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Pediatric Rheumatology Online Journal

Dima Chbeir, Jean Gaschignard, Ronan Bonnefoy, Constance Beyler, Isabelle Melki, Albert Faye, Ulrich Meinzer
BACKGROUND: Kawasaki disease (KD) is an acute febrile systemic vasculitis that affects small and medium blood vessels. Intensified treatments for the most severely affected patients have been proposed recently, and the early identification of KD patients at high risk for coronary artery aneurysms (CAA) is crucial. However, the risk scoring systems developed in Japan have not been validated in European populations, and little data is available concerning the link between initial echocardiogram findings other than high z-scores and cardiac prognosis...
July 18, 2018: Pediatric Rheumatology Online Journal
Hanna Rahimi, Marinka Twilt, Troels Herlin, Lynn Spiegel, Thomas Klit Pedersen, Annelise Küseler, Peter Stoustrup
BACKGROUND: Little is known about the chronicity of orofacial symptoms and how this influences the oral health-related quality of life in juvenile idiopathic arthritis (JIA). Therefore, our objectives were to study the long-term changes in self-reported orofacial symptoms, and to define the impact of orofacial symptoms on oral health-related quality of life in JIA. METHODS: At baseline (T0), 157 consecutive JIA patients ≤20 years completed a patient pain questionnaire that incorporates domains related to the orofacial area...
July 13, 2018: Pediatric Rheumatology Online Journal
Giovanna Ferrara, Greta Mastrangelo, Patrizia Barone, Francesco La Torre, Silvana Martino, Giovanni Pappagallo, Angelo Ravelli, Andrea Taddio, Francesco Zulian, Rolando Cimaz
BACKGROUND: Conventional pharmacological therapies for the treatment of juvenile idiopathic arthritis (JIA) consist of non-biological, disease-modifying antirheumatic drugs, among which methotrexate (MTX) is the most commonly prescribed. However, there is a lack of consensus-based clinical and therapeutic recommendations for the use of MTX in the management of patients with JIA. Therefore, the Methotrexate Advice and RecommendAtions on Juvenile Idiopathic Arthritis (MARAJIA) Expert Meeting was convened to develop evidence-based recommendations for the use of MTX in the treatment of JIA...
July 11, 2018: Pediatric Rheumatology Online Journal
Hermine I Brunner, Lisa G Rider, Daniel J Kingsbury, Dominic Co, Rayfel Schneider, Ellen Goldmuntz, Karen B Onel, Edward H Giannini, Daniel J Lovell
IMPORTANCE: Specialized research networks are essential to achieve drug approvals for rare pediatric diseases. Such networks help realize the potential of global legislation enacted upon the recognition that most children are treated with drugs whose most beneficial dose and regimen have not been established in pediatric patients. The Pediatric Rheumatology Collaborative Study Group (PRCSG) is a North American clinical trials network that is specialized in the performance of clinical trials of new therapies for pediatric populations with rheumatic diseases...
July 11, 2018: Pediatric Rheumatology Online Journal
Qianzi Zhao, Lawrence K Jung
BACKGROUND: To understand the relationship between regulatory B cells (Bregs) and juvenile idiopathic arthritis (JIA), we analyzed the percentages of Bregs and their function in peripheral blood (PB) and synovial fluid (SF) of JIA patients. METHODS: Twenty-one JIA patients and 11 children with growing pain but without known rheumatic diseases as controls were included. The B cell phenotype and intracellular production of IL-10 of Bregs were assessed by flow cytometry...
July 4, 2018: Pediatric Rheumatology Online Journal
Fajer Altammar, Bianca Lang
BACKGROUND: Kawasaki Disease (KD), the leading cause of acquired heart disease in children in the developed world, is extremely rare in neonates. We present a case of incomplete KD in a neonate and a review of the literature on neonatal KD. CASE PRESENTATION: A previously healthy full term 15 day old Caucasian male with an unremarkable antenatal and perinatal history, presented on Day 2 of illness with fever, rash, irritability, and poor feeding. Examination revealed fever (39...
July 3, 2018: Pediatric Rheumatology Online Journal
Daniel J Lovell, Jason A Dare, Megan Francis-Sedlak, Julie Ball, Brian D LaMoreaux, Emily Von Scheven, Adam Reinhardt, Rita Jerath, Oral Alpan, Ramesh Gupta, Donald Goldsmith, Andrew Zeft, Henry Naddaf, Beth Gottlieb, Lawrence Jung, Robert J Holt
BACKGROUND: Juvenile idiopathic arthritis (JIA) is an inflammatory arthritis of unknown etiology, which lasts for greater than 6 weeks with onset before 16 years of age. JIA is the most common chronic rheumatic disease in children. NSAIDs have been the mainstay of initial management with naproxen (NAP) being commonly used, but they may cause serious side effects such as gastric ulcers which can be reduced by concomitant administration of proton pump inhibitors, such as esomeprazole (ESO)...
