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focal segmental glomerulosclerosis

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https://www.readbyqxmd.com/read/29350347/low-birth-weight-is-a-conditioning-factor-for-podocyte-alteration-and-steroid-dependance-in-children-with-nephrotic-syndrome
#1
Giovanni Conti, Dominique De Vivo, Claudia Fede, Stefania Arasi, Angela Alibrandi, Roberto Chimenz, Domenico Santoro
BACKGROUND: Low birth weight (LBW) is associated with reduced nephron endowment. Clinical-pathologic features of post adaptive focal segmental glomerulosclerosis (FSGS) have been observed in subjects with prematurity and very LBW. METHODS: We aimed to investigate the correlation between LBW and outcome in a cohort of 89 children with idiopathic nephrotic syndrome (NS) (2-12 years-old at onset, followed for > 3 years), of whom 21 with LBW (birth weight < 10th percentile for gestational age, gender, ethnicity, and maternal parity or birth weight < 2500 g)...
January 19, 2018: Journal of Nephrology
https://www.readbyqxmd.com/read/29340328/nephrin-loss-can-be-used-to-predict-remission-and-long-term-renal-outcome-in%C3%A2-patients-with-minimal-change-disease
#2
Nina A van de Lest, Malu Zandbergen, Daphne H T IJpelaar, Ron Wolterbeek, Jan A Bruijn, Ingeborg M Bajema, Marion Scharpfenecker
Introduction: Minimal change disease is a common cause of nephrotic syndrome. In general, patients with minimal change disease respond to corticosteroids and have excellent long-term renal survival. However, some patients have less favorable outcome. These patients are often thought to have progressed to focal segmental glomerulosclerosis. We previously reported that a segmental loss of podocyte markers is present before the development of focal segmental glomerulosclerosis in a rat model...
January 2018: KI Reports
https://www.readbyqxmd.com/read/29340321/randomized-clinical-trial-design-to-assess-abatacept-in-resistant-nephrotic-syndrome
#3
Howard Trachtman, Debbie S Gipson, Michael Somers, Cathie Spino, Sharon Adler, Lawrence Holzman, Jeffrey B Kopp, John Sedor, Sandra Overfield, Ayanbola Elegbe, Michael Maldonado, Anna Greka
Introduction: Treatment-resistant nephrotic syndrome is a rare form of glomerular disease that occurs in children and adults. No Food and Drug Administration-approved treatments consistently achieve remission of proteinuria and preservation of kidney function. CD80 (B7-1) can be expressed on injured podocytes, and administration of abatacept (modified CTLA4-Ig based on a natural ligand to CD80) has been associated with sustained normalization of urinary protein excretion and maintenance of glomerular filtration rate in experimental and clinical settings...
January 2018: KI Reports
https://www.readbyqxmd.com/read/29340309/strongyloides-stercoralis-associated-tip-variant-focal-segmental-glomerulosclerosis
#4
Massini Merzkani, Nupur N Uppal, Daniel W Ross, Pranisha Gautam-Goyal, Prashant Malhotra, Hitesh H Shah, Kenar D Jhaveri, Vivette D D'Agati
No abstract text is available yet for this article.
January 2018: KI Reports
https://www.readbyqxmd.com/read/29337702/changing-concepts-of-hiv-infection-and-renal-disease
#5
Jean Hou, Cynthia C Nast
PURPOSE OF REVIEW: Human immunodeficiency virus (HIV)-associated nephropathy (HIVAN) was identified as the major renal manifestation of HIV infection early in the HIV epidemic. However, HIV infection now is associated with a different spectrum of renal lesions leading to chronic kidney disease. This review examines the changes in kidney injury occurring in the current HIV era and the factors involved in this transformation of disease expression. RECENT FINDINGS: The incidence of HIVAN and opportunistic infections in HIV-infected individuals has declined in concert with the use of effective combination antiretroviral agents...
January 13, 2018: Current Opinion in Nephrology and Hypertension
https://www.readbyqxmd.com/read/29337051/fermt2-links-cortical-actin-structures-plasma-membrane-tension-and-focal-adhesion-function-to-stabilize-podocyte-morphology
#6
M Yasuda-Yamahara, M Rogg, J Frimmel, P Trachte, M Helmstaedter, P Schroder, M Schiffer, C Schell, T B Huber
Simplification and retraction of podocyte protrusions, generally termed as foot process effacement, is a uniform pathological pattern observed in the majority of glomerular disease, including focal segmental glomerulosclerosis. However, it is still incompletely understood how the interaction of cortical actin structures, actomyosin contractility and focal adhesions, is being orchestrated to control foot process morphology in health and disease. By uncovering the functional role of fermitin family member 2 (FERMT2 or kindlin-2) in podocytes, we provide now evidence, how cell-extracellular matrix (ECM) interactions modulate membrane tension and actomyosin contractility...
