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Focal segmental glomerulosclerosis fsgs

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https://www.readbyqxmd.com/read/29035193/renal-outcome-in-iga-nephropathy-according-to-oxford-classification-and-ultrastructural-analysis-in-a-brazilian-center%C3%A2
#1
Giovana Mariani, Leandro L L Freitas, Ricardo L Zollner, Maria Almerinda V F Ribeiro Alves
AIMS: Correlate clinical and histologic features with renal outcome in patients with biopsy-proven IgA nephropathy (IgAN). MATERIALS AND METHODS: Retrospective analysis of records and renal tissue of IgAN patients. Histology was revised according to MEST score of Oxford classification. Focal segmental glomerulosclerosis (FSGS) features were assessed by light microscopy. Electron microscopy review searched for podocyte effacement. RESULTS: 67 patients were included, 56...
October 16, 2017: Clinical Nephrology
https://www.readbyqxmd.com/read/28992235/treatment-by-immunoadsorption-for-recurrent-focal-segmental-glomerulosclerosis-after-paediatric-kidney-transplantation-a-multicentre-french-cohort
#2
Lise Allard, Theresa Kwon, Saoussen Krid, Justine Bacchetta, Arnaud Garnier, Robert Novo, Georges Deschenes, Rémi Salomon, Gwenaëlle Roussey, Emma Allain-Launay
Background: Primary focal segmental glomerulosclerosis (FSGS) frequently recurs after kidney transplantation (KTx) in children. This can lead to delayed graft loss. As the management of children with recurrent FSGS is not well established, apheresis strategies could be a cornerstone to control the disease. Immunoadsorption (IA) is a recent apheresis therapy. There have been few studies examining IA in this setting. We report the results of IA for management of recurrent FSGS after KTx in children in France...
July 28, 2017: Nephrology, Dialysis, Transplantation
https://www.readbyqxmd.com/read/28982981/the-hippo-pathway-regulator-kibra-promotes-podocyte-injury-by-inhibiting-yap-signaling-and-disrupting-actin-cytoskeletal-dynamics
#3
Kristin Meliambro, Jenny S Wong, Justina Ray, Rhodora C Calizo, Sara Towne, Beatriz Cole, Fadi El Salem, Ronald E Gordon, Lewis Kaufman, John C He, Evren U Azeloglu, Kirk N Campbell
Kidney podocytes represent a key constituent of the glomerular filtration barrier. Identifying the molecular mechanisms of podocyte injury and survival is important for better understanding and management of kidney diseases. KIBRA (KIdney BRAin protein), an upstream regulator of the Hippo signaling pathway encoded by the Wwc1 gene, shares the pro-injury properties of its putative binding partner dendrin and antagonizes the pro-survival signaling of the downstream Hippo pathway effector YAP (Yes-associated protein) in Drosophila and MCF10A cells...
October 5, 2017: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/28975106/reporting-renal-biopsies-from-cyprus-a-systematic-approach
#4
Düriye Deren Oygar, Guy H Neild
BACKGROUND: The etiology of renal disease varies in different parts of the world. In the Middle East, half of all patients reaching end-stage are categorised as either unknown etiology or hypertension-related nephropathy. OBJECTIVES: To report a renal biopsy series, in a reproducible format and manner, so that data can be compared directly among other series. PATIENTS AND METHODS: Biopsies of native kidneys were performed in a 10-year period, at a tertiary referral hospital that provides the entire nephrology service for north Cyprus...
July 2017: Journal of Nephropathology
https://www.readbyqxmd.com/read/28975100/impact-of-%C3%AE-h1-and-%C3%AE-h2-cytokines-in-the-progression-of-idiopathic-nephrotic-syndrome-due-to-focal-segmental-glomerulosclerosis-and-minimal-change-disease
#5
Maria Stangou, Μichael Spartalis, Dimitra-Vasilia Daikidou, Theodora Kouloukourgiotou, Erasmia Sampani, Ioanna-Theologia Lambropoulou, Afroditi Pantzaki, Αikaterini Papagianni, George Efstratiadis
BACKGROUND: Differential diagnosis between primary focal segmental glomerulosclerosis (FSGS) and minimal change disease (MCD) is sometimes difficult as nephrotic syndrome is the main clinical symptom in both diseases. OBJECTIVES: This study has attempted to evaluate the urinary excretion of Th1 and Th2 cytokines as potential biomarkers in distinguishing the two types of nephrotic syndrome, and predicting outcome of renal function. PATIENTS AND METHODS: Thirty-six patients with FSGS (M/F 22/14, Age; 41...
