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Paediatric rheumatology

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https://www.readbyqxmd.com/read/30100561/consensus-proposal-for-taxonomy-and-definition-of-the-autoinflammatory-diseases-aids-a-delphi-study
#1
REVIEW
Eldad Ben-Chetrit, Marco Gattorno, Ahmet Gul, Daniel L Kastner, Helen J Lachmann, Isabelle Touitou, Nicolino Ruperto
Autoinflammatory diseases (AIDs) are a relatively new family of disorders, defined about 19 years ago. Some of them are hereditary and some are not. The names given to these diseases do not follow any systematic guidelines, and sometimes the same disorder carries several names. The aim of this study is to refine the definition of AIDs and to provide some conventions for their naming. We focused mainly on monogenetic AIDs. Delphi technique, which enables consensus among a group of experts through internet and mail communication and questionnaires, was employed...
August 12, 2018: Annals of the Rheumatic Diseases
https://www.readbyqxmd.com/read/30051293/are-the-2016-eular-acr-printo-classification-criteria-for-macrophage-activation-syndrome-applicable-to-patients-with-adult-onset-still-s-disease
#2
Yoshifumi Tada, Satomi Inokuchi, Akihito Maruyama, Rie Suematsu, Mariko Sakai, Yuri Sadanaga, Nobuyuki Ono, Yojiro Arinobu, Syuichi Koarada
The objectives of this study are to determine whether the 2016 European League Against Rheumatism/American College of Rheumatology/Paediatric Rheumatology International Trials Organization classification criteria for macrophage activation syndrome (MAS) complicating systemic juvenile idiopathic arthritis (SJIA) can be used to identify MAS in patients with adult-onset Still's disease (AOSD). Using laboratory data from 76 AOSD patients with and without MAS, we analyzed the ability of the collective and individual constitutive elements of the 2016 MAS in SJIA criteria and additional laboratory measures to discriminate between AOSD patients with (n = 16) and without (n = 60) MAS...
July 26, 2018: Rheumatology International
https://www.readbyqxmd.com/read/29972659/the-role-of-imaging-in-juvenile-idiopathic-arthritis
#3
Clara Malattia, Mariangela Rinaldi, Alberto Martini
The prognosis of juvenile idiopathic arthritis (JIA) has changed dramatically due to the availability of novel drugs. Prompt diagnosis and treatment are essential to prevent permanent joint damage. As a result, methods to improve JIA diagnosis and prognosis are of high priority to tailor treatment strategies and maximize their efficacy. Musculoskeletal ultrasound and magnetic resonance imaging are more sensitive than clinical examination and radiography in the detection of joint involvement and might play a substantial role to optimize the management of JIA...
July 20, 2018: Expert Review of Clinical Immunology
https://www.readbyqxmd.com/read/29961686/growth-during-tocilizumab-therapy-for-polyarticular-course-juvenile-idiopathic-arthritis-2-year-data-from-a-phase-iii-clinical-trial
#4
Kamal N Bharucha, Hermine I Brunner, Inmaculada Calvo Penadés, Irina Nikishina, Nadina Rubio-Pérez, Sheila Oliveira, Katarzyna Kobusinska, Heinrike Schmeling, Flavio Sztajnbok, Frank Weller-Heinemann, Elena Zholobova, Francesco Zulian, Roger Allen, Jeffrey Chaitow, James Frane, Chris Wells, Nicolino Ruperto, Fabrizio De Benedetti
OBJECTIVE: Evaluate growth in patients with polyarticular-course juvenile idiopathic arthritis (pcJIA) treated with tocilizumab (TCZ) for up to 2 years in a phase III trial. METHODS: Patients with pcJIA lasting at least 6 months and inadequate response to methotrexate received open-label TCZ intravenously every 4 weeks (randomly assigned to 8 or 10 mg/kg if they weighed < 30 kg; received 8 mg/kg if they weighed ≥ 30 kg) for 16 weeks. Patients with JIA American College of Rheumatology 30 response at Week 16 were randomly assigned to TCZ or placebo for 24 weeks, with an open-label extension through Week 104...
July 1, 2018: Journal of Rheumatology
https://www.readbyqxmd.com/read/29914516/reliability-of-loscat-score-for-activity-and-tissue-damage-assessment-in-a-large-cohort-of-patients-with-juvenile-localized-scleroderma
#5
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
https://www.readbyqxmd.com/read/29912602/comparing-immunoglobulin-a-vasculitis-henoch-sch%C3%A3-nlein-purpura-in-children-and-adults-a-single-centre-study-from-turkey
#6
E D Batu, A Sarı, A Erden, H E Sönmez, B Armağan, U Kalyoncu, Ö Karadağ, Y Bilginer, A Akdoğan, S Kiraz, S Özen
OBJECTIVE: Immunoglobulin A vasculitis/Henoch-Schönlein purpura (IgAV/HSP) is a systemic vasculitis involving small vessels with the deposition of immune complexes containing IgA. It is the most common primary systemic vasculitis of childhood and is much less common in adults. Our aim was to investigate the differences and similarities between adult and paediatric patients with IgAV/HSP. METHOD: We retrospectively evaluated the medical records of 35 adult and 159 paediatric (˂ 18 years old) patients with a clinical diagnosis of IgAV/HSP who were seen at the Departments of Rheumatology and Pediatric Rheumatology, Hacettepe University, Ankara, Turkey...
