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Nephrotic syndrom

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https://www.readbyqxmd.com/read/29457738/reproducibility-and-feasibility-of-strategies-for-morphologic-assessment-of-renal-biopsies-using-the-nephrotic-syndrome-study-network-digital-pathology-scoring-system
#1
Jarcy Zee, Jeffrey B Hodgin, Laura H Mariani, Joseph P Gaut, Matthew B Palmer, Serena M Bagnasco, Avi Z Rosenberg, Stephen M Hewitt, Lawrence B Holzman, Brenda W Gillespie, Laura Barisoni
CONTEXT: - Testing reproducibility is critical for the development of methodologies for morphologic assessment. Our previous study using the descriptor-based Nephrotic Syndrome Study Network Digital Pathology Scoring System (NDPSS) on glomerular images revealed variable reproducibility. OBJECTIVE: - To test reproducibility and feasibility of alternative scoring strategies for digital morphologic assessment of glomeruli and explore use of alternative agreement statistics...
February 19, 2018: Archives of Pathology & Laboratory Medicine
https://www.readbyqxmd.com/read/29457021/difficult-renal-pathological-classification-in-a-case-of-pediatric-nephrotic-syndrome
#2
Hiroshi Yamaguchi, Atsutoshi Shiratori, Taku Nakagawa, Kyoko Kanda, Shigeo Hara, Norishige Yoshikawa, Ryojiro Tanaka
The underlying histopathology is very important in determining patient management, as the histopathology usually has direct repercussions on the treatment response and clinical course. However, the impact of the method used to assess renal biopsies, i.e., light microscopy (LM), immunofluorescence (IF), and electron microscopy (EM), on the occurrence of a difficult biopsy classification in the native kidneys of pediatric nephrotic patients is unknown. A 12-month-old Japanese boy was diagnosed with nephrotic syndrome (NS); he was administered prednisolone (60 mg/m2 /day), and a continuous albumin infusion was started...
September 2017: Case Reports in Nephrology and Dialysis
https://www.readbyqxmd.com/read/29456400/unilateral-transient-watershed-cerebral-infarct-in-a-6-year-old-girl-with-frequently-relapsing-nephrotic-syndrome
#3
Kapil Bhalla, Deepali Garg, Mahima Rajan, Jaya Shankar Kaushik, Geeta Gathwala
Nephrotic syndrome is a prothrombotic state with predisposition to venous sinus thrombosis and arterial vascular stroke. Watershed infarcts in junction of arterial territory develop in hypotensive hypovolemic state. These border zone infarcts are usually bilateral in the absence of unilateral arterial stenosis or microembolism. We report a 6-year-girl of frequently relapsing nephrotic syndrome who developed sudden onset hemiparesis with aphasia. Magnetic resonance (MR) imaging brain revealed unilateral watershed infarct in territory between the major cerebral arterial vessels with evidence of restricted diffusion and normal vessel anatomy on MR angiography...
January 2018: Journal of Natural Science, Biology, and Medicine
https://www.readbyqxmd.com/read/29456220/importance-of-renal-biopsy-in-patients-aged-60-years-and-older-experience-from-a-tertiary-care-hospital
#4
Pallav Gupta, Devinder Singh Rana
As the life expectancy is increasing, there is a rise in elderly population and consequent increase in the patients with renal disease. There is an inconsistency between clinical and histopathological diagnosis in elderly, and so renal biopsy is important in these patients to decide appropriate clinical management and prognosis. This study outlines the importance of renal biopsy in elderly and describes the clinical and pathologic spectrum of renal diseases in patient ≥60 years. All patients (age ≥60 years) undergoing renal biopsies from January 2011 to December 2014 were included in this retrospective study...
January 2018: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/29446731/vitamin-d-receptor-a-novel-therapeutic-target-for-kidney-diseases
#5
Shikun Yang, Aimei Li, JianWen Wang, Jun Liu, Yachun Han, Wei Zhang, Yan Chun Li, Hao Zhang
BACKGROUND: Kidney disease is a serious problem that adversely affects human health, but critical knowledge is lacking on how to effectively treat established chronic kidney disease. Mounting evidence from animal and clinical studies has suggested that vitamin D receptor (VDR) activation has beneficial effects on various renal diseases. METHOD: A structured search of published research literature regarding VDR structure and function, VDR in various renal diseases (e...
