion, Anthony R French, Phillip I Tarr
OBJECTIVE: Accumulating evidence links juvenile idiopathic arthritis (JIA) to nonhost factors such as gut microbes. We hypothesize that children with new-onset JIA have increased intestinal bacterial translocation and circulating lipopolysaccharide (LPS). METHODS: We studied systemic treatment-naive patients with JIA [polyarticular JIA, n = 22, oligoarticular JIA, n = 31, and spondyloarthropathies (SpA), n = 16], patients with established inflammatory bowel disease-related arthritis (IBD-RA, n = 11), and 34 healthy controls...
September 15, 2017: Journal of Rheumatology
https://www.readbyqxmd.com/read/28900629/siae-rare-variants-in-juvenile-idiopathic-arthritis-and-primary-antibody-deficiencies
#4
Eirini Sevdali, Elena Tsitsami, Maria Tsinti, Evangelia Farmaki, Efimia Papadopoulou-Alataki, Anastasios E Germenis, Matthaios Speletas
Sialic acid acetylesterase (SIAE) deficiency was suggested to lower the levels of ligands for sialic acid-binding immunoglobulin-like receptors, decreasing the threshold for B-cell activation. In humans, studies of rare heterozygous loss-of-function mutations in SIAE gene in common autoimmune diseases, including juvenile idiopathic arthritis (JIA), yielded inconsistent results. Considering the distinct pathogenesis of the two main subtypes of JIA, autoinflammatory systemic (sJIA) and autoimmune oligo/polyarticular (aJIA), and a predisposition to autoimmunity displayed by patients and families with primary antibody deficiencies (PADs), the aim of our study was to analyze whether SIAE rare variants are associated with both the phenotype of JIA and the autoimmunity risk in families with PADs...
2017: Journal of Immunology Research
https://www.readbyqxmd.com/read/28871670/-early-detection-of-endothelial-dysfunction-in-children-with-autoimmune-diseases-by-a-novel-noninvasive-technique
#5
Liron Borenstein-Levin, Riva Brik, Giora Pillar, Yonatan Butbul Aviel
INTRODUCTION: Atherosclerosis is emerging as one of the most important causes of morbidity and mortality among patients with different rheumatologic disease. Endothelial dysfunction may be an early sign of atherosclerosis. OBJECTIVES: To evaluate the occurrence of endothelial dysfunction in children with autoimmune diseases, including juvenile idiopathic arthritis (JIA), systemic lupus erythematosus (SLE) and dermatomyositis, using a novel noninvasive technique...
July 2017: Harefuah
https://www.readbyqxmd.com/read/28870142/validation-of-the-standardized-universal-pain-evaluations-for-rheumatology-providers-for-children-and-youth-super-kidz
#6
Nadia J Luca, Jennifer N Stinson, Brian M Feldman, Susanne M Benseler, Dorcas Beaton, Sarah Campillo, Claire LeBlanc, Margaret van Wyk, Ahmed M Bayoumi
Study Design Longitudinal observational clinimetric study with repeated measures. Background No validated multi-dimensional pain measure for children and youth with juvenile idiopathic arthritis (JIA) exists. Objectives To determine the test-retest reliability, construct validity, and responsiveness of English and French versions of the Standardized Universal Pain Evaluations for Rheumatology Providers for Children and Youth (SUPER-KIDZ). Methods Measurement properties of SUPER-KIDZ (older child, younger child, and parent versions) were prospectively evaluated in patients aged 4-18 years with JIA at 2 centres...
September 4, 2017: Journal of Orthopaedic and Sports Physical Therapy
https://www.readbyqxmd.com/read/28865469/comics-as-an-educational-tool-for-children-with-juvenile-idiopathic-arthritis
#7
Amir Mendelson, Noa Rabinowicz, Yonit Reis, Gil Amarilyo, Liora Harel, Philip J Hashkes, Yosef Uziel
BACKGROUND: This study examined whether the comic book Neta and the Medikidz Explain JIA would improve disease-related knowledge and treatment adherence among patients with juvenile idiopathic arthritis (JIA). METHODS: In this prospective cohort study, JIA patients answered 20 multiple-choice knowledge questions about their disease, before and after reading the comic book. Demographic, clinical, health-related quality of life and adherence data were recorded and correlated to the responses...
