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Systemic juvenile idiopathic arthritis

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https://www.readbyqxmd.com/read/28331226/the-histone-deacetylase-inhibitor-givinostat-itf2357-exhibits-potent-anti-tumor-activity-against-crlf2-rearranged-bcp-all
#1
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
https://www.readbyqxmd.com/read/28328098/plasma-exchange-successfully-treated-macrophage-activation-syndrome-in-rheumatoid-factor-positive-polyarticular-juvenile-idiopathic-arthritis-with-combined-pneumonia
#2
Lianjie Shi, Fanlei Hu, Chuanhui Xu, Huaqun Zhu, Di Qie, Chuanjie Yuan, Yuhong Tao, Hanmin Liu
Macrophage activation syndrome (MAS) is one of the serious complications associated with rheumatic diseases, especially systemic juvenile idiopathic arthritis (sJIA). Here we describe a 9-year-old girl with rheumatoid factor (RF)-positive polyarticular JIA, not sJIA, combined with pneumonia who was successfully treated by plasma exchange. She was diagnosed with RF-positive polyarticular JIA based on positive RF and multiple joint swelling and tenderness 3 years ago. She was admitted in our hospital with myalgia for 2 days and a high fever for half a day...
March 22, 2017: International Journal of Rheumatic Diseases
https://www.readbyqxmd.com/read/28296284/pro-inflammatory-cytokine-environments-can-drive-il-17-over-expression-by-%C3%AE-%C3%AE-t-cells-in-systemic-juvenile-idiopathic-arthritis
#3
Christoph Kessel, Katrin Lippitz, Toni Weinhage, Claas Hinze, Helmut Wittkowski, Dirk Holzinger, Ndate Fall, Alexei A Grom, Niklas Gruen, Dirk Foell
OBJECTIVE: Systemic-onset juvenile idiopathic arthritis (sJIA) is speculated to follow a biphasic course with an initial systemic disease phase driven by innate immune mechanisms and interleukin (IL)-1β as a key cytokine, while a second chronic arthritic phase may be dominated by adaptive immunity and cytokines such as IL-17A. Although a recent mouse model points to a critical role of IL-17 expressing γδT cells in disease pathology, in human patients both the prevalence of IL-17 as well as the role of producing cells are still unclear...
March 15, 2017: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/28293458/the-novel-use-of-combined-il-1-and-il-6-inhibition-in-a-patient-with-severe-aggressive-erosive-systemic-onset-juvenile-idiopathic-arthritis
#4
Martin Lee, John Isaacs
Systemic-onset juvenile idiopathic arthritis (SoJIA) is a form of juvenile idiopathic arthritis (JIA) that typically presents with prominent systemic features and accounts for approximately 10-15% of children with JIA. Pro-inflammatory cytokine pathways are thought to be involved in its pathogenesis, including interleukin-1 (IL-1) and interleukin-6 (IL-6), and laboratory tests demonstrate a prominent inflammatory response with high CRP and ferritin levels. We present a case of severe, aggressive, erosive SoJIA in a 17-year-old male resistant to multiple biologic therapies treated with a novel combination of IL-1 and IL-6 blockade along with subcutaneous methotrexate...
March 2017: European Journal of Rheumatology
https://www.readbyqxmd.com/read/28291375/juvenile-idiopathic-arthritis-what-is-the-utility-of-ultrasound
#5
Hershernpal A S Basra, Paul D Humphries
Juvenile Idiopathic Arthritis is a heterogeneous condition and an important cause of acquired disability in children. Evidence supports early treatment to prevent future complications. This relies on prompt diagnosis, achieved by a high index of clinical suspicion and supportive evidence, including the detection of joint and or tendon inflammation. Ultrasound is a readily accessible, well tolerated, safe and accurate modality for assessing joints and the surrounding soft tissues. It can also be used to guide therapy into those joints and tendon sheaths, resistant to systemic treatments...
