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https://www.readbyqxmd.com/read/29138824/genetic-mutational-testing-of-chinese-children-with-familial-hematuria-with-biopsy%C3%A2-proven-fsgs
#1
Yongzhen Li, Ying Wang, Qingnan He, Xiqiang Dang, Yan Cao, Xiaochuan Wu, Shuanghong Mo, Xiaoxie He, Zhuwen Yi
Focal segmental glomerulosclerosis (FSGS) is a pathological lesion rather than a disease, with a diverse etiology. FSGS may result from genetic and non‑genetic factors. FSGS is considered a podocyte disease due to the fact that in the majority of patients with proven‑FSGS, the lesion results from defects in the podocyte structure or function. However, FSGS does not result exclusively from podocyte‑associated genes, however also from other genes including collagen IV‑associated genes. Patients who carry the collagen type IVA3 chain (COL4A3) or COL4A4 mutations usually exhibit Alport Syndrome (AS), thin basement membrane neuropathy or familial hematuria (FH)...
November 10, 2017: Molecular Medicine Reports
https://www.readbyqxmd.com/read/29137483/molecular-profiling-in-iga-nephropathy-and-focal-and-segmental-glomerulosclerosis
#2
I Tycová, P Hrubá, D Maixnerová, E Girmanová, P Mrázová, L Straňavová, R Zachoval, M Merta, J Slatinská, M Kollár, E Honsová, V Tesař, O Viklický
The aim of the study was to characterize by molecular profiling two glomerular diseases: IgA nephropathy (IgAN) and focal segmental glomerulosclerosis (FSGS) and to identify potential molecular markers of IgAN and FSGS progression. The expressions of 90 immune-related genes were compared in biopsies of patients with IgAN (n=33), FSGS (n=17) and in controls (n=11) using RT-qPCR. To identify markers of disease progression, gene expression was compared between progressors and non-progressors in 1 year follow-up...
November 10, 2017: Physiological Research
https://www.readbyqxmd.com/read/29130775/results-of-native-and-transplant-kidney-biopsies-of-children-in-a-single-center-over-a-15-years-period
#3
Emel Isiyel, Kibriya Fidan, Bahar Buyukkaragoz, Meltem Akcaboy, Yasar Kandur, Ipek Isik Gonul, Necla Buyan, Sevcan Bakkaloglu, Oguz Soylemezoglu
Renal biopsy is an important diagnostic procedure in pediatric nephrology. This study retrospectively investigates the indications, results and complications in a single tertiary children's hospital in Turkey. We evaluated the native and transplant kidney biopsies in Gazi University Pediatric Nephrology Department between 2001 and 2015. A total of 196 biopsies (144 natives and 52 transplants) were included into the study. The mean age of the patients was respectively 10.8 ± 3.5, 13.9 ± 1.5 years...
November 2017: Renal Failure
https://www.readbyqxmd.com/read/29117638/renal-transplant-outcomes-in-primary-fsgs-compared-with-other-recipients-and-risk-factors-for-recurrence-a-national-review-of-the-irish-transplant-registry
#4
S Cormican, C Kennedy, P O'Kelly, B Doyle, A Dorman, A Awan, P J Conlon
INTRODUCTION: Primary focal segmental glomerular sclerosis (p-FSGS) is commonly complicated by recurrence (r-FSGS) post-transplantation. Our objective was to describe Irish outcomes for transplantation after ESRD due to p-FSGS, specifically rates of, and treatments for, r-FSGS. PATIENTS AND METHODS: Irish patients with biopsy proven FSGS were identified from the Irish National Kidney Transplant database (1982-2015). Medical record review was performed to identify predictors of r-FSGS and treatments for r-FSGS...
