583 results match your criteria Pediatric Rheumatology Online Journal[Journal]


Clinical features and characteristics of uveitis associated with juvenile idiopathic arthritis in Japan: first report of the pediatric rheumatology association of Japan (PRAJ).

Pediatr Rheumatol Online J 2019 Apr 11;17(1):15. Epub 2019 Apr 11.

Department of Lifetime Clinical Immunology, Graduate School of Medical and Dental Sciences, Tokyo Medical and Dental University, 1-5-45 Yushima, Bunkyo-ku, Tokyo, 113-8510, Japan.

Background: Although there are many reports on Juvenile Idiopathic arthritis-associated uveitis (JIA-U) from various countries, especially from Europe and North America, there are few reports from Asia. Our aim was to investigate the epidemiology, characteristics and predictors of JIA-U in Japan.

Methods: Data were retrospectively collected on 726 patients with JIA from medical records as of April 2016 at 15 medical centers specialized in pediatric rheumatic diseases. Read More

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http://dx.doi.org/10.1186/s12969-019-0318-5DOI Listing
April 2019
1 Read

Measurement properties and performance of an eight-minute submaximal treadmill test in patients with juvenile idiopathic arthritis: a controlled study.

Pediatr Rheumatol Online J 2019 Apr 8;17(1):14. Epub 2019 Apr 8.

Department of Rheumatology, Oslo University Hospital, Rikshospitalet, Oslo, Norway.

Background: Poor cardiorespiratory fitness is previously reported in patients with juvenile idiopathic arthritis (JIA) measured both by maximal and submaximal exercise tests, but a submaximal exercise test with acceptable measurement properties is currently lacking for both clinical and research purposes in this patient population. The objectives of this study were to evaluate the measurement properties and performance of a submaximal treadmill test in patients with JIA, and to compare the results with those obtained in controls.

Methods: Fifty-nine patients (50 girls), aged 10-16 years, with oligo- (n = 30) and polyarticular (n = 29) JIA, and 59 age- and sex-matched controls performed an eight-minute submaximal treadmill test for estimating peak oxygen uptake (VO) followed by a maximal treadmill test measuring VO directly. Read More

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https://ped-rheum.biomedcentral.com/articles/10.1186/s12969-
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http://dx.doi.org/10.1186/s12969-019-0316-7DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6454717PMC
April 2019
5 Reads

Increase expression of CD177 in Kawasaki disease.

Pediatr Rheumatol Online J 2019 Apr 3;17(1):13. Epub 2019 Apr 3.

Department of Pediatrics, Kaohsiung Chang Gung Memorial Hospital and Chang Gung University College of Medicine, #123 Da-Pei Road, Niaosong District, Kaohsiung, 83301, Taiwan.

Background: Kawasaki disease (KD) is the most common acute coronary vasculitis disease to occur in children. Its incidence has been attributed to the combined effects of infection, genetics, and immunity. Although the etiopathogenesis of KD remains unknown, we have performed a survey of global genetic DNA methylation status and transcripts expression in KD patients in order to determine their contribution to the pathogenesis of KD. Read More

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https://ped-rheum.biomedcentral.com/articles/10.1186/s12969-
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http://dx.doi.org/10.1186/s12969-019-0315-8DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6446352PMC
April 2019
3 Reads

Screening of asymptomatic rheumatic heart disease among refugee/migrant children and youths in Italy.

Pediatr Rheumatol Online J 2019 Apr 2;17(1):12. Epub 2019 Apr 2.

Médecins Sans Frontières-Operational Centre Brussels, Italy Mission, Rome, Italy.

Background: Rheumatic heart disease (RHD) is a chronic condition responsible of congestive heart failure, stroke and arrhythmia. Almost eradicated in high-income countries (HIC), it persists in low- and middle-income countries. The purpose of the study was to assess the feasibility and meaningfulness of ultrasound-based RHD screening among the population of unaccompanied foreign minors in Italy and determine the burden of asymptomatic RHD among this discrete population. Read More

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https://ped-rheum.biomedcentral.com/articles/10.1186/s12969-
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http://dx.doi.org/10.1186/s12969-019-0314-9DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6444853PMC
April 2019
3 Reads

Efficacy and cost savings with the use of a minimal sedation / anxiolysis protocol for intra-articular corticosteroid injections in children with juvenile idiopathic arthritis: a retrospective review of prospectively collected data.

Pediatr Rheumatol Online J 2019 Mar 20;17(1):11. Epub 2019 Mar 20.

Department of Pediatric Anesthesiology, UPMC Children's Hospital of Pittsburgh, 4401 Penn Avenue Pittsburgh, Pittsburgh, PA, 15224, USA.

