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https://www.readbyqxmd.com/read/28236143/c3-glomerulopathy-and-eculizumab-a-report-on-four-paediatric-cases
#1
Célia Lebreton, Justine Bacchetta, Frédérique Dijoud, Lucie Bessenay, Véronique Fremeaux-Bacchi, Anne Laure Sellier-Leclerc
BACKGROUND: Eculizumab may be used to treat C3-glomerulopathy (C3G), a rare but severe glomerular disease. DIAGNOSIS AND TREATMENT: Patients 1, 2 and 3 were diagnosed with nephritic syndrome with alternative complement pathway activation (low C3, C3Nef-positive) and C3G at the age of 9, 13 and 12 years, respectively. Treatment with eculizumab normalized proteinuria within 1, 2 and 7 months, respectively. Proteinuria relapsed when eculizumab was withdrawn, but the re-introduction of eculizumab normalized proteinuria...
February 24, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28214961/obesity-and-kidney-disease-hidden-consequences-of-the-epidemic
#2
EDITORIAL
Csaba P Kovesdy, Susan L Furth, Carmine Zoccali
Obesity has become a worldwide epidemic, and its prevalence has been projected to grow by 40% in the next decade. This increasing prevalence has implications for the risk of diabetes, cardiovascular disease and also for Chronic Kidney Disease. A high body mass index is one of the strongest risk factors for new-onset Chronic Kidney Disease. In individuals affected by obesity, a compensatory hyperfiltration occurs to meet the heightened metabolic demands of the increased body weight. The increase in intraglomerular pressure can damage the kidneys and raise the risk of developing Chronic Kidney Disease in the long-term...
February 2017: Journal of Nephrology
https://www.readbyqxmd.com/read/28205394/obesity-and-kidney-disease-hidden-consequences-of-the-epidemic
#3
Csaba P Kovesdy, Susan L Furth, Carmine Zoccali
Obesity has become a worldwide epidemic, and its prevalence has been projected to grow by 40% in the next decade. This increasing prevalence has implications for the risks of diabetes, cardiovascular disease and also for Chronic Kidney Disease. A high body mass index is one of the strongest risk factors for new-onset Chronic Kidney Disease. In individuals affected by obesity, a series of complex pathophysiologic changes occur that lead to the development of Chronic Kidney Disease. These include on the one hand effects mediated by the downstream consequences of obesity (such as diabetes mellitus and hypertension), but also direct effects of adipose tissue, via humoral factors such as leptin, adiponectin, resistin and visfatin)...
March 2017: Journal of Renal Care
https://www.readbyqxmd.com/read/28194887/atgl-deficiency-induces-podocyte-apoptosis-and-leads-to-glomerular-filtration-barrier-damage
#4
Wen Chen, Youzhao Jiang, Jian Han, Jiongyu Hu, Ting He, Tiantian Yan, Huang Na, Qiong Zhang, Hao Mei, Yong Liao, Yuesheng Huang, Bing Chen
Abnormal lipid metabolism, renal lipid accumulation and lipotoxicity are associated with the pathological features of glomerulopathy. However, the mechanisms by which lipid accumulation leads to the development or progression of this disease have not been fully elucidated. In this work, we have identified a role for the rate-limiting enzyme in lipolysis, adipose triglyceride lipase (ATGL; also called patatin-like phospholipase domain-containing protein 2(PNPLP2), in renal lipid metabolism and kidney disease...
February 14, 2017: FEBS Journal
https://www.readbyqxmd.com/read/28178697/obesity-and-kidney-disease-hidden-consequences-of-the-epidemic
#5
Csaba P Kovesdy, Susan L Furth, Carmine Zoccali
Obesity has become a worldwide epidemic, and its prevalence has been projected to grow by 40% in the next decade. This increasing prevalence has implications for the risk of diabetes, cardiovascular disease, and also for chronic kidney disease. A high body mass index is one of the strongest risk factors for new-onset chronic kidney disease. In individuals affected by obesity, a compensatory hyperfiltration occurs to meet the heightened metabolic demands of the increased body weight. The increase in intraglomerular pressure can damage the kidneys and raise the risk of developing chronic kidney disease in the long-term...
February 8, 2017: American Journal of Nephrology
https://www.readbyqxmd.com/read/28171864/obesity-and-kidney-disease-hidden-consequences-of-the-epidemic
#6
Csaba P Kovesdy, Susan L Furth, Carmine Zoccali
Obesity has become a worldwide epidemic, and its prevalence has been projected to grow by 40% in the next decade. This increasing prevalence has implications for the risk of diabetes, cardiovascular disease, and also for chronic kidney disease. A high body mass index is one of the strongest risk factors for new-onset chronic kidney disease. In individuals affected by obesity, a compensatory hyperfiltration occurs to meet the heightened metabolic demands of the increased body weight. The increase in intraglomerular pressure can damage the kidneys and raise the risk of developing chronic kidney disease in the long-term...
