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Recent items - Pediatric Rheumatology

Predictors of response to methotrexate in juvenile idiopathic arthritis
13/08/2014 12:04
Background: The response to methotrexate so far is unpredictable in patients with juvenile idiopathic arthritis. Thus such predictors have to be determined in a large patient cohort. Methods: Demographic, clinical, articular and laboratory variables
Health related quality of life and parental perceptions of child vulnerability among parents of a child with juvenile idiopathic arthritis: results from a web-based survey
07/08/2014 06:00
Background: A chronic illness, such as Juvenile Idiopathic Arthritis (JIA), has an impact on the whole family, especially on parents caring for the ill child. Therefore the aim of this study is to evaluate parental Health Related Quality of Life (HRQ
Blau Syndrome, the prototypic auto-inflammatory granulomatous disease
06/08/2014 04:08
Blau syndrome is a monogenic disease resulting from mutations in the pattern recognition receptor NOD2, and is phenotypically characterized by the triad of granulomatous polyarthritis, dermatitis and uveitis. This paper reviews briefly the classical
Assessment of musculoskeletal abnormalities in children with mucopolysaccharidoses using pGALS
02/08/2014 02:13
Background: Children with mucopolysaccharidoses (MPS) often have musculoskeletal (MSK) abnormalities. Paediatric Gait, Arms, Legs, and Spine (pGALS), is a simple MSK assessment validated in school-age children to detect abnormal joints. We aimed to i
Developing a provisional, international Minimal Dataset for Juvenile Dermatomyositis: for use in clinical practice to inform research
21/07/2014 18:09
Background: Juvenile dermatomyositis (JDM) is a rare but severe autoimmune inflammatory myositis of childhood. International collaboration is essential in order to undertake clinical trials, understand the disease and improve long-term outcome. The a
Deregulation of the IL-1beta axis in chronic recurrent multifocal osteomyelitis
18/07/2014 00:28
Background: This study aims to investigate the inflammasome response in peripheral blood mononuclear cells (PBMCs) and the expression of inflammasome components in bone biopsies from patients with chronic recurrent multifocal osteomyelitis (CRMO). Me
Safety of celecoxib and nonselective nonsteroidal anti-inflammatory drugs in juvenile idiopathic arthritis: results of the phase 4 registry
16/07/2014 23:19
Background: This study aimed to assess long-term safety and developmental data on juvenile idiopathic arthritis (JIA) patients treated in routine clinical practice with celecoxib or nonselective nonsteroidal anti-inflammatory drugs (nsNSAIDs). Method
Development of positive antinuclear antibodies and rheumatoid factor in systemic juvenile idiopathic arthritis points toward an autoimmune phenotype later in the disease course
16/07/2014 18:00
Background: Systemic juvenile idiopathic arthritis (sJIA) is commonly considered an autoinflammatory disease. However, sJIA patients may develop aggressive arthritis without systemic inflammation later in the disease, resembling an autoimmune phenoty
A cross-sectional electromyography assessment in linear scleroderma patients
12/07/2014 18:39
Background: Muscle atrophy and asymmetric extremity growth is a common feature of linear scleroderma (LS). Extra-cutaneous features are also common and primary neurologic involvement, with sympathetic dysfunction, may have a pathogenic role in subcut
High dose anakinra for treatment of severe neonatal Kawasaki disease: a case report
11/07/2014 23:25
We report an 11-week-old female who presented with Kawasaki disease (KD) complicated by macrophage activation syndrome (MAS). The infant presented to the hospital with persistent fever, cough, diarrhea, and emesis, among other symptoms. Her condition
Magnetic resonance imaging findings in juvenile spondyloarthropathy and effects of treatment observed on subsequent imaging
11/07/2014 10:08
Background: Magnetic resonance imaging (MRI) is often used to diagnose and monitor treatment effects of juvenile spondyloarthropathy (SpA). Our objective was to describe MRI findings in juvenile SpA and determine predictors of active sacroiliitis and
A survey of steroid-related osteoporosis diagnosis, prevention and treatment practices of pediatric rheumatologists in North America
09/07/2014 05:50
Background: The purpose of our study is to assess practices of North American pediatric rheumatologists regarding monitoring, prevention, and treatment of low bone mineral density (BMD) in children on long-term glucocorticoid treatment.Long-term gluc
The clinical effectiveness of intra-articular corticosteroids for arthritis of the lower limb in juvenile idiopathic arthritis: a systematic review
11/06/2014 23:51
Background: Juvenile Idiopathic Arthritis (JIA) commonly affects joints of the lower limb including the knee, ankle, subtalar and other foot joints. Intra-articular corticosteroid injections (IACIs) are considered to be effective for short-term relie
Anti-type II collagen antibodies, anti-CCP, IgA RF and IgM RF are associated with joint damage, assessed eight years after onset of juvenile idiopathic arthritis (JIA)
11/06/2014 16:16
Background: Early appearance of antibodies specific for native human type II collagen (anti-CII) characterizes an early inflammatory and destructive phenotype in adults with rheumatoid arthritis (RA). The objective of this study was to investigate th