March 14, 2017: British Journal of Radiology
https://www.readbyqxmd.com/read/28290112/protecting-bone-health-in-pediatric-rheumatic-diseases-pharmacological-considerations
#6
REVIEW
Yujuan Zhang, Diana Milojevic
Bone health in children with rheumatic conditions may be compromised due to several factors related to the inflammatory disease state, delayed puberty, altered life style, including decreased physical activities, sun avoidance, suboptimal calcium and vitamin D intake, and medical treatments, mainly glucocorticoids and possibly some disease-modifying anti-rheumatic drugs. Low bone density or even fragility fractures could be asymptomatic; therefore, children with diseases of high inflammatory load, such as systemic onset juvenile idiopathic arthritis, juvenile dermatomyositis, systemic lupus erythematosus, and those requiring chronic glucocorticoids may benefit from routine screening of bone health...
March 13, 2017: Paediatric Drugs
https://www.readbyqxmd.com/read/28288653/inflammatory-bowel-disease-following-anti-interleukin-1-treatment-in-systemic-juvenile-idiopathic-arthritis
#7
Boris Hügle, Fabian Speth, Johannes-Peter Haas
BACKGROUND: Inflammatory bowel disease can develop in the context of some rheumatic diseases in childhood, including juvenile idiopathic arthritis (JIA). Inflammatory bowel disease (IBD) is frequently associated with other immune-mediated diseases; however, systemic onset JIA (sJIA) has not previously been connected to IBD. Treatment of sJIA has significantly changed in recent years, possibly causing changes in inflammatory patterns. Therefore, data from the German Center for Pediatric and Adolescent Rheumtology from 2010 until 2015 were analyzed by retrospective chart review...
March 14, 2017: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/28283733/high-sensitive-crp-as-a-predictive-marker-of-long-term-outcome-in-juvenile-idiopathic-arthritis
#8
Mikel Alberdi-Saugstrup, Marek Zak, Susan Nielsen, Troels Herlin, Ellen Nordal, Lillemor Berntson, Anders Fasth, Marite Rygg, Klaus Müller
To evaluate whether C-reactive protein (CRP), including variation within the normal range, is predictive of long-term disease outcome in Juvenile Idiopathic Arthritis (JIA). Consecutive patients with newly diagnosed JIA were included prospectively from defined geographic areas of the Nordic countries from 1997 to 2000. Inclusion criteria were availability of a baseline serum sample within 12 months after disease onset and 8-year clinical assessment data. Systemic onset JIA was not included. CRP was measured by high-sensitive ELISA (detection limit of 0...
March 10, 2017: Rheumatology International
https://www.readbyqxmd.com/read/28283723/intravoxel-incoherent-motion-magnetic-resonance-imaging-of-the-knee-joint-in-children-with-juvenile-idiopathic-arthritis
#9
Fabian Hilbert, Annette Holl-Wieden, Alexander Sauer, Herbert Köstler, Henning Neubauer
BACKGROUND: MRI of synovitis relies on use of a gadolinium-based contrast agent. Diffusion-weighted MRI (DWI) visualises thickened synovium but is of limited use in the presence of joint effusion. OBJECTIVE: To investigate the feasibility and diagnostic accuracy of diffusion-weighted MRI with intravoxel incoherent motion (IVIM) for diagnosing synovitis in the knee joint of children with juvenile idiopathic arthritis. MATERIALS AND METHODS: Twelve consecutive children with confirmed or suspected juvenile idiopathic arthritis (10 girls, median age 11 years) underwent MRI with contrast-enhanced T1-weighted imaging and DWI at 1...
March 10, 2017: Pediatric Radiology
https://www.readbyqxmd.com/read/28277841/safety-of-tocilizumab-in-the-treatment-of-juvenile-idiopathic-arthritis
#10
Ricardo Machado Xavier, Sandra Helena Machado
Tocilizumab (TCZ) is a recombinant humanized monoclonal antibody and IL-6 receptor antagonist, currently approved for the treatment of systemic juvenile idiopathic arthritis (sJIA) and polyarticular juvenile idiopathic arthritis (pJIA) in children aged 2 years or older refractory to conventional treatment. The most common adverse events in patients treated with TCZ were infections, especially in the respiratory tract. The most frequent laboratory abnormalities were altered liver function, neutropenia and elevated cholesterol levels...
March 6, 2017: Expert Opinion on Drug Safety
https://www.readbyqxmd.com/read/28266235/canakinumab-for-first-line-steroid-free-treatment-in-a-child-with-systemic-onset-juvenile-idiopathic-arthritis
#11
G Horneff, J Peitz, J Kekow, D Foell
No abstract text is available yet for this article.