January 11, 2018: Matrix Biology: Journal of the International Society for Matrix Biology
https://www.readbyqxmd.com/read/29328453/aldosterone-is-involved-in-the-pathogenesis-of-obesity-related-glomerulopathy-through-activation-of-wnt-%C3%AE-catenin-signaling-in-podocytes
#7
Jia-Jia Zhu, Yi-Pu Chen, Min Yang, Bao-Li Liu, Jing Dong, Hong-Rui Dong, Hong-Liang Rui, Hong Cheng
Obesity-related glomerulopathy (ORG) is morphologically characterized by glomerulomegaly with or without observable focal segmental glomerulosclerosis under light microscope, with decreased podocyte density and number, and with increased foot‑process width observed under electron microscope. The severity of podocyte injury is correlated with the degree of proteinuria and renal dysfunction. However, the pathogenesis of ORG is not well understood. The aim of the present study was to explore the possible pathogenic role of aldosterone (ALDO) in ORG...
January 5, 2018: Molecular Medicine Reports
https://www.readbyqxmd.com/read/29327821/chronic-kidney-disease-in-patients-with-chronic-hepatitis-c-virus-infection
#8
Omer Shahab, Pegah Golabi, Zobair M Younossi
Hepatitis C virus (HCV) infection affects many organs in the body, including the liver, kidneys, skin, joints and others. Although the hepatic manifestation of HCV has been widely studied, the extrahepatic manifestaions of HCV have not been fully appreciated. Studies have shown that patients with HCV have a higher risk of chronic kidney disease and end-stage renal disease, as well as poorer outcomes after kidney transplantation. Given these findings, it is important to screen HCV patients for presence of renal impairement in a timely manner...
January 10, 2018: Minerva Gastroenterologica e Dietologica
https://www.readbyqxmd.com/read/29321142/differentiating-primary-genetic-and-secondary-fsgs-in-adults-a-clinicopathologic-approach
#9
An S De Vriese, Sanjeev Sethi, Karl A Nath, Richard J Glassock, Fernando C Fervenza
FSGS describes a renal histologic lesion with diverse causes and pathogenicities that are linked by podocyte injury and depletion. Subclasses of FSGS include primary, genetic, and secondary forms, the latter comprising maladaptive, viral, and drug-induced FSGS. Despite sharing certain clinical and histologic features, these subclasses differ noticeably in management and prognosis. Without an accepted nongenetic biomarker that discriminates among these FSGS types, classification of patients is often challenging...
January 10, 2018: Journal of the American Society of Nephrology: JASN
https://www.readbyqxmd.com/read/29320993/distribution-of-glomerular-diseases-in-taiwan-preliminary-report-of-national-renal-biopsy-registry-publication-on-behalf-of-taiwan-society-of-nephrology
#10
Hsien-Fu Chiu, Hung-Chun Chen, Kuo-Cheng Lu, Kuo-Hsiung Shu
BACKGROUND: Despite the development of biomarkers and noninvasive imaging tools, biopsy remains the only method for correctly diagnosing patients with unexplained hematuria, proteinuria and renal failure. Renal biopsy has been performed for several decades in Taiwan; however, a national data registry is still lacking until 2013. METHODS: The Renal Biopsy Registry Committee was established within the Taiwan Society of Nephrology in January 2013. A biopsy registry format, including basic demographic data, baseline clinical features, laboratory data, and clinical and pathological diagnosis was developed...
January 10, 2018: BMC Nephrology
https://www.readbyqxmd.com/read/29319763/current-distribution-pattern-of-biopsy-proven-glomerular-disease-in-salvador-brazil-40-years-after-an-initial-assessment
#11
Washington Luis Conrado Dos-Santos, Glória Maria Maranhão Sweet, Labene Gondim Azevêdo, Maria Brandão Tavares, Maria Fernanda Sanches Soares, Caroline Vilas Boas de Melo, Márcia Fernanda Melo Carneiro, Rilma Ferreira de Souza Santos, Márcia Cristina Conrado, Daniela Teixeira Leal Braga, Marcia Carvalho Bessa, Nathanael de Freitas Pinheiro Junior, Marília Bahiense-Oliveira
INTRODUCTION: A report on the prevalence of glomerular disease diagnosed via renal biopsy in Salvador, BA, Brazil was published in 1973 and showed a predominance of membranoproliferative glomerulonephritis, which was frequently associated with hepatosplenic schistosomiasis. OBJECTIVE: In this study, we investigate the potential changes in the distribution of glomerular diseases after a period of important epidemiological transition in Brazil. METHODS: Pathology reports of all patients subjected to kidney biopsy from 2003 to 2015 in a referral nephrology service were reviewed...
