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Pediatric Rheumatology Online Journal

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https://www.readbyqxmd.com/read/29137644/functional-resonance-magnetic-imaging-fmri-in-adolescents-with-idiopathic-musculoskeletal-pain-a-paradigm-of-experimental-pain
#1
Juliana Molina, Edson Amaro, Liana Guerra Sanches da Rocha, Liliana Jorge, Flavia Heloisa Santos, Claudio A Len
BACKGROUND: Studies on functional magnetic resonance imaging (fMRI) have shown that adults with musculoskeletal pain syndromes tolerate smaller amount of pressure (pain) as well as differences in brain activation patterns in areas related to pain.The objective of this study was to evaluate, through fMRI, the brain activation in adolescents with idiopathic musculoskeletal pain (IMP) while performing an experimental paradigm of pain. METHODS: The study included 10 consecutive adolescents with idiopathic musculoskeletal pain (average age 16...
November 14, 2017: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/29132381/patients-with-juvenile-idiopathic-arthritis-in-clinical-remission-with-positive-power-doppler-signal-in-joint-ultrasonography-have-an-increased-rate-of-clinical-flare-a-prospective-study
#2
Vanessa Bugni Miotto E Silva, Sônia de Aguiar Vilela Mitraud, Rita Nely Vilar Furtado, Jamil Natour, Claudio Arnaldo Len, Maria Teresa de Sande E Lemos Ramos Ascensão Terreri
BACKGROUND: Ultrasonography (US) studies carried out on joints of juvenile idiopathic arthritis (JIA) patients in clinical remission demonstrate the presence of subclinical synovitis. The significance of subclinical synovitis and the positive power Doppler (PD) signal on US in JIA in clinical remission is not well understood. The objectives of this study were to assess whether the changes detected by US in patients with JIA in clinical remission can predict disease flare and to evaluate factors associated with flare and joint damage over 30 months of follow-up...
November 13, 2017: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/29121953/risk-of-tuberculosis-among-alabama-children-and-adolescents-treated-with-tumor-necrosis-factor-inhibitors-a-retrospective-study
#3
Matthew L Stoll, James Aaron Grubbs, Timothy Beukelman, Melissa L Mannion, Traci W Jester, Randy Q Cron, Marilyn J Crain
BACKGROUND: Tumor Necrosis Factor inhibitors (TNFi) have dramatically improved the outlook for patients with inflammatory arthritides and bowel disease (IBD), but are associated with increased infection risks, including tuberculosis (TB). Pediatric inflammatory diseases are uncommon, and the risk of TB in children taking TNFi remains unclear. The objective of this study was to report the incidence of TB disease among TNFi recipients at a single pediatric medical center serving most of Alabama compared to that of the general population of Alabama children...
November 9, 2017: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/29116003/protocols-on-classification-monitoring-and-therapy-in-children-s-rheumatology-pro-kind-results-of-the-working-group-polyarticular-juvenile-idiopathic-arthritis
#4
REVIEW
Gerd Horneff, Ariane Klein, Gerd Ganser, Michaela Sailer-Höck, Annette Günther, Ivan Foeldvari, Frank Weller-Heinemann
OBJECTIVE: Several effective pharmacologic treatment options for polyarticual juvenile idiopathic arthritis (JIA) have emerged but initial treatment is heterogeneous in Germany. Therefore, the German Society of Pediatric Rheumatolgy has established a commission to develop consensus "Protocols on classification, monitoring and therapy in children's rheumatology (PRO-KIND)" to harmonize diagnostic and treatment approaches for new-onset JIA in Germany. METHODS: A set of definitions for in- and exclusion, diagnostic workup, parameters for the evaluation of disease activity criteria, therapeutic options, medication dosing, monitoring recommendations, targets, definitions of a therapy failure and four therapeutic algorithms developed by a working group were agreed by web based survey to which all members of the GKJR have been invited...
November 7, 2017: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/29089059/effective-induction-therapy-for-anti-srp-associated-myositis-in-childhood-a-small-case-series-and-review-of-the-literature
#5
E L Binns, E Moraitis, S Maillard, S Tansley, N McHugh, T S Jacques, L R Wedderburn, C Pilkington, S A Yasin, K Nistala
BACKGROUND: Anti-Signal Recognition Particle associated myopathy is a clinically and histopathologically distinct subgroup of Juvenile Idiopathic Inflammatory Myositis, which is under-recognised in children and fails to respond to conventional first line therapies. We present three cases where remission was successfully induced using combination therapy with intensive rehabilitation. CASE PRESENTATIONS: Three new patients are reported. All 3 cases presented with profound, rapid-onset, proximal myopathy and markedly raised CK, but no rash...
