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Pediatric Rheumatology Online Journal

Courtney B Crayne, Esraa Eloseily, Melissa L Mannion, Saji P Azerf, Peter Weiser, Timothy Beukelman, Matthew L Stoll, Daniel I Feig, T Prescott Atkinson, Randy Quentin Cron
BACKGROUND: Henoch-Schonlein purpura (HSP) is a small vessel vasculitis that is characterized by non-thrombocytopenic purpura, abdominal pain, arthritis, and glomerulonephritis. Typically, HSP is self-limited requiring only supportive care, but more severe cases may require corticosteroid (CS) treatment. Rarely, a subset of these patients has persistent rash, arthritis, abdominal involvement, or renal disease despite treatment with CS, or has disease recurrence on CS tapering. Refractory HSP has been effectively treated with a variety of CS sparing therapies...
November 14, 2018: Pediatric Rheumatology Online Journal
Michael C Kwa, Jonathan I Silverberg, Kaveh Ardalan
BACKGROUND: Juvenile dermatomyositis (JDM) is a rare autoimmune disease that causes significant morbidity and quality of life impairment. Little is known about the inpatient burden of JDM in the US. Our goal was to determine the prevalence and risk factors for hospitalization with juvenile dermatomyositis and assess inpatient burden of JDM. METHODS: Data on 14,401,668 pediatric hospitalizations from the 2002-2012 Nationwide Inpatient Sample (NIS) was analyzed. ICD-9-CM coding was used to identify hospitalizations with a diagnosis of JDM...
November 13, 2018: Pediatric Rheumatology Online Journal
Georgina Tiller, Joanne Buckle, Roger Allen, Jane Munro, Peter Gowdie, Angela Cox, Jonathan Akikusa
BACKGROUND: The advent of new treatments for Juvenile Idiopathic Arthritis (JIA) has prompted interest in systematically studying the outcomes of patients treated in the 'modern era'. Such data provide both benchmarks for assessing local outcomes and important information for use in counselling families of newly diagnosed patients. While data are available for cohorts in Europe and North America, no such data exist for Australian patients. The aim was to examine the demographics, treatment and outcomes at 12 months of an inception cohort of newly diagnosed patients with JIA at a single tertiary referral paediatric rheumatology centre in Australia...
November 9, 2018: Pediatric Rheumatology Online Journal
Grace O'Sullivan, Siobhán O'Higgins, Line Caes, Sophia Saetes, Brian E McGuire, Jennifer Stinson
BACKGROUND: Juvenile Idiopathic Arthritis (JIA) affects over 1000 children and adolescents in Ireland, potentially impacting health-related quality-of-life. Accessible self-management strategies, including Internet-based interventions, can support adolescents in Ireland where specialist rheumatology care is geographically-centralised within the capital city. This study interviewed adolescents with JIA, their parents, and healthcare professionals to (i) explore the self-management needs of Irish adolescents; and (ii) evaluate the acceptability of an adapted version of a Canadian JIA self-management programme (Teens Taking Charge: Managing Arthritis Online, or TTC) for Irish users...
November 8, 2018: Pediatric Rheumatology Online Journal
Kristin M Houghton, Heather M Macdonald, Heather A McKay, Jaime Guzman, Ciarán Duffy, Lori Tucker
BACKGROUND: Arthritis in childhood can be associated with muscle weakness around affected joints, low bone mass and low bone strength. Exercise is recognized as an important part of management of children with juvenile idiopathic arthritis (JIA) but the exercise prescription to best promote bone and muscle health is unknown. We therefore aimed to: 1. assess feasibility and safety of a 6-month home- and group-based exercise program for children with JIA; 2. estimate the effect of program participation on bone mass and strength, muscle function and clinical outcomes and 3...
October 22, 2018: Pediatric Rheumatology Online Journal
Liane D Heale, Saunya Dover, Y Ingrid Goh, Victoria A Maksymiuk, Greg D Wells, Brian M Feldman
BACKGROUND: Children and adolescents with juvenile idiopathic arthritis (JIA) are less physically active than their healthy peers and are at high risk of missing out on the general health benefits of physical activity. Wearable activity trackers are a promising option for intervening in this population with potential advantages over traditional exercise prescriptions. The objectives of this study were to: (1) determine the feasibility of a wearable activity tracker intervention in adolescents with JIA; and (2) estimate the variability in response to a wearable activity tracker intervention on the physical activity levels of adolescents with JIA...
October 22, 2018: Pediatric Rheumatology Online Journal
Jennifer C Cooper, Kelly Rouster-Stevens, Tracey B Wright, Joyce J Hsu, Marisa S Klein-Gitelman, Stacy P Ardoin, Laura E Schanberg, Hermine I Brunner, B Anne Eberhard, Linda Wagner-Weiner, Jay Mehta, Kathleen Haines, Deborah K McCurdy, Thomas A Phillips, Zhen Huang, Emily von Scheven
BACKGROUND: To reduce treatment variability and facilitate comparative effectiveness studies, the Childhood Arthritis and Rheumatology Research Alliance (CARRA) published consensus treatment plans (CTPs) including one for juvenile proliferative lupus nephritis (LN). Induction immunosuppression CTPs outline treatment with either monthly intravenous (IV) cyclophosphamide (CYC) or mycophenolate mofetil (MMF) in conjunction with one of three corticosteroid (steroid) CTPs: primarily oral, primarily IV or mixed oral/IV...