February 8, 2017: Nephron
https://www.readbyqxmd.com/read/28149647/hepatitis-c-and-kidney-disease-a-narrative-review
#7
REVIEW
Rashad S Barsoum, Emad A William, Soha S Khalil
Hepatitis-C (HCV) infection can induce kidney injury, mostly due to formation of immune-complexes and cryoglobulins, and possibly to a direct cytopathic effect. It may cause acute kidney injury (AKI) as a part of systemic vasculitis, and augments the risk of AKI due to other etiologies. It is responsible for mesangiocapillary or membranous glomerulonephritis, and accelerates the progression of chronic kidney disease due to other causes. HCV infection increases cardiovascular and liver-related mortality in patients on regular dialysis...
March 2017: Journal of Advanced Research
https://www.readbyqxmd.com/read/28139294/c5-inhibition-prevents-renal-failure-in-a-mouse-model-of-lethal-c3-glomerulopathy
#8
Allison Lesher Williams, Damodar Gullipalli, Yoshiyasu Ueda, Sayaka Sato, Lin Zhou, Takashi Miwa, Kenneth S Tung, Wen-Chao Song
C3 glomerulopathy is a potentially life-threatening disease of the kidney caused by dysregulated alternative pathway complement activation. The specific complement mediator(s) responsible for kidney injury in C3 glomerulopathy are yet to be defined and no specific therapy is currently available. We previously developed a mouse model of lethal C3 glomerulopathy with factor H and properdin gene double mutations. Therefore, we used this model to examine the role of C5 and C5a receptor (C5aR) in the pathogenesis of the disease...
January 27, 2017: Kidney International
https://www.readbyqxmd.com/read/28098116/membranous-glomerulopathy-and-massive-cervical-lymphadenopathy-due-to-immunoglobulin-g4-disease
#9
Kamel El-Reshaid, Shaikha Al-Bader, John Madda
A 32-year-old male presented with acute and severe nephrotic syndrome as well as massive right cervical lymphadenopathy for <2 years. Computed tomography scan of the chest, abdomen, and pelvis did not reveal any lymphadenopathy. Histopathology and immunohistochemical testing of his lymph node biopsy showed infiltrate enriched with immunoglobulin G4 (IgG4)-positive plasma cells. His kidney biopsy showed granular membranous deposits of IgG4 in the basement membrane without interstitial infiltrate. Antiphospholipid 2 receptor antibodies were absent excluding its "idiopathic" nature...
January 2017: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/28069603/treatment-of-b-cell-disorder-improves-renal-outcome-of-patients-with-monoclonal-gammopathy-associated-c3-glomerulopathy
#10
Sophie Chauvet, Véronique Frémeaux-Bacchi, Florent Petitprez, Alexandre Karras, Laurent Daniel, Stéphane Burtey, Gabriel Choukroun, Yahsou Delmas, Dominique Guerrot, Arnaud François, Moglie Le Quintrec, Vincent Javaugue, David Ribes, Laurence Vrigneaud, Bertrand Arnulf, Jean Michel Goujon, Pierre Ronco, Guy Touchard, Frank Bridoux
The high frequency of monoclonal gammopathy in adult patients with C3 glomerulopathy (C3G) emphasizes the role of the monoclonal immunoglobulin (MIg) in the occurrence of renal disease and raises the issue of the therapeutic management. The aim of the study was to evaluate the effect of chemotherapy in a large cohort of patients with MIg-associated C3G. Fifty adult patients with MIg and biopsy-proven C3G were extracted from the French national database of C3G. We retrospectively compared renal outcomes in patients who received or not chemotherapy targeting the underlying B-cell clone...
January 9, 2017: Blood
https://www.readbyqxmd.com/read/28069266/the-clinicopathologic-characteristics-of-kidney-diseases-related-to-monotypic-iga-deposits
#11
Marguerite Vignon, Camille Cohen, Stanislas Faguer, Laure-Hélène Noel, Celine Guilbeau, Marion Rabant, Sarah Higgins, Aurélie Hummel, Alexandre Hertig, Hélène Francois, Moglie Lequintrec, Eve Vilaine, Bertrand Knebelmann, Jacques Pourrat, Dominique Chauveau, Jean-Michel Goujon, Vincent Javaugue, Guy Touchard, Khalil El Karoui, Frank Bridoux
Monoclonal gammopathy of renal significance (MGRS) regroups renal disorders caused by a monoclonal immunoglobulin without overt hematological malignancy. MGRS includes tubular disorders, glomerular disorders with organized deposits, and glomerular disorders with non-organized deposits, such as proliferative glomerulonephritis with monoclonal IgG deposits. Since glomerular involvement related to monotypic IgA deposits is poorly described we performed retrospective analysis and defined clinico-biological characteristics, renal pathology, and outcome in 19 referred patients...