February 14, 2018: Current Medicinal Chemistry
https://www.readbyqxmd.com/read/29446486/interleukin-17a-participates-in-podocyte-injury-by-inducing-il-1%C3%AE-secretion-through-ros-nlrp3-inflammasome-caspase-1-pathway
#6
Junli Yan, Yao Li, Haiping Yang, Li Zhang, Baohui Yang, Mo Wang, Qiu Li
Studies show that the Th17/IL-17A axis plays an important role in the pathogenesis of kidney diseases. Previously, we also showed that IL-17A may play a role in the pathogenesis of primary nephrotic syndrome; however, the underlying mechanism(s) is unclear. The aim of this study was to explore the molecular mechanism of IL-17A inducing podocyte injury in vitro. In this study, the NLRP3 inflammsome activation and the morphology of podocytes were detected by western blot and immunofluorescence .The results showed that podocytes persistently expressed IL-17A receptor, and that NLRP3 inflammasome in these cells was activated upon exposure to IL-17A...
February 15, 2018: Scandinavian Journal of Immunology
https://www.readbyqxmd.com/read/29445323/urine-proteomics-of-primary-membranous-nephropathy-using-nanoscale-liquid-chromatography-tandem-mass-spectrometry-analysis
#7
Lu Pang, Qianqian Li, Yan Li, Yi Liu, Nan Duan, Haixia Li
Background: Primary membranous nephropathy (PMN) is an important cause of nephrotic syndrome in adults. Urine proteome may provide important clues of pathophysiological mechanisms in PMN. In the current study, we analyzed and compared the proteome of urine from patients with PMN and normal controls. Methods: We performed two technical replicates (TMT1 and TMT2) to analyze and compare the urine proteome from patients with PMN and normal controls by tandem mass tag (TMT) technology coupled with nanoscale liquid chromatography tandem mass spectrometry analysis (LC-MS/MS)...
2018: Clinical Proteomics
https://www.readbyqxmd.com/read/29441828/fluoride-associated-ultrastructural-changes-and-apoptosis-in-human-renal-tubule-a-pilot-study
#8
J A Quadri, S Sarwar, A Sinha, M Kalaivani, A K Dinda, A Bagga, T S Roy, T K Das, A Shariff
The susceptibility of the kidneys to fluoride toxicity can largely be attributed to its anatomy and function. As the filtrate moves along the complex tubular structure of each nephron, it is concentrated in the proximal and distal tubules and collecting duct. It has been frequently observed that the children suffering from renal impairments also have some symptoms of dental and skeletal fluorosis. The findings suggest that fluoride somehow interferes with renal anatomy and physiology, which may lead to renal pathogenesis...
January 1, 2018: Human & Experimental Toxicology
https://www.readbyqxmd.com/read/29441190/nephrotic-syndrome-in-very-elderly-should-we-treat-aggressively
#9
Abhilash Koratala, Jogiraju Tantravahi
Chronic kidney disease and acute kidney injury are being increasingly recognized in very elderly patients, aged 80 or more. In cases of suspected glomerulonephritis with or without nephrotic syndrome, the clinical decision-making of whether to obtain a renal biopsy and treat with immunosuppressive therapy should not be based on advanced age alone but take into consideration the patient's functional status and overall prognosis. Herein, we report a case of an elderly patient with minimal change disease who benefitted from a timely renal biopsy and aggressive immunosuppressive therapy...
January 8, 2018: Clinics and Practice
https://www.readbyqxmd.com/read/29436729/adverse-events-linked-with-the-use-of-chimeric-and-humanized-anti-cd20-antibodies-in-children-with-idiopathic-nephrotic-syndrome
#10
Alice Bonanni, Marta Calatroni, Matteo D'Alessandro, Sara Signa, Enrica Bertelli, Michela Cioni, Eddi Di Marco, Roberto Biassoni, Gianluca Caridi, Giulia Ingrasciotta, Roberta Bertelli, Armando Di Donato, Maurizio Bruschi, Alberto Canepa, Giorgio Piaggio, Pietro Ravani, Gian Marco Ghiggeri
AIMS: Anti-CD20 antibodies are increasingly being used to treat idiopathic nephrotic syndrome (INS) in children. While they may allow steroid and calcineurin-inhibitor withdrawal, repeated infusions of anti-CD20 antibodies are often required to maintain remission. Data on their potential toxicity in INS are needed to consider repeated infusions. METHODS: We reported data on side effects related to the use of rituximab (a chimeric anti-body; 130 pts) and ofatumumab (a humanized anti-body; 37 pts) in children with INS (steroid-dependent and steroid/calcineurin-inhibitor dependent disease) treated in a national referral center during a 9-year period (400 treatments; follow-up 1-9 years)...
