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Nephrotic Syndrome

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1. Nephrotic syndrome

Nephrotic syndrome Evidence Maps - Trip Database or use your Google+ account Turning Research Into Practice ALL of these words: Title only Anywhere in the document ANY of these words: Title only Anywhere in the document This EXACT phrase: Title only Anywhere in the document EXCLUDING words: Title only Anywhere in the document Timeframe: to: Combine searches by placing the search numbers in the top search box and pressing the search button. An example search might look like (#1 or #2) and (#3

2018 Trip Evidence Maps

4. Assessment of nephrotic syndrome

Assessment of nephrotic syndrome Assessment of nephrotic syndrome - Differential diagnosis of symptoms | BMJ Best Practice You'll need a subscription to access all of BMJ Best Practice Search  Assessment of nephrotic syndrome Last reviewed: February 2019 Last updated: June 2018 Summary Nephrotic syndrome is defined as the presence of proteinuria (>3.5 g/24 hours), hypoalbuminaemia (<30 g/L), and peripheral oedema. Hyperlipidaemia and thrombotic disease are also frequently seen. Despite heavy (...) proteinuria and lipiduria, the urine contains few cells or casts. This is in contrast to nephritic syndrome, which is typically defined as the presence of acute kidney injury (renal dysfunction), hypertension, and an active urinary sediment (red cells and red cell casts). Patients presenting with isolated heavy proteinuria without the other components of nephrotic syndrome are described as having nephrotic-range proteinuria and is usually indicative of an underlying glomerulopathy. In this case

2018 BMJ Best Practice

5. Sodium retention and edema in nephrotic syndrome: new models and therapeutic targets

Sodium retention and edema in nephrotic syndrome: new models and therapeutic targets Sodium Retention and Edema in Nephrotic Syndrome: New Models and Therapeutic Targets – Clinical Correlations Search Sodium Retention and Edema in Nephrotic Syndrome: New Models and Therapeutic Targets July 23, 2019 4 min read By Maria Smith Peer Reviewed The mechanism of sodium retention and edema formation in patients with nephrotic syndrome has long been highly contested in the nephrology world (...) and edema in nephrotic syndrome. Recently, studies have found evidence for an alternate model for 6 proposed a mechanism of renal-aberrant response to nephrotic urine, based on their study of nephrotic rats. They detected increased levels of plasminogen filtered into nephrotic urine and demonstrated that the plasminogen is activated into plasmin in the renal tubules by a protein called urokinase-type plasminogen activator (uPA). They found that plasmin then proteolytically cleaves and activates

2019 Clinical Correlations

6. Prevalence of Asthma and Allergies and Risk of Relapse in Childhood Nephrotic Syndrome: Insight into Nephrotic Syndrome Cohort. (PubMed)

Prevalence of Asthma and Allergies and Risk of Relapse in Childhood Nephrotic Syndrome: Insight into Nephrotic Syndrome Cohort. To determine the lifetime prevalence of allergies in childhood nephrotic syndrome, the seasonality of presentation and relapses, and the impact of allergies on subsequent relapses.In a longitudinal cohort of children with nephrotic syndrome (ages 1-18 years), assessment for allergic diseases was conducted using the validated and modified version of the International (...) Study of Asthma and Allergies in Childhood questionnaire at enrollment. Outcomes included frequently relapsing nephrotic syndrome, relapse rates, and the relapse-free duration after initial steroid therapy.Among 277 participants, the majority were male (65%) with a median age of 3.7 years (IQR 2.8-5.8) at presentation. A total of 64% reported lifetime allergies with 20% having asthma, 33% wheezing, 27% eczema, and 24% rhinitis. Over 3.3 years of follow-up, presence of asthma and allergies

2019 Journal of Pediatrics

7. Omega-3 Fatty Acids for Proteinuria due to Nephrotic Syndrome: A Review of Clinical Effectiveness and Cost-Effectiveness

