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Rheumatology pediatrics

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https://www.readbyqxmd.com/read/28807357/development-and-initial-validation-of-the-macrophage-activation-syndrome-primary-hemophagocytic-lymphohistiocytosis-score-a-diagnostic-tool-that-differentiates-primary-hemophagocytic-lymphohistiocytosis-from-macrophage-activation-syndrome
#1
Francesca Minoia, Francesca Bovis, Sergio Davì, Antonella Insalaco, Kai Lehmberg, Susan Shenoi, Sheila Weitzman, Graciela Espada, Yi-Jin Gao, Jordi Anton, Toshiyuki Kitoh, Ozgur Kasapcopur, Helga Sanner, Rosa Merino, Itziar Astigarraga, Maria Alessio, Michael Jeng, Vyacheslav Chasnyk, Kim E Nichols, Zeng Huasong, Caifeng Li, Concetta Micalizzi, Nicolino Ruperto, Alberto Martini, Randy Q Cron, Angelo Ravelli, AnnaCarin Horne
OBJECTIVE: To develop and validate a diagnostic score that assists in discriminating primary hemophagocytic lymphohistiocytosis (pHLH) from macrophage activation syndrome (MAS) related to systemic juvenile idiopathic arthritis. STUDY DESIGN: The clinical, laboratory, and histopathologic features of 362 patients with MAS and 258 patients with pHLH were collected in a multinational collaborative study. Eighty percent of the population was assessed to develop the score and the remaining 20% constituted the validation sample...
August 11, 2017: Journal of Pediatrics
https://www.readbyqxmd.com/read/28795234/demographic-and-clinical-characteristics-of-patients-with-serum-sickness-like-reaction
#2
Alaaddin Yorulmaz, Fatih Akın, Ahmet Sert, Mehmet Akif Ağır, Rıza Yılmaz, Şükrü Arslan
In this study, we aimed to review the demographic, clinical, and laboratory characteristics of patients who were followed up and treated with the diagnosis of serum sickness-like reactions (SSLR) in our pediatric rheumatology clinic retrospectively and emphasize the importance of early diagnosis and treatment. The files of 29 patients who were hospitalized in the pediatric rheumatology clinic between September 2016 and March 2017 with the diagnosis of type 3 hypersensitivity reaction were reviewed retrospectively...
August 9, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28774449/imaging-of-juvenile-idiopathic-arthritis
#3
REVIEW
Dimitriou Christos, Boitsios Grammatina, Phu-Quoc Lê, Laurence Goffin, Badot Valérie, Paolo Simoni
Juvenile idiopathic arthritis is an umbrella term covering several distinct categories that share common features. The European League Against Rheumatism and the Pediatric Rheumatology European Society have published a consensus article with recommendations to guide radiologists and clinicians in choosing the best imaging technique for each particular clinical setting. A reproducible, accurate, validated, and long-established scoring system to use in everyday practice for monitoring and predicting long-term response to therapy is still to be developed on MR imaging for each joint...
September 2017: Radiologic Clinics of North America
https://www.readbyqxmd.com/read/28753165/pediatric-rheumatologic-diseases-a-review-for-primary-care-nps
#4
Malinda Teague
Rheumatologic diseases are uncommon in children but have the potential for significant morbidity, mortality, and disability. This article reviews the most common rheumatologic diseases in children, their diagnostic evaluations, and the NP's role in the management and coordination of these conditions.
July 27, 2017: Nurse Practitioner
https://www.readbyqxmd.com/read/28743360/signs-and-symptoms-of-rheumatic-diseases-as-first-manifestation-of-pediatric-cancer-diagnosis-and-prognosis-implications
#5
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...
July 2017: Revista Brasileira de Reumatologia
https://www.readbyqxmd.com/read/28741897/recent-advances-in-pediatric-rheumatology-january-to-march-2017
#6
Sandesh Guleria, Rakesh Kumar Pilania
No abstract text is available yet for this article.
