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https://www.readbyqxmd.com/read/27817791/-advances-in-clinical-research-on-c1q-nephropathy
#1
Juan Yang, Bi-Li Zhang
C1q nephropathy is a rare type of glomerulonephritis manifested as the deposition of C1q in the glomerular mesangium during immunofluorescent staining. Systemic lupus erythematosus and type I membranoproliferative glomerulonephropathy need to be excluded in the diagnosis of C1q nephropathy. C1q nephropathy has various manifestations under a light microscope, mainly including minimal change disease, focal segmental glomerulosclerosis, and proliferative glomerulonephritis. This disease is mainly manifested as persistent proteinuria or nephrotic syndrome and occurs more frequently in boys...
November 2016: Zhongguo Dang Dai Er Ke za Zhi, Chinese Journal of Contemporary Pediatrics
https://www.readbyqxmd.com/read/27621641/rituximab-use-in-adult-primary-glomerulopathy-where-is-the-evidence
#2
REVIEW
Samir G Mallat, Houssam S Itani, Rana M Abou-Mrad, Rima Abou Arkoub, Bassem Y Tanios
Rituximab is a chimeric anti-CD20 antibody that results in depletion of B-cell lymphocytes. It is currently used in the treatment of a variety of autoimmune diseases, in addition to CD20-positive lymphomas. The use of rituximab in the treatment of the adult primary glomerular diseases has emerged recently, although not yet established as first-line therapy in international guidelines. In patients with steroid-dependent minimal change disease or frequently relapsing disease, and in patients with idiopathic membranous nephropathy (IMN), several retrospective and prospective studies support the use of rituximab to induce remission, whereas in idiopathic focal and segmental glomerulosclerosis (FSGS), the use of rituximab has resulted in variable results...
2016: Therapeutics and Clinical Risk Management
https://www.readbyqxmd.com/read/27235854/experimentally-induced-anti-myeloperoxidase-vasculitis-does-not-require-properdin-masp-2-or-bone-marrow-derived-c5
#3
Simon J Freeley, Reena J Popat, Kiran Parmar, Martin Kolev, Beverley J Hunt, Cordula M Stover, Willhelm Schwaeble, Claudia Kemper, Michael G Robson
Anti-neutrophil cytoplasmic antibody vasculitis is a systemic autoimmune disease with glomerulonephritis and pulmonary haemorrhage as major clinical manifestations. The name reflects the presence of autoantibodies to myeloperoxidase and proteinase-3, which bind to both neutrophils and monocytes. Evidence of the pathogenicity of these autoantibodies is provided by the observation that injection of anti-myeloperoxidase antibodies into mice causes a pauci-immune focal segmental necrotizing glomerulonephritis which is histologically similar to the changes seen on renal biopsy in patients...
September 2016: Journal of Pathology
https://www.readbyqxmd.com/read/27130200/patterns-of-childhood-steroid-sensitive-and-steroid-resistant-nephrotic-syndrome-in-saudi-children-clinicopathological-study-of-87-cases
#4
Abdulla A Alharthi
Nephrotic syndrome (NS) is one of the most common causes of chronic kidney disease in children. Mostly, NS is controlled by steroids. In spite of this, 10% to 20% of the patients have steroid resistant NS (SRNS), and the rest have steroid sensitive NS (SSNS). Eighty-seven children with NS (66 SSNS; 21SRNS) were retrospectively studied within the past 10 years in Taif region of Saudi Arabia. Regarding outcome, 66 (76%) patient responded to the steroid therapy, while 21 (24%) patients characterize as SRNS. Out of 66 SSNS, 25 children revealed complete remission, 26 were diagnosed as steroid dependent, and 15 were identified as frequent relapses...
April 29, 2016: Clinical Pediatrics
https://www.readbyqxmd.com/read/27036111/acthar-gel-in-the-treatment-of-nephrotic-syndrome-a-multicenter-retrospective-case-series
#5
MULTICENTER STUDY
Arvind Madan, Snezana Mijovic-Das, Ana Stankovic, Geoffrey Teehan, Amber S Milward, Anupa Khastgir
BACKGROUND: Current first-line anti-proteinuric treatments for nephrotic syndrome (NS) do not produce an effective response in all patients and are not tolerated by some patients. Additional effective and tolerable treatment options in NS are strongly needed. This retrospective case series is the largest to date to examine Acthar gel (adrenocorticotropic hormone, ACTH) in patients with varied-etiology NS. METHODS: This multicenter retrospective case series included adult patients with NS (N = 44) treated with Acthar gel at 6 clinical practices...
