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Pediatric Rheumatology Online Journal

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https://www.readbyqxmd.com/read/29343274/coexistent-sickle-cell-anemia-and-autoimmune-disease-in-eight-children-pitfalls-and-challenges
#1
Valerie Li-Thiao-Te, Florence Uettwiller, Pierre Quartier, Florence Lacaille, Brigitte Bader-Meunier, Valentine Brousse, Mariane de Montalembert
BACKGROUND: Patients with sickle cell disease (SCD) present a defective activation of the alternate complement pathway that increases the risk of infection and is thought to predispose to autoimmune disease (AID). However, coexisting AID and SCD is rarely reported, suggesting possible underdiagnosis due to an overlapping of the symptoms. STUDY DESIGN: Among 603 patients with SCD followed between 1999 and June 2016, we retrospectively searched for patients with coexisting SCD and AID...
January 17, 2018: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/29343257/pigmented-villonodular-synovitis-in-pediatric-population-review-of-literature-and-a-case-report
#2
Mohsen Karami, Mehryar Soleimani, Reza Shiari
BACKGROUND: Pigmented villonodular synovitis (PVNS) is a rare proliferative process in children that mostly affects the knee joint. CASE PRESENTATION: The study follows the case of a 3-year-old boy presenting recurrent patellar dislocation and PVNS. Due to symptoms such as chronic arthritis, he had been taking prednisolone and methotrexate for 6 months before receiving a definitive diagnosis. After a period of showing no improvements from his treatment, he was referred to our center and was diagnosed with local PVNS using magnetic resonance imaging (MRI)...
January 17, 2018: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/29316941/homocysteine-folate-hs-c-reactive-protein-tumor-necrosis-factor-alpha-and-inflammatory-proteins-are-these-biomarkers-related-to-nutritional-status-and-cardiovascular-risk-in-childhood-onset-systemic-lupus-erythematosus
#3
Roberta Garcia Salomão, Luciana Martins de Carvalho, Clarice Izumi, Érika Silva Czernisz, José César Rosa, Sonir Roberto Rauber Antonini, Ana Carolina Bueno, Maria Olímpia Ribeiro do Vale Almada, Carolina de Almeida Coelho-Landell, Alceu Afonso Jordão, Virgínia Paes Leme Ferriani, Jacqueline Pontes Monteiro
BACKGROUND: Childhood-onset systemic lupus erythematosus (c-SLE) is a chronic autoimmune disease which increases cardiovascular risk factors (CRF) such as elevated homocysteine, TNF-α, and hs-C reactive protein. METHODS: We evaluated BMI, waist circumference (WC), 24-h recalls, SLEDAI-2 K, SLICC/ACR-DI, serum levels of homocysteine, folate, TNF-α, hs-C reactive protein, lipid profile, proteomic data, and duration of corticosteroid therapy in 19 c-SLE and 38 healthy volunteers...
January 9, 2018: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/29310668/prior-to-extension-transcriptomes-of-fibroblast-like-synoviocytes-from-extended-and-polyarticular-juvenile-idiopathic-arthritis-are-indistinguishable
#4
AnneMarie C Brescia, Megan M Simonds, Suzanne M McCahan, Kathleen E Sullivan, Carlos D Rose
BACKGROUND: Our intent was to identify differences between the transcriptome of fibroblast-like synoviocytes (FLS) in oligoarticular juvenile idiopathic arthritis (JIA) before extension when compared to persistent subtype of JIA, when the two are clinically indistinguishable. Additionally, we sought to determine if differences between the transcriptomes of FLS from extended-to-be and polyarticular course JIA could be detected. Our hypothesis was that intrinsic differences in the transcriptome of the FLS from extended-to-be JIA would distinguish them from persistent oligoarticular JIA, before the course is clinically apparent...
January 8, 2018: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/29304824/patient-characteristics-associated-with-response-to-nsaid-monotherapy-in-children-with-systemic-juvenile-idiopathic-arthritis
#5
Anjali Sura, Christopher Failing, Julie Sturza, Jasmine Stannard, Meredith Riebschleger
BACKGROUND: Systemic juvenile idiopathic arthritis (sJIA) is an auto-inflammatory disease characterized by fever, arthritis, and ≥1 of rash, generalized lymphadenopathy, hepato/splenomegaly, and serositis. Non-steroidal anti-inflammatory drugs (NSAIDs) are among the initial treatments of sJIA, but there is currently no evidence indicating which children should undergo a trial of NSAID monotherapy and which should not. Our objective is to identify presentation characteristics which are associated with response and lack of response to a trial of NSAID monotherapy...
January 5, 2018: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/29298697/cone-beam-computed-tomography-for-the-assessment-of-linear-scleroderma-of-the-face
#6
C Di Giovanni, S Puggina, A Meneghel, F Vittadello, G Martini, F Zulian
BACKGROUND: To date, standardized methods for assessing the disease progression of linear scleroderma of the face (LSF) are lacking. OBJECTIVES: We investigated whether Cone Beam Computed Tomography (CBCT) may represent a reliable tool for assessing linear scleroderma of the face (LSF). METHODS: Ten patients with LSF and five age-matched controls underwent CBCT assessment. The transverse sections at three anatomic levels of the maxillofacial bones were analyzed...
