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Focal segmental glomerulosclerosis fsgs

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https://www.readbyqxmd.com/read/27900977/unconventional-strategies-in-the-battle-of-focal-and-segmental-glomerulosclerosis
#1
Satish Mendonca, R S V Kumar, Devika Gupta, Pooja Gupta, Satish Barki, M L Sharma
A 24-year-old male presented with classic features of the nephrotic syndrome. An initial renal biopsy revealed minimal change disease and thereafter, a second biopsy showed features of focal and segmental glomerulosclerosis. There was no response to conventional immunosuppression, and the patient had to be given rituximab; in spite of this, he went on to develop end-stage renal disease. He continued to have heavy proteinuria leading to severe hypoalbuminemia, thrombosis, infections, and malnutrition, placing the patient in a life-threatening situation...
November 2016: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/27900314/renal-and-cardiovascular-morbidities-associated-with-apol1-status-among-african-american-and-non-african-american-children-with-focal-segmental-glomerulosclerosis
#2
Robert P Woroniecki, Derek K Ng, Sophie Limou, Cheryl A Winkler, Kimberly J Reidy, Mark Mitsnefes, Matthew G Sampson, Craig S Wong, Bradley A Warady, Susan L Furth, Jeffrey B Kopp, Frederick J Kaskel
BACKGROUND AND OBJECTIVES: African-American (AA) children with focal segmental glomerulosclerosis (FSGS) have later onset disease that progresses more rapidly than in non-AA children. It is unclear how APOL1 genotypes contribute to kidney disease risk, progression, and cardiovascular morbidity in children. DESIGN SETTING PARTICIPANTS AND MEASUREMENTS: We examined the prevalence of APOL1 genotypes and associated cardiovascular phenotypes among children with FSGS in the Chronic Kidney Disease in Children (CKiD) study; an ongoing multicenter prospective cohort study of children aged 1-16 years with mild to moderate kidney disease...
2016: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/27884116/kidney-disease-in-the-elderly-biopsy-based-data-from-14-renal-centers-in-poland
#3
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/27847659/interferon-induced-focal-segmental-glomerulosclerosis
#4
Yusuf Kayar, Nuket Bayram Kayar, Nadir Alpay, Jamshid Hamdard, Iskender Ekinci, Sebnem Emegil, Rabia Bag Soydas, Birol Baysal
Behçet's disease is an inflammatory disease of unknown etiology which involves recurring oral and genital aphthous ulcers and ocular lesions as well as articular, vascular, and nervous system involvement. Focal segmental glomerulosclerosis (FSGS) is usually seen in viral infections, immune deficiency syndrome, sickle cell anemia, and hyperfiltration and secondary to interferon therapy. Here, we present a case of FSGS identified with kidney biopsy in a patient who had been diagnosed with Behçet's disease and received interferon-alpha treatment for uveitis and presented with acute renal failure and nephrotic syndrome associated with interferon...
2016: Case Reports in Nephrology
https://www.readbyqxmd.com/read/27816064/increased-wnt-and-notch-signaling-a-clue-to-the-renal-disease-in-schimke-immuno-osseous-dysplasia
#5
Marie Morimoto, Clara Myung, Kimberly Beirnes, Kunho Choi, Yumi Asakura, Arend Bokenkamp, Dominique Bonneau, Milena Brugnara, Joel Charrow, Estelle Colin, Amira Davis, Georges Deschenes, Mattia Gentile, Mario Giordano, Andrew K Gormley, Rajeshree Govender, Mark Joseph, Kory Keller, Evelyne Lerut, Elena Levtchenko, Laura Massella, Christy Mayfield, Behzad Najafian, David Parham, Jurgen Spranger, Peter Stenzel, Uluc Yis, Zhongxin Yu, Jonathan Zonana, Glenda Hendson, Cornelius F Boerkoel
BACKGROUND: Schimke immuno-osseous dysplasia (SIOD) is a multisystemic disorder caused by biallelic mutations in the SWI/SNF-related matrix-associated actin-dependent regulator of chromatin, subfamily A-like 1 (SMARCAL1) gene. Changes in gene expression underlie the arteriosclerosis and T-cell immunodeficiency of SIOD; therefore, we hypothesized that SMARCAL1 deficiency causes the focal segmental glomerulosclerosis (FSGS) of SIOD by altering renal gene expression. We tested this hypothesis by gene expression analysis of an SIOD patient kidney and verified these findings through immunofluorescent analysis in additional SIOD patients and a genetic interaction analysis in Drosophila...
