Pediatric Rheumatology

http://www.ped-rheum.com/

List of Papers (Total 2,596)

Assessment of transition readiness in adolescents and young adults with chronic health conditions

Transition from pediatric to adult health care is a vulnerable period for adolescents and young adults. Challenges include paucity of validated measures to assess patients’ transition readiness. We evaluated the Transition Readiness Assessment Questionnaire (TRAQ) in adolescents and young adults with rheumatic, gastrointestinal, and endocrine disorders. We examined whether baseline ...

Comics as an educational tool for children with juvenile idiopathic arthritis

This study examined whether the comic book Neta and the Medikidz Explain JIA would improve disease-related knowledge and treatment adherence among patients with juvenile idiopathic arthritis (JIA). In this prospective cohort study, JIA patients answered 20 multiple-choice knowledge questions about their disease, before and after reading the comic book. Demographic, clinical, ...

The spectrum of rheumatic in-patient diagnoses at a pediatric hospital in Kenya

Background Pediatric rheumatic diseases are chronic illnesses that can cause considerable disease burden to children and their families. There is limited epidemiologic data on these diseases in East Africa. The aim of this study was to assess the spectrum of pediatric rheumatic diagnoses in an in-patient setting and determine the accuracy of ICD-10 codes in identifying these ...

Endothelial function in children with a history of henoch schonlein purpura

Background Although Henoch-Schonlein purpura (HSP) is the most common form of systemic vasculitis in children, the long term effect of HSP on endothelial function is still not clear. The aim of our study was to evaluate the long term effect of HSP on endothelial function in children and adolescents. Methods This research was an observational prospective study. The study group ...

Atypical monoarthritis presentation in children with oligoarticular juvenile idiopathic arthritis: a case series

Background Oligoarticular juvenile idiopathic arthritis (oligoJIA), the most common chronic inflammatory arthritis of childhood, usually involves the knees and ankles. Severe oligoJIA monoarthritis presenting in a joint other than knees and ankles, is rare. Findings We report four children who presented with severe isolated arthritis of the hip, wrist or elbow and were diagnosed ...

Childhood Arthritis and Rheumatology Research Alliance consensus clinical treatment plans for juvenile dermatomyositis with skin predominant disease

Background Juvenile dermatomyositis (JDM) is the most common form of the idiopathic inflammatory myopathies in children. A subset of children have the rash of JDM without significant weakness, and the optimal treatments for these children are unknown. The goal of this study was to describe the development of consensus clinical treatment plans (CTPs) for children with JDM who have ...

Disease status, reasons for discontinuation and adverse events in 1038 Italian children with juvenile idiopathic arthritis treated with etanercept

Background Data from routine clinical practice are needed to further define the efficacy and safety of biologic medications in children with juvenile idiopathic arthritis (JIA). The aim of this analysis was to investigate the disease status, reasons for discontinuation and adverse events in Italian JIA patients treated with etanercept (ETN). Methods In 2013, all centers of the ...

Evidence-based decision support for pediatric rheumatology reduces diagnostic errors

Background The number of trained specialists world-wide is insufficient to serve all children with pediatric rheumatologic disorders, even in the countries with robust medical resources. We evaluated the potential of diagnostic decision support software (DDSS) to alleviate this shortage by assessing the ability of such software to improve the diagnostic accuracy of non-specialists. ...

Macrophage activation syndrome triggered by coeliac disease: a unique case report

Background Macrophage activation syndrome is described as a “clinical syndrome of hyperinflammation resulting in an uncontrolled and ineffective immune response” in the context of an autoinflammatory or rheumatic disease. Current associations of macrophage activation syndrome with autoimmune disease most notably include a host of rheumatological conditions and inflammatory bowel ...

Fatigue in patients with Juvenile Idiopathic Arthritis: relationship to perceived health, physical health, self-efficacy, and participation

Background Fatigue is common in patients with JIA and affects daily life negatively. We assessed the presence and severity of fatigue in patients with JIA, including factors presumed associated with fatigue (e.g., disease activity, disability, pain, physical activity, exercise capacity, and self-efficacy), and whether fatigue is related to participation in physical education ...

Monitoring change in volume of calcifications in juvenile idiopathic inflammatory myopathy: a pilot study using low dose computed tomography

Background Dystrophic calcifications may occur in patients with J uvenile Idiopathic Inflammatory Myopathy (JIIM) as well as other connective tissue and metabolic diseases, but a reliable method of measuring the volume of these calcifications has not been established. The purpose of this study is to determine the feasibility of low dose, limited slice, Computed Tomography (CT) to ...

Assessment of left atrial mechanical functions and atrial electromechanical delay in Juvenile idiopathic arthritis by tissue Doppler echocardiography

Background Juvenile idiopathic arthritis (JIA) is a systemic chronic inflammatory disease. Studies using tissue Doppler imaging (TDI) for the evaluation of cardiac functions of children with JIA are limited. Thus, this study was conducted to evaluate Left ventricular function, left atrial mechanical functions and atrial electromechanical delay in JIA. Methods This study was carried ...

