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https://www.readbyqxmd.com/read/29881250/stat4-gene-polymorphism-in-two-major-autoimmune-diseases-multiple-sclerosis-and-juvenile-onset-systemic-lupus-erythematosus-and-its-relation-to-disease-severity
#1
Rania S Nageeb, Alaa A Omran, Ghada S Nageeb, Manal A Yousef, Yassir A A Mohammad, Amal Fawzy
Background: Multiple sclerosis (MS) and systemic lupus erythematosus (SLE) are chronic autoimmune mediated diseases with strong genetic and environmental components. The aim of this study is to evaluate the association of STAT4 gene polymorphism with multiple sclerosis (MS) and juvenile onset systemic lupus erythematosus (JO-SLE) and its relation to disease severity. Methods: Group 1 consisted of 40 MS patients while group 2 included 40 JO-SLE patients. Forty healthy volunteers (controls) were included in this study...
2018: The Egyptian journal of neurology, psychiatry and neurosurgery
https://www.readbyqxmd.com/read/29773269/juvenile-onset-systemic-lupus-erythematosus-jsle-pathophysiological-concepts-and-treatment-options
#2
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/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/29467038/clinical-predictors-of-proteinuric-remission-following-an-ln-flare-evidence-from-the-uk-jsle-cohort-study
#4
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/29457995/translation-and-validation-of-the-transition-readiness-assessment-questionnaire-traq
#5
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/29353100/vitamin-d-and-juvenile-systemic-lupus-erythematosus-lights-shadows-and-still-unresolved-issues
#6
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/29320971/the-effect-of-nutritional-intervention-on-the-lipid-profile-and-dietary-intake-of-adolescents-with-juvenile-systemic-lupus-erythematosus-a-randomized-controlled-trial
#7
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
#8
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/29233038/juvenile-systemic-lupus-erythematosus-in-turkey-demographic-clinical-and-laboratory-features-with-disease-activity-and-outcome
#9
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
https://www.readbyqxmd.com/read/29209549/macrophage-activation-syndrome-a-report-of-two-cases-and-a-literature-review
#10
Asaad Alkoht, Ibrahem Hanafi, Basheer Khalil
Macrophage activation syndrome (MAS) is a severe, potentially fatal condition that may complicate autoimmune diseases, and it belongs to hemophagocytic lymphohistiocytosis (HLH) disorders. MAS occurs in adults and children. However, it is rare in juvenile systemic lupus erythematosus (jSLE), and it is extremely rare to be the initial presentation of jSLE. Here, we report two patients with juvenile SLE who initially presented with MAS. One of the two patients is 4 years old. This is the youngest reported patient to our knowledge...
2017: Case Reports in Rheumatology
https://www.readbyqxmd.com/read/29199882/association-study-of-mecp2-gene-single-nucleotide-polymorphisms-in-juvenile-onset-systemic-lupus-erythematosus-patients-from-iran
#11
Mahdi Mahmoudi, Saeed Aslani, Elham Hamzeh, Vahid Ziaee, Shiva Poursani, Mohammad Hossein Nicknam, Nima Rezaei
INTRODUCTION: Juvenile-onset systemic lupus erythematosus is a multigenic autoimmune disorder. Polymorphisms of MECP2 gene have been reported to increase the risk of adult-onset SLE. In this study, we aimed to analyze if MECP2 gene polymorphisms could impress the proneness to JSLE in Iranian population. MATERIAL AND METHODS: Polymorphisms of MECP2 gene were genotyped in 50 Iranian JSLE patients and 426 matched healthy controls employing the real-time PCR allelic discrimination technique...
December 2017: Fetal and Pediatric Pathology
https://www.readbyqxmd.com/read/28956469/alpha-beta-double-negative-t-cells-in-children-with-systemic-lupus-erythematosus-the-relation-to-disease-activity-and-characteristics
#12
Zeinab A El-Sayed, Rasha H El-Owaidy, Neama L Mohamed, Beshoy A Shehata
OBJECTIVES: We aimed to investigate alpha beta double negative (αβ DN) T-cell percentages in juvenile systemic lupus erythematosus (JSLE) and their relation to disease activity and organ involvement. METHODS: This prospective study was carried out over a period of 12 months and included 21 JSLE patients and 20 healthy matched controls. SLE disease activity index 2000 (SLEDAI-2K) scores were recorded in addition to αβ DN T-cell percentages measurement at enrollment and after remission...
