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Focal and segmental glomerulosclerosis

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https://www.readbyqxmd.com/read/28724797/increasing-the-level-of-peroxisome-proliferator-activated-receptor-%C3%AE-coactivator-1%C3%AE-in-podocytes-results-in-collapsing-glomerulopathy
#1
Szu-Yuan Li, Jihwan Park, Chengxiang Qiu, Seung Hyeok Han, Matthew B Palmer, Zoltan Arany, Katalin Susztak
Inherited and acquired mitochondrial defects have been associated with podocyte dysfunction and chronic kidney disease (CKD). Peroxisome proliferator-activated receptor γ coactivator-1α (PGC1α) is one of the main transcriptional regulators of mitochondrial biogenesis and function. We hypothesized that increasing PGC1α expression in podocytes could protect from CKD. We found that PGC1α and mitochondrial transcript levels are lower in podocytes of patients and mouse models with diabetic kidney disease (DKD)...
July 20, 2017: JCI Insight
https://www.readbyqxmd.com/read/28724378/erratum-to-significance-of-the-urokinase-type-plasminogen-activator-and-its-receptor-in-the-progression-of-focal-segmental-glomerulosclerosis-in-clinical-and-mouse-models
#2
Jin-Shuen Chen, Li-Chien Chang, Chung-Ze Wu, Tzu-Ling Tseng, Jui-An Lin, Yuh-Feng Lin, Chao-Wen Cheng
No abstract text is available yet for this article.
July 19, 2017: Journal of Biomedical Science
https://www.readbyqxmd.com/read/28722118/at2-r-deficiency-mediated-podocyte-loss-via-activation-of-ectopic-hedgehog-interacting-protein-hhip-gene-expression
#3
Min-Chun Liao, Xin-Ping Zhao, Shiao-Ying Chang, Chao-Sheng Lo, Isabelle Chenier, Tomoko Takano, Julie R Ingelfinger, Shao-Ling Zhang
Angiotensin II type 2 receptor (AT2 R) deficiency in AT2 R knockout (KO) mice has been linked to congenital abnormalities of the kidney and urinary tract; however, the mechanisms by which this occurs are poorly understood. In this study, we examined whether AT2 R deficiency impaired glomerulogenesis and mediated podocyte loss/dysfunction in vivo and in vitro. Nephrin-cyan fluorescent protein (CFP)-transgenic (Tg) and Nephrin/AT2 RKO mice were used to assess glomerulogenesis, while wild-type and AT2 RKO mice were used to evaluate maturation of podocyte morphology/function...
July 19, 2017: Journal of Pathology
https://www.readbyqxmd.com/read/28707483/investigational-drugs-in-development-for-focal-segmental-glomerulosclerosis
#4
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...
July 14, 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
#5
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
#6
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...
July 8, 2017: Pflügers Archiv: European Journal of Physiology
https://www.readbyqxmd.com/read/28682562/-renal-diseases-related-to-myh9-disorders
#7
Dario Galeano, Luca Zanoli, Vincenzo L'Imperio, Pasquale Fatuzzo, Antonio Granata
Mutations in MYH9 gene encoding the nonmuscle myosin heavy chain IIA (NMMHC-IIA) are related to a number of rare autosomal-dominant disorders which has been known as May-Hegglin disease, Sebastian syndrome, Fechtner syndrome and Epstein syndrome. Their common clinical features are congenital macrothrombocytopaenia and polymorphonuclear inclusion bodies, in addition to a variable risk of developing proteinuria, chronic kidney disease progressing toward end stage, sensorineural deafness and presenile cataracts...
April 2017: Giornale Italiano di Nefrologia: Organo Ufficiale Della Società Italiana di Nefrologia
https://www.readbyqxmd.com/read/28664408/focal-segmental-glomerulosclerosis-why-does-it-occur-segmentally
#8
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...
June 29, 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
#9
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...
June 29, 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
#10
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
#11
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
#12
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
#13
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...
June 23, 2017: Pflügers Archiv: European Journal of Physiology
https://www.readbyqxmd.com/read/28643120/the-incidence-of-the-nephrotic-syndrome-in-childhood-in-germany
#14
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
https://www.readbyqxmd.com/read/28642464/serum-c3-and-renal-outcome-in-patients-with-primary-focal-segmental-glomerulosclerosis
#15
Jian Liu, Jingyuan Xie, Xiaoyan Zhang, Jun Tong, Xu Hao, Hong Ren, Weiming Wang, Nan Chen
The role of complement (C) in the pathogenesis or progression of focal segmental glomerulosclerosis (FSGS) is uncertain. The present study assessed the relationship between serum C3, the baseline characteristics, and the progression of FSGS in the cohort and identified the clinical implications of serum C3 levels in patients with FSGS. Compared to the patients with C3 ≥ 85 mg/dL (N = 474), those with C3 < 85 mg/dL (N = 117) presented a higher level of serum creatinine, lower levels of eGFR, hemoglobin, proteinuria, triglyceride, cholesterol, IgA, as well as, severe tubulointerstitial injury (TI)...