Omega-3 Fatty Acids for Proteinuria due to Nephrotic Syndrome: A Review of Clinical Effectiveness and Cost-Effectiveness Omega-3 Fatty Acids for Proteinuria due to Nephrotic Syndrome: A Review of Clinical Effectiveness and Cost-Effectiveness | CADTH.ca Find the information you need Omega-3 Fatty Acids for Proteinuria due to Nephrotic Syndrome: A Review of Clinical Effectiveness and Cost-Effectiveness Omega-3 Fatty Acids for Proteinuria due to Nephrotic Syndrome: A Review of Clinical (...) Effectiveness and Cost-Effectiveness Published on: February 5, 2016 Project Number: RC0741-000 Product Line: Research Type: Drug Report Type: Summary with Critical Appraisal Result type: Report Question What is the clinical effectiveness of omega-3 fatty acid supplementation for reduction of proteinuria in patients with nephrotic syndrome? What is the cost-effectiveness of omega-3 fatty acid supplementation for reduction of proteinuria in patients with nephrotic syndrome? Key Message Four systematic reviews

2016 Canadian Agency for Drugs and Technologies in Health - Rapid Review

8. Study protocol: mycophenolate mofetil as maintenance therapy after rituximab treatment for childhood-onset, complicated, frequently-relapsing nephrotic syndrome or steroid-dependent nephrotic syndrome: a multicenter double-blind, randomized, placebo-contr (PubMed)

Study protocol: mycophenolate mofetil as maintenance therapy after rituximab treatment for childhood-onset, complicated, frequently-relapsing nephrotic syndrome or steroid-dependent nephrotic syndrome: a multicenter double-blind, randomized, placebo-contr Idiopathic nephrotic syndrome (INS) is the most common chronic glomerular disease in children. Approximately 80-90% of patients with childhood INS have steroid-sensitive nephrotic syndrome (SSNS), and can obtain remission with steroid therapy (...) , while the remainder have steroid-resistant nephrotic syndrome (SRNS). Furthermore, approximately 50% of children with SSNS develop frequently-relapsing nephrotic syndrome (FRNS) or steroid-dependent nephrotic syndrome (SDNS). Children with FRNS/SDNS are usually treated with immunosuppressive agents such as cyclosporine, cyclophosphamide, or mizoribine in Japan. However, 10-20% of children receiving immunosuppressive agents still show frequent relapse and/or steroid dependence during or after

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2018 BMC Nephrology

9. Cost-Effectiveness of Administering Rituximab for Steroid-Dependent Nephrotic Syndrome and Frequently Relapsing Nephrotic Syndrome: A Preliminary Study in Japan (PubMed)

Cost-Effectiveness of Administering Rituximab for Steroid-Dependent Nephrotic Syndrome and Frequently Relapsing Nephrotic Syndrome: A Preliminary Study in Japan With regard to the use of rituximab for patients with steroid-dependent nephrotic syndrome and frequently relapsing nephrotic syndrome, not only has the regimen not been clinically verified but also there is a lack of health economics evidence. Therefore, we conducted a prospective clinical study on 30 patients before (with steroids

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2017 Scientific reports

10. Learning to live with nephrotic syndrome: experiences of adult patients and parents of children with nephrotic syndrome (PubMed)

Learning to live with nephrotic syndrome: experiences of adult patients and parents of children with nephrotic syndrome People living with nephrotic syndrome (NS) need to develop an in-depth understanding of their condition in order to participate in treatment decisions, develop self-management skills and integrate illness into daily life. However, the learning needs of adult patients and parents of children with NS are unknown. We therefore explored patient and parent perspectives on learning

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2017 Nephrology Dialysis Transplantation

11. NPHS2 V260E Is a Frequent Cause of Steroid-Resistant Nephrotic Syndrome in Black South African Children (PubMed)

NPHS2 V260E Is a Frequent Cause of Steroid-Resistant Nephrotic Syndrome in Black South African Children In South Africa (SA), steroid-resistant nephrotic syndrome (SRNS) is more frequent in black than in Indian children.Seeking a genetic basis for this disparity, we enrolled 33 Indian and 31 black children with steroid-sensitive nephrotic syndrome (SSNS) and SRNS from KwaZulu-Natal, SA; SRNS children underwent kidney biopsy. We sequenced NPHS2 and genotyped APOL1 in 15 SSNS and 64 SRNS

