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https://www.readbyqxmd.com/read/29333701/the-childhood-arthritis-rheumatology-research-alliance-consensus-treatment-plans-towards-comparative-effectiveness-in-the-pediatric-rheumatic-diseases
#1
REVIEW
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 approaches. Innovative comparative effectiveness approaches are needed to efficiently study treatment approaches 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/29324237/dna-induction-of-mdm2-promotes-proliferation-of-human-renal-mesangial-cells-and-alters-peripheral-b-cells-subsets-in-pediatric-systemic-lupus-erythematosus
#2
Chen-Xing Zhang, Ji Chen, Li Cai, Jing Wu, Jia-Yuan Wang, Lan-Fang Cao, Wei Zhou, Tong-Xin Chen
The study is aimed to investigate the role of MDM2 in the pathogenesis of lupus nephritis (LN) in pediatric SLE (pSLE). We confirmed that MDM2 expression was increased in peripheral blood mononuclear cells (PBMCs) as well as renal specimen of SLE compared with that of controls by western blot and immunofluorescence staining. Percentage of apoptotic and necrotic CD4+T, CD8+T and B cells were detected by flow cytometry respectively and levels of plasma cell free DNA (cfDNA) were quantified in SLE and healthy controls (HC)...
January 8, 2018: Molecular Immunology
https://www.readbyqxmd.com/read/29320972/rituximab-use-in-pediatric-lupus-anticoagulant-hypoprothrombinemia-syndrome-report-of-three-cases-and-review-of-the-literature
#3
K Cetin Gedik, S Siddique, C L Aguiar
Lupus anticoagulant hypoprothrombinemia syndrome (LA-HPS) is a rare condition that may predispose both to thrombosis and bleeding due to positive lupus anticoagulant (LA) and factor II (FII) deficiency. It can be seen in association with infections or systemic lupus erythematosus (SLE) and may require glucocorticoids (GCs) and/or immunosuppressive medications. Pediatric LA-HPS cases in the literature and three cases that received only rituximab (RTX) for LA-HPS (in addition to GCs) at two institutions between January 2010 and June 2017 were analyzed descriptively...
January 1, 2018: Lupus
https://www.readbyqxmd.com/read/29318909/how-to-discern-folliculotropic-mycosis-fungoides-from-follicular-mucinosis-using-a-pediatric-case
#4
Drew A Emge, Daniel J Lewis, Phyu P Aung, Madeleine Duvic
Folliculotropic mycosis fungoides (FMF) is a variant of mycosis fungoides (MF) with folliculotropic, atypical lymphocytes that may or may not have mucin deposition surrounding the hair follicle. Follicular mucinosis (FM) is a primary or secondary finding in FMF, lupus, or collagen vascular diseases that is only a histological process of mucin deposition surrounding the hair follicles. We present a case of a 6-year-old boy who had features of both FMF and primary follicular mucinosis (PFM). The case reveals key insights on FMF with concurrent FM in pediatric patients and how to differentiate between FMF and PFM...
January 1, 2018: Journal of Cutaneous Medicine and Surgery
https://www.readbyqxmd.com/read/29314753/functional-mannose-binding-lectin-levels-in-patients-with-pediatric-onset-systemic-lupus-erythematosus-in-remission
#5
Siyaram Didel, Deepti Suri, Amit Rawat, Surjit Singh
BACKGROUND: Complement system plays a crucial role in the pathogenesis of systemic lupus erythematosus (SLE). Excessive activation of complement causes tissue injury and SLE occurrence is typically associated with congenital deficiencies of the early components of the classical complement pathway. Mannose-binding lectin (MBL) is recognized as important causative factor in the etio-pathogenesis of SLE and MBL-2 gene has evolved as a candidate gene for SLE susceptibility. AIM: This study was done to assess the functional MBL (fMBL) level in SLE patients in remission and its co-relation with disease severity and outcome...
January 4, 2018: International Journal of Rheumatic Diseases
https://www.readbyqxmd.com/read/29301470/risk-of-complications-of-ultrasound-guided-renal-biopsy-for-adult-and-pediatric-patients-with-systemic-lupus-erythematosus
#6
Y S Sun, I T Sun, H K Wang, A H Yang, C Y Tsai, C J Huang, D F Huang, C C Lai
Objective The objective of this paper is to identify the risk of complications of real-time ultrasound-guided renal biopsy in adult and pediatric patients with systemic lupus erythematosus (SLE). Materials and methods This retrospective study examined outcomes of 296 renal biopsy procedures in 275 SLE patients. Imaging-confirmed symptomatic hematoma was regarded as a major complication when intervention (blood transfusion, angiographic embolization, or surgery) was required or as a minor complication otherwise...
