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Systemic juvenile idiopathic arthritis

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https://www.readbyqxmd.com/read/28513071/juvenile-idiopathic-arthritis-in-multiplex-families-longitudinal-follow-up
#1
Munira Al Marri, Alya Qari, Sulaiman M Al-Mayouf
OBJECTIVE: To describe the physical, social, educational and employment status and clinical outcomes of patients with juvenile idiopathic arthritis (JIA) from multiplex families. METHODS: All familial JIA patients were treated and had regular follow-up between 1990 and 2015 at King Faisal Specialist Hospital and Research Center (KFSH-RC), Riyadh, were included. Demographic data, disease duration, active arthritis and articular and extra-articular damage at last follow-up visit were reviewed...
May 16, 2017: International Journal of Rheumatic Diseases
https://www.readbyqxmd.com/read/28511684/access-to-pediatric-rheumatology-care-for-juvenile-idiopathic-arthritis-in-the-united-arab-emirates
#2
Khulood Khawaja, Mustafa Al-Maini
BACKGROUND: This study looks at access to care for Juvenile Idiopathic Arthritis through pediatric rheumatology in the UAE, as an example of multi-ethnic society. METHODS: Patients with a diagnosis of Juvenile idiopathic arthritis were identified through the hospital electronic medical records system from January 1st 2011 to December 31st 2014. All residents of the United Arab Emirates hold an Emirates identity card. We divided our patients into two groups: Emirati-Emirates, who are native Emirati children and hold the Emirati nationality, as stated on their Emirates identity card, and who therefore have full, comprehensive access to free medical care; and non-Emirati-Emirates, who represent other nationalities, as stated on their Emirates identity card...
May 16, 2017: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/28499374/clinical-improvement-of-renal-amyloidosis-in-a-patient-with-systemic-onset-juvenile-idiopathic-arthritis-who-received-tocilizumab-treatment-a-case-report-and-literature-review
#3
Songkiat Chantarogh, Soamarat Vilaiyuk, Thipwimol Tim-Aroon, Suchin Worawichawong
BACKGROUND: Juvenile idiopathic arthritis (JIA) is a common rheumatic disease in children and adolescents. Although JIA may cause secondary amyloidosis, this is a rare complication in patients with JIA and other rheumatic diseases. Many previous studies have revealed that common heterozygous or homozygous mutations in the MEFV gene are associated with systemic-onset JIA (SJIA). CASE PRESENTATION: We herein report a case involving a 19-year-old female patient with difficult-to-control SJIA...
May 12, 2017: BMC Nephrology
https://www.readbyqxmd.com/read/28499329/biologic-therapy-modifies-clinical-and-laboratory-features-of-macrophage-activation-syndrome-associated-with-systemic-juvenile-idiopathic-arthritis
#4
Grant S Schulert, Francesca Minoia, John Bohnsack, Randy Q Cron, Soah Hashad, Isabelle Kone-Paut, Mikhail Kostik, Daniel Lovell, Despoina Maritsi, Peter A Nigrovic, Priyankar Pal, Angelo Ravelli, Masaki Shimizu, Valda Stanevicha, Sebastiaan Vastert, Andreas Woerner, Fabrizio de Benedetti, Alexei A Grom
OBJECTIVE: To assess performance of the 2016 macrophage activation syndrome (MAS) classification criteria for patients with systemic juvenile idiopathic arthritis (systemic JIA) who develop MAS while treated with biologic medications. METHODS: A systematic literature review was performed to identify patients with MAS while treated with IL-1 and IL-6 blocking agents. Clinical and laboratory information was compared to a large previously compiled historical cohort...
May 12, 2017: Arthritis Care & Research
https://www.readbyqxmd.com/read/28451698/-still-s-disease-in-children-and-adults
#5
C M Hedrich, C Günther, M Aringer
Systemic juvenile idiopathic arthritis (sJIA) is characterized by fever, arthritis, and other signs of systemic inflammation. Historically, sJIA was named Still's disease after George Frederic Still, who first reported patients. Individuals who manifest after the 16(th) birthday are diagnosed with adult onset Still's disease (AOSD). The pathophysiology of sJIA and AOSD are incompletely understood. Increased activation of inflammasomes and the expression of proinflammatory cytokines play a central role. S100 proteins, which can activate Toll-like receptors, thus, maintaining positive feedback loops, have also been detected at increased levels in sera from sJIA patients...