November 5, 2016: Orphanet Journal of Rare Diseases
https://www.readbyqxmd.com/read/27801486/focal-segmental-glomerulosclerosis-in-children
#6
Manel Jellouli, Kamel Abidi, Mouna Askri, Meriem Ferjani, Ouns Naija, Yousra Hammi, Rym Goucha, Tahar Gargah
Background Focal segmental glomerulosclerosis (FSGS) represents 20% of nephrotic syndrome in children. The clinical course and prognosis is heterogeneous in children. The aim of this study was to analyze treatment and outcome of children with FSGS. Methods This retrospective study was conducted in the Department of Pediatrics in Charles Nicolle Hospital during a 15-year period (1996-2010). Results There were 30 children, 16 boys and 14 girls. The mean age was 7 ± 4 years. Nephrotic syndrome was observed in 26 patients, hematuria was noticed in 2 patients and renal insufficiency was detected in 2 patients at presentation...
May 2016: La Tunisie Médicale
https://www.readbyqxmd.com/read/27797890/long-term-outcome-of-kidney-transplantation-in-recipients-with-focal-segmental-glomerulosclerosis
#7
Anna Francis, Peter Trnka, Steven J McTaggart
BACKGROUND AND OBJECTIVES: FSGS can recur after kidney transplantation and is associated with poor graft outcomes. We aimed to assess the incidence of FSGS recurrence post-transplant and determine the effect of graft source on recurrence and graft survival in patients with biopsy-proven FSGS. DESIGN, SETTING, PARTICIPANTS, & MEASUREMENTS: Using the Australian and New Zealand Dialysis and Transplant Registry, we assessed incidence of FSGS, the influence of donor type on the risk of FSGS recurrence, and graft loss in recipients with ESRD caused by primary FSGS using Kaplan-Meier and logistic regression analyses...
November 7, 2016: Clinical Journal of the American Society of Nephrology: CJASN
https://www.readbyqxmd.com/read/27795629/clinicopathological-correlation-and-treatment-response-of-primary-focal-segmental-glomerulosclerosis-in-adults-and-adolescents
#8
J Dhanapriya, T Dineshkumar, N Gopalakrishnan, R Sakthirajan, T Balasubramaniyan
The incidence of focal segmental glomerulosclerosis (FSGS) is approximately 10% in children <6 years, 20% in adolescents, and 20-25% in adults. A retrospective observational study was done to document clinicopathological correlation, treatment response, and risk factors in the progression of chronic kidney disease (CKD) of primary FSGS in adults and adolescents. A total of 170 patients were studied with a mean follow-up of 4.32 ± 1.2 years. FSGS not otherwise specified was the most common subtype (56%) followed by tip variant (24%)...
September 2016: Indian Journal of Nephrology
https://www.readbyqxmd.com/read/27752010/a-clinicopathologic-study-of-glomerular-disease-a-single-center-five-year-retrospective-study-from-northwest-india
#9
Pankaj Beniwal, Lalit Pursnani, Sanjeev Sharma, R K Garsa, Mohit Mathur, Prasad Dharmendra, Vinay Malhotra, Dhanajai Agarwal
Studies published from centers across India have reported different and contradicting patterns of glomerular disease. In this retrospective study, we report our experience from a Tertiary Care Center in Northwest India. A total of 702 renal biopsies performed between 2008 and 2013 were reviewed of which 80 were excluded from the study because of having insufficient records or if the biopsies were taken from an allograft. The study included 411 males (66.1 %) and 211 females (33.9%) with an age range of 12-70 years (mean 30...
September 2016: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/27752004/cyclosporine-therapy-in-steroid-dependent-or-steroid-resistant-idiopathic-focal-and-segmental-glomerulosclerosis
#10
Imen Gorsane, I Helal, I Yacoub, F Ben Hamida, E Abderrahim, T Ben Abdallah
Focal and segmental glomerulosclerosis (FSGS) is a heterogeneous entity. Previous few studies have evaluated the efficacy of calcineurin inhibitors in primary FSGS and have suggested positive benefit. In this single-center, retrospective study (1975-2014), we report our experience in Tunisian adults with primary FSGS treated with cyclosporine A (CsA). It includes patients histologically proven FSGS and managed in the Charles Nicolle Hospital at Tunis, Tunisia. The dose of CsA was adjusted to maintain a whole blood trough level of 80-150 ng/mL...