Background: Intra-articular corticosteroid injections (IACI) are frequently used in the treatment of juvenile idiopathic arthritis. There is a paucity of evidence-based research describing methods of pain and anxiety control for this procedure. IACI were mostly performed under general anesthesia for children younger than 13 years old in our institution as of 2014. Read More

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http://dx.doi.org/10.1186/s12969-019-0312-yDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6425704PMC
March 2019
2 Reads

Different histological classifications for Henoch-Schönlein purpura nephritis: which one should be used?

Pediatr Rheumatol Online J 2019 Feb 28;17(1):10. Epub 2019 Feb 28.

Department of Pediatrics, Hacettepe University, Ankara, Turkey.

Background: Nephritis is the most important chronic complication of IgA Vasculitis (IgAV)/Henoch-Schönlein purpura (IGAV/HSP) and thus the main prognostic factor of this most common childhood vasculitis. Since the prognosis and treatment selection depends on the mode of interpretation of biopsy material, in this manuscript we have presented several issues related to the uneven application of different histological classifications in IgAV/Henoch-Schönlein purpura nephritis (HSPN). The nephritis of IgAV/IGAV/HSP will be abbreviated as HSPN for this paper. Read More

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http://dx.doi.org/10.1186/s12969-019-0311-zDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6393980PMC
February 2019
1 Read

Pediatric anti-NMDA receptor encephalitis with catatonia: treatment with electroconvulsive therapy.

Pediatr Rheumatol Online J 2019 Feb 18;17(1). Epub 2019 Feb 18.

Section of Pediatric Rheumatology, Department of Pediatrics, University of Chicago Medical Center, 5841 S. Maryland Avenue, MC 5044, Chicago, 60637, IL, USA.

Background: Anti-NMDA receptor encephalitis, an autoimmune disease associated with antibodies against N-methyl-D-aspartate (NMDA) receptors, is being diagnosed more frequently, especially in children and young adults. Acute neurological and psychiatric manifestations are the common presenting symptoms. Diagnosing anti-NMDA receptor encephalitis is often challenging given the wide range of clinical presentation, and may be further complicated by its overlap of symptoms, brain MRI changes, and CSF findings with other entities affecting the brain. Read More

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http://dx.doi.org/10.1186/s12969-019-0310-0DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6378721PMC
February 2019
3 Reads

Development and pilot testing of the treatment and education approach for childhood-onset lupus (TEACH): a cognitive behavioral treatment.

Pediatr Rheumatol Online J 2019 Feb 18;17(1). Epub 2019 Feb 18.

University of Cincinnati College of Medicine, Cincinnati Children's Hospital Medical Center, 3333 Burnet Ave, Cincinnati, OH, 45229, USA.

Background: To develop and test the feasibility and initial effectiveness of the Treatment and Education Approach for Childhood-onset Lupus (TEACH) protocol, a 6-session cognitive behavioral therapy (CBT) intervention for adolescents and young adults (AYA) with childhood-onset systemic lupus erythematosus (cSLE).

Methods: Females with cSLE (n = 14; ages 13-19 years, M = 16.21 years) presenting to a pediatric rheumatology clinic subsequently completed the protocol, which was iteratively modified based on participant/interventionist feedback. Read More

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http://dx.doi.org/10.1186/s12969-019-0307-8DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6380024PMC
February 2019
4 Reads

Calm in the midst of cytokine storm: a collaborative approach to the diagnosis and treatment of hemophagocytic lymphohistiocytosis and macrophage activation syndrome.

Pediatr Rheumatol Online J 2019 Feb 14;17(1). Epub 2019 Feb 14.

Division of Immunolgy, Boston Children's Hospital, Boston, MA, USA.

Background: Hemophagocytic lymphohistiocytosis (HLH) and macrophage activation syndrome (MAS) were historically thought to be distinct entities, often managed in isolation. In fact, these conditions are closely related. A collaborative approach, which incorporates expertise from subspecialties that previously treated HLH/MAS independently, is needed. Read More

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https://ped-rheum.biomedcentral.com/articles/10.1186/s12969-
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http://dx.doi.org/10.1186/s12969-019-0309-6DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6376762PMC
February 2019
7 Reads

Participation in school sports among children and adolescents with juvenile idiopathic arthritis in the German National Paediatric Rheumatologic Database, 2000-2015: results from a prospective observational cohort study.

Pediatr Rheumatol Online J 2019 Feb 11;17(1). Epub 2019 Feb 11.

Epidemiology Unit, German Rheumatism Research Centre Berlin and Department of Rheumatology and Clinical Immunology, Charité University Medicine Berlin, Chariteplatz 1, 10117, Berlin, Germany.

Background: Regular school sports can help adolescents achieve the recommended amount of daily physical activity and provide knowledge, attitudes and behavioral skills that are needed in order to adopt and maintain a physically active lifestyle. Furthermore, it reaches all children including those that are at risk for engaging in more sedentary types of behavior. Since adolescents with juvenile idiopathic arthritis (JIA) are less involved in physical and social activities than their healthy peers, the objectives were to (1) estimate the prevalence of participation in school sports among patients with JIA; (2) determine the correlates associated with school sports absenteeism; and (3) investigate whether attendance in school sports has changed in the era of biologics. Read More

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http://dx.doi.org/10.1186/s12969-019-0306-9DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6371582PMC
February 2019

The pediatric rheumatology objective structured clinical examination: progressing from a homegrown effort toward a reliable and valid national formative assessment.

