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https://www.readbyqxmd.com/read/29020719/diagnosis-and-management-of-nephrotic-syndrome
#1
Oonagh McCloskey, Alexander P Maxwell
Nephrotic syndrome is defined by a triad of clinical features: oedema, substantial proteinuria (> 3.5 g/24 hours) and hypoalbuminaemia (< 30 g/L). It is often associated with hyperlipidaemia, thromboembolism and an increased risk of infection. Nephrotic syndrome develops following pathological injury to renal glomeruli. This may be a primary problem, with a disease specific to the kidneys, or secondary to a systemic disorder such as diabetes mellitus. The most common cause in children is minimal change glomerulonephritis...
February 2017: Practitioner
https://www.readbyqxmd.com/read/28978692/the-erm-protein-moesin-regulates-cd8-regulatory-t-cell-homeostasis-and-self-tolerance
#2
Hiroki Satooka, Daisuke Nagakubo, Tomomi Sato, Takako Hirata
The ezrin-radixin-moesin (ERM) proteins are a family of membrane-associated proteins that link membrane proteins with actin filaments in the cell cortex and regulate many cellular processes, including cell shape determination, membrane transport, and signal transduction. Lymphocytes predominantly express two ERM members, ezrin and moesin. Mutations in the moesin gene in humans are associated with primary immunodeficiency with profound lymphopenia, and moesin-deficient mice exhibit a similar lymphopenia phenotype...
October 4, 2017: Journal of Immunology: Official Journal of the American Association of Immunologists
https://www.readbyqxmd.com/read/28955333/accelerated-systemic-autoimmunity-in-the-absence-of-somatic-hypermutation-in-564igi-a-mouse-model-of-systemic-lupus-with-knocked-in-heavy-and-light-chain-genes
#3
Gabrielle McDonald, Carlos O Medina, Monika Pilichowska, John F Kearney, Reiko Shinkura, Erik Selsing, Henry H Wortis, Tasuku Honjo, Thereza Imanishi-Kari
564Igi mice have knocked-in immunoglobulin (Ig) heavy (H) and light (L) chain genes that encode an autoantibody recognizing RNA. Previously, we showed that these mice produce pathogenic IgG autoantibodies when activation-induced deaminase (AID) is expressed in pre-B and immature B cells but not when it is expressed only in mature B cells. AID has two functions; it is necessary for somatic hypermutation (SHM) and class switch recombination (CSR). To determine the role of each of these functions in the generation of pathogenic autoantibodies, we generated 564Igi mice that carry a mutant AID-encoding gene, Aicda (Aicda(G23S)), which is capable of promoting CSR but not SHM...
2017: Frontiers in Immunology
https://www.readbyqxmd.com/read/28949280/childhood-nephrotic-syndrome-in-tropical-africa-then-and-now
#4
Wasiu A Olowu, Adebowale Ademola, Adebukola B Ajite, Yauba M Saad
This descriptive and comparative review examines the changing epidemiology, treatment, renal and patient outcome of childhood nephrotic syndrome (NS) in tropical Africa (TpAfr). In the 1960s to 1980s, corticosteroid-resistant non-minimal change disease (nMCD) including quartan malaria nephropathy (QMN) was the dominant renal histopathology type. The overall incidence of NS was 0.35-1.34% of hospital admissions. Median age at onset of NS ranged between 4.0 and 12.0 years while the mean (SD) age range was 5.8 (3...
September 26, 2017: Paediatrics and International Child Health
https://www.readbyqxmd.com/read/28937057/outcome-of-childhood-lupus-nephritis-in-saudi-children
#5
Sulaiman Mohammed Al-Mayouf, Ali AlAmeer, Areej Alfattani, Abdullah Alsonbul
Our aim in this study is to report the long-term renal outcome of a cohort of Saudi children with systemic lupus erythematosus (SLE). All patients with childhood lupus nephritis (cLN) proved by renal biopsy seen between January 2000 and June 2015 were reviewed. The renal outcome was assessed according to serum creatinine level, protein/creatinine ratio at the last follow-up visit, and/or evidence of renal impairment during follow-up period and end-stage renal disease (ESRD). Additional outcome measures include accrual damage measured by pediatric adaptation of the Systemic Lupus International Collaborating Clinics/American College of Rheumatology Damage Index (pSDI), and death related to SLE was determined...