March 2017: Kidney International
https://www.readbyqxmd.com/read/28058212/complement-related-kidney-diseases-recurrence-after-transplantation
#12
REVIEW
Maurizio Salvadori, Elisabetta Bertoni
The recurrence of renal disease after renal transplantation is becoming one of the main causes of graft loss after kidney transplantation. This principally concerns some of the original diseases as the atypical hemolytic uremic syndrome (HUS), the membranoproliferative glomerulonephritis (MPGN), in particular the MPGN now called C3 glomerulopathy. Both this groups of renal diseases are characterized by congenital (genetic) or acquired (auto-antibodies) modifications of the alternative pathway of complement...
December 24, 2016: World Journal of Transplantation
https://www.readbyqxmd.com/read/28031959/averting-the-legacy-of-kidney-disease-focus-on-childhood
#13
REVIEW
Julie R Ingelfinger, Kamyar Kalantar-Zadeh, Franz Schaefer
World Kidney Day 2016 focuses on kidney disease in childhood and the antecedents of adult kidney disease that can begin in earliest childhood. Chronic kidney disease (CKD) in childhood differs from that in adults, as the largest diagnostic group among children includes congenital anomalies and inherited disorders, with glomerulopathies and kidney disease in the setting of diabetes being relatively uncommon. In addition, many children with acute kidney injury will ultimately develop sequelae that may lead to hypertension and CKD in later childhood or in adult life...
June 2016: Future Science OA
https://www.readbyqxmd.com/read/27994856/c3-glomerulopathy-in-adults-a-distinct-patient-subset-showing-frequent-association-with-monoclonal-gammopathy-and-poor-renal-outcome
#14
Isaac E Lloyd, Alexander Gallan, Hunter K Huston, Kalani L Raphael, Dylan V Miller, Monica P Revelo, Mazdak A Khalighi
BACKGROUND: C3 glomerulopathy (C3G) includes both C3 glomerulonephritis (C3GN) and dense deposit disease (DDD) and is defined by C3-dominant deposits on immunofluorescence. Dysfunction of the alternative pathway (AP) of complement is central to the pathophysiology of C3G and young patients often harbor genetic alterations of AP mediators. Recently, a link between C3G and paraproteinemia has been established. We performed this study to better characterize older patients with C3G where this association is more frequently seen...
December 2016: Clinical Kidney Journal
https://www.readbyqxmd.com/read/27994854/consequences-of-morbid-obesity-on-the-kidney-where-are-we-going
#15
Maruja Navarro Díaz
Obesity and morbid obesity are modifiable risk factors for the development and progression of kidney disease. Obesity has reached epidemic proportions and is currently an important health problem in Europe, so it is necessary to develop therapeutic and preventive strategies. The obesity-related glomerulopathy has been defined as a secondary form of focal segmental glomerulosclerosis, and its most characteristic feature is glomerulomegaly. The renal evolution of patients with obesity-related glomerulopathy (ORG) who have not been treated is unfavourable...
December 2016: Clinical Kidney Journal
https://www.readbyqxmd.com/read/27989322/atypical-hemolytic-uremic-syndrome-and-c3-glomerulopathy-conclusions-from-a-kidney-disease-improving-global-outcomes-kdigo-controversies-conference
#16
Timothy H J Goodship, H Terence Cook, Fadi Fakhouri, Fernando C Fervenza, Véronique Frémeaux-Bacchi, David Kavanagh, Carla M Nester, Marina Noris, Matthew C Pickering, Santiago Rodríguez de Córdoba, Lubka T Roumenina, Sanjeev Sethi, Richard J H Smith
In both atypical hemolytic uremic syndrome (aHUS) and C3 glomerulopathy (C3G) complement plays a primary role in disease pathogenesis. Herein we report the outcome of a 2015 Kidney Disease: Improving Global Outcomes (KDIGO) Controversies Conference where key issues in the management of these 2 diseases were considered by a global panel of experts. Areas addressed included renal pathology, clinical phenotype and assessment, genetic drivers of disease, acquired drivers of disease, and treatment strategies. In order to help guide clinicians who are caring for such patients, recommendations for best treatment strategies were discussed at length, providing the evidence base underpinning current treatment options...