September 28, 2017: Modern Rheumatology
https://www.readbyqxmd.com/read/28887660/urinary-interleukin-22-binding-protein-as-a-marker-of-lupus-nephritis-in-egyptian-children-with-juvenile-systemic-lupus-erythematosus
#13
Ahmed Mohamed Mahmoud Badr, Yomna Farag, Maie Abdelshafy, Nermine Magdi Riad
Juvenile systemic lupus erythematosus (JSLE) is a multi-system autoimmune inflammatory disease. Generally, 60% of patients will develop lupus nephritis (LN); thus, early recognition and treatment is associated with better outcome. Interleukin 22 (IL-22) is involved in tissue inflammation and is regulated by interleukin 22 binding protein (IL-22BP). This study aimed to use IL-22BP as a non-invasive marker for disease activity in JSLE and LN. This is a cross-sectional study conducted on 82 subjects: 51 JSLE patients and 31 healthy controls of matched age and gender...
February 2018: Clinical Rheumatology
https://www.readbyqxmd.com/read/28704174/evaluation-of-pulmonary-artery-pressure-in-patients-with-juvenile-systemic-lupus-erythematosus-jsle
#14
Amra Adrovic, Reyhan Dedeoglu, Sezgin Sahin, Kenan Barut, Aida Koka, Dicle Cengiz, Funda Oztunc, Ozgur Kasapcopur
Juvenile systemic lupus erythematosus (jSLE) is a chronic multisystemic autoimmune disease. Previous studies among adults have shown impaired right ventricular (RV) function in patients with SLE. Also, these patients may develop pulmonary artery hypertension (PAH), which is one of the most threatening complications of SLE. Nevertheless, studies on PAH among jSLE patients are still rare. The aim of this study was to assess the RV function in jSLE patients by Doppler echocardiography (Echo Doppler). We also estimated pulmonary artery systolic pressure (PASP) and mean pulmonary artery pressure (mPAP) in these patients...
February 20, 2018: Bosnian Journal of Basic Medical Sciences
https://www.readbyqxmd.com/read/28628467/juvenile-and-adult-onset-systemic-lupus-erythematosus-a-comparative-study-in-a-large-cohort-from-the-spanish-society-of-rheumatology-lupus-registry-relesser
#15
MULTICENTER STUDY
Vicenç Torrente-Segarra, Tarek Carlos Salman Monte, Iñigo Rúa-Figueroa, Fernando Sánchez-Alonso, Francisco Javier López-Longo, María Galindo-Izquierdo, Jaime Calvo-Alén, Alejandro Olivé-Marqués, Jesús Ibañez-Ruán, Loreto Horcada, Ana Sánchez-Atrio, Carlos Montilla, Rafael Benito Melero González, Elvira Díez-Álvarez, Victor Martinez-Taboada, José Luis Andreu, Olaia Fernández-Berrizbeitia, José Ángel Hernández-Beriain, Marian Gantes, Blanca Hernández-Cruz, Ángela Pecondón-Español, Carlos Marras, Gema Bonilla, José M Pego-Reigosa
OBJECTIVES: We aimed to describe juvenile-onset systemic lupus erythematosus (jSLE) features and to establish its differences compared to adult-onset SLE (aSLE) from a large national database. METHODS: Data from patients (≥4 ACR criteria) included in Spanish Society of Rheumatology Lupus Registry (RELESSER) were analysed. Sociodemographic, clinical, serological, activity, treatment, cumulative damage, comorbidities and severity data were collected. Patients with disease onset <18 years were described and compared to those with disease onset ≥18 years...