September 2017: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/28933339/incidence-and-outcome-of-lupus-nephritis-in-french-polynesia%C3%A2
#6
Antoine Delarche, Catherine Lumbroso, Alain Fournier, Ronan Delaval, Jacques Pourrat, Pascale Testevuide, Sylvie Leou, Christophe Cordonnier, Dominique Chauveau, Stanislas Faguer
OBJECTIVE: Outcomes of systemic lupus erythematosus (SLE) and lupus nephritis (LN) are highly heterogeneous among some populations because of interactions between genetic, epigenetic, environmental, and socioeconomic factors. A better characterization of social and ethnic disparities in mixed populations may thus help to develop individualized treatment regimens. MATERIALS AND METHODS: Retrospective observational study including all patients with LN diagnosed between January 1993 and January 2014 in the only Nephrology Department of French Polynesia...
September 21, 2017: Clinical Nephrology
https://www.readbyqxmd.com/read/28863792/genetics-of-immune-mediated-glomerular-diseases-focus-on-complement
#7
REVIEW
Marina Noris, Giuseppe Remuzzi
The spectrum of immune-mediated glomerular diseases is wide, ranging from rare diseases with well-recognized genetic origins to more common and multifactorial diseases. Immune-mediated glomerular injury is complex and involves both the innate and the adaptive immune systems. In the past 20 years a huge effort has been undertaken to unravel the genetic basis of immune-mediated glomerular diseases. The discovery of abnormalities in genes encoding proteins of the alternative pathway of complement in more than 50% of patients with atypical hemolytic uremic syndrome (aHUS), and in approximately 20% of patients with membranoproliferative glomerulonephritis (MPGN), has highlighted the role of this complement pathway in the pathogenesis of immune-mediated glomerular diseases...
September 2017: Seminars in Nephrology
https://www.readbyqxmd.com/read/28806242/generalized-lymph-node-fdg-uptake-as-the-first-manifestation-of-systemic-lupus-erythematosus
#8
Antoine Girard, Jessica Ohnona, Jean-François Bernaudin, Françoise Montravers, Claude Bachmeyer
We report 2 cases of young patients referred for FDG PET/CT for peripheral lymphadenopathy and moderate fever associated with pericarditis in 1 patient and glomerulonephritis in the other patient. FDG PET/CT showed a very similar appearance in these 2 patients, with highly avid lymph nodes with axillary predominance and diffuse FDG uptake in spleen and bone marrow. Systemic lupus erythematosus with an unusual presentation of generalized lymphadenopathy was diagnosed in both patients. This FDG distribution pattern should be kept in mind when PET is performed for etiological investigation of fever of unknown origin...
October 2017: Clinical Nuclear Medicine
https://www.readbyqxmd.com/read/28800777/bortezomib-treatment-induces-a-higher-mortality-rate-in-lupus-model-mice-with-a-higher-disease-activity
#9
Tomoko Ikeda, Hiroshi Fujii, Masato Nose, Yukiko Kamogawa, Tsuyoshi Shirai, Yuko Shirota, Tomonori Ishii, Hideo Harigae
BACKGROUND: Bortezomib (Bz) is a proteasome inhibitor that directly targets antibody-producing plasma cells. We recently reported the first randomized control trial that evaluated the effects of Bz in patients with systemic lupus erythematosus (SLE). In that study, we demonstrated that Bz treatment is associated with many adverse reactions in patients with refractory disease. In the present study, we examine the therapeutic and toxic effects of Bz on MRL/MpJ-lpr/lpr (MRL/lpr) mice with severe disease activity...
August 11, 2017: Arthritis Research & Therapy
https://www.readbyqxmd.com/read/28794288/kidney-disease-in-oman-a-view-of-the-current-and-future-landscapes
#10
Intisar Hamed Al Alawi, Issa Al Salmi, Adhra Al Mawali, John A Sayer
Oman is located in the southeast of Arabian Peninsula with a relatively young population of about 3 831 553 people. The Ministry of Health, which is the healthcare provider, is facing a challenge with the increased levels of noncommunicable diseases including chronic kidney disease. A growing number of patients progress to end-stage kidney disease (ESKD), demanding renal replacement therapy. In 2014, there were 1339 of ESKD patients receiving dialysis and almost 1400 patients received kidney transplants. The estimated annual incidence of ESKD is 120 patients per million population...
July 2017: Iranian Journal of Kidney Diseases
https://www.readbyqxmd.com/read/28762061/direct-antiglobulin-coombs-test-in-systemic-lupus-erythematosus-patients
#11
Thelma Skare, Leandro Picelli, Thiago Alberto G Dos Santos, Renato Nisihara
The objective of the study is to study the positivity of Coombs test or direct antiglobulin test (DAT) in systemic lupus erythematosus (SLE) patients and its relationship with disease's clinical and serological profile. Retrospective study of 373 SLE patients seen at single Rheumatology Unit. Epidemiological data (age, gender, age at disease onset, auto declared ethnic background and tobacco use), clinical (malar rash, photosensitivity, oral ulcers, discoid lesions, serositis, glomerulonephritis, convulsions, psychosis, hemolytic anemia, leukopenia, lymphocytopenia and arthritis), and serological profile (anti ds DNA, anti Ro/SS-A; anti La/SS-B, Anti RNP, Anti Sm, aCl (anticardiolipin) IgG, aCl Ig M, LA or lupus anticoagulant, rheumatoid factor and direct Coombs) were collected...
