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Pediatric Rheumatology Online Journal

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https://www.readbyqxmd.com/read/27894310/monitoring-change-in-volume-of-calcifications-in-juvenile-idiopathic-inflammatory-myopathy-a-pilot-study-using-low-dose-computed-tomography
#1
Maria Ibarra, Cynthia Rigsby, Gabrielle A Morgan, Christina L Sammet, Chiang-Ching Huang, Dong Xu, Ira N Targoff, Lauren M Pachman
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 measure objectively in-situ calcification volumes in patients with JIIM over time. METHODS: Ten JIIM patients (eight JDM, two Overlap) with calcifications were prospectively recruited over a 2-year period to undergo two limited, low dose, four-slice CT scans...
November 29, 2016: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/27881174/juvenile-arthritis-caused-by-a-novel-famin-lacc1-mutation-in-two-children-with-systemic-and-extended-oligoarticular-course
#2
Tilmann Kallinich, Anne Thorwarth, Sae-Lim von Stuckrad, Angela Rösen-Wolff, Hella Luksch, Patrick Hundsdoerfer, Kirsten Minden, Peter Krawitz
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 functions and is involved in regulation of inflammasome activation and promotion of ROS production. CASE PRESENTATION: We describe two siblings with severe familial forms of juvenile arthritis in which whole-exome-sequencing revealed a novel homozygous frameshift mutation (NM_153218...
November 24, 2016: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/27881171/assessment-of-left-atrial-mechanical-functions-and-atrial-electromechanical-delay-in-juvenile-idiopathic-arthritis-by-tissue-doppler-echocardiography
#3
Azza Z El Eraky, Nesrin M Handoka, Mona Sayed Ghaly, Samah Ismail Nasef, Nahed A Eldahshan, Ahmed M Ibrahim, Sherein Shalaby
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 out as a across sectional study. A total of 34 patients with active JIA and 34 controls were included...
November 24, 2016: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/27835954/characteristics-and-outcome-of-children-with-juvenile-dermatomyositis-in-cape-town-a-cross-sectional-study
#4
Lawrence Owino Okong'o, Monika Esser, Jo Wilmshurst, Christiaan Scott
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 and outcomes of patients satisfying the Bohan and Peter criteria for probable JDM seen between 2004 and 2013 in three hospitals in Cape Town, South Africa...
November 11, 2016: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/27835952/lipoprotein-cholesterol-fractions-are-related-to-markers-of-inflammation-in-children-and-adolescents-with-juvenile-idiopathic-arthritis-a-cross-sectional-study
#5
Anna-Helene Bohr, Freddy Karup Pedersen, Claus Henrik Nielsen, Klaus Gottlob Müller
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 with inflammation as well as with overweight and low PA. METHODS: Two hundred ten patients with JIA were included in this descriptive cross-sectional study...
November 11, 2016: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/27832822/sarcoidosis-presenting-as-granulomatous-myositis-in-a-16-year-old-adolescent
#6
Amir B Orandi, Eric Eutsler, Cole Ferguson, Andrew J White, Maleewan Kitcharoensakkul
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 hydroxylase. There are limited reports of isolated granulomatous myositis causing hypercalcemia in pediatric patients...
November 10, 2016: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/27832795/bone-histomorphometric-changes-in-children-with-rheumatic-disorders-on-chronic-glucocorticoids
#7
Jennifer Harrington, Douglas Holmyard, Earl Silverman, Etienne Sochett, Marc Grynpas
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 findings with clinical and radiological variables. METHODS: Children on chronic glucocorticoids for an underlying rheumatic disease were referred for evaluation of bone fragility given the presence of vertebral compression fractures...
November 10, 2016: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/27756328/-sometimes-i-feel-like-a-pharmacist-identity-and-medication-use-among-adolescents-with-juvenile-arthritis
#8
J E McDonagh, K L Shaw, J Prescott, F J Smith, R Roberts, N J Gray
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 long-term condition. The aim of this study was to examine the relationships between identity and medication use among young people with juvenile arthritis...
October 19, 2016: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/27658465/arterial-dissection-in-childhood-takayasu-arteritis-not-as-rare-as-thought
#9
Florence A Aeschlimann, Lars Grosse-Wortmann, Susanne M Benseler, Ronald M Laxer, Diane Hebert, Rae S M Yeung
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 performed. Patients with arterial dissection at cTA diagnosis were reported in detail and compared to the remaining single center retrospective cohort of children without dissection...
September 22, 2016: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/27650128/the-human-microbiome-and-juvenile-idiopathic-arthritis
#10
REVIEW
Anouk Verwoerd, Nienke M Ter Haar, Sytze de Roock, Sebastiaan J Vastert, Debby Bogaert
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 the development of a wide array of immune-mediated diseases, including inflammatory bowel disease, type 1 diabetes and rheumatoid arthritis...
September 20, 2016: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/27646340/decreasing-patient-cost-and-travel-time-through-pediatric-rheumatology-telemedicine-visits
#11
Elizabeth A Kessler, Ashley K Sherman, Mara L Becker
BACKGROUND: There is a critical shortage of pediatric rheumatologists in the US. Substantial travel to clinics can impose time and monetary burdens on families. The aim of this study was to evaluate the cost of in-person pediatric rheumatology visits for families and determine if telemedicine clinics resulted in time and cost savings. Factors associated with interest in telemedicine were also explored. METHODS: Surveys were offered to parents and guardians of patients in Pediatric Rheumatology follow-up clinics in Kansas City, Missouri, the primary site of in-person care, and at a telemedicine outreach site 160 miles away, in Joplin, Missouri...