January 1, 2018: Lupus
https://www.readbyqxmd.com/read/29289551/role-of-tubulointerstitial-lesions-in-predicting-renal-outcome-among-pediatric-onset-lupus-nephritis-a-retrospective-cohort-study
#7
Chao-Yi Wu, Hui-Ping Chien, Huang-Yu Yang, Tsung-Chieh Yao, Min-Hua Tseng, Mei-Chin Yu, Kuo-Wei Yeh, Jing-Long Huang
BACKGROUND: Raising evidence suggested a prognostic utility of tubulointerstitial lesions in lupus nephritis (LN). The exact prevalence of tubulointerstitial abnormalities and its predictive value among pediatric onset systemic lupus erythematous (pSLE) cases, however, remained unknown. METHODS: Sixty-seven pSLE subjects diagnosed with LN with initial renal samples available were enrolled and followed for an average of 6.49 ± 3.06 years. Renal histology was evaluated according to the International Society of Nephrology/Renal Pathology Society classification, National Institute of Health classification and tubulointerstitial activity index (TIAI)...
December 15, 2017: Journal of Microbiology, Immunology, and Infection, Wei Mian Yu Gan Ran za Zhi
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/29224679/a-review-on-sle-and-malignancy
#9
REVIEW
May Y Choi, Kelsey Flood, Sasha Bernatsky, Rosalind Ramsey-Goldman, Ann E Clarke
Systemic lupus erythematosus (SLE) is a chronic, systemic autoimmune disease characterized by autoantibody production, complement activation, and immune complex deposition. It predominantly affects young and middle-aged women. While improvements in the diagnosis and treatment of SLE have altered prognosis, morbidity and mortality rates remain higher than the general population. In addition to renal injury, cardiovascular disease, and infection, malignancy is known to be a significant cause of death in this population...
June 2017: Best Practice & Research. Clinical Rheumatology
https://www.readbyqxmd.com/read/29214394/serious-adverse-events-associated-with-anti-tumor-necrosis-factor-alpha-agents-in-pediatric-onset-inflammatory-bowel-disease-and-juvenile-idiopathic-arthritis-in-a-real-life-setting
#10
Serena Pastore, Samuele Naviglio, Arianna Canuto, Loredana Lepore, Stefano Martelossi, Alessandro Ventura, Andrea Taddio
OBJECTIVES: Anti-tumor necrosis factor alpha (anti-TNF-α) agents are generally well tolerated, yet they can be associated with serious adverse events (SAEs) in a minority of patients. We examined the incidence of SAEs in a pediatric referral center for chronic rheumatologic and gastroenterological inflammatory disorders. METHODS: Retrospective analysis of SAEs occurring during treatment with anti-TNF-α agents in patients with juvenile idiopathic arthritis (JIA) (n = 78)  or pediatric-onset inflammatory bowel disease (IBD) (n = 105) seen at the Institute for Maternal and Child Health IRCCS "Burlo Garofolo" in Trieste, Italy, between June 2001 and February 2016...
December 6, 2017: Paediatric Drugs
https://www.readbyqxmd.com/read/29208331/autoimmune-and-medication-induced-lymphadenopathies
#11
REVIEW
Alejandro A Gru, Dennis P O'Malley, Aliso Viejo
This article will provide a discussion of some common autoimmune disorders that could affect the lymph nodes and potentially mimic B and T-cell lymphomas. Some of these disorders are more characteristic of individuals in the pediatric age group (autoimmune lymphoproliferative syndrome, Kawasaki disease), while others present in older individuals (rheumatoid arthritis, lupus erythematosus, sarcoidosis). A common finding that groups all of these disorders together is the overall relative preservation of the architecture, a feature that can be particularly helpful to distinguish them from many B and T-cell lymphomas...
November 28, 2017: Seminars in Diagnostic Pathology
https://www.readbyqxmd.com/read/29152588/the-delivery-science-rapid-analysis-program-a-research-and-operational-partnership-at-kaiser-permanente-northern-california
#12
Julie A Schmittdiel, Rick Dlott, Joseph D Young, Michael B Rothmann, Wendy Dyer, Alyce S Adams
Introduction: Health care researchers and delivery system leaders share a common mission to improve health care quality and outcomes. However, differing timelines, incentives, and priorities are often a barrier to research and operational partnerships. In addition, few funding mechanisms exist to generate and solicit analytic questions that are of interest to both research and to operations within health care settings, and provide rapid results that can be used to improve practice and outcomes...
October 2017: Learning Health Systems
https://www.readbyqxmd.com/read/29101552/long-term-renal-outcome-in-pediatric-glomerulonephritis-associated-with-crescent-formation
#13
Pornpimol Rianthavorn, Manunya Chacranon
BACKGROUND: Information on long-term renal outcome of pediatric glomerulonephritis associated with crescent formation is limited. A single center retrospective study was conducted to assess long-term renal survival and to determine whether the 2010 classification for antineutrophil cytoplasmic antibody (ANCA)-associated glomerulonephritis can predict renal outcome in pediatric glomerulonephritis associated with crescent formation. METHODS: Biopsy and clinical data of children, aged ≤ 18 years with ≥ 10 glomeruli and ≥ 10% crescentic glomeruli during January 1998 to December 2015, were reviewed...