April 27, 2017: Der Hautarzt; Zeitschrift Für Dermatologie, Venerologie, und Verwandte Gebiete
https://www.readbyqxmd.com/read/28444097/chronic-polyarthritis-as-the-first-manifestation-of-childhood-systemic-polyarteritis-nodosa
#6
Glaucia Vanessa Novak, Koken Hayashi, Kohei Sampa, Yosuke Okumura, Gabriela Ribeiro Viola Ferreira, Clovis Artur Silva
Arthritis has been reported as an acute pattern, generally evanescent with oligoarthritis, mostly affecting knees and ankles in childhood systemic polyarteritis nodosa. However, chronic polyarthritis with morning stiffness mimicking juvenile idiopathic arthritis has not been reported. We describe the case of a 4-year old girl who had additive and chronic polyarthritis with edema, tenderness, pain on motion and morning stiffness for 2 months. After 45 days, she also presented painful subcutaneous nodules and erythematous-violaceous lesions in the extensor region of upper and lower limbs...
January 2017: Einstein
https://www.readbyqxmd.com/read/28427313/sensitivity-of-a-juvenile-subject-specific-musculoskeletal-model-of-the-ankle-joint-to-the-variability-of-operator-dependent-input
#7
Iain Hannah, Erica Montefiori, Luca Modenese, Joe Prinold, Marco Viceconti, Claudia Mazzà
Subject-specific musculoskeletal modelling is especially useful in the study of juvenile and pathological subjects. However, such methodologies typically require a human operator to identify key landmarks from medical imaging data and are thus affected by unavoidable variability in the parameters defined and subsequent model predictions. The aim of this study was to thus quantify the inter- and intra-operator repeatability of a subject-specific modelling methodology developed for the analysis of subjects with juvenile idiopathic arthritis...
May 2017: Proceedings of the Institution of Mechanical Engineers. Part H, Journal of Engineering in Medicine
https://www.readbyqxmd.com/read/28420491/telemedicine-for-patients-with-rheumatic-diseases-systematic-review-and-proposal-for-research-agenda
#8
REVIEW
Matteo Piga, Ignazio Cangemi, Alessandro Mathieu, Alberto Cauli
OBJECTIVE: To systematically review the scientific literature regarding tele-rheumatology and draw conclusions about feasibility, effectiveness, and patient satisfaction. METHODS: PubMed, Scopus, and Cochrane database searches were performed (April 2016) using relevant MeSH and keyword terms for telemedicine and rheumatic diseases. Articles were selected if reporting outcomes for feasibility, effectiveness, and patient satisfaction and methodologically appraised using the Cochrane Collaboration's tool for assessing risk of bias and a modified version of CONSORT 2010 Statement...
March 22, 2017: Seminars in Arthritis and Rheumatism
https://www.readbyqxmd.com/read/28418334/juvenile-idiopathic-arthritis
#9
Kenan Barut, Amra Adrovic, Sezgin Şahin, Özgür Kasapçopur
Juvenile idiopathic arthritis is the most common chronic rheumatic disease of unknown aetiology in childhood and predominantly presents with peripheral arthritis. The disease is divided into several subgroups, according to demographic characteristics, clinical features, treatment modalities and disease prognosis. Systemic juvenile idiopathic arthritis, which is one of the most frequent disease subtypes, is characterized by recurrent fever and rash. Oligoarticular juvenile idiopathic arthritis, common among young female patients, is usually accompanied by anti-nuclear antibodie positivity and anterior uveitis...
April 5, 2017: Balkan Medical Journal
https://www.readbyqxmd.com/read/28416939/a-systemic-onset-juvenile-idiopathic-arthritis-patient-with-reduced-anakinra-treatment-admitted-with-an-attack
#10
Dilek Yilmaz, Mediha Akcan, Semiha Terlemez, Ferah Sonmez, Abdullah Baris Akcan
Interleukin-1 plays an important role in the pathogenesis of systemic-onset juvenile idiopathic arthritis (SoJIA), and the use of anti-interleukin-1 therapy has been increasing. We report a case of a 14-year-old male patient with SoJIA. He was in remission with anakinra treatment for almost 2 years. When we extended the therapeutic range and decreased the dose (1 mg/kg twice a week), he developed symptoms mimicking pulmonary embolism and cardiac ischemia. Increased cardiac enzyme levels and echocardiographic findings were interpreted as myopericarditis...
