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Rheumatology pediatrics

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https://www.readbyqxmd.com/read/28079914/intravenous-immunoglobulin-in-pediatric-rheumatology-when-to-use-it-and-what-is-the-evidence
#1
Martha M Rodriguez, Linda Wagner-Weiner
Intravenous immunoglobulin (IVIG) is given to children with a variety of rheumatologic illnesses. The mechanism of action by which it exerts therapeutic effects is not well understood and likely differs in the medical conditions for which it is given. IVIG is approved by the US Food and Drug Administration and is the standard of care for Kawasaki disease, but most IVIG use in pediatric rheumatology is "off-label. " The literature supports the use of IVIG for juvenile dermatomyositis, although it is unclear whether its use should be limited to those children with more severe or refractory disease...
January 1, 2017: Pediatric Annals
https://www.readbyqxmd.com/read/28079913/intravenous-immunoglobulin-in-the-treatment-of-hematologic-disorders-in-pediatrics
#2
Gabriela Villanueva, Jill L O de Jong, Jennifer L McNeer
Intravenous immunoglobulin (IVIG) is pooled immunoglobulin G derived from human blood donors. It was introduced in the early 1980s to treat immunodeficiency disorders. Since then, its use has expanded to other fields such as neurology, rheumatology, and hematology. IVIG has been used to provide passive immunity in qualitative and quantitative immunoglobulin disorders, to neutralize antibodies in immune-mediated diseases, and as an immune modulatory agent. The difficulty of producing IVIG in high quantities, in addition to a growing list of "off-label" indications, has resulted in a worldwide shortage and increase in cost...
January 1, 2017: Pediatric Annals
https://www.readbyqxmd.com/read/28077146/childhood-arthritis-and-rheumatology-research-alliance-consensus-clinical-treatment-plans-for-juvenile-dermatomyositis-with-skin-predominant-disease
#3
Susan Kim, Philip Kahn, Angela B Robinson, Bianca Lang, Andrew Shulman, Edward J Oberle, Kenneth Schikler, Megan Lea Curran, Lilliana Barillas-Arias, Charles H Spencer, Lisa G Rider, Adam M Huber
BACKGROUND: Juvenile dermatomyositis (JDM) is the most common form of the idiopathic inflammatory myopathies in children. A subset of children have the rash of JDM without significant weakness, and the optimal treatments for these children are unknown. The goal of this study was to describe the development of consensus clinical treatment plans (CTPs) for children with JDM who have active skin rashes, without significant muscle involvement, referred to as skin predominant JDM in this manuscript...
January 11, 2017: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/28070763/uveitis-in-childhood-onset-systemic-lupus-erythematosus-patients-a-multicenter-survey
#4
Paola Pinheiro Kahwage, Mariana Paes Leme Ferriani, João M Furtado, Luciana Martins de Carvalho, Gecilmara Salviato Pileggi, Francisco Hugo Rodrigues Gomes, Maria Teresa Terreri, Claudia Saad Magalhães, Rosa Maria Rodrigues Pereira, Silvana Brasilia Sacchetti, Roberto Marini, Eloisa Bonfá, Clovis Artur Silva, Virgínia Paes Leme Ferriani
The aim of this study is to assess uveitis prevalence in a large cohort of childhood-onset systemic lupus erythematosus (cSLE) patients. A retrospective multicenter cohort study including 852 cSLE patients was performed in ten pediatric rheumatology centers (Brazilian cSLE group). An investigator meeting was held and all participants received database training. Uveitis was diagnosed through clinical assessment by the uveitis expert ophthalmologist of each center. Patients with and without uveitis were assessed for lupus clinical/laboratory features and treatments...
January 9, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28058540/juvenile-dermatomyositis-a-tertiary-center-experience
#5
Kenan Barut, Pinar Ozge Avar Aydin, Amra Adrovic, Sezgin Sahin, Ozgur Kasapcopur
Juvenile dermatomyositis (JDM) is a rare chronic inflammatory disease of unknown etiology and primarily involves muscle and skin. It is the most common idiopathic inflammatory myopathy of childhood. This study aimed to evaluate demographic and clinical features, laboratory data, treatment modalities, and outcome of patients with JDM at a referral pediatric rheumatology center in Turkey. We retrospectively reviewed medical records of patients diagnosed with JDM between the years 2003-2016 at the Pediatric Rheumatology Department Cerrahpasa Medical Faculty...