June 18, 2018: Scandinavian Journal of Rheumatology
https://www.readbyqxmd.com/read/29871839/-haemophagocytic-syndromes-the-importance-of-early-diagnosis-and-treatment
#7
Itziar Astigarraga, Luis I Gonzalez-Granado, Luis M Allende, Laia Alsina
Haemophagocytic syndrome, or haemophagocytic lymphohistiocytosis (HLH), is a disorder with high mortality, typically recognised at paediatric age. Without proper treatment, HLH can be fatal. The risk of a rapid progression to multi-organ failure and central nervous system involvement leading to long-term sequelae, are the most feared consequences of a diagnostic delay. Therefore, HLH is a medical emergency that paediatricians should be able to identify in a patient with fever and progressive worsening of general condition...
June 2, 2018: Anales de Pediatría: Publicación Oficial de la Asociación Española de Pediatría (A.E.P.)
https://www.readbyqxmd.com/read/29786780/correction-to-the-italian-version-of-the-juvenile-arthritis-multidimensional-assessment-report-jamar
#8
Alessandro Consolaro, Francesca Bovis, Angela Pistorio, Rolando Cimaz, Fabrizio De Benedetti, Angela Miniaci, Fabrizia Corona, Valeria Gerloni, Silvana Martino, Serena Pastore, Patrizia Barone, Sara Pieropan, Elisabetta Cortis, Rosa Anna Podda, Romina Gallizzi, Adele Civino, Francesco La Torre, Donato Rigante, Rita Consolini, Maria Cristina Maggio, Silvia Magni-Manzoni, Francesca Perfetti, Giovanni Filocamo, Claudia Toppino, Francesco Licciardi, Marco Garrone, Silvia Scala, Elisa Patrone, Monica Tonelli, Daniela Tani, Angelo Ravelli, Alberto Martini, Nicolino Ruperto
The family name of author Francesco La Torre was incorrect in the published article. The correct family name should read as La Torre F.
July 2018: Rheumatology International
https://www.readbyqxmd.com/read/29718410/the-multifaceted-presentation-of-chronic-recurrent-multifocal-osteomyelitis-a-series-of-486-cases-from-the-eurofever-international-registry
#9
Hermann Girschick, Martina Finetti, Francesca Orlando, Susanne Schalm, Antonella Insalaco, Gerd Ganser, Susan Nielsen, Troels Herlin, Isabelle Koné-Paut, Silvana Martino, Marco Cattalini, Jordi Anton, Sulaiman Mohammed Al-Mayouf, Michael Hofer, Pierre Quartier, Christina Boros, Jasmin Kuemmerle-Deschner, Denise Pires Marafon, Maria Alessio, Tobias Schwarz, Nicolino Ruperto, Alberto Martini, Annette Jansson, Marco Gattorno
No abstract text is available yet for this article.
April 30, 2018: Rheumatology
https://www.readbyqxmd.com/read/29690965/childhood-onset-systemic-lupus-erythematosus-a-cohort-study
#10
Shakeel Shakeel, Syed Rehan Ali, Farida Karim, Sidra Ishaq, Iman Qaiser, Kanwal Nayani, Ayesha Ahmed
OBJECTIVE: To determine the clinical and immunological characteristics and short-term outcome of children with systemic lupus erythematosus (SLE). STUDY DESIGN: A descriptive cohort study. PLACE AND DURATION OF STUDY: Paediatric Rheumatology Clinic, The Aga Khan University Hospital, Karachi, from January 2011 to December 2015. METHODOLOGY: Clinical and immunological profile and short-term outcome of children less than 16 years of age admitted in the paediatric ward, with the diagnosis of SLE was studied...