June 26, 2018: Pediatric Rheumatology Online Journal
Claas H Hinze, Prasad T Oommen, Frank Dressler, Andreas Urban, Frank Weller-Heinemann, Fabian Speth, Elke Lainka, Jürgen Brunner, Heike Fesq, Dirk Foell, Wolfgang Müller-Felber, Ulrich Neudorf, Christoph Rietschel, Tobias Schwarz, Ulrike Schara, Johannes-Peter Haas
BACKGROUND: Juvenile dermatomyositis (JDM) is the most common inflammatory myopathy in childhood and a major cause of morbidity among children with pediatric rheumatic diseases. The management of JDM is very heterogeneous. The JDM working group of the Society for Pediatric Rheumatology (GKJR) aims to define consensus- and practice-based strategies in order to harmonize diagnosis, treatment and monitoring of JDM. METHODS: The JDM working group was established in 2015 consisting of 23 pediatric rheumatologists, pediatric neurologists and dermatologists with expertise in the management of JDM...
June 25, 2018: Pediatric Rheumatology Online Journal
Kim Ohl, Helge Nickel, Halima Moncrieffe, Patricia Klemm, Anja Scheufen, Dirk Föll, Viktor Wixler, Angela Schippers, Norbert Wagner, Lucy R Wedderburn, Klaus Tenbrock
BACKGROUND: Inflammatory effector T cells trigger inflammation despite increased numbers of Treg cells in the synovial joint of patients suffering from juvenile idiopathic arthritis (JIA). The cAMP response element (CREM)α is known to play a major role in regulation of T cells in SLE, colitis, and EAE. However, its role in regulation of effector T cells within the inflammatory joint is unknown. METHODS: CREM expression was analyzed in synovial fluid cells from oligoarticular JIA patients by flow cytometry...
June 20, 2018: Pediatric Rheumatology Online Journal
Claas H Hinze, Fabian Speth, Prasad T Oommen, Johannes-Peter Haas
BACKGROUND: Juvenile Dermatomyositis (JDM) is a rare pediatric autoimmune disease with broad variations of the individual course. Data on the optimal management are mostly lacking. Currently treatment decisions are often based on experts' opinions. In order to develop consensus-based treatment strategies for JDM in Germany a survey was pursued to analyze the current clinical practice. METHODS: An online survey addressing all members of the Society for Pediatric Rheumatology (GKJR) in Germany and Austria and pediatric neurologists with expertise in JDM was performed in February/March of 2016...
June 20, 2018: Pediatric Rheumatology Online Journal
Anna Agazzi, Gloria Fadanelli, Fabio Vittadello, Francesco Zulian, Giorgia Martini
OBJECTIVES: To assess reliability of the two indexes of Localized Scleroderma Cutaneous Assessment Tool (LoSCAT), the modified Localized Scleroderma Skin Severity Index (mLoSSI) and the Localized Scleroderma Skin Damage Index (LoSDI), when applied by clinicians with different experience in scoring and managing patients with JLS. Secondary aim was to compare LoSCAT and infrared thermography (IRT) in monitoring lesions over time. METHODS: Consecutive children with Juvenile Localized Scleroderma (JLS) were blindly evaluated by three examiners with different experience in Paediatric Rheumatology and with no experience in LoSCAT use...
June 18, 2018: Pediatric Rheumatology Online Journal
Yuzaburo Inoue, Kanako Mitsunaga, Takeshi Yamamoto, Koki Chiba, Fumiya Yamaide, Taiji Nakano, Yoshinori Morita, Akiko Yamaide, Shuichi Suzuki, Takayasu Arima, Ken-Ichi Yamaguchi, Minako Tomiita, Naoki Shimojo, Yoichi Kohno
BACKGROUND: Bisphosphonates are recommended for use as first-line therapy for the prevention and treatment of glucocorticoid-induced osteoporosis in adults. However, the appropriate usage of bisphosphonates for the prevention or treatment of glucocorticoid-induced osteoporosis in children remains unclear. METHODS: We performed a cross-sectional study to clarify the factors associated with the development of glucocorticoid-induced bone loss and osteoporosis in patients with childhood-onset rheumatic disease and to investigate the impact of the early use of alendronate...
June 18, 2018: Pediatric Rheumatology Online Journal
Suzanne Parsons, Wendy Thomson, Katharine Cresswell, Bella Starling, Janet E McDonagh
BACKGROUND: Involving people of all ages in health research is now widely advocated. To date, no studies have explored whether and how young people with chronic rheumatic conditions want to be involved in influencing health research. This study aimed to explore amongst young people with rheumatic conditions, 1) their experiences of research participation and involvement 2) their beliefs about research involvement and 3) beliefs about how young people's involvement should be organized in the future...
