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nephrotic syndrome review

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https://www.readbyqxmd.com/read/28427453/the-italian-society-for-pediatric-nephrology-sinepe-consensus-document-on-the-management-of-nephrotic-syndrome-in-children-part-i-diagnosis-and-treatment-of-the-first-episode-and-the-first-relapse
#1
REVIEW
Andrea Pasini, Elisa Benetti, Giovanni Conti, Luciana Ghio, Marta Lepore, Laura Massella, Daniela Molino, Licia Peruzzi, Francesco Emma, Carmelo Fede, Antonella Trivelli, Silvio Maringhini, Marco Materassi, Giovanni Messina, Giovanni Montini, Luisa Murer, Carmine Pecoraro, Marco Pennesi
This consensus document is aimed at providing an updated, multidisciplinary overview on the diagnosis and treatment of pediatric nephrotic syndrome (NS) at first presentation. It is the first consensus document of its kind to be produced by all the pediatric nephrology centres in Italy, in line with what is already present in other countries such as France, Germany and the USA. It is based on the current knowledge surrounding the symptomatic and steroid treatment of NS, with a view to providing the basis for a separate consensus document on the treatment of relapses...
April 21, 2017: Italian Journal of Pediatrics
https://www.readbyqxmd.com/read/28392820/genetics-of-hereditary-nephrotic-syndrome-a-clinical-review
#2
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/28391338/what-are-we-missing-in-the-clinical-trials-of-focal-segmental-glomerulosclerosis
#3
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/28391337/managing-glomerular-disease-in-pregnancy
#4
Michelle A Hladunewich, Kate Bramham, Belinda Jim, Sharon Maynard
Glomerular disease, especially lupus nephritis, is common in young women of childbearing age. As such, practicing nephrologists must have adequate knowledge and expertise to prepare these women for pregnancy, to diagnose and manage the numerous pregnancy-associated complications that are common in this vulnerable patient population and finally to support these young women who often struggle to manage both their young children along with an often onerous chronic disease. In this article we will review the pre-pregnancy counseling these women should ideally receive to allow them to make an informed decision about proceeding with a pregnancy, an approach to the diagnosis of pregnancy-associated worsening of kidney function and proteinuria as well as pregnancy-safe management strategies, including immunosuppression, antihypertensive agents and other medications necessary for the management of nephrotic syndrome...
January 1, 2017: Nephrology, Dialysis, Transplantation
https://www.readbyqxmd.com/read/28378029/nephrotoxicity-in-children-with-frequently-relapsing-nephrotic-syndrome-receiving-long-term-cyclosporine-treatment
#5
Yuko Hamasaki, Fumiyo Komaki, Kenji Ishikura, Riku Hamada, Tomoyuki Sakai, Hiroshi Hataya, Kentaro Ogata, Takashi Ando, Masataka Honda
BACKGROUND: Steroid-sparing drugs, such as cyclosporine, are recommended as treatment for children with frequently relapsing nephrotic syndrome (FRNS) and steroid-related toxicities. We recently reported a high rate of relapsing nephrotic syndrome 2 years after discontinuation of cyclosporine treatment, suggesting that long-term treatment is necessary. Cyclosporine-associated nephrotoxicity (CAN) is a potential side effect of long-term cyclosporine treatment. METHODS: We retrospectively reviewed pediatric patients with FRNS treated with cyclosporine for ≥3 years at a single center between 1999 and 2012...
April 4, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28348914/brincidofovir-use-after-foscarnet-crystal-nephropathy-in-a-kidney-transplant-recipient-with-multiresistant-cytomegalovirus-infection
#6
Romain Vial, Christine Zandotti, Sophie Alain, Alexandre Decourt, Noémie Jourde-Chiche, Raj Purgus, Charleric Bornet, Laurent Daniel, Valérie Moal, Tristan Legris
Background. Cytomegalovirus (CMV) antiviral drug resistance constitutes an increasing challenge in transplantation. Foscarnet is usually proposed when resistance for ganciclovir is suspected, but its use is limited by its nephrotoxicity. Case Presentation. We report a case of multiresistant CMV disease in a kidney transplant recipient. Foscarnet was prescribed after ganciclovir treatment failure in a patient with two mutations in the UL97 viral gene. Foscarnet induced biopsy-proven kidney crystal precipitation that resulted in severe acute transplant failure and nephrotic syndrome...