July 31, 2017: Clinical Rheumatology
https://www.readbyqxmd.com/read/28755900/assessment-of-urinary-tweak-levels-in-mexican-patients-with-untreated-lupus-nephritis-an-exploratory-study
#12
Fabiola Reyes-Martínez, Monserrat Pérez-Navarro, Adrián Rodríguez-Matías, Virgilia Soto-Abraham, Gabriela Gutierrez-Reyes, Zaira Medina-Avila, Rafael Valdez-Ortiz
OBJECTIVES: Urinary levels of TWEAK (uTWEAK) may be correlated with the degree of lupus nephritis (LN) activity. Our objective was to determine the sensitivity and specificity of uTWEAK in Mexican patients with untreated active lupus nephritis. METHODS: An exploratory study was performed; four groups of patients were analyzed as follows: 1) patients with systemic lupus erythematosus (SLE) without renal activity (SLE-LN), 2) patients with SLE with renal activity (SLE+LN), 3) patients with other types of glomerulopathy (glomerulonephritis, GMN), 4) and healthy patients (controls)...
July 26, 2017: Nefrología: Publicación Oficial de la Sociedad Española Nefrologia
https://www.readbyqxmd.com/read/28745692/-changes-in-the-complement-system-in-membranoproliferative-glomerulonephritis
#13
V A Yurova, L A Bobrova, N L Kozlovskaya, Yu V Korotchaeva, A G Serova, L V Kozlov, S S Andina, K A Demyanova, A M Kuchieva, S V Roshchupkina
AIM: To compare the clinical manifestations membranoproliferative glomerulonephritis (MPGN) in its idiopathic variant, lupus nephritis (LN), and C3 glomerulopathy (C3-GP), by comparing them with changes in the complement system. SUBJECTS AND METHODS: The clinic of nephrology followed up 42 patients with different types of MPGN in 2013 to 2015. The study included 35 patients divided into 3 groups: 1) 8 patients with C3-GP, 2) 13 with idiopathic MPGN; 3) 14 with Class IV LN...
2017: Terapevticheskiĭ Arkhiv
https://www.readbyqxmd.com/read/28745688/-risk-factors-for-anemia-in-the-early-stages-of-chronic-kidney-disease
#14
Yu S Milovanov, L V Kozlovskaya, L Yu Milovanova, M M Markina, V V Kozlov, M V Taranova, V V Fomin
AIM: To identify the early markers of anemia in chronic kidney disease (CKD) in patients with chronic glomerulonephritis (CGN) and glomerulonephritis (GN) in systemic diseases. SUBJECTS AND METHODS: Seventy-nine patients with some male preponderance who were aged 21 to 65 years (45.3±11.1 years) and had CKD (CGN and GN) in systemic diseases (systemic lupus erythematosus and Wegener's granulomatosis) in the early stages (Stages I-II) of CKD were examined. GN was diagnosed by a lifetime renal biopsy...
2017: Terapevticheskiĭ Arkhiv
https://www.readbyqxmd.com/read/28719617/microrna-155-deficiency-leads-to-decreased-autoantibody-levels-and-reduced-severity-of-nephritis-and-pneumonitis-in-pristane-induced-lupus
#15
Harald Leiss, Wilhelm Salzberger, Barbara Jacobs, Irina Gessl, Nicolas Kozakowski, Stephan Blüml, Antonia Puchner, Attila Kiss, Bruno K Podesser, Josef S Smolen, Georg H Stummvoll
OBJECTIVE: We herein examine the role of endogenous miR155 in the development of systemic manifestations in pristane induced lupus. MATERIALS AND METHODS: Systemic lupus in miR155-deficient and wild type mice was induced upon injection of pristane and analyzed after 8 months, PBS-injected mice served as controls. Glomerulonephritis and pneumonitis were quantified using the kidney biopsy score and a newly adapted histomorphometric image analysis system; lung tissue was further analyzed by tissue cytometry...
