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https://www.readbyqxmd.com/read/28516235/clinical-imaging-and-genotypical-features-of-three-deceased-and-five-surviving-cases-with-ada2-deficiency
#1
Sezgin Sahin, Amra Adrovic, Kenan Barut, Serdal Ugurlu, Eda Tahir Turanli, Huri Ozdogan, Ozgur Kasapcopur
Deficiency of adenosine deaminase type 2 (DADA2) is a rare form of autoinflammatory disorder with limited reported cases. In this paper, we have presented the clinico-immunological, radiological and genetic characteristics of five surviving and three deceased childhood-onset DADA2 patients. We aimed to compare surviving and deceased patients in terms of clinical features and treatment modalities. Moreover, we have evaluated the causes of death in our DADA2 subjects together with the previously reported cases...
May 17, 2017: Rheumatology International
https://www.readbyqxmd.com/read/28514463/pediatric-psoriasis-comorbidity-screening-guidelines
#2
Emily Osier, Audrey S Wang, Megha M Tollefson, Kelly M Cordoro, Stephen R Daniels, Andrew Eichenfield, Joel M Gelfand, Alice B Gottlieb, Alexa B Kimball, Mark Lebwohl, Nehal N Mehta, Amy S Paller, Jeffrey B Schwimmer, Dennis M Styne, Abby S Van Voorhees, Wynnis L Tom, Lawrence F Eichenfield
Importance: Psoriasis is a complex inflammatory skin condition associated with serious medical comorbidities in adults, including obesity, hypertension, dyslipidemia, type 2 diabetes mellitus, psoriatic arthritis, nonalcoholic fatty liver disease, depression, anxiety, and decreased quality of life. Because psoriasis begins in childhood in almost one-third of patients, early identification of risk may be critical to minimizing effects on future health. Objective: To develop the first set of guidelines for comorbidity screening for patients with pediatric psoriasis based on current evidence...
May 17, 2017: JAMA Dermatology
https://www.readbyqxmd.com/read/28511684/access-to-pediatric-rheumatology-care-for-juvenile-idiopathic-arthritis-in-the-united-arab-emirates
#3
Khulood Khawaja, Mustafa Al-Maini
BACKGROUND: This study looks at access to care for Juvenile Idiopathic Arthritis through pediatric rheumatology in the UAE, as an example of multi-ethnic society. METHODS: Patients with a diagnosis of Juvenile idiopathic arthritis were identified through the hospital electronic medical records system from January 1st 2011 to December 31st 2014. All residents of the United Arab Emirates hold an Emirates identity card. We divided our patients into two groups: Emirati-Emirates, who are native Emirati children and hold the Emirati nationality, as stated on their Emirates identity card, and who therefore have full, comprehensive access to free medical care; and non-Emirati-Emirates, who represent other nationalities, as stated on their Emirates identity card...
May 16, 2017: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/28503078/variations-in-the-clinical-practice-of-physicians-managing-takayasu-arteritis-a-nationwide-survey
#4
Lillian Barra, Patrick Liang, Susanne M Benseler, David A Cabral, Aurore Fifi-Mah, Yueyang Li, Nataliya Milman, Marinka Twilt, Elaine Yacyshyn, Christian Pagnoux
OBJECTIVE: Takayasu arteritis (TAK) is a large vessel vasculitis that predominately affects young women and can cause severe ischemic complications. Given the rarity of TAK, the management of this condition is challenging. We aim to describe current rheumatologist practices for the management of TAK and identify discrepancies and gaps in knowledge. METHODS: An online survey (developed by the Canadian Vasculitis Network and approved by the Canadian Rheumatology Association) containing 48 questions with regard to the diagnosis, monitoring and treatment of TAK was distributed to 495 Canadian adult and pediatric rheu-matologists by email...
2017: Open Access Rheumatology: Research and Reviews
https://www.readbyqxmd.com/read/28502061/validation-of-administrative-case-ascertainment-algorithms-for-chronic-childhood-arthritis-in-manitoba-canada
#5
Natalie Jane Shiff, Kiem Oen, Rasheda Rabbani, Lisa M Lix
We validated case ascertainment algorithms for juvenile idiopathic arthritis (JIA) in the provincial health administrative databases of Manitoba, Canada. A population-based pediatric rheumatology clinical database from April 1st 1980 to March 31st 2012 was used to test case definitions in individuals diagnosed at ≤15 years of age. The case definitions varied the number of diagnosis codes (1, 2, or 3), time frame (1, 2 or 3 years), time between diagnoses (ever, >1 day, or ≥8 weeks), and physician specialty...