October 22, 2018: Pediatric Rheumatology Online Journal
Kristine Risum, Bjørge Herman Hansen, Anne Marit Selvaag, Øyvind Molberg, Hanne Dagfinrud, Helga Sanner
BACKGROUND: Knowledge about objectively measured levels of physical activity (PA) and PA participation (included facilitators and barriers for PA) in patients with juvenile idiopathic arthritis (JIA) diagnosed in the era of biologics is limited. We aimed to compare objectively measured PA in patients with oligo- and polyarticular JIA diagnosed in the biologic era with controls and to examine associations between PA and disease variables; furthermore, to explore participation in PA, physical education (PE) and facilitators and barriers for PA participation in patients and controls...
October 17, 2018: Pediatric Rheumatology Online Journal
Livia de Freitas Keppeke, Juliana Molina, Vanessa Bugni Miotto E Silva, Maria Teresa de Sande E Lemos Ramos Ascensão Terreri, Gerson Dierley Keppeke, Teresa Helena Schoen, Claudio Arnaldo Len
BACKGROUND: Adherence to treatment for chronic diseases is lower in children than in adults, less extensively studied in children and is associated with multiple related factors. The aim of this study is to perform a descriptive analysis of psycho-cognitive aspects of primary caregivers of pediatric patients with chronic rheumatic diseases, as well as socioeconomic and clinical factors, family functioning and treatment satisfaction. METHODS: Primary caregivers of 90 patients were included...
October 12, 2018: Pediatric Rheumatology Online Journal
Pondtip Jongvilaikasem, Edward B McNeil, Pornsak Dissaneewate, Prayong Vachvanichsanong
BACKGROUND: Morbidity and mortality in childhood onset systemic lupus erythematosus (SLE) is more severe than adult onset SLE. Long-term follow up is needed to determine the prognosis. The objectives of this study are to describe the mortality of childhood SLE in a single tertiary care centre over three decades, compare trends in survival over time, and determine predictors for survival. METHODS: We retrospectively reviewed the medical records of children aged < 18 years who were diagnosed with SLE at the Department of Pediatrics, Songklanagarind Hospital, Thailand, from 1985 to 2016...
September 29, 2018: Pediatric Rheumatology Online Journal
Linda Lei, Sabina Muhammad, Muthana Al-Obaidi, Neil Sebire, Iek Leng Cheng, Despina Eleftheriou, Paul Brogan
BACKGROUND: We previously described an endogamous Pakistani kindred in whom we identified a novel homozygous missense mutation in the PRKCD gene encoding for protein kinase C δ (PKCδ) as a cause of monogenic systemic lupus erythematosus (SLE). PKCδ has a role in the negative regulation of B cells. Given the nature of the disease, a logical targeted therapeutic approach in these patients is B cell depletion. Indeed, the 3 siblings all had a marked clinical response and resolution of symptoms with rituximab, although 2 of the siblings had severe reactions to rituximab thus precluding further treatment with this...
September 26, 2018: Pediatric Rheumatology Online Journal
Federica Vanoni, Roberta Caorsi, Sandra Aeby, Marie Cochard, Jordi Antón, Stefan Berg, Riva Brik, Pavla Dolezalova, Isabelle Koné-Paut, Benedicte Neven, Seza Ozen, Pascal Pillet, Silvia Stojanov, Carine Wouters, Marco Gattorno, Michaël Hofer
BACKGROUND: Diagnosis of Periodic Fever, Aphthous stomatitis, Pharyngitis and Cervical Adenitis (PFAPA) syndrome is currently based on the modified Marshall's criteria, but no validated evidence based classification criteria for PFAPA has been established so far. METHODS: A multistep process, based on the Delphi and Nominal Group Technique was conducted. After 2 rounds of e-mail Delphi survey involving 21 experts in autoinflammation we obtained a list of variables that were discussed in an International Consensus Conference...
September 21, 2018: Pediatric Rheumatology Online Journal
Joanie Sims-Gould, Douglas L Race, Heather Macdonald, Kristin M Houghton, Ciarán M Duffy, Lori B Tucker, Heather A McKay
BACKGROUND: Physical activity is essential for ensuring optimal physical function and fitness in children with juvenile idiopathic arthritis (JIA). Although exercise intervention trials informed current clinical practice, few studies addressed why children with JIA do or do not participate in exercise interventions. We aimed to describe perceived barriers and facilitators to the uptake and adherence to a 6-month home-based exercise intervention for children diagnosed with JIA and their parents...
