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Focal segmental glomerulosclerosis fsgs

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https://www.readbyqxmd.com/read/29217578/a-small-molecule-inhibitor-of-trpc5-ion-channels-suppresses-progressive-kidney-disease-in-animal-models
#1
Yiming Zhou, Philip Castonguay, Eriene-Heidi Sidhom, Abbe R Clark, Moran Dvela-Levitt, Sookyung Kim, Jonas Sieber, Nicolas Wieder, Ji Yong Jung, Svetlana Andreeva, Jana Reichardt, Frank Dubois, Sigrid C Hoffmann, John M Basgen, Mónica S Montesinos, Astrid Weins, Ashley C Johnson, Eric S Lander, Michael R Garrett, Corey R Hopkins, Anna Greka
Progressive kidney diseases are often associated with scarring of the kidney's filtration unit, a condition called focal segmental glomerulosclerosis (FSGS). This scarring is due to loss of podocytes, cells critical for glomerular filtration, and leads to proteinuria and kidney failure. Inherited forms of FSGS are caused by Rac1-activating mutations, and Rac1 induces TRPC5 ion channel activity and cytoskeletal remodeling in podocytes. Whether TRPC5 activity mediates FSGS onset and progression is unknown. We identified a small molecule, AC1903, that specifically blocks TRPC5 channel activity in glomeruli of proteinuric rats...
December 8, 2017: Science
https://www.readbyqxmd.com/read/29214160/mir-217-is-a-useful-diagnostic-biomarker-and-regulates-human-podocyte-cells-apoptosis-via-targeting-tnfsf11-in-membranous-nephropathy
#2
Jing Li, Bin Liu, Hen Xue, Qiao Qiao Zhou, Ling Peng
Background: MicroRNAs have recently been verified as useful diagnostic biomarkers in various diseases. In this study, we investigated whether miR-217 is a useful diagnostic biomarker and the possible pathological mechanism of miR-217 in this disease. Methods: Patients with focal segmental glomerulosclerosis (FSGS), membranous nephropathy (MN), and diabetic nephropathy (DN) and control patients were enrolled in this study. The miR-217 inhibitor and mimics were transfected into human podocyte cells to investigate the pathological mechanism of miR-217 in this disease...
2017: BioMed Research International
https://www.readbyqxmd.com/read/29212162/rituximab-treatment-in-adults-with-refractory-minimal-change-disease-or-focal-segmental-glomerulosclerosis
#3
Hong Ren, Li Lin, Pingyan Shen, Xiao Li, Jingyuan Xie, Xiaoxia Pan, Wen Zhang, Nan Chen
Rituximab (RTX) may benefit patients with glomerular disease who suffer from focal segmental glomerular sclerosis (FSGS) or minimal change disease (MCD). Here, we have described our experience treating 6 FSGS and 9 MCD patients with steroid-dependent/refractory nephrotic syndrome (NS) with RTX. Patients received RTX (375 mg/m2) intravenously on days 1, 8, 23, and 29. During a median follow-up of 8 months (range, 3-36 months) after RTX administration, all patients achieved complete or partial remission. Relapses decreased by approximately 30-fold compared with the year preceding RTX treatment, and an 89...
November 7, 2017: Oncotarget
https://www.readbyqxmd.com/read/29198656/long-term-outcomes-of-kidney-transplant-recipients-with-primary-idiopathic-focal-segmental-glomerulosclerosis
#4
O Staeck, F Halleck, K Budde, D Khadzhynov
BACKGROUND: Few data exist on recurrence rates, treatment response, and long-term outcomes in kidney transplant recipients (KTR) with primary focal segmental glomerulosclerosis (FSGS). METHODS: This retrospective, observational study included 1218 consecutive KTR during 2002 to 2016. All patients with primary idiopathic FSGS were identified through application of strict diagnostic criteria. Outcomes were followed over an average of 70.4 months. RESULTS: We identified 48 KTR (3...
