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Focal and segmental glomerulosclerosis

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https://www.readbyqxmd.com/read/28822451/-the-value-of-serum-free-light-chain-in-differential-diagnosis-of-monoclonal-gammopathy-of-renal-significance
#1
C Li, Y B Wen, H Li, W Su, J Li, J F Cai, L M Chen, X M Li, X W Li
Objective: To investigate the value of serum free light chain (FLC) in differential diagnosis of monoclonal gammopathy of renal significance (MGRS). Methods: Forty-nine hospitalized patients who underwent renal biopsy in Peking Union Medical College Hospital between January 2013 and December 2015 were included. Monoclonal gammopathy was detected by serum protein electrophoresis (SPE), serum immunofixation electrophoresis (IFE), urine IFE and serum FLC. All patients were classified as MGRS (n=32) and monoclonal gammopathy of undetermined significance (MGUS) (n=17)...
August 8, 2017: Zhonghua Yi Xue za Zhi [Chinese medical journal]
https://www.readbyqxmd.com/read/28800680/pharmacological-inhibition-of-fsgs-related-trpc6-gain-of-function-mutants-by-semisynthetic-larixol-derived-compounds
#2
Nicole Urban, Sonja Neuser, Anika Hentschel, Sebastian Köhling, Jörg Rademann, Michael Schaefer
BACKGROUND AND PURPOSE: Gain of function mutations in TRPC6 can cause autosomal dominant forms of focal segmental glomerulosclerosis (FSGS). Validated inhibitors of TRPC6 that are biologically active on FSGS-related TRPC6 mutants are eagerly sought. EXPERIMENTAL APPROACH: We synthesized new TRPC6-inhibiting modulators from larixol, a resiniferous constituent of Larix decidua, and tested the potency and selectivity in cell lines stably expressing various TRP channel isoforms...
August 11, 2017: British Journal of Pharmacology
https://www.readbyqxmd.com/read/28799058/is-cd44-in-glomerular-parietal-epithelial-cells-a-pathological-marker-of-renal-function-deterioration-in-primary-focal-segmental-glomerulosclerosis
#3
Brunna Pinto Froes, Stanley de Almeida Araújo, Eduardo Alves Bambirra, Eduardo Araújo Oliveira, Ana Cristina Simões E Silva, Sérgio Veloso Brant Pinheiro
BACKGROUND: The search for risk factors for chronic kidney disease in children with focal segmental glomerulosclerosis (FSGS) is important in defining prognosis and individualized treatment. This study preliminarily investigated whether CD44 immunostaining in glomerular parietal epithelial cells (PECs) is a prognostic marker in pediatric FSGS. METHODS: In this retrospective study, 26 patients with FSGS, biopsied from 1985 to 2010, were evaluated. Immunohistochemistry for CD44 was performed in all cases...
August 10, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28797008/resveratrol-attenuates-adriamycin-induced-focal-segmental-glomerulosclerosis-through-c3ar-c5ar-sphingosine-kinase-1-pathway
#4
Guoyong Liu, Qiang Wang, Yan Shi, Xiaofei Peng, Hong Liu, Youming Peng, Liyu He
BACKGROUND/AIM: Focal segmental glomerulosclerosis (FSGS) typically presents with nephrotic range proteinuria, which could eventually develop into end-stage renal disease. Resveratrol (RSV) is a natural polyphenol compound, which has been reported to suppress inflammatory response and renal interstitial fibrosis. This study is aimed at evaluating the renoprotective effect of RSV treatment on adriamycin-induced FSGS. METHODS: In Balb/c mice, adriamycin nephropathy was induced by adriamycin (10 mg/kg body weight, diluted in normal saline) via a tail vein on day 0...
August 11, 2017: Pharmacology
https://www.readbyqxmd.com/read/28791668/obesity-related-glomerulopathy-pathogenesis-pathologic-clinical-characteristics-and-treatment
#5
REVIEW
Tianhua Xu, Zitong Sheng, Li Yao
In light of the rapid increase in the number of obesity incidences worldwide, obesity has become an independent risk factor for chronic kidney disease. Obesity-related glomerulopathy (ORG) is characterized by glomerulomegaly in the presence or absence of focal and segmental glomerulosclerosis lesions. IgM and complement 3 (C3) nonspecifically deposit in lesions without immune-complex-type deposits during ORG immunofluorescence. ORG-associated glomerulomegaly and focal and segmental glomerulosclerosis can superimpose on other renal pathologies...