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2018 Kidney international reports

12. Parental Whole-Exome Sequencing Enables Sialidosis Type II Diagnosis due to an NEU1 Missense Mutation as an Underlying Cause of Nephrotic Syndrome in the Child (PubMed)

Parental Whole-Exome Sequencing Enables Sialidosis Type II Diagnosis due to an NEU1 Missense Mutation as an Underlying Cause of Nephrotic Syndrome in the Child Monogenetic renal diseases, including recessively inherited nephrotic syndromes, represent a significant health burden despite being rare conditions. Precise diagnosis, including identification of the underlying molecular cause, is especially difficult in low-income countries and/or if affected individuals are unavailable for biochemical (...) testing. Whole-exome sequencing (WES) has opened up novel diagnostic perspectives for these settings. However, sometimes the DNA of affected individuals is not suitable for WES due to low amounts or degradation.We report on the use of parental WES with implementation of specific stepwise variant filtering to identify the underlying molecular cause of the childhood-onset nephrotic syndrome as nephrosialidosis resulting from a mutation in NEU1.Sequencing both parents enabled a nephrosialidosis diagnosis

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2018 Kidney international reports

13. Direct-Acting Oral Anticoagulants as Prophylaxis Against Thromboembolism in the Nephrotic Syndrome (PubMed)

Direct-Acting Oral Anticoagulants as Prophylaxis Against Thromboembolism in the Nephrotic Syndrome We report 2 cases of apixaban use as prophylaxis against thromboembolism in the nephrotic syndrome (NS), and review the existing literature on direct-acting oral anticoagulant (DOAC) use in this scenario. Our cases appear to be the first reported use of apixaban as prophylaxis against thromboembolism in NS. We report our systematic review of the existing literature on direct-acting oral

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2018 Kidney international reports

14. Effect of Combined Gluten-Free, Dairy-Free Diet in Children With Steroid-Resistant Nephrotic Syndrome: An Open Pilot Trial (PubMed)

Effect of Combined Gluten-Free, Dairy-Free Diet in Children With Steroid-Resistant Nephrotic Syndrome: An Open Pilot Trial Steroid-resistant nephrotic syndrome (SRNS) affects both children and adults and has a high rate of progression to end-stage renal disease. Although a subset of patients have well-characterized genetic mutation(s), in the majority of cases, the etiology is unknown. Over the past 50 years, a number of case reports have suggested the potential impact of dietary changes (...) in controlling primary nephrotic syndrome, especially gluten and dairy restrictions.We have designed a prospective, open-label, nonrandomized, pilot clinical trial, to study the effect of a gluten-free and dairy-free (GF/DF) diet in children with SRNS. The study will be organized as a 4-week summer camp to implement a GF/DF diet in a tightly controlled and monitored setting. Blood, urine, and stool samples will be collected at different time points during the study.The primary end point is a reduction

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2018 Kidney international reports

15. Rituximab in The Management of Pediatric Steroid-Resistant Nephrotic Syndrome: A Systematic Review

Rituximab in The Management of Pediatric Steroid-Resistant Nephrotic Syndrome: A Systematic Review To evaluate the efficacy and safety of rituximab in children with steroid-resistant nephrotic syndrome.A systematic review evaluating the efficacy and safety of rituximab in children with steroid-resistant nephrotic syndrome was performed. Data from studies, performed before April 2017 were collected, from MEDLINE, Cochrane Library, Scopus, and Web of Science. Study eligibility criteria included (...) clinical trials and observational studies with a minimal sample size of 5 patients, regarding treatment with rituximab in children with steroid-resistant nephrotic syndrome. Independent extraction of articles by 2 investigators using predefined data fields was performed.We included 7 case series and 1 open-label randomized controlled trial. Among them, 3 studies were multicenter. A total of 226 patients were included. Mean age at onset was 5.6 ± 1.1 years. Mean number of rituximab administrations