May 13, 2017: Rheumatology International
https://www.readbyqxmd.com/read/28475276/cytokine-biomarkers-of-disease-activity-and-therapeutic-response-after-initiating-methotrexate-therapy-in-patients-with-juvenile-idiopathic-arthritis
#6
Ryan S Funk, Marcia A Chan, Mara L Becker
STUDY OBJECTIVE: To evaluate the relationship between plasma cytokine levels with disease activity and therapeutic response in patients with juvenile idiopathic arthritis after initiating methotrexate (MTX) therapy. DESIGN: Single-center, observational, prospective cohort study. SETTING: Outpatient pediatric rheumatology clinic at a tertiary care academic pediatric hospital. PATIENTS: Sixty-one patients diagnosed with juvenile idiopathic arthritis who started therapy with standard-dose methotrexate (MTX) 15 mg/m(2) /week; at 3 months, treating physicians were given the option of maintaining the MTX dose, increasing the MTX dose, or adding etanercept, based on their clinical judgment...
May 5, 2017: Pharmacotherapy
https://www.readbyqxmd.com/read/28464558/prescribing-for-children-with-rheumatologic-disease-perceived-treatment-approaches-between-pediatric-and-adult-rheumatologists
#7
Heather Van Mater, Stephen J Balevic, Gary L Freed, Sarah J Clark
OBJECTIVE: To compare practice patterns and prescribing differences for juvenile idiopathic arthritis (JIA) between adult and pediatric rheumatologists, the perceived educational needs, and factors that enhance or impede co-management. METHODS: Two parallel, cross sectional surveys focusing on JIA were administered in 2009 to a random sample of 193 pediatric rheumatologists (PR) and 500 adult rheumatologists (AR) using the American College of Rheumatology membership file...
May 2, 2017: Arthritis Care & Research
https://www.readbyqxmd.com/read/28461821/assessment-of-neutrophil-to-lymphocyte-ratio-and-mean-platelet-volume-in-pediatric-familial-mediterranean-fever-patients
#8
Ozge Basaran, Nermin Uncu, Banu Acar Celikel, Fatma Aydın, Nilgun Cakar
BACKGROUND: Blood neutrophil to lymphocyte ratio (NLR) and mean platelet volume (MPV) both have been used as a simple marker of inflammation in many disorders. Here, we aimed to investigate the relationship between NLR, MPV, and familial Mediterranean fever (FMF). MATERIALS AND METHODS: In this retrospective study, the files of FMF patients in pediatric rheumatology outpatient clinic were reviewed. There were 160 participants (68.4%) in the FMF patient group and 74 participants (31...
2017: Journal of Research in Medical Sciences: the Official Journal of Isfahan University of Medical Sciences
https://www.readbyqxmd.com/read/28461649/canadian-rheumatology-association-meeting-the-westin-ottawa-ottawa-ontario-canada-february-8-11-2017
#9
Earl D Silverman
The 72nd Annual Meeting of The Canadian Rheumatology Association (CRA) was held at The Westin Ottawa, Ottawa, Ontario, Canada, February 8-11, 2017. The program consisted of presentations covering original research, symposia, awards, and lectures. Highlights of the meeting include the following 2017 award winners: Dr. Vinod Chandran, Young Investigator; Dr. Jacques P. Brown, Distinguished Investigator; Dr. David Robinson, Teacher-Educator; Dr. Michel Zummer, Distinguished Rheumatologist; Ms. Rebecca Gole, Best Abstract on SLE Research by a Trainee - Ian Watson Award; Ms...
