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Dermatomyositis childhood

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https://www.readbyqxmd.com/read/29773230/medications-received-by-patients-with-juvenile-dermatomyositis
#1
Takayuki Kishi, Nastaran Bayat, Michael M Ward, Adam M Huber, Lan Wu, Gulnara Mamyrova, Ira N Targoff, William J Warren-Hicks, Frederick W Miller, Lisa G Rider
OBJECTIVE: Few controlled studies are available to guide treatment decisions in juvenile dermatomyositis (JDM). This study evaluated therapies received, changes of treatment over time, and factors associated with medication choices in JDM. METHODS: We performed a retrospective analysis of the number and type of therapies and duration of treatment for 320 patients with JDM enrolled in a North American registry. Kaplan-Meier and logistic regression analysis were used to assess the association of demographic and clinical features and autoantibodies with medication usage...
March 28, 2018: Seminars in Arthritis and Rheumatism
https://www.readbyqxmd.com/read/29770465/expression-of-myxovirus-resistance-protein-a-a-possible-marker-of-muscle-disease-activity-and-autoantibody-specificities-in-juvenile-dermatomyositis
#2
Sirisucha Soponkanaporn, Claire T Deakin, Peter W Schutz, Lucy R Marshall, Shireena A Yasin, Cerise M Johnson, Erdal Sag, Sarah L Tansley, Neil J McHugh, Lucy R Wedderburn, Thomas S Jacques
AIMS: To evaluate the relationship between expression of Myxovirus-resistance protein A (MxA) protein on muscle biopsies by immunohistochemistry and disease activity in JDM patients. Also, another aim was to investigate whether the expression of MxA is related with myositis-specific autoantibodies (MSA) status in JDM patients. METHODS: 103 patients (median aged 6.3, IQR 0.5-15.9) enrolled in the Juvenile Dermatomyositis Cohort and Biomarker Study (JDCBS). Muscle biopsies were stained with MxA and scored...
May 16, 2018: Neuropathology and Applied Neurobiology
https://www.readbyqxmd.com/read/29673367/comparing-the-importance-of-quality-measurement-themes-in-juvenile-idiopathic-inflammatory-myositis-between-patients-and-families-and-healthcare-professionals
#3
Heather O Tory, Ruy Carrasco, Thomas Griffin, Adam M Huber, Philip Kahn, Angela Byun Robinson, David Zurakowski, Susan Kim
BACKGROUND: A standardized set of quality measures for juvenile idiopathic inflammatory myopathies (JIIM) is not in use. Discordance has been shown between the importance ascribed to quality measures between patients and families and physicians. The objective of this study was to assess and compare the importance of various aspects of high quality care to patients with JIIM and their families with healthcare providers, to aid in future development of comprehensive quality measures. METHODS: Surveys were developed by members of the Childhood Arthritis and Rheumatology Research Alliance (CARRA) Juvenile Dermatomyositis Workgroup through a consensus process and administered to patients and families through the CureJM Foundation and to healthcare professionals through CARRA...
April 19, 2018: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/29457372/clinical-characteristics-and-factors-associated-with-disability-and-impaired-quality-of-life-in-children-with-juvenile-systemic-sclerosis
#4
Brandi E Stevens, Kathryn S Torok, Suzanne C Li, Nicole Hershey, Megan Curran, Gloria C Higgins, Katharine F Moore, C Egla Rabinovich, Samuel Dodson, Anne M Stevens
OBJECTIVE: To investigate clinical manifestations of juvenile systemic sclerosis (jSSc), including disease characteristics and patient quality of life, through the multinational Childhood Arthritis and Rheumatology Research Alliance (CARRA) Legacy Registry. METHODS: Subjects with jSSc were prospectively enrolled between 2010 and 2013. Diagnosis of jSSc was determined by the enrolling pediatric rheumatologist, with disease onset required prior to age 18. Collected data included demographics, disease characteristics, medication exposure, and quality of life metrics...
