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Pediatric Rheumatology Online Journal

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https://www.readbyqxmd.com/read/28427473/intestinal-beh%C3%A3-et-and-crohn-s-disease-two-sides-of-the-same-coin
#1
REVIEW
Simona Valenti, Romina Gallizzi, Dominique De Vivo, Claudio Romano
Behçet's disease (BD) and Crohn's disease (CD) are chronic immune-mediated, inflammatory disorders affecting many different systems (joints, skin, eyes, gastrointestinal and biliary tracts). Both disorders have fluctuating courses and when gastrointestinal symptoms are prevalent, differential diagnosis can be difficult. BD involves the gastrointestinal tract in 10-15% of cases with localized lesions in the ileocecal region. The clinical picture is heterogeneous with various clusters of disease expression. CD is a chronic inflammatory disorder, which can affect any part of the intestinal tract, as well as extra-intestinal tissue...
April 20, 2017: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/28427414/observational-study-of-interleukin-21-il-21-does-not-distinguish-kawasaki-disease-from-other-causes-of-fever-in-children
#2
Rachel Engelberg, Meghan Martin, Brian H Wrotniak, Mark Daniel Hicar
BACKGROUND: Kawasaki disease (KD) is a febrile childhood vasculitis of unknown etiology. The diagnosis is highly concerning as over a quarter of children who fail to receive timely treatment with intravenous immunoglobulin (IVIG) will develop coronary aneurysms. Diagnosis relies on proper symptomatology and is supported by non-specific markers of inflammation. Previous studies have identified elevated plasma levels of interleukin-21 (IL-21) as a sensitive and specific biomarker in KD...
April 20, 2017: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/28424093/a-survey-of-national-and-multi-national-registries-and-cohort-studies-in-juvenile-idiopathic-arthritis-challenges-and-opportunities
#3
Timothy Beukelman, Janneke Anink, Lillemor Berntson, Ciaran Duffy, Justine A Ellis, Mia Glerup, Jaime Guzman, Gerd Horneff, Lianne Kearsley-Fleet, Ariane Klein, Jens Klotsche, Bo Magnusson, Kirsten Minden, Jane E Munro, Martina Niewerth, Ellen Nordal, Nicolino Ruperto, Maria Jose Santos, Laura E Schanberg, Wendy Thomson, Lisette van Suijlekom-Smit, Nico Wulffraat, Kimme Hyrich
BACKGROUND: To characterize the existing national and multi-national registries and cohort studies in juvenile idiopathic arthritis (JIA) and identify differences as well as areas of potential future collaboration. METHODS: We surveyed investigators from North America, Europe, and Australia about existing JIA cohort studies and registries. We excluded cross-sectional studies. We captured information about study design, duration, location, inclusion criteria, data elements and collection methods...
April 19, 2017: Pediatric Rheumatology Online Journal
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
#4
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/28416004/clinical-study-of-children-with-takayasu-arteritis-a-retrospective-study-from-a-single-center-in-china
#5
Ye Feng, Xuemei Tang, Mingyue Liu, Juan Zhou, Xiaodong Zhao, Qiu Li
BACKGROUND: Delayed diagnosis of childhood Takayasu arteritis (TA) is common due to its atypical symptoms. The objective of the present study was to summarize the clinical features of childhood TA to raise awareness and improve management. METHODS: Eleven children diagnosed with TA at our hospital were enrolled. Clinical information, diagnosis, treatment, and outcome were then examined retrospectively. The Pediatric Vasculitis Activity Score (PVAS) and the Indian Takayasu Clinical Activity Score (ITAS2010) were used to assess disease activity...
April 17, 2017: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/28410589/lung-involvement-in-childhood-onset-granulomatosis-with-polyangiitis
#6
REVIEW
Giovanni Filocamo, Sofia Torreggiani, Carlo Agostoni, Susanna Esposito
Granulomatosis with polyangiitis is an ANCA-associated systemic vasculitis with a low incidence in the pediatric population. Lung involvement is a common manifestation in children affected by granulomatosis with polyangiitis, both at disease's onset and during flares. Its severity is variable, ranging from asymptomatic pulmonary lesions to dramatic life-threatening clinical presentations such as diffuse alveolar haemorrhage. Several radiologic findings have been described, but the most frequent abnormalities detected are nodular lesions and fixed infiltrates...
April 14, 2017: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/28407779/histologic-effects-of-mandibular-protrusion-splints-in-antigen-induced-tmj-arthritis-in-rabbits
#7
Julia von Bremen, Kernt Köhler, Krystyna Siudak, Daniel Zahner, Sabine Ruf
BACKGROUND: Although it is common clinical practice to treat children with Juvenile Idiopathic Arthritis (JIA) with functional appliances, the scientific evidence for this is limited. The aim of this study was to study the histologic effects of mandibular protrusion splints in temporomandibular joint (TMJ) arthritis in rabbits. METHODS: Twenty-eight ten-week old New Zealand white rabbits were randomly divided into four groups: AO (TMJ arthritis, no splint), AS (TMJ arthritis, mandibular splint advancement), OS (no arthritis, mandibular splint advancement) and OO (no arthritis, no splint)...