January 5, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28040333/signs-and-symptoms-of-rheumatic-diseases-as-first-manifestation-of-pediatric-cancer-diagnosis-and-prognosis-implications
#6
Mariana Bertoldi Fonseca, Francisco Hugo Rodrigues Gomes, Elvis Terci Valera, Gecilmara Salviato Pileggi, Paula Braga Gonfiantini, Marcela Braga Gonfiantini, Virgínia Paes Leme Ferriani, Luciana Martins de Carvalho
OBJECTIVE: To assess the prevalence and describe the clinical, laboratory and radiological findings, treatment and outcome of children with cancer initially referred to a tertiary outpatient pediatric rheumatology clinic. METHODS: Retrospective analysis of medical records from patients identified in a list of 250 new patients attending the tertiary Pediatric Rheumatology Clinic, Ribeirão Preto Medical School hospital, University of São Paulo, from July 2013 to July 2015, whose final diagnosis was cancer...
December 16, 2016: Revista Brasileira de Reumatologia
https://www.readbyqxmd.com/read/28025240/safety-of-quadrivalent-meningococcal-conjugate-vaccine-in-11-to-21-year-olds
#7
Hung-Fu Tseng, Lina S Sy, Bradley K Ackerson, Rulin C Hechter, Sara Y Tartof, Mendel Haag, Jeffrey M Slezak, Yi Luo, Christine A Fischetti, Harp S Takhar, Yan Miao, Marianne Cunnington, Zendi Solano, Steven J Jacobsen
BACKGROUND: Meningococcal conjugate vaccination is recommended in the United States. This study evaluates the safety of quadrivalent meningococcal conjugate vaccine in a cohort aged 11 to 21 years. METHODS: This cohort study with self-controlled case-series analysis was conducted at Kaiser Permanente Southern California. Individuals receiving MenACWY-CRM, a quadrivalent meningococcal conjugate vaccine, during September 30, 2011 to June 30, 2013, were included. Twenty-six prespecified events of interest (EOIs), including neurologic, rheumatologic, hematologic, endocrine, renal, pediatric, and pediatric infectious disease EOIs, were identified through electronic health records 1 year after vaccination...
January 2017: Pediatrics
https://www.readbyqxmd.com/read/28013435/evaluation-of-quality-indicators-and-disease-damage-in-childhood-onset-systemic-lupus-erythematosus-patients
#8
Julia G Harris, Kristyn I Maletta, Evelyn M Kuhn, Judyann C Olson
The aim of this study was to describe compliance with select quality indicators and assess organ-specific dysfunction in a childhood-onset systemic lupus erythematosus population by using a validated damage index and to evaluate associations between compliance with quality indicators and disease damage. A retrospective chart review was performed on patients diagnosed with systemic lupus erythematosus prior to age 18 followed at a single center in the USA from 1999 to 2012 (n = 75). Data regarding quality indicators and outcome variables, including the Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index, were collected...
December 24, 2016: Clinical Rheumatology
https://www.readbyqxmd.com/read/27994096/paediatric-rheumatology-clinic-population-in-southeast-asia-are-we-different
#9
Thaschawee Arkachaisri, Swee-Ping Tang, Tassalapa Daengsuwan, Gun Phongsamart, Soamarat Vilaiyuk, Sirirat Charuvanij, Sook Fun Hoh, Justin Hung Tiong Tan, Lena Das, Elizabeth Ang, Wendy Lim, Yiong Huak Chan, Christine B Bernal
OBJECTIVES: To examine the descriptive epidemiology of the patient population referred to paediatric rheumatology centres (PRCs) in Southeast Asia (SEA) and to compare the frequency of conditions encountered with other PRC populations. METHODS: A web-based Registry for Childhood Onset Paediatric Rheumatic Diseases was established in 2009 and seven PRCs in four SEA countries, where paediatric rheumatologists are available, participated in a prospective 24 month data collection (43 months for Singapore)...
