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Focal segmental glomerulosclerosis fsgs

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https://www.readbyqxmd.com/read/29456224/alport-s-syndrome-with-focal-segmental-glomerulosclerosis-lesion-pattern-to-recognize
#1
Afnan A Alsahli, Sara I Alshahwan, Amal O Alotaibi, Khaled O Alsaad, Nourah Aloudah, Mahfooz Farooqui, Abdullah A Al Sayyari
The association between Alport's syndrome (AS) and focal segmental glomerulosclerosis (FSGS) in the same patient is complex and rarely reported. We report a case of a 42-year-old male presenting with proteinuria, microscopic hematuria, elevated serum creatinine and hypertension with unremarkable physical examination apart from obesity. The renal biopsy showed well-established FSGS pattern of injury with mild interstitial fibrosis and tubular atrophy, while the electron microscopic examination demonstrated glomerular basement membranes (GBM) changes compatible with AS...
January 2018: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/29456219/descriptive-analysis-of-glomerulonephritis-by-histological-type-and-their-progression-among-adults-in-a-tertiary-care-center-in-sri-lanka
#2
Kawmadi W Gunawardena, Eranga S Wijewickrama, Carukshi Arambepola, Rushika D Lanerolle
Prevalence of different glomerulonephritides and their clinical course vary geographically. Our objectives are to assess the prevalence of different histological types of glomerulonephritis (GN) based on the light microscopic histology and to assess their progression according to histological type. A retrospective cross-sectional study was carried out among adult patients (>18 years) with a histological diagnosis of GN at the University Professorial Unit over a period of six months. Information including demographic data, renal biopsy findings, and progression of the disease through serum creatinine (SCr) level were collected through existing clinic records of consenting patients...
January 2018: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/29395351/end-stage-renal-disease-and-mortality-outcomes-across-different-glomerulonephropathies-in-a-large-diverse-us-population
#3
John J Sim, Simran K Bhandari, Michael Batech, Aviv Hever, Teresa N Harrison, Yu-Hsiang Shu, Dean A Kujubu, Tracy Y Jonelis, Michael H Kanter, Steven J Jacobsen
OBJECTIVE: To compare renal function decline, incident end-stage renal disease (ESRD), and mortality among patients with 5 common glomerular diseases in a large diverse population. PATIENTS AND METHODS: A retrospective cohort study (between January 1, 2000, and December 31, 2011) of patients with glomerulonephropathy using the electronic health record of an integrated health system was performed. Estimated glomerular filtration rate (eGFR) change, incident ESRD, and mortality were compared among patients with biopsy-proven focal segmental glomerulosclerosis (FSGS), membranous glomerulonephritis (MN), minimal change disease (MCD), immunoglobulin A nephropathy (IgAN), and lupus nephritis (LN)...
January 9, 2018: Mayo Clinic Proceedings
https://www.readbyqxmd.com/read/29382012/coenzyme-q10-supplementation-therapy-for-2-children-with-proteinuria-renal-disease-and-adck4-mutation-case-reports-and-literature-review
#4
Chunyue Feng, Qiong Wang, Jingjing Wang, Fei Liu, Huijun Shen, Haidong Fu, Jianhua Mao
RATIONALE: Mitochondrial nephropathy has a poor prognosis and often progresses to the end-stage renal disease. Renal pathology often is focal segmental glomerulosclerosis (FSGS) and does not respond to steroid therapy or immunosuppressive therapy. Some patients are benefited from the therapy of coenzyme Q10, which affect the synthesis pathway of coenzyme Q10. PATIENT CONCERNS: Herein, we report 2 cases of children with proteinuria renal disease with ADCK4 mutation...
November 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29378953/disease-causing-mutation-in-%C3%AE-actinin-4-promotes-podocyte-detachment-through-maladaptation-to-periodic-stretch
#5
Di Feng, Jacob Notbohm, Ava Benjamin, Shijie He, Minxian Wang, Lay-Hong Ang, Minaspi Bantawa, Mehdi Bouzid, Emanuela Del Gado, Ramaswamy Krishnan, Martin R Pollak
α-Actinin-4 (ACTN4) bundles and cross-links actin filaments to confer mechanical resilience to the reconstituted actin network. How this resilience is built and dynamically regulated in the podocyte, and the cause of its failure in ACTN4 mutation-associated focal segmental glomerulosclerosis (FSGS), remains poorly defined. Using primary podocytes isolated from wild-type (WT) and FSGS-causing point mutant Actn4 knockin mice, we report responses to periodic stretch. While WT cells largely maintained their F-actin cytoskeleton and contraction, mutant cells developed extensive and irrecoverable reductions in these same properties...
January 29, 2018: Proceedings of the National Academy of Sciences of the United States of America
https://www.readbyqxmd.com/read/29363327/the-mechanical-challenges-and-cytoskeletal-impairments-in-focal-segmental-glomerulosclerosis
#6
Di Feng, Clark DuMontier, Martin R Pollak
Focal segmental glomerulosclerosis (FSGS) is a histologically defined form of kidney injury typically mediated by podocyte dysfunction. Podocytes rely on their intricate actin-based cytoskeleton to maintain the glomerular filtration barrier in the face of mechanical challenges resulting from pulsatile blood flow and filtration of this blood flow. This review summarizes the mechanical challenges faced by podocytes in the form of stretch and shear stress, both of which may play a role in the progression of podocyte dysfunction and detachment...
January 24, 2018: American Journal of Physiology. Renal Physiology
https://www.readbyqxmd.com/read/29350347/low-birth-weight-is-a-conditioning-factor-for-podocyte-alteration-and-steroid-dependance-in-children-with-nephrotic-syndrome
#7
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/29337702/changing-concepts-of-hiv-infection-and-renal-disease
#8
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/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/29319763/current-distribution-pattern-of-biopsy-proven-glomerular-disease-in-salvador-brazil-40-years-after-an-initial-assessment
#10
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/29277509/mortality-and-allograft-loss-trends-among-us-pediatric-kidney-transplant-recipients-with-and-without-focal-segmental-glomerulosclerosis
#11
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/29259860/steroid-resistant-nephrotic-syndrome-caused-by-co-inheritance-of-mutations-at-nphs1-and-adck4-genes-in-two-chinese-siblings
#12
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
#13
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
#14
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/29234893/the-clinical-relevance-of-plasma-cd147-basigin-in-biopsy-proven-kidney-diseases
#15
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/29225906/patient-engagement-in-kidney-research-opportunities-and-challenges-ahead
#16
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/29217578/a-small-molecule-inhibitor-of-trpc5-ion-channels-suppresses-progressive-kidney-disease-in-animal-models
#17
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
#18
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
#19
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/29198656/long-term-outcomes-of-kidney-transplant-recipients-with-primary-idiopathic-focal-segmental-glomerulosclerosis
#20
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
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