April 13, 2017: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/28403889/muscle-mri-at-the-time-of-questionable-disease-flares-in-juvenile-dermatomyositis-jdm
#8
Rabheh Abdul-Aziz, Chack-Yung Yu, Brent Adler, Sharon Bout-Tabaku, Katherine E Lintner, Melissa Moore-Clingenpeel, Charles H Spencer
BACKGROUND: The course of JDM has improved substantially over the last 70 years with early and aggressive treatments. Yet it remains difficult to detect disease flares as symptoms may be mild; signs of rash and muscle weakness vary widely and are often equivocal; laboratory tests of muscle enzyme levels are often normal; electromyography and muscle biopsy are invasive. Alternative tools are needed to help decide if more aggressive treatment is needed. Our objective is to determine the effectiveness of muscle Magnetic Resonance Imaging (MRI) in detecting JDM flares, and how an MRI affects physician's decision-making regarding treatment...
April 12, 2017: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/28403864/health-related-quality-of-life-among-swedish-children-with-juvenile-idiopathic-arthritis-parent-child-discrepancies-gender-differences-and-comparison-with-a-european-cohort
#9
Veronica Lundberg, Catharina Eriksson
BACKGROUND: This study investigates gender differences in self-reports and between parent and child reports in Health-related Quality of Life (HRQOL), measured with disease-specific and generic instruments for chronic disease. Comparison of HRQOL results in this Juvenile Idiopathic Arthritis (JIA) sample to a European cohort of children with JIA and one of children with other health conditions are also made. METHODS: Fifty-three children with juvenile idiopathic arthritis (JIA), aged 8-18 years, and their parents completed the condition-specific DISABKIDS for JIA, and the DISABKIDS generic instrument for chronic conditions (DCGM-37) in a cross-sectional study...
April 12, 2017: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/28399931/pilot-study-comparing-the-childhood-arthritis-rheumatology-research-alliance-carra-systemic-juvenile-idiopathic-arthritis-consensus-treatment-plans
#10
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/28399930/radiographic-damage-in-hands-and-wrists-of-patients-with-juvenile-idiopathic-arthritis-after-29%C3%A2-years-of-disease-duration
#11
Anne M Selvaag, Eva Kirkhus, Lena Törnqvist, Vibke Lilleby, Hanne A Aulie, Berit Flatø
BACKGROUND: There are few studies on radiographic outcome after long-term disease duration in juvenile idiopathic arthritis (JIA). We wanted to evaluate 29-year radiographic outcome in hands/wrists and predictors of damage in patients with long-term active JIA. METHODS: Patients diagnosed from 1980 to 1985, who had active disease at 15-, 23- or 29-year follow-up and arthritis in the wrists during the disease course, were reexamined with radiographs of hands/wrists...
April 11, 2017: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/28399927/bk-viruria-and-viremia-in-children-with-systemic-lupus-erythematosus
#12
Nirupama Gupta, Cuong Q Nguyen, Renee F Modica, Melissa E Elder, Eduardo H Garin
BACKGROUND: BK virus (BKV) is a ubiquitous polyoma virus that lies dormant in the genitourinary tract once acquired in early childhood. In states of cellular immunodeficiency, the virus can reactivate to cause hemorrhagic cystitis and nephritis. Children with systemic lupus erythematosus (SLE) have an increased risk of developing infectious complications secondary to their immunocompromised state from the administration of several immuno-modulatory drugs. Currently, there are no data regarding the prevalence of BK viruria or viremia in children with SLE...
April 11, 2017: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/28399893/ultrasound-guided-steroid-tendon-sheath-injections-in-juvenile-idiopathic-arthritis-a-10-year-single-center-retrospective-study
#13
Shannon E Peters, Ronald M Laxer, Bairbre L Connolly, Dimitri A Parra
BACKGROUND: The aims of this study were to: (a) Identify tendon sheaths most commonly treated with steroid injections in a pediatric patient population with Juvenile Idiopathic Arthritis (JIA); (b) Describe technical aspects of the procedure; (c) Characterize sonographic appearance of tenosynovitis in JIA; (d) Assess agreement between clinical request and sites injected. METHODS: This was a 10 year single-center retrospective study (May 2006-April 2016) of patients with JIA referred by Rheumatology for ultrasound-guided tendon sheath injections...
