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Paediatric rheumatology

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https://www.readbyqxmd.com/read/30328080/paediatric-rheumatology-helen-foster-and-paul-a-brogan-eds-published-by-oxford-university-press-uk-second-edition-2018-isbn-978-0-19-873875-6
#1
Raju Khubchandani
No abstract text is available yet for this article.
October 16, 2018: Indian Journal of Pediatrics
https://www.readbyqxmd.com/read/30266876/big-data-and-stratified-medicine-what-does-it-mean-for-children
#2
REVIEW
Wen Y Ding, Michael W Beresford, Moin A Saleem, Athimalaipet V Ramanan
Stratified medicine in paediatrics is increasingly becoming a reality, as our understanding of disease pathogenesis improves and novel treatment targets emerge. We have already seen some success in paediatrics in targeted therapies such as cystic fibrosis for specific cystic fibrosis transmembrane conductance regulator variants. With the increased speed and decreased cost of processing and analysing data from rare disease registries, we are increasingly able to use a systems biology approach (including '-omics') to screen across populations for molecules and genes of interest...
September 28, 2018: Archives of Disease in Childhood
https://www.readbyqxmd.com/read/30249262/the-barriers-and-facilitators-to-the-implementation-of-national-clinical-programmes-in-ireland-using-the-mrc-framework-for-process-evaluations
#3
Catherine D Darker, Gail H Nicolson, Aine Carroll, Joe M Barry
BACKGROUND: A major healthcare reform agenda in Ireland is underway which underpins the establishment of a series of National Clinical Programmes (NCPs), which aim to take an evidence based approach to improve quality, access and value. The current study aimed to determine the enablers and barriers to implementation of the NCPs. METHODS: A qualitative methodology advocated by the Medical Research Council (MRC) framework on conducting process evaluations of complex interventions guided this research...
September 24, 2018: BMC Health Services Research
https://www.readbyqxmd.com/read/30234239/-an-alternative-proposal-for-managing-morphological-examination-of-urinary-sediment-and-increasing-its-appropriateness
#4
Cristina Robbiano, Ilenia Infusino, Federica Braga, Alberto Dolci, Mauro Panteghini
BACKGROUND: The morphological examination of urinary sediment (MEUS) is traditionally associated with urinalysis (UA), with workload implications and the need for automation of its execution. METHODS: Considering MEUS as a test requiring specialized knowhow and skill for its execution, since 2005 in our laboratory it is performed for inpatients only upon specific request. Eleven years after, we have analyzed the long-term impact of this approach on the provided service...
September 2018: Giornale Italiano di Nefrologia: Organo Ufficiale Della Società Italiana di Nefrologia
https://www.readbyqxmd.com/read/30232192/clinical-trials-in-children-and-adolescents-with-systemic-lupus-erythematosus-methodological-aspects-regulatory-landscape-and-future-opportunities
#5
Hermine I Brunner, Alberto Martini, Daniel J Lovell, Nicolino Ruperto
Childhood-onset systemic lupus erythematosus (cSLE) is rare in many regions of the world, including Europe. Access to approved medications for cSLE is currently limited, among others, due to a lack of high-quality evidence from clinical trials. The objectives of the study were to evaluate the current regulatory framework regarding medication approvals, delineate barriers to clinical trial conduct, and strategies to improve access to new medications for cSLE. Relevant methodological and regulatory aspects, epidemiological data, study designs and outcome measures are reviewed, and the results of a survey among Paediatric Rheumatology International Trials Organisation/Pediatric Rheumatology Collaborative Study Group investigators are presented...
September 19, 2018: Annals of the Rheumatic Diseases
https://www.readbyqxmd.com/read/30167316/transition-and-transfer-of-the-patient-with-paediatric-onset-lupus-a-practical-approach-for-paediatric-and-adult-rheumatology-practices
#6
Rebecca E Sadun, Laura E Schanberg
The prevalence of paediatric-onset SLE (pSLE) is estimated at 1million people worldwide and accounts for a significant proportion of SLE morbidity, mortality and cost. Patients with pSLE are especially vulnerable during and immediately following transfer from paediatric to adult rheumatology care, when substantial delays in care and increased disease activity are common. Transition is the process through which adolescents and young adults (AYA) develop the skills needed to succeed in the adult healthcare environment, a process that typically takes several years and may span a patient's time in paediatric and adult clinics...
2018: Lupus Science & Medicine
https://www.readbyqxmd.com/read/30100561/consensus-proposal-for-taxonomy-and-definition-of-the-autoinflammatory-diseases-aids-a-delphi-study
#7
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
#8
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
#9
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...
August 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
#10
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...
August 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
#11
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
#12
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
#13
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...
August 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
#14
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
#15
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.
August 1, 2018: Rheumatology
https://www.readbyqxmd.com/read/29690965/childhood-onset-systemic-lupus-erythematosus-a-cohort-study
#16
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
#17
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
#18
MULTICENTER STUDY
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
#19
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
#20
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
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