Pediatr Rheumatol Online J 2019 Feb 8;17(1). Epub 2019 Feb 8.

Division of Rheumatology, Perelman School of Medicine, University of Pennsylvania, Children's Hospital of Philadelphia, 3401 Civic Center Blvd., Philadelphia, PA, 19104, USA.

Background: Of 37 pediatric rheumatology fellowship training programs in the United States, many have three or fewer fellows at a given time, making large-scale assessment of fellow performance difficult. An objective structured clinical examination (OSCE) is a scenario-based simulation method that assesses individual performance, thus indirectly measuring training program effectiveness. This study describes the development and implementation of two national pediatric rheumatology OSCEs and methods used for programmatic improvement. Read More

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http://dx.doi.org/10.1186/s12969-019-0308-7DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6367759PMC
February 2019
1 Read

The effects of TNF-alpha inhibitor therapy on the incidence of infection in JIA children: a meta-analysis.

Pediatr Rheumatol Online J 2019 Jan 18;17(1). Epub 2019 Jan 18.

Department of Paediatrics, Medical School, University of Pécs, 7. József Attila street, Pécs, 7623, Hungary.

Background: Juvenile Idiopathic arthritis (JIA) is the most common chronic rheumatic disease in childhood. The diagnosis is based on the underlying symptoms of arthritis with an exclusion of other diseases Biologic agents are increasingly used on the side of disease-modifying anti-rheumatic drugs (DMARD) in JIA treatment.

Main Body: The aim of this meta-analysis was to investigate the observed infections in JIA children during tumor necrosis factor (TNF)-alpha inhibitor therapy. Read More

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http://dx.doi.org/10.1186/s12969-019-0305-xDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6339290PMC
January 2019
7 Reads

Coronary artery dilation associated with anti-synthetase syndrome in an adolescent.

Pediatr Rheumatol Online J 2019 Jan 10;17(1). Epub 2019 Jan 10.

Department of Pediatrics, University of Texas McGovern Medical School, 6431 Fannin St. MSB 3.228, Houston, TX, USA.

Background: Idiopathic inflammatory myopathies (IIM) are a group of systemic autoimmune disorders primarily affecting skeletal muscle. Pediatric coronary artery dilation is frequently discussed in Kawasaki disease. However, it has yet to be reported in the IIMs or antisynthetase syndrome. Read More

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https://ped-rheum.biomedcentral.com/articles/10.1186/s12969-
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http://dx.doi.org/10.1186/s12969-019-0304-yDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6329119PMC
January 2019
10 Reads

The effectiveness of a multidisciplinary intervention strategy for the treatment of symptomatic joint hypermobility in childhood: a randomised, single Centre parallel group trial (The Bendy Study).

Pediatr Rheumatol Online J 2019 Jan 8;17(1). Epub 2019 Jan 8.

Norfolk and Norwich University Hospital NHS Trust, Colney Lane, Norwich, UK.

Introduction: Joint hypermobility is common in childhood and can be associated with musculoskeletal pain and dysfunction. Current management is delivered by a multidisciplinary team, but evidence of effectiveness is limited. This clinical trial aimed to determine whether a structured multidisciplinary, multisite intervention resulted in improved clinical outcomes compared with standard care. Read More

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https://ped-rheum.biomedcentral.com/articles/10.1186/s12969-
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http://dx.doi.org/10.1186/s12969-018-0298-xDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6325876PMC
January 2019
14 Reads

Kawasaki disease shock syndrome: clinical characteristics and possible use of IL-6, IL-10 and IFN-γ as biomarkers for early recognition.

Pediatr Rheumatol Online J 2019 Jan 5;17(1). Epub 2019 Jan 5.

Department of Rheumatology Immunology and Allergy, Children's Hospital, Zhejiang University School of Medicine, No.57, Zhugan Lane, Yan-an Road, Hangzhou, 310003, China.

Background: As an acute febrile and inflammatory disease, Kawasaki disease (KD) could develop Kawasaki disease shock syndrome (KDSS) sometimes. However its pathogenesis was still not well known. This study was to learn more about the clinical features and evaluate the role of cytokines in the pathogenesis of KDSS. Read More

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http://dx.doi.org/10.1186/s12969-018-0303-4DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6321686PMC
January 2019
7 Reads

Clinical phenotypes and biologic treatment use in juvenile dermatomyositis-associated calcinosis.

Pediatr Rheumatol Online J 2018 Dec 29;16(1):84. Epub 2018 Dec 29.