December 19, 2016: Rheumatology
https://www.readbyqxmd.com/read/27993144/disease-status-reasons-for-discontinuation-and-adverse-events-in-1038-italian-children-with-juvenile-idiopathic-arthritis-treated-with-etanercept
#10
Sara Verazza, Sergio Davì, Alessandro Consolaro, Francesca Bovis, Antonella Insalaco, Silvia Magni-Manzoni, Rebecca Nicolai, Denise Pires Marafon, Fabrizio De Benedetti, Valeria Gerloni, Irene Pontikaki, Francesca Rovelli, Rolando Cimaz, Achille Marino, Francesco Zulian, Giorgia Martini, Serena Pastore, Chiara Sandrin, Fabrizia Corona, Marta Torcoletti, Giovanni Conti, Claudia Fede, Patrizia Barone, Marco Cattalini, Elisabetta Cortis, Luciana Breda, Alma Nunzia Olivieri, Adele Civino, Rosanna Podda, Donato Rigante, Francesco La Torre, Gianfranco D'Angelo, Mauro Jorini, Romina Gallizzi, Maria Cristina Maggio, Rita Consolini, Alessandro De Fanti, Valentina Muratore, Maria Giannina Alpigiani, Nicolino Ruperto, Alberto Martini, Angelo Ravelli
BACKGROUND: Data from routine clinical practice are needed to further define the efficacy and safety of biologic medications in children with juvenile idiopathic arthritis (JIA). The aim of this analysis was to investigate the disease status, reasons for discontinuation and adverse events in Italian JIA patients treated with etanercept (ETN). METHODS: In 2013, all centers of the Italian Pediatric Rheumatology Study Group were asked to make a census of patients given ETN after January 2000...
December 20, 2016: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/27988434/anti-ro-ssa-and-anti-la-ssb-antibodies-association-with-mild-lupus-manifestations-in-645-childhood-onset-systemic-lupus-erythematosus
#11
REVIEW
Glaucia V Novak, Mariana Marques, Verena Balbi, Natali W S Gormezano, Kátia Kozu, Ana P Sakamoto, Rosa M R Pereira, Maria T Terreri, Claudia S Magalhães, Andressa Guariento, Adriana M E Sallum, Roberto Marini, Virginia Paes Leme Ferriani, Cássia Maria Barbosa, Tânia Caroline Monteiro de Castro, Valéria C Ramos, Eloisa Bonfá, Clovis A Silva
BACKGROUND: To our knowledge there are no studies assessing anti-Ro/SSA and anti-La/SSB autoantibodies in a large population of childhood-systemic lupus erythematosus(cSLE) patients. METHODS: This was a retrospective multicenter cohort study performed in 10 Pediatric Rheumatology services, São Paulo state, Brazil. Anti-Ro/SSA and anti-La/SSB antibodies were measured by enzyme linked immunosorbent assay(ELISA) in 645 cSLE patients. RESULTS: Anti-Ro/SSA and anti-La/SSB antibodies were evidenced in 209/645(32%) and 102/645(16%) of cSLE patients, respectively...
December 14, 2016: Autoimmunity Reviews
https://www.readbyqxmd.com/read/27964737/evidence-based-decision-support-for-pediatric-rheumatology-reduces-diagnostic-errors
#12
Michael M Segal, Balu Athreya, Mary Beth F Son, Irit Tirosh, Jonathan S Hausmann, Elizabeth Y N Ang, David Zurakowski, Lynn K Feldman, Robert P Sundel
BACKGROUND: The number of trained specialists world-wide is insufficient to serve all children with pediatric rheumatologic disorders, even in the countries with robust medical resources. We evaluated the potential of diagnostic decision support software (DDSS) to alleviate this shortage by assessing the ability of such software to improve the diagnostic accuracy of non-specialists. METHODS: Using vignettes of actual clinical cases, clinician testers generated a differential diagnosis before and after using diagnostic decision support software...