September 2, 2017: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/28861733/patterns-of-uveitis-in-children-at-the-apex-institute-for-eye-care-in-india-analysis-and-review-of-literature
#8
Brijesh Takkar, Pradeep Venkatesh, Nripen Gaur, Sat Pal Garg, Rajpal Vohra, Supriyo Ghose
AIM: To study patterns of uveitis in Indian children and compare with data sets published earlier in the literature. METHODS: Consecutive patients below 16 years of age presenting to the uvea clinic of a tertiary eye care center were included prospectively through the period of July 2009-August 2013. Children with retinal vasculitis, exogenous endophthalmitis and masquerade syndromes were excluded from analysis. Uveitis was classified as per the nomenclature system adopted by the International Uveitis Study Group...
August 31, 2017: International Ophthalmology
https://www.readbyqxmd.com/read/28861674/-ocular-involvement-in-rheumatoid-arthritis-connective-tissue-diseases-and-vasculitis
#9
REVIEW
I Kötter, N Stübiger, C Deuter
There are many interfaces between ophthalmologists and rheumatologists. On the one hand ophthalmologists face the question if an inflammation of the eye is caused by systemic inflammatory rheumatic diseases and on the other hand rheumatologists have to consider that ocular manifestations are relatively common in some inflammatory rheumatic diseases. Furthermore, these ocular manifestations may influence therapeutic decisions of the rheumatologist. This article summarizes which ocular inflammations can be associated with rheumatoid arthritis, connective tissue diseases and vasculitides...
August 31, 2017: Zeitschrift Für Rheumatologie
https://www.readbyqxmd.com/read/28861079/tocilizumab-contributes-to-the-inflammatory-status-of-mature-dendritic-cells-through-interleukin-6-receptor-subunits-modulation
#10
Daniel Meley, Audrey Héraud, Valerie Gouilleux-Gruart, Fabrice Ivanes, Florence Velge-Roussel
Tocilizumab, a humanized anti-IL-6 receptor α (IL-6Rα) is widely used in the treatment of a panel of pathologies such as adult and juvenile rheumatoid arthritis (RA) and the systemic form of juvenile idiopathic arthritis in children. Its indications are expected to be largely extended to other inflammatory diseases in close future. Dendritic cells (DCs) appear to be deeply involved in the immunopathology of these diseases, yet the effects of tocilizumab on these cells were poorly studied. In this study, we explored the effect of tocilizumab on the regulation of IL-6R subunits [gp130, soluble form of IL-6Rα (sIL-6Rα), and mIL-6Rα] in human monocyte-derived DCs...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/28860338/hif1a-is-a-critical-downstream-mediator-for-hemophagocytic-lymphohistiocytosis
#11
Rui Huang, Yoshihiro Hayashi, Xiaomei Yan, Jiachen Bu, Jieyu Wang, Yue Zhang, Yile Zhou, Yuting Tang, Lingyun Wu, Zefeng Xu, Xin Liu, Qianfei Wang, Jianfeng Zhou, Zhijian Xiao, James P Bridges, Rebecca A Marsh, Kejian Zhang, Michael B Jordan, Yuhua Li, Gang Huang
Hemophagocytic lymphohistiocytosis is a life-threatening syndrome characterized by overwhelming immune activation. A steroid and chemotherapy-based regimen remains as the first-line of therapy but it has substantial morbidity. Thus, novel, less toxic therapy for hemophagocytic lymphohistiocytosis is urgently needed. Although differences exist between familial hemophagocytic lymphohistiocytosis and secondary hemophagocytic lymphohistiocytosis, they have many common features. Using bioinformatic analysis with familial hemophagocytic lymphohistiocytosis and systemic juvenile idiopathic arthritis, which is associated with secondary hemophagocytic lymphohistiocytosis, we identified a common hypoxia-inducible factor 1A (HIF1A) signature...
August 31, 2017: Haematologica
https://www.readbyqxmd.com/read/28858208/inflammatory-diseases-and-growth-effects-on-the-gh-igf-axis-and-on-growth-plate
#12
REVIEW
Francesca Cirillo, Pietro Lazzeroni, Chiara Sartori, Maria Elisabeth Street
This review briefly describes the most common chronic inflammatory diseases in childhood, such as cystic fibrosis (CF), inflammatory bowel diseases (IBDs), juvenile idiopathic arthritis (JIA), and intrauterine growth restriction (IUGR) that can be considered, as such, for the changes reported in the placenta and cord blood of these subjects. Changes in growth hormone (GH) secretion, GH resistance, and changes in the insulin-like growth factor (IGF) system are described mainly in relationship with the increase in nuclear factor-κB (NF-κB) and pro-inflammatory cytokines...