Juvenile arthritis caused by a novel FAMIN (LACC1) mutation in two children with systemic and extended oligoarticular course

Background The pathophysiological origin of juvenile idiopathic arthritis (JIA) is largely unknown. However, individuals with presumably pathogenic mutations in FAMIN have been reported, associating this gene with a rare subtype of this disorder. FAMIN, that is formerly also referred to as LACC1 or C13orf31, has recently been shown to play a crucial role in immune-metabolic ...

Characteristics and outcome of children with juvenile dermatomyositis in Cape Town: a cross-sectional study

Background Juvenile dermatomyositis (JDM) is a rare idiopathic inflammatory childhood myopathy of uncertain aetiology. The demographic and clinical presentation of JDM may differ by race and geographic regions. Few studies have described the characteristics of JDM patients from Africa. Methods We conducted a retrospective observational study to determine clinical characteristics ...

Lipoprotein cholesterol fractions are related to markers of inflammation in children and adolescents with juvenile idiopathic arthritis: a cross sectional study

Background The purpose of the study is to determine levels of total cholesterol (TC), low-density, and high-density lipoprotein fractions of cholesterol (LDLc and HDLc), in patients with juvenile idiopathic arthritis (JIA), and relate those to disease activity, overweight, and physical activity (PA), testing the hypothesis that the levels of cholesterol fractions are associated ...

Bone histomorphometric changes in children with rheumatic disorders on chronic glucocorticoids

Background Rheumatic diseases are associated with an increased fracture risk. The tissue level characteristics of the bone involvement in children have not been well elucidated. Our objectives were to describe the bone micro-architectural characteristics in children with rheumatic diseases on chronic glucocorticoids, and to determine associations between micro-architectural ...

Sarcoidosis presenting as granulomatous myositis in a 16-year-old adolescent

Background Sarcoidosis is a multi-system disease characterized by the presence of non-caseating epithelioid granulomas in affected tissues, including skeletal muscle. These organized collections of immune cells have important pathophysiologic action including cytokine production leading to inflammation as well as enzymatic conversion of cholecalciferol to calcitriol via 1-α ...

“Sometimes I feel like a pharmacist”: identity and medication use among adolescents with juvenile arthritis

Background Taking medicines as intended is difficult for everybody, but young people going through adolescence have greater problems than adults and younger children. One of the most important things that happen during the teenage years is the development of individual identities, which might not remain constant during this time and can be affected deeply by the diagnosis of a ...

Arterial dissection in childhood Takayasu Arteritis: not as rare as thought

Background Arterial vessel wall dissection is a rare, life-threatening and rarely described complication in childhood Takayasu Arteritis (cTA). Prevalence and risk factors for arterial dissection in cTA are unknown. We sought to study the prevalence and analyse risk factors for arterial dissection in cTA. Findings A single center retrospective review of all children with cTA was ...

The human microbiome and juvenile idiopathic arthritis

Juvenile idiopathic arthritis (JIA) is the most common rheumatic disease in childhood. The pathogenesis of JIA is thought to be the result of a combination of host genetic and environmental triggers. However, the precise factors that determine one’s susceptibility to JIA remain to be unravelled. The microbiome has received increasing attention as a potential contributing factor to ...

Parents’ information needs and influential factors when making decisions about TNF-α inhibitors

Background Parents struggle when making treatment decisions for children with arthritis or other chronic conditions. Understanding their decision-making process is an essential step towards improving the decision-making experience. The objective of this study was to describe parents’ information needs and the influences on their decision making about treatment with TNF-α ...

Treatment with high-dose recombinant human hyaluronidase-facilitated subcutaneous immune globulins in patients with juvenile dermatomyositis who are intolerant to intravenous immune globulins: a report of 5 cases

Background High-dose intravenous immune globulins (IVIg) are frequently used in refractory juvenile dermatomyositis (JDM) but are often poorly tolerated. High-dose recombinant human hyaluronidase-facilitated subcutaneous immune globulins (fSCIg) allow the administration of much higher doses of immune globulins than conventional subcutaneous immune globulin therapy and may be an ...

Monogenic polyarteritis: the lesson of ADA2 deficiency

The deficiency of Adenosine Deaminase 2 (DADA2) is a new autoinflammatory disease characterised by an early onset vasculopathy with livedoid skin rash associated with systemic manifestations, CNS involvement and mild immunodeficiency. This condition is secondary to autosomal recessive mutations of CECR1 (Cat Eye Syndrome Chromosome Region 1) gene, mapped to chromosome 22q11.1, that ...

Analysis of health care claims during the peri-transfer stage of transition from pediatric to adult care among juvenile idiopathic arthritis patients

Background To investigate the utilization of health care services before and after transfer from pediatric to adult rheumatology care in clinical practice. Methods Using US commercial claims data from January 2005 through August 2012, we identified individuals with a JIA diagnosis code from a pediatric rheumatologist followed by any diagnosis code from an adult rheumatologist. ...

Abnormal body composition, cardiovascular endurance, and muscle strength in pediatric SLE

Background Children with SLE are known to have higher fat mass compared to their peers but there are no published data regarding exercise capacity as measured by cardiovascular endurance and muscle strength. In our pilot study of ten children with SLE, we sought to examine body composition, cardiovascular endurance, and isometric muscle strength. Findings Ten pediatric SLE patients ...