May 24, 2018: Pediatric Rheumatology Online Journal
Sarah L Finch, Alan M Rosenberg, Hassan Vatanparast
BACKGROUND: Vitamin D has been implicated in the pathogenesis of autoimmune diseases. While the roles of vitamin D in other autoimmune diseases have been investigated, less is known about the role of vitamin D in chronic childhood arthritis. MAIN BODY: This review summarizes and evaluates evidence relating to 25-hydroxyvitamin D (25(OH)D) and chronic childhood arthritis. A scoping literature review was conducted using Ovid Medline, Ovid Embase, Cumulative Index to Nursing and Allied Health Literature, Web of Science and Scopus...
May 16, 2018: Pediatric Rheumatology Online Journal
Xin-Yan Zhang, Ting-Ting Yang, Xiu-Fen Hu, Yu Wen, Feng Fang, Hui-Ling Lu
BACKGROUND: The pathogenesis of Kawasaki disease are still not well understood. It was designed to investigate the relationship between adipokines including chemerin, omentin-1, adiponectin and acute Kawasaki disease. METHODS: Enzyme-linked immunosorbent (ELISA) was used to detect serum levels of chemerin, omentin-1, adiponectin, and inflammatory cytokines IL-1β and TNF-α in 80 cases of patients diagnosed with Kawasaki disease (KD). In addition, 20 cases of children with fever and 20 cases of healthy children were selected as febrile and normal controls...
May 8, 2018: Pediatric Rheumatology Online Journal
Matthew L Stoll, Chung H Kau, Peter D Waite, Randy Q Cron
BACKGROUND: Arthritis involving the temporomandibular joint (TMJ) complicates 40 - 96% of cases of juvenile idiopathic arthritis (JIA), potentially leading to devastating changes to form and function. Optimal evaluation and management of this joint remains a matter of ongoing discussion. METHODS: We performed a PubMed search for all articles with keywords "temporomandibular" and "arthritis", covering the dates 2002 through February 28, 2018. A separate PubMed search was performed for all articles with keywords "temporomandibular joint", "arthritis", and "treatment" covering the same dates...
April 25, 2018: Pediatric Rheumatology Online Journal
Hsiao-Feng Chou, Yu-Hung Wu, Che-Sheng Ho, Yu-Hsuan Kao
BACKGROUND: This study aimed to evaluate the demographic, clinical features, laboratory data, pathology and other survey in pediatric patients with cryofibrinogenemia. METHODS: A 12-year retrospective chart review identified eight pediatric patients at Mackay Memorial Hospital, Taipei, Taiwan. RESULTS: The female-to-male ratio was 3:1. The mean age at symptom onset and of diagnosis was 10.3 ± 4.6 years and 12.3 ± 4 years, respectively...
April 24, 2018: Pediatric Rheumatology Online Journal
Merav Heshin-Bekenstein, Liat Perl, Aimee O Hersh, Emily von Scheven, Ed Yelin, Laura Trupin, Jinoos Yazdany, Erica F Lawson
BACKGROUND: To compare final height to mid-parental target height among adults with childhood-onset systemic lupus erythematosus (cSLE) versus adult-onset SLE (aSLE), and to evaluate the impact of age at SLE onset on final height. METHODS: Data derived from the Lupus Outcomes Study, a longitudinal cohort of adults with SLE, was used for this cross-sectional analysis (N = 728). Participants aged 18-63 years with complete height data were included (N = 566) and were classified as cSLE if age at diagnosis was < 18 years (N = 72)...
April 23, 2018: Pediatric Rheumatology Online Journal
Romina Gallizzi, Caterina Pidone, Luca Cantarini, Martina Finetti, Marco Cattalini, Giovanni Filocamo, Antonella Insalaco, Donato Rigante, Rita Consolini, Maria Cristina Maggio, Adele Civino, Silvana Martino, Alma Nunzia Olivieri, Giovanna Fabio, Serena Pastore, Angela Mauro, Diana Sutera, Giuseppe Trimarchi, Nicolino Ruperto, Marco Gattorno, Rolando Cimaz
Following publication of the original article [1], the authors reported that the names of two institutional authors - EUROFEVER and the Paediatric Rheumatology International Trials Organisation (PRINTO) - had been unintentionally omitted in the final online version of the manuscript. The corrected author list is shown in this Correction..
April 23, 2018: Pediatric Rheumatology Online Journal
Heather O Tory, Ruy Carrasco, Thomas Griffin, Adam M Huber, Philip Kahn, Angela Byun Robinson, David Zurakowski, Susan Kim
BACKGROUND: A standardized set of quality measures for juvenile idiopathic inflammatory myopathies (JIIM) is not in use. Discordance has been shown between the importance ascribed to quality measures between patients and families and physicians. The objective of this study was to assess and compare the importance of various aspects of high quality care to patients with JIIM and their families with healthcare providers, to aid in future development of comprehensive quality measures. METHODS: Surveys were developed by members of the Childhood Arthritis and Rheumatology Research Alliance (CARRA) Juvenile Dermatomyositis Workgroup through a consensus process and administered to patients and families through the CureJM Foundation and to healthcare professionals through CARRA...
April 19, 2018: Pediatric Rheumatology Online Journal
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