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Focal and segmental glomerulosclerosis

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https://www.readbyqxmd.com/read/29043143/focal-segmental-glomerulosclerosis-associated-with-mitochondrial-disease
#1
Kenneth Lim, David Steele, Andrew Fenves, Ravi Thadhani, Eliot Heher, Amel Karaa
Primary mitochondrial diseases (MD) are complex, heterogeneous inherited diseases caused by mutations in either the mitochondrial or nuclear DNA. Glomerular diseases in MD have been reported with tRNA mutation m.3243A>G causing a syndrome of mitochondrial encephalomyopathy, lactic acidosis and stroke-like episodes (MELAS). We describe here a case of focal segmental glomerulosclerosis (FSGS) associated with a new tRNA mutation site. A 34-year-old man with a history of living related kidney transplantation, diabetes, hearing loss, and developmental delay presented to the outpatient clinic with complaints of new behavioral difficulties, worsening symptoms, and brain involvement on imaging...
2017: Clin Nephrol Case Stud
https://www.readbyqxmd.com/read/29041955/pluripotent-nontumorigenic-multilineage-differentiating-stress-enduring-cells-muse-cells-a-seven-year-retrospective
#2
REVIEW
Samantha C Fisch, María L Gimeno, Julia D Phan, Ariel A Simerman, Daniel A Dumesic, Marcelo J Perone, Gregorio D Chazenbalk
Multilineage differentiating stress enduring (Muse) cells, discovered in the spring of 2010 at Tohoku University in Sendai, Japan, were quickly recognized by scientists as a possible source of pluripotent cells naturally present within mesenchymal tissues. Muse cells normally exist in a quiescent state, singularly activated by severe cellular stress in vitro and in vivo. Muse cells have the capacity for self-renewal while maintaining pluripotent cell characteristics indicated by the expression of pluripotent stem cell markers...
October 18, 2017: Stem Cell Research & Therapy
https://www.readbyqxmd.com/read/29039058/recurrent-and-de-novo-glomerulonephritis-following-renal-transplantation-higher-rates-of-rejection-and-lower-graft-survival
#3
Safak Mirioglu, Yasar Caliskan, Yagmur Goksoy, Sibel Gulcicek, Yasemin Ozluk, Irem Sarihan, Nurhan Seyahi, Isin Kilicaslan, Aydin Turkmen, Mehmet Sukru Sever
PURPOSE: In this retrospective study with case-control design, we aimed to determine the clinical and pathological characteristics of post-transplant glomerulonephritis (GN), and their effects on transplant recipients. METHODS: One hundred and twenty renal transplant recipients with biopsy-proven recurrent or de novo primary GN were compared with two matched control groups including 120 transplant recipients with nonrecurrent primary GN (nonrecurrent GN group) and 120 transplant recipients with non-GN etiology (non-GN group)...
October 16, 2017: International Urology and Nephrology
https://www.readbyqxmd.com/read/29038896/inducible-atf3-nfat-axis-aggravates-podocyte-injury
#4
Hong Zhang, Shun Liang, Yue Du, Ruizhao Li, Chaosheng He, Wenjian Wang, Shuangxin Liu, Zhiming Ye, Xinling Liang, Wei Shi, Bin Zhang
Podocyte injury and loss contribute to proteinuria, glomerulosclerosis, and eventually kidney failure. Activating transcription factor 3 (ATF3) is a stress inducible transcription factor that is transiently expressed following stimulation. However, we show for the first time an induction of ATF3 in podocytes from patients with chronic kidney disease, including minimal change disease, focal segmental glomerulosclerosis, and diabetic nephropathy. The role of ATF3 induction in podocytes under chronic conditions is currently unknown...
October 16, 2017: Journal of Molecular Medicine: Official Organ of the "Gesellschaft Deutscher Naturforscher und Ärzte"
https://www.readbyqxmd.com/read/29038887/mutations-in-inf2-may-be-associated-with-renal-histology-other-than-focal-segmental-glomerulosclerosis
#5
Anja K Büscher, Nora Celebi, Peter F Hoyer, Hanns-Georg Klein, Stefanie Weber, Julia Hoefele
BACKGROUND: In 2010, INF2 mutations were associated with autosomal-dominant focal segmental glomerulosclerosis (FSGS), clinically presenting with moderate proteinuria in adolescence. However, in the meantime, cases with more severe clinical courses have been described, including progression to end-stage renal disease (ESRD) during childhood. INF2 mutations in patients with isolated FSGS are clustered in exons 2 to 4, encoding the diaphanous inhibitory domain, involved in the regulation of the podocyte actin cytoskeleton...