August 8, 2017: Frontiers of Medicine
https://www.readbyqxmd.com/read/28776307/genetic-basis-of-adult-onset-nephrotic-syndrome-and-focal-segmental-glomerulosclerosis
#6
REVIEW
Jian Liu, Weiming Wang
Nephrotic syndrome (NS) is one of the most common glomerular diseases with signs of nephrosis, heavy proteinuria, hypoalbuminemia, and edema. Dysfunction of glomerular filtration barrier causes protein loss through the kidneys. Focal segmental glomerulosclerosis (FSGS) accounts for nearly 20% of NS among children and adults. Adult-onset FSGS/NS is often associated with low response to steroid treatment and immunosuppressive medication and poor renal survival. Several genes involved in NS and FSGS have been identified by linkage analysis and next-generation sequencing...
August 3, 2017: Frontiers of Medicine
https://www.readbyqxmd.com/read/28774288/the-impact-of-igm-deposits-on-the-outcome-of-nephrotic-syndrome-in-children
#7
Sandra Juozapaite, Rimante Cerkauskiene, Arvydas Laurinavicius, Augustina Jankauskiene
BACKGROUND: The significance of IgM deposits in glomerular mesangium has been controversial since they were first described due to the variations in the both the definitions used and described impact on clinical outcome. The aim of our study was to evaluate the significance of the IgM deposits in the glomerular mesangium for outcomes of nephrotic syndrome (NS) in children. METHODS: Forty-five children with NS who underwent renal biopsy at tertiary pediatric hospital from January 1st, 2000 to December 31st, 2015 and the pathology diagnosis of minimal change disease, focal segmental glomerulosclerosis and mesangial hypercellularity (MH) were retrospectively analyzed...
August 3, 2017: BMC Nephrology
https://www.readbyqxmd.com/read/28763127/european-veterinary-renal-pathology-service-a-survey-over-a-7-year-period-2008-2015
#8
L Aresu, V Martini, S L Benali, C Brovida, R E Cianciolo, R Dalla Riva, D Trez, J J Van Der Lugt, A Van Dongen, E Zini
BACKGROUND: The European Veterinary Renal Pathology Service (EVRPS) is the first Web-based registry for canine renal biopsy specimens in Europe. HYPOTHESIS/OBJECTIVES: The aim was to verify whether differences exist between the clinical and laboratory presentation of dogs with nephropathy according to renal pathological findings, as defined by light and electron microscopy of renal biopsy specimens submitted to EVRPS. ANIMALS: Renal biopsy specimens of dogs were collected from the archive of the service (n = 254)...
August 1, 2017: Journal of Veterinary Internal Medicine
https://www.readbyqxmd.com/read/28761228/kidney-disease-in-human-immunodeficiency-virus-seropositive-patients-absence-of-human-immunodeficiency-virus-associated-nephropathy-was-a-characteristic-feature
#9
J Prakash, V Ganiger, S Prakash, M Sivasankar, S Sunder, U Singh
Human immunodeficiency virus (HIV) infection can cause a broad spectrum of renal diseases. However, there is paucity of Indian data on the patterns of renal lesions in HIV-seropositive patients. The aim of the present study was to delineate the spectrum of renal lesions in HIV/acquired immunodeficiency syndrome patients. In this prospective study, all HIV-positive patients of both genders aged >18 years were screened for renal disease. Patients with proteinuria of more than 1 g/24 h were subjected to renal biopsy...
July 2017: Indian Journal of Nephrology
https://www.readbyqxmd.com/read/28753930/pregnancy-proteinuria-plant-based-supplemented-diets-and-focal-segmental-glomerulosclerosis-a-report-on-three-cases-and-critical-appraisal-of-the-literature
#10
Rossella Attini, Filomena Leone, Benedetta Montersino, Federica Fassio, Fosca Minelli, Loredana Colla, Maura Rossetti, Cristiana Rollino, Maria Grazia Alemanno, Antonella Barreca, Tullia Todros, Giorgina Barbara Piccoli
Chronic kidney disease (CKD) is increasingly recognized in pregnant patients. Three characteristics are associated with a risk of preterm delivery or small for gestational age babies; kidney function reduction, hypertension, and proteinuria. In pregnancy, the anti-proteinuric agents (ACE-angiotensin converting enzyme-inhibitors or ARBS -angiotensin receptor blockers) have to be discontinued for their potential teratogenicity, and there is no validated approach to control proteinuria. Furthermore, proteinuria usually increases as an effect of therapeutic changes and pregnancy-induced hyperfiltration...