September 20, 2016: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/27641835/parents-information-needs-and-influential-factors-when-making-decisions-about-tnf-%C3%AE-inhibitors
#12
Ellen A Lipstein, Daniel J Lovell, Lee A Denson, Sandra C Kim, Charles Spencer, Maria T Britto
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 inhibitors. METHODS: Survey domains were developed based on qualitative data and cognitive interviewing...
September 15, 2016: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/27609179/monogenic-polyarteritis-the-lesson-of-ada2-deficiency
#13
REVIEW
Roberta Caorsi, Federica Penco, Francesca Schena, Marco Gattorno
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 encodes for the enzymatic protein adenosine deaminase 2 (ADA2). By now 19 different mutations in CECR1 gene have been detected...
September 8, 2016: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/27576444/chronic-recurrent-multifocal-osteomyelitis-crmo-advancing-the-diagnosis
#14
M R Roderick, R Shah, V Rogers, A Finn, A V Ramanan
BACKGROUND: Chronic recurrent multifocal osteomyelitis (CRMO) is a little known inflammatory bone disease occurring primarily in children and adolescents. Delays in referral and diagnosis may lead to prolonged courses of antibiotics with in-patient care, unnecessary radiation exposure from multiple plain radiographs or bone scans and repeated surgery including bone biopsies. Children (aged < 18 years) diagnosed with CRMO between January 2005 and December 2012, reviewed at Bristol Royal Hospital for Children were included and all available data collected...
August 30, 2016: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/27475753/safety-and-efficacy-of-pregabalin-in-adolescents-with-fibromyalgia-a-randomized-double-blind-placebo-controlled-trial-and-a-6-month-open-label-extension-study
#15
Lesley M Arnold, Kenneth N Schikler, Lucinda Bateman, Tahira Khan, Lynne Pauer, Pritha Bhadra-Brown, Andrew Clair, Marci L Chew, Joseph Scavone
BACKGROUND: Fibromyalgia (FM) is a common pain condition characterized by widespread musculoskeletal pain and tenderness. Pregabalin is an approved treatment for adults in the United States, but there are no approved treatments for adolescents with FM. METHODS: This was a 15-week, randomized, double-blind, placebo-controlled study and 6-month open-label safety trial of flexible-dose pregabalin (75-450 mg/day) for the treatment of adolescents (12-17 years) with FM...
July 30, 2016: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/27387754/physical-activity-in-children-with-juvenile-idiopathic-arthritis-compared-to-controls
#16
G J F Joyce Bos, Otto T H M Lelieveld, Wineke Armbrust, Pieter J J Sauer, Jan H B Geertzen, Pieter U Dijkstra
BACKGROUND: To compare physical activity (PA) in children with juvenile idiopathic arthritis (JIA) with controls and to analyse the effect of disease specific factors on PA in children with JIA treated according to current treatment regimes. METHODS: PA was measured with a 7-day activity diary and expressed as physical activity level (PAL). Moderate to vigorous physical activity (MVPA) (hours/day) and sedentary time (hours/day) was determined. In children with JIA, medication, the number of swollen and/or painful joints, disease activity, functional ability, pain and well-being was determined...
July 7, 2016: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/27349388/pfapa-syndrome-a-review-on-treatment-and-outcome
#17
REVIEW
Federica Vanoni, Katerina Theodoropoulou, Michaël Hofer
The syndrome of periodic fever, aphthous stomatitis, pharyngitis and cervical adenitis (PFAPA syndrome) is the most common cause of periodic fever in childhood. The current pharmacological treatment includes corticosteroids, which usually are efficacious in the management of fever episodes, colchicine, for the prophylaxis of febrile episodes, and other medication for which efficacy has not been proven so far. Tonsillectomy is an option for selected patients. Usually PFAPA syndrome resolves during adolescence, but there is increasing evidence that this condition may persist into adulthood...
June 27, 2016: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/27130211/pediatric-complex-regional-pain-syndrome-a-review
#18
REVIEW
Rotem Weissmann, Yosef Uziel
Complex regional pain syndrome (CRPS) is a chronic, intensified localized pain condition that can affect children and adolescents as well as adults, but is more common among adolescent girls. Symptoms include limb pain; allodynia; hyperalgesia; swelling and/or changes in skin color of the affected limb; dry, mottled skin; hyperhidrosis and trophic changes of the nails and hair. The exact mechanism of CRPS is unknown, although several different mechanisms have been suggested. The diagnosis is clinical, with the aid of the adult criteria for CRPS...
April 29, 2016: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/27121190/juvenile-idiopathic-arthritis-associated-uveitis
#19
REVIEW
Sarah L N Clarke, Ethan S Sen, Athimalaipet V Ramanan
Juvenile idiopathic arthritis (JIA) is the most common rheumatic disease of childhood, with JIA-associated uveitis its most common extra-articular manifestation. JIA-associated uveitis is a potentially sight-threatening condition and thus carries a considerable risk of morbidity. The aetiology of the condition is autoimmune in nature with the predominant involvement of CD4(+) T cells. However, the underlying pathogenic mechanisms remain unclear, particularly regarding interplay between genetic and environmental factors...
April 27, 2016: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/27112923/therapeutic-advances-in-the-treatment-of-vasculitis
#20
REVIEW
Despina Eleftheriou, Paul A Brogan
Considerable therapeutic advances for the treatment of vasculitis of the young have been made in the past 10 years, including the development of outcome measures that facilitate clinical trial design. Notably, these include: a recognition that some patients with Kawasaki Disease require corticosteroids as primary treatment combined with IVIG; implementation of rare disease trial design for polyarteritis nodosa to deliver the first randomised controlled trial for children; first clinical trials involving children for anti-neutrophil cytoplasmic antibody (ANCA) vasculitis; and identification of monogenic forms of vasculitis that provide an understanding of pathogenesis, thus facilitating more targeted treatment...
April 26, 2016: Pediatric Rheumatology Online Journal
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