May 1, 2017: Journal of Rheumatology
https://www.readbyqxmd.com/read/28457525/retrospective-study-evaluating-treatment-decisions-and-outcomes-of-childhood-uveitis-not-associated-with-juvenile-idiopathic-arthritis
#10
Elham Sardar, Perrine Dusser, Antoine Rousseau, Bahram Bodaghi, Marc Labetoulle, Isabelle Koné-Paut
OBJECTIVE: To evaluate treatment, ocular complications and outcomes of children with pediatric uveitis not associated with juvenile idiopathic arthritis. STUDY DESIGN: This was a retrospective chart review of pediatric uveitis in children under 16 years of age, recruited from the pediatric rheumatology department at Bicêtre Hospital from 2005 to 2015. Patients with juvenile idiopathic arthritis-associated and infectious uveitis were excluded. We used the Standardization of Uveitis Nomenclature Working Group to classify uveitis, disease activity, and treatment end points...
April 27, 2017: Journal of Pediatrics
https://www.readbyqxmd.com/read/28453344/pediatric-hereditary-angioedema-as-a-cause-of-acute-compartment-syndrome-of-the-hand-and-forearm-a-case-report
#11
Chelsea Venditto, Zachary Jager, John LoGiudice, Hani Matloub
BACKGROUND: Compartment syndrome of the upper extremity is a surgical emergency that, when left untreated, can have dire consequences. Its causes are numerous, one of which is the uncommon entity hereditary angioedema, an autosomal dominant disease resulting in edema in a variety of potential locations, including the extremities. This is only the second time hereditary angioedema has been mentioned in the literature as a cause of compartment syndrome. METHODS: We present a case of hereditary angioedema leading to hand and forearm compartment syndrome in a 13-year-old pediatric patient...
May 2017: Hand: Official Journal of the American Association for Hand Surgery
https://www.readbyqxmd.com/read/28444038/difficulties-in-accessing-services-that-are-of-medium-complexity-in-small-municipalities-a-case-study
#12
Camila Ribeiro Silva, Brigida Gimenez Carvalho, Luiz Cordoni, Elisabete de Fátima Pólo de Almeida Nunes
The study aimed to describe the specialized health services and to identify areas of greater difficulty of access to specialized consultations offered by SUS in small cities in the 18th Regional Health Area of Paraná State, Brazil, using case study methodology. The data were collected between January and April 2015. Managers, management teams and the board of directors of the CIS (Consórcio Intermunicipal de Saúde) were interviewed. The 21 studied specialist areas were rated like Sufficient Quota, Insufficient Quota, Inexistent Supply, and Assistance Gap...
April 2017: Ciência & Saúde Coletiva
https://www.readbyqxmd.com/read/28437291/behcet-disease-initially-presenting-as-deep-venous-thrombosis-a-case-report
#13
Arun Gurunathan, David Teachey, Kudakwashe R Chikwava, Char Witmer, Ami V Desai
Behcet disease is a potentially life-threatening multisystemic vasculitis with thrombotic tendency. Mucocutaneous ulcers, arthritis, and uveitis are the most recognizable features, but may be absent at the time of medical evaluation. We report a case in which a 8-year old patient presented with spontaneous bilateral lower extremity deep venous thromboses, and screening for rheumatologic symptoms led to diagnosing Behcet. This case demonstrates that deep venous thromboses can be the initial event bringing a patient with Behcet to medical attention, highlighting the importance of screening for underlying rheumatologic diseases in pediatric patients who present with unprovoked thrombosis...
April 21, 2017: Journal of Pediatric Hematology/oncology
https://www.readbyqxmd.com/read/28434948/international-and-multidisciplinary-expert-recommendations-for-the-use-of-biologics-in-systemic-lupus-erythematosus
#14
REVIEW
Jean-François Kleinmann, Florence Tubach, Véronique Le Guern, Alexis Mathian, Christophe Richez, David Saadoun, Karim Sacre, Jérémie Sellam, Raphaèle Seror, Zahir Amoura, Emmanuel Andres, Sylvain Audia, Brigitte Bader-Meunier, Gilles Blaison, Bernard Bonnotte, Patrice Cacoub, Sophie Caillard, Laurent Chiche, Olivier Chosidow, Nathalie Costedoat-Chalumeau, Claire Daien, Eric Daugas, Nairouz Derdèche, Andrea Doria, Olivier Fain, Fadi Fakhouri, Dominique Farge, Cem Gabay, Sylvie Guillo, Eric Hachulla, Najia Hajjaj-Hassouni, Mohamed Hamidou, Frédéric A Houssiau, Noémie Jourde-Chiche, Isabelle Koné-Paut, Aïcha Ladjouz-Rezig, Olivier Lambotte, Dan Lipsker, Xavier Mariette, Nicolas Martin-Silva, Thierry Martin, François Maurier, Roderich Meckenstock, Arsène Mékinian, Olivier Meyer, Shirine Mohamed, Jacques Morel, Bruno Moulin, Denis Mulleman, Thomas Papo, Vincent Poindron, Xavier Puéchal, Leonardo Punzi, Pierre Quartier, Laurent Sailler, Amar Smail, Martin Soubrier, Agnès Sparsa, Zoubida Tazi-Mezalek, Leith Zakraoui, Stéphane Zuily, Jean Sibilia, Jacques-Eric Gottenberg
BACKGROUND/PURPOSE: Despite conventional immunosuppressants, active and steroid-dependent systemic lupus erythematosus (SLE) represents a therapeutic challenge. Only one biologic, belimumab, has been approved, but other biologics are sometimes used off-label. Given the lack of evidence-based data in some clinical situations encountered in real life, we developed expert recommendations for the use of biologics for SLE. METHODS: The recommendations were developed by a formal consensus method...
