Pediatr Rheumatol
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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 sleep problems in children with JIA. The mechanism(s) of the relationships characterizing the development or exacerbation of sleep problems in children with JIA are still unknown, however studies have reported an association. The purpose of this study was to synthesize existing research related to sleep problems in children with JIA. ⋯ This review supports an association between poor sleep and increased symptoms related to JIA, specifically the experience of pain. However, results need to be interpreted cautiously given the inconsistent findings regarding factors associated with sleep problems in JIA, the limited evidence available, and its low quality. Furthermore it is not yet determined if the poor sleep patterns predate the symptoms reported with JIA. More research is vital to understanding the factors that predict or perpetuate poor sleep in children and adolescents diagnosed with JIA.
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A reduction of ADA2 activity due to autosomal recessive loss of function mutations in CECR1 results in a newly described vasculopathic phenotype reminiscent of polyarteritis nodosa, with manifestations ranging from fatal systemic vasculitis with multiple strokes in children to limited cutaneous disease in middle-aged individuals. Evidence indicates that ADA2 is essential for the endothelial integrity of small vessels. However, CECR1 is not expressed, nor is the ADA2 protein detectable, in cultured human endothelial cells, thus implicating additional cell types or circulating factors in disease pathogenesis. ⋯ We hypothesise that ADA2 may act as a regulator of neutrophil activation, and that a reduction of ADA2 activity results in significant endothelial damage via a neutrophil-driven process.
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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 (HRQOL) and parental perceptions of child vulnerability (PPCV) and associated variables in parents of a child with JIA. ⋯ The HRQOL of JIA parents was comparable to the HRQOL of parents of a healthy child. JIA parents of a child with active arthritis showed worse HRQOL than parents of a child without active arthritis. Parents perceived their child with JIA as vulnerable.
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Observational Study
Procedural pain and patient-reported side effects with weekly injections of subcutaneous methotrexate in children with rheumatic disorders.
Despite the widespread use of subcutaneous methotrexate in treating pediatric rheumatic disorders, the amount of pain associated with the injections has not been quantified. Our study aims 1) to quantify the amount of pain associated with subcutaneous injections of methotrexate, 2) to explore predictors of pain, 3) to determine the frequency of patient-reported clinical adverse effects of methotrexate, and 4) identify coping strategies of patients and caregivers. ⋯ Subcutaneous injections of methotrexate are associated with a mild amount of pain. Presence of side effects may amplify the amount of perceived pain. Clinicians can apply this knowledge when counseling patients and family members about methotrexate therapy.
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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 Board of Pediatrics (ABP). Programs have been evaluated every 5 years by the ACGME to remain credentialed and graduates had to pass a written exam to be certified. There has been no report yet that details not just what training fellows should receive in the 32 US PR training programs but what training the trainees are actually receiving. ⋯ Most US PR training programs as a whole provide an appropriate training by current ACGME, American College of Rheumatology (ACR), and ABP standards in: 1) number of continuity clinics; 2) sufficient on-call activities for weekday nights and weekends; 3) joint interdisciplinary conferences; 4) electives 5) didactic activities; 6) scholarly activities; and 7) exposure to diverse rheumatology diseases. Areas of concern were uniformity & standardization of training, need for a customized PR training curriculum, more mentorship, free electives, training in musculoskeletal ultrasound, need for a hands-on OSCE certification exam and more exposure to ACGME competencies.