October 6, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29035193/renal-outcome-in-iga-nephropathy-according-to-oxford-classification-and-ultrastructural-analysis-in-a-brazilian-center%C3%A2
#6
Giovana Mariani, Leandro L L Freitas, Ricardo L Zollner, Maria Almerinda V F Ribeiro Alves
AIMS: Correlate clinical and histologic features with renal outcome in patients with biopsy-proven IgA nephropathy (IgAN). MATERIALS AND METHODS: Retrospective analysis of records and renal tissue of IgAN patients. Histology was revised according to MEST score of Oxford classification. Focal segmental glomerulosclerosis (FSGS) features were assessed by light microscopy. Electron microscopy review searched for podocyte effacement. RESULTS: 67 patients were included, 56...
October 16, 2017: Clinical Nephrology
https://www.readbyqxmd.com/read/29027319/pregnancy-in-immunoglobulin-m-nephropathy
#7
Meryem Hocaoğlu, Sabahat Alışır Ecder, Abdulkadir Turgut, Ateş Karateke
Immunoglobulin nephropathy is an uncommon glomerular disease and a relatively less recognized clinico-immunopathological entity in the domain of glomerulonephritis, often thought to be a bridge between minimal change disease and focal segmental glomerulosclerosis. It is characterized by asymptomatic hematuria, episodes of macroscopic hematuria and proteinuria. Corticosteroids remain the mainstay of therapeutic strategies for such patients. We present what we believe to be the first known case of successful pregnancy in a 40-year-old woman with a 12-year history of underlying immunoglobulin nephropathy...
October 13, 2017: Journal of Obstetrics and Gynaecology Research
https://www.readbyqxmd.com/read/29020719/diagnosis-and-management-of-nephrotic-syndrome
#8
Oonagh McCloskey, Alexander P Maxwell
Nephrotic syndrome is defined by a triad of clinical features: oedema, substantial proteinuria (> 3.5 g/24 hours) and hypoalbuminaemia (< 30 g/L). It is often associated with hyperlipidaemia, thromboembolism and an increased risk of infection. Nephrotic syndrome develops following pathological injury to renal glomeruli. This may be a primary problem, with a disease specific to the kidneys, or secondary to a systemic disorder such as diabetes mellitus. The most common cause in children is minimal change glomerulonephritis...
February 2017: Practitioner
https://www.readbyqxmd.com/read/28992235/treatment-by-immunoadsorption-for-recurrent-focal-segmental-glomerulosclerosis-after-paediatric-kidney-transplantation-a-multicentre-french-cohort
#9
Lise Allard, Theresa Kwon, Saoussen Krid, Justine Bacchetta, Arnaud Garnier, Robert Novo, Georges Deschenes, Rémi Salomon, Gwenaëlle Roussey, Emma Allain-Launay
Background: Primary focal segmental glomerulosclerosis (FSGS) frequently recurs after kidney transplantation (KTx) in children. This can lead to delayed graft loss. As the management of children with recurrent FSGS is not well established, apheresis strategies could be a cornerstone to control the disease. Immunoadsorption (IA) is a recent apheresis therapy. There have been few studies examining IA in this setting. We report the results of IA for management of recurrent FSGS after KTx in children in France...
July 28, 2017: Nephrology, Dialysis, Transplantation
https://www.readbyqxmd.com/read/28982981/the-hippo-pathway-regulator-kibra-promotes-podocyte-injury-by-inhibiting-yap-signaling-and-disrupting-actin-cytoskeletal-dynamics
#10
Kristin Meliambro, Jenny S Wong, Justina Ray, Rhodora C Calizo, Sara Towne, Beatriz Cole, Fadi El Salem, Ronald E Gordon, Lewis Kaufman, John C He, Evren U Azeloglu, Kirk N Campbell
Kidney podocytes represent a key constituent of the glomerular filtration barrier. Identifying the molecular mechanisms of podocyte injury and survival is important for better understanding and management of kidney diseases. KIBRA (KIdney BRAin protein), an upstream regulator of the Hippo signaling pathway encoded by the Wwc1 gene, shares the pro-injury properties of its putative binding partner dendrin and antagonizes the pro-survival signaling of the downstream Hippo pathway effector YAP (Yes-associated protein) in Drosophila and MCF10A cells...