2017: Case Reports in Transplantation
https://www.readbyqxmd.com/read/28341877/short-courses-of-daily-prednisolone-during-upper-respiratory-tract-infections-reduce-relapse-frequency-in-childhood-nephrotic-syndrome
#7
Asiri S Abeyagunawardena, R S Thalgahagoda, Pathum V Dissanayake, Shamali Abeyagunawardena, Y A Illangasekera, Umeshi I Karunadasa, Richard S Trompeter
BACKGROUND: Relapses of childhood nephrotic syndrome (NS) are frequently precipitated by viral upper respiratory tract infections (URTIs). A review of the literature reveals that in patients with steroid-dependent NS on alternate day corticosteroids, a short course of daily corticosteroid therapy during the course of an URTI may reduce relapse frequency. OBJECTIVE: To assess the effect of a short course of low-dose corticosteroid therapy during the course of an URTI on relapse frequency in patients with steroid-sensitive NS who have not been taking any treatment for a minimum period of 3 months...
March 24, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28321320/calcineurin-inhibitors-in-the-treatment-of-primary-focal-segmental-glomerulosclerosis-a-systematic-review-and-meta-analysis-of-the-literature
#8
REVIEW
Louis-Philippe Laurin, Patrick H Nachman, Bethany J Foster
PURPOSE OF REVIEW: Primary focal segmental glomerulosclerosis (FSGS) is the most common cause of nephrotic syndrome in adults. Glucocorticoids have been evaluated in the treatment of primary FSGS in numerous retrospective studies. Evidence suggesting a role for including calcineurin inhibitors (CNIs) in early therapy remains limited. The aim of this study was to systematically review the literature examining the efficacy of CNIs in the treatment of primary FSGS both as first-line therapy and as an adjunctive agent in steroid-resistant patients, with respect to remission in proteinuria and renal survival...
2017: Canadian Journal of Kidney Health and Disease
https://www.readbyqxmd.com/read/28296375/-endomyocardial-fibrosis-associated-to-antiphospholipid-syndrome-case-report-and-review
#9
Carlos A Parquet, Jorge Curotto-Grasiosi, Rogelio A Machado, Bruno Peressotti, Matías B Padilla, Alberto L Bolaño
A 29-year-old man with a history of seizures, was admitted due to an episode of unconsciousness recovered and hypertension with renal disfunction. The electrocardiogram mimicked a hypertrophic cardiomyopathy, but, by Doppler echocardiography, this was discarded because it suggested endomyocardial fibrosis which was confirmed by cardiac magnetic resonance imaging with late enhancement. Since the episode of unconsciousness, brain imaging studies were performed showing vascular sequelae and microangiopathic lesions...
March 2017: Revista Médica del Instituto Mexicano del Seguro Social
https://www.readbyqxmd.com/read/28291548/the-role-of-interleukin-17a-in-the-pathogenesis-of-kidney-diseases
#10
REVIEW
Charlotte Cortvrindt, Reinhart Speeckaert, Alena Moerman, Joris R Delanghe, Marijn M Speeckaert
Being a member of the IL-17 family, comprising six structurally related ligands, IL-17A is a cytokine, produced by multiple cell types, such as CD4(+)αβ T cells, γδ T cells, natural killer cells, neutrophils, macrophages, dendritic cells, lymphoid tissue inducer cells, mast cells and plasma cells. IL-17A participates in tissue inflammation by inducing the expression of chemokines, proinflammatory cytokines and matrix metalloproteases. Besides its role in host defence against infectious diseases, IL-17A is involved in different autoimmune and inflammatory diseases...
April 2017: Pathology
https://www.readbyqxmd.com/read/28286744/endocytic-trafficking-at-the-mature-podocyte-slit-diaphragm
#11
REVIEW
Agnieszka Swiatecka-Urban
Endocytic trafficking couples cell signaling with the cytoskeletal dynamics by organizing a crosstalk between protein networks in different subcellular compartments. Proteins residing in the plasma membrane are internalized and transported as cargo in endocytic vesicles (i.e., endocytosis). Subsequently, cargo proteins can be delivered to lysosomes for degradation or recycled back to the plasma membrane. The slit diaphragm is a modified tight junction connecting foot processes of the glomerular epithelial cells, podocytes...
