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Focal and segmental glomerulosclerosis

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https://www.readbyqxmd.com/read/29238427/hiv-associated-nephropathy-in-africa-pathology-clinical-presentation-and-strategy-for-prevention
#1
REVIEW
Nazik Elmalaika Husain, Mohamed H Ahmed, Ahmed O Almobarak, Sufian K Noor, Wadie M Elmadhoun, Heitham Awadalla, Clare L Woodward, Dushyant Mital
The human immunodeficiency virus (HIV) infection can lead to progressive decline in renal function known as HIV-associated nephropathy (HIVAN). Importantly, individuals of African ancestry are more at risk of developing HIVAN than their European descent counterparts. An in-depth search on Google Scholar, Medline and PubMed was conducted using the terms "HIVAN" and "pathology and clinical presentation", in addition to "prevalence and risk factors for HIVAN", with special emphasis on African countries for any articles published between 1990 and 2017...
January 2018: Journal of Clinical Medicine Research
https://www.readbyqxmd.com/read/29234893/the-clinical-relevance-of-plasma-cd147-basigin-in-biopsy-proven-kidney-diseases
#2
Yoshiko Mori, Tomohiro Masuda, Tomoki Kosugi, Tomoki Yoshioka, Mayuko Hori, Hiroshi Nagaya, Kayaho Maeda, Yuka Sato, Hiroshi Kojima, Noritoshi Kato, Takuji Ishimoto, Takayuki Katsuno, Yukio Yuzawa, Kenji Kadomatsu, Shoichi Maruyama
BACKGROUND: Precise understanding of kidney disease activity is needed to design therapeutic strategies. CD147/basigin is involved in the pathogenesis of acute kidney injury and renal fibrosis through inflammatory cell infiltration. The present study examined the clinical relevance of CD147 in biopsy-proven kidney diseases that lead to the progression of chronic kidney disease. METHODS: Kidney biopsy specimens and plasma and urine samples were obtained from patients with kidney diseases, including IgA nephropathy (IgAN), Henoch-Schönlein purpura nephritis (HSPN), diabetic kidney disease (DKD), focal segmental glomerulosclerosis (FSGS), and membranous nephropathy (MN), who underwent renal biopsy between 2011 and 2014...
December 12, 2017: Clinical and Experimental Nephrology
https://www.readbyqxmd.com/read/29227285/shifts-in-podocyte-histone-h3k27me3-regulate-mouse-and-human-glomerular-disease
#3
Syamantak Majumder, Karina Thieme, Sri N Batchu, Tamadher A Alghamdi, Bridgit B Bowskill, M Golam Kabir, Youan Liu, Suzanne L Advani, Kathryn E White, Laurette Geldenhuys, Karthik K Tennankore, Penelope Poyah, Ferhan S Siddiqi, Andrew Advani
Histone protein modifications control fate determination during normal development and dedifferentiation during disease. Here, we set out to determine the extent to which dynamic changes to histones affect the differentiated phenotype of ordinarily quiescent adult glomerular podocytes. To do this, we examined the consequences of shifting the balance of the repressive histone H3 lysine 27 trimethylation (H3K27me3) mark in podocytes. Adriamycin nephrotoxicity and subtotal nephrectomy (SNx) studies indicated that deletion of the histone methylating enzyme EZH2 from podocytes decreased H3K27me3 levels and sensitized mice to glomerular disease...
December 11, 2017: Journal of Clinical Investigation
https://www.readbyqxmd.com/read/29225906/patient-engagement-in-kidney-research-opportunities-and-challenges-ahead
#4
REVIEW
Amber O Molnar, Moumita Barua, Ana Konvalinka, Kara Schick-Makaroff
Purpose of Review: Patient engagement in research is increasingly recognized as an important component of the research process and may facilitate translation of research findings. To heighten awareness on this important topic, this review presents opportunities and challenges of patient engagement in research, drawing on specific examples from 4 areas of Canadian kidney research conducted by New Investigators in the Kidney Research Scientist Core Education and National Training (KRESCENT) Program...
2017: Canadian Journal of Kidney Health and Disease
https://www.readbyqxmd.com/read/29225801/histologic-regression-of-fibrillary-glomerulonephritis-the-first-report-of-biopsy-proven-spontaneous-resolution-of-disease
#5
Miroslav Sekulic, Samih H Nasr, Joseph P Grande, Lynn D Cornell
Fibrillary glomerulonephritis (FGN) is a rare immune complex type glomerulonephritis characterized by glomerular deposition of randomly oriented fibrils measuring 10-30 nm in thickness, and typically presents with proteinuria with or without renal insufficiency and hematuria. We present a case in which a patient initially presented at age 41 years with nephrotic-range proteinuria and hypertension; a kidney biopsy showed FGN. The patient was treated with angiotensin receptor blockage only, without immunosuppression as per patient preference, and the level of protein in the urine improved...