July 25, 2017: International Journal of Rheumatic Diseases
https://www.readbyqxmd.com/read/28725955/biologics-in-juvenile-idiopathic-arthritis-a-narrative-review
#7
REVIEW
Federica Vanoni, Francesca Minoia, Clara Malattia
In the past years, pediatric rheumatology has seen a revolution in the treatments for rheumatic diseases, particularly juvenile idiopathic arthritis. Even if nonsteroidal anti-inflammatory drugs (NSAID), intra-articular corticosteroids (IAC) injections, and methotrexate remain the mainstay of the treatment for JIA patients, in aggressive disease, these treatments may be not sufficient to reach disease remission and to prevent long-term disability. Comprehension of immunological mechanisms involved in the pathogenesis of the diseases allowed to conceive new drugs targeting specific steps of the immune response...
July 20, 2017: European Journal of Pediatrics
https://www.readbyqxmd.com/read/28719732/genome-wide-association-meta-analysis-reveals-novel-juvenile-idiopathic-arthritis-susceptibility-loci
#8
Laura A McIntosh, Miranda C Marion, Marc Sudman, Mary E Comeau, Mara L Becker, John F Bohnsack, Tasha E Fingerlin, Thomas A Griffin, J Peter Haas, Daniel J Lovell, Lisa A Maier, Peter A Nigrovic, Sampath Prahalad, Marilynn Punaro, Carlos D Rosé, Carol A Wallace, Carol A Wise, Halima Moncrieffe, Timothy D Howard, Carl D Langefeld, Susan D Thompson
OBJECTIVE: Juvenile idiopathic arthritis (JIA) is the most common childhood rheumatic disease and has a strong genomic component. To date, JIA genetic association studies have had limited sample sizes, used heterogeneous patient populations, or included only candidate regions. This study focuses on identifying new associations among oligoarticular and polyarticular rheumatoid factor negative (RF(-) ) JIA, which are clinically similar and the most prevalent JIA disease subtypes. METHODS: Three cohorts totaling 2,751 oligoarticular and polyarticular RF(-) JIA cases were genotyped on the Affymetrix Genome-Wide SNP Array 6...
July 18, 2017: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/28717937/clinical-presentation-and-outcomes-of-childhood-onset-membranous-lupus-nephritis
#9
Maria Pereira, Eyal Muscal, Karen Eldin, M John Hicks, Anna Carmela P Sagcal-Gironella, Marietta DeGuzman, Scott E Wenderfer
BACKGROUND: Best practices for managing childhood-onset membranous lupus nephritis (MLN) are not yet established. Most studies involve primarily or exclusively adult cohorts or pediatric cohorts with combinations of pure or mixed membranous and proliferative nephritis. METHODS: We performed a single-center cohort study of consecutively diagnosed children with pure MLN from 1990 and 2016. Patients received care in Houston, Texas, one of the most diverse metropolitan areas in North America...
July 17, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28695435/evaluation-of-development-of-subclinical-atherosclerosis-in-children-with-uveitis
#10
Seçil Conkar, Suzan Güven Yılmaz, İlker Özgür Koska, Afig Berdeli, Sevgi Mir
Uveitis is a chronic inflammatory disease. Chronic inflammation has been shown to have a role in pathogenesis of atherosclerosis. Atherosclerosis is the most important risk factor of cardiovascular diseases and is shown to start as early as childhood. In this study, we investigated the presence of subclinical atherosclerosis in children with uveitis. Seventy five patients who were diagnosed as having uveitis in ophthalmology and pediatric rheumatology clinics were included in the study. Patients with hypertension, obesity, dyslipidemia, diabetes, and with history of early cardiovascular disease were excluded...
July 10, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28693580/telemedicine-and-other-care-models-in-pediatric-rheumatology-an-exploratory-study-of-parents-perceptions-of-barriers-to-care-and-care-preferences
#11
Danielle R Bullock, Richard K Vehe, Lei Zhang, Colleen K Correll
BACKGROUND: The United States pediatric rheumatology workforce is committed to a mission of providing children access to pediatric rheumatology care. With a limited number and distribution of pediatric rheumatologists, telemedicine has been proposed as one way to meet this mission, yet the adoption of this modality has been slower than expected. The purpose of this study was to explore the parent perspective on barriers to accessing pediatric rheumatology care and to explore the acceptability of telemedicine and other alternative care models...