July 19, 2017: Nutrients
https://www.readbyqxmd.com/read/28752288/application-of-next-generation-sequencing-technology-to-diagnosis-and-treatment-of-focal-segmental-glomerulosclerosis
#11
REVIEW
Yutaka Harita
A broad range of genetic and non-genetic factors can lead to kidney injury that manifests as focal segmental glomerulosclerosis (FSGS), which can be classified into primary (idiopathic) and secondary forms. Previous genetic approaches to familial or sporadic cases of FSGS or steroid-resistant nephrotic syndrome identified causal mutations in a subset of genes. Recently, next-generation sequencing (NGS) approaches are becoming a part of a standard assessment in medical genetics. Current knowledge of the comprehensive genomic information is changing the way we think about FSGS and draws attention not only to identification of novel causal genes, but also to potential roles for combinations of mutations in multiple genes, mutations with complex inheritance, and susceptibility genes with variable penetrance carrying relatively minor but significant effects...
July 27, 2017: Clinical and Experimental Nephrology
https://www.readbyqxmd.com/read/28748893/complication-rate-and-diagnostic-yield-of-percutaneous-native-kidney-biopsies-a-10-year-experience-at-a-tertiary-care-hospital-in-pakistan
#12
Sonia Yaqub, Nayla Ahmed, Urooj Fatima, Ayesha Maqbool, Waqar Ashif, Syed A Hussain
The use of an automated biopsy device, and real-time ultrasound for percutaneous kidney biopsies (PKBs) has improved the likelihood of obtaining adequate tissue for diagnosis and also has reduced the complications associated with the procedure. We aimed to determine the frequency and type of complications associated with PKB and to determine the diagnostic yield. It was a retrospective file-based review of cases who underwent PKB of native kidney between January 2003 and December 2013 at the Aga Khan University Hospital in Karachi, Pakistan...
July 2017: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/28748888/childhood-idiopathic-steroid-resistant-nephrotic-syndrome-at-a-single-center-in-khartoum
#13
El-Tigani M A Ali, Hanna F K Makki, Mohamed B Abdelraheem, Salwa O Makke, Rashid A Allidir
Prevalence, clinicopathological features, and outcome of childhood idiopathic steroid-resistant nephrotic syndrome (ISRNS) vary in different countries. We report on these parameters in a single center in Khartoum. We retrospectively reviewed all the records of children with idiopathic nephrotic syndrome (INS) followed up in the pediatric renal unit, Soba Hospital, Khartoum between 2001 and 2012. ISRNS was defined as no remission within four weeks of daily prednisolone at a dose of 60 mg/m2. In 430 children with INS 130 (28%) had SRNS with a mean age of 7...
July 2017: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/28748887/nondiabetic-renal-disease-in-patients-with-type-2-diabetes
#14
Ikram Mami, Amel Harzallah, Hayet Kaaroud, Raja Aoudia, Fethi Ben Hamida, Rim Goucha, Taieb Ben Abdallah
Diabetic nephropathy (DN) is one of the major complications of type 2 diabetes mellitus (T2DM). The diagnosis of DN is mostly clinical. Kidney biopsy is indicated only if nondiabetic renal disease (NDRD) is suspected. This study is aimed to assess the prevalence of NDRD and to determine predictor and prognostic factors of DN, NDRD. It was a retrospective analytic study including T2DM patients in whom renal biopsies were performed at our department from 1988 to 2014. Seventy-five patients were included. Mean age was 52...
July 2017: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/28748184/complement-in-non-antibody-mediated-kidney-diseases
#15
REVIEW
Andrea Angeletti, Joselyn Reyes-Bahamonde, Paolo Cravedi, Kirk N Campbell
The complement system is part of the innate immune response that plays important roles in protecting the host from foreign pathogens. The complement components and relative fragment deposition have long been recognized to be strongly involved also in the pathogenesis of autoantibody-related kidney glomerulopathies, leading to direct glomerular injury and recruitment of infiltrating inflammation pathways. More recently, unregulated complement activation has been shown to be associated with progression of non-antibody-mediated kidney diseases, including focal segmental glomerulosclerosis, C3 glomerular disease, thrombotic microangiopathies, or general fibrosis generation in progressive chronic kidney diseases...
