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Focal segmental glomerulosclerosis fsgs

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https://www.readbyqxmd.com/read/28822451/-the-value-of-serum-free-light-chain-in-differential-diagnosis-of-monoclonal-gammopathy-of-renal-significance
#1
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
#2
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
#3
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
#4
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
https://www.readbyqxmd.com/read/28791668/obesity-related-glomerulopathy-pathogenesis-pathologic-clinical-characteristics-and-treatment
#5
REVIEW
Tianhua Xu, Zitong Sheng, Li Yao
In light of the rapid increase in the number of obesity incidences worldwide, obesity has become an independent risk factor for chronic kidney disease. Obesity-related glomerulopathy (ORG) is characterized by glomerulomegaly in the presence or absence of focal and segmental glomerulosclerosis lesions. IgM and complement 3 (C3) nonspecifically deposit in lesions without immune-complex-type deposits during ORG immunofluorescence. ORG-associated glomerulomegaly and focal and segmental glomerulosclerosis can superimpose on other renal pathologies...
August 8, 2017: Frontiers of Medicine
https://www.readbyqxmd.com/read/28776307/genetic-basis-of-adult-onset-nephrotic-syndrome-and-focal-segmental-glomerulosclerosis
#6
REVIEW
Jian Liu, Weiming Wang
Nephrotic syndrome (NS) is one of the most common glomerular diseases with signs of nephrosis, heavy proteinuria, hypoalbuminemia, and edema. Dysfunction of glomerular filtration barrier causes protein loss through the kidneys. Focal segmental glomerulosclerosis (FSGS) accounts for nearly 20% of NS among children and adults. Adult-onset FSGS/NS is often associated with low response to steroid treatment and immunosuppressive medication and poor renal survival. Several genes involved in NS and FSGS have been identified by linkage analysis and next-generation sequencing...
August 3, 2017: Frontiers of Medicine
https://www.readbyqxmd.com/read/28763127/european-veterinary-renal-pathology-service-a-survey-over-a-7-year-period-2008-2015
#7
L Aresu, V Martini, S L Benali, C Brovida, R E Cianciolo, R Dalla Riva, D Trez, J J Van Der Lugt, A Van Dongen, E Zini
BACKGROUND: The European Veterinary Renal Pathology Service (EVRPS) is the first Web-based registry for canine renal biopsy specimens in Europe. HYPOTHESIS/OBJECTIVES: The aim was to verify whether differences exist between the clinical and laboratory presentation of dogs with nephropathy according to renal pathological findings, as defined by light and electron microscopy of renal biopsy specimens submitted to EVRPS. ANIMALS: Renal biopsy specimens of dogs were collected from the archive of the service (n = 254)...
August 1, 2017: Journal of Veterinary Internal Medicine
https://www.readbyqxmd.com/read/28752288/application-of-next-generation-sequencing-technology-to-diagnosis-and-treatment-of-focal-segmental-glomerulosclerosis
#8
REVIEW
Yutaka Harita
A broad range of genetic and non-genetic factors can lead to kidney injury that manifests as focal segmental glomerulosclerosis (FSGS), which can be classified into primary (idiopathic) and secondary forms. Previous genetic approaches to familial or sporadic cases of FSGS or steroid-resistant nephrotic syndrome identified causal mutations in a subset of genes. Recently, next-generation sequencing (NGS) approaches are becoming a part of a standard assessment in medical genetics. Current knowledge of the comprehensive genomic information is changing the way we think about FSGS and draws attention not only to identification of novel causal genes, but also to potential roles for combinations of mutations in multiple genes, mutations with complex inheritance, and susceptibility genes with variable penetrance carrying relatively minor but significant effects...
July 27, 2017: Clinical and Experimental Nephrology
https://www.readbyqxmd.com/read/28748893/complication-rate-and-diagnostic-yield-of-percutaneous-native-kidney-biopsies-a-10-year-experience-at-a-tertiary-care-hospital-in-pakistan
#9
Sonia Yaqub, Nayla Ahmed, Urooj Fatima, Ayesha Maqbool, Waqar Ashif, Syed A Hussain
The use of an automated biopsy device, and real-time ultrasound for percutaneous kidney biopsies (PKBs) has improved the likelihood of obtaining adequate tissue for diagnosis and also has reduced the complications associated with the procedure. We aimed to determine the frequency and type of complications associated with PKB and to determine the diagnostic yield. It was a retrospective file-based review of cases who underwent PKB of native kidney between January 2003 and December 2013 at the Aga Khan University Hospital in Karachi, Pakistan...