2017: Frontiers in Pediatrics
https://www.readbyqxmd.com/read/28258791/kidney-disease-and-venous-thromboembolism-does-being-woman-make-the-difference
#12
REVIEW
A Gigante, F Di Mario, A Pierucci, A Amoroso, F S Pignataro, L Napoleone, S Basili, V Raparelli
The risk of venous thromboembolism (VTE) is increased across the spectrum of chronic kidney disease (CKD), from mild to more advanced CKD, and typically characterizes nephrotic syndrome (NS). VTE risk in patients with kidney disease may be due to underlying hemostatic abnormalities, including activation of pro-thrombotic factors, inhibition of endogenous anticoagulation systems, enhanced platelet activation and aggregation, and decreased fibrinolytic activity. The mechanisms involved differ depending on the cause of the kidney impairment (i...
March 1, 2017: European Journal of Internal Medicine
https://www.readbyqxmd.com/read/28223549/membranous-nephropathy-associated-with-hepatitis-c-virus-infection-treated-with-corticosteroids-and-ledipasvir-sofosbuvir-a-case-report-and-review-of-literature
#13
Qinjie Weng, Xiao Li, Hong Ren, Jingyuan Xie, Xiaoxia Pan, Jing Xu, Nan Chen
BACKGROUND: Membranous nephropathy (MN) is the most common cause of nephrotic syndrome in adults. As many clinical cases have reported, it may be associated with hepatitis C virus (HCV) infection. Antiviral therapy can be various. CASE SUMMARY: We report a case of patient with chronic HCV infection and MN, who presented with was proteinuria. He was treated with ledipasvir and sofosbuvir (Harvoni; Gilead Sciences, Foster City, CA) and was found to be virus-free. CONCLUSION: We have reported this case to provide insight into whether Ledipasvir-Sofosbuvir should be administered for HCV-related glomerulonephritis...
March 28, 2017: Oncotarget
https://www.readbyqxmd.com/read/28176955/urinary-excretion-of-il-1%C3%AE-il-6-and-il-8-cytokines-during-relapse-and-remission-of-idiopathic-nephrotic-syndrome
#14
Amal A Al-Eisa, Maysoun Al Rushood, Rajaa J Al-Attiyah
BACKGROUND/AIM: The role of pro-inflammatory cytokines in the immunopathogenesis of idiopathic nephrotic syndrome had been widely postulated. Reports on the release of cytokines, during idiopathic nephrotic syndrome (INS) activation, were conflicting in defining a specific interleukin pattern during relapse and remission of the disease. The aim of this study was to explore the role of IL-1β, IL-6 and IL-8 in the pathophysiology of INS during relapse and remission. PATIENTS AND METHODS: A total of 37 INS patients were included...
2017: Journal of Inflammation Research
https://www.readbyqxmd.com/read/28173653/-coenzyme-q-10-treatment-for-one-child-with-coq6-gene-mutation-induced-nephrotic-syndrome-and-literature-review
#15
Q Cao, G M Li, H Xu, Q Shen, L Sun, X Y Fang, H M Liu, W Guo, Y H Zhai, B B Wu
Objective: To summarize the clinical manifestation and molecular characteristics of COQ6 mutation induced nephrotic syndrome, and to evaluate efficacy of CoQ(10) therapy. Method: Clinical data of the case with infantile nephrotic syndrome was summarized, including clinical manifestations, laboratory findings and family investigation. The patient received CoQ(10) 30 mg/(kg·d) therapy. Urine protein/creatinine ratio, serum albumin and creatinine were detected to assess the efficacy of the therapy. Result: (1) The 10 months old boy was presented with nephrotic level proteinuria and hypoalbuminemia...
