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idiopathic nephrotic syndrome

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https://www.readbyqxmd.com/read/28534103/rituximab-for-steroid-dependent-or-frequently-relapsing-idiopathic-nephrotic-syndrome-in-adults-a-retrospective-multicenter-study-in-spain
#1
Iara DaSilva, Ana Huerta, Luis Quintana, Beatriz Redondo, Elena Iglesias, Juliana Draibe, Manuel Praga, José Ballarín, Montserrat Díaz-Encarnación
BACKGROUND: Patients with difficult-to-treat idiopathic nephrotic syndrome (INS), steroid-dependent nephrotic syndrome (SDNS), or frequently relapsing nephrotic syndrome (FRNS) require long-term immunosuppressive therapy. Rituximab offers an alternative treatment for patients with disease that has not responded to multiple therapies. OBJECTIVE: Our objective was to determine the efficacy and safety of rituximab in adult patients with difficult-to-treat (SDNS or FRNS) INS...
May 22, 2017: BioDrugs: Clinical Immunotherapeutics, Biopharmaceuticals and Gene Therapy
https://www.readbyqxmd.com/read/28533882/-idiopathic-nephrotic-syndrome-ins-in-children-in-dakar-about-40-cases
#2
Younoussa Keita, Ahmed Tall Lemrabott, Assane Sylla, Babacar Niang, El Hadji Fary Ka, Chérif Mohamed Dial, Aliou Abdoulaye Ndongo, Amadou Sow, Claude Moreira, Abdou Niang, Ousmane Ndiaye, Boucar Diouf, Mouhamadou Guélaye Sall
INTRODUCTION: This study aimed to analyze the diagnostic, therapeutic, and evolutionary features of nephrosis in children in a pediatric department in Dakar. METHODS: The study was carried out in the Department of Pediatrics at the Aristide Le Dantec Hospital. We conducted a retrospective study over a period of 3 years from 1 January 2012 to 31 December 2014. All patients aged 2-12 years with idiopathic nephrotic syndrome were included in the study. RESULTS: Forty cases of nephrosis were collected, that is to say a prevalence of 23% among patients with kidney disease treated in the Department of Pediatrics...
2017: Pan African Medical Journal
https://www.readbyqxmd.com/read/28487395/safety-of-rituximab-compared-with-steroids-and-cyclophosphamide-for-idiopathic-membranous-nephropathy
#3
Jan A J G van den Brand, Piero Ruggenenti, Antonietta Chianca, Julia M Hofstra, Annalisa Perna, Barbara Ruggiero, Jack F M Wetzels, Giuseppe Remuzzi
Guidelines recommend steroid plus cyclical cyclophosphamide (St-Cp) therapy for patients with idiopathic membranous nephropathy at high risk of progression to ESRD. Rituximab (Rtx) may be a safer alternative. In this retrospective, observational cohort study, we compared time to any adverse event (primary outcome); serious or nonserious events; partial and complete remission of the nephrotic syndrome; and a composite of doubling of serum creatinine, ESRD, or death between 100 Rtx-treated patients and 103 patients who received daily St-Cp We monitored patients with standardized protocols and adjusted for baseline characteristics by Cox regression...
May 9, 2017: Journal of the American Society of Nephrology: JASN
https://www.readbyqxmd.com/read/28483380/rac1-activation-in-podocytes-induces-the-spectrum-of-nephrotic-syndrome
#4
Richard Robins, Cindy Baldwin, Lamine Aoudjit, Jean-François Côté, Indra R Gupta, Tomoko Takano
Hyper-activation of Rac1, a small GTPase, in glomerular podocytes has been implicated in the pathogenesis of familial proteinuric kidney diseases. However, the role of Rac1 in acquired nephrotic syndrome is unknown. To gain direct insights into this, we generated a transgenic mouse model expressing a doxycycline-inducible constitutively active form of Rac1 (CA-Rac1) in podocytes. Regardless of the copy number, proteinuria occurred rapidly within five days, and the histology resembled minimal change disease...
May 5, 2017: Kidney International
https://www.readbyqxmd.com/read/28483237/biochemical-alteration-in-children-with-idiopathic-nephrotic-syndrome-associated-with-an-increased-risk-of-sensorineural-hearing-loss-additional-insights-in-cochlear-renal-relationship
#5
Ghada Mohamed El Mashad, Wafaa Moustafa M Abo El Fotoh, Ahmed Mahmoud Zein El Abedein, Fatma Abd El Raoof Abd El Sadek
OBJECTIVES: Children with Idiopathic Nephrotic Syndrome (INS) are at risk of hearing loss due to the adverse impact of medications and related immunological and genetic factors on both cochlea and kidney. So this work was planned to evaluate hearing status in children with INS and to clarify the possible associated risk factors by interpreting the clinical and laboratory profiles of those children. METHODS: Ninety children with INS aged 5-14 years [30 patients with steroid-sensitive nephrotic syndrome (SSNS), 30 patients with steroid dependent/frequently relapsing nephrotic syndrome (SDNS/FRNS), and 30 patients with steroid-resistant nephrotic syndrome (SRNS)], and 90 age and sex matched normal controls were enrolled into this study...