February 2017: Eurasian Journal of Medicine
https://www.readbyqxmd.com/read/28416023/the-new-childhood-arthritis-and-rheumatology-research-alliance-carra-registry-design-rationale-and-characteristics-of-patients-enrolled-in-the-first-12%C3%A2-months
#11
Timothy Beukelman, Yukiko Kimura, Norman T Ilowite, Kelly Mieszkalski, Marc D Natter, Grendel Burrell, Brian Best, Jason Jones, Laura E Schanberg
BACKGROUND: Herein we describe the history, design, and rationale of the new Childhood Arthritis and Rheumatology Research Alliance (CARRA) Registry and present the characteristics of patients with juvenile idiopathic arthritis (JIA) enrolled in the first 12 months of operation. METHODS: The CARRA Registry began prospectively collecting data in the United States and Canada in July 2015 to evaluate the safety of therapeutic agents in persons with childhood-onset rheumatic disease, initially restricted to JIA...
April 17, 2017: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/28401744/fdg-pet-in-macrophage-activation-syndrome-associated-with-systemic-juvenile-idiopathic-arthritis
#12
Masaki Shimizu, Yasuhiro Ikawa, Mao Mizuta, Maiko Takakura, Natsumi Inoue, Ryosei Nishimura, Akihiro Yachie
We herein describe a case of systemic juvenile idiopathic arthritis (s-JIA)-associated macrophage activation syndrome (MAS) in which the (18F) fluorodeoxyglucose positron emission tomography ((18)(F) FDG-PET) findings were characteristic. The pattern of greater (18)(F) FDG accumulation into the spleen compared with the liver was more remarkable in this patient compared with s-JIA. This pattern, however, was also observed in cases of acute leukemia. In the present patient, serum interleukin (IL)-18 was extremely elevated (255 000 pg/mL), whereas in leukemia patients it is mildly elevated (360-1480 pg/mL)...
April 2017: Pediatrics International: Official Journal of the Japan Pediatric Society
https://www.readbyqxmd.com/read/28400731/il-1-inhibition-may-have-an-important-role-in-treating-refractory-kawasaki-disease
#13
REVIEW
Perrine Dusser, Isabelle Koné-Paut
Kawasaki disease (KD) is an acute inflammatory vasculitis occurring in young children before 5 years and representing at this age, the main cause of acquired heart disease. A single infusion of 2 g/kg of intravenous immunoglobulins along with aspirin has reduced the frequency of coronary artery aneurysms from 25 to 5%. However, 10-20% of patients do not respond to standard treatment and have an increased risk of cardiac complications and death. The development of more potent therapeutic approaches of KD is an urgent need...
2017: Frontiers in Pharmacology
https://www.readbyqxmd.com/read/28399931/pilot-study-comparing-the-childhood-arthritis-rheumatology-research-alliance-carra-systemic-juvenile-idiopathic-arthritis-consensus-treatment-plans
#14
Yukiko Kimura, Sriharsha Grevich, Timothy Beukelman, Esi Morgan, Peter A Nigrovic, Kelly Mieszkalski, T Brent Graham, Maria Ibarra, Norman Ilowite, Marisa Klein-Gitelman, Karen Onel, Sampath Prahalad, Marilynn Punaro, Sarah Ringold, Dana Toib, Heather Van Mater, Jennifer E Weiss, Pamela F Weiss, Laura E Schanberg
OBJECTIVES: To assess the feasibility of studying the comparative effectiveness of the Childhood Arthritis and Rheumatology Research Alliance (CARRA) consensus treatment plans (CTPs) for systemic Juvenile Idiopathic Arthritis (JIA) using an observational registry. METHODS: Untreated systemic JIA patients enrolled in the CARRA Registry were begun on one of 4 CTPs chosen by the treating physician and patient/family (glucocorticoid [GC] alone; methotrexate [MTX] ± GC; IL1 inhibitor [IL1i] ± GC; IL6 inhibitor [IL6i] ± GC)...
April 11, 2017: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/28394851/development-and-validation-of-the-self-reported-promis-pediatric-pain-behavior-item-bank-and-short-form-scale
#15
Natoshia R Cunningham, Susmita Kashikar-Zuck, Constance Mara, Kenneth R Goldschneider, Dennis A Revicki, Carlton Dampier, David D Sherry, Lori Crosby, Adam Carle, Karon F Cook, Esi M Morgan
Pain behaviors are important indicators of functioning in chronic pain; however, no self-reported pain behavior instrument has been developed for pediatric populations. The purpose of this study was to create a brief pediatric measure of patient-reported pain behaviors as part of the Patient- Reported Outcome Measurement Information System (PROMIS). A pool of 47 candidate items for this measure had been previously developed through qualitative research. In this study, youth with chronic pain associated with juvenile fibromyalgia (JFM), juvenile idiopathic arthritis (JIA), or sickle cell disease (SCD) (ages 8 to 18 years) from three pediatric centers completed all 47 candidate items for development of the pain behavior item bank along with established measures of pain interference, depressive symptoms, fatigue, average pain intensity, and pain catastrophizing...