March 31, 2016: BMC Nephrology
https://www.readbyqxmd.com/read/26994577/the-clinicopathologic-characteristics-and-outcome-of-atypical-anti-glomerular-basement-membrane-nephritis
#6
Samih H Nasr, A Bernard Collins, Mariam Priya Alexander, Daniel F Schraith, Loren Herrera Hernandez, Mary E Fidler, Sanjeev Sethi, Nelson Leung, Fernando C Fervenza, Lynn D Cornell
Classic anti-glomerular basement membrane (GBM) disease presents with rapidly progressive glomerulonephritis (GN) with or without pulmonary hemorrhage. On biopsy typical disease displays bright polytypic linear GBM staining for IgG by immunofluorescence and diffuse crescentic/necrotizing GN on light microscopy. Here, we studied 20 patients with atypical anti-GBM nephritis typified by bright linear GBM staining for immunoglobulins but without a diffuse crescentic phenotype. Patients had hematuria, proteinuria, and mild renal insufficiency, without pulmonary hemorrhage...
April 2016: Kidney International
https://www.readbyqxmd.com/read/26971743/proliferative-glomerulonephritis-with-monoclonal-immunoglobulin-g-deposits-complicated-by-immunoglobulin-a-nephropathy-in-the-renal-allograft
#7
Anri Sawada, Kunio Kawanishi, Shigeru Horita, Junki Koike, Kazuho Honda, Ayami Ochi, Mizuki Komoda, Yoichiro Tanaka, Kohei Unagami, Masayoshi Okumi, Tomokazu Shimizu, Hideki Ishida, Kazunari Tanabe, Yoji Nagashima, Kosaku Nitta
Immunoglobulin (Ig) A nephropathy (IgAN) is a known autoimmune disease due to abnormal glycosylation of IgA1, and occasionally, IgG co-deposition occurs. The prognosis of IgG co-deposition with IgAN is adverse, as shown in the previous studies. However, in the clinical setting, monoclonality of IgG co-deposition with IgAN has not been observed. We describe a case of proliferative glomerulonephritis with monoclonal IgG deposits (PGNMID) combined with IgAN in a renal allograft. A-21-year-old man developed end-stage renal failure with unknown aetiology and underwent living-donor kidney transplantation from his mother 2 years after being diagnosed...
July 2016: Nephrology
https://www.readbyqxmd.com/read/26788876/frequency-of-the-original-kidney-disease-and-its-effect-on-the-outcome-of-kidney-transplant-in-the-urology-nephrology-center-mansoura-university
#8
Mohamed E Mashaly, Mabrouk I Ismail, Esam E Lotfy, Ahmed F Donia, Ihab W Wafa, Mohamed A Foda, Ahmed A Denewar, Mohamed H Abbas, Ahmed A Shokeir
OBJECTIVES: Renal allograft function and graft survival depends on many factors, including the source of the graft, immunologic matching between donor and recipient, incidence of acute rejection, and recurrence of the original kidney disease. This work aimed to evaluate the effects of the original kidney disease on patient and graft survival. MATERIALS AND METHODS: This was a retrospective, single-center study that included 2189 kidney transplant recipients who were transplanted at The Urology and Nephrology Centre, Mansoura University, between 1976 and 2010...
April 2016: Experimental and Clinical Transplantation
https://www.readbyqxmd.com/read/26774466/differences-in-initial-hemodialysis-vascular-access-use-among-glomerulonephritis-subtypes-in-the-united-states
#9
COMPARATIVE STUDY
Michelle M O'Shaughnessy, Maria E Montez-Rath, Yuanchao Zheng, Richard A Lafayette, Wolfgang C Winkelmayer
BACKGROUND: The type of vascular access used for hemodialysis affects patient morbidity and mortality. Whether vascular access types differ by glomerulonephritis (GN) subtype in the US hemodialysis population has not been investigated. STUDY DESIGN: Cross-sectional observational study. SETTING & PARTICIPANTS: We identified all adult (aged ≥ 18 years) patients within the US Renal Data System who initiated hemodialysis therapy from July 2005 through December 2011 with a diagnosis of end-stage renal disease attributed to any of 4 primary (focal segmental glomerulosclerosis, immunoglobulin A nephropathy [reference group], membranous nephropathy, and membranoproliferative GN) or 2 secondary (lupus nephritis and vasculitis) GN subtypes...
April 2016: American Journal of Kidney Diseases: the Official Journal of the National Kidney Foundation
https://www.readbyqxmd.com/read/26684161/aetiology-of-paediatric-end-stage-renal-failure-in-jordan-a-multicentre-study
#10
K F Akl, J H Albaramki, I Hazza, R Haddidi, S H Saleh, R Haddad, S Ajarmeh, R S Al-Assaf, E Al-Qadi
OBJECTIVE: The purpose of this study was to find out the aetiology of end-stage renal failure (ESRF) in children in Jordan. SUBJECTS AND METHODS: This was a multicentre retrospective study at five participating hospitals. Data collection included medical record review for age, gender, aetiology of ESRF, modality of renal replacement therapy (RRT) and outcome. End-stage renal failure was defined as estimated glomerular filtration rate (eGFR) < 15 ml/min/1.73m2...