October 2017: Jornal Brasileiro de Nefrologia: ʹorgão Oficial de Sociedades Brasileira e Latino-Americana de Nefrologia
https://www.readbyqxmd.com/read/29319762/complement-system-and-c4d-expression-in-cases-of-membranous-nephropathy
#12
Fabiano Bichuette Custódio, Crislaine Aparecida da Silva, Fernanda Rodrigues Helmo, Juliana Reis Machado, Marlene Antônia Dos Reis
INTRODUCTION: Membranous nephropathy (MN) is one of the major causes of nephrotic syndrome. The complement system plays a key role in the pathophysiology of MN. OBJECTIVES: To identify the complement pathway possibly activated in MN cases and correlate the presence of C4d with more severe clinical and histological markers. METHODS: Sixty nine cases from renal biopsy with membranous nephropathy were investigated. The presence of C1q was analyzed by direct immunofluorescence; and expression of C4d by immunohistochemistry...
October 2017: Jornal Brasileiro de Nefrologia: ʹorgão Oficial de Sociedades Brasileira e Latino-Americana de Nefrologia
https://www.readbyqxmd.com/read/29310486/focal-segmental-glomerulosclerosis-in-children-complicated-by-posterior-reversible-encephalopathy-syndrome
#13
Magdalena Stârcea, Cristina Gavrilovici, Mihaela Munteanu, Ingrith Miron
An uncommon side effect of cyclosporine A (CsA) use is posterior reversible encephalopathy syndrome (PRES). PRES usually develops because of disturbed capacity of posterior cerebral blood flow to autoregulate an acute rise in blood pressure. We present the case of a 10-year-old girl who was previously diagnosed in our department with focal segmental glomerulosclerosis. She was treated with CsA and developed seizures, progressive loss of consciousness, and visual disturbance on the 7th day of treatment. Brain magnetic resonance imaging showed degeneration of white matter with diffuse demyelination in the parietal and posterior occipital lobes, consistent with the diagnosis of PRES...
January 1, 2018: Journal of International Medical Research
https://www.readbyqxmd.com/read/29277509/mortality-and-allograft-loss-trends-among-us-pediatric-kidney-transplant-recipients-with-and-without-focal-segmental-glomerulosclerosis
#14
Chia-Shi Wang, Jennifer Gander, Rachel E Patzer, Larry A Greenbaum
BACKGROUND: Pediatric patients with focal segmental glomerulosclerosis (FSGS) have high rates of disease recurrence and allograft failure after kidney transplantation, but there are few data for long-term survival posttransplantation. STUDY DESIGN: Retrospective cohort study. SETTING & PARTICIPANTS: 12,303 pediatric patients (aged <18 years), including 1,408 (11%) patients with FSGS, who received a first kidney transplant in 1990 through 2009 and were followed up through June 2015 were identified from the US Renal Data System database...
December 22, 2017: American Journal of Kidney Diseases: the Official Journal of the National Kidney Foundation
https://www.readbyqxmd.com/read/29276101/cd44-is-required-for-the-pathogenesis-of-experimental-crescentic-glomerulonephritis-and-collapsing-focal-segmental-glomerulosclerosis
#15
Jennifer Eymael, Shagun Sharma, Markus A Loeven, Jack F Wetzels, Fieke Mooren, Sandrine Florquin, Jeroen K Deegens, Brigith K Willemsen, Vikram Sharma, Toin H van Kuppevelt, Marinka A Bakker, Tammo Ostendorf, Marcus J Moeller, Henry B Dijkman, Bart Smeets, Johan van der Vlag
A key feature of glomerular diseases such as crescentic glomerulonephritis and focal segmental glomerulosclerosis is the activation, migration and proliferation of parietal epithelial cells. CD44-positive activated parietal epithelial cells have been identified in proliferative cellular lesions in glomerular disease. However, it remains unknown whether CD44-positive parietal epithelial cells contribute to the pathogenesis of scarring glomerular diseases. Here, we evaluated this in experimental crescentic glomerulonephritis and the transgenic anti-Thy1...