February 13, 2018: British Journal of Clinical Pharmacology
https://www.readbyqxmd.com/read/29436096/nephrotic-syndrome-complicated-with-femoral-vein-catheter-related-phlegmasia-cerulea-dolens-a-case-report
#11
Wen-Chun Chen, Shuo-Ming Ou
Nephrotic syndrome is a well-known risk factor of deep vein thrombosis (DVT). Catheter-related DVT under the setting of nephrotic syndrome may be presented as a more fulminant form, phlegmasia cerulea dolens. Phlegmasia cerulea dolens may lead to severe obstruction of venous drainage of the extremities and presents with compartment syndrome that impairs arterial perfusion. Aggressive management with thrombolysis and/or thrombectomy are considered with simultaneous anticoagulant treatment.
February 13, 2018: Hemodialysis International
https://www.readbyqxmd.com/read/29433915/a-novel-assay-provides-sensitive-measurement-of-physiologically-relevant-changes-in-albumin-permeability-in-isolated-human-and-rodent-glomeruli
#12
Sara Desideri, Karen L Onions, Yan Qiu, Raina D Ramnath, Matthew J Butler, Christopher R Neal, Matthew L R King, Andrew E Salmon, Moin A Saleem, Gavin I Welsh, C Charles Michel, Simon C Satchell, Andrew H J Salmon, Rebecca R Foster
Increased urinary albumin excretion is a key feature of glomerular disease but has limitations as a measure of glomerular permeability. Here we describe a novel assay to measure the apparent albumin permeability of single capillaries in glomeruli, isolated from perfused kidneys cleared of red blood cells. The rate of decline of the albumin concentration within the capillary lumen was quantified using confocal microscopy and used to calculate apparent permeability. The assay was extensively validated and provided robust, reproducible estimates of glomerular albumin permeability...
February 9, 2018: Kidney International
https://www.readbyqxmd.com/read/29429831/predictors-of-poor-outcome-in-children-with-steroid-sensitive-nephrotic-syndrome
#13
Kirtisudha Mishra, Sandeep Kumar Kanwal, Sushma Veeranna Sajjan, Vikram Bhaskar, Bimbadhar Rath
BACKGROUND AND OBJECTIVE: Nearly 50% of the children with steroid sensitive nephrotic syndrome (SSNS) have a frequently relapsing (FR) or steroid dependent (SD) course, experiencing steroid toxicities and complications of immunosuppression. The study aimed to compare parameters between children with infrequent relapsing (IFR) and FR/SD nephrotic syndrome and to identify the factors associated with a FR/SD course. METHODS: A retrospective analysis of medical records from 2009 to 2014, of children with SSNS attending the pediatric nephrology clinic in a tertiary care medical college and hospital...
February 8, 2018: Nefrología: Publicación Oficial de la Sociedad Española Nefrologia
https://www.readbyqxmd.com/read/29429752/patterns-of-clinical-response-to-eculizumab-in-patients-with-c3-glomerulopathy
#14
Moglie Le Quintrec, Anne-Laure Lapeyraque, Arnaud Lionet, Anne-Laure Sellier-Leclerc, Yahsou Delmas, Véronique Baudouin, Eric Daugas, Stéphane Decramer, Leila Tricot, Mathilde Cailliez, Philippe Dubot, Aude Servais, Catherine Mourey-Epron, Franck Pourcine, Chantal Loirat, Véronique Frémeaux-Bacchi, Fadi Fakhouri
BACKGROUND: Cases reports and small series of patients with C3 glomerulopathy have reported variable efficacy of eculizumab. STUDY DESIGN: Case series of C3 glomerulopathy. SETTING & PARTICIPANTS: Pediatric and adult patients with C3 glomerulopathy treated with eculizumab between 2010 and 2016 were identified through the C3 glomerulopathy French registry database, and a questionnaire was sent to participating French pediatric and adult nephrology centers, as well as one pediatric referral center in Québec, Canada...
February 8, 2018: American Journal of Kidney Diseases: the Official Journal of the National Kidney Foundation
https://www.readbyqxmd.com/read/29426974/infusion-reactions-associated-with-rituximab-treatment-for-childhood-onset-complicated-nephrotic-syndrome
#15
Koichi Kamei, Masao Ogura, Mai Sato, Shuichi Ito, Kenji Ishikura
BACKGROUND: Infusion reaction (IR) is defined as an adverse event within 24 h after monoclonal antibody infusion. In non-Hodgkin lymphoma, IR incidence following rituximab treatment is high (77-80%), but there are no data in complicated nephrotic syndrome. METHODS: Records of rituximab infusions in patients with complicated nephrotic syndrome between February 2006 and December 2014 at the National Center for Child Health and Development were reviewed. Rituximab was administered at doses of 375 mg/m2...
