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focal segmental glomerulosclerosis

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https://www.readbyqxmd.com/read/28445873/pregnancy-outcomes-in-patients-with-glomerular-disease-attending-a-single-academic-center-in-north-carolina
#1
Michelle M O'Shaughnessy, Meghan A Jobson, Katy Sims, Abigail L Liberty, Patrick H Nachman, William F Pendergraft
BACKGROUND: Contemporary data regarding pregnancy outcomes in US patients with primary glomerular diseases are lacking. We aimed to report fetal and maternal outcomes among women with biopsy-proven primary glomerular disease who received obstetric care at a single large academic US center. METHODS: All women with a biopsy-confirmed primary glomerular disease diagnosis and without end-stage kidney disease who received obstetric care at the University of North Carolina (UNC) Hospitals (1996-2015) were identified using the Glomerular Disease Collaborative Network registry and the UNC Hospitals Perinatal Database...
April 27, 2017: American Journal of Nephrology
https://www.readbyqxmd.com/read/28441404/angiopoietin-like-4-and-minimal-change-disease
#2
Gabriel Cara-Fuentes, Alfons Segarra, Cecilia Silva-Sanchez, Heiman Wang, Miguel A Lanaspa, Richard J Johnson, Eduardo H Garin
BACKGROUND: Minimal Change Disease (MCD) is the most common type of nephrotic syndrome in children. Angiopoietin-like-4 (Angplt4) has been proposed as mediator of proteinuria in MCD. The aim of this study was to evaluate the role of Angptl4 as a biomarker in MCD. METHODS: Patients with biopsy-proven primary MCD, focal segmental glomerulosclerosis, membranous nephropathy (60, 52 and 52 respectively) and 18 control subjects had urinary and serum Angptl4 measured by Elisa...
2017: PloS One
https://www.readbyqxmd.com/read/28436233/cyclosporine-based-immunosuppressive-therapy-for-patients-with-steroid-resistant-focal-segmental-glomerulosclerosis-a-meta-analysis
#3
Yuan-Yow Chiou, Yi-Che Lee, Mei-Ju Chen
OBJECTIVE: Focal segmental glomerulosclerosis (FSGS) is a leading cause of end-stage kidney disease that requires immunosuppressive treatment as therapy. Few studies have been specifically designed to assess the efficacy of cyclosporine (CSA) in patients with steroid-resistant FSGS. This study investigated the efficacy of CSA-based therapy in steroid-resistant FSGS. METHODS: Medline, Cochrane, EMBASE, and Google Scholar databases were searched through April 30, 2014 using the keywords "cyclosporine," "steroid-resistant," "focal segmental glomerulosclerosis," and "FSGS...
April 22, 2017: Current Medical Research and Opinion
https://www.readbyqxmd.com/read/28435991/polycythemia-capillary-rarefaction-and-focal-glomerulosclerosis-in-two-adolescents-born-extremely-low-birth-weight-and-premature
#4
Nariaki Asada, Takanori Tsukahara, Megumi Furuhata, Daisuke Matsuoka, Shunsuke Noda, Kuniaki Naganuma, Akinori Hashiguchi, Midori Awazu
BACKGROUND: Low birthweight infants have a reduced number of nephrons and are at high risk of chronic kidney disease. Preterm birth and/or intrauterine growth restriction (IUGR) may also affect peritubular capillary development, as has been shown in other organs. CASE-DIAGNOSIS/TREATMENT: We report two patients with a history of preterm birth and extremely low birthweight who showed polycythemia and renal capillary rarefaction. Patient 1 and 2, born at 25 weeks of gestation with a birthweight of 728 and 466 g, showed mild proteinuria at age 8 and 6 years, respectively...
April 24, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28407405/fibrinogen-links-podocyte-injury-with-toll-like-receptor-4-and-is-associated-with-disease-activity-in-fsgs-patients
#5
Hongtian Wang, Chunxia Zheng, Xiaodong Xu, Yue Zhao, Yinghui Lu, Zhihong Liu
AIM: Fibrinogen (Fg) is reported to participate in inflammation through Toll-like receptor 4 (TLR4). However, it remains unknown whether Fg might induce podocyte damage through TLR4 and be related to disease activity in patients with focal segmental glomerulosclerosis (FSGS). METHODS: We observed Fg-induced alterations in actin and apoptosis in cultured human podocytes transfected with or without TLR4 siRNA. Expression of TLR4, phospho-p38 MAPK and phospho-NF-κB p65 was evaluated by quantitative reverse transcription polymerase chain reaction (qRT-PCR) or western blotting, and we analysed urinary Fg levels in adriamycin-treated mice and double immunofluorescence staining for TLR4, Fg and podocin...