July 2017: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/28745696/-obesity-related-glomerulopathy-mechanisms-of-development-clinical-course
#10
REVIEW
I M Kutyrina
Obesity and overweight are recognized as epidemics of non-communicable diseases in the 21st century. The kidneys are a target organ for obesity, damage to which is considered to be an independent risk factor for the development of renal failure. Obesity-related glomerulopathy (OGP) is one of the types of renal injury in obesity, which is characterized by the development of proteinuria in patients with a body mass index (BMI) of >30 kg/m2 in the absence of other causes of kidney damage. The pathogenesis of OGP is multifactorial...
2017: Terapevticheskiĭ Arkhiv
https://www.readbyqxmd.com/read/28707483/investigational-drugs-in-development-for-focal-segmental-glomerulosclerosis
#11
REVIEW
Howard Trachtman
Focal segmental glomerulosclerosis is an important cause of end stage kidney disease and is a paradigm for the study of glomerular scarring. There are no FDA approved treatments for this condition. Current therapies, assessed based on reduction in proteinuria, are generally effective in a subset of patients which suggests that FSGS is a heterogeneous group of glomerular disorders or podocytopathies that converge on a common histopathological phenotype. Areas covered: We searched for investigational drugs agents that target different pathophysiological pathways using the key words 'FSGS' and 'podocyte' in American and European clinical trial registers (clinicaltrials...
August 2017: Expert Opinion on Investigational Drugs
https://www.readbyqxmd.com/read/28700988/high-dose-rituximab-ineffective-for-focal-segmental-glomerulosclerosis-a-long-term-observation-study
#12
Dario Roccatello, Savino Sciascia, Daniela Rossi, Mirella Alpa, Carla Naretto, Massimo Radin, Antonella Barreca, Roberta Fenoglio, Simone Baldovino, Elisa Menegatti
BACKGROUND: A beneficial effect of rituximab (RTX) on focal segmental glomerulosclerosis (FSGS) in pediatric patients or in transplant recipients has been reported in isolated cases. However, the use of RTX in adult patients with idiopathic FSGS needs further investigation. METHODS: Eight patients who had biopsy-proven FSGS (63.9 ± 14.0, range 40-81 years, 4 women, 4 men) with major risk factors precluding corticosteroids or conventional immunosuppression were treated with a high dose of RTX (8 weekly doses of 375 mg/m2) and prospectively followed up for at least 2 years (29...
July 13, 2017: American Journal of Nephrology
https://www.readbyqxmd.com/read/28689240/supar-and-chronic-kidney-disease-a-podocyte-story
#13
REVIEW
Martin Zeier, Jochen Reiser
The soluble urokinase-type plasminogen activator receptor (suPAR) is a circulating signaling molecule derived from immature myeloid cells. Elevated levels of suPAR have been linked to the pathogenesis of the kidney disease focal and segmental glomerulosclerosis. Here, suPAR acts on podocytes by activating αvβ3 integrins. Large observational studies showed that suPAR also predicts chronic kidney disease incidence and progression by predating the disease by several years prior to any other known marker of renal dysfunction...
August 2017: Pflügers Archiv: European Journal of Physiology
https://www.readbyqxmd.com/read/28664408/focal-segmental-glomerulosclerosis-why-does-it-occur-segmentally
#14
REVIEW
Michio Nagata, Namiko Kobayashi, Satoshi Hara
Podocyte loss is the fundamental basis of glomerulosclerosis. Focal segmental glomerulosclerosis (FSGS) is a progressive glomerular disease, and its glomerular features are a prototype of podocyte loss-driven glomerulosclerosis. The glomerular pathology of FSGS is characterized by a focal and segmental location of the sclerotic lesions in human FSGS; segmental sclerosis often shows simultaneous intra- and extra-capillary changes, including parietal cell migration, capillary collapse, hyaline deposition, and intra-capillary thrombi and occasional hypercellularity...
August 2017: Pflügers Archiv: European Journal of Physiology
https://www.readbyqxmd.com/read/28664407/combined-use-of-electron-microscopy-and-intravital-imaging-captures-morphological-and-functional-features-of-podocyte-detachment
#15
REVIEW
James L Burford, Georgina Gyarmati, Isao Shirato, Wilhelm Kriz, Kevin V Lemley, János Peti-Peterdi
The development of podocyte injury and albuminuria in various glomerular pathologies is still incompletely understood due to technical limitations in studying the glomerular filtration barrier (GFB) in real-time. We aimed to directly visualize the early morphological and functional changes of the GFB during the development of focal segmental glomerulosclerosis (FSGS) using a combination of transmission electron microscopy (TEM) and in vivo multiphoton microscopy (MPM) in the rat puromycin aminonucleoside (PAN) model...