February 18, 2018: Arthritis Care & Research
https://www.readbyqxmd.com/read/29363513/anti-nt5c1a-autoantibodies-are-associated-with-more-severe-disease-in-patients-with-juvenile-myositis
#5
Richard M Yeker, Iago Pinal-Fernandez, Takayuki Kishi, Katherine Pak, Ira N Targoff, Frederick W Miller, Lisa G Rider, Andrew L Mammen
OBJECTIVES: Autoantibodies recognising cytosolic 5'-nucleotidase 1A (NT5C1A) are found in adult patients with myositis and other autoimmune diseases. They are especially prevalent in adults with inclusion body myositis (IBM), in which they are associated with more severe weakness and higher mortality. This study was undertaken to define the prevalence and clinical features associated with anti-NT5C1A autoantibodies in juvenile myositis. METHODS: We screened sera from 380 patients with juvenile myositis, 30 patients with juvenile idiopathic arthritis (JIA) and 92 healthy control children for anti-NT5C1A autoantibodies...
May 2018: Annals of the Rheumatic Diseases
https://www.readbyqxmd.com/read/29333701/the-childhood-arthritis-and-rheumatology-research-alliance-consensus-treatment-plans-toward-comparative-effectiveness-in-the-pediatric-rheumatic-diseases
#6
Sarah Ringold, Peter A Nigrovic, Brian M Feldman, George A Tomlinson, Emily von Scheven, Carol A Wallace, Adam M Huber, Laura E Schanberg, Suzanne C Li, Pamela F Weiss, Robert C Fuhlbrigge, Esi M Morgan, Yukiko Kimura
The pediatric rheumatic diseases are a heterogeneous group of rare diseases, posing a number of challenges for the use of traditional clinical and translational research methods. Innovative comparative effectiveness approaches are needed to efficiently study treatment strategies and disease outcomes. The Childhood Arthritis and Rheumatology Research Alliance (CARRA) developed the consensus treatment plan (CTP) approach as a comparative effectiveness tool for research in pediatric rheumatology. CTPs are treatment strategies, developed by consensus methods among CARRA members, intended to reduce variation in treatment approaches, standardize outcome measurements, and allow for comparison of the effectiveness of different approaches with the goal of improving disease outcomes...
January 15, 2018: Arthritis & Rheumatology
https://www.readbyqxmd.com/read/29245175/development-and-testing-of-a-hybrid-measure-of-muscle-strength-in-juvenile-dermatomyositis-for-use-in-routine-care
#7
Giulia Camilla Varnier, Silvia Rosina, Cristina Ferrari, Angela Pistorio, Alessandro Consolaro, Francesca Bovis, Sara Dalprà, Clarissa Pilkington, Susan Maillard, Adele Civino, Elena Tsitsami, Jaime de Inocencio, Marija Jelusic, Jelena Vojinovic, Graciela Espada, Balahan Makay, Maria Martha Katsicas, Polixeni Pratsidou-Gertsi, Dragana Lazarevic, Anand Prahalad Rao, Denise Pires Marafon, Nicolino Ruperto, Alberto Martini, Angelo Ravelli
OBJECTIVE: To develop and test a hybrid measure of muscle strength for juvenile dermatomyositis (JDM), which is based on the combination of the Manual Muscle Testing 8 (MMT8) and the Childhood Myositis Assessment Scale (CMAS), but is more comprehensive than the former and more feasible than the latter. METHODS: The hybrid MMT/CMAS (hMC) is composed of all 8 items of the MMT8 and 3 items of the CMAS: 1) time of head lift; 2) assessment of abdominal muscles; 3) floor rise...
December 15, 2017: Arthritis Care & Research
https://www.readbyqxmd.com/read/29170979/predictors-of-changes-in-disease-activity-among-children-with-juvenile-dermatomyositis-enrolled-in-the-childhood-arthritis-and-rheumatology-research-alliance-carra-legacy-registry
#8
Divya Challa, Cynthia S Crowson, Timothy B Niewold, Ann M Reed
Determinants of changes in disease activity among patients with juvenile dermatomyositis (JDM) are unknown. Our objective was to develop predictive models to predict changes in disease activity using the CARRA Legacy Registry. The CARRA Legacy Registry included 658 subjects with definite or probably JDM with 297 subjects with a one follow-up visit after baseline, and we studied the 65 subjects with active disease at baseline. Linear regression models were used to build risk scores for changes in disease activity adjusted for baseline disease activity, age, sex, and disease duration...