Takayasu Arteritis in the pediatric population: a contemporary United States-Based Single Center Cohort
04/06/2014 19:37
Background: Takayasu Arteritis is an idiopathic, chronic, large vessel vasculitis involving the aorta and its primary branches. Few studies have been done in pediatric patients to date with the largest case series of US patients published in 2003 con
The role of sleep in Juvenile idiopathic arthritis patients and their caregivers
03/06/2014 22:17
Juvenile idiopathic arthritis is a chronic disease that may lead to various consequences for patients and caregivers, especially in relation to sleep quality. Sleep is an essential process for homeostasis of the organism. In general, caregivers of ch
Sleep problems and associated factors in children with juvenile idiopathic arthritis: a systematic review
02/06/2014 19:40
Background: Sleep problems are common among children with chronic illnesses such as Juvenile Idiopathic Arthritis (or JIA). However, little is known about the frequency and severity of sleep disturbance(s) and the factors that are associated with sle
Clinical features of childhood granulomatosis with polyangiitis (wegener's granulomatosis)
26/05/2014 15:17
Background: Granulomatosis with polyangiitis (GPA), formerly known as Wegener's granulomatosis (WG), belongs to the group of ANCA-associated necrotizing vasculitides. This study describes the clinical picture of the disease in a large cohort of GPA p
Tuberculin skin test and ELISPOT/T. SPOT.TB in children and adolescents with juvenile idiopathic arthritis
23/05/2014 00:04
Background: There are controversies regarding the accuracy of the tuberculin skin test (TST) and methods based on the production of interferon gamma by sensitized T cells for the diagnosis of latent tuberculosis infection (LTBI) in pediatrics and imm
Blood gene expression profiling in pediatric systemic lupus erythematosus and systemic juvenile idiopathic arthritis: from bench to bedside
08/05/2014 02:25
Blood gene expression profiling has led to major advances in the field of rheumatology over the last few decades. Specifically, DNA microarray technology has been integral in increasing our knowledge of key players in the pathogenesis of some rare pe
Childhood Cogan syndrome with aortitis and anti-neutrophil cytoplasmic antibody -associated glomerulonephritis
25/04/2014 20:58
Cogan syndrome is a systemic disease manifesting interstitial keratitis, sensorineural hearing loss, tinnitus, and rotatory vertigo. Renal complications of this syndrome are very rare. We encountered an adolescent with Cogan syndrome complicated by a
An intra-articular ganglion cyst in a patient with juvenile idiopathic arthritis
24/04/2014 02:04
We report an intra-articular ganglion cyst (IAGC) presenting as knee pain and a mass in a patient with longstanding Juvenile Idiopathic Arthritis (JIA). We could not find a similar case of an IAGC occurring in the knee of JIA patients in the literatu
Treatment of juvenile idiopathic arthritis: a revolution i care
23/04/2014 04:38
A generation ago, children with arthritis faced a lifetime of pain and disability. Today, there are a multitude of treatment options, including a variety of biologics targeting key cytokines and other inflammatory mediators. While non-steroidal anti-
The involvement of the vasa vasorum in the development of vasculitis in animal model of Kawasaki disease
30/03/2014 07:59
Background: Kawasaki Disease (KD) involves a diffuse and systemic vasculitis of unknown etiology that mainly affects infants and children. Although a considerable number of analyses of the clinical, histopathological and molecular biological details
Adolescent obesity, joint pain, and hypermobility
29/03/2014 09:30
Background: Obesity associated with joint pain of the lower extremities is likely due to excessive mechanical load on weight bearing joints. Additional mechanical factors may explain the association between obesity and joint pain.FindingsWe character
Feasibility of an online and a face-to-face version of a self-management program for young adults with a rheumatic disease: experiences of young adults and peer leaders
26/03/2014 04:51
Background: Based on the self-efficacy theory, an online and a face-to-face self-management programs 'Challenge your Arthritis' for young adults with a rheumatic disease have recently been developed. These two courses are led by young peer leaders. T
Cervical spine involvement in patients with juvenile idiopathic arthritis - MRI follow-up study
04/03/2014 22:04
Background: To describe MRI and clinical findings in patients with juvenile idiopathic arthritis with cervical spine involvement at onset and follow-up under therapy. Methods: 13 patients with signs of cervical spine involvement in juvenile idiopathi
Current pediatric rheumatology fellowship training in the United States: what fellows actually do
10/02/2014 08:31
Background: Pediatric Rheumatology (PR) training in the US has existed since the 1970's. In the early 1990's, the training was formalized into a three year training program by the American College of Graduate Medical Education (ACGME) and American Bo
Reviewer Acknowledgement 2013
01/02/2014 02:36
Contributing reviewersThe Editors of Pediatric Rheumatology would like to thank all our reviewers who have contributed to the journal in Volume 11 (2013).