November 8, 2017: Clinical Transplantation
https://www.readbyqxmd.com/read/29115212/endemic-influences-of-political-regimes-healthcare-systems-and-preferences-on-the-frequencies-and-incidences-of-nephropathies-in-eastern-saxony-germany%C3%A2
#5
Sam Sedaghat, Eleène Sahmila Heshmati, Thomas Frese, Wilma Gertrud Rasche, Filip Barinka, Florian Fahr, Joachim Beige, Stephan Schiekofer, Franz Maximilian Rasche
INTRODUCTION: The possible confounding influence of investigator-related preferences, available histological techniques, and healthcare systems on the frequencies and incidences of primary and secondary nephropathies was evaluated in this long-term observation. MATERIALS AND METHODS: The observation time from 1983 to 2010 was divided in regard to the political regimes: a) prior to and after German reunification: German Democratic Republic (GDR, period 1 from 1983 to 1990)/Federal Republic of Germany (FRG, period 2 from 1990 to 2010); and the two heads of the division of nephrology, b) conductor 1 (1983 - 2006) and conductor 2 (2006 - 2010)...
November 8, 2017: Clinical Nephrology
https://www.readbyqxmd.com/read/29110886/the-incidence-of-primary-vs-secondary-focal-segmental-glomerulosclerosis-a-clinicopathologic-study
#6
Musab S Hommos, An S De Vriese, Mariam P Alexander, Sanjeev Sethi, Lisa Vaughan, Ladan Zand, Kharmen Bharucha, Nicola Lepori, Andrew D Rule, Fernando C Fervenza
OBJECTIVES: To describe the change in the incidence rates of primary and secondary focal segmental glomerulosclerosis (FSGS) from 1994 through 2013 in Olmsted County, Minnesota, and to identify the clinical and biopsy characteristics that distinguish primary from secondary FSGS. PATIENTS AND METHODS: Olmsted County adult residents with native kidney biopsy from January 1, 1994, through December 31, 2013, and FSGS as the only glomerulopathy were identified. The clinical and pathologic characterstics of primary and secondary FSGS were described and compared, and incidence rates were calculated...
October 27, 2017: Mayo Clinic Proceedings
https://www.readbyqxmd.com/read/29106637/glomerular-disease-frequencies-by-race-sex-and-region-results-from-the-international-kidney-biopsy-survey
#7
Michelle M O'Shaughnessy, Susan L Hogan, Bawana D Thompson, Rosanna Coppo, Agnes B Fogo, J Charles Jennette
Background: Large-scale studies comparing glomerular disease frequencies across continents are lacking. Methods: We surveyed 29 nephropathology laboratories in four continents using a standardized data collection form. We obtained recent consecutive kidney biopsy diagnosis frequencies at each center and summary demographics for each diagnosis. This report focuses on glomerular disease frequencies by region and race/ethnicity. Results: Among 42 603 glomerular disease diagnoses reported (median age 47 years, 52% male, 57% white), from a total of 60 340 diagnoses, glomerular disease subtype frequencies differed considerably by continent...
July 2, 2017: Nephrology, Dialysis, Transplantation
https://www.readbyqxmd.com/read/29095250/determination-of-the-optimal-target-level-of-proteinuria-in-the-management-of-patients-with-glomerular-diseases-by-using-different-definitions-of-proteinuria
#8
Youn Kyung Kee, Chan-Yun Yoon, Seung Jun Kim, Sung Jin Moon, Chan Ho Kim, Jung Tak Park, Beom Jin Lim, Tae Ik Chang, Ea Wha Kang, Jeong Hae Kie, Tae-Hyun Yoo, Hyun Joo Jeong, Shin-Wook Kang, Seung Hyeok Han
Proteinuria is a major determinant of adverse renal outcome, and its reduction slows renal progression in glomerular diseases. However, the optimal target of proteinuria in glomerular diseases is unclear, and discrepancies in the definition of proteinuria produce ambiguous findings. Here we investigated the optimal target of proteinuria by using different definitions of proteinuria. We analyzed 574 IgA nephropathy (IgAN), 175 membranous nephropathy (MGN), and 177 focal segmental glomerulosclerosis (FSGS) cases from 3 Korean kidney centers...