November 23, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/29120812/imaging-findings-in-systemic-childhood-diseases-presenting-with-dermatologic-manifestations
#9
REVIEW
Adam Z Fink, Julia K Gittler, Radhika N Nakrani, Jonathan Alis, Einat Blumfield, Terry L Levin
PURPOSE: Many childhood diseases often present with skin abnormalities with which radiologists are largely unfamiliar. Knowledge of associated dermatologic manifestations may aid the radiologist in confirming the diagnosis and recommending targeted imaging of affected organs. METHODS: We review the imaging findings in childhood diseases associated with dermatologic manifestations. FINDINGS: Diseases include dermatologic findings which herald underlying malignancy (Neuroblastoma, leukemia/lymphoma, Langerhans cell histiocytosis),are associated with risk of malignancy (Epidermolysis Bullosa, basal cell nevus syndrome, Cowden's syndrome, Tuberous Sclerosis),or indicate a systemic inflammatory/immune disorder (Kawasaki's disease, Henoch Schonlein Purpura, systemic lupus erythematosus, scleroderma, sarcoidosis, dermatomyositis and immune thrombocytopenic purpura)...
October 31, 2017: Clinical Imaging
https://www.readbyqxmd.com/read/29090371/cutaneous-manifestations-of-dermatomyositis-a-comprehensive-review
#10
REVIEW
Carlo Mainetti, Benedetta Terziroli Beretta-Piccoli, Carlo Selmi
Dermatomyositis (DM) is an idiopathic inflammatory myopathy characterized by the presence of skin lesions and inflammation of skeletal muscles; however, this feature may be absent in amyopathic DM. DM is a rare disease, occurring at any age, and has two peaks of incidence: one in childhood between 5 and 15 years of age and one in adulthood between 40 and 60 years, with a female preponderance. DM has been associated with malignancy; therefore, every newly diagnosed patient should undergo screening investigations, but evidence-based guidelines on their extension are lacking...
December 2017: Clinical Reviews in Allergy & Immunology
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
#11
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/28954874/fibrous-arthropathy-associated-with-morphea-a-new-cause-of-diffuse-acquired-joint-contractures
#12
Etienne Merlin, Sylvain Breton, Sylvie Fraitag, Jean-Louis Stéphan, Carine Wouters, Christine Bodemer, Brigitte Bader-Meunier
Etiologies for childhood-onset diffuse joint contractures encompass a large group of inherited disorders and acquired diseases, in particular a subtype of juvenile idiopathic arthritis called "dry polyarthritis," dermatomyositis, and systemic sclerosis. We report on 2 boys, aged 5 and 8 years, who developed acquired symmetric painless joint contractures preceding the development of superficial plaques of morphea by 7 to 13 months. There was no other clinical involvement, biological inflammation, or autoantibodies...
October 2017: Pediatrics
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
#13
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/28911766/juvenile-dermatomyositis-key-roles-of-muscle-magnetic-resonance-imaging-and-early-aggressive-treatment
#14
O Corral-Magaña, A F Bauzá-Alonso, M M Escudero-Góngora, L Lacruz, A Martín-Santiago
Juvenile dermatomyositis is a rare systemic connective tissue disease with onset during childhood. It presents clinically with proximal muscle weakness and characteristic skin involvement. Diagnosis is based on the Bohan and Peter criteria, though many authors are now substituting biopsy with muscle magnetic resonance imaging (MRI) for both diagnosis and follow-up. Without intensive early treatment, complications such as calcinosis cutis and lipodystrophy can develop in the chronic phases of the disease. Early recognition is therefore key to management...
September 11, 2017: Actas Dermo-sifiliográficas
https://www.readbyqxmd.com/read/28895209/focal-hyperkeratosis-overlying-the-palmar-faces-of-interphalangeal-and-metacarpophalangeal-joints-revealing-a-juvenile-dermatomyositis
#15
C Abasq-Thomas, V Devauchelle, S Fraitag, N Jay, G Gourier, A Le Duc Pennec, E Brenaut, L Misery
Dermatomyositis (DM) is an idiopathic inflammatory myopathy characterized by an inflammatory infiltrate primarily affecting the skeletal muscle and skin [1]. It is the commonest of the idiopathic inflammatory myopathies of childhood, comprising 85% of cases. It has an annual incidence estimated to range between 1.9 and 4.1 per million children [2, 3]. This article is protected by copyright. All rights reserved.
September 12, 2017: Journal of the European Academy of Dermatology and Venereology: JEADV
https://www.readbyqxmd.com/read/28610606/biologic-therapies-for-refractory-juvenile-dermatomyositis-five-years-of-experience-of-the-childhood-arthritis-and-rheumatology-research-alliance-in-north-america
#16
C H Spencer, K Rouster-Stevens, H Gewanter, G Syverson, R Modica, K Schmidt, H Emery, C Wallace, S Grevich, K Nanda, Y D Zhao, S Shenoi, S Tarvin, S Hong, C Lindsley, J E Weiss, M Passo, K Ede, A Brown, K Ardalan, W Bernal, M L Stoll, B Lang, R Carrasco, C Agaiar, L Feller, H Bukulmez, R Vehe, H Kim, H Schmeling, D Gerstbacher, M Hoeltzel, B Eberhard, R Sundel, S Kim, A M Huber, A Patwardhan
BACKGROUND: The prognosis of children with juvenile dermatomyositis (JDM) has improved remarkably since the 1960's with the use of corticosteroid and immunosuppressive therapy. Yet there remain a minority of children who have refractory disease. Since 2003 the sporadic use of biologics (genetically-engineered proteins that usually are derived from human genes) for inflammatory myositis has been reported. In 2011-2016 we investigated our collective experience of biologics in JDM through the Childhood Arthritis and Rheumatology Research Alliance (CARRA)...
June 13, 2017: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/28550457/treatment-of-juvenile-dermatomyositis-an-update
#17
REVIEW
Charalampia Papadopoulou, Lucy R Wedderburn
The idiopathic inflammatory myopathies of childhood consist of a heterogeneous group of autoimmune diseases characterised by proximal muscle weakness and pathognomonic skin rashes. The overall prognosis of juvenile myositis has improved significantly over recent years, but the long-term outcome differs substantially from patient to patient, suggestive of distinct clinical phenotypes with variable responses to treatment. High doses of corticosteroids remain the cornerstone of therapy along with other immunosuppressant therapies depending on disease severity and response...
October 2017: Paediatric Drugs
https://www.readbyqxmd.com/read/28535894/association-between-demyelinating-disease-and-autoimmune-rheumatic-disease-in-a-pediatric-population
#18
Ana Luiza M Amorim, Nadia C Cabral, Fabiane M Osaku, Claudio A Len, Enedina M L Oliveira, Maria Teresa Terreri
INTRODUCTION: Multiple sclerosis (MS) and neuromyelitis optica (NMO) are demyelinating diseases of the central nervous system. Autoimmunity in patients with demyelinating disease and in their families has been broadly investigated and discussed. Recent studies show a higher incidence of rheumatic autoimmune diseases among adult patients with MS or NMO and their families, but there are no studies in the pediatric population. OBJECTIVE: To evaluate an association of MS and NMO with autoimmune rheumatic diseases in pediatric patients...
May 2017: Revista Brasileira de Reumatologia
https://www.readbyqxmd.com/read/28410093/race-income-and-disease-outcomes-in-juvenile-dermatomyositis
#19
COMPARATIVE STUDY
Kathryn Phillippi, Mark Hoeltzel, Angela Byun Robinson, Susan Kim
OBJECTIVE: To determine the relationships among race, income, and disease outcomes in children with juvenile dermatomyositis (JDM). STUDY DESIGN: Data from 438 subjects with JDM enrolled in the Childhood Arthritis and Rheumatology Research Alliance (CARRA) Legacy Registry were analyzed. Demographic data included age, sex, race, annual family income, and insurance status. Clinical outcomes included muscle strength, presence of rash, calcinosis, weakness, physical function, and quality of life measures...
May 2017: Journal of Pediatrics
https://www.readbyqxmd.com/read/28396024/coexisting-juvenile-dermatomyositis-and-sickle-cell-disease-maintaining-a-high-degree-of-suspicion
#20
Ellen Fraint, Maureen Leffler, Corinna L Schultz
Juvenile dermatomyositis is an idiopathic inflammatory myopathy of childhood not previously described in a patient with sickle cell disease. We present a case of an 11-year-old girl with sickle cell disease who was diagnosed subsequently with juvenile dermatomyositis, and highlight the diagnostic and therapeutic challenges of these concurrent chronic diseases.
July 2017: Journal of Pediatrics
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