Division of Pediatric Rheumatology, Department of Pediatrics, St. Louis, MO, USA.

Background: Few risk factors have been identified for the development of calcinosis among patients with Juvenile Dermatomyositis, and currently no clinical phenotype has been associated with its development. We analyzed a large database of patients to further elucidate any relationships among patients with and without calcinosis.

Method: The CARRA legacy registry recruited pediatric rheumatology patients from 55 centers across North America from 2010 through 2014, including over 650 subjects with Juvenile Dermatomyositis. Read More

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http://dx.doi.org/10.1186/s12969-018-0299-9DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6311016PMC
December 2018
3 Reads

Arterial properties in adults with long-lasting active juvenile idiopathic arthritis compared to healthy controls.

Pediatr Rheumatol Online J 2018 Dec 29;16(1):85. Epub 2018 Dec 29.

Department of Circulation and Imaging, Faculty of Medicine and Health Science, Norwegian University of Science and Technology, NTNU, Trondheim, Norway.

Background: The data on cardiovascular risk and systemic arterial properties in patients with long-lasting juvenile idiopathic arthritis (JIA) is limited. The objective of this study was to describe systemic arterial properties including characteristic impedance (Z), total arterial compliance (C), and peripheral vascular resistance (R) in patients with long-lasting active JIA compared with matched controls, and to assess the relation to JIA disease variables and traditional cardiovascular risk factors.

Findings: Methods: Eighty-one JIA patients (median age 38. Read More

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http://dx.doi.org/10.1186/s12969-018-0302-5DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6310961PMC
December 2018

Coronary artery status of patients with transient fever 24-36 h after first IVIG infusion did not differ from that seen in responsive patients.

Pediatr Rheumatol Online J 2018 Dec 29;16(1):83. Epub 2018 Dec 29.

Department of Pediatrics, University of Ulsan College of Medicine, Seoul, South Korea.

Background: Current management guidelines for patients with Kawasaki disease (KD) differ in their recommendations for fever observation times when determining resistance to initial intravenous immunoglobulin (IVIG). This retrospective study assessed coronary artery status in patients with transient fever 24-36 h after the completion of a first IVIG infusion.

Methods: Children with KD treated with IVIG between January 2006 and February 2017 were included. Read More

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http://dx.doi.org/10.1186/s12969-018-0301-6DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6311065PMC
December 2018
6 Reads

Body composition and phase angle as an indicator of nutritional status in children with juvenile idiopathic arthritis.

Pediatr Rheumatol Online J 2018 Dec 27;16(1):82. Epub 2018 Dec 27.

Institute of Nursing and Health Sciences, Faculty of Medicine, University of Rzeszów, Al. mjr. W. Kopisto 2 a, 35-310, Rzeszów, Poland.

Background: Juvenile idiopathic arthritis (JIA) is the most common chronic, systemic autoimmune connective tissue disease diagnosed in children and adolescents. An important aspect of monitoring of children with JIA is a precise assessment of the nutritional status to identify children and adolescents at risk of malnutrition. The aim of the study was to assess the body composition and phase angle in children diagnosed with JIA in comparison to age and sex matched healthy children since there are scarce reports in paediatric patients. Read More

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http://dx.doi.org/10.1186/s12969-018-0297-yDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6307257PMC
December 2018
1 Read

Living with autoinflammatory diseases: identifying unmet needs of children, adolescents and adults.

Pediatr Rheumatol Online J 2018 Dec 20;16(1):81. Epub 2018 Dec 20.

Rheumatology, Department of Pediatrics and autoinflammation reference center Tuebingen, University Children's Hospital Tuebingen, Tuebingen, Germany.

Background: Autoinflammatory diseases (AIDs) illnesses of the innate immunity resulting in clinical signs and symptoms of systemic inflammation and loss of organ functions. While pathophysiological mechanisms are heavily studied and increasingly well understood, psychosocial needs are much less explored. The disease impact on the everyday life of patients including school and work is poorly studied. Read More

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http://dx.doi.org/10.1186/s12969-018-0300-7DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6302479PMC
December 2018
2 Reads

Multi-centre national audit of juvenile localised scleroderma: describing current UK practice in disease assessment and management.

Pediatr Rheumatol Online J 2018 Dec 18;16(1):80. Epub 2018 Dec 18.

Department of Paediatric Rheumatology, Alder Hey Children's NHS Foundation Trust, Eaton Road, Liverpool, L12 2AP, UK.

Objective: To describe current United Kingdom practice in assessment and management of patients with juvenile localised scleroderma (JLS) compared to Paediatric Rheumatology European Society (PRES) scleroderma working party recommendations.

Methods: Patients were included if they were diagnosed with JLS and were under the care of paediatric rheumatology between 04/2015-04/2016. Retrospective data was collected in eleven UK centres using a standardised proforma and collated centrally. Read More

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http://dx.doi.org/10.1186/s12969-018-0295-0DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6299547PMC
December 2018
5 Reads

Off-label use of tocilizumab to treat non-juvenile idiopathic arthritis in pediatric rheumatic patients: a literature review.