November 3, 2017: Clinical and Experimental Nephrology
https://www.readbyqxmd.com/read/29078111/markers-of-coagulation-activation-inflammation-and-fibrinolysis-as-predictors-of-poor-outcomes-after-pediatric-venous-thromboembolism-a-systematic-review-and-meta-analysis
#14
REVIEW
Ayesha Zia, Joy Russell, Ravi Sarode, Surendranath R Veeram, Shellie Josephs, Kendra Malone, Song Zhang, Janna Journeycake
BACKGROUND: Sequelae of venous thromboembolism (VTE) in children include recurrence, development of post thrombotic syndrome (PTS) when venous return from a limb is affected and chronic thromboembolic pulmonary hypertension (CTEPH) after pulmonary embolism. Identification of laboratory-based risk factors may be useful for individualized risk assessment for VTE sequelae. Coagulation activation and inflammation may contribute to their pathophysiology. We performed a systematic review to investigate the association between biomarkers of coagulation activation, inflammation and fibrinolysis and adverse VTE outcomes in children and young adults...
October 7, 2017: Thrombosis Research
https://www.readbyqxmd.com/read/29067848/distinct-clinical-correlates-of-immune-thrombocytopenic-purpura-at-diagnosis-of-childhood-onset-and-adult-sle
#15
Gladys Cherres Xavier Esteves, Natali Weniger Spelling Gormezano, Oriany L Pereira, David Kern, Clovis Almeida Silva, Rosa Maria Rodrigues Pereira, Katia Tomie Kozu, Eloisa Bonfá, Nadia Emi Aikawa
OBJECTIVES: To compare clinical and laboratorial features between childhood-onset systemic lupus erythematosus (cSLE) and adult SLE (aSLE) at concomitant diagnosis of immune thrombocytopenic purpura (ITP). METHODS: This study evaluated 56 cSLE and 73 aSLE patients regularly followed at Pediatric and Rheumatology Divisions of the same University hospital with ITP (platelets count <100,000/mm3 in the absence of other causes) at lupus onset. RESULTS: Median current age was 11...
October 25, 2017: Modern Rheumatology
https://www.readbyqxmd.com/read/29050884/initial-digital-vasculitis-in-a-large-multicenter-cohort-of-childhood-onset-systemic-lupus-erythematosus
#16
Ana Paula Sakamoto, Clovis Artur Silva, Marco Felipe Castro da Silva, Anandreia Simões Lopes, Gleice Clemente Souza Russo, Adriana Maluf Elias Sallum, Katia Kozu, Eloisa Bonfá, Claudia Saad-Magalhães, Rosa Maria Rodrigues Pereira, Claudio Arnaldo Len, Maria Teresa Terreri
OBJECTIVES: To assess clinical digital vasculitis (DV) as an initial manifestation of childhood-onset systemic lupus erythematosus (cSLE) within a large population. METHODS: Multicenter cross-sectional study including 852 cSLE patients (ACR criteria) followed in ten Pediatric Rheumatology centers in São Paulo State, Brazil. RESULTS: DV was observed in 25/852 (3%) cSLE patients. Periungual hemorrhage was diagnosed in 12 (48%), periungual infarction in 7 (28%), tip finger ulceration in 4 (16%), painful nodules in 1 (4%) and gangrene in 1 (4%)...
October 16, 2017: Revista Brasileira de Reumatologia
https://www.readbyqxmd.com/read/29042869/clinical-characteristics-of-hypertensive-encephalopathy-in-pediatric-patients
#17
Chang Hoon Ahn, Seung-A Han, Young Hwa Kong, Sun Jun Kim
PURPOSE: The aim of this study was to assess the clinical characteristics of hypertensive encephalopathy according to the underlying etiologies in children. METHODS: We retrospectively evaluated 33 pediatric patients who were diagnosed as having hypertensive encephalopathy in Chonbuk National University Children's Hospital. Among the patients, 18 were excluded because of incomplete data or because brain magnetic resonance imaging (MRI) was not performed. Finally, 17 patients were enrolled and divided into a renal-origin hypertension group and a non-renal-origin hypertension group according to the underlying cause...
August 2017: Korean Journal of Pediatrics
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
#18
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/29020480/bilateral-hemorrhagic-retinopathy-with-roth-spots-in-pediatric-onset-systemic-lupus-erythematosus-and-associated-thrombocytopenia-a-case-report-and-review-of-literature
#19
Jie Zhang, Yingbai Chen, Zikui Yu, Lin Liu
No abstract text is available yet for this article.
October 11, 2017: Ocular Immunology and Inflammation
https://www.readbyqxmd.com/read/28990859/hematoxylin-bodies-in-pediatric-bone-marrow-aspirates-and-their-utility-in-the-diagnosis-of-systemic-lupus-erythematosus
#20
Min Xu, Karen M Chisholm, Guang Fan, Anne M Stevens, Joe C Rutledge
In our recent case report, the finding of lupus erythematosus (LE) cells in a bone marrow aspirate led to the diagnosis of systemic lupus erythematosus (SLE) and appropriate treatment, although the patient was not clinically suspected to have SLE. To determine whether LE cells are present in the bone marrow aspirates of SLE patients, but overlooked in routine bone marrow morphology review, bone marrow aspirates from 30 pediatric patients (15 with SLE and 15 with other diagnoses) evaluated by rheumatologists were reviewed...
January 1, 2017: Pediatric and Developmental Pathology
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