September 2016: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/27748392/minimal-change-disease-and-idiopathic-fsgs-manifestations-of-the-same-disease
#11
REVIEW
Rutger J Maas, Jeroen K Deegens, Bart Smeets, Marcus J Moeller, Jack F Wetzels
Minimal change disease (MCD) and focal segmental glomerulosclerosis (FSGS) are the key histological findings in patients with idiopathic nephrotic syndrome (INS). Although MCD and idiopathic FSGS are often considered to represent separate entities based on differences in their presenting characteristics, histology and outcomes, little evidence exists for this separation. We propose that MCD and idiopathic FSGS are different manifestations of the same progressive disease. The gradual development of FSGS in patients with non-remitting or relapsing INS has been well documented...
December 2016: Nature Reviews. Nephrology
https://www.readbyqxmd.com/read/27744657/pathogenesis-of-focal-segmental-glomerulosclerosis
#12
REVIEW
Beom Jin Lim, Jae Won Yang, Woo Sung Do, Agnes B Fogo
Focal segmental glomerulosclerosis (FSGS) is characterized by focal and segmental obliteration of glomerular capillary tufts with increased matrix. FSGS is classified as collapsing, tip, cellular, perihilar and not otherwise specified variants according to the location and character of the sclerotic lesion. Primary or idiopathic FSGS is considered to be related to podocyte injury, and the pathogenesis of podocyte injury has been actively investigated. Several circulating factors affecting podocyte permeability barrier have been proposed, but not proven to cause FSGS...
November 2016: Journal of Pathology and Translational Medicine
https://www.readbyqxmd.com/read/27725544/umbilical-cord-blood-transplantation-associated-nephrotic-syndrome-successfully-treated-by-low-density-lipoprotein-apheresis
#13
Yuka Sugawara, Kenjiro Honda, Daisuke Katagiri, Motonobu Nakamura, Takahisa Kawakami, Ryo Nasu, Akimasa Hayashi, Yukako Shintani, Akihiro Tojo, Eisei Noiri, Mineo Kurokawa, Masashi Fukayama, Masaomi Nangaku
The development of nephrotic syndrome (NS) after umbilical cord transplantation (UBT) has been reported in only four cases to date. We herein report the case of a 50-year-old woman who developed NS 94 days after UBT. She fell into oliguria and required dialysis. A kidney biopsy revealed focal and segmental glomerulosclerosis. Although glucocorticoid monotherapy did not improve her condition, the addition of low-density lipoprotein (LDL) apheresis resulted in remission of NS, a drastic improvement in her renal function, and withdrawal from dialysis...
2016: Internal Medicine
https://www.readbyqxmd.com/read/27701157/truncating-wilms-tumor-suppressor-gene-1-mutation-in-an-xx-female-with-adult-onset-focal-segmental-glomerulosclerosis-and-streak-ovaries-a-case-report
#14
Julia Hoefele, Markus J Kemper, Ulf Schoenermarck, Susanna Mueller, Hanns-Georg Klein, Anja Lemke
About 30% of children with nephrotic syndrome (NS) have inherited forms. Among them, mutations in Wilms tumor suppressor gene 1 (WT1) are a well characterized cause associated with steroid-resistant NS, Wilms tumor, and urogenital malformation in males. However, the role of WT1 mutations in adult-onset focal segmental glomerulosclerosis (FSGS) is unclear. We report the case of a 38-year-old female with FSGS. She had been diagnosed with streak ovaries during diagnostic workup for infertility. Mutational analysis identified the heterozygous mutation c...
October 5, 2016: Nephron
https://www.readbyqxmd.com/read/27651302/primary-focal-segmental-glomerulosclerosis-mirnas-and-targeted-therapies
#15
REVIEW
Johannes Leierer, Gert Mayer, Andreas Kronbichler
BACKGROUND: Primary focal segmental glomerulosclerosis (FSGS) is a common cause of nephrotic syndrome. AIMS: The pathogenic steps leading to primary FSGS are still obscure, although evidence suggests that circulatory factor(s) are involved in the onset of disease. RESULTS: Recent technical advances allow the analysis of miRNA expression in tissues and body fluids, leading to reports of miRNAs involved in the molecular mechanisms of FSGS-aetiopathogenesis...