April 13, 2017: Nephrology
https://www.readbyqxmd.com/read/28405841/focal-segmental-glomerulosclerosis-and-medullary-nephrocalcinosis-in-children-with-adck4-mutations
#6
Eujin Park, Hee Gyung Kang, Young Hun Choi, Kyoung Bun Lee, Kyung Chul Moon, Hyeon Joo Jeong, Michio Nagata, Hae Il Cheong
BACKGROUND: Mutations in the AarF domain containing kinase 4 gene (ADCK4), one of the novel genes causing steroid-resistant nephrotic syndrome (SRNS), usually manifest as isolated adolescent-onset focal segmental glomerulosclerosis (FSGS). ADCK4 interacts with components of the coenzyme Q10 (CoQ10) biosynthesis pathway. METHODS: The incidence and phenotypes of patients with ADCK4 mutations were investigated in a cohort of Korean pediatric patients with SRNS. RESULTS: Among the 53 patients enrolled in the study the incidence of ADCK4-associated FSGS was 7...
April 12, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28392820/genetics-of-hereditary-nephrotic-syndrome-a-clinical-review
#7
REVIEW
Tae-Sun Ha
Advances in podocytology and genetic techniques have expanded our understanding of the pathogenesis of hereditary steroid-resistant nephrotic syndrome (SRNS). In the past 20 years, over 45 genetic mutations have been identified in patients with hereditary SRNS. Genetic mutations on structural and functional molecules in podocytes can lead to serious injury in the podocytes themselves and in adjacent structures, causing sclerotic lesions such as focal segmental glomerulosclerosis or diffuse mesangial sclerosis...
March 2017: Korean Journal of Pediatrics
https://www.readbyqxmd.com/read/28391347/therapeutics-for-apol1-nephropathies-putting-out-the-fire-in-the-podocyte
#8
Jurgen Heymann, Cheryl A Winkler, Maarten Hoek, Katalin Susztak, Jeffrey B Kopp
APOL1 nephropathies comprise a range of clinical and pathologic syndromes, which can be summarized as focal segmental glomerulosclerosis, in various guises, and arterionephrosclerosis, otherwise known as hypertensive kidney diseases. Current therapies for these conditions may achieve therapeutic targets, reduction in proteinuria and control of blood pressure, respectively, but often fail to halt the progressive decline in kidney function. It appears that current therapies fail to address certain underlying critical pathologic processes that are driven, particularly in podocytes and microvascular cells, by the APOL1 renal risk genetic variants...
January 1, 2017: Nephrology, Dialysis, Transplantation
https://www.readbyqxmd.com/read/28391338/what-are-we-missing-in-the-clinical-trials-of-focal-segmental-glomerulosclerosis
#9
Ladan Zand, Richard J Glassock, An S De Vriese, Sanjeev Sethi, Fernando C Fervenza
Focal segmental glomerulosclerosis (FSGS) is a lesion and not a disease. This conundrum is the crux of controversies regarding interventions to alter its natural history. In the broadest sense, the lesion can be primary (idiopathic), or secondary to a process originating outside the kidneys or to a genetic mutation. The organ-based target is the podocyte, and the mechanisms responsible for the podocytopathy are numerous and diverse. Recurrence of primary FSGS in renal allografts provides the best evidence for the existence of a circulating factor or factors, the nature of which remains uncertain...
January 1, 2017: Nephrology, Dialysis, Transplantation
https://www.readbyqxmd.com/read/28391333/clinical-trials-in-minimal-change-disease
#10
Pietro Ravani, Enrica Bertelli, Simardeep Gill, Gian Marco Ghiggeri
Minimal change disease (MCD) is a pathological condition characterized by subtle glomerular lesions causing massive and reversible proteinuria that is usually steroid sensitive. Recurrence of symptoms of active disease following successful treatment (including proteinuria, oedema and oliguria) and steroid toxicity requires the use of other drugs to attain or maintain remission. Unresolved MCD is considered the initial step in the pathological pathway leading to focal and segmental glomerulosclerosis (FSGS)...
January 1, 2017: Nephrology, Dialysis, Transplantation
https://www.readbyqxmd.com/read/28386663/a-child-with-phenylketonuria-and-focal-segmental-glomerulosclerosis-the-bright-side-of-proteinuria
#11
Fatma Rabah, Khalid Al-Thihli, Mohamed El-Naggari, Ibtisam B Elnour
Phenylketonuria (PKU) is the most common inborn error of amino acid metabolism. Phenylalanine hydroxylase is the underlying deficient enzyme. If left untreated, growth failure, microcephaly, global developmental delay, seizures and severe intellectual impairment would characterize the clinical picture of PKU. On the other side of protein homeostasis lies nephrotic syndrome. It is a well-known quantitative defect due to significant proteinuria. Focal segmental glomerulosclerosis (FSGS) is a special congenital variant affecting children and adults...