March 7, 2017: Scandinavian Journal of Rheumatology
https://www.readbyqxmd.com/read/28257625/association-of-il-10-gene-polymorphisms-and-susceptibility-to-juvenile-idiopathic-arthritis-in-egyptian-children-and-adolescents-a-case-control-study
#12
Manar M Fathy, Hosam F Elsaadany, Yasser F Ali, Mohsen A A Farghaly, Mohammed E Hamed, Hany E Ibrahim, Maha A Noah, Mayy A N Allah, Shaimaa S A Elashkar, Nasser I Abdelsalam, Hind M Abdelrahman, Ahmed R Ahmed, Heba G Anany, Sanaa M Ismail, Boshra R Ibrahim, Nashwa M Al Azizi, Heba H Gawish, Ghada M Al-Akad, Rehab M Nabil, Dalia S Fahmy, Salah F Alsayed
BACKGROUND: Juvenile Idiopathic Arthritis (JIA) is the most common chronic arthritis in children worldwide. Among anti-inflammatory cytokines, interleukin-10 (IL-10) is a key immunosuppressive cytokine involved in the pathogenesis of JIA. To date, only a few studies concerned the association of interleukin-10 gene polymorphisms with JIA. In this study, we aimed to investigate 3 cytokine single-nucleotide polymorphisms situated at positions -1082(G/A), -819(C/T), and -592(C/A) in the promoter region of the IL-10 gene to determine whether this polymorphism could be a marker of susceptibility to JIA in Egyptian children and adolescents...
January 17, 2017: Italian Journal of Pediatrics
https://www.readbyqxmd.com/read/28251541/profile-of-pediatric-idiopathic-inflammatory-myopathies-from-a-tertiary-care-center-of-eastern-india
#13
Sumantra Sarkar, Tanushree Mondal, Arpan Saha, Rakesh Mondal, Supratim Datta
OBJECTIVES: To report data on Idiopathic inflammatory myopathies (IIM) from eastern India. METHODS: All IIM patients diagnosed over the last 5 y (2011-2016) were included through a retrospective review of records from the hospital and specialty clinic at Institute of Postgraduate Medical Education & Research (I.P.G.M.E.&R.), Kolkata. RESULTS: Out of the 11 IIM patients, 9 had Juvenile dermatomyositis (JDM) and 2 had overlap myositis (OM) [with systemic lupus erythematosus (SLE) and scleroderma]...
March 2, 2017: Indian Journal of Pediatrics
https://www.readbyqxmd.com/read/28251009/idiopathic-pulmonary-hemosiderosis-in-a-child-with-recurrent-macrophage-activation-syndrome-secondary-to-systemic-juvenile-idiopathic-arthritis
#14
Kenan Barut, Sezgin Sahin, Amra Adrovic, Velat Sen, Ozgur Kasapcopur
Macrophage activation syndrome, a severe complication of systemic juvenile idiopathic arthritis and other inflammatory diseases, represents one of the most important rheumatological emergencies. Delayed diagnosis could lead to life-threatening complications. Pulmonary hemosiderosis has been classically characterized by a triad of anemia, hemoptysis, and lung infiltrates on chest radiogram. Although the majority of patients of pulmonary hemosiderosis are considered idiopathic, secondary hemosiderosis associated with known diseases could be seen...
2017: Case Reports in Pediatrics
https://www.readbyqxmd.com/read/28241879/time-to-diagnosis-in-juvenile-idiopathic-arthritis-a-french-perspective
#15
Laura Aoust, Linda Rossi-Semerano, Isabelle Koné-Paut, Perrine Dusser
BACKGROUND: Juvenile idiopathic arthritis (JIA) is a rare disease that is not widely known by paediatricians and general practitioner (GP) leading to diagnostic error and delayed care provision. We aimed to analyse patient's journey and time to diagnosis of JIA (delay from the first symptom to the diagnosis of JIA). We performed a retrospective cohort study of 67 patients diagnosed with JIA and seen in the paediatric rheumatology department of the Kremlin Bicêtre Hospital, between July 2002 and January 2015...