December 2017: Transplantation Proceedings
https://www.readbyqxmd.com/read/29191574/-collapsing-focal-segmental-glomerulosclerosis-induced-by-cytomegalovirus-a%C3%A2-case-report
#5
Clarisse Grèze, Cyril Garrouste, Jean-Louis Kemeny, Carole Philipponnet, Julien Aniort, Anne-Élisabeth Heng
Focal segmental glomerulosclerosis (FSGS) is a common cause of nephrotic syndrome in child and adult. The collapsing forms are of poor renal prognosis and are usually secondary to viral infections with, first and foremost, the human immunodeficiency virus. Among other viral etiologies, cytomegalovirus (CMV) is an uncommon cause. We report a case of a 32years-old patient with collapsing focal segmental glomerulosclerosis induced by cytomegalovirus with initial acute renal failure and proteinuria at 12.4g/24h...
November 27, 2017: Néphrologie & Thérapeutique
https://www.readbyqxmd.com/read/29191133/focal-segmental-glomerulosclerosis-in-related-miniature-schnauzer-dogs
#6
Wilson Yau, Lisa Mausbach, Meryl P Littman, Rachel E Cianciolo, Cathy A Brown
Focal segmental glomerulosclerosis (FSGS) recently has been recognized as a common cause of proteinuria in dogs in general, and in Miniature Schnauzer dogs in particular. This study describes the morphologic features present in the kidneys of 8 related proteinuric Miniature Schnauzer dogs. The FSGS, characterized by solidification of portions of the capillary tuft, affected 32% to 49% of examined glomeruli in these dogs. Synechiae, often accompanied by hyalinosis, were present in 13% to 54% of glomeruli and were more prevalent in older dogs...
January 1, 2017: Veterinary Pathology
https://www.readbyqxmd.com/read/29190634/focal-segmental-glomerulosclerosis-associated-with-chronic-progressive-external-ophthalmoplegia-and-mitochondrial-dna-a3243g-mutation
#7
Kaori Narumi, Eikan Mishima, Yukako Akiyama, Tetsuro Matsuhashi, Takashi Nakamichi, Kiyomi Kisu, Shuhei Nishiyama, Hajime Ikenouchi, Akio Kikuchi, Rumiko Izumi, Mariko Miyazaki, Takaaki Abe, Hiroshi Sato, Sadayoshi Ito
Focal segmental glomerulosclerosis (FSGS) is caused by various etiologies, with mitochondrial dysfunction being one of the causes. FSGS is known to be associated with mitochondrial encephalomyopathy, lactic acidosis, and stroke-like episodes (MELAS), which is a subclass of mitochondrial disease. However, it has rarely been reported in other mitochondrial disease subclasses. Here, we reported a 20-year-old man diagnosed with FSGS associated with chronic progressive external ophthalmoplegia (CPEO) due to mitochondrial DNA (mtDNA) 3243A>G mutation...
November 30, 2017: Nephron
https://www.readbyqxmd.com/read/29189487/rituximab-and-therapeutic-plasma-exchange-in-recurrent-focal-segmental-glomerulosclerosis-postkidney-transplantation
#8
Sami Alasfar, Dany Matar, Robert A Montgomery, Niraj Desai, Bonnie Lonze, Vikas Vujjini, Michelle M Estrella, John Manllo Dieck, Gebran Khneizer, Sanja Sever, Jochen Reiser, Nada Alachkar
BACKGROUND: Focal segmental glomerulosclerosis (FSGS) is a common cause of end stage renal disease (ESRD) with a high rate of recurrence after kidney transplantation. Several factors such as white race, rapid progression, and previous allograft failure due to recurrence were found to be risks of recurrence. Data are limited on the benefits of rituximab and/or therapeutic plasma exchange (TPE) in preventing recurrence. In this study, we sought to assess the efficacy of rituximab and TPE for the prevention and treatment of recurrent FSGS post kidney transplantation...