December 2017: Clinical Kidney Journal
https://www.readbyqxmd.com/read/29217578/a-small-molecule-inhibitor-of-trpc5-ion-channels-suppresses-progressive-kidney-disease-in-animal-models
#6
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
#7
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
#8
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/29206334/non-diabetic-renal-diseases-in-patients-with-type-2-diabetes-a-single-center-study
#9
Jian-Zhen Fan, Rong Wang
OBJECTIVE: To analyse the pathological characteristics and distribution of renal injury in selected type 2 diabetic patients. Comparison between diabetic nephropathy (DN) and non-diabetic renal disease (NDRD) in clinical characteristics, to find important predictors for NDRD. METHODS: To conduct retrospective analysis of clinical, laboratory and pathohistological data of type 2 diabetic patients in whom renal biopsies were performed from March 2010 to September 2014 in Shandong Provincial Hospital affiliated to Shandong University (n = 88)...
December 5, 2017: Internal Medicine Journal
https://www.readbyqxmd.com/read/29198665/isolated-crystalloid-podocytopathy-with-focal-segmental-glomerulosclerosis-in-renal-allograft-an-unusual-presentation-of-post-transplant-monoclonal-gammopathy-of-renal-significance-a-case-report
#10
A-H Yang, C-C Loong, N-C Lin, C-Y Yang
BACKGROUND: Monoclonal gammopathy of renal significance denotes a spectrum of hematologic disorders that cause direct or indirect renal damage. CASE PRESENTATION: A 51-year-old man had received a living-donor kidney transplant from his wife in 2008. He had gradual increased proteinuria 4 years later. His renal biopsy results revealed cytoplasmic crystalloid inclusions in the podocytes. No crystalloid inclusion was found in other renal cells. Despite that immunofluorescent examination failed to show light-chain deposition, the serum immuno-electrophoresis revealed monoclonal immunoglobulin-Gκ...
December 2017: Transplantation Proceedings
https://www.readbyqxmd.com/read/29198656/long-term-outcomes-of-kidney-transplant-recipients-with-primary-idiopathic-focal-segmental-glomerulosclerosis
#11
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/29193639/oligonephronia-and-wolf-hirschhorn-syndrome-a-further-observation
#12
Antonio Gatto, Pietro Ferrara, Chiara Leoni, Roberta Onesimo, Marcella Zollino, Francesco Emma, Giuseppe Zampino
Wolf-Hirschhorn syndrome (WHS) is a rare chromosomal disorder caused by a partial deletion of chromosome 4 (4p16.3p16.2). We describe a case of a male 9 years old children with WHS proteinuria and hypertension. Laboratory data showed creatinine 1.05 mg/dl, GFR 65.9 ml/min/1.73 m2 , cholesterol 280 mg/dl, triglyceride 125 mg/dl with electrolytes in the normal range. Urine collection showed protein 2.72 g/L with a urine protein/creatinine ratio (UP /UCr ratio) of 4.2 and diuresis of 1,100 ml. Renal ultrasound showed reduced kidney dimensions with diffusely hyperechogenic cortex and poorly visualized pyramids...
November 28, 2017: American Journal of Medical Genetics. Part A
https://www.readbyqxmd.com/read/29191574/-collapsing-focal-segmental-glomerulosclerosis-induced-by-cytomegalovirus-a%C3%A2-case-report
#13
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
#14
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
#15
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
#16
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
#17
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/29179962/the-spectrum-of-glomerular-disease-between-the-years-2003-and-2015-in-columbia-a-review-of-12-613-cases
#18
Luis Eduardo Barrera-Herrera, Rocío Del Pilar López Panqueva, Adriana Alejandra Flórez Vargas, Rafael Enrique Andrade Pérez
BACKGROUND: The prevalence of glomerular disease (GD) varies according to the different socio-demographic characteristics of each population. For the first time we present the prevalence of the different forms of GD among patients from several different areas of Columbia. METHODS: Data from 12,613 renal biopsies studied at our University Hospital between 2003 and 2015 was reviewed. Pathology results were classified according to a list of renal diseases proposed by various authors...
January 2017: Revista Española de Patología
https://www.readbyqxmd.com/read/29167190/an-outcomes-based-definition-of-proteinuria-remission-in-focal-segmental-glomerulosclerosis
#19
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
#20
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
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