May 11, 2015: West Indian Medical Journal
https://www.readbyqxmd.com/read/26640779/long-term-outcomes-in-children-with-steroid-resistant-nephrotic-syndrome-treated-with-calcineurin-inhibitors
#11
Nathan T Beins, Katherine M Dell
BACKGROUND: Steroid-resistant nephrotic syndrome (SRNS) is an important cause of chronic kidney disease (CKD) in children that often progresses to end-stage renal disease (ESRD). Calcineurin inhibitors (CNIs) have been shown to be effective in inducing short-term remission in some patients with SRNS. However, there are little data examining their long-term impact on ESRD progression rates. METHODS: We performed a retrospective chart review of all patients treated for SRNS with CNIs at our institution from 1995 to 2013...
2015: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/26440274/-glomerulonephritides
#12
REVIEW
J Floege
Many forms of glomerulonephritis, even the more common types belong to the so-called rare diseases; however, they are very important, for example with respect to health economics as they often affect young people. An example is immunoglobulin A (IgA) nephropathy, which is the most common cause of end stage renal disease in young adults. This review summarizes the current knowledge on the pathogenesis, clinical presentation and therapy of the most common European types of glomerulonephritis with a special focus on the most recently acquired knowledge on IgA nephropathy, membranous glomerulonephritis, minimal change nephropathy and focal segmental glomerulosclerosis (FSGS)...
November 2015: Der Internist
https://www.readbyqxmd.com/read/25997642/angiotensin-converting-enzyme-genotype-is-not-a-significant-genetic-risk-factor-for-idiopathic-nephrotic-syndrome-in-croatian-children
#13
Danko Batinić, Jadranka Sertić, Marijana Ćorić, Paško Konjevoda, Danica Batinić, Danko Milošević
AIM: The association of the angiotensin-converting enzyme (ACE) gene insertion/deletion (I/D) polymorphism with idiopathic nephrotic syndrome (INS) is controversial. Only scarce information on European populations is available. The aim of the study was to investigate the distribution of the ACE gene I/D polymorphism and its impact on INS in children from Croatia. MATERIALS AND METHODS: Ninety-five children with INS were investigated: 30 with minimal change disease (MCD), 35 with mesangial proliferative glomerulonephritis (MesPGN) and 30 with focal segmental glomerulosclerosis (FSGS)...
2015: Nephron
https://www.readbyqxmd.com/read/25894566/nephrotic-syndrome-and-thrombotic-microangiopathy-caused-by-cobalamin-c-deficiency
#14
Jens C Koenig, Frank Rutsch, Clemens Bockmeyer, Matthias Baumgartner, Bodo B Beck, Brigitta Kranz, Martin Konrad
BACKGROUND: Cobalamin C (CblC) defects are inherited autosomal recessive disorders of vitamin B12 metabolism due to mutations in the MMACHC gene. Renal manifestations include thrombotic microangiopathy (TMA), acute or chronic renal failure, tubulointerstitial nephritis, and proximal renal tubular acidosis. However, reports about glomerular pathologies are scarce. CASE REPORT: A 4-year-old boy presented with nephrotic syndrome, arterial hypertension, and chronic anemia but no signs of hemolysis...
July 2015: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/25881669/micrornas-in-glomerular-diseases-from-pathophysiology-to-potential-treatment-target
#15
REVIEW
Francesco P Schena, Fabio Sallustio, Grazia Serino
miRNAs are regulators of gene expression in diverse biological and pathological courses in life. Their discovery may be considered one of the most important steps in the story of modern biology. miRNAs are packed within exosomes and released by cells for cellular communications; they are present in bodily fluids. Their study opens the way for understanding the pathogenetic mechanisms of many diseases; furthermore, as potential candidate biomarkers, they can be measured in bodily fluids for non-invasive monitoring of disease outcomes...
June 2015: Clinical Science (1979-)
https://www.readbyqxmd.com/read/25859369/the-need-for-improved-uptake-of-the-kdigo-glomerulonephritis-guidelines-into-clinical-practice-in-canada-a-survey-of-nephrologists
#16
Sean Barbour, Monica Beaulieu, Jagbir Gill, Gabriela Espino-Hernandez, Heather N Reich, Adeera Levin
BACKGROUND: The lack of glomerulonephritis (GN) guidelines has historically contributed to substantial variability in the treatment of GN. We hypothesize that there are barriers to GN guideline implementation leading to incomplete translation of the 2012 KDIGO GN guidelines into patient care, and that current practice patterns deviate from guideline recommendations. METHODS: Adult nephrologists in Canada (N = 390) were surveyed using a web-based tool. The survey of 40 questions captured physician demographics, self-reported GN case load, treatment approaches and barriers to guideline implementation...