July 11, 2017: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/28626961/comparing-polyarteritis-nodosa-in-children-and-adults-a-single-center-study
#12
Abdulsamet Erden, Ezgi D Batu, Hafize E Sönmez, Alper Sarı, Berkan Armagan, Zehra S Arıcı, Emre Bilgin, Umut Kalyoncu, Ömer Karadağ, Yelda Bilginer, Ali Ihsan Ertenli, Seza Özen
OBJECTIVE: Polyarteritis nodosa (PAN) is a necrotizing vasculitis of medium/small arteries. We aimed to examine the characteristics of adult- and childhood-onset PAN. METHODS: Fifteen pediatric (˂ 18 years) and 22 adult PAN patients who fulfilled the Ankara 2008 and American College of Rheumatology 1990 criteria, respectively, were included in the study. RESULTS: Five children had cutaneous and all the rest of the patients had systemic PAN...
June 19, 2017: International Journal of Rheumatic Diseases
https://www.readbyqxmd.com/read/28621800/a-consensus-review-on-malignancy-associated-hemophagocytic-lymphohistiocytosis-in-adults
#13
REVIEW
Naval Daver, Kenneth McClain, Carl E Allen, Sameer A Parikh, Zaher Otrock, Cristhiam Rojas-Hernandez, Boris Blechacz, Sa Wang, Milen Minkov, Michael B Jordan, Paul La Rosée, Hagop M Kantarjian
Hemophagocytic lymphohistiocytosis (HLH) is a syndrome of severe immune activation and dysregulation resulting in extreme and often life-threatening inflammation. HLH has been well recognized in pediatric populations, and most current diagnostic and therapeutic guidelines are based on pediatric HLH. Recently there has been recognition of HLH in adults, especially secondary to immune deregulation by an underlying rheumatologic, infectious, or malignant condition. This review is focused on malignancy-associated HLH (M-HLH), in which possible mechanisms of pathogenesis include severe inflammation, persistent antigen stimulation by the tumor cells, and loss of immune homeostasis because of chemotherapy, hematopoietic stem cell transplantation, or infection...
June 16, 2017: Cancer
https://www.readbyqxmd.com/read/28612093/clinical-trial-design-in-juvenile-idiopathic-arthritis
#14
Stephen J Balevic, Mara L Becker, Michael Cohen-Wolkowiez, Laura E Schanberg
Randomized clinical trials provide the gold standard evidence base to guide clinical practice. Despite major advances in trial design, pediatric clinical trials are still difficult to perform and pose unique challenges, including the need to consider the impact of developmental changes in trial design. Advances within pediatric rheumatology combined with the need to comply with legislative requirements have driven new approaches to performing pediatric clinical trials such as utilization of large research networks, incorporation of patient and family stakeholders in the planning and implementation of clinical trials, and the development of novel trial designs...
June 13, 2017: Paediatric Drugs
https://www.readbyqxmd.com/read/28610606/biologic-therapies-for-refractory-juvenile-dermatomyositis-five-years-of-experience-of-the-childhood-arthritis-and-rheumatology-research-alliance-in-north-america
#15
C H Spencer, K Rouster-Stevens, H Gewanter, G Syverson, R Modica, K Schmidt, H Emery, C Wallace, S Grevich, K Nanda, Y D Zhao, S Shenoi, S Tarvin, S Hong, C Lindsley, J E Weiss, M Passo, K Ede, A Brown, K Ardalan, W Bernal, M L Stoll, B Lang, R Carrasco, C Agaiar, L Feller, H Bukulmez, R Vehe, H Kim, H Schmeling, D Gerstbacher, M Hoeltzel, B Eberhard, R Sundel, S Kim, A M Huber, A Patwardhan
BACKGROUND: The prognosis of children with juvenile dermatomyositis (JDM) has improved remarkably since the 1960's with the use of corticosteroid and immunosuppressive therapy. Yet there remain a minority of children who have refractory disease. Since 2003 the sporadic use of biologics (genetically-engineered proteins that usually are derived from human genes) for inflammatory myositis has been reported. In 2011-2016 we investigated our collective experience of biologics in JDM through the Childhood Arthritis and Rheumatology Research Alliance (CARRA)...