October 31, 2017: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/29041934/h-syndrome-5-new-cases-from-the-united-states-with-novel-features-and-responses-to-therapy
#6
Jessica L Bloom, Clara Lin, Lisa Imundo, Stephen Guthery, Shelly Stepenaskie, Csaba Galambos, Amy Lowichik, John F Bohnsack
BACKGROUND: H Syndrome is an autosomal recessive disorder characterized by cutaneous hyperpigmentation, hypertrichosis, and induration with numerous systemic manifestations. The syndrome is caused by mutations in SLC29A3, a gene located on chromosome 10q23, which encodes the human equilibrative transporter 3 (hENT3). Less than 100 patients with H syndrome have been described in the literature, with the majority being of Arab descent, and only a few from North America. CASE PRESENTATION: Here we report five pediatric patients from three medical centers in the United States who were identified to have H syndrome by whole exome sequencing...
October 17, 2017: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/29029629/effects-of-age-and-gender-on-reference-levels-of-biomarkers-comprising-the-pediatric-renal-activity-index-for-lupus-nephritis-p-rail
#7
Michael R Bennett, Qing Ma, Jun Ying, Prasad Devarajan, Hermine Brunner
BACKGROUND: Systemic Lupus Erythematosus (SLE) is a multisystem autoimmune disease that disproportionately effects women and children of minorities. Renal involvement (lupus nephritis, or LN) occurs in up to 80% of children with SLE and is a major determinant of poor prognosis. We have developed a non-invasive pediatric Renal Activity Index for Lupus (p-RAIL) that consists of laboratory measures that reflect histologic LN activity. These markers are neutrophil gelatinase associated lipocalin (NGAL), kidney injury molecule-1 (KIM-1), monocyte chemotactic protein (MCP-1), adiponectin (APN), ceruloplasmin (CP) and hemopexin (HPX)...
October 13, 2017: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/29029616/short-term-effectiveness-and-experiences-of-a-peer-guided-web-based-self-management-intervention-for-young-adults-with-juvenile-idiopathic-arthritis
#8
Judy Ammerlaan, Harmieke van Os-Medendorp, Nienke de Boer-Nijhof, Lieske Scholtus, Aike A Kruize, Philomine van Pelt, Berent Prakken, Hans Bijlsma
BACKGROUND: A web-based self-management intervention guided by peer-trainers was developed to support young adults' self-management in coping with Juvenile Idiopathic Arthritis (JIA). To investigate its effectiveness, a randomized controlled trial (RCT) was conducted. In addition, the content of the chat and participants' goals were studied to identify underlying processes. METHODS: An RCT with a six-month follow up period was conducted among 72 young adults with JIA, aged between 16 and 25 years old, randomly assigned to the intervention or to the usual care control group...
October 13, 2017: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/29025422/readout-segmented-multi-shot-diffusion-weighted-mri-of-the-knee-joint-in-patients-with-juvenile-idiopathic-arthritis
#9
Alexander Sauer, Mengxia Li, Annette Holl-Wieden, Thomas Pabst, Henning Neubauer
BACKGROUND: Diffusion-weighted MRI has been proposed as a new technique for imaging synovitis without intravenous contrast application. We investigated diagnostic utility of multi-shot readout-segmented diffusion-weighted MRI (multi-shot DWI) for synovial imaging of the knee joint in patients with juvenile idiopathic arthritis (JIA). METHODS: Thirty-two consecutive patients with confirmed or suspected JIA (21 girls, median age 13 years) underwent routine 1.5 T MRI with contrast-enhanced T1w imaging (contrast-enhanced MRI) and with multi-shot DWI (RESOLVE, b-values 0-50 and 800 s/mm(2))...
October 12, 2017: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/28950892/next-generation-sequencing-identifies-mutations-in-gnptg-gene-as-a-cause-of-familial-form-of-scleroderma-like-disease
#10
Abdelali Zrhidri, Saadia Amasdl, Jaber Lyahyai, Hanane Elouardi, Bouchra Chkirate, Laure Raymond, Grégory Egéa, Mohamed Taoudi, Said El Mouatassim, Abdelaziz Sefiani
BACKGROUND: Scleroderma is a multisystem disease, characterized by fibrosis of skin and internal organs, immune dysregulation, and vasculopathy. The etiology of the disease remains unknown, but it is likely multifactorial. However, the genetic basis for this condition is defined by multiple genes that have only modest effect on disease susceptibility. METHODS: Three Moroccan siblings, born from non-consanguineous Moroccan healthy parents were referred for genetic evaluation of familial scleroderma...