February 9, 2018: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29424201/hereditary-hyperhomocysteinemia-associated-with-nephrotic-syndrome-complicated-by-artery-thrombosis-and-chronic-thromboembolic-pulmonary-hypertension-a-case-report
#16
Paolo Ruggeri, Federica Lo Bello, Francesco Nucera, Michele Gaeta, Francesco Monaco, Gaetano Caramori, Giuseppe Girbino
We present here the case of a 30-year-old man with a long term history of nephrotic syndrome (NS) who developed an episode of acute left main pulmonary artery thrombosis complicated by a lung abscess. During the hospital admission was also identified a concomitant hyperhomocysteinemia. After an atypical resection of the left upper pulmonary lobe and the starting of long term anticoagulation the patient was discharged but did not attend the planned follow up visits until one year later when he was seen again for severe dyspnea and exercise intolerance...
December 19, 2017: Monaldi Archives for Chest Disease, Archivio Monaldi Per le Malattie del Torace
https://www.readbyqxmd.com/read/29423203/causes-and-predictors-of-mortality-in-biopsy-proven-lupus-nephritis-the-sarawak-experience
#17
Cheng Lay Teh, Vui Eng Phui, Guo Ruey Ling, Lui-Sian Ngu, Sharifah Aishah Wan, Clare Hui-Hong Tan
Background: Lupus nephritis (LN) is a serious manifestation of systemic lupus erythematosus that can be fatal if left untreated. The causes and prognostic predictors of mortality in LN have been well studied in developed countries but evidence is lacking for developing countries. The objective of this study was to investigate the causes and predictors of mortality in a cohort of Malaysian patients with biopsy-proven LN. Methods: We retrospectively studied all patients with biopsy-proven LN treated in Sarawak General Hospital during the period of 2000-15...
February 2018: Clinical Kidney Journal
https://www.readbyqxmd.com/read/29423202/azithromycin-suppressed-relapses-of-idiopathic-nephrotic-syndrome-in-a-child
#18
Hiromichi Hara, Daishi Hirano
Long-term immunosuppressive therapy with severe adverse effects is indispensable to maintain disease remission in frequently relapsing nephrotic syndrome (NS) in children. Hence, development of new therapy with less toxicity for relapses of NS is required. We demonstrated a case of a 2-year-old boy with frequently relapsing NS, whose frequent relapses were successfully treated with azithromycin. Azithromycin treatment prevented the need for long-term immunosuppressive therapy in this case. Azithromycin could be a new treatment option for relapse of NS, with few adverse effects, in selected cases...
February 2018: Clinical Kidney Journal
https://www.readbyqxmd.com/read/29421774/efficacy-and-safety-of-rituximab-in-children-with-steroid-and-cyclosporine-resistant-and-steroid-and-cyclosporine-dependent-nephrotic-syndrome
#19
Rozita Hoseini, Kamran Sabzian, Hasan Otukesh, Nazanin Zafaranloo, Parsa Panahi, Nahid Rahimzadeh, Shahrbanoo Nakhaie, Mohsen Akhavan Sepehi
INTRODUCTION: There is evidence of the effectiveness of rituximab in treatment of nephrotic syndrome in children. The present study aimed to assess safety and the therapeutic effectiveness of rituximab in steroid- and cyclosporine-resistant pediatric nephrotic syndrome. MATERIALS AND METHODS: Forty-three children with steroid- and cyclosporine-resistant or steroid- and cyclosporine-dependent noncongenital nephrotic syndrome were included in the study to receive intravenous rituximab, 375 mg/m2/wk, for 4 weeks...
January 2018: Iranian Journal of Kidney Diseases
https://www.readbyqxmd.com/read/29416285/a-rare-case-of-renal-vein-thrombosis-secondary-to-klebsiella-pneumoniae-pyelonephritis
#20
Kelly L Lurz, Dany N Hanna, Brian H McGreen, Francis J Schanne
Renal vein thrombosis (RVT) is most often an implication of nephrotic syndrome. Pyelonephritis has been associated at a much lower rate, with the incidence of Klebsiella pneumoniae causation being extremely rare. In our case, a 35-year-old female patient presented with right-sided K. pneumoniae-positive acute pyelonephritis complicated by perinephric abscess and renal vein thrombosis. She was successfully treated with anticoagulation and extended antibiotic therapy. The possibility of RVT in patients with K...
January 2018: Urology Annals
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