March 2017: Kidney International
https://www.readbyqxmd.com/read/27932161/sofosbuvir-monotherapy-for-asymptomatic-and-noncirrhotic-hepatitis-c-infection-in-a-renal-retransplantation-recipient-a-case-report
#17
G Deng, J Ma, S Shen, Q Li, L Peng, S Meng, J Zhou, J Wu, D Liu
Renal transplant recipients infected with hepatitis C virus (HCV) have a high risk of progressing to cirrhosis, end-stage liver diseases, and hepatocellular carcinoma. It is also considered as an independent risk for graft loss and is correlated with proteinuria, transplant glomerulopathy, HCV-associated glomerulonephritis, and chronic rejection. Previous therapy involving interferon alfa and ribavirin led to treatment complications, including toxicity, anemia, sepsis, and drug-drug interactions with calcineurin inhibitors, as well as reduced tolerability and efficacy...
November 2016: Transplantation Proceedings
https://www.readbyqxmd.com/read/27913625/absence-of-mir-146a-in-podocytes-increases-risk-of-diabetic-glomerulopathy-via-up-regulation-of-erbb4-and-notch-1
#18
Ha Won Lee, Samia Q Khan, Shehryar Khaliqdina, Mehmet M Altintas, Florian Grahammer, Jimmy L Zhao, Kwi Hye Koh, Nicholas J Tardi, Mohd Hafeez Faridi, Terese Geraghty, David J Cimbaluk, Katalin Susztak, Luis F Moita, David Baltimore, Pierre-Louis Tharaux, Tobias B Huber, Matthias Kretzler, Markus Bitzer, Jochen Reiser, Vineet Gupta
Podocyte injury is an early event in diabetic kidney disease and is a hallmark of glomerulopathy. MicroRNA-146a (miR-146a) is highly expressed in many cell types under homeostatic conditions, and plays an important anti-inflammatory role in myeloid cells. However, its role in podocytes is unclear. Here, we show that miR-146a expression levels decrease in the glomeruli of patients with type 2 diabetes (T2D), which correlates with increased albuminuria and glomerular damage. miR-146a levels are also significantly reduced in the glomeruli of albuminuric BTBR ob/ob mice, indicating its significant role in maintaining podocyte health...
January 13, 2017: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/27884116/kidney-disease-in-the-elderly-biopsy-based-data-from-14-renal-centers-in-poland
#19
Agnieszka Perkowska-Ptasinska, Dominika Deborska-Materkowska, Artur Bartczak, Tomasz Stompor, Tomasz Liberek, Barbara Bullo-Piontecka, Anna Wasinska, Agnieszka Serwacka, Marian Klinger, Jolanta Chyl, Michal Kuriga, Robert Malecki, Krzysztof Marczewski, Bogdan Hryniewicz, Tadeusz Gregorczyk, Monika Wieliczko, Stanislaw Niemczyk, Olga Rostkowska, Leszek Paczek, Magdalena Durlik
BACKGROUND: Longer life expectancy is associated with an increasing prevalence of kidney disease. Aging itself may cause renal damage, but the spectrum of kidney disorders that affect elderly patients is diverse. Few studies, mostly form US, Asia and West Europe found differences in the prevalence of some types of kidney diseases between elderly and younger patients based on renal biopsy findings, with varied proportion between glomerulopathies and arterionephrosclerosis as a dominant injury found...
November 25, 2016: BMC Nephrology
https://www.readbyqxmd.com/read/27858997/a-novel-assay-to-assess-the-effect-of-pharmaceutical-compounds-on-the-differentiation-of-podocytes
#20
Frances Kindt, Elke Hammer, Stefan Kemnitz, Antje Blumenthal, Paul Klemm, Rabea Schlüter, Susan E Quaggin, Jens van den Brandt, Georg Fuellen, Uwe Völker, Karlhans Endlich, Nicole Endlich
BACKGROUND AND PURPOSE: Therapeutic options for treating glomerulopathies, the main cause of chronic kidney disease, are limited. Podocyte dedifferentiation is a major event in the pathogenesis of glomerulopathies. The goal of the present study was, therefore, to develop an assay to monitor podocyte differentiation suitable for compound screening. EXPERIMENTAL APPROACH: We isolated and cultured glomeruli from transgenic mice, expressing cyan fluorescent protein (CFP) under the control of the promoter of nephrin, a marker of podocyte differentiation...
January 2017: British Journal of Pharmacology
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