September 20, 2018: Pediatric Rheumatology Online Journal
Lucio Ventura-Ríos, Enrique Faugier, Laura Barzola, L B De la Cruz-Becerra, Guadalupe Sánchez-Bringas, Andrés Rodríguez García, Rocío Maldonado, Johannes Roth, Cristina Hernández-Díaz
BACKGROUND: Musculoskeletal Ultrasonography (MSUS) is an important tool for the clinical assessment in Juvenile Idiopathic Arthritis (JIA). The objective of this study was to evaluate the reliability of MSUS to detect elementary lesions: synovitis, tenosynovitis, cartilage damage and bone erosions in the wrist and metacarpal (MCP) joints of patients with JIA. METHODS: Thirty children in various subgroups of JIA according to ILAR criteria, were included in this cross-sectional study...
September 17, 2018: Pediatric Rheumatology Online Journal
Casper G Schoemaker, Wineke Armbrust, Joost F Swart, Sebastiaan J Vastert, Jorg van Loosdregt, Anouk Verwoerd, Caroline Whiting, Katherine Cowan, Wendy Olsder, Els Versluis, Rens van Vliet, Marlous J Fernhout, Sanne L Bookelman, Jeannette Cappon, J Merlijn van den Berg, Ellen Schatorjé, Petra C E Hissink Muller, Sylvia Kamphuis, Joke de Boer, Otto T H M Lelieveld, Janjaap van der Net, Karin R Jongsma, Annemiek van Rensen, Christine Dedding, Nico M Wulffraat
BACKGROUND: Research on Juvenile Idiopathic Arthritis (JIA) should support patients, caregivers/parents (carers) and clinicians to make important decisions in the consulting room and eventually to improve the lives of patients with JIA. Thus far these end-users of JIA-research have rarely been involved in the prioritisation of future research. MAIN BODY: Dutch organisations of patients, carers and clinicians will collaboratively develop a research agenda for JIA, following the James Lind Alliance (JLA) methodology...
September 15, 2018: Pediatric Rheumatology Online Journal
Jacqueline K K Sit, Winnie K Y Chan
BACKGROUND: Accumulated damage is an important prognostic factor in systemic lupus erythematous. However, the pattern of disease damage and its risk factors have not been well studied in childhood-onset systemic lupus erythematosus (cSLE) in Asia. The objectives are to evaluate the pattern of damage and to identify the risk factors for accumulated damage in an Asian group of cSLE. METHODS: A retrospective chart review was conducted on a group of 59 patients with cSLE...
September 10, 2018: Pediatric Rheumatology Online Journal
Wenji Chen, Shiyan Mo, Gui Luo, Yanyan Wang, Xiaohu Deng, Jian Zhu, Wei Zhao
BACKGROUND: As one kind of osteochondrodysplasia, progressive pseudorheumatoid dysplasia (PPD) is also known as spondyloepiphyseal dysplasia tarda with progressive arthropathy or arthropathy progressive pseudorheumatoid of childhood. PPD is a very rare disease, especially in China, and has an estimated prevalence of 1/1000000 due to lacking definite prevalence survey. It is an autosomal recessive disorder caused by gene mutation of Wntl inducible signaling pathway protein 3 (WISP3). Its basic pathological change is persistent degeneration and loss of articular cartilage in multiple joints...
September 10, 2018: Pediatric Rheumatology Online Journal
Jessica L Bloom, Jeffrey R Darst, Lori Prok, Jennifer B Soep
BACKGROUND: Henoch-Schonlein Purpura (HSP) is one of the most common vasculitides of childhood, with 10-20 cases per 100,000 children. It frequently occurs following an infectious trigger and involves IgA and C3 deposition in small vessel walls. HSP is characterized by palpable purpura plus IgA deposition on biopsy, arthritis/arthralgia, renal involvement (hematuria and/or proteinuria), and/or abdominal pain. It is not generally recognized as a cause of dilated coronary arteries. CASE PRESENTATION: We describe the first reported case of HSP presenting with dilated coronary arteries...
September 4, 2018: Pediatric Rheumatology Online Journal
Veronica Lundberg, Viveca Lindh, Catharina Eriksson, Solveig Petersen, Eva Eurenius
Following publication of the original article [1], the authors reported an error in the data of their article: one girl was by mistake scored as a boy. The authors have made new analyses of the corrected data. The corrected data and the new analyses are listed in this Correction.
August 22, 2018: Pediatric Rheumatology Online Journal
Megan Cann, Athimalaipet V Ramanan, Andrew Crawford, Andrew D Dick, Sarah L N Clarke, Fatima Rashed, Catherine M Guly
BACKGROUND: There is a paucity of data on the ocular outcomes in paediatric non-infectious uveitis since the introduction of the biologic agents. The purpose of this study was to outline the clinical characteristics of children with non-infectious uveitis and determine the visual outcomes and ocular complication rates in the modern era. METHODS: Children with non-infectious uveitis from January 2011 to December 2015 were identified. Data was collected at baseline, 1, 3, 5, and 10 years post diagnosis...
August 6, 2018: Pediatric Rheumatology Online Journal
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