2018 EvidenceUpdates

16. Efficacy of Rituximab vs Tacrolimus in Pediatric Corticosteroid-Dependent Nephrotic Syndrome: A Randomized Clinical Trial

Efficacy of Rituximab vs Tacrolimus in Pediatric Corticosteroid-Dependent Nephrotic Syndrome: A Randomized Clinical Trial Calcineurin inhibitors are an established first-line corticosteroid-sparing therapy for patients with corticosteroid-dependent nephrotic syndrome (CDNS), whereas B-lymphocyte-depleting therapy is mostly used as a rescue for calcineurin inhibitor-resistant cases. The positive efficacy and safety profile of rituximab raises the question of whether it could be used as a first

2018 EvidenceUpdates

17. Idiopathic nephrotic syndrome in children. (PubMed)

Idiopathic nephrotic syndrome in children. The incidence of idiopathic nephrotic syndrome (NS) is 1·15-16·9 per 100 000 children, varying by ethnicity and region. The cause remains unknown but the pathogenesis of idiopathic NS is thought to involve immune dysregulation, systemic circulating factors, or inherited structural abnormalities of the podocyte. Genetic risk is more commonly described among children with steroid-resistant disease. The mainstay of therapy is prednisone for the vast

2018 Lancet

18. Low molecular weight heparin may benefit nephrotic remission in steroid-sensitive nephrotic syndrome via inhibiting elastase (PubMed)

Low molecular weight heparin may benefit nephrotic remission in steroid-sensitive nephrotic syndrome via inhibiting elastase Low molecular weight heparin (LMWH) has a structure similar to heparan sulfate, which exerts anti‑inflammatory effects via inhibiting elastase (Ela) activity. Release of Ela along the glomerular capillary wall may induce glomerular injury and proteinuria. The present study aimed to investigate the influence of LMWH on steroid‑sensitive nephrotic syndrome (SSNS (...) ) and the potential underlying mechanism. A total of 40 SSNS patients and 20 healthy controls were recruited. SSNS patients were treated with LMWH and prednisone simultaneously (LMWH+pred group) or with prednisone alone (pred group). Proteinuria, urinary glycosaminoglycans (GAGs), serum Ela and urinary creatinine levels were measured. The nephrotic period of SSNS was 15.93±5.78 days. The nephrotic period of SSNS in LMWH+pred group was significantly reduced compared with the pred group (14.13±4.56 vs. 18.63±6.49

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2017 Molecular medicine reports

19. NephCure Accelerating Cures Institute: A Multidisciplinary Consortium to Improve Care for Nephrotic Syndrome (PubMed)

NephCure Accelerating Cures Institute: A Multidisciplinary Consortium to Improve Care for Nephrotic Syndrome NephCure Accelerating Cures Institute (NACI) is a collaborative organization sponsored by NephCure Kidney International and the University of Michigan. The Institute is composed of 7 cores designed to improve treatment options and outcomes for patients with glomerular disease: Clinical Trials Network, Data Warehouse, Patient-Reported Outcomes (PRO) and Endpoints Consortium, Clinical (...) Trials Consulting Team, Quality Initiatives, Education and Engagement, and Data Coordinating Center.The Trials Network includes 22 community- and hospital-based nephrology practices, 14 of which are trial-only sites. Eight sites participate in the NACI Registry, and as of October 2017, 1054 patients are enrolled with diagnoses including but not limited to focal segmental glomerulosclerosis, minimal change disease, membranous nephropathy, IgA nephropathy, and childhood-onset nephrotic syndrome

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2017 Kidney international reports

20. Immunoglobulin G and M levels in childhood nephrotic syndrome: two centers Egyptian study (PubMed)

Immunoglobulin G and M levels in childhood nephrotic syndrome: two centers Egyptian study Idiopathic nephrotic syndrome (INS) is the most common glomerular disease in children. Immune cell subsets may play a role in pathogenesis of INS. We aimed to assess immunoglobulin G (IgG) and immunoglobulin M (IgM) levels in children with nephrotic syndrome (NS) to predict prognosis of the disease and response to treatment.This prospective case control study was done in Pediatric Nephrology Units

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2017 Electronic physician

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