Pediatr Rheumatol Online J 2018 Dec 14;16(1):79. Epub 2018 Dec 14.

Department of Rheumatology, Ajou University of medical school, 164 Worldcup-ro, Yeongtong-gu, Suwon, 16499, Republic of Korea.

Tocilizumab, an anti-interleukin-6 (IL-6) agent, is indicated as a treatment for several autoimmune or inflammatory diseases, including rheumatoid arthritis and juvenile idiopathic arthritis (JIA). IL-6 plays roles in both immune system dysregulation and inflammation, and thus efforts to extend the utility of tocilizumab in patients with autoinflammatory conditions are ongoing. Here, we survey the literature on the off-label use of tocilizumab in patients with juvenile-onset rheumatic diseases including juvenile systemic lupus erythematosus (SLE), juvenile dermatomyositis (DM), vasculitis, juvenile scleroderma, and other autoinflammatory diseases. Read More

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https://ped-rheum.biomedcentral.com/articles/10.1186/s12969-
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http://dx.doi.org/10.1186/s12969-018-0296-zDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6295005PMC
December 2018
8 Reads

Disease burden and social impact of pediatric chronic nonbacterial osteomyelitis from the patient and family perspective.

Pediatr Rheumatol Online J 2018 Dec 14;16(1):78. Epub 2018 Dec 14.

Pediatric Rheumatology, Seattle Children's Hospital, University of Washington, Seattle, WA, USA.

Background: Chronic nonbacterial osteomyelitis (CNO) is an autoinflammatory bone disorder that if left untreated can result in bone destruction and severe continuing pain due to persistent inflammation. The impact this chronic disease has on the daily lives of affected children and their families is not well known. The purpose of this study is to understand the disease burden and socioeconomic and psychological impact of CNO from the patients' and families' perspectives and identify areas of improvement for patient care and reduced disease burden based on patients' and families' responses. Read More

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http://dx.doi.org/10.1186/s12969-018-0294-1DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6295016PMC
December 2018
1 Read

Educational initiatives and training for paediatric rheumatology in Europe.

Pediatr Rheumatol Online J 2018 Dec 7;16(1):77. Epub 2018 Dec 7.

Department of Paediatrics, University Medical Center Utrecht, Utrecht University, Utrecht, Netherlands.

The Paediatric Rheumatology European Society (PReS) has over many years, developed a portfolio of educational activities to address increasing educational needs of workforce and support young clinicians to acquire skills to develop new knowledge and deliver clinical care in the future. These educational activities aim to facilitate growth of paediatric rheumatology and ultimately improve the clinical care for children and families. This article describes the current portfolio of PReS educational activities and their relevance to the international paediatric rheumatology community. Read More

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https://ped-rheum.biomedcentral.com/articles/10.1186/s12969-
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http://dx.doi.org/10.1186/s12969-018-0289-yDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6286498PMC
December 2018
3 Reads

Protective parents and permissive children: what qualitative interviews with parents and children can tell us about the feasibility of juvenile idiopathic arthritis trials.

Pediatr Rheumatol Online J 2018 Dec 4;16(1):76. Epub 2018 Dec 4.

Department of Psychological Sciences, University of Liverpool, Whelan Building, Brownlow Hill, Liverpool, L69 3GB, UK.

Background: Patient recruitment can be very challenging in paediatric studies, especially in relatively uncommon conditions, such as juvenile idiopathic arthritis (JIA). However, involving children and young people (CYP) in the design of such trials could promise a more rapid trajectory towards making evidence-based treatments available. Studies involving CYP are advocated in the literature but we are not aware of any early stage feasibility studies that have qualitatively accessed the perspectives of parents and CYP with a long term condition to inform design and conduct of a trial. Read More

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http://dx.doi.org/10.1186/s12969-018-0293-2DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6278083PMC
December 2018
3 Reads

Feasibility of a musculoskeletal ultrasound intervention to improve adherence in juvenile idiopathic arthritis: a proof-of concept trial.

Pediatr Rheumatol Online J 2018 Nov 22;16(1):75. Epub 2018 Nov 22.

Cincinnati Children's Hospital Medical Center, Behavioral Medicine and Clinical Psychology, Center for Adherence and Self-Management, 3333 Burnet Ave, MLC 7039, Cincinnati, OH, 45229, USA.

Background: Non-adherence is a prevalent and modifiable issue in juvenile idiopathic arthritis (JIA) that currently lacks provider-based intervention. Education surrounding disease status is one way in which families remain engaged in their care. Musculoskeletal ultrasound is one such form of demonstrative, real-time education that may impact the way patients and caregivers self-manage their disease. Read More

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http://dx.doi.org/10.1186/s12969-018-0292-3DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6251087PMC
November 2018
19 Reads

Chronic non bacterial osteitis- a multicentre study.

