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Focal and segmental glomerulosclerosis

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https://www.readbyqxmd.com/read/29457800/comment-to-focal-segmental-glomerulosclerosis-associated-with-mitochondrial-disease-by-lim-et-al-in-clin-nephrol-case-stud-2017-5-20-25
#1
COMMENT
https://www.readbyqxmd.com/read/29456224/alport-s-syndrome-with-focal-segmental-glomerulosclerosis-lesion-pattern-to-recognize
#2
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
#3
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/29415466/the-role-of-trio-a-rho-guanine-nucleotide-exchange-factor-in-glomerular-podocytes
#4
Mirela Maier, Cindy Baldwin, Lamine Aoudjit, Tomoko Takano
Nephrotic syndrome is a kidney disease featured by heavy proteinuria. It is caused by injury to the specialized epithelial cells called "podocytes" within the filtration unit of the kidney, glomerulus. Previous studies showed that hyperactivation of the RhoGTPase, Rac1, in podocytes causes podocyte injury and glomerulosclerosis (accumulation of extracellular matrix in the glomerulus). However, the mechanism by which Rac1 is activated during podocyte injury is unknown. Trio is a guanine nucleotide exchange factor (GEF) known to activate Rac1...
February 6, 2018: International Journal of Molecular Sciences
https://www.readbyqxmd.com/read/29398134/kidney-disease-in-the-setting-of-hiv-infection-conclusions-from-a-kidney-disease-improving%C3%A2-global-outcomes-kdigo-controversies%C3%A2-conference
#5
Charles R Swanepoel, Mohamed G Atta, Vivette D D'Agati, Michelle M Estrella, Agnes B Fogo, Saraladevi Naicker, Frank A Post, Nicola Wearne, Cheryl A Winkler, Michael Cheung, David C Wheeler, Wolfgang C Winkelmayer, Christina M Wyatt
HIV-positive individuals are at increased risk for kidney disease, including HIV-associated nephropathy, noncollapsing focal segmental glomerulosclerosis, immune-complex kidney disease, and comorbid kidney disease, as well as kidney injury resulting from prolonged exposure to antiretroviral therapy or from opportunistic infections. Clinical guidelines for kidney disease prevention and treatment in HIV-positive individuals are largely extrapolated from studies in the general population, and do not fully incorporate existing knowledge of the unique HIV-related pathways and genetic factors that contribute to the risk of kidney disease in this population...
February 2, 2018: Kidney International
https://www.readbyqxmd.com/read/29395351/end-stage-renal-disease-and-mortality-outcomes-across-different-glomerulonephropathies-in-a-large-diverse-us-population
#6
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/29394927/quantification-of-cancer-risk-in-glomerulonephritis
#7
James Goya Heaf, Alastair Hansen, Gunnar Hellmund Laier
BACKGROUND: The association of increased cancer risk with glomerulonephritis (GN) is well known, but controversy exists concerning which types of GN are involved, and the size of the association. A national registry survey was performed to assess the size of this association, and the temporal relationship of cancer diagnosis to GN diagnosis. METHODS: All patients with biopsy-proven GN between 1985 and 2015 in Denmark were extracted from The Danish Renal Biopsy Registry and the National Pathology Data Bank...
February 2, 2018: BMC Nephrology
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
#8
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/29380515/acute-hemodialysis-in-a-young-man-with-severe-symptomatic-hyponatremia-and-kidney-injury
#9
Catherine Courteau, Alex Al Khoury, Rene P Michel, Catherine L Weber
A 35-year-old man presented with severe hypo-osmolar hyponatremia (serum sodium 99 mmol/L), profound nonoliguric renal failure (serum creatinine 1240 μmol/L), and nephrotic range proteinuria. Computed tomography of the abdomen revealed nephromegaly and no obstruction. The patient was admitted to the intensive care unit (ICU) and conventional hemodialysis was initiated. To avoid rapid sodium correction, we prescribed concurrent dialysate flow, a low dialysate sodium concentration, a small surface area dialyzer, and a low blood flow rate...
January 30, 2018: Hemodialysis International
https://www.readbyqxmd.com/read/29378953/disease-causing-mutation-in-%C3%AE-actinin-4-promotes-podocyte-detachment-through-maladaptation-to-periodic-stretch
#10
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
#11
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/29361670/podocyte-specific-knockin-of-pten-protects-kidney-from-hyperglycemia
#12
Huizhen Wang, Ziwei Feng, Jianteng Xie, Feng Wen, Menglei Jv, Tiantian Liang, Jing Li, Yanhui Wang, Yangyang Zuo, Sheng Li, Ruizhao Li, Zhilian Li, Bin Zhang, Xinling Liang, Shuangxin Liu, Wei Shi, Wenjian Wang
Aim Phosphatase and tensin homolog deleted on chromosome 10 (PTEN) has been proved to be downregulated in podocytes challenged with high glucose (HG), and knockout of PTEN in podocytes aggravated the progression of diabetic kidney disease (DKD). However, whether podocyte-specific knockin of PTEN protects the kidney against hyperglycemia in vivo remains undefined. Methods The inducible podocyte-specific PTEN knockin (PPKI) mice were generated by crossing newly created transgenic loxP-stop-loxP-PTEN mice with podocin-iCreERT2 mice...
January 17, 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
#13
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
#14
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
#15
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
#16
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
#17
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
#18
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
#19
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
#20
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
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