April 7, 2017: Metabolic Brain Disease
https://www.readbyqxmd.com/read/28370393/removal-of-focal-segmental-glomerulosclerosis-fsgs-factor-supar-using-cytosorb
#12
Heiko Schenk, Janina Müller-Deile, Roland Schmitt, Jan Hinrich Bräsen, Hermann Haller, Mario Schiffer
Treatment of primary focal segmental glomerulosclerosis (FSGS) and its recurrence after kidney transplantation associated with rapid deterioration of kidney function remains to be challenging despite advances in immunosuppressive therapy. The presence of circulating factors has been postulated to be a pivotal player in the pathogenesis of FSGS, although suPAR and CLCF-1 have been identified as the most promising causative factors. The potential therapeutic effect of suPAR elimination in an FSGS patient using CytoSorb, a hemoadsorption device that gained attention in the cytokine elimination in septic patients, was studied...
March 29, 2017: Journal of Clinical Apheresis
https://www.readbyqxmd.com/read/28356673/remission-of-post-transplant-focal-segmental-glomerulosclerosis-with-angiotensin-receptor-blockers
#13
S B Bansal, S K Sethi, P Jha, R Duggal, V Kher
Recurrence of focal segmental glomerulosclerosis (FSGS) is common after kidney transplantation. Plasmapheresis (PP) is considered to be the most effective treatment; however, results are variable and relapse is common after stopping plasmapheresis. Here, we report an unusual case of recurrent FSGS, who achieved complete remission with angiotensin receptor blocker therapy.
March 2017: Indian Journal of Nephrology
https://www.readbyqxmd.com/read/28355399/comparative-analysis-of-primary-and-secondary-glomerulopathies-in-the-northeast-of-brazil-data-from-the-pernambuco-registry-of-glomerulopathies-repeg
#14
Denise Maria do Nascimento Costa, Lucila Maria Valente, Pedro Alves da Cruz Gouveia, Filipe Wanick Sarinho, Gisele Vajgel Fernandes, Maria Alina Gomes de Mattos Cavalcante, Camila Barbosa Lyra de Oliveira, Carolina de Andrade Jordão de Vasconcelos, Emanuel Sávio Cavalcante Sarinho
INTRODUCTION: In Brazil, glomerulopathies are the third leading cause of chronic renal disease, accounting for 11% of dialysis patients. Studies on the prevalence of this disease in Northeastern Brazil are scarce. OBJECTIVE: The aim was to describe the findings of biopsies and to conduct a comparative analysis on the clinical laboratory presentation of primary glomerulopathies (PG) and secondary glomerulopathies (SG). METHODS: This was a retrospective study conducted at two public teaching hospitals in the state of Pernambuco, Northeastern Brazil...
March 2017: Jornal Brasileiro de Nefrologia: ʹorgão Oficial de Sociedades Brasileira e Latino-Americana de Nefrologia
https://www.readbyqxmd.com/read/28355395/obesity-and-kidney-disease
#15
Geraldo Bezerra da Silva Junior, Ana Carla Sobral Novaes Bentes, Elizabeth De Francesco Daher, Sheila Maria Alvim de Matos
Obesity has been pointed out as an important cause of kidney diseases. Due to its close association with diabetes and hypertension, excess weight and obesity are important risk factors for chronic kidney disease (CKD). Obesity influences CKD development, among other factors, because it predisposes to diabetic nephropathy, hypertensive nephrosclerosis and focal and segmental glomerulosclerosis. Excess weight and obesity are associated with hemodynamic, structural and histological renal changes, in addition to metabolic and biochemical alterations that lead to kidney disease...
March 2017: Jornal Brasileiro de Nefrologia: ʹorgão Oficial de Sociedades Brasileira e Latino-Americana de Nefrologia
https://www.readbyqxmd.com/read/28352022/analysis-of-renal-diseases-detected-in-renal-biopsies-of-adult-patients-a-single-center-experience
#16
Salman Imtiaz, Murtaza F Drohlia, Kiran Nasir, Beena Salman, Aasim Ahmad
Renal biopsy is crucial while evaluating for the diagnosis of glomerular, vascular, tubulointerstitial, and genetic diseases. It gives vital information which helps in estimating the disease prognosis, progression, and management. This is the retrospective analysis of all adult patients aged above 18 years, who underwent percutaneous renal biopsy at The Kidney Center Post Graduate Training Institute, Karachi, over a duration of 18 years, i.e., January 1, 1996, to December 2013. Renal graft biopsies and those which were inadequate were excluded from analysis...
