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Focal segmental glomerulosclerosis fsgs

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
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
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...
October 17, 2016: Nature Reviews. Nephrology
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...
October 16, 2016: Journal of Pathology and Translational Medicine
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
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
Johannes Leierer, Gert Mayer, Andreas Kronbichler
Primary focal and segmental glomerulosclerosis (FSGS) is a common cause of nephrotic syndrome. The pathogenic steps leading to primary FSGS are still obscure, although evidence suggests that circulatory factor(s) are involved in onset of disease. 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. Moreover, investigations have also highlighted miRNAs that might serve as biomarkers for primary FSGS...
September 21, 2016: European Journal of Clinical Investigation
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
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
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 Rep
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...
2016: BMC Nephrology
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
Yu Wang, Chunxia Zheng, Feng Xu, Zhihong Liu
Primary focal segmental glomerulosclerosis (FSGS) and minimal change disease (MCD) are common types of primary glomerular disease; they share numerous clinical and pathological similarities but have different treatment regimens and prognoses. It is therefore necessary to distinguish between them and to explore the mechanism underlying their differences. Fibrinogen is reportedly involved in podocyte damage and in renal fibrosis in vitro and in animal models of kidney disease. We thus tested urinary fibrinogen, serum fibrinogen, and renal fibrinogen deposition levels in a cohort comprising 50 patients with FSGS and 40 patients with MCD...
September 23, 2016: Biochemical and Biophysical Research Communications
Vlad Florin Iovănescu, Adriana Florentina Constantinescu, Costin Teodor Streba, Sorin Ioan Zaharie, Cristin Constantin Vere, Eugen Mandache, Mircea Niculae Penescu, Eugen MoŢa
Chronic viral hepatitis B and C may associate different extrahepatic manifestations and renal disease is the most frequent. Kidney damage is represented in most cases by glomerulopathies, which include membranous nephropathy, membranoproliferative glomerulonephritis (MPGN), IgA nephropathy, focal and segmental glomerulosclerosis and diabetic nephropathy. We conducted a retrospective study on 639 patients diagnosed with chronic viral hepatitis B and C and different renal diseases. Complete evaluation of liver and renal status was performed and, in selected cases, renal biopsy...
2016: Romanian Journal of Morphology and Embryology, Revue Roumaine de Morphologie et Embryologie
Theophilus I Umeizudike, Jacob O Awobusuyi, Christiana O Amira, Taslim B Bello, Monica O Mabayoje, Adebowale O Adekoya, Olufemi O Adelowo, Mumuni A Amisu
BACKGROUND: The burden of chronic kidney disease (CKD) in Nigeria is quite alarming. The prevalence of CKD ranges from 11 - 23.5%. Hypertension and chronic glomerulonephritis (CGN) remain the two leading causes of CKD in Nigeria. The etiology of CKD in many of these patients remains unknown, as few biopsies are done. In order to demystify the various glomerular diseases that culminate in CGN, performing a kidney biopsy offers a ray of hope. Few studies on renal biopsies have emanated from Nigeria; this study, however, is unique as the histopathological analysis involves light, immunofluorescence, and electron microscopies...
August 10, 2016: Clinical Nephrology
Syed Sajid Hussain Shah, Farkhanda Hafeez
OBJECTIVE: To compare the efficacy of tacrolimus versus cyclosporine (Calcineurin Inhibitors) in the management of childhood steroid-resistant nephritic syndrome (SRNS). STUDY DESIGN: Quasi-experimental study. PLACE AND DURATION OF STUDY: Department of Paediatric Nephrology at The Children's Hospital and Institute of Child Health, Lahore, from August 2014 to September 2015. METHODOLOGY: Patients of either gender aged 1 - 12 years, with the diagnosis of mesangioproliferative glomerulonephritis (MesangioPGN), focal segmental glomerulosclerosis (FSGS) or minimal-change disease (MCD) were included...
July 2016: Journal of the College of Physicians and Surgeons—Pakistan: JCPSP
Andreas Kronbichler, Moin A Saleem, Björn Meijers, Jae Il Shin
Focal segmental glomerulosclerosis (FSGS) is one of the primary glomerular disorders in both children and adults which can progress to end-stage renal failure. Although there are genetic and secondary causes, circulating factors have also been regarded as an important factor in the pathogenesis of FSGS, because about 40% of the patients with FSGS have recurrence after renal transplantation. Soluble urokinase-type plasminogen activator receptor (suPAR) is a soluble form of uPAR, which is a membrane-bound protein linked to GPI in various immunologically active cells, including podocytes...
2016: Journal of Immunology Research
Roxana Cleper, Irit Krause, Nathan Bar Nathan, Maya Mor, Amit Dagan, Irith Weissman, Yaakov Frishberg, Ruty Rachamimov, Eitan Mor, Miriam Davidovits
: From 1982 to 2011, 53 kidney transplantations (KT) for pediatric focal segmental glomerulosclerosis (FSGS) were recorded in the National Israeli Kidney Transplant Registry (NIKTR): 22-primary (1◦) FSGS, 25-proved/suspected genetic-secondary (2◦) FSGS, six lost/incomplete files/other. Half (56%) of 23 patients with 2◦ FSGS were Israeli-Arabs vs 29% of 1◦ FSGS KT recipients. 1◦ FSGS recurrence occurred in 64% (14/22) of 22 KT in 17 patients aged (median) 14 years vs 1/25 of 2◦ FSGS (P<...
October 2016: Clinical Transplantation
Abdullah Kashgary, Jessica M Sontrop, Lihua Li, Ahmed A Al-Jaishi, Zainab N Habibullah, Roaa Alsolaimani, William F Clark
BACKGROUND: Evidence on the role of plasma exchange for treating recurrent post-transplant focal segmental glomerulosclerosis (FSGS) comes largely from individual cases and uncontrolled series. We conducted a systematic review and meta-analysis to estimate the remission rate after treatment with plasma exchange, and to determine if remission varied with patient or treatment characteristics. METHODS: We searched MEDLINE, EMBASE, Science Citation Index Expanded, and the Conference Proceedings Citation Index (Science and BIOSIS) for studies of patients with post-transplant recurrent FSGS who were treated with plasma exchange after recurrence (1950-2012)...
July 29, 2016: BMC Nephrology
Yuan Wu, Pengzhi Hu, Hongbo Xu, Jinzhong Yuan, Lamei Yuan, Wei Xiong, Xiong Deng, Hao Deng
Focal segmental glomerulosclerosis (FSGS) is the most common glomerular histological lesion associated with high-grade proteinuria and end-stage renal disease. Histologically, FSGS is characterized by focal segmental sclerosis with foot process effacement. The aim of this study was to identify the disease-causing mutation in a four-generation Chinese family with FSGS. A novel missense mutation, c.1856G>A (p.Gly619Asp), in the collagen type IV alpha-4 gene (COL4A4) was identified in six patients and it co-segregated with the disease in this family...
July 29, 2016: Journal of Cellular and Molecular Medicine
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