July 2017: Journal of Nephropathology
https://www.readbyqxmd.com/read/28966119/wt1-is-necessary-for-the-proliferation-and-migration-of-cells-of-renin-lineage-following-kidney-podocyte-depletion
#6
Natalya V Kaverina, Diana G Eng, Andrea D Largent, Ilse Daehn, Anthony Chang, Kenneth W Gross, Jeffrey W Pippin, Peter Hohenstein, Stuart J Shankland
Wilms' tumor suppressor 1 (WT1) plays an important role in cell proliferation and mesenchymal-epithelial balance in normal development and disease. Here, we show that following podocyte depletion in three experimental models, and in patients with focal segmental glomerulosclerosis (FSGS) and membranous nephropathy, WT1 increased significantly in cells of renin lineage (CoRL). In an animal model of FSGS in RenWt1(fl/fl) reporter mice with inducible deletion of WT1 in CoRL, CoRL proliferation and migration to the glomerulus was reduced, and glomerular disease was worse compared with wild-type mice...
October 10, 2017: Stem Cell Reports
https://www.readbyqxmd.com/read/28965116/new-anti-nephrin-antibody-mediated-podocyte-injury-model-using-a-c57bl-6-mouse-strain
#7
Kazuhiro Takeuchi, Shokichi Naito, Nagako Kawashima, Naoko Ishigaki, Takashi Sano, Kouju Kamata, Yasuo Takeuchi
BACKGROUND: Focal segmental glomerulosclerosis (FSGS) is considered a subset of the podocytopathies. The molecular pathogenesis of podocytopathy is still unknown. There has not been an experimental animal model of isolated podocytopathy induced by antibody in C57BL/6 strain, which is widely used as the genetic background. Nephrin is closely associated with the slit diaphragm of the glomerular podocyte, and has recently received attention as a potential therapeutic target. The function of nephrin, especially its role in FSGS development via podocytopathy, is being elucidated...
September 30, 2017: Nephron
https://www.readbyqxmd.com/read/28951873/multiple-targets-for-novel-therapy-of-fsgs-associated-with-circulating-permeability-factor
#8
REVIEW
Virginia J Savin, Mukut Sharma, Jianping Zhou, David Genochi, Ram Sharma, Tarak Srivastava, Amna Ilahe, Pooja Budhiraja, Aditi Gupta, Ellen T McCarthy
A plasma component is responsible for altered glomerular permeability in patients with focal segmental glomerulosclerosis. Evidence includes recurrence after renal transplantation, remission after plasmapheresis, proteinuria in infants of affected mothers, transfer of proteinuria to experimental animals, and impaired glomerular permeability after exposure to patient plasma. Therapy may include decreasing synthesis of the injurious agent, removing or blocking its interaction with cells, or blocking signaling or enhancing cell defenses to restore the permeability barrier and prevent progression...
2017: BioMed Research International
https://www.readbyqxmd.com/read/28948129/sudden-collapse-in-the-first-trimester-report-of-hyperacute-renal-failure-secondary-to-collapsing-glomerulopathy-as-the-initial-presentation-of-lupus
#9
Pooja Sethi, Jennifer Treece, Chidinma Onweni
Hyperacute renal failure is rarely the initial presentation of systemic lupus erythematosus (SLE). Pregnancy can predispose untreated lupus nephritis to acute renal failure. Collapsing glomerulopathy (CG) type of renal failure is not a new clinicopathological entity. There have been documented cases prior to 1979. It is thought that detection bias coupled with the predilection for HIV has caused this form of glomerulopathy to be incorrectly named or diagnosed as 'malignant focal segmental glomerulosclerosis (FSGS)'...
July 24, 2017: Curēus
https://www.readbyqxmd.com/read/28939949/focal-segmental-glomerulosclerosis-associated-with-cutaneous-and-systemic-plasmacytosis
#10
Shinsuke Isobe, Naro Ohashi, Naoko Katahashi, Sayaka Ishigaki, Naoko Tsuji, Takayuki Tsuji, Akihiko Kato, Yoshihide Fujigaki, Akira Shimizu, Hideo Yasuda
Cutaneous and systemic plasmacytosis (CSP) is a rare lymphoproliferative disorder that mainly affects middle-aged Asian individuals. Although Castleman disease is often complicated with various renal involvements, glomerulonephritis associated with CSP, which is considered as a variant of Castleman disease, is rare. This report presents the case of a 41-year-old Japanese man with nephrotic syndrome associated with CSP. Renal biopsy findings showed focal segmental glomerulosclerosis (FSGS) and diffusely mild segmental mesangial proliferation...