April 18, 2017: Autoimmunity Reviews
https://www.readbyqxmd.com/read/28432766/2017-acr-arhp-pediatric-rheumatology-symposium
#15
(no author information available yet)
For a searchable version of these abstracts, please visit www.acrabstracts.org.
April 2017: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/28420068/pentraxin-3-levels-are-associated-with-vasculitis-and-disease-activity-in-childhood-onset-systemic-lupus-erythematosus
#16
S Sahin, A Adrovic, K Barut, S Durmus, R Gelisgen, H Uzun, O Kasapcopur
Objectives Childhood-onset systemic lupus erythematosus (cSLE) is a multisystemic autoimmune disease characterized by inflammatory organ damage by means of vasculitis. Pentraxin-3 (PTX3) is expressed locally at the sites of inflammatory processes, predominantly from endothelial cells. In adult studies, PTX3 has shown to be an indicator of active vasculitis both in large-vessel and small-vessel vasculitides, as well as in SLE. Moreover, in SLE it has found to be correlated with disease activity, and with some of the clinical manifestations and laboratory parameters...
January 1, 2017: Lupus
https://www.readbyqxmd.com/read/28412707/application-of-the-2016-eular-acr-printo-classification-criteria-for-macrophage-activation-syndrome-in-patients-with-adult-onset-still-disease
#17
Sung Soo Ahn, Byung-Woo Yoo, Seung Min Jung, Sang-Won Lee, Yong-Beom Park, Jason Jungsik Song
OBJECTIVE: To evaluate the clinical significance of the 2016 European League Against Rheumatism (EULAR)/American College of Rheumatology (ACR)/Pediatric Rheumatology International Trials Organization (PRINTO) classification criteria for macrophage activation syndrome (MAS) in patients with adult-onset Still disease (AOSD). METHODS: We performed a retrospective analysis of patients with AOSD with fever who were admitted to Severance Hospital between 2005 and 2016...
April 15, 2017: Journal of Rheumatology
https://www.readbyqxmd.com/read/28399893/ultrasound-guided-steroid-tendon-sheath-injections-in-juvenile-idiopathic-arthritis-a-10-year-single-center-retrospective-study
#18
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
#19
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/28394238/anti-ribosomal-p-antibody-a-multicenter-study-in-childhood-onset-systemic-lupus-erythematosus-patients
#20
C C M Valões, B C Molinari, A C G Pitta, N W S Gormezano, S C L Farhat, K Kozu, A M E Sallum, S Appenzeller, A P Sakamoto, M T Terreri, R M R Pereira, C S Magalhães, J C O A Ferreira, C M Barbosa, F H Gomes, E Bonfá, C A Silva
Objectives Anti-ribosomal P protein (anti-P) autoantibodies are highly specific for systemic lupus erythematosus (SLE). However, the evaluation of this autoantibody in childhood-onset SLE (cSLE) populations has been limited to a few small series, hampering the interpretation of the clinical and laboratorial associations. Therefore, the objective of this multicenter cohort study was to evaluate demographic, clinical/laboratorial features, and disease damage score in cSLE patients with and without the presence of anti-P antibody...
April 2017: Lupus
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