April 11, 2017: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/28399875/asas-definition-for-sacroiliitis-on-mri-in-spa-applicable-to-children
#14
Nele Herregods, Joke Dehoorne, Filip Van den Bosch, Jacob Lester Jaremko, Joke Van Vlaenderen, Rik Joos, Xenofon Baraliakos, Gaëlle Varkas, Koenraad Verstraete, Dirk Elewaut, Lennart Jans
BACKGROUND: The Assessment of Spondyloarthritis International Society (ASAS) definition for a 'positive' Magnetic Resonance Imaging (MRI) for sacroiliitis is well studied and validated in adults, but studies about the value of this definition in children are lacking. The aim of this study is to evaluate whether the adult ASAS definition of a positive MRI of the sacroiliac joints can be applied to children with a clinical suspicion of Juvenile Spondyloarthritis (JSpA). METHODS: Two pediatric musculoskeletal radiologists blinded to clinical data independently retrospectively reviewed sacroiliac (SI) joint MRI in 109 children suspected of sacroiliitis...
April 11, 2017: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/28381287/munchausen-by-proxy-syndrome-mimicking-systemic-autoinflammatory-disease-case-report-and-review-of-the-literature
#15
Helmut Wittkowski, Claas Hinze, Sigrid Häfner-Harms, Vinzenz Oji, Katja Masjosthusmann, Martina Monninger, Ulrike Grenzebach, Dirk Foell
BACKGROUND: Systemic autoinflammatory diseases (SAIDs) represent a growing number of monogenic, polygenic or multifactorial disorders that are often difficult to diagnose. CASE PRESENTATION: Here we report a patient who was initially erroneously diagnosed and treated for SAID. Symptoms consisted of recurrent fever, erythematous and/or blistering skin lesions, angioedema, susceptibility to bleeding, external ear infections and reversible anisocoria in the absence of laboratory evidence of systemic inflammation...
April 5, 2017: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/28356102/methods-for-analyzing-observational-longitudinal-prognosis-studies-for-rheumatic-diseases-a-review-worked-example-using-a-clinic-based-cohort-of-juvenile-dermatomyositis-patients
#16
REVIEW
Lily Siok Hoon Lim, Eleanor Pullenayegum, Rahim Moineddin, Dafna D Gladman, Earl D Silverman, Brian M Feldman
Most outcome studies of rheumatic diseases report outcomes ascertained on a single occasion. While single assessments are sufficient for terminal or irreversible outcomes, they may not be sufficiently informative if outcomes change or fluctuate over time. Consequently, longitudinal studies that measure non-terminal outcomes repeatedly afford a better understanding of disease evolution.Longitudinal studies require special analytic methods. Newer longitudinal analytic methods have evolved tremendously to deal with common challenges in longitudinal observational studies...
March 29, 2017: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/28320400/predictors-for-intravenous-immunoglobulin-resistance-and-coronary-artery-lesions-in-kawasaki-disease
#17
Tian Xie, Ying Wang, Songling Fu, Wei Wang, Chunhong Xie, Yiying Zhang, Fangqi Gong
BACKGROUND: To assess the predictors for intravenous immunoglobulin (IVIG) resistance and coronary artery lesions (CALs) in Kawasaki disease (KD). METHODS: A total of 560 KD patients were reviewed retrospectively, including 410 complete KD (cKD) and 150 incomplete KD (iKD) patients. The laboratory data were compared between the IVIG-resistant and IVIG-responsive groups, as well as between the coronary artery lesions (CALs+) and without coronary artery lesions (CALs-) groups...
March 21, 2017: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/28288653/inflammatory-bowel-disease-following-anti-interleukin-1-treatment-in-systemic-juvenile-idiopathic-arthritis
#18
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/28257644/a-single-center-analysis-of-henoch-schonlein-purpura-nephritis-with-nephrotic-proteinuria-in-children
#19
Dan Feng, Wen-Yan Huang, Sheng Hao, Xiao-Ling Niu, Ping Wang, Ying Wu, Guang-Hua Zhu
BACKGROUND: In children with Henoch-Schonlein purpura nephritis (HSPN), the degree of proteinuria has been proven to be not only a sign of kidney damage, but also an accelerator of kidney disease progression. Nephrotic proteinuria at disease onset has been proposed as a predictor of a poor renal outcome. This study aims to assess the clinical and pathological features of HSPN with nephrotic proteinuria in a single center. METHODS: One hundred thirty-seven patients with HSPN who visited Shanghai Children's Hospital from January 2009 to December 2013 were retrospectively reviewed...
March 4, 2017: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/28231857/missed-opportunities-for-timely-diagnosis-of-pediatric-lupus-in-south-africa-a-qualitative-study
#20
Laura B Lewandowski, Melissa H Watt, Laura E Schanberg, Nathan M Thielman, Christiaan Scott
BACKGROUND: Systemic Lupus Erythematosus (SLE) is a serious multisystem autoimmune disease, which is more aggressive in children and people of African descent. In South Africa, pediatric SLE (pSLE) patients are at high risk for severe disease. Similar to pSLE worldwide, South African children and adolescents with SLE require subspecialized medical care. The aim of this study is to describe the care-seeking experiences of families and examine factors that contribute to delays in the diagnosis of pSLE...
February 23, 2017: Pediatric Rheumatology Online Journal
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