September 26, 2017: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/28934971/assessment-classification-and-treatment-of-calcinosis-as-a-complication-of-juvenile-dermatomyositis-a-survey-of-pediatric-rheumatologists-by-the-childhood-arthritis-and-rheumatology-research-alliance-carra
#11
A B Orandi, K W Baszis, V R Dharnidharka, A M Huber, M F Hoeltzel
BACKGROUND: There is no standardized approach to the management of JDM-associated calcinosis and its phenotypes. Current knowledge of treatment outcomes is confined to small series and case reports. We describe physician perspectives toward diagnostic approach, classification and treatment directly targeting calcinosis, independent of overall JDM therapy. METHODS: An electronic survey of 22 questions was organized into sections regarding individual practices of assessment, classification and treatment of calcinosis, including perceived successes of therapies...
September 21, 2017: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/28888223/assessment-of-transition-readiness-in-adolescents-and-young-adults-with-chronic-health-conditions
#12
Paul T Jensen, Gabrielle V Paul, Stephanie LaCount, Juan Peng, Charles H Spencer, Gloria C Higgins, Brendan Boyle, Manmohan Kamboj, Christopher Smallwood, Stacy P Ardoin
BACKGROUND: 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 TRAQ scores and other demographic variables predicted transition to adult care over a three year follow up period...
September 9, 2017: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/28865469/comics-as-an-educational-tool-for-children-with-juvenile-idiopathic-arthritis
#13
Amir Mendelson, Noa Rabinowicz, Yonit Reis, Gil Amarilyo, Liora Harel, Philip J Hashkes, Yosef Uziel
BACKGROUND: 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). METHODS: 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, health-related quality of life and adherence data were recorded and correlated to the responses...
September 2, 2017: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/28830457/growth-and-weight-gain-in-children-with-juvenile-idiopathic-arthritis-results-from-the-reacch-out-cohort
#14
Jaime Guzman, Tristan Kerr, Leanne M Ward, Jinhui Ma, Kiem Oen, Alan M Rosenberg, Brian M Feldman, Gilles Boire, Kristin Houghton, Paul Dancey, Rosie Scuccimarri, Alessandra Bruns, Adam M Huber, Karen Watanabe Duffy, Natalie J Shiff, Roberta A Berard, Deborah M Levy, Elizabeth Stringer, Kimberly Morishita, Nicole Johnson, David A Cabral, Maggie Larché, Ross E Petty, Ronald M Laxer, Earl Silverman, Paivi Miettunen, Anne-Laure Chetaille, Elie Haddad, Lynn Spiegel, Stuart E Turvey, Heinrike Schmeling, Bianca Lang, Janet Ellsworth, Suzanne E Ramsey, Johannes Roth, Sarah Campillo, Susanne Benseler, Gaëlle Chédeville, Rayfel Schneider, Shirley M L Tse, Roxana Bolaria, Katherine Gross, Debbie Feldman, Bonnie Cameron, Roman Jurencak, Jean Dorval, Claire LeBlanc, Claire St Cyr, Michele Gibbon, Rae S M Yeung, Ciarán M Duffy, Lori B Tucker
BACKGROUND: With modern treatments, the effect of juvenile idiopathic arthritis (JIA) on growth may be less than previously reported. Our objective was to describe height, weight and body mass index (BMI) development in a contemporary JIA inception cohort. METHODS: Canadian children newly-diagnosed with JIA 2005-2010 had weight and height measurements every 6 months for 2 years, then yearly up to 5 years. These measurements were used to calculate mean age- and sex-standardized Z-scores, and estimate prevalence and cumulative incidence of growth impairments, and the impact of disease activity and corticosteroids on growth...
August 22, 2017: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/28830446/autoimmune-phenotype-with-type-i-interferon-signature-in-two-brothers-with-ada2-deficiency-carrying-a-novel-cecr1-mutation
#15
Andrea Skrabl-Baumgartner, Barbara Plecko, Wolfgang M Schmidt, Nadja König, Michael Hershfield, Ursula Gruber-Sedlmayr, Min Ae Lee-Kirsch
BACKGROUND: Loss-of-function CECR1 mutations cause polyarteritis nodosa (PAN) with childhood onset, an autoinflammatory disorder without significant signs of autoimmunity. Herein we describe the unusual presentation of an autoimmune phenotype with constitutive type I interferon activation in siblings with adenosine deaminase 2 (ADA2) deficiency. CASE PRESENTATION: We describe two siblings with early-onset recurrent strokes, arthritis, oral ulcers, discoid rash, peripheral vascular occlusive disease and high antinuclear antibody titers...