Pediatr Rheumatol Online J 2018 Nov 22;16(1):74. Epub 2018 Nov 22.

Departments of Paediatric Rheumatology and Immunology, Bristol Royal Hospital for Children, Bristol, BS2 8BJ, UK.

Objective: To understand the demographics, clinical features and treatment outcomes of Chronic Non-bacterial Osteitis (CNO) from three tertiary paediatric rheumatology services in the United Kingdom.

Methods: Children less than 18 years of age diagnosed with CNO between 2001 to 2016 from one tertiary service and between 2001 to 2017 from two tertiary services were included. Clinical notes were reviewed and all pertinent data were collected on a pre-defined proforma. Read More

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http://dx.doi.org/10.1186/s12969-018-0290-5DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6251121PMC
November 2018
17 Reads

Long-term follow-up of autologous hematopoietic stem cell transplantation for refractory juvenile dermatomyositis: a case-series study.

Pediatr Rheumatol Online J 2018 Nov 20;16(1):72. Epub 2018 Nov 20.

Division of Rheumatology and Immunology, The Affiliated Children's Hospital, Capital Institute of Pediatrics, Beijing, 100020, China.

Objective: To follow up the refractory juvenile dermatomyositis (JDM) with autologous hematopoietic stem cell transplantation (AHSCT) in a long time and to investigate whether AHSCT is effective and safe to treat refractory JDM.

Methods: We collected the AHSCT and follow-up data of three patients with refractory JDM who received autologous peripheral blood CD34+ cell transplantation in our hospital between June 2004 and July 2015. Those data include: hight, weight, routine blood and urine tests, ESR, CK, ALT, AST, LDH, renal functional tests, lymphocyte subpopulations, HRCT and muscle MRI. Read More

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http://dx.doi.org/10.1186/s12969-018-0284-3DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6245778PMC
November 2018
17 Reads

Health-related quality of life in children with inflammatory brain disease.

Pediatr Rheumatol Online J 2018 Nov 20;16(1):73. Epub 2018 Nov 20.

Rheumatology, Department of Pediatrics, Alberta Children's Hospital, 2888 Shaganappi Trail NW, Calgary, AB, T3B 6A8, Canada.

Objective: To quantify the impact of inflammatory brain diseases in the pediatric population on health-related quality of life, including the subdomains of physical, emotional, school and social functioning.

Methods: This was a multicenter, observational cohort study of children (< 18 years of age) diagnosed with inflammatory brain disease (IBrainD). Patients were included if they had completed at least one Health Related Quality of Life Questionnaire (HRQoL). Read More

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http://dx.doi.org/10.1186/s12969-018-0291-4DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6245877PMC
November 2018
20 Reads

Rituximab treatment for chronic steroid-dependent Henoch-Schonlein purpura: 8 cases and a review of the literature.

Pediatr Rheumatol Online J 2018 Nov 14;16(1):71. Epub 2018 Nov 14.

Department of Pediatrics, Division of Rheumatology, University of Alabama at Birmingham, 1600 7th Ave S, CPPN G10, Birmingham, AL, 35233, USA.

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. Read More

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https://ped-rheum.biomedcentral.com/articles/10.1186/s12969-
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http://dx.doi.org/10.1186/s12969-018-0285-2DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6236882PMC
November 2018
36 Reads

Inpatient burden of juvenile dermatomyositis among children in the United States.

Pediatr Rheumatol Online J 2018 Nov 13;16(1):70. Epub 2018 Nov 13.

Division of Rheumatology, Departments of Pediatrics and Medical Social Sciences, Ann & Robert H. Lurie Children's Hospital of Chicago/Northwestern University Feinberg School of Medicine, 225 E Chicago Ave Box 50, Chicago, IL, 60611, USA.

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. Read More

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http://dx.doi.org/10.1186/s12969-018-0286-1DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6234588PMC
November 2018
3 Reads

Juvenile idiopathic arthritis managed in the new millennium: one year outcomes of an inception cohort of Australian children.

Pediatr Rheumatol Online J 2018 Nov 9;16(1):69. Epub 2018 Nov 9.

Department of Rheumatology, The Royal Children's Hospital, 50 Flemington Rd, Parkville, Melbourne, VIC, 3052, Australia.

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. Read More

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https://ped-rheum.biomedcentral.com/articles/10.1186/s12969-
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http://dx.doi.org/10.1186/s12969-018-0288-zDOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6230231PMC
November 2018
27 Reads

Self-management needs of Irish adolescents with Juvenile Idiopathic Arthritis (JIA): how can a Canadian web-based programme meet these needs?

Pediatr Rheumatol Online J 2018 Nov 8;16(1):68. Epub 2018 Nov 8.

The Hospital for Sick Children, Toronto, Canada.