June 2017: International Journal of Pediatric Otorhinolaryngology
https://www.readbyqxmd.com/read/28481137/angiotensin-converting-enzyme-gene-polymorphism-in-children-with-idiopathic-nephrotic-syndrome-effect-on-biopsy-findings
#6
Maryam Monajemzadeh, Mahshid Hesami, Reza Shahsiah, Mohammad Vasei, Safoora Hooshmand, Parin Tanzifi, Niloofar Hajizadeh, Neamatollah Ataei, Mehryar Mehrkash, Faezeh Javadi Larijani, Mastaneh Moghtaderi, Behnaz Bazargani, Reza Khorvash, Narjes Soleimanifar
OBJECTIVE: Angiotensin converting enzyme (ACE) converts angiotensin I into angiotensin II. The ACE gene shows an I/D polymorphism, which correlates with ACE concentrations. The aim of this study is to evaluate the distribution of the ACE I/D genotype in children with idiopathic nephrotic syndrome (INS) and healthy controls and study the effect of this polymorphism on clinical and pathologic findings. METHODS: ACE gene I/D polymorphism of 104 patients with INS and 119 controls were determined...
May 8, 2017: Fetal and Pediatric Pathology
https://www.readbyqxmd.com/read/28476686/mutation-spectrum-of-genes-associated-with-steroid-resistant-nephrotic-syndrome-in-chinese-children
#7
Ying Wang, Xiqiang Dang, Qingnan He, Yan Zhen, Xiaoxie He, Zhuwen Yi, Kuichun Zhu
Approximately 20% of children with idiopathic nephrotic syndrome do not respond to steroid therapy. More than 30 genes have been identified as disease-causing genes for the steroid-resistant nephrotic syndrome (SRNS). Few reports were from the Chinese population. The coding regions of genes commonly associated with SRNS were analyzed to characterize the gene mutation spectrum in children with SRNS in central China. The first phase study involved 38 children with five genes (NPHS1, NPHS2, PLCE1, WT1, and TRPC6) by Sanger sequencing...
May 2, 2017: Gene
https://www.readbyqxmd.com/read/28469399/a-novel-biomarker-panel-to-identify-steroid-resistance-in-childhood-idiopathic-nephrotic-syndrome
#8
Michael R Bennett, LaTawnya Pleasant, Christopher Haffner, Qing Ma, Wendy D Haffey, Jun Ying, Michael Wagner, Kenneth D Greis, Prasad Devarajan
Idiopathic nephrotic syndrome (NS) is the most common glomerular disorder of childhood. Response to initial treatment with corticosteroids is an indicator of prognosis, as resistant patients often present more progressive disease. In this cross-sectional pilot study, we set out to discover a panel of noninvasive biomarkers that could distinguish steroid-resistant nephrotic syndrome (SRNS) from steroid-sensitive nephrotic syndrome (SSNS). Information gleaned from such a panel could yield more individualized treatment plans and prevent unnecessary steroid exposure in patients unlikely to respond...
2017: Biomarker Insights
https://www.readbyqxmd.com/read/28465799/immunoglobulin-g-and-m-levels-in-childhood-nephrotic-syndrome-two-centers-egyptian-study
#9
Ghada Mohamed El Mashad, Soha Abd El Hady Ibrahim, Sameh Abd Allah Abdelnaby
INTRODUCTION: 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. METHODS: This prospective case control study was done in Pediatric Nephrology Units at Minoufia and Benha University Hospitals, during the period from 1st March 2014 to 30th June 2015...
February 2017: Electronic Physician
https://www.readbyqxmd.com/read/28451893/immunology-of-idiopathic-nephrotic-syndrome
#10
REVIEW
Manuela Colucci, Giorgia Corpetti, Francesco Emma, Marina Vivarelli
The pathogenesis of idiopathic nephrotic syndrome (INS) is as yet unknown, but several lines of evidence indicate that the immune system may play a crucial pathogenic role in non-genetic INS. The most important of these are, first, the effectiveness of therapy based on immunosuppression and, second, a vast body of data derived both from experimental models and from patient studies that implicate T cells and more recently B cells as major players in INS pathogenesis. However, recent findings also suggest a direct role of podocytes as drivers of the disease process, and the interplay between the glomerulus and the immune system is still being elucidated...
April 27, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
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
#11
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/28420061/metabolic-profiling-reveals-new-serum-biomarkers-of-lupus-nephritis
#12
J Li, X-W Xie, H Zhou, B Wang, M-J Zhang, F-Y Tang
Metabolomics has been applied to explore altered metabolite profiles in disease and identify unique metabolic signatures specific to certain pathologies. The aim of the current study is to characterize the metabolic profile of patients diagnosed with lupus nephritis (LN) and explore new insights into underlying disease processes. A metabolomic approach using ultra-high-performance liquid chromatography coupled with high-resolution mass spectrometry (UPLC-HRMS) was developed in serum samples from 32 LN patients, 30 idiopathic nephrotic syndrome (INS) patients and 28 healthy controls (HCs)...
January 1, 2017: Lupus
https://www.readbyqxmd.com/read/28405891/renalase-in-children-with-glomerular-kidney-diseases
#13
Piotr Skrzypczyk, Joanna Przychodzień, Małgorzata Mizerska-Wasiak, Elżbieta Kuźma-