March 2017: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/28352020/profile-of-glomerular-diseases-associated-with-hepatitis-b-and-c-a-single-center-experience-from-india
#17
Nishad Raveendran, Pankaj Beniwal, Amith Vijay Leon D'Souza, Rajendra Singh Tanwar, Piyush Kimmatkar, Dhananjai Agarwal, Vinay Malhotra
Hepatitis B and C are known to affect kidneys in a number of ways. Glomerular diseases associated with hepatitis B and C include membranous nephropathy (MN), membranoproliferative glomerulonephritis (MPGN), focal segmental glomerulosclerosis, immunoglobulin A nephropathy, rarely amyloidosis, and fibrillary and immunotactoid glomerulopathy. In a retrospective analysis of kidney biopsy of 534 patients, we found 16 (2.9%) patients of hepatitis B and 11 (2.05%) patients of hepatitis C with glomerular disease. The most common form of glomerulonephritis in hepatitis B patient was MN and in hepatitis C patient was MPGN...
March 2017: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/28352011/biopsy-of-small-kidneys-a-safe-and-a-useful-guide-to-potentially-treatable-kidney-disease
#18
Kamel El-Reshaid, Wael El-Reshaid, Dalal Al-Bader, Jozsef Varro, John Madda, Hosameldin Tawfik Sallam
Over the past four years, all patients with unexplained rapid progression of their renal disease were subjected to kidney biopsy, despite their small size (<9 cm), to define its etiology. Children, pregnant women, morbidly obese patients, and those with an unstable cardiovascular state, septicemia, bleeding diathesis as well as those kidney size with size <6 cm were excluded from the study. Doppler ultrasound was used to exclude renovascular/ischemic nephropathy. The procedure was performed by an interventional radiologist using a biopsy gun technique and under ultrasound guidance...
March 2017: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/28339906/interstitial-fibrosis-scored-on-whole-slide-digital-imaging-of-kidney-biopsies-is-a-predictor-of-outcome-in-proteinuric-glomerulopathies
#19
Laura H Mariani, Sebastian Martini, Laura Barisoni, Pietro A Canetta, Jonathan P Troost, Jeffrey B Hodgin, Matthew Palmer, Avi Z Rosenberg, Kevin V Lemley, Hui-Ping Chien, Jarcy Zee, Abigail Smith, Gerald B Appel, Howard Trachtman, Stephen M Hewitt, Matthias Kretzler, Serena M Bagnasco
Background.: Interstitial fibrosis (IF), tubular atrophy (TA) and interstitial inflammation (II) are known determinants of progression of renal disease. Standardized quantification of these features could add value to current classification of glomerulopathies. Methods.: We studied 315 participants in the Nephrotic Syndrome Study Network (NEPTUNE) study, including biopsy-proven minimal change disease (MCD = 98), focal segmental glomerulosclerosis (FSGS = 121), membranous nephropathy (MN = 59) and IgA nephropathy (IgAN = 37)...
February 27, 2017: Nephrology, Dialysis, Transplantation
https://www.readbyqxmd.com/read/28339709/clinicopathologic-correlations-of-renal-pathology-in-the-adult-population-of-poland
#20
Agnieszka Perkowska-Ptasinska, Artur Bartczak, Malgorzata Wagrowska-Danilewicz, Agnieszka Halon, Krzysztof Okon, Aldona Wozniak, Marian Danilewicz, Henryk Karkoszka, Andrzej Marszalek, Jolanta Kowalewska, Andrzej Mroz, Agnieszka Korolczuk, Andrzej Oko, Alicja Debska-Slizien, Beata Naumnik, Zbigniew Hruby, Marian Klinger, Kazimierz Ciechanowski, Marek Myslak, Wladyslaw Sulowicz, Andrzej Rydzewski, Andrzej Wiecek, Jacek Manitius, Tadeusz Gregorczyk, Stanislaw Niemczyk, Michal Nowicki, Ryszard Gellert, Tomasz Stompor, Monika Wieliczko, Krzysztof Marczewski, Leszek Paczek, Olga Rostkowska, Dominika Deborska-Materkowska, Grazyna Bogdanowicz, Andrzej Milkowski, Magdalena Durlik
Background: This is the first report on the epidemiology of biopsy-proven kidney diseases in Poland. Methods: The Polish Registry of Renal Biopsies has collected information on all (n = 9394) native renal biopsies performed in Poland from 2009 to 2014. Patients' clinical data collected at the time of biopsy, and histopathological diagnoses were used for epidemiological and clinicopathologic analysis. Results: There was a gradual increase in the number of native renal biopsies performed per million people (PMP) per year in Poland in 2009-14, starting from 36 PMP in 2009 to 44 PMP in 2014...
April 1, 2017: Nephrology, Dialysis, Transplantation
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