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. Read More

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https://ped-rheum.biomedcentral.com/articles/10.1186/s12969-
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http://dx.doi.org/10.1186/s12969-018-0287-0DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6225653PMC
November 2018
6 Reads

Feasibility and safety of a 6-month exercise program to increase bone and muscle strength in children with juvenile idiopathic arthritis.

Pediatr Rheumatol Online J 2018 Oct 22;16(1):67. Epub 2018 Oct 22.

Division of Rheumatology, K4-123 ACB, British Columbia Children's Hospital, 4480 Oak Street, Vancouver, BC, V6H 3V4, Canada.

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. Read More

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https://ped-rheum.biomedcentral.com/articles/10.1186/s12969-
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http://dx.doi.org/10.1186/s12969-018-0283-4DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6198360PMC
October 2018
6 Reads

A wearable activity tracker intervention for promoting physical activity in adolescents with juvenile idiopathic arthritis: a pilot study.

Pediatr Rheumatol Online J 2018 Oct 22;16(1):66. Epub 2018 Oct 22.

Division of Rheumatology, The Hospital for Sick Children, 555 University Ave, Toronto, ON, M5G 1X8, Canada.

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. Read More

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https://ped-rheum.biomedcentral.com/articles/10.1186/s12969-
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http://dx.doi.org/10.1186/s12969-018-0282-5DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6198499PMC
October 2018
12 Reads

Pilot study comparing the childhood arthritis and rheumatology research alliance consensus treatment plans for induction therapy of juvenile proliferative lupus nephritis.

Pediatr Rheumatol Online J 2018 Oct 22;16(1):65. Epub 2018 Oct 22.

University of California, San Francisco, 550 16th Street, 5th Floor, San Francisco, CA, 94158, USA.

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. The acceptability and in-practice use of these CTPs are unknown. Read More

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https://ped-rheum.biomedcentral.com/articles/10.1186/s12969-
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http://dx.doi.org/10.1186/s12969-018-0279-0DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6196456PMC
October 2018
12 Reads

Physical activity in patients with oligo- and polyarticular juvenile idiopathic arthritis diagnosed in the era of biologics: a controlled cross-sectional study.

Pediatr Rheumatol Online J 2018 Oct 17;16(1):64. Epub 2018 Oct 17.

Department of Rheumatology, Oslo University Hospital, Rikshospitalet, Oslo, Norway.

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.

Methods: The study cohort included 60 patients (30 persistent oligo JIA/30 poly-articular disease) and 60 age- and sex-matched controls. Read More

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http://dx.doi.org/10.1186/s12969-018-0281-6DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6192283PMC
October 2018
3 Reads

Psychological characteristics of caregivers of pediatric patients with chronic rheumatic disease in relation to treatment adherence.

Pediatr Rheumatol Online J 2018 Oct 12;16(1):63. Epub 2018 Oct 12.

Pediatric Rheumatology Unit, Allergy, Immunology and Rheumatology Division, Department of Pediatrics, Universidade Federal de São Paulo/Escola Paulista de Medicina (UNIFESP/EPM), Borges Lagoa Street, 802, Vila Clementino, São Paulo, SP, 04083-001, Brazil.

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. Read More

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http://dx.doi.org/10.1186/s12969-018-0280-7DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6186042PMC
October 2018
1 Read

Improvement of survival rates in the last decade in Thai childhood-onset systemic lupus erythematosus.

Pediatr Rheumatol Online J 2018 Sep 29;16(1):62. Epub 2018 Sep 29.

Department of Pediatrics, Faculty of Medicine, Prince of Songkla University, Hat Yai, Songkhla, 90110, Thailand.

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. Read More

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http://dx.doi.org/10.1186/s12969-018-0274-5DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6162941PMC
September 2018
3 Reads

Successful use of ofatumumab in two cases of early-onset juvenile SLE with thrombocytopenia caused by a mutation in protein kinase C δ.

Pediatr Rheumatol Online J 2018 Sep 26;16(1):61. Epub 2018 Sep 26.

Infection, Inflammation and Rheumatology Section, Infection, Immunity, Inflammation and Physiological Medicine Programme, UCL Institute of Child Health, London, UK.

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. Read More

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http://dx.doi.org/10.1186/s12969-018-0278-1DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6158832PMC
September 2018
6 Reads

Towards a new set of classification criteria for PFAPA syndrome.

Pediatr Rheumatol Online J 2018 Sep 21;16(1):60. Epub 2018 Sep 21.

Unité Romande d'Immuno-rhumatologie Pédiatrique, CHUV, University of Lausanne, Lausanne and HUG, Geneva, Switzerland.

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. Read More

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http://dx.doi.org/10.1186/s12969-018-0277-2DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6151014PMC
September 2018
3 Reads

"I just want to get better": experiences of children and youth with juvenile idiopathic arthritis in a home-based exercise intervention.