Results of a multinational survey regarding the diagnosis and treatment of temporomandibular joint involvement in juvenile idiopathic arthritis
25/01/2014 11:01
Background: Temporomandibular joint (TMJ) involvement occurs in up to 80% of patients with juvenile idiopathic arthritis (JIA). Currently they are no standardized procedures regarding diagnosis and treatment of this common complication of JIA. The ai
MEFV gene mutations and cardiac phenotype in children with familial Mediterranean fever: a cohort study
17/01/2014 03:54
Background: Familial Mediterranean fever (FMF) is the most common autoinflammatory disorder in the world. It is characterized by recurrent febrile inflammatory attacks of serosal and synovial membranes. MEFV gene mutations are responsible for the dis
The pro-inflammatory potential of T cells in juvenile-onset systemic lupus erythematosus
16/01/2014 17:13
Background: T cells are important to systemic lupus erythematosus (SLE) disease progression. This study determined the pro-inflammatory potential of T cells within the rare condition juvenile-onset SLE (JSLE).MethodIL-17A and Th1/Th2-related cytokine
Prolonged improvement of childhood onset systemic lupus erythematosus following systematic administration of rituximab and cyclophosphamide
15/01/2014 04:02
Background: Although the combination of cyclophosphamide and rituximab has been utilized in case reports, there are no previous reports of the long term outcome of SLE treated systematically with this regimen. We report a pilot study to evaluate the
Juvenile dermatomyositis in South African children is characterised by frequent dystropic calcification: a cross sectional study
07/01/2014 23:18
Background: To describe Juvenile dermatomyositis (JDM) that has rarely been reported in Sub-Saharan Africa in children. Methods: Retrospective record review of children with JDM attending a tertiary hospital in South Africa. Results: Twenty-one child
Gout in a 15-year-old boy with juvenile idiopathic arthritis: a case study
06/01/2014 14:01
Joint pain is a common complaint in pediatrics and is most often attributed to overuse or injury. In the face of persistent, severe, or recurrent symptoms, the differential typically expands to include bony or structural causes versus rheumatologic c
Assessment of disease activity by patients with juvenile idiopathic arthritis and the parents compared to the assessment by pediatric rheumatologists
24/12/2013 19:34
Background: Self assessment of arthritis is important for recognition of disease activity and early initiation of therapy. Proper interpretation of physical symptoms is necessary for this. The purpose was to investigate the assessment by patients and
Autoinflammatory bone disorders with special focus on chronic recurrent multifocal osteomyelitis (CRMO)
23/12/2013 11:15
Sterile bone inflammation is the hallmark of autoinflammatory bone disorders, including chronic nonbacterial osteomyelitis (CNO) with its most severe form chronic recurrent multifocal osteomyelitis (CRMO). Autoinflammatory osteopathies are the result
Safety and feasibility of a home-based six week resistance training program in juvenile idiopathic arthritis
21/12/2013 00:08
Background: Juvenile idiopathic arthritis (JIA), among the most common chronic diseases of childhood, can be associated with attenuated physical activity levels, reduced fitness, decreased functionality and pain. This pilot study aimed to determine t
Profiling risk factors for chronic uveitis in juvenile idiopathic arthritis: a new model for EHR-based research
03/12/2013 22:06
Background: Juvenile idiopathic arthritis is the most common rheumatic disease in children. Chronic uveitis is a common and serious comorbid condition of juvenile idiopathic arthritis, with insidious presentation and potential to cause blindness. Kno
pGALS - paediatric Gait Arms Legs and Spine - A simple examination of the musculoskeletal system
12/11/2013 19:16
We describe pGALS (paediatric Gait, Arms, Legs and Spine) - a simple quick musculoskeletal assessment to distinguish abnormal from normal joints in children and young people. The use of pGALS is aimed at the non-specialist in paediatric musculoskelet
The pediatric rheumatology quality of life scale: validation of the English version in a US cohort of juvenile idiopathic arthritis
08/11/2013 20:05
Background: This study aims to validate the English version of the Pediatric Rheumatology Quality of Life Scale (PRQL), a concise Health Related Quality of Life (HRQoL) measure, in a US cohort of children with juvenile idiopathic arthritis (JIA). Met
Hypergammaglobulinemia in the pediatric population as a marker for underlying autoimmune disease: a retrospective cohort study
01/11/2013 12:23
Background: The significance of hypergammaglobulinemia as a marker of immune activation is unknown, as a differential diagnosis for hypergammaglobulinemia in children has not been adequately established. The goal of this study was to identify conditi