May 2018: Journal of the College of Physicians and Surgeons—Pakistan: JCPSP
https://www.readbyqxmd.com/read/29685142/correction-to-a-national-cohort-study-on-pediatric-beh%C3%A3-et-s-disease-cross-sectional-data-from-an-italian-registry
#11
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
https://www.readbyqxmd.com/read/29669569/an-international-delphi-survey-for-the-definition-of-the-variables-for-the-development-of-new-classification-criteria-for-periodic-fever-aphtous-stomatitis-pharingitis-cervical-adenitis-pfapa
#12
Federica Vanoni, Silvia Federici, Jordi Antón, Karyl S Barron, Paul Brogan, Fabrizio De Benedetti, Fatma Dedeoglu, Erkan Demirkaya, Veronique Hentgen, Tilmann Kallinich, Ronald Laxer, Ricardo Russo, Natasa Toplak, Yosef Uziel, Alberto Martini, Nicolino Ruperto, Marco Gattorno, Michael Hofer
BACKGROUND: Diagnosis of Periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA) is currently based on a set of criteria proposed in 1999 modified from Marshall's criteria. Nevertheless no validated evidence based set of classification criteria for PFAPA has been established so far. The aim of this study was to identify candidate classification criteria PFAPA syndrome using international consensus formation through a Delphi questionnaire survey. METHODS: A first open-ended questionnaire was sent to adult and pediatric clinicians/researchers, asking to identify the variables thought most likely to be helpful and relevant for the diagnosis of PFAPA...
April 18, 2018: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/29652654/patients-with-juvenile-idiopathic-arthritis-become-adults-the-role-of-transitional-care
#13
REVIEW
Fabrizio Conti, Irene Pontikaki, Mariella D'Andrea, Angelo Ravelli, Fabrizio De Benedetti
Most juvenile idiopathic arthritis (JIA) patients need to attend adult rheumatology centres to continue the clinical management of their disease and to receive adequate long-term treatment. Transition from the paediatric to the adult health care team is a critical moment in the clinical history of these patients, but unfortunately, about 50% of the transfer processes to adult rheumatology are not successful, putting these patients at high risk of unfavourable outcomes. There are several obstacles to the success of transitional care for JIA patients, such as the absence of specific criteria for the assessment of disease activity, the lack of specific treatment recommendations for JIA adult patients, the poor adolescent-specific training for adult rheumatologists, and the shortage of resources...
April 13, 2018: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/29600947/disease-activity-overweight-physical-activity-and-screen-time-in-a-cohort-of-patients-with-juvenile-idiopathic-arthritis
#14
Gilad Sherman, Dan Nemet, Veronica Moshe, Alessandro Consolaro, Angelo Ravelli, Nicolino Ruperto, Yosef Uziel
OBJECTIVES: Physical activity is an important part of children's health and social development. Juvenile idiopathic arthritis (JIA) can lead to decreased physical activity and quality of life. This study characterised clinical aspects, physical activity rates, obesity, and screen time in a group of JIA patients. METHODS: Ninety-seven JIA patients in the Paediatric Rheumatology Clinic at Meir Medical Center were evaluated over a 6-month period and compared by statistical methods to 98 age-matched healthy controls...
March 21, 2018: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/29593902/confidence-amongst-multidisciplinary-professionals-in-managing-paediatric-rheumatic-disease-in-australia
#15
Samuel Cassidy, Andrea Coda, Kerry West, Gordon Hendry, Debra Grech, Julie Jones, Fiona Hawke, Davinder Singh-Grewal
Objective: Interprofessional collaboration is a crucial component of care for children with rheumatic disease. Interprofessional care, when delivered appropriately, prevents disability and improves long-term prognosis in this vulnerable group. Methods: The aim of this survey was to explore allied health professionals' and nurses' confidence in treating paediatric rheumatology patients. Results: Overall, 117 participants were recruited, 77...
2018: Arthritis
https://www.readbyqxmd.com/read/29592918/consensus-based-recommendations-for-the-management-of-uveitis-associated-with-juvenile-idiopathic-arthritis-the-share-initiative
#16
Tamas Constantin, Ivan Foeldvari, Jordi Anton, Joke de Boer, Severine Czitrom-Guillaume, Clive Edelsten, Raz Gepstein, Arnd Heiligenhaus, Clarissa A Pilkington, Gabriele Simonini, Yosef Uziel, Sebastian J Vastert, Nico M Wulffraat, Anne-Mieke Haasnoot, Karoline Walscheid, Annamária Pálinkás, Reshma Pattani, Zoltán Györgyi, Richárd Kozma, Victor Boom, Andrea Ponyi, Angelo Ravelli, Athimalaipet V Ramanan
BACKGROUND: In 2012, a European initiative called S ingle Hub and Access point for pediatric Rheumatology in Europe (SHARE) was launched to optimise and disseminate diagnostic and management regimens in Europe for children and young adults with rheumatic diseases. Juvenile idiopathic arthritis (JIA) is the most common rheumatic disease in children and uveitis is possibly its most devastating extra-articular manifestation. Evidence-based guidelines are sparse and management is mostly based on physicians' experience...