September 22, 2017: CEN Case Reports
https://www.readbyqxmd.com/read/28937082/treatment-of-steroid-resistant-focal-segmental-glomerulosclerosis-with-rituximab-a-case-report-and-review-of-literature
#11
Mohamed Hasham Varwani, Ahmed Sokwala
Various immunomodulating agents have been tried for the treatment of steroid-resistant focal segmental glomerulosclerosis (FSGS) in the native kidney. A few case series and small studies have reported mixed results with the use of Rituximab for this indication. We report on the case of a 76-year-old male with steroid-resistant FSGS successfully treated with rituximab and remained in remission at the end of six months. A review of the literature highlights the paucity of data on this subject. We conclude that rituximab is a potentially useful treatment for steroid resistant FSGS and larger controlled studies are needed to further define its role in this setting...
September 2017: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/28879428/difficult-to-treat-idiopathic-nephrotic-syndrome-established-drugs-open-questions-and-future-options
#12
REVIEW
Markus J Kemper, Lisa Valentin, Michael van Husen
The idiopathic nephrotic syndrome in childhood can be classified according to the International Study of Kidney Disease in Children (ISKDC) based on the response to steroids. Typically, steroid-sensitive nephrotic syndrome (SSNS) is characterised by minimal changes in disease (MCD) histology, whereas in steroid-resistant nephrotic syndrome (SRNS) focal segmental glomerulosclerosis (FSGS) is the most prevalent lesion. Patients with SSNS may develop frequent relapses and/or steroid dependency, which can be difficult to treat...
September 6, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28857521/igm-as-a-novel-predictor-of-disease-progression-in-secondary-focal-segmental-glomerulosclerosis
#13
Arijana Pačić, Petar Šenjug, Jasna Bacalja, Miroslav Tišljar, Ivica Horvatić, Stela Bulimbašić, Mladen Knotek, Krešimir Galešić, Danica Galešić Ljubanović
AIM: To determine the role of immunoglobulin M (IgM) deposits in clinical manifestations, disease outcome, and treatment response of idiopathic and secondary focal segmental glomerulosclerosis (FSGS). METHODS: Kidney biopsy specimens of 171 patients diagnosed with FSGS (primary and secondary) and 50 control patients were retrospectively included in the study. For each patient, clinical and outcome data were obtained and compared to morphological parameters, including immunofluorescence analysis of mesangial IgM and complement 3 (C3) deposits analyzed on kidney biopsy samples...
August 31, 2017: Croatian Medical Journal
https://www.readbyqxmd.com/read/28852486/diabetes-deafness-and-renal-disease
#14
Iolanda Godinho, Joana Gameiro, Sofia Jorge, Fernando Abreu, Marta Neves, José António Lopes, António Gomes da Costa
Deafness, kidney disease and diabetes are not a usual association, neither is a family history of these diseases. We present the case of a 47-year-old woman with non-nephrotic proteinuria, no haematuria, normal renal function, sensorineural hearing loss, recently diagnosed diabetes and maculopathy. There was a maternal family history of deafness, diabetes and renal disease. Renal biopsy revealed focal and segmental glomerulosclerosis (FSGS), leading to the pursuit of an m.3243A > G mitochondrial mutation and diagnosis of maternally inherited diabetes and deafness...
August 2017: Clinical Kidney Journal
https://www.readbyqxmd.com/read/28842513/transcription-and-translation-of-apol1-variants-commentary
#15
Samina Ejaz
It is highly significant to document molecular alterations existing in the normal cells prior to the onset of any disease. Knowledge of genetic mutations and associated molecular mechanisms will be helpful for better diagnosis and management of diseases. Major focus of this commentary is to provide a comprehensive understanding about apolipoprotein 1 gene (APOL1), protein encoded by this gene (apoL1) and mechanistic details regarding role of apoL1 in the lysis of Trypanosoma brucei Information about APOL1 genetic variants, APOL1G1 and APOL1G2, is provided along with the association of these variants with hypertension-attributed end-stage renal disease (ESRD) and focal segmental glomerulosclerosis (FSGS)...