January 3, 2018: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/29287597/-next-generation-sequencing-identifies-mutations-in-gnptg-gene-as-a-cause-of-familial-form-of-scleroderma-like-disease
#7
LETTER
Afolake T Arowolo, Henry A Adeola, Nonhlanhla P Khumalo
No abstract text is available yet for this article.
December 29, 2017: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/29287595/dealing-with-chronic-non-bacterial-osteomyelitis-a-practical-approach
#8
REVIEW
Andrea Taddio, Giovanna Ferrara, Antonella Insalaco, Manuela Pardeo, Massimo Gregori, Martina Finetti, Serena Pastore, Alberto Tommasini, Alessandro Ventura, Marco Gattorno
BACKGROUND: Chronic Non-Bacterial Osteomyelitis (CNO) is an inflammatory disorder that primarily affects children. Although underestimated, its incidence is rare. For these reasons, no diagnostic and no therapeutic guidelines exist. The manuscript wants to give some suggestions on how to deal with these patients in the every-day clinical practice. MAIN BODY: CNO is characterized by insidious onset of bone pain with local swelling. Systemic symptoms such as fever, skin involvement and arthritis may be sometimes present...
December 29, 2017: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/29282090/pharmacokinetic-and-safety-profile-of-tofacitinib-in-children-with-polyarticular-course-juvenile-idiopathic-arthritis-results-of-a-phase-1-open-label-multicenter-study
#9
Nicolino Ruperto, Hermine I Brunner, Zbigniew Zuber, Nikolay Tzaribachev, Daniel J Kingsbury, Ivan Foeldvari, Gerd Horneff, Elzbieta Smolewska, Richard K Vehe, Anasuya Hazra, Rong Wang, Charles A Mebus, Christine Alvey, Manisha Lamba, Sriram Krishnaswami, Thomas C Stock, Min Wang, Ricardo Suehiro, Alberto Martini, Daniel J Lovell
BACKGROUND: Juvenile idiopathic arthritis (JIA) is the most common pediatric rheumatic disease and a leading cause of childhood disability. The objective of this study was to characterize the PK, safety, and taste acceptability of tofacitinib in patients with JIA. METHODS: This Phase 1, open-label, multiple-dose (twice daily [BID] for 5 days) study of tofacitinib in patients with active (≥ 5 joints) polyarticular course JIA was conducted from March 2013-December 2015...
December 28, 2017: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/29282086/mental-health-care-for-youth-with-rheumatologic-diseases-bridging-the-gap
#10
REVIEW
Alaina M Davis, Tamar B Rubinstein, Martha Rodriguez, Andrea M Knight
Youth with rheumatologic diseases have a high prevalence of comorbid mental health disorders. Individuals with comorbid mental health disorders are at increased risk for adverse outcomes related to mental health as well as their underlying rheumatologic disease. Early identification and treatment of mental health disorders has been shown to improve outcomes, but current systems of care fall short in providing adequate mental health services to those in need. Pediatric rheumatologists are uniquely positioned to provide mental health screening and intervention for youth with rheumatologic diseases due to the frequency of patient encounters and ongoing therapeutic relationship with patients and families...
December 28, 2017: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/29268757/a-national-cohort-study-on-pediatric-beh%C3%A3-et-s-disease-cross-sectional-data-from-an-italian-registry
#11
Romina Gallizzi, Caterina Pidone, Luca Cantarini, Martina Finetti, Marco Cattalini, Giovanni Filocamo, Antonella Insalaco, Donato Rigante, Rita Consolini, Maria Cristina Maggio, Adele Civino, Silvana Martino, Alma Nunzia Olivieri, Giovanna Fabio, Serena Pastore, Angela Mauro, Diana Sutera, Giuseppe Trimarchi, Nicolino Ruperto, Marco Gattorno, Rolando Cimaz
BACKGROUND: Behçet's disease is a rare multi-systemic inflammatory disease with unknown etiology which involves principally oral and genital mucosa, skin and eyes. Average age at onset of the disease is about 25-30 years, but it may be diagnosed before the age of 16. It is not very rare in Italy, even though there are limited data concerning epidemiology. Aim of this study is to describe the baseline data of an Italian cohort of patients with as having BD or probable BD. METHODS: We described the baseline data of the first national epidemiological study on children coming from 16 Italian Pediatric Rheumatologic Centers diagnosed by the treating physicians as having Behçet's Disease...
December 21, 2017: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/29178931/anatomy-of-the-sacroiliac-joints-in-children-and-adolescents-by-computed-tomography
#12
Anna Zejden, Anne Grethe Jurik
BACKGROUND: Diagnosing sacroiliitis by magnetic resonance imaging (MRI) in children/adolescents can be difficult due to the growth-related changes. This study analyzed the normal osseous anatomy of the sacroiliac joints (SIJ) in a juvenile population using computed tomography (CT). METHODS: The anatomy of the SIJ was retrospectively analyzed in 124 trauma patients aged 9 months - <18 years by CT, based on 2 mm slices in axial, semi-axial and semi-coronal planes...
November 25, 2017: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/29137644/functional-resonance-magnetic-imaging-fmri-in-adolescents-with-idiopathic-musculoskeletal-pain-a-paradigm-of-experimental-pain
#13
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
#14
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
#15
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
#16
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
#17
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
#18
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
#19
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
#20
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
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