December 21, 2017: Kidney International
https://www.readbyqxmd.com/read/29273332/global-glomerulosclerosis-with-nephrotic-syndrome-the-clinical-importance-of-age%C3%A2-adjustment
#16
Musab S Hommos, Caihong Zeng, Zhihong Liu, Jonathan P Troost, Avi Z Rosenberg, Matthew Palmer, Walter K Kremers, Lynn D Cornell, Fernando C Fervenza, Laura Barisoni, Andrew D Rule
Globally sclerotic glomeruli (GSG) occur with both normal aging and kidney disease. However, it is unknown whether any GSG or only GSG exceeding that expected for age is clinically important. To evaluate this, we identified patients with a glomerulopathy that often presents with nephrotic syndrome (focal segmental glomerulosclerosis, membranous nephropathy, or minimal change disease) in the setting of the Nephrotic Syndrome Study Network (NEPTUNE), China-Digital Kidney Pathology (DiKiP), and the Southeast Minnesota cohorts...
December 19, 2017: Kidney International
https://www.readbyqxmd.com/read/29259860/steroid-resistant-nephrotic-syndrome-caused-by-co-inheritance-of-mutations-at-nphs1-and-adck4-genes-in-two-chinese-siblings
#17
Hongwen Zhang, Fang Wang, Xiaoyu Liu, Xuhui Zhong, Yong Yao, Huijie Xiao
Hereditary nephrotic syndrome often presents with steroid-resistance and onset within the first year of life. Mutations in genes highly expressed in podocytes have been found in two thirds of these patients, especially NPHS1 and NPHS2 among at least 29 genetic causes that have been discovered. We reported two siblings with steroid-resistant nephrotic syndrome caused by co-inheritance of mutations at NPHS1 (c.1339G>A, p.E447K) and ACDK4 (c.748G>C, p.D250H) genes. The siblings presented with steroid-resistant nephrotic syndrome and pathological lesions of focal segmental glomerulosclerosis (FSGS), while the elder sister also developed hypertension, renal failure and cardiac dysfunction...
November 2017: Intractable & Rare Diseases Research
https://www.readbyqxmd.com/read/29250696/nephrotic-syndrome-after-autologous-hematopoietic-stem-cell-transplantation-a-case-report
#18
Bogdan Obrişcă, Adriana Roxana JurubiŢă, Andreea Gabriella Andronesi, Mihaela Gherghiceanu, Gener Ismail, George Mitroi, Mihai Cristian Hârza
Nephrotic syndrome (NS) is a rare complication of hematopoietic cell transplantation (HCT) and is thought to represent a renal manifestation of chronic graft-versus-host disease (cGVHD). Glomerulopathies occur less often in recipients of autologous as compared to allogeneic HCT and, in this setting, renal pathology is less well characterized. This case report describes a 54-year-old man admitted for the evaluation of a nephrotic-range proteinuria. His past medical history included a ? light-chain secreting multiple myeloma (MM) for which he underwent autologous HCT...
2017: Romanian Journal of Morphology and Embryology, Revue Roumaine de Morphologie et Embryologie
https://www.readbyqxmd.com/read/29249233/abatacept-efficacy-in-steroid-resistant-minimal-change-disease-revealed-by-the-speed-of-proteinuria-reduction-after-the-start-of-abatacept%C3%A2
#19
David Dado, Samir Parikh, Isabelle Ayoub, Brad Rovin, Tibor Nadasdy, Lee Hebert
A unique characteristic of the response of minimal-change disease (MCD) or focal and segmental glomerulosclerosis (FSGS) to steroid therapy is that the remission of proteinuria occurs quickly, for example, within 4 - 6 weeks of the onset of steroid therapy, even in those with severe nephrotic syndrome. Remission of proteinuria in MCD and FSGS can also occur spontaneously (not steroid induced). However, spontaneous remission usually proceeds over several months or longer. Recently, there have been several reports that abatacept can induce proteinuria remission in MCD and FSGS...
December 18, 2017: Clinical Nephrology
https://www.readbyqxmd.com/read/29241625/vinculin-is-required-to-maintain-glomerular-barrier-integrity
#20
Franziska Lausecker, Xuefei Tian, Kazunori Inoue, Zhen Wang, Christopher E Pedigo, Hossam Hassan, Chang Liu, Margaret Zimmer, Stephanie Jinno, Abby L Huckle, Hellyeh Hamidi, Robert S Ross, Roy Zent, Christoph Ballestrem, Rachel Lennon, Shuta Ishibe
Cell-matrix interactions and podocyte intercellular junctions are key for maintaining the glomerular filtration barrier. Vinculin, a cytoplasmic protein, couples actin filaments to integrin-mediated cell-matrix adhesions and to cadherin-based intercellular junctions. Here, we examined the role of vinculin in podocytes by the generation of a podocyte-specific knockout mouse. Mice lacking podocyte vinculin had increased albuminuria and foot process effacement following injury in vivo. Analysis of primary podocytes isolated from the mutant mice revealed defects in cell protrusions, altered focal adhesion size and signaling, as well as impaired cell migration...
December 11, 2017: Kidney International
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