December 13, 2016: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/27943079/assessment-of-type-i-interferon-signaling-in-pediatric-inflammatory-disease
#13
Gillian I Rice, Isabelle Melki, Marie-Louise Frémond, Tracy A Briggs, Mathieu P Rodero, Naoki Kitabayashi, Anthony Oojageer, Brigitte Bader-Meunier, Alexandre Belot, Christine Bodemer, Pierre Quartier, Yanick J Crow
PURPOSE: Increased type I interferon is considered relevant to the pathology of a number of monogenic and complex disorders spanning pediatric rheumatology, neurology, and dermatology. However, no test exists in routine clinical practice to identify enhanced interferon signaling, thus limiting the ability to diagnose and monitor treatment of these diseases. Here, we set out to investigate the use of an assay measuring the expression of a panel of interferon-stimulated genes (ISGs) in children affected by a range of inflammatory diseases...
December 9, 2016: Journal of Clinical Immunology
https://www.readbyqxmd.com/read/27927641/genetic-architecture-distinguishes-systemic-juvenile-idiopathic-arthritis-from-other-forms-of-juvenile-idiopathic-arthritis-clinical-and-therapeutic-implications
#14
Michael J Ombrello, Victoria L Arthur, Elaine F Remmers, Anne Hinks, Ioanna Tachmazidou, Alexei A Grom, Dirk Foell, Alberto Martini, Marco Gattorno, Seza Özen, Sampath Prahalad, Andrew S Zeft, John F Bohnsack, Norman T Ilowite, Elizabeth D Mellins, Ricardo Russo, Claudio Len, Maria Odete E Hilario, Sheila Oliveira, Rae S M Yeung, Alan M Rosenberg, Lucy R Wedderburn, Jordi Anton, Johannes-Peter Haas, Angela Rosen-Wolff, Kirsten Minden, Klaus Tenbrock, Erkan Demirkaya, Joanna Cobb, Elizabeth Baskin, Sara Signa, Emily Shuldiner, Richard H Duerr, Jean-Paul Achkar, M Ilyas Kamboh, Kenneth M Kaufman, Leah C Kottyan, Dalila Pinto, Stephen W Scherer, Marta E Alarcón-Riquelme, Elisa Docampo, Xavier Estivill, Ahmet Gül, Carl D Langefeld, Susan Thompson, Eleftheria Zeggini, Daniel L Kastner, Patricia Woo, Wendy Thomson
OBJECTIVES: Juvenile idiopathic arthritis (JIA) is a heterogeneous group of conditions unified by the presence of chronic childhood arthritis without an identifiable cause. Systemic JIA (sJIA) is a rare form of JIA characterised by systemic inflammation. sJIA is distinguished from other forms of JIA by unique clinical features and treatment responses that are similar to autoinflammatory diseases. However, approximately half of children with sJIA develop destructive, long-standing arthritis that appears similar to other forms of JIA...
December 7, 2016: Annals of the Rheumatic Diseases
https://www.readbyqxmd.com/read/27916349/pediatric-pyoderma-gangrenosum-is-it-just-big-wounds-on-little-adults
#15
Tahereh Soleimani, Sarah E Sasor, Leigh Spera, Barry E Eppley, Juan Socas, Michael W Chu, Sunil S Tholpady
BACKGROUND: Pyoderma gangrenosum (PG) is an uncommon, ulcerative, cutaneous condition, often caused by surgical trauma that can masquerade as a pyogenic disease process requiring debridement and antibiotics. Treatment is, however, medical, with delay leading to significant morbidity. In addition, medical workup for coincident disorders has been suggested. The purpose of this study was to test the hypothesis that pediatric PG has differing disease associations and therefore requires a differing medical workup...
November 2016: Journal of Surgical Research
https://www.readbyqxmd.com/read/27914595/intra-articular-injection-in-patients-with-juvenile-idiopathic-arthritis-factors-associated-with-a-good-response
#16
Ana Luiza Garcia Cunha, Vanessa Bugni Miotto E Silva, Fabiane Mitie Osaku, Luísa Brasil Niemxeski, Rita Nely Vilar Furtado, Jamil Natour, Maria Teresa de Sande, Lemos Ramos Ascensão Terreri
INTRODUCTION: Intra-articular injection of corticosteroids (IIC) for treatment of patients with juvenile idiopathic arthritis (JIA) is increasingly used in Pediatric Rheumatology. OBJECTIVES: To describe the clinical course of patients undergoing IIC in our Pediatric Rheumatology Unit. METHODS: Retrospective study of patients with JIA undergoing IIC from January 2008 to December 2012, with a minimum follow-up of six months after the injection...