October 5, 2017: Journal of Biological Chemistry
https://www.readbyqxmd.com/read/28975106/reporting-renal-biopsies-from-cyprus-a-systematic-approach
#11
Düriye Deren Oygar, Guy H Neild
BACKGROUND: The etiology of renal disease varies in different parts of the world. In the Middle East, half of all patients reaching end-stage are categorised as either unknown etiology or hypertension-related nephropathy. OBJECTIVES: To report a renal biopsy series, in a reproducible format and manner, so that data can be compared directly among other series. PATIENTS AND METHODS: Biopsies of native kidneys were performed in a 10-year period, at a tertiary referral hospital that provides the entire nephrology service for north Cyprus...
July 2017: Journal of Nephropathology
https://www.readbyqxmd.com/read/28975100/impact-of-%C3%AE-h1-and-%C3%AE-h2-cytokines-in-the-progression-of-idiopathic-nephrotic-syndrome-due-to-focal-segmental-glomerulosclerosis-and-minimal-change-disease
#12
Maria Stangou, Μichael Spartalis, Dimitra-Vasilia Daikidou, Theodora Kouloukourgiotou, Erasmia Sampani, Ioanna-Theologia Lambropoulou, Afroditi Pantzaki, Αikaterini Papagianni, George Efstratiadis
BACKGROUND: Differential diagnosis between primary focal segmental glomerulosclerosis (FSGS) and minimal change disease (MCD) is sometimes difficult as nephrotic syndrome is the main clinical symptom in both diseases. OBJECTIVES: This study has attempted to evaluate the urinary excretion of Th1 and Th2 cytokines as potential biomarkers in distinguishing the two types of nephrotic syndrome, and predicting outcome of renal function. PATIENTS AND METHODS: Thirty-six patients with FSGS (M/F 22/14, Age; 41...
July 2017: Journal of Nephropathology
https://www.readbyqxmd.com/read/28966119/wt1-is-necessary-for-the-proliferation-and-migration-of-cells-of-renin-lineage-following-kidney-podocyte-depletion
#13
Natalya V Kaverina, Diana G Eng, Andrea D Largent, Ilse Daehn, Anthony Chang, Kenneth W Gross, Jeffrey W Pippin, Peter Hohenstein, Stuart J Shankland
Wilms' tumor suppressor 1 (WT1) plays an important role in cell proliferation and mesenchymal-epithelial balance in normal development and disease. Here, we show that following podocyte depletion in three experimental models, and in patients with focal segmental glomerulosclerosis (FSGS) and membranous nephropathy, WT1 increased significantly in cells of renin lineage (CoRL). In an animal model of FSGS in RenWt1(fl/fl) reporter mice with inducible deletion of WT1 in CoRL, CoRL proliferation and migration to the glomerulus was reduced, and glomerular disease was worse compared with wild-type mice...
October 10, 2017: Stem Cell Reports
https://www.readbyqxmd.com/read/28965116/new-anti-nephrin-antibody-mediated-podocyte-injury-model-using-a-c57bl-6-mouse-strain
#14
Kazuhiro Takeuchi, Shokichi Naito, Nagako Kawashima, Naoko Ishigaki, Takashi Sano, Kouju Kamata, Yasuo Takeuchi
BACKGROUND: Focal segmental glomerulosclerosis (FSGS) is considered a subset of the podocytopathies. The molecular pathogenesis of podocytopathy is still unknown. There has not been an experimental animal model of isolated podocytopathy induced by antibody in C57BL/6 strain, which is widely used as the genetic background. Nephrin is closely associated with the slit diaphragm of the glomerular podocyte, and has recently received attention as a potential therapeutic target. The function of nephrin, especially its role in FSGS development via podocytopathy, is being elucidated...
September 30, 2017: Nephron
https://www.readbyqxmd.com/read/28961608/nivolumab-associated-nephrotic-syndrome-in-a-patient-with-renal-cell-carcinoma-a-case-report
#15
Robin A Daanen, Rutger J H Maas, Rutger H T Koornstra, Eric J Steenbergen, Carla M L van Herpen, Annelieke E C A B Willemsen
INTRODUCTION: Immune checkpoint inhibitors have taken an important place in the treatment of different types of malignancies. These drugs are known to have specific immune-mediated adverse events. We describe a case of severe nephrotic syndrome secondary to treatment with nivolumab in a patient with renal cell carcinoma. CASE PRESENTATION: A 62-year-old man was treated with nivolumab for papillary renal cell carcinoma type 2 for 8 weeks when he was admitted to the hospital with a severe nephrotic syndrome and acute kidney injury...