Pediatr Rheumatol Online J 2018 Sep 20;16(1):59. Epub 2018 Sep 20.

University of British Columbia, Vancouver, Canada.

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. Read More

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http://dx.doi.org/10.1186/s12969-018-0273-6DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6148995PMC
September 2018
1 Read

Reliability of ultrasonography to detect inflammatory lesions and structural damage in juvenile idiopathic arthritis.

Pediatr Rheumatol Online J 2018 Sep 17;16(1):58. Epub 2018 Sep 17.

Laboratorio de ultrasonido musculoesquelético y articular, Instituto Nacional de Rehabilitación, Luis Guillermo Ibarra Ibarra, Calzada México-Xochimilco 289, Arenal de Guadalupe, Tlalpan, 14389, Mexico city, Mexico.

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. Read More

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http://dx.doi.org/10.1186/s12969-018-0275-4DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6142317PMC
September 2018
4 Reads

Dutch juvenile idiopathic arthritis patients, carers and clinicians create a research agenda together following the James Lind Alliance method: a study protocol.

Pediatr Rheumatol Online J 2018 Sep 15;16(1):57. Epub 2018 Sep 15.

Pediatric Rheumatology and Immunology, Wilhelmina Children's Hospital, University Medical Center Utrecht, Utrecht, Netherlands.

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. Read More

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http://dx.doi.org/10.1186/s12969-018-0276-3DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6139167PMC
September 2018
9 Reads

Risk factors for damage in childhood-onset systemic lupus erythematosus in Asians: a case control study.

Pediatr Rheumatol Online J 2018 Sep 10;16(1):56. Epub 2018 Sep 10.

Department of Paediatrics, Queen Elizabeth Hospital, 30 Gascoigne Road, Kowloon, Hong Kong, Special Administrative Region of China.

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. Read More

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http://dx.doi.org/10.1186/s12969-018-0271-8DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6131800PMC
September 2018
2 Reads

Progressive pseudorheumatoid dysplasia with new-found gene mutation of Wntl inducible signaling pathway protein 3.

Pediatr Rheumatol Online J 2018 Sep 10;16(1):55. Epub 2018 Sep 10.

Department of Rheumatology, Hainan Branch of Chinese People's Liberation Army General Hospital, Haitang Bay, Sanya, China.

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). Read More

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http://dx.doi.org/10.1186/s12969-018-0272-7DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6131911PMC
September 2018
4 Reads

A case of Henoch-Schonlein Purpura with dilated coronary arteries.

Pediatr Rheumatol Online J 2018 Sep 4;16(1):54. Epub 2018 Sep 4.

Department of Pediatric Rheumatology, Children's Hospital Colorado, 13123 East 16th Avenue, B311, Aurora, CO, 80045, USA.

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. Read More

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https://ped-rheum.biomedcentral.com/articles/10.1186/s12969-
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http://dx.doi.org/10.1186/s12969-018-0270-9DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6123917PMC
September 2018
23 Reads

Correction to: Health-related quality of life in girls and boys with juvenile idiopathic arthritis: self- and parental reports in a cross-sectional study.

Pediatr Rheumatol Online J 2018 08 22;16(1):53. Epub 2018 Aug 22.

Department of Public health and Clinical medicine, Epidemiology and Global health, Umeå University, Umeå, Sweden.

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. Read More

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http://dx.doi.org/10.1186/s12969-018-0269-2DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6106743PMC
August 2018
1.622 Impact Factor

Outcomes of non-infectious Paediatric uveitis in the era of biologic therapy.

Pediatr Rheumatol Online J 2018 Aug 6;16(1):51. Epub 2018 Aug 6.

University Hospitals Bristol NHS Foundation Trust, Bristol, UK.

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. Read More

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http://dx.doi.org/10.1186/s12969-018-0266-5DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6080499PMC
August 2018
21 Reads

Successful implementation of a clinical transition pathway for adolescents with juvenile-onset rheumatic and musculoskeletal diseases.

Pediatr Rheumatol Online J 2018 Aug 3;16(1):50. Epub 2018 Aug 3.

Department of Rheumatology, Erasmus University Medical Centre, Postal box 2040, 3000, CA, Rotterdam, the Netherlands.

Background: In 2008 a clinical transition pathway for young people with juvenile-onset rheumatic and musculoskeletal diseases (jRMD) aiming at improving transitional care was instituted. Historical data on drop-out rate in our clinic was 35%, one year before the implementation of the transition pathway. This study aims to I) evaluate the effectiveness of the clinical transition pathway, II) evaluate the experiences and satisfaction of YP with the transitional process and evaluate their perceived self-management skills. Read More

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https://ped-rheum.biomedcentral.com/articles/10.1186/s12969-
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http://dx.doi.org/10.1186/s12969-018-0268-3DOI Listing
http://www.ncbi.nlm.nih.gov/pmc/articles/PMC6091100PMC
August 2018
10 Reads