February 2, 2017: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/28112586/genetic-variation-in-the-glucocorticoid-pathway-involved-in-interindividual-differences-in-the-glucocorticoid-treatment
#16
Qian-Qian Song, Wan-Ying Xie, Yong-Jun Tang, Jun Zhang, Jie Liu
Glucocorticoids (GCs) are widely used for treating asthma, rheumatoid arthritis, nephrotic syndrome, acute lymphoblastic leukemia and other autoimmune diseases. However, in a subgroup of patients, failure to respond to GCs is known as GC resistance or GC insensitivity. This represents an important barrier to effective treatment and a clinical problem requiring an urgent solution. Genetic variation in the GC pathway is a significant factor in interindividual differences in GC treatment. This article reviews the pharmacogenetics of GCs in diverse diseases based on the GC pathway...
February 2017: Pharmacogenomics
https://www.readbyqxmd.com/read/28112049/kidney-disease-in-hepatitis-b-surface-antigen-positive-children-experience-from-a-centre-in-south-west-nigeria-and-a-review-of-the-nigerian-literature
#17
Adanze O Asinobi, Adebowale D Ademola, Clement A Okolo, Adedayo A Adepoju, Susan M Samuel, Wendy E Hoy
BACKGROUND: Kidney disease is an important extra-hepatic manifestation of hepatitis B virus (HBV) infection. However, there is paucity of recent literature on kidney disease in children and adolescents with HBV infection from several parts of sub-Saharan Africa including Nigeria. OBJECTIVE: To review the pattern of kidney disease in hepatitis B surface antigen (HBsAg)-positive children and adolescents seen at a tertiary hospital in south-west Nigeria. METHODS: A retrospective study was undertaken of HBsAg-seropositive children with kidney disease managed at University College Hospital, Ibadan, from January 2004 to December 2015...
January 23, 2017: Paediatrics and International Child Health
https://www.readbyqxmd.com/read/28098115/a-novel-fibrillin-1-mutation-in-an-egyptian-marfan-family-a-proband-showing-nephrotic-syndrome-due-to-focal-segmental-glomerulosclerosis
#18
Mohammad Al-Haggar, Ashraf Bakr, Yahya Wahba, Paul J Coucke, Fatma El-Hussini, Mona Hafez, Riham Eid, Abdel-Rahman Eid, Amr Sarhan, Ali Shaltout, Ayman Hammad, Sohier Yahia, Ahmad El-Rifaie, Dina Abdel-Hadi
Marfan syndrome (MFS), the founding member of connective tissue disorder, is an autosomal dominant disease; it is caused by a deficiency of the microfibrillar protein fibrillin-1 (FBN1) and characterized by involvement of three main systems; skeletal, ocular, and cardiovascular. More than one thousand mutations in FBN1 gene on chromosome 15 were found to cause MFS. Nephrotic syndrome (NS) had been described in very few patients with MFS being attributed to membranoproliferative glomerulonephritis secondary to infective endocarditis...
January 2017: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/28078995/sphingolipids-in-genetic-and-acquired-forms-of-chronic-kidney-diseases
#19
Norishi Ueda
Sphingolipids (SLs) regulate apoptosis, proliferation, and stress response. SLs, including ceramide, glycosphingolipids (glucosylceramide, lactosylceramide, and gangliosides) and sphingosine-1-phosphate (S1P), play a role in the pathogenesis and progression of genetic (lysosomal storage disease, congenital nephrotic syndrome and polycystic kidney disease) and non-genetic forms of chronic kidney diseases (CKDs). SLs metabolism defects promote complications (cardiovascular events, etc.) via oxidant stress in CKDs...
January 12, 2017: Current Medicinal Chemistry
https://www.readbyqxmd.com/read/28072731/focal-segmental-glomerulosclerosis-lagged-behind-the-onset-of-rheumatoid-arthritis-by-7-years-a-case-report-and-literature-review
#20
Yang Liu, Hong-Yan Wen, Li-Hua Wang, Chen Wang
INTRODUCTION: The co-existence of focal segmental glomerulosclerosis (FSGS) and rheumatoid arthritis (RA), presenting either together or in succession, is very rare. A variety of histopathological features in the clinical renal disease may occur in RA. Only 8 studies have previously reported this poorly understood connection. CLINICAL FINDINGS/DIAGNOSES: A case of a 54-year-old male with RA lasting for more than 7 years developed cheirarthritis as the first signs...
January 2017: Medicine (Baltimore)
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