February 28, 2017: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/28229369/course-outcome-and-complications-in-children-with-systemic-onset-juvenile-idiopathic-arthritis
#16
Mansi Dewoolkar, Rolando Cimaz, Pranav Raman Chickermane, Raju P Khubchandani
OBJECTIVES: To assess the course, outcome and complications in a mono-centric cohort of 53 patients with systemic onset juvenile idiopathic arthritis (s-JIA). METHODS: In an observational study, 53 consecutive patients diagnosed with s-JIA on or before October 2009 were enrolled and followed up between October 2009 and September 2012. At each 6-12 weekly visit, clinical examination, laboratory investigations and details of on-going treatment were recorded. Disease course was classified as monocyclic, intermittent and persistent...
February 23, 2017: Indian Journal of Pediatrics
https://www.readbyqxmd.com/read/28225869/extremely-elevated-il-18-levels-may-help-distinguish-systemic-onset-juvenile-idiopathic-arthritis-from-other-febrile-diseases
#17
Y Xia, P Cui, Q Li, F Liang, C Li, J Yang
The aim of this research was to explore whether IL-18 can be a serological marker for the diagnosis of systemic-onset juvenile idiopathic arthritis (sJIA). A total of 23 sJIA patients (13 males, median age 8.2), 20 acute lymphoblastic leukemia (ALL) patients, 18 patients with severe infections (SIF), 26 Kawasaki disease (KD) patients, 18 juvenile idiopathic arthritis (JIA) patients, and 25 healthy control patients were selected for this study. Enzyme-linked immunosorbent assays (ELISAs) were used to determine the serum concentrations of the S100A8, S100A9, and IL-6 proteins...
February 16, 2017: Brazilian Journal of Medical and Biological Research, Revista Brasileira de Pesquisas Médicas e Biológicas
https://www.readbyqxmd.com/read/28217950/impact-of-uveitis-on-quality-of-life-in-adult-patients-with-juvenile-idiopathic-arthritis
#18
Anne-Mieke J W Haasnoot, Naïlah F M Sint Jago, Janneke Tekstra, Joke H de Boer
OBJECTIVE: To establish the impact of uveitis on the Quality of Life (QOL) in adult patients with juvenile idiopathic arthritis (JIA). METHODS: Adult patients with a history of JIA, with (n=31) or without (n=51) chronic anterior uveitis, were included. Their scores on three validated QOL questionnaires (National Eye Institute Visual Functioning Questionnaire (NEI VFQ-25), Medical Outcomes Study 36-Item Short Form Health Survey (SF-36) and EuroQol-5D (EQ-5D)) were analyzed to find factors that could influence QOL...
February 19, 2017: Arthritis Care & Research
https://www.readbyqxmd.com/read/28202118/-clinical-and-laboratory-features-of-macrophage-activation-syndrome
#19
Li Guo, Mei-Ping Lu, Gui-Juan Dong, Li-Ping Teng, Yi-Ping Xu, Li-Xia Zou, Qi Zheng
OBJECTIVE: To study the clinical and laboratory features of macrophage activation syndrome (MAS) at the early stage of diagnosis, and to explore a method for early identification of MAS. METHODS: A retrospective analysis was performed for the demographic data, clinical and laboratory features, and treatment outcomes of 21 MAS patients. RESULTS: Of the 21 MAS patients, 14 had systemic juvenile idiopathic arthritis, 5 had Kawasaki disease (KD), and 2 had connective tissue disease (CTD) as primary diseases...
February 2017: Zhongguo Dang Dai Er Ke za Zhi, Chinese Journal of Contemporary Pediatrics
https://www.readbyqxmd.com/read/28182665/padi4-and-the-hla-drb1-shared-epitope-in-juvenile-idiopathic-arthritis
#20
Kaori Hisa, Masakatsu D Yanagimachi, Takuya Naruto, Takako Miyamae, Masako Kikuchi, Rhoki Hara, Tomoyuki Imagawa, Shumpei Yokota, Masaaki Mori
OBJECTIVE: Both genetic and environmental factors are associated with susceptibility to juvenile idiopathic arthritis (JIA). Many studies have reported that both a 'shared epitope' (SE) encoded by several HLA-DRB1 alleles and the peptidyl arginine deiminase type 4 (PADI4) gene polymorphisms are associated with susceptibility to rheumatoid arthritis (RA). However, it is uncertain whether JIA and RA share the latter genetic risk factor. Therefore, here we investigated relationships between HLA-SE and PADI4 polymorphisms with clinical subtypes of JIA...
2017: PloS One
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