November 2017: Medicine (Baltimore)
https://www.readbyqxmd.com/read/29071895/-effect-of-mild-moxibustion-with-different-terms-at-different-acupoints-on-renal-functions-and-renal-pathological-changes-in-focal-segmental-glomerulosclerosis-rats
#9
Lin Li, Yu-Xia Sun, Jia-Yi Ma, Chun-Ling Zhang, Yi Li
OBJECTIVE: To observe changes of urinary microprotein, and serum creatinine, urea nitrogen and uric acid levels in focal segmental glomerulosclerosis (FSGS) rats treated by mild moxibustion, so as to explore the mechanism of moxibustion underlying improvement of FSGS. METHODS: SD rats were randomized into normal control (normal), sham operation (sham), model, medication (Losartan), moxibustion-Shenshu (BL 23) and moxibustion-Geshu (BL 17) groups. The latter two moxibustion groups were further divided into 10 min, 20 min and 30 min subgroups (n=6 in each group/subgroups)...
December 25, 2016: Zhen Ci Yan Jiu, Acupuncture Research
https://www.readbyqxmd.com/read/29064158/the-natural-compound-2-3-5-4-tetrahydroxystilbene-2-o-%C3%AE-d-glucoside-protects-against-adriamycin-induced-nephropathy-through-activating-the-nrf2-keap1-antioxidant-pathway
#10
En-Yuan Lin, Uyanga Bayarsengee, Ching-Chiung Wang, Yung-Hsiao Chiang, Chao-Wen Cheng
2,3,5,4'-Tetrahydroxystilbene-2-O-β-d-glucoside (THSG) is an active compound extracted from Polygonum multiflorum Thunb. This herb and radix Polygoni Multiflori preparata have been used to treat arteriosclerosis, hyperlipidemia, hypercholesterolemia, and diabetes for thousands of years. This study aimed to investigate the protective effects of THSG in an Adriamycin (AD)-induced focal segmental glomerulosclerosis (FSGS) mouse model and the underlying mechanisms in an in vitro system. Mice were treated with THSG (2...
October 24, 2017: Environmental Toxicology
https://www.readbyqxmd.com/read/29043143/focal-segmental-glomerulosclerosis-associated-with-mitochondrial-disease
#11
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/29038887/mutations-in-inf2-may-be-associated-with-renal-histology-other-than-focal-segmental-glomerulosclerosis
#12
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
#13
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/28993503/myh9-e1841k-mutation-augments-proteinuria-and-podocyte-injury-and-migration
#14
Sylvia Cechova, Fan Dong, Fang Chan, Michael J Kelley, Phillip Ruiz, Thu H Le
Intronic variants of the MYH9 gene that encodes the nonmuscle myosin heavy chain IIA are associated with diabetic nephropathy in European Americans and with sickle cell disease-associated nephropathy. However, the causal functional variants of MYH9 have remained elusive. Rare missense mutations in MYH9 cause macrothrombocytopenia and are occasionally associated with development of nephropathy. The E1841K mutation is among the common MYH9 missense mutations and has been associated with nephropathy in some carriers...
October 9, 2017: Journal of the American Society of Nephrology: JASN
https://www.readbyqxmd.com/read/28992235/treatment-by-immunoadsorption-for-recurrent-focal-segmental-glomerulosclerosis-after-paediatric-kidney-transplantation-a-multicentre-french-cohort
#15
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/28989175/glomerular-disease-loss-of-epas1-promotes-fsgs
#16
Jack M Heintze
No abstract text is available yet for this article.
November 2017: Nature Reviews. Nephrology
https://www.readbyqxmd.com/read/28982981/the-hippo-pathway-regulator-kibra-promotes-podocyte-injury-by-inhibiting-yap-signaling-and-disrupting-actin-cytoskeletal-dynamics
#17
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
#18
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
#19
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
#20
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
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