November 20, 2017: Transplantation
https://www.readbyqxmd.com/read/29184911/kidney-transplant-recipients-with-primary-membranous-glomerulonephritis-have-a-higher-risk-of-acute-rejection-compared-with-other-primary-glomerulonephritides
#9
Tripti Singh, Brad Astor, Weixiong Zhong, Didier Mandelbrot, Arjang Djamali, Sarah Panzer
Background: Despite being the leading cause of graft failure, there is a lack of published data about the rates of rejection in kidney transplant patients with glomerulonephritis as the cause of end-stage renal disease. Methods: We examined all consecutive adult (>18 years) renal transplant recipients with biopsy-proven native renal glomerular disease who underwent kidney transplant between 1994 and 2013. Glomerulonephritis groups included were IgA nephropathy (IgAN) (N = 306), focal segmental glomerulosclerosis (FSGS) (N = 298), membranous nephropathy (MN) (N = 81), and lupus nephritis (LN) (N = 177)...
November 2017: Transplantation Direct
https://www.readbyqxmd.com/read/29167190/an-outcomes-based-definition-of-proteinuria-remission-in-focal-segmental-glomerulosclerosis
#10
Jonathan P Troost, Howard Trachtman, Patrick H Nachman, Matthias Kretzler, Cathie Spino, Radko Komers, Sarah Tuller, Kalyani Perumal, Susan F Massengill, Elaine S Kamil, Gia Oh, David T Selewski, Patrick Gipson, Debbie S Gipson
BACKGROUND AND OBJECTIVES: Proteinuria is used as an indicator of FSGS disease activity, but its use as a clinical trial end point is not universally accepted. The goal of this study was to refine proteinuria definitions associated with long-term kidney survival. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: Data on 466 patients with primary FSGS with proteinuria (urine protein-to-creatinine ratio >1 g/g) were analyzed from five independent cohorts. Proteinuria by months 1, 4, and 8 after study baseline was categorized by conventional definitions of complete (<0...
November 22, 2017: Clinical Journal of the American Society of Nephrology: CJASN
https://www.readbyqxmd.com/read/29167172/podocytes-exhibit-a-specialized-protein-quality-control-employing-derlin-2-in-kidney-disease
#11
Guohui Ren, Nicholas J Tardi, Fumihiko Matsuda, Kwi Hye Koh, Phillip Ruiz, Changli Wei, Mehmet Mete Altintas, Hidde Ploegh, Jochen Reiser
Podocytes are terminally differentiated cells of the kidney filtration barrier with a limited proliferative capacity, and are the primary glomerular target for various sources of cellular stress. Accordingly, it is particularly important for podocytes to cope with stress efficiently to circumvent cell death and avoid compromising renal function. Improperly folded proteins within the endoplasmic reticulum (ER) are associated with increased cellular injury and cell death. To relieve ER stress, protein quality control mechanisms like ER-associated degradation (ERAD) are initiated...
November 22, 2017: American Journal of Physiology. Renal Physiology
https://www.readbyqxmd.com/read/29155846/soluble-cd40-ligand-directly-alters-glomerular-permeability-and-may-act-as-a-circulating-permeability-factor-in-fsgs
#12
Sophie Doublier, Cristina Zennaro, Luca Musante, Tiziana Spatola, Giovanni Candiano, Maurizio Bruschi, Luca Besso, Massimo Cedrino, Michele Carraro, Gian Marco Ghiggeri, Giovanni Camussi, Enrico Lupia
CD40/CD40 ligand (CD40L) dyad, a co-stimulatory bi-molecular complex involved in the adaptive immune response, has also potent pro-inflammatory actions in haematopoietic and non-haematopoietic cells. We describe here a novel role for soluble CD40L (sCD40L) as modifier of glomerular permselectivity directly acting on glomerular epithelial cells (GECs). We found that stimulation of CD40, constitutively expressed on GEC cell membrane, by the sCD40L rapidly induced redistribution and loss of nephrin in GECs, and increased albumin permeability in isolated rat glomeruli...