August 2017: Pflügers Archiv: European Journal of Physiology
https://www.readbyqxmd.com/read/28663063/development-of-focal-segmental-glomerulosclerosis-patient-reported-outcome-measures-symptom-diary-and-symptom-impact-questionnaire
#16
Susan D Mathias, Susan Vallow, Debbie S Gipson, Kevin S Thorneloe, Dennis Sprecher
BACKGROUND: Focal segmental glomerulosclerosis (FSGS) is a kidney disease that affects patients' functioning and well-being. This study aimed to develop patient-reported outcome questionnaires to measure patient experiences related to FSGS. STUDY DESIGN: Qualitative patient interviews to identify important symptoms and concepts (concept elicitation) formed the basis for the development of 2 questionnaires, one on symptoms and one on their impact. Additional qualitative interviews were implemented to evaluate/refine the questionnaires (cognitive debriefing)...
June 26, 2017: American Journal of Kidney Diseases: the Official Journal of the National Kidney Foundation
https://www.readbyqxmd.com/read/28658768/clinicopathological-analysis-of-glomerular-disease-of-adult-onset-nephrotic-syndrome-in-an-indian-cohort-a-retrospective-study
#17
Mayur Suryawanshi, Swapnil Karnik, Sanjeet Roy
INTRODUCTION: Primary glomerular disease presenting with adult onset nephrotic syndrome are a major cause of chronic renal failure worldwide. The spectrum of renal disease presenting with nephrotic syndrome has undergone a gradual change globally over the course of time. However, there still exist regional differences in the incidence of primary glomerular diseases causing adult onset nephrotic syndrome. AIM: To observe the spectrum of renal diseases presenting with adult onset nephrotic syndrome with comparative analysis of changing trends over the last five decades with regards to Western and Indian literature...
May 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28658762/role-of-immunofluorescence-in-adult-onset-nephrotic-syndrome-a-study-in-a-tertiary-care-centre-of-western-india
#18
Sharada Rane, Prerana Mutyal, Nicholas Dcunha, Mayur Parkhi, Meenal Jadhav
INTRODUCTION: Study of renal Immunofluorescence (IF) is an ancillary but essential technique in evaluation of renal biopsies in glomerulopathies and also it enlightens on the pathogenesis of nephrotic syndrome. AIM: To determine the role of IF in evaluating definite subtyping and diagnosis of adult onset nephrotic syndrome and attempting clinicopathological correlation. MATERIALS AND METHODS: A total of 52 patients of adult onset nephrotic syndrome were evaluated clinically and with pertinent investigations; and subjected to USG guided percutaneous renal biopsy which was processed and stained for light microscopy and for immunofluorescence by direct method (DIF) using antibodies against IgG, IgM, IgA and C3...
May 2017: Journal of Clinical and Diagnostic Research: JCDR
https://www.readbyqxmd.com/read/28646406/podocytes-and-the-quest-for-precision-medicines-for-kidney-diseases
#19
REVIEW
Peter Mundel
In this review, I describe a 30-year journey in the quest for precision medicines for patients with kidney diseases. In 1987, when I started my reseach career, most scientists studying glomerular disease biology were focused on mesangial cells. The crucial role of the podocyte in many kidney diseases characterized by proteinuria, including focal segmental glomerulosclerosis (FSGS) and diabetic nephropathy, had not yet been recognized. We were not aware of genetic causes or drivers of kidney diseases nor of molecular markers and cell culture systems for mechanistic studies of podocyte biology...
August 2017: Pflügers Archiv: European Journal of Physiology
https://www.readbyqxmd.com/read/28643120/the-incidence-of-the-nephrotic-syndrome-in-childhood-in-germany
#20
Ingo Franke, Malik Aydin, Corinna Elke Llamas Lopez, Lisa Kurylowicz, Rainer Ganschow, Michael Lentze, Mark Born
BACKGROUND: The incidence of childhood nephrotic syndrome (NS) in Germany is not well known. METHODS: An ESPED-based nationwide collection of epidemiological data of children in 2005 and 2006. RESULT: The mean age of NS at onset was 5.5 ± 3.7 years. The gender ratio of boys to girls was 1.8:1. The average length of stay was 15.5 ± 11.2 days, with younger children remaining significantly longer in hospital. Steroid-resistance was more common in children ≥8 years (p = 0...
June 22, 2017: Clinical and Experimental Nephrology
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