August 25, 2017: Bioscience Reports
https://www.readbyqxmd.com/read/28832357/recurrent-glomerular-disease-after-kidney-transplantation
#16
Christopher D Blosser, Roy D Bloom
PURPOSE OF REVIEW: With improving short-term kidney transplant outcomes, recurrent glomerular disease is being increasingly recognized as an important cause of chronic allograft failure. Further understanding of the risks and pathogenesis of recurrent glomerular disease enable informed transplant decisions, along with the development of preventive and treatment strategies. RECENT FINDINGS: Multiple observational studies have highlighted differences in rates and outcomes for various recurrent glomerular diseases, although these rates have not markedly improved over the last decade...
November 2017: Current Opinion in Nephrology and Hypertension
https://www.readbyqxmd.com/read/28822451/-the-value-of-serum-free-light-chain-in-differential-diagnosis-of-monoclonal-gammopathy-of-renal-significance
#17
C Li, Y B Wen, H Li, W Su, J Li, J F Cai, L M Chen, X M Li, X W Li
Objective: To investigate the value of serum free light chain (FLC) in differential diagnosis of monoclonal gammopathy of renal significance (MGRS). Methods: Forty-nine hospitalized patients who underwent renal biopsy in Peking Union Medical College Hospital between January 2013 and December 2015 were included. Monoclonal gammopathy was detected by serum protein electrophoresis (SPE), serum immunofixation electrophoresis (IFE), urine IFE and serum FLC. All patients were classified as MGRS (n=32) and monoclonal gammopathy of undetermined significance (MGUS) (n=17)...
August 8, 2017: Zhonghua Yi Xue za Zhi [Chinese medical journal]
https://www.readbyqxmd.com/read/28800680/pharmacological-inhibition-of-fsgs-related-trpc6-gain-of-function-mutants-by-semisynthetic-larixol-derived-compounds
#18
Nicole Urban, Sonja Neuser, Anika Hentschel, Sebastian Köhling, Jörg Rademann, Michael Schaefer
BACKGROUND AND PURPOSE: Gain of function mutations in TRPC6 can cause autosomal dominant forms of focal segmental glomerulosclerosis (FSGS). Validated inhibitors of TRPC6 that are biologically active on FSGS-related TRPC6 mutants are eagerly sought. EXPERIMENTAL APPROACH: We synthesized new TRPC6-inhibiting modulators from larixol, a resiniferous constituent of Larix decidua, and tested the potency and selectivity in cell lines stably expressing various TRP channel isoforms...
August 11, 2017: British Journal of Pharmacology
https://www.readbyqxmd.com/read/28799058/is-cd44-in-glomerular-parietal-epithelial-cells-a-pathological-marker-of-renal-function-deterioration-in-primary-focal-segmental-glomerulosclerosis
#19
Brunna Pinto Froes, Stanley de Almeida Araújo, Eduardo Alves Bambirra, Eduardo Araújo Oliveira, Ana Cristina Simões E Silva, Sérgio Veloso Brant Pinheiro
BACKGROUND: The search for risk factors for chronic kidney disease in children with focal segmental glomerulosclerosis (FSGS) is important in defining prognosis and individualized treatment. This study preliminarily investigated whether CD44 immunostaining in glomerular parietal epithelial cells (PECs) is a prognostic marker in pediatric FSGS. METHODS: In this retrospective study, 26 patients with FSGS, biopsied from 1985 to 2010, were evaluated. Immunohistochemistry for CD44 was performed in all cases...
August 10, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28797008/resveratrol-attenuates-adriamycin-induced-focal-segmental-glomerulosclerosis-through-c3ar-c5ar-sphingosine-kinase-1-pathway
#20
Guoyong Liu, Qiang Wang, Yan Shi, Xiaofei Peng, Hong Liu, Youming Peng, Liyu He
BACKGROUND/AIM: Focal segmental glomerulosclerosis (FSGS) typically presents with nephrotic range proteinuria, which could eventually develop into end-stage renal disease. Resveratrol (RSV) is a natural polyphenol compound, which has been reported to suppress inflammatory response and renal interstitial fibrosis. This study is aimed at evaluating the renoprotective effect of RSV treatment on adriamycin-induced FSGS. METHODS: In Balb/c mice, adriamycin nephropathy was induced by adriamycin (10 mg/kg body weight, diluted in normal saline) via a tail vein on day 0...
August 11, 2017: Pharmacology
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