November 2016: Revista Brasileira de Reumatologia
https://www.readbyqxmd.com/read/27909087/stand-up-and-be-counted-measuring-and-mapping-the-rheumatology-workforce-in-canada
#17
Claire E H Barber, Lauren Jewett, Elizabeth M Badley, Diane Lacaille, Alfred Cividino, Vandana Ahluwalia, Henry Averns, Cory Baillie, Janet Ellsworth, Janet Pope, Deborah Levy, Christine Charnock, Claire McGowan, J Carter Thorne, Cheryl Barnabe, Michel Zummer, Katie Lundon, Robert S McDougall, John G Thomson, Elaine A Yacyshyn, Dianne Mosher, Julie Brophy, Thanu Nadarajah Ruban, Deborah A Marshall
OBJECTIVE: To characterize the practicing rheumatologist workforce, the Canadian Rheumatology Association (CRA) launched the Stand Up and Be Counted workforce survey in 2015. METHODS: The survey was distributed electronically to 695 individuals, of whom 519 were expected to be practicing rheumatologists. Demographic and practice information were elicited. We estimated the number of full-time equivalent rheumatologists per 75,000 population from the median proportion of time devoted to clinical practice multiplied by provincial rheumatologist numbers from the Canadian Medical Association...
December 1, 2016: Journal of Rheumatology
https://www.readbyqxmd.com/read/27902749/risk-factors-associated-with-renal-involvement-in-childhood-henoch-sch%C3%A3-nlein-purpura-a-meta-analysis
#18
Han Chan, Yan-Ling Tang, Xiao-Hang Lv, Gao-Fu Zhang, Mo Wang, Hai-Ping Yang, Qiu Li
BACKGROUND AND OBJECTIVE: Henoch-Schönlein purpura (HSP) is an important cause of chronic kidney disease in children. This meta-analysis identified risk factors associated with renal involvement in childhood HSP. METHODS: PubMed, Embase, and Web of Science were searched. The quality of all eligible studies was assessed using the Newcastle-Ottawa scale criteria. An analysis of possible risk factors was conducted to report the odds ratio (OR) and weighted mean difference (WMD)...
2016: PloS One
https://www.readbyqxmd.com/read/27902668/pneumatosis-intestinalis-due-to-child-abuse
#19
Stephanie Anne Deutsch, Cindy W Christian
Pneumatosis intestinalis (PI) and the presence of portal venous gas (PVG) are commonly considered pathognomonic for necrotizing enterocolitis in the neonatal period; however, these 2 radiographic findings have been documented in all age groups in a variety of clinical settings and medical conditions including respiratory, cardiac, rheumatologic, gastrointestinal disorders, and traumatic injury. In children, intramural dissection of intestinal gas in the absence of clinical symptoms suggestive of necrotizing enterocolitis should raise concern for a traumatic etiology, including injuries sustained from child physical abuse...
November 29, 2016: Pediatric Emergency Care
https://www.readbyqxmd.com/read/27890486/towards-a-multidisciplinary-approach-to-understand-and-manage-obesity-and-related-diseases
#20
REVIEW
Stephan C Bischoff, Yves Boirie, Tommy Cederholm, Michael Chourdakis, Cristina Cuerda, Nathalie M Delzenne, Nicolaas E Deutz, Denis Fouque, Laurence Genton, Carmen Gil, Berthold Koletzko, Miguel Leon-Sanz, Raanan Shamir, Joelle Singer, Pierre Singer, Nanette Stroebele-Benschop, Anders Thorell, Arved Weimann, Rocco Barazzoni
Overnutrition and sedentary lifestyle result in overweight or obesity defined as abnormal or excessive fat accumulation that may impair health. According to the WHO, the worldwide prevalence of obesity nearly doubled between 1980 and 2008. In 2008, over 50% of both men and women in the WHO European Region were overweight, and approximately 23% of women and 20% of men were obese. Comprehensive diagnostic and therapeutic approaches should include nutritional treatment to favor the best metabolic and nutritional outcome, as well as to induce potential disease-specific benefits from selected nutritional regimens...
November 16, 2016: Clinical Nutrition: Official Journal of the European Society of Parenteral and Enteral Nutrition
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