The identification of a novel splicing mutation in C1qB in a Japanese family with C1q deficiency: a case report
28/10/2013 06:23
C1q deficiency is a rare disease that is associated with a high probability of developing systemic lupus erythematosus. We report a 4-year-old Japanese girl who presented with fever, facial erythema, joint pain, and oral ulceration. Complement defici
Meta-analysis confirms association between TNFA-G238A variant and JIA, and between PTPN22-C1858T variant and oligoarticular, RF-polyarticular and RF-positive polyarticular JIA
26/10/2013 03:50
Background: Although more than 100 non-HLA variants have been tested for associations with juvenile idiopathic arthritis (JIA) in candidate gene studies, only a few have been replicated. We sought to replicate reported associations of single nucleoti
Moroccan parents caring for children with juvenile idiopathic arthritis: positive and negative aspects of their experiences
20/10/2013 19:00
Background: Juvenile idiopathic arthritis (JIA) can lead to serious disability in children and adolescents, requiring intensive home care usually provided by parents .These parents must also cope with physical, familial, social and financial constrai
Elevated levels of CXCL10 in the Periodic Fever, Aphthous stomatitis, Pharyngitis and cervical Adenitis syndrome (PFAPA) during and between febrile episodes; an indication of a persistent activation of the innate immune system
18/10/2013 01:10
Background: The Periodic Fever, Aphthous stomatitis, Pharyngitis and cervical Adenitis syndrome (PFAPA) is the most common periodic fever syndrome in childhood. Clinically, PFAPA may resemble autoinflammatory diseases, but the etiology is not fully u
Temporomandibular signs, symptoms, joint alterations and disease activity in juvenile idiopathic arthritis -- an observational study
17/10/2013 20:09
Background: Juvenile idiopathic arthritis (JIA) is a heterogeneous disease that frequently affects also the temporomandibular joint (TMJ) and associated structures. The main aim of this observational study was to describe systematically orofacial cli
Chorea, a little-known manifestation in systemic lupus erythematosus: short literature review and four case reports
17/10/2013 00:02
Chorea is a movement disorder that may be found in children due to several causes. Here we focus especially on Systemic Lupus Erythematosus associated chorea. First we outline its epidemiology, hypothesized pathogenesis, clinical presentation and tre
Malignancies in children and young adults on etanercept: summary of cases from clinical trials and post marketing reports
02/10/2013 06:30
Background: Malignancy risk may be increased in chronic inflammatory conditions that are mediated by tumor necrosis factor (TNF), such as juvenile idiopathic arthritis (JIA), but the role of TNF in human cancer biology is unclear. In response to a 20
Understanding treatment decision making in juvenile idiopathic arthritis: a qualitative assessment
01/10/2013 00:08
Background: The increase in therapeutic options for juvenile idiopathic arthritis (JIA) has added complexity to treatment decisions. Shared decision making has the potential to help providers and families work together to choose the best possible opt
Clinical patterns of juvenile idiopathic arthritis in Zambia
14/09/2013 10:56
Background: Juvenile idiopathic arthritis (JIA) is a heterogeneous group of disorders with different disease manifestations among various populations. There are few reports of JIA among indigenous Africans especially sub-Saharan Africa. We present he
DC-SIGN gene promoter variants and IVIG treatment response in Kawasaki disease
06/09/2013 01:24
Background: Genetic variants in the inhibiting FcgammaRIIB mediate anti-inflammatory responses and influence IVIG refractoriness (IVIG-R). However, these variants are rare in Asian and Hispanic populations so other genes in the pathway could be poten
Evaluation of anti-citrullinated type II collagen and anti-citrullinated vimentin antibodies in patients with juvenile idiopathic arthritis
29/08/2013 22:06
Background: To determine the prevalence and significance of anti-citrullinated vimentin and anti-citrullinated type II collagen antibodies and elucidate their role in the disease process of juvenile idiopathic arthritis (JIA). Methods: Sera were obta
Exercise in children with joint hypermobility syndrome and knee pain: a randomised controlled trial comparing exercise into hypermobile versus neutral knee extension
14/08/2013 03:49
Background: Knee pain in children with Joint Hypermobility Syndrome (JHS) is traditionally managed with exercise, however the supporting evidence for this is scarce. No trial has previously examined whether exercising to neutral or into the hypermobi