August 31, 2017: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/28837372/update-upon-efficacy-and-safety-of-etanercept-for-the-treatment-of-spondyloarthritis-and-juvenile-idiopathic-arthritis
#13
Giuseppe Murdaca, Simone Negrini, Ottavia Magnani, Elena Penza, Marco Pellecchio, Rossella Gulli, Paola Mandich, Francesco Puppo
TNF-α inhibitors have demonstrated efficacy both as monotherapy and in combination with disease-modifying anti-rheumatic drugs (DMARDs) in the treatment of chronic inflammatory immune-mediated diseases such as rheumatoid arthritis, Crohn's disease, ulcerative colitis, ankylosing spondylitis (AS), psoriasis (Ps) and/or psoriatic arthritis (PsA) and may be administered off-label to treat disseminated granuloma annulare, systemic lupus erythematosus and systemic sclerosis. There are several TNF-α inhibitors available for clinical use including infliximab, adalimumab, golimumab, certolizumab pegol and etanercept...
August 24, 2017: Modern Rheumatology
https://www.readbyqxmd.com/read/28830457/growth-and-weight-gain-in-children-with-juvenile-idiopathic-arthritis-results-from-the-reacch-out-cohort
#14
Jaime Guzman, Tristan Kerr, Leanne M Ward, Jinhui Ma, Kiem Oen, Alan M Rosenberg, Brian M Feldman, Gilles Boire, Kristin Houghton, Paul Dancey, Rosie Scuccimarri, Alessandra Bruns, Adam M Huber, Karen Watanabe Duffy, Natalie J Shiff, Roberta A Berard, Deborah M Levy, Elizabeth Stringer, Kimberly Morishita, Nicole Johnson, David A Cabral, Maggie Larché, Ross E Petty, Ronald M Laxer, Earl Silverman, Paivi Miettunen, Anne-Laure Chetaille, Elie Haddad, Lynn Spiegel, Stuart E Turvey, Heinrike Schmeling, Bianca Lang, Janet Ellsworth, Suzanne E Ramsey, Johannes Roth, Sarah Campillo, Susanne Benseler, Gaëlle Chédeville, Rayfel Schneider, Shirley M L Tse, Roxana Bolaria, Katherine Gross, Debbie Feldman, Bonnie Cameron, Roman Jurencak, Jean Dorval, Claire LeBlanc, Claire St Cyr, Michele Gibbon, Rae S M Yeung, Ciarán M Duffy, Lori B Tucker
BACKGROUND: With modern treatments, the effect of juvenile idiopathic arthritis (JIA) on growth may be less than previously reported. Our objective was to describe height, weight and body mass index (BMI) development in a contemporary JIA inception cohort. METHODS: Canadian children newly-diagnosed with JIA 2005-2010 had weight and height measurements every 6 months for 2 years, then yearly up to 5 years. These measurements were used to calculate mean age- and sex-standardized Z-scores, and estimate prevalence and cumulative incidence of growth impairments, and the impact of disease activity and corticosteroids on growth...
August 22, 2017: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/28822720/juvenile-idiopathic-arthritis-practice-patterns-among-oral-and-maxillofacial-surgeons
#15
Brian E Kinard, Shelly Abramowicz
PURPOSE: The purpose of this investigation is to assess the current clinical practices by oral and maxillofacial surgeons (OMSs) in the United States regarding diagnosis and treatment of temporomandibular joint (TMJ) involvement in children with juvenile idiopathic arthritis (JIA). MATERIALS AND METHODS: We implemented a cross-sectional survey of academic OMSs in the United States via an electronic survey. The survey included respondent demographic data, patient volumes, diagnostic methods, and management practices for children with JIA and TMJ involvement...
July 25, 2017: Journal of Oral and Maxillofacial Surgery
https://www.readbyqxmd.com/read/28815559/characteristic-elevation-of-soluble-tnf-receptor-ii-to-i-ratio-in-macrophage-activation-syndrome-with-systemic-juvenile-idiopathic-arthritis
#16
Masaki Shimizu, Natsumi Inoue, Mao Mizuta, Yasuo Nakagishi, Akihiro Yachie
To investigate the clinical significance of soluble tumor necrosis factor receptor (sTNFR) II/I ratio as an indicator of the diagnosis of macrophage activation syndrome (MAS) complicating systemic juvenile idiopathic arthritis (s-JIA), we measured the serum sTNFRI and II levels in 117 patients with s-JIA, including 29 patients with MAS, 15 with Epstein-Barr virus-induced hemophagocytic lymphohistiocytosis (EBVHLH), 15 with Kawasaki disease (KD), and 28 healthy controls (HCs). We determined their correlation with measurements of disease activity and severity...