June 13, 2017: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/28599656/transitional-care-for-rheumatic-conditions-in-europe-current-clinical-practice-and-available-resources
#16
Daniel Clemente, Leticia Leon, Helen Foster, Loreto Carmona, Kirsten Minden
OBJECTIVE: To assess European pediatric rheumatology providers' current clinical practices and resources used in the transition from child-centered to adult-oriented care. METHODS: European pediatric rheumatologists were invited to complete a 17-item anonymized e-survey assessing current transition practices, transition policy awareness, and needs in advance of the publication of EULAR/PReS recommendations on transition. RESULTS: The response rate was 121/276 (44%), including responses from 115 centers in 22 European Union countries...
June 9, 2017: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/28583183/design-and-implementation-of-a-decision-aid-for-juvenile-idiopathic-arthritis-medication-choices
#17
William B Brinkman, Ellen A Lipstein, Janalee Taylor, Pamela J Schoettker, Katherine Naylor, Karla Jones, Sheetal S Vora, Catherine C Mims, Elizabeth Roth-Wojcicki, Beth Gottlieb, Nancy Griffin, Carole Lannon, Esi Morgan
BACKGROUND: Randomized trials have demonstrated the efficacy of patient decision aids to facilitate shared decision making in clinical situations with multiple medically reasonable options for treatment. However, little is known about how best to implement these tools into routine clinical practice. In addition, reliable implementation of decision aids has been elusive and spread within pediatrics has been slow. We sought to develop and reliably implement a decision aid for treatment of children with juvenile idiopathic arthritis...
June 5, 2017: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/28558790/challenges-in-transitioning-adolescents-and-young-adults-with-rheumatologic-diseases-to-adult-care-in-a-developing-country-the-brazilian-experience
#18
Catherine Gusman Anelli, Ana Luiza Mendes Amorim, Fabiane Mitie Osaku, Maria Teresa Terreri, Claudio Arnaldo Len, Andreas Reiff
BACKGROUND: Transition guidelines and recommendations for developing countries are limited and best transition practices in young patients with chronic medical conditions have been poorly examined. This study evaluates transition practices from pediatric to adult rheumatology care in Brazil. METHODS: Practicing pediatric rheumatologists registered in the Brazilian Society of Rheumatology were e-surveyed with SurveyMonkey® using the Chira et al. questionnaire that had been used previously to evaluate transition practices of pediatric rheumatologists from the Childhood Arthritis and Rheumatology Research Alliance (CARRA) in the USA and Canada...
May 30, 2017: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/28544820/delays-to-care-in-pediatric-lupus-patients-from-the-childhood-arthritis-and-rheumatology-research-alliance-legacy-registry
#19
Tamar B Rubinstein, Wenzhu B Mowrey, Norman T Ilowite, Dawn M Wahezi
OBJECTIVES: Prompt treatment for lupus is important to prevent morbidity. A potential barrier to early treatment of pediatric lupus is delayed presentation to a pediatric rheumatologist. To better understand factors contributing to delayed presentation among pediatric lupus patients, we examined differences in demographic and clinical characteristics of lupus patients within the Childhood Arthritis and Rheumatology Research Alliance (CARRA) Legacy Registry with regards to time between symptom onset and presentation to a pediatric rheumatologist...
May 23, 2017: Arthritis Care & Research
https://www.readbyqxmd.com/read/28538014/precision-medicine-in-pediatric-rheumatology
#20
Joo Guan Yeo, Chin Teck Ng, Salvatore Albani
PURPOSE OF REVIEW: Precision medicine is the tailoring of medical care to subcategories of disease. In pediatric rheumatology, these subcategories must first be defined by their specific molecular immunological profiles, and then the effects of growth and puberty, developmental immunological changes, and differences in treatment options and adherence considered when designing therapeutic strategies. In the present review, we summarize the unmet needs in pediatric rheumatology before such precision medical care can be effectively delivered to affected patients...
September 2017: Current Opinion in Rheumatology
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