November 2017: Clinical and Experimental Rheumatology
https://www.readbyqxmd.com/read/28545118/atopy-in-children-with-juvenile-systemic-lupus-erythematosus-is-associated-with-severe-disease
#16
Ruru Guo, Yanqing Zhou, Liangjing Lu, Lanfang Cao, Junjia Cao
The influence of co-existing atopy on the prognosis of juvenile systemic lupus erythematosus (JSLE) was assessed in this study. Patients diagnosed with JSLE between October 2005 and April 2016 were enrolled in a prospective study and followed up for 2 years. Management of patients was evaluated using the systemic lupus erythematosus disease activity index 2000 (SLEDAI-2K) score and laboratory variables. Eighty JSLE patients were enrolled at diagnosis and divided into those with (n = 35) and without (n = 45) atopy...
2017: PloS One
https://www.readbyqxmd.com/read/28506339/-effect-of-allergic-rhinitis-on-disease-condition-and-treatment-in-patients-with-juvenile-onset-systemic-lupus-erythematosus
#17
RANDOMIZED CONTROLLED TRIAL
Yan-Qing Zhou, Lan-Fang Cao, Ru-Ru Guo, Le-Ping Li, Hui-Fen Fang
OBJECTIVE: To investigate the effect of allergic rhinitis (AR) and its intervention on disease condition and medications in patients with juvenile-onset systemic lupus erythematosus (JSLE). METHODS: The clinical data of 96 children diagnosed with JSLE were collected, and according to the presence or absence of AR or other allergic diseases, they were divided into AR group (n=44), non-AR group (n=20), and non-allergic group (n=32). The children in the AR group were randomly administered with or without intervention (n=22 each)...
May 2017: Zhongguo Dang Dai Er Ke za Zhi, Chinese Journal of Contemporary Pediatrics
https://www.readbyqxmd.com/read/28477309/oral-ulcers-in-juvenile-onset-systemic-lupus-erythematosus-a-review-of-the-literature
#18
REVIEW
Pongsawat Rodsaward, Titipong Prueksrisakul, Tawatchai Deekajorndech, Steven W Edwards, Michael W Beresford, Direkrit Chiewchengchol
Oral ulcers are the most common mucosal sign in juvenile-onset systemic lupus erythematosus (JSLE). The ulcers are one of the key clinical features; however, the terminology of oral ulcers, especially in JSLE patients, is often vague and ill-defined. In fact, there are several clinical manifestations of oral ulcers in JSLE, and some lesions occur when the disease is active, indicating that early management of the disease should be started. Oral ulcers are classified as lupus erythematosus (LE) specific, where the lesional biopsy shows a unique pattern of mucosal change in LE, and LE nonspecific, where the ulcers and their histopathological findings can be found in other oral diseases...
December 2017: American Journal of Clinical Dermatology
https://www.readbyqxmd.com/read/28385126/do-classic-blood-biomarkers-of-jsle-identify-active-lupus-nephritis-evidence-from-the-uk-jsle-cohort-study
#19
E M D Smith, A L Jorgensen, M W Beresford
Background Lupus nephritis (LN) affects up to 80% of juvenile-onset systemic lupus erythematosus (JSLE) patients. The value of commonly available biomarkers, such as anti-dsDNA antibodies, complement (C3/C4), ESR and full blood count parameters in the identification of active LN remains uncertain. Methods Participants from the UK JSLE Cohort Study, aged <16 years at diagnosis, were categorized as having active or inactive LN according to the renal domain of the British Isles Lupus Assessment Group score...
January 1, 2017: Lupus
https://www.readbyqxmd.com/read/28378099/increased-serum-sfas-strail-and-reduced-sfasl-in-juvenile-onset-systemic-lupus-erythematosus
#20
Bernadete L Liphaus, Maria Helena B Kiss, Solange Carrasco, Patrícia Palmeira, Claudia Goldenstein-Schainberg, Magda Carneiro-Sampaio
The aims of this study were to assess serum Fas, FasL, TRAIL, and Bcl-2 levels in patients with juvenile-onset systemic lupus erythematosus (JSLE) and to evaluate their relations with disease activity parameters and nephritis. Forty-eight JSLE patients, 33 juvenile idiopathic arthritis (JIA, inflammatory controls) patients and 40 healthy controls were enrolled. sFas, sFasL, sTRAIL, and sBcl-2 serum levels were measured by ELISA. Disease activity parameters included SLEDAI score, ESR, anti-dsDNA antibodies, C3, and C4 levels...
December 2017: Clinical Rheumatology
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