June 22, 2017: Scientific Reports
https://www.readbyqxmd.com/read/28639628/multiple-socioeconomic-deprivation-and-impact-on-survival-in-patients-with-primary-glomerulonephritis
#16
Emily P McQuarrie, Bruce Mackinnon, Samira Bell, Stewart Fleming, Valerie McNeice, Graham Stewart, Jonathan G Fox, Colin C Geddes
Background: The impact of multiple socio-economic deprivation on patient outcomes in primary renal diseases is unknown. We aimed to assess whether risk of death or requiring renal replacement therapy (RRT) in patients with primary glomerulonephritis (GN) was higher in patients living in an area of multiple socio-economic deprivation. Methods: Patients undergoing native renal biopsy between 2000 and 2014 were identified. Baseline demographics, postcode at time of biopsy, follow-up blood pressure, proteinuria and time to death or RRT were recorded...
February 2017: Clinical Kidney Journal
https://www.readbyqxmd.com/read/28632965/frequent-col4-mutations-in-familial-microhematuria-accompanied-by-later-onset-alport-nephropathy-due-to-focal-segmental-glomerulosclerosis
#17
Louiza Papazachariou, Gregory Papagregoriou, Despina Hadjipanagi, Panagiota Demosthenous, Konstantinos Voskarides, Constantina Koutsofti, Kostas Stylianou, Petros Ioannou, Dimitris Xydakis, Ioannis Tzanakis, Antonia Papadaki, Nicolaos Kallivretakis, Nicolaos Nikolakakis, Garyfalia Perysinaki, Daniel P Gale, Athanasios Diamantopoulos, Pavlos Goudas, Dimitris Goumenos, Andreas Soloukides, Ioannis Boletis, Christina Melexopoulou, Eleni Georgaki, Elena Frysira, Fifi Komianou, Dimitrios Grekas, Christos Paliouras, Polichronis Alivanis, George Vergoulas, Alkis Pierides, Eugenios Daphnis, Constantinos Deltas
Familial microscopic hematuria (FMH) is associated with a genetically heterogeneous group of conditions including the collagen-IV nephropathies, the heritable C3/CFHR5 nephropathy and the glomerulopathy with fibronectin deposits. The clinical course varies widely, ranging from isolated benign familial hematuria to end-stage renal disease (ESRD) later in life. We investigated 24 families using Next Generation Sequencing (NGS) for five genes: COL4A3, COL4A4, COL4A5, CFHR5 and FN1. In 17 families (71%), we found 15 pathogenic mutations in COL4A3/A4/A5, nine of them novel...
June 20, 2017: Clinical Genetics
https://www.readbyqxmd.com/read/28630634/moxibustion-alleviates-injury-in-a-rat-focal-segmental-glomerulosclerosis-model
#18
Yi Li, Yuxia Sun, Chunling Zhang, Ke Wang, Peicheng Shen, Di Huang, Wen Ma, Jin Zhang, Lin Li, Liqun He
OBJECTIVES: To evaluate the therapeutic effects of moxibustion at Shenshu (BL-23) and Geshu (BL-17) acupoints in a focal segmental glomerulosclerosis (FSGS) model in rats. METHODS: A FSGS rat model was established by single nephrectomy and repeated injection of doxorubicin. The FSGS rats were randomly divided into the model group, losartan (positive control) group, Shenshu moxibustion group, and Geshu moxibustion group. Molecular indicators of kidney function and renal pathological changes were monitored...
2017: Evidence-based Complementary and Alternative Medicine: ECAM
https://www.readbyqxmd.com/read/28626447/autoimmune-thyroiditis-and-glomerulopathies
#19
REVIEW
Domenico Santoro, Carmela Vadalà, Rossella Siligato, Michele Buemi, Salvatore Benvenga
Autoimmune thyroiditis (AIT) is generally associated with hypothyroidism. It affects ~2% of the female population and 0.2% of the male population. The evidence of thyroid function- and thyroid autoantibody-unrelated microproteinuria in almost half of patients with AIT and sometimes heavy proteinuria as in the nephrotic syndrome point to a link of AIT with renal disease. The most common renal diseases observed in AIT are membranous nephropathy, membranoproliferative glomerulonephritis, minimal change disease, IgA nephropathy, focal segmental glomerulosclerosis, antineutrophil cytoplasmic autoantibody (ANCA) vasculitis, and amyloidosis...
2017: Frontiers in Endocrinology
https://www.readbyqxmd.com/read/28626180/pregnancy-is-a-risk-factor-for-secondary-focal-segmental-glomerulosclerosis-in-women-with-a-history-of-very-low-birth-weight
#20
Mari Tanaka, Sachio Iwanari, Yasushi Tsujimoto, Keisuke Taniguchi, Koichiro Hagihara, Daiki Fumihara, Syo Miki, Saeko Shimoda, Masaki Ikeda, Hiroya Takeoka
Low birth weight (LBW) has been known to increase the susceptibility to renal injury in adulthood. A 26-year-old woman developed proteinuria in early pregnancy; she had been born with very LBW. The clinical course was progressive, and an emergency Caesarean section was performed at 36 weeks due to acute kidney injury. A renal biopsy provided a diagnosis of post-adaptive focal segmental glomerulosclerosis. Increased demand for glomerular filtration during early pregnancy appeared to have initiated the renal injury...
2017: Internal Medicine
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