2017: PloS One
https://www.readbyqxmd.com/read/28652957/a-retrospective-study-of-acute-renal-failure-in-children-its-incidence-etiology-complications-and-prognosis
#16
Kadar Ismail Hassan, Jama Hodan M, Chunfu Li
BACKGROUND: Acute renal failure (ARF) developed due to various causes and may lead to significant morbidity and mortality among pediatric patients. OBJECTIVES: The study was conducted to determine the incidence, etiology, outcome of treatment and clinical presentation of ARF in pediatric patients in Somalia. METHODS: Comprehensive case history of 39 pediatric patients below 12 years of age, admitted with renal diseases in four tertiary care hospitals in Hargeisa and Borama cities in Somalia during December 2015 to November 2016...
May 25, 2017: Curēus
https://www.readbyqxmd.com/read/28612005/renal-limited-lupus-like-nephritis-how-much-of-a-lupus
#17
Asmaa S Abu Maziad, Jose Torrealba, Mouin G Seikaly, Jared R Hassler, Allen R Hendricks
Systemic lupus erythematosus (SLE) is an autoimmune disease characterized by development of autoantibodies to nuclear and cytoplasmic antigens. A small subset of SLE patients who had the typical clinical features of SLE was reported to show persistently negative antinuclear antibody tests. Our report describes a 5-year-old male who presented with histopathological findings suggestive of lupus nephritis with no clinical signs or symptoms of SLE and negative autoantibodies. He was treated with corticosteroids, mycophenolate mofetil, and monthly intravenous cyclophosphamide...
May 2017: Case Reports in Nephrology and Dialysis
https://www.readbyqxmd.com/read/28592713/pulmonary-limited-granulomatosis-with-polyangiitis-coexisting-with-mixed-connective-tissue-disease
#18
Masaru Ando, Akihiko Goto, Mari Yamasue, Yuko Usagawa, Hiroaki Oka, Takehiko Shigenaga, Jun-Ichi Kadota
Granulomatosis with polyangiitis (GPA) is a systemic disease characterized by necrotizing, granulomatous vasculitis of the upper and lower respiratory tracts and glomerulonephritis, and is classified as a classical or limited form. The classical form of GPA demonstrates the involvement of the upper respiratory tract, sinuses, lungs and kidneys, whereas the limited form is characterized by the lack of the renal involvement with female predominance. On the other hand, mixed connective tissue disease (MCTD) shows the clinical and laboratorial features of systemic lupus erythematosus, systemic sclerosis and polymyositis, along with high titers of anti-ribonucleoprotein antibodies and is characterized by good response to corticosteroid therapy and favorable prognosis...
June 2017: Tohoku Journal of Experimental Medicine
https://www.readbyqxmd.com/read/28584626/mmp2-and-mmp9-associate-with-crescentic-glomerulonephritis
#19
Tessa M Phillips, Mitali Fadia, Tom N Lea-Henry, Jonathan Smiles, Giles D Walters, Simon H Jiang
Background: Systemic lupus erythematosus (SLE) is a systemic autoimmune disease characterized by multiple organ involvement. Lupus nephritis (LN) is a common manifestation with a wide variety of histological appearances. Matrix metalloproteinases (MMP) 2 and 9 are gelatinases capable of degrading glomerular basement membrane type IV collagen, which have been associated with LN. We examine the expression of MMP2 and MMP9 in different classes of LN. Methods: MMP2 and MMP9 expression was detected by immunohistochemistry in sections from renal biopsy specimens with class III, class IV and class V LN (total n = 31), crescentic immunoglobulin A nephropathy (n = 6), pauci-immune glomerulonephritis (n = 7), minimal change disease (n = 2), mesangiocapillary glomerulonephritis (n = 7), diabetic nephropathy (n = 12) and histologically normal controls (n = 8)...
April 2017: Clinical Kidney Journal
https://www.readbyqxmd.com/read/28584258/cathepsin-s-inhibition-combines-control-of-systemic-and-peripheral-pathomechanisms-of-autoimmune-tissue-injury
#20
Maia Tato, Santhosh V Kumar, Yajuan Liu, Shrikant R Mulay, Solange Moll, Bastian Popper, Jonathan N Eberhard, Dana Thomasova, Arne Christian Rufer, Sabine Gruner, Wolfgang Haap, Guido Hartmann, Hans-Joachim Anders
Cathepsin(Cat)-S processing of the invariant chain-MHC-II complex inside antigen presenting cells is a central pathomechanism of autoimmune-diseases. Additionally, Cat-S is released by activated-myeloid cells and was recently described to activate protease-activated-receptor-(PAR)-2 in extracellular compartments. We hypothesized that Cat-S blockade targets both mechanisms and elicits synergistic therapeutic effects on autoimmune tissue injury. MRL-(Fas)lpr mice with spontaneous autoimmune tissue injury were treated with different doses of Cat-S inhibitor RO5459072, mycophenolate mofetil or vehicle...
June 5, 2017: Scientific Reports
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