August 16, 2017: Clinical and Experimental Immunology
https://www.readbyqxmd.com/read/28811270/developing-and-evaluating-jiapp-acceptability-and-usability-of-a-smartphone-app-system-to-improve-self-management-in-young-people-with-juvenile-idiopathic-arthritis
#17
Ran A Cai, Dominik Beste, Hema Chaplin, Socrates Varakliotis, Linda Suffield, Francesca Josephs, Debajit Sen, Lucy R Wedderburn, Yiannakis Ioannou, Stephen Hailes, Despina Eleftheriou
BACKGROUND: Flare-ups in juvenile idiopathic arthritis (JIA) are characterized by joint pain and swelling and often accompanied with fatigue, negative emotions, and reduced participation in activities. To minimize the impact of JIA on the physical and psychosocial development and well-being of young people (YP), it is essential to regularly monitor disease activity and side effects, as well as to support self-management such as adherence to treatment plans and engagement in general health-promoting behaviors...
August 15, 2017: JMIR MHealth and UHealth
https://www.readbyqxmd.com/read/28807357/development-and-initial-validation-of-the-macrophage-activation-syndrome-primary-hemophagocytic-lymphohistiocytosis-score-a-diagnostic-tool-that-differentiates-primary-hemophagocytic-lymphohistiocytosis-from-macrophage-activation-syndrome
#18
Francesca Minoia, Francesca Bovis, Sergio Davì, Antonella Insalaco, Kai Lehmberg, Susan Shenoi, Sheila Weitzman, Graciela Espada, Yi-Jin Gao, Jordi Anton, Toshiyuki Kitoh, Ozgur Kasapcopur, Helga Sanner, Rosa Merino, Itziar Astigarraga, Maria Alessio, Michael Jeng, Vyacheslav Chasnyk, Kim E Nichols, Zeng Huasong, Caifeng Li, Concetta Micalizzi, Nicolino Ruperto, Alberto Martini, Randy Q Cron, Angelo Ravelli, AnnaCarin Horne
OBJECTIVE: To develop and validate a diagnostic score that assists in discriminating primary hemophagocytic lymphohistiocytosis (pHLH) from macrophage activation syndrome (MAS) related to systemic juvenile idiopathic arthritis. STUDY DESIGN: The clinical, laboratory, and histopathologic features of 362 patients with MAS and 258 patients with pHLH were collected in a multinational collaborative study. Eighty percent of the population was assessed to develop the score and the remaining 20% constituted the validation sample...
August 11, 2017: Journal of Pediatrics
https://www.readbyqxmd.com/read/28805021/towards-establishing-a-standardized-magnetic-resonance-imaging-scoring-system-for-temporomandibular-joints-in-juvenile-idiopathic-arthritis
#19
Mirkamal A Tolend, Marinka Twilt, Randy Q Cron, Nikolay Tzaribachev, Saurabh Guleria, Thekla von Kalle, Bernd Koos, Elka Miller, Jennifer Stimec, Yoginder Vaid, Tore A Larheim, Troels Herlin, Lynn Spiegel, Emilio Inarejos, Rahim Moineddin, Marion A van Rossum, Rotraud K Saurenmann, Andrea S Doria, Christian J Kellenberger
OBJECTIVES: The temporomandibular joints (TMJs) are frequently affected in children with juvenile idiopathic arthritis (JIA). Early detection is challenging, as major variation is present in scoring TMJ pathology on Magnetic Resonance Imaging (MRI). Consensus-driven development and validation of a MRI scoring system for TMJs has important clinical utility in timely improvement of diagnosis, and serving as an outcome measure. We report on a multi-institutional collaboration towards developing a TMJ MRI scoring system for JIA...
August 13, 2017: Arthritis Care & Research
https://www.readbyqxmd.com/read/28791286/autoimmune-thrombotic-thrombocytopenic-purpura-two-rare-cases-associated-with-juvenile-idiopathic-arthritis-and-multiple-sclerosis
#20
Despoina Dimopoulou, Athina Dimosiari, Eudokia Mandala, Theodoros Dimitroulas, Alaxandros Garyfallos
Secondary thrombotic microangiopathies are associated with several underlying conditions, with most of them being resolved after the treatment of background disease. Thrombotic thrombocytopenic purpura (TTP) is a rare microangiopathy presenting with anemia, thrombocytopenia, and neurological deficits, occurring most often in various autoimmune diseases due to inhibition of ADAMTS13 by autoantibodies, as well as in pregnant women with or without an autoimmune substrate. In this article, we report two newly diagnosed TTP cases, who have not been published so far...
2017: Frontiers in Medicine
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