April 5, 2017: Pain
https://www.readbyqxmd.com/read/28386137/measurement-of-blood-calprotectin-mrp-8-mrp-14-levels-in-patients-with-juvenile-idiopathic-arthritis
#16
Jaryna Bojko
OBJECTIVES: The aim of the investigation was to compare blood calprotectin (MRP8/14, S100A 8/9) levels in patients with systemic-onset, polyarticular, RF-negative and oligoarticular subtypes of juvenile idiopathic arthritis (JIA), and to explore links between blood calprotectin levels and clinical and laboratory markers of JIA activity. MATERIAL AND METHODS: Measurement of calprotectin in blood serum was performed in 160 patients with JIA followed up at Lviv Regional Council Public Institution "Western-Ukrainian Specialised Children's Medical Centre"...
2017: Reumatologia
https://www.readbyqxmd.com/read/28378099/increased-serum-sfas-strail-and-reduced-sfasl-in-juvenile-onset-systemic-lupus-erythematosus
#17
Bernadete L Liphaus, Maria Helena B Kiss, Solange Carrasco, Patrícia Palmeira, Claudia Goldenstein-Schainberg, Magda Carneiro-Sampaio
The aims of this study were to assess serum Fas, FasL, TRAIL, and Bcl-2 levels in patients with juvenile-onset systemic lupus erythematosus (JSLE) and to evaluate their relations with disease activity parameters and nephritis. Forty-eight JSLE patients, 33 juvenile idiopathic arthritis (JIA, inflammatory controls) patients and 40 healthy controls were enrolled. sFas, sFasL, sTRAIL, and sBcl-2 serum levels were measured by ELISA. Disease activity parameters included SLEDAI score, ESR, anti-dsDNA antibodies, C3, and C4 levels...
April 4, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28372862/corrigendum-to-mir-146a-modulates-macrophage-polarization-in-systemic-juvenile-idiopathic-arthritis-by-targeting-inhba-mol-immunol-77-2016-205-212
#18
Dan Li, Mingyue Duan, Yuan Feng, Lingling Geng, Xiaoqing Li, Wanggang Zhang
No abstract text is available yet for this article.
March 31, 2017: Molecular Immunology
https://www.readbyqxmd.com/read/28365583/predictors-of-relapse-after-discontinuing-systemic-treatment-in-childhood-autoimmune-chronic-uveitis
#19
Gabriele Simonini, Claudia Bracaglia, Marco Cattalini, Andrea Taddio, Alice Brambilla, Cinzia De Libero, Denise Pires Marafon, Roberto Caputo, Rolando Cimaz
OBJECTIVE: To identify clinical predictors of relapse in childhood autoimmune chronic uveitis after stopping systemic treatment. METHODS: A retrospective, multicenter, cohort study. RESULTS: Ninety-four children in remission, receiving no treatments and with at least a 6-month followup, were enrolled. A higher probability of maintaining remission after discontinuing treatment was shown in idiopathic compared with juvenile idiopathic arthritis uveitis (Mantel-Cox chi-square = 23...
April 1, 2017: Journal of Rheumatology
https://www.readbyqxmd.com/read/28331226/the-histone-deacetylase-inhibitor-givinostat-itf2357-exhibits-potent-anti-tumor-activity-against-crlf2-rearranged-bcp-all
#20
A M Savino, J Sarno, L Trentin, M Vieri, G Fazio, M Bardini, C Bugarin, G Fossati, K Davis, G Gaipa, S Izraeli, L H Meyer, G P Nolan, A Biondi, G Te Kronnie, C Palmi, G Cazzaniga
Leukemias bearing CRLF2 and JAK2 gene alterations are characterized by aberrant JAK/STAT signaling and poor prognosis. The HDAC inhibitor givinostat/ITF2357 has been shown to exert antineoplastic activity against both systemic juvenile idiopathic arthritis and myeloproliferative neoplasms through inhibition of the JAK/STAT pathway. These findings led us to hypothesize that givinostat might also act against CRLF2-rearranged BCP-ALL, which lack effective therapies. Here, we found that givinostat inhibited proliferation and induced apoptosis of BCP-ALL CRLF2-rearranged cell lines, positive for exon 16 JAK2 mutations...
March 23, 2017: Leukemia: Official Journal of the Leukemia Society of America, Leukemia Research Fund, U.K
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