November 2016: European Journal of Clinical Investigation
https://www.readbyqxmd.com/read/27650730/collapsing-glomerulopathy-is-common-in-the%C3%A2-setting-of-thrombotic-microangiopathy-of-the%C3%A2-native-kidney
#16
David Buob, Mélanie Decambron, Viviane Gnemmi, Marie Frimat, Maxime Hoffmann, Raymond Azar, Jean-Dominique Gheerbrant, Thomas Guincestre, Christian Noël, Marie-Christine Copin, François Glowacki
Thrombotic microangiopathy (TMA) is a poorly recognized cause of collapsing glomerulopathy. The frequency and significance of collapsing glomerulopathy associated with renal TMA have not been specifically studied in native kidney biopsy specimens. Here we retrospectively documented clinicopathologic features of 53 patients with histologically proven TMA in the native kidney, with special emphasis on changes due to focal segmental glomerulosclerosis (FSGS). Histological TMA was related to hypertensive nephropathy in 21 patients, genetic complement abnormalities in 9, drugs in 7, and to other causes in 16 patients...
September 17, 2016: Kidney International
https://www.readbyqxmd.com/read/27621641/rituximab-use-in-adult-primary-glomerulopathy-where-is-the-evidence
#17
REVIEW
Samir G Mallat, Houssam S Itani, Rana M Abou-Mrad, Rima Abou Arkoub, Bassem Y Tanios
Rituximab is a chimeric anti-CD20 antibody that results in depletion of B-cell lymphocytes. It is currently used in the treatment of a variety of autoimmune diseases, in addition to CD20-positive lymphomas. The use of rituximab in the treatment of the adult primary glomerular diseases has emerged recently, although not yet established as first-line therapy in international guidelines. In patients with steroid-dependent minimal change disease or frequently relapsing disease, and in patients with idiopathic membranous nephropathy (IMN), several retrospective and prospective studies support the use of rituximab to induce remission, whereas in idiopathic focal and segmental glomerulosclerosis (FSGS), the use of rituximab has resulted in variable results...
2016: Therapeutics and Clinical Risk Management
https://www.readbyqxmd.com/read/27610422/apol1-risk-alleles-are-associated-with-more-severe-arteriosclerosis-in-renal-resistance-vessels-with-aging-and-hypertension
#18
Michael D Hughson, Wendy E Hoy, Susan A Mott, Victor G Puelles, John F Bertram, Cheryl L Winkler, Jeffrey B Kopp
The increased risk of end-stage kidney disease (ESKD) among hypertensive African Americans is partly related to APOL1 allele variants. Hypertension-associated arterionephrosclerosis consists of arteriosclerosis, glomerulosclerosis, and cortical fibrosis. The initial glomerulosclerosis, attributed to preglomerular arteriosclerosis and ischemia, consists of focal global glomerulosclerosis (FGGS), but in biopsy studies, focal segmental glomerulosclerosis (FSGS) is found with progression to ESKD, particularly in African Americans...
May 2016: KI Reports
https://www.readbyqxmd.com/read/27600725/collapsing-glomerulopathy-in-a-young-woman-with-apol1-risk-alleles-following-acute-parvovirus-b19-infection-a-case-report-investigation
#19
Whitney Besse, Sherry Mansour, Karan Jatwani, Cynthia C Nast, Ursula C Brewster
BACKGROUND: Collapsing Glomerulopathy (CG), also known as the collapsing variant of Focal Segmental Glomerulosclerosis (FSGS), is distinct in both its clinical severity and its pathophysiologic characteristics from other forms of FSGS. This lesion occurs disproportionally in patients carrying two APOL1 risk alleles, and is the classic histologic lesion resulting from Human Immunodeficiency Virus (HIV) infection of podocytes. Other viral infections, including parvovirus B19, and drugs such as interferon that perturb the immune system, have also been associated with CG...
September 6, 2016: BMC Nephrology
https://www.readbyqxmd.com/read/27588375/apol1-associated-end-stage-renal-disease-in-a-living-kidney-transplant-donor
#20
N A Zwang, A Shetty, N Sustento-Reodica, E J Gordon, J Leventhal, L Gallon, J J Friedewald
Homozygosity for apolipoprotein-L1 (APOL1) risk variants has emerged as an important predictor of renal disease in individuals of African descent over the past several years. Additionally, these risk variants may be important predictors of renal allograft failure when present in a living or deceased donor. Currently, there is no universal recommendation for screening of potential donors. We present a case of end-stage renal disease with focal segmental glomerulosclerosis in a living donor 7 years following donor nephrectomy...
October 6, 2016: American Journal of Transplantation
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