November 2017: Journal of Immunotherapy
https://www.readbyqxmd.com/read/28958905/-uro-and-nephrotoxic-effects-of-drugs-of-abuse-literature-review-and-pharmaco-epidemiological-survey-in-france-and-in-the-marseille-area
#16
Marion Gully, Élisabeth Frauger, Michel Spadari, Liselotte Pochard, Vanessa Pauly, Fanny Romain, Bertrand Gondouin, Marion Sallée, Julie Moussi-Frances, Stéphane Burtey, Bertrand Dussol, Laurent Daniel, Joëlle Micallef, Noémie Jourde-Chiche
A great diversification of drugs of abuse has been observed in recent years, both in the populations using them and in the types of drugs. Although dependency and psychiatric disorders associated with the abuse of these substances is well known, somatic complications, uro-nephrotoxicity in particular, are less recognized. We propose here an overview of the products used by drugs abusers in France, through the analysis of the national pharmaco-epidemiological study Observation des produits psychotropes illicites ou détournés de leur utilisation médicamenteuse (OPPIDUM)...
September 25, 2017: Néphrologie & Thérapeutique
https://www.readbyqxmd.com/read/28951873/multiple-targets-for-novel-therapy-of-fsgs-associated-with-circulating-permeability-factor
#17
REVIEW
Virginia J Savin, Mukut Sharma, Jianping Zhou, David Genochi, Ram Sharma, Tarak Srivastava, Amna Ilahe, Pooja Budhiraja, Aditi Gupta, Ellen T McCarthy
A plasma component is responsible for altered glomerular permeability in patients with focal segmental glomerulosclerosis. Evidence includes recurrence after renal transplantation, remission after plasmapheresis, proteinuria in infants of affected mothers, transfer of proteinuria to experimental animals, and impaired glomerular permeability after exposure to patient plasma. Therapy may include decreasing synthesis of the injurious agent, removing or blocking its interaction with cells, or blocking signaling or enhancing cell defenses to restore the permeability barrier and prevent progression...
2017: BioMed Research International
https://www.readbyqxmd.com/read/28949388/microrna-135a-is-involved-in-podocyte-injury-in-a-transient-receptor-potential-channel-1-dependent-manner
#18
Xianggui Yang, Dongming Wu, Hongfei Du, Fang Nie, Xueli Pang, Ying Xu
Transient receptor potential (TRP) cation channels are essential for normal cellular physiology, and their abnormal expression may lead to a number of disorders, including podocytopathy. Therefore, it is crucial to understand the mechanisms underlying the regulation of TRP channels. In the present study, microRNA (miR)-135a was found to be upregulated in patients with focal segmental glomerulosclerosis and mice treated with adriamycin (ADR). In cultured podocytes, transforming growth factor (TGF)-β and ADR were found to promote miR‑135a expression...
September 25, 2017: International Journal of Molecular Medicine
https://www.readbyqxmd.com/read/28949280/childhood-nephrotic-syndrome-in-tropical-africa-then-and-now
#19
Wasiu A Olowu, Adebowale Ademola, Adebukola B Ajite, Yauba M Saad
This descriptive and comparative review examines the changing epidemiology, treatment, renal and patient outcome of childhood nephrotic syndrome (NS) in tropical Africa (TpAfr). In the 1960s to 1980s, corticosteroid-resistant non-minimal change disease (nMCD) including quartan malaria nephropathy (QMN) was the dominant renal histopathology type. The overall incidence of NS was 0.35-1.34% of hospital admissions. Median age at onset of NS ranged between 4.0 and 12.0 years while the mean (SD) age range was 5.8 (3...
September 26, 2017: Paediatrics and International Child Health
https://www.readbyqxmd.com/read/28948129/sudden-collapse-in-the-first-trimester-report-of-hyperacute-renal-failure-secondary-to-collapsing-glomerulopathy-as-the-initial-presentation-of-lupus
#20
Pooja Sethi, Jennifer Treece, Chidinma Onweni
Hyperacute renal failure is rarely the initial presentation of systemic lupus erythematosus (SLE). Pregnancy can predispose untreated lupus nephritis to acute renal failure. Collapsing glomerulopathy (CG) type of renal failure is not a new clinicopathological entity. There have been documented cases prior to 1979. It is thought that detection bias coupled with the predilection for HIV has caused this form of glomerulopathy to be incorrectly named or diagnosed as 'malignant focal segmental glomerulosclerosis (FSGS)'...
July 24, 2017: Curēus
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