2017: Frontiers in Medicine
https://www.readbyqxmd.com/read/28745696/-obesity-related-glomerulopathy-mechanisms-of-development-clinical-course
#16
REVIEW
I M Kutyrina
Obesity and overweight are recognized as epidemics of non-communicable diseases in the 21st century. The kidneys are a target organ for obesity, damage to which is considered to be an independent risk factor for the development of renal failure. Obesity-related glomerulopathy (OGP) is one of the types of renal injury in obesity, which is characterized by the development of proteinuria in patients with a body mass index (BMI) of >30 kg/m2 in the absence of other causes of kidney damage. The pathogenesis of OGP is multifactorial...
2017: Terapevticheskiĭ Arkhiv
https://www.readbyqxmd.com/read/28724797/increasing-the-level-of-peroxisome-proliferator-activated-receptor-%C3%AE-coactivator-1%C3%AE-in-podocytes-results-in-collapsing-glomerulopathy
#17
Szu-Yuan Li, Jihwan Park, Chengxiang Qiu, Seung Hyeok Han, Matthew B Palmer, Zoltan Arany, Katalin Susztak
Inherited and acquired mitochondrial defects have been associated with podocyte dysfunction and chronic kidney disease (CKD). Peroxisome proliferator-activated receptor γ coactivator-1α (PGC1α) is one of the main transcriptional regulators of mitochondrial biogenesis and function. We hypothesized that increasing PGC1α expression in podocytes could protect from CKD. We found that PGC1α and mitochondrial transcript levels are lower in podocytes of patients and mouse models with diabetic kidney disease (DKD)...
July 20, 2017: JCI Insight
https://www.readbyqxmd.com/read/28724378/erratum-to-significance-of-the-urokinase-type-plasminogen-activator-and-its-receptor-in-the-progression-of-focal-segmental-glomerulosclerosis-in-clinical-and-mouse-models
#18
Jin-Shuen Chen, Li-Chien Chang, Chung-Ze Wu, Tzu-Ling Tseng, Jui-An Lin, Yuh-Feng Lin, Chao-Wen Cheng
No abstract text is available yet for this article.
July 19, 2017: Journal of Biomedical Science
https://www.readbyqxmd.com/read/28722118/at2-r-deficiency-mediated-podocyte-loss-via-activation-of-ectopic-hedgehog-interacting-protein-hhip-gene-expression
#19
Min-Chun Liao, Xin-Ping Zhao, Shiao-Ying Chang, Chao-Sheng Lo, Isabelle Chenier, Tomoko Takano, Julie R Ingelfinger, Shao-Ling Zhang
Angiotensin II type 2 receptor (AT2 R) deficiency in AT2 R knockout (KO) mice has been linked to congenital abnormalities of the kidney and urinary tract; however, the mechanisms by which this occurs are poorly understood. In this study, we examined whether AT2 R deficiency impaired glomerulogenesis and mediated podocyte loss/dysfunction in vivo and in vitro. Nephrin-cyan fluorescent protein (CFP)-transgenic (Tg) and Nephrin/AT2 RKO mice were used to assess glomerulogenesis, while wild-type and AT2 RKO mice were used to evaluate maturation of podocyte morphology/function...
July 19, 2017: Journal of Pathology
https://www.readbyqxmd.com/read/28707483/investigational-drugs-in-development-for-focal-segmental-glomerulosclerosis
#20
REVIEW
Howard Trachtman
Focal segmental glomerulosclerosis is an important cause of end stage kidney disease and is a paradigm for the study of glomerular scarring. There are no FDA approved treatments for this condition. Current therapies, assessed based on reduction in proteinuria, are generally effective in a subset of patients which suggests that FSGS is a heterogeneous group of glomerular disorders or podocytopathies that converge on a common histopathological phenotype. Areas covered: We searched for investigational drugs agents that target different pathophysiological pathways using the key words 'FSGS' and 'podocyte' in American and European clinical trial registers (clinicaltrials...
August 2017: Expert Opinion on Investigational Drugs
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