August 22, 2017: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/28821293/incidence-and-predictors-of-uveitis-in-juvenile-idiopathic-arthritis-in-a-nordic-long-term-cohort-study
#16
Ellen Nordal, Veronika Rypdal, Terje Christoffersen, Kristiina Aalto, Lillemor Berntson, Anders Fasth, Troels Herlin, Susan Nielsen, Suvi Peltoniemi, Bjørn Straume, Marek Zak, Marite Rygg
BACKGROUND: The incidence of uveitis associated with juvenile idiopathic arthritis (JIA) varies around the world. Our aim was to investigate the incidence and predictors of uveitis in a Nordic population-based cohort. METHODS: Consecutive JIA cases from defined geographical areas in Denmark, Finland, Sweden and Norway with disease onset between January 1997 to June 2000 were followed for median 98 months in this prospective longitudinal cohort study. Potential clinical and immunological predictors of uveitis were identified with logistic regression analysis...
August 18, 2017: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/28797267/joint-cartilage-thickness-and-automated-determination-of-bone-age-and-bone-health-in-juvenile-idiopathic-arthritis
#17
Marinka Twilt, Dan Pradsgaard, Anne Helene Spannow, Arne Horlyck, Carsten Heuck, Troels Herlin
BACKGROUND: BoneXpert is an automated method to calculate bone maturation and bone health index (BHI) in children with juvenile idiopathic arthritis (JIA). Cartilage thickness can also be seen as an indicator for bone health and arthritis damage. The objective of this study was to evaluate the relation between cartilage thickness, bone maturation and bone health in patients with JIA. METHODS: Patients with JIA diagnosed according ILAR criteria included in a previous ultrasonography (US) study were eligible if hand radiographs were taken at the same time as the US examination...
August 10, 2017: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/28784185/immunogenicity-and-safety-of-influenza-vaccination-in-patients-with-juvenile-idiopathic-arthritis-on-biological-therapy-using-the-microneutralization-assay
#18
M S Camacho-Lovillo, A Bulnes-Ramos, W Goycochea-Valdivia, L Fernández-Silveira, E Núñez-Cuadros, O Neth, P Pérez-Romero
BACKGROUND: Seasonal influenza virus vaccination should be considered in all pediatric patients with rheumatic diseases. Few studies have addressed influenza vaccination safety and efficacy in this group. We aim to prospectively evaluate immunogenicity and safety of the trivalent inactivated influenza vaccine including A/H1N1, A/H3N2 and B strains in children with juvenile idiopathic arthritis (JIA) receiving biological therapy. METHODS: Thirty-five children diagnosed with JIA and 6 healthy siblings were included...
August 7, 2017: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/28784161/changes-in-clinical-and-laboratory-features-of-kawasaki-disease-noted-over-time-in-daejeon-korea
#19
Hong-Ryang Kil, Jae-Won Yu, Sung-Churl Lee, Jung-Woo Rhim, Kyung-Yil Lee
BACKGROUND: Kawasaki disease (KD) becomes one of the common diseases in Korea. Changes in clinical features and laboratory findings of KD were evaluated over a period of 10 years. METHODS: We reviewed the medical records of KD patients and compared the clinical and laboratory features of two KD patient groups: those admitted from 2000 to 2004 (group A, 284 cases) and those admitted from 2010 to 2014 (group B, 331 cases). RESULTS: There were a total of 615 KD patients (mean age: 29...
August 7, 2017: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/28784150/clinical-practice-variation-and-need-for-pediatric-specific-treatment-guidelines-among-rheumatologists-caring-for-children-with-anca-associated-vasculitis-an-international-clinician-survey
#20
Clara Westwell-Roper, Joanna M Lubieniecka, Kelly L Brown, Kimberly A Morishita, Cherry Mammen, Linda Wagner-Weiner, Eric Yen, Suzanne C Li, Kathleen M O'Neil, Sivia K Lapidus, Paul Brogan, Rolando Cimaz, David A Cabral
BACKGROUND: Because pediatric antineutrophil cytoplasmic antibody-associated vasculitis is rare, management generally relies on adult data. We assessed treatment practices, uptake of existing clinical assessment tools, and interest in pediatric treatment protocols among rheumatologists caring for children with granulomatosis with polyangiitis (GPA) and microscopic polyangiitis (MPA). METHODS: A needs-assessment survey developed by an international working group of pediatric rheumatologists and two nephrologists was circulated internationally...
August 7, 2017: Pediatric Rheumatology Online Journal
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