August 2018: Annals of the Rheumatic Diseases
https://www.readbyqxmd.com/read/29582130/conventional-radiography-in-juvenile-idiopathic-arthritis-joint-recommendations-from-the-french-societies-for-rheumatology-radiology-and-paediatric-rheumatology
#17
Pauline Marteau, Catherine Adamsbaum, Linda Rossi-Semerano, Michel De Bandt, Irène Lemelle, Chantal Deslandre, Tu Anh Tran, Anne Lohse, Elisabeth Solau-Gervais, Christelle Sordet, Pascal Pillet, Brigitte Bader-Meunier, Julien Wipff, Cécile Gaujoux-Viala, Sylvain Breton, Valérie Devauchelle-Pensec
BACKGROUND: Juvenile idiopathic arthritis (JIA) can cause structural damage. However, data on conventional radiography (CR) in JIA are scant. OBJECTIVE: To provide pragmatic guidelines on CR in each non-systemic JIA subtype. METHODS: A multidisciplinary task force of 16 French experts (rheumatologists, paediatricians, radiologists and one patient representative) formulated research questions on CR assessments in each non-systemic JIA subtype...
March 26, 2018: European Radiology
https://www.readbyqxmd.com/read/29533755/efficacy-and-safety-of-canakinumab-in-patients-with-still-s-disease-exposure-response-analysis-of-pooled-systemic-juvenile-idiopathic-arthritis-data-by-age-groups
#18
Eugen Feist, Pierre Quartier, Bruno Fautrel, Rayfel Schneider, Paolo Sfriso, Petros Efthimiou, Luca Cantarini, Karine Lheritier, Karolynn Leon, Chetan S Karyekar, Antonio Speziale
OBJECTIVES: To describe the efficacy, safety, and exposure-response relationship of canakinumab in a subgroup of patients with systemic juvenile idiopathic arthritis (SJIA) aged ≥16 years, representative of adult-onset Still's disease (AOSD) patients, and to compare this subgroup with those of children and young adolescents with SJIA by pooling clinical data collected during the development programme of canakinumab. METHODS: Safety and efficacy data on canakinumab-treated patients were pooled from 4 SJIA studies (NCT00426218, NCT00886769, NCT00889863, and NCT00891046)...
March 2, 2018: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/29481737/subcutaneous-abatacept-in-patients-with-polyarticular-course-juvenile-idiopathic-arthritis-results-from-a-phase-iii-open-label-study
#19
Hermine I Brunner, Nikolay Tzaribachev, Gabriel Vega-Cornejo, Ingrid Louw, Alberto Berman, Inmaculada Calvo Penadés, Jordi Antón, Francisco Ávila-Zapata, Rubén Cuttica, Gerd Horneff, Ivan Foeldvari, Vladimir Keltsev, Daniel J Kingsbury, Diego Oscar Viola, Rik Joos, Bernard Lauwerys, Maria Eliana Paz Gastañaga, Maria Elena Rama, Carine Wouters, John Bohnsack, Johannes Breedt, Michel Fischbach, Thomas Lutz, Kirsten Minden, Tatiana Miraval, Mahmood M T M Ally, Nadina Rubio-Pérez, Elisabeth Solau Gervais, Riana van Zyl, Xiaohui Li, Marleen Nys, Robert Wong, Subhashis Banerjee, Daniel J Lovell, Alberto Martini, Nicolino Ruperto
OBJECTIVE: To investigate the pharmacokinetics, effectiveness, and safety of subcutaneous (SC) abatacept treatment over 24 months in patients with polyarticular-course juvenile idiopathic arthritis (JIA). METHODS: In this phase III, open-label, international, multicenter, single-arm study, patients with polyarticular JIA (cohort 1, ages 6-17 years and cohort 2, ages 2-5 years) in whom treatment with ≥1 disease-modifying antirheumatic drug was unsuccessful received weight-tiered SC abatacept weekly: 10 to <25 kg (50 mg), 25 to <50 kg (87...
July 2018: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/29453217/time-spent-in-inactive-disease-before-mtx-withdrawal-is-relevant-with-regard-to-the-flare-risk-in-patients-with-jia
#20
Jens Klotsche, Kirsten Minden, Martina Niewerth, Gerd Horneff
OBJECTIVES: To determine the reasons of methotrexate (MTX) discontinuation, frequency of adverse events (AE) and whether the time in inactive disease before MTX withdrawal disease is associated with the risk of disease flare. METHODS: Patients with juvenile idiopathic arthritis (JIA) beginning treatment with MTX were prospectively observed in the national JIA biologic register Biologika in der Kinderrheumatologie/Biologics in Paediatric Rheumatology and its follow-up register Juvenile arthritis Methotrexate/Biologics long-term Observation...
July 2018: Annals of the Rheumatic Diseases
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