December 2014: Clinical Kidney Journal
https://www.readbyqxmd.com/read/25722314/-in-process-citation
#17
Hans-Peter Marti, Christina Dörje, Erik H Strøm
Recurrence of glomerulonephritis after kidney transplantation is especially in the long term an important cause of renal allograft failure. The exact frequency depends on the one hand how the diagnosis of recurrence was established, either on clinical grounds or histologically via a kidney transplant biopsy, and on the other hand on the type of the underlying or primary glomerular disease. The consequences of a relapse on allograft function and survival vary, depending on the primary disease. For example, recurrences after IgA nephropathy occur depending on the length of the observation period in over 50 % of the allografts with often relatively slow progression...
March 2015: Therapeutische Umschau. Revue Thérapeutique
https://www.readbyqxmd.com/read/25709493/low-dose-synthetic-adrenocorticotropic-hormone-analog-therapy-for-nephrotic-patients-results-from-a-single-center-pilot-study
#18
Paolo Lorusso, Anna Bottai, Emanuela Mangione, Maurizio Innocenti, Adamasco Cupisti, Maria Francesca Egidi
INTRODUCTION: This report describes our experience using a low-dose synthetic adrenocorticotropic hormone (ACTH) analog for patients affected by nephrotic syndrome who had not responded to or had relapsed after steroid and immunosuppressive treatments. PATIENTS AND METHODS: Eighteen adult nephrotic patients with an estimated glomerular filtration rate >30 mL/min were recruited. Histological pictures included ten of membranous nephropathy, three of membranous proliferative glomerulonephritis, three of minimal change, and two of focal segmental glomerular sclerosis...
2015: International Journal of Nephrology and Renovascular Disease
https://www.readbyqxmd.com/read/25635037/spectrum-of-steroid-resistant-and-congenital-nephrotic-syndrome-in-children-the-podonet-registry-cohort
#19
MULTICENTER STUDY
Agnes Trautmann, Monica Bodria, Fatih Ozaltin, Alaleh Gheisari, Anette Melk, Marta Azocar, Ali Anarat, Salim Caliskan, Francesco Emma, Jutta Gellermann, Jun Oh, Esra Baskin, Joanna Ksiazek, Giuseppe Remuzzi, Ozlem Erdogan, Sema Akman, Jiri Dusek, Tinatin Davitaia, Ozan Özkaya, Fotios Papachristou, Agnieszka Firszt-Adamczyk, Tomasz Urasinski, Sara Testa, Rafael T Krmar, Lidia Hyla-Klekot, Andrea Pasini, Z Birsin Özcakar, Peter Sallay, Nilgun Cakar, Monica Galanti, Joelle Terzic, Bilal Aoun, Alberto Caldas Afonso, Hanna Szymanik-Grzelak, Beata S Lipska, Sven Schnaidt, Franz Schaefer
BACKGROUND AND OBJECTIVES: Steroid-resistant nephrotic syndrome is a rare kidney disease involving either immune-mediated or genetic alterations of podocyte structure and function. The rare nature, heterogeneity, and slow evolution of the disorder are major obstacles to systematic genotype-phenotype, intervention, and outcome studies, hampering the development of evidence-based diagnostic and therapeutic concepts. To overcome these limitations, the PodoNet Consortium has created an international registry for congenital nephrotic syndrome and childhood-onset steroid-resistant nephrotic syndrome...
April 7, 2015: Clinical Journal of the American Society of Nephrology: CJASN
https://www.readbyqxmd.com/read/25595442/end-stage-renal-disease-in-japanese-children-a-nationwide-survey-during-2006-2011
#20
Motoshi Hattori, Mayumi Sako, Tetsuji Kaneko, Akira Ashida, Akira Matsunaga, Tohru Igarashi, Noritomo Itami, Toshiyuki Ohta, Yoshimitsu Gotoh, Kenichi Satomura, Masataka Honda, Takashi Igarashi
BACKGROUND: End-stage renal disease (ESRD) in children is considered a rare, but serious condition. Epidemiological and demographic information on pediatric ESRD patients around the world is important to better understand this disease and to improve patient care. The Japanese Society for Pediatric Nephrology (JSPN) reported epidemiological and demographic data in 1998. Since then, however, there has been no nationwide survey on Japanese children with ESRD. METHODS: The JSPN conducted a cross-sectional nationwide survey in 2012 to update information on the incidence, primary renal disease, initial treatment modalities, and survival in pediatric Japanese patients with ESRD aged less than 20 years during the period 2006-2011...
October 2015: Clinical and Experimental Nephrology
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