2017: PloS One
https://www.readbyqxmd.com/read/29142983/efficacy-and-safety-of-sparsentan-compared-with-irbesartan-in-patients-with-primary-focal-segmental-glomerulosclerosis-randomized-controlled-trial-design-duet
#13
Radko Komers, Debbie S Gipson, Peter Nelson, Sharon Adler, Tarak Srivastava, Vimal K Derebail, Kevin E Meyers, Pablo Pergola, Meghan E MacNally, Jennifer L Hunt, Alvin Shih, Howard Trachtman
Introduction: Primary focal segmental glomerulosclerosis (FSGS) is a leading cause of nephrotic syndrome and end-stage renal disease. There are no US Food and Drug Administration-approved therapies for FSGS, and treatment often fails to reduce proteinuria. Endothelin is an important factor in the pathophysiology of podocyte disorders, including FSGS. Sparsentan is a first-in-class, orally active, dual-acting angiotensin receptor blocker (ARB) and highly selective endothelin Type A receptor antagonist...
July 2017: KI Reports
https://www.readbyqxmd.com/read/29141939/dapagliflozin-in-focal-segmental-glomerulosclerosis-a-combined-human-rodent-pilot-study
#14
Harindra Rajasekeran, Heather N Reich, Michelle A Hladunewich, Daniel Cattran, Julie A Lovshin, Yuliya Lytvyn, Petter Bjornstad, Vesta Lai, Josephine Tse, Leslie Cham, Syamantak Majumder, Bridgit B Bowskill, M Golam Kabir, Suzanne L Advani, Ian W Gibson, Manish M Sood, Andrew Advani, David Z I Cherney
BACKGROUND: Focal segmental glomerulosclerosis (FSGS) is an important cause of non-diabetic chronic kidney disease (CKD). Sodium glucose cotransporter-2 inhibition (SGLT2i) therapy attenuates the progression of diabetic nephropathy, but it remains unclear whether SGLT2i provides renoprotection in non-diabetic CKD such as FSGS. OBJECTIVE: The primary aim of this pilot study was to determine the effect of 8 weeks of dapagliflozin on GFR in humans and in experimental FSGS...
November 15, 2017: American Journal of Physiology. Renal Physiology
https://www.readbyqxmd.com/read/29138824/genetic-mutational-testing-of-chinese-children-with-familial-hematuria-with-biopsy%C3%A2-proven-fsgs
#15
Yongzhen Li, Ying Wang, Qingnan He, Xiqiang Dang, Yan Cao, Xiaochuan Wu, Shuanghong Mo, Xiaoxie He, Zhuwen Yi
Focal segmental glomerulosclerosis (FSGS) is a pathological lesion rather than a disease, with a diverse etiology. FSGS may result from genetic and non‑genetic factors. FSGS is considered a podocyte disease due to the fact that in the majority of patients with proven‑FSGS, the lesion results from defects in the podocyte structure or function. However, FSGS does not result exclusively from podocyte‑associated genes, however also from other genes including collagen IV‑associated genes. Patients who carry the collagen type IVA3 chain (COL4A3) or COL4A4 mutations usually exhibit Alport Syndrome (AS), thin basement membrane neuropathy or familial hematuria (FH)...
November 10, 2017: Molecular Medicine Reports
https://www.readbyqxmd.com/read/29137483/molecular-profiling-in-iga-nephropathy-and-focal-and-segmental-glomerulosclerosis
#16
I Tycová, P Hrubá, D Maixnerová, E Girmanová, P Mrázová, L Straňavová, R Zachoval, M Merta, J Slatinská, M Kollár, E Honsová, V Tesař, O Viklický
The aim of the study was to characterize by molecular profiling two glomerular diseases: IgA nephropathy (IgAN) and focal segmental glomerulosclerosis (FSGS) and to identify potential molecular markers of IgAN and FSGS progression. The expressions of 90 immune-related genes were compared in biopsies of patients with IgAN (n=33), FSGS (n=17) and in controls (n=11) using RT-qPCR. To identify markers of disease progression, gene expression was compared between progressors and non-progressors in 1 year follow-up...
November 10, 2017: Physiological Research
https://www.readbyqxmd.com/read/29130775/results-of-native-and-transplant-kidney-biopsies-of-children-in-a-single-center-over-a-15-years-period
#17
Emel Isiyel, Kibriya Fidan, Bahar Buyukkaragoz, Meltem Akcaboy, Yasar Kandur, Ipek Isik Gonul, Necla Buyan, Sevcan Bakkaloglu, Oguz Soylemezoglu
Renal biopsy is an important diagnostic procedure in pediatric nephrology. This study retrospectively investigates the indications, results and complications in a single tertiary children's hospital in Turkey. We evaluated the native and transplant kidney biopsies in Gazi University Pediatric Nephrology Department between 2001 and 2015. A total of 196 biopsies (144 natives and 52 transplants) were included into the study. The mean age of the patients was respectively 10.8 ± 3.5, 13.9 ± 1.5 years...
November 2017: Renal Failure
https://www.readbyqxmd.com/read/29115212/endemic-influences-of-political-regimes-healthcare-systems-and-preferences-on-the-frequencies-and-incidences-of-nephropathies-in-eastern-saxony-germany%C3%A2
#18
Sam Sedaghat, Eleène Sahmila Heshmati, Thomas Frese, Wilma Gertrud Rasche, Filip Barinka, Florian Fahr, Joachim Beige, Stephan Schiekofer, Franz Maximilian Rasche
INTRODUCTION: The possible confounding influence of investigator-related preferences, available histological techniques, and healthcare systems on the frequencies and incidences of primary and secondary nephropathies was evaluated in this long-term observation. MATERIALS AND METHODS: The observation time from 1983 to 2010 was divided in regard to the political regimes: a) prior to and after German reunification: German Democratic Republic (GDR, period 1 from 1983 to 1990)/Federal Republic of Germany (FRG, period 2 from 1990 to 2010); and the two heads of the division of nephrology, b) conductor 1 (1983 - 2006) and conductor 2 (2006 - 2010)...
December 2017: Clinical Nephrology
https://www.readbyqxmd.com/read/29114699/glomerulonephritis-disease-pattern-at-saudi-tertiary-care-center
#19
Khalid I AlMatham, Ali F AlFayez, Raghdah A AlHarthi, Falah S AlMutairi, Fahad S Alrasheedi, Abdulrehman Mustafa, Mustafa Ahmed, Basim A AlMatouq, Fadil A AlRowaei
To assess changes in the pattern of glomerular diseases to help guide optimal allocation of resources, to focus future reasearch, and improve outcomes. Methods: A retrospective chart review was conducted on kidney biopsies taken between 2007 and 2016 at a single tertiary care center in Saudi Arabia (King Fahad Medical City, Riyadh) to evaluate the prevalence and pattern of glomerulonephritis (GN). Results: The most common primary GN in 102 biopsies from adult patients with a mean age of 28.9 ± 13.6 years and 40...
November 2017: Saudi Medical Journal
https://www.readbyqxmd.com/read/29110886/the-incidence-of-primary-vs-secondary-focal-segmental-glomerulosclerosis-a-clinicopathologic-study
#20
Musab S Hommos, An S De Vriese, Mariam P Alexander, Sanjeev Sethi, Lisa Vaughan, Ladan Zand, Kharmen Bharucha, Nicola Lepori, Andrew D Rule, Fernando C Fervenza
OBJECTIVES: To describe the change in the incidence rates of primary and secondary focal segmental glomerulosclerosis (FSGS) from 1994 through 2013 in Olmsted County, Minnesota, and to identify the clinical and biopsy characteristics that distinguish primary from secondary FSGS. PATIENTS AND METHODS: Olmsted County adult residents with native kidney biopsy from January 1, 1994, through December 31, 2013, and FSGS as the only glomerulopathy were identified. The clinical and pathologic characterstics of primary and secondary FSGS were described and compared, and incidence rates were calculated...
October 27, 2017: Mayo Clinic Proceedings
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