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juvenile systemic lupus

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https://www.readbyqxmd.com/read/29773269/juvenile-onset-systemic-lupus-erythematosus-jsle-pathophysiological-concepts-and-treatment-options
#1
REVIEW
Christian M Hedrich, Eve M D Smith, Michael W Beresford
The systemic autoimmune/inflammatory condition systemic lupus erythematosus (SLE) manifests before the age of 16 years in 10-20% of all cases. Clinical courses are more severe, and organ complications are more common in patients with juvenile SLE. Varying gender distribution in different age groups and increasing severity with younger age and the presence of monogenic disease in early childhood indicate distinct differences in the pathophysiology of juvenile versus adult-onset SLE. Regardless of these differences, classification criteria and treatment options are identical...
August 2017: Best Practice & Research. Clinical Rheumatology
https://www.readbyqxmd.com/read/29773266/genetic-interferonopathies-an-overview
#2
REVIEW
Despina Eleftheriou, Paul A Brogan
Interferonopathies comprise an expanding group of monogenic diseases characterised by disturbance of the homeostatic control of interferon (IFN)-mediated immune responses. Although differing in the degree of phenotypic expression and severity, the clinical presentation of these diseases shows a considerable degree of overlap, reflecting their common pathogenetic mechanisms. Increased understanding of the molecular basis of these Mendelian disorders has led to the identification of targeted therapies for these diseases, which could also be of potential relevance for non-genetic IFN-mediated diseases such as systemic lupus erythematosus and juvenile dermatomyositis...
August 2017: Best Practice & Research. Clinical Rheumatology
https://www.readbyqxmd.com/read/29721693/nutritional-intervention-in-patients-with-juvenile-systemic-lupus-erythematosus-protective-effect-against-the-increase-in-fat-mass
#3
Thais Ortiz Abad, Roseli Oselka Sarni, Simone Guerra da Silva, Daniele Machado, Fabíola I Suano-Souza, Claudio Arnaldo Len, Maria Teresa Terreri
Patients with juvenile systemic lupus erythematosus (JSLE) usually have an increase in fat mass and decrease in lean body mass. The purpose of this study was to assess the effect of a nutritional intervention on changes in body composition and food consumption of adolescents with JSLE compared with a control group and its variation over time and to assess the association of total fat mass with clinical parameters. This randomized controlled study evaluated 31 girls. Body composition and food intake were evaluated at baseline...
May 2, 2018: Rheumatology International
https://www.readbyqxmd.com/read/29672804/imaging-features-of-the-juvenile-inflammatory-arthropathies
#4
Iwona Sudoł-Szopińska, Lennart Jans, Anne Grethe Jurik, Robert Hemke, Iris Eshed, Nathalie Boutry
We discuss the imaging of several juvenile inflammatory arthropathies including juvenile idiopathic arthritis, juvenile systemic lupus erythematosus, juvenile scleroderma, juvenile dermatomyositis, and chronic recurrent multifocal osteomyelitis. Juvenile idiopathic arthritis is the most common autoimmune chronic systemic disease of connective tissue in children. The remaining systemic juvenile connective tissue diseases are rare. However, they require early diagnosis and initiation of treatment to prevent injury, not only to the musculoskeletal system but also to the internal organs, and even death...
April 2018: Seminars in Musculoskeletal Radiology
https://www.readbyqxmd.com/read/29662164/transcription-factors-operate-across-disease-loci-with-ebna2-implicated-in-autoimmunity
#5
John B Harley, Xiaoting Chen, Mario Pujato, Daniel Miller, Avery Maddox, Carmy Forney, Albert F Magnusen, Arthur Lynch, Kashish Chetal, Masashi Yukawa, Artem Barski, Nathan Salomonis, Kenneth M Kaufman, Leah C Kottyan, Matthew T Weirauch
Explaining the genetics of many diseases is challenging because most associations localize to incompletely characterized regulatory regions. Using new computational methods, we show that transcription factors (TFs) occupy multiple loci associated with individual complex genetic disorders. Application to 213 phenotypes and 1,544 TF binding datasets identified 2,264 relationships between hundreds of TFs and 94 phenotypes, including androgen receptor in prostate cancer and GATA3 in breast cancer. Strikingly, nearly half of systemic lupus erythematosus risk loci are occupied by the Epstein-Barr virus EBNA2 protein and many coclustering human TFs, showing gene-environment interaction...
April 16, 2018: Nature Genetics
https://www.readbyqxmd.com/read/29467038/clinical-predictors-of-proteinuric-remission-following-an-ln-flare-evidence-from-the-uk-jsle-cohort-study
#6
Eve M D Smith, Peng Yin, Andrea L Jorgensen, Michael W Beresford
BACKGROUND: Proteinuria is a well-known risk factor for progression of renal dysfunction in a variety of chronic kidney diseases. In adult-onset Systemic Lupus Erytematosus (SLE) patients with lupus nephritis (LN), proteinuria takes a significant period of time to normalise, with proteinuric remission being associated with improved renal survival and reductions in mortality. The length of time required to attain proteinuric remission has not previously been investigated in Juvenile-onset SLE (JSLE)...
February 21, 2018: Pediatric Rheumatology Online Journal
https://www.readbyqxmd.com/read/29460702/comparison-between-disease-onset-patterns-of-egyptian-juvenile-and-adult-systemic-lupus-erythematosus-single-centre-experience
#7
H H Abdel-Nabi, R A Abdel-Noor
Background Systemic lupus erythematosus (SLE) is a complex autoimmune disease that occurs worldwide in both children and adults, with different disease manifestations, activity and severity between them. Objectives To analyse the difference in disease onset patterns and activity in Egyptian children and adults with SLE. Methods A retrospective cohort study conducted on 298 Egyptian SLE patients, 215 adults (a-SLE) (>18 years) and 83 children (j-SLE) (≤18 years). Disease onset, clinical manifestations and laboratory investigations were recorded...
May 2018: Lupus
https://www.readbyqxmd.com/read/29457995/translation-and-validation-of-the-transition-readiness-assessment-questionnaire-traq
#8
Catherine G Anelli, Claudio A Len, Maria Teresa R A Terreri, Gleice C S Russo, Andreas O Reiff
OBJECTIVE: To translate and validate the Brazilian Portuguese version of the Transition Readiness Assessment Questionnaire in a population of adolescents and young adults with chronic rheumatologic disorders. This questionnaire evaluates the patient's readiness for making the transition from the pediatric health service to adult care. METHODS: The four-phase methodology for the translation and validation of generic questionnaires was followed, including translation, back-translation, pilot testing and clinical validation of the final tool...
February 16, 2018: Jornal de Pediatria
https://www.readbyqxmd.com/read/29457372/clinical-characteristics-and-factors-associated-with-disability-and-impaired-quality-of-life-in-children-with-juvenile-systemic-sclerosis
#9
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/29431056/characteristics-of-systemic-lupus-erythematosus-in-a-sample-of-the-egyptian-population-a-retrospective-cohort-of-1109-patients-from-a-single-center
#10
K T El Hadidi, B M Medhat, N M Abdel Baki, H Abdel Kafy, W Abdelrahaman, A Y Yousri, D H Attia, M Eissa, D El Dessouki, I Elgazzar, F T Elgengehy, N El Ghobashy, H El Hadary, G El Mardenly, H El Naggar, A M El Nahas, R M El Refai, H Allah El Rwiny, R M Elsman, M Galal, S Ghoniem, L A Maged, S M Sally, H Naji, S Saad, M Shaaban, M Sharaf, N Sobhy, R M Soliman, T S El Hadidi
Introduction Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease that can vary among different ethnic and racial groups. Objective The objective of this paper is to study the prevalence of various manifestations of SLE in a sample of the Egyptian population. Patients and methods Information in this study was derived from the medical records of SLE patients who sought medical advice at a private clinic in Cairo from January 1980 to June 2016. Results This study included 1109 SLE patients, of whom 114 (10...
May 2018: Lupus
https://www.readbyqxmd.com/read/29385862/analysis-of-serum-il-38-in-juvenile-onset-systemic-lupus-erythematosus
#11
Yusuke Takeuchi, Tomoko Seki, Norimoto Kobayashi, Kenji Sano, Tomonari Shigemura, Hisashi Shimojo, Kazuhiko Matsumoto, Kazunaga Agematsu
No abstract text is available yet for this article.
February 23, 2018: Modern Rheumatology
https://www.readbyqxmd.com/read/29371779/hyperferritinemia-in-hemophagocytic-lymphohistiocytosis-a-single-institution-experience-in-pediatric-patients
#12
Surupa Basu, Biplab Maji, Santanu Barman, Apurba Ghosh
Hemophagocytic lymphohistiocytosis (HLH) is an inflammatory condition that may run a rapid fatal course and calls for prompt diagnosis. Early intervention with steroids and other immunosuppressive drugs can contain the disease process and favours positive outcome. Ferritin ≥500 ng/ml is a HLH diagnostic criterion. We evaluated the diagnostic potential of admission ferritin, in children with HLH. Pediatric patients of a referral teaching hospital from Feb 2010-Oct 2013 having been investigated for ferritin on admission were included...
January 2018: Indian Journal of Clinical Biochemistry: IJCB
https://www.readbyqxmd.com/read/29366725/performance-of-the-2012-systemic-lupus-international-collaborating-clinics-classification-criteria-versus-the-1997-american-college-of-rheumatology-classification-criteria-in-adult-and-juvenile-systemic-lupus-erythematosus-a-systematic-review-and-meta-analysis
#13
REVIEW
Esther A R Hartman, Annet van Royen-Kerkhof, Johannes W G Jacobs, Paco M J Welsing, Ruth D E Fritsch-Stork
OBJECTIVE: To evaluate the performance in classifying systemic lupus erythematosus by the 2012 Systemic Lupus International Collaborating Clinics criteria (SLICC'12), versus the revised American College of Rheumatology criteria from 1997 (ACR'97) in adult and juvenile SLE patients. METHODS: A systematic literature search was conducted in PubMed and Embase for studies comparing SLICC'12 and ACR'97 with clinical diagnosis. A meta-analysis was performed to estimate the sensitivity and specificity of SLICC'12 and ACR'97...
March 2018: Autoimmunity Reviews
https://www.readbyqxmd.com/read/29353100/vitamin-d-and-juvenile-systemic-lupus-erythematosus-lights-shadows-and-still-unresolved-issues
#14
REVIEW
Stefano Stagi, Donato Rigante
Systemic lupus erythematosus (SLE) and juvenile SLE (jSLE) are autoimmune disorders naturally associated with several genetic, environmental, hormonal, and immunological contributing factors. It has been assumed that vitamin D deficiency may have a role in the immune activation of patients with SLE and play an active part in many comorbidities and even complications. A host of clinical studies suggested that vitamin D exerts inhibitory effects on many immunological abnormalities associated with SLE, also in children and adolescents, while different reports have hypothesized that vitamin D may be associated with accelerated cardiovascular disease in SLE...
March 2018: Autoimmunity Reviews
https://www.readbyqxmd.com/read/29343274/coexistent-sickle-cell-anemia-and-autoimmune-disease-in-eight-children-pitfalls-and-challenges
#15
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/29333701/the-childhood-arthritis-and-rheumatology-research-alliance-consensus-treatment-plans-toward-comparative-effectiveness-in-the-pediatric-rheumatic-diseases
#16
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/29320971/the-effect-of-nutritional-intervention-on-the-lipid-profile-and-dietary-intake-of-adolescents-with-juvenile-systemic-lupus-erythematosus-a-randomized-controlled-trial
#17
S G L da Silva, M T Terreri, T T O Abad, D Machado, F L A Fonseca, S Hix, F I Suano-Souza, R O S Sarni, C A Len
Objective This study sought to evaluate the effects of a nutritional intervention on the lipid metabolism biomarkers associated with cardiovascular risk, and their variation over time, in juvenile systemic lupus erythematosus (JSLE) patients. This study also investigated the relationships between these biomarkers and dietary intake, nutritional status, disease variables, and medication used. Methods A total of 31 10- to 19-year-old female adolescents with JSLE for at least six months were analyzed. The participants were randomly allocated to two groups: nutritional intervention or control...
April 2018: Lupus
https://www.readbyqxmd.com/read/29276866/association-of-stat4-gene-single-nucleotide-polymorphisms-with-iranian-juvenile-onset-systemic-lupus-erythematosus-patients
#18
Arash Salmaninejad, Mahdi Mahmoudi, Saeed Aslani, Shiva Poursani, Vahid Ziaee, Nima Rezaei
Salmaninejad A, Mahmoudi M, Aslani S, Poursani S, Ziaee V, Rezaei N. Association of STAT4 gene single nucleotide polymorphisms with Iranian juvenile-onset systemic lupus erythematosus patients. Turk J Pediatr 2017; 59: 144-149. Juvenile-onset systemic lupus erythematosus (JSLE) is a complex autoimmune disease, characterized by multi-organ involvement. Single nucleotide polymorphisms (SNPs) of signal transducer and activator of transcription 4 (STAT4) gene have been reported to have relationship with the risk of several autoimmune diseases...
2017: Turkish Journal of Pediatrics
https://www.readbyqxmd.com/read/29243035/systemic-lupus-erythematosus-and-ocular-involvement-an-overview
#19
REVIEW
Rosanna Dammacco
Systemic lupus erythematosus (SLE) is a multisystem autoimmune disease of undefined etiology and with remarkably heterogeneous clinical features. Virtually any organ system can be affected, including the eye. SLE-related eye involvement can be diagnosed in approximately one-third of the patients and is usually indicative of disease activity. An early diagnosis and the adoption of suitable therapeutic measures are necessary to prevent sight-threatening consequences, especially in patients with juvenile SLE. Periocular lesions, such as eyelid involvement and orbital inflammation, are relatively rare and, in case of orbital masses, may require a biopsy control...
December 14, 2017: Clinical and Experimental Medicine
https://www.readbyqxmd.com/read/29233038/juvenile-systemic-lupus-erythematosus-in-turkey-demographic-clinical-and-laboratory-features-with-disease-activity-and-outcome
#20
S Sahin, A Adrovic, K Barut, N Canpolat, Y Ozluk, I Kilicaslan, S Caliskan, L Sever, O Kasapcopur
Objectives This paper aims to assess in a retrospective fashion the clinical and laboratory features, severity and outcome of juvenile systemic lupus erythematosus (jSLE) from a referral center in Turkey. Methods We have included all jSLE patients ( n = 92) diagnosed according to the revised American College of Rheumatology 1997 criteria between January 2004 and January 2017. Results The most prevalent clinical feature in our cohort was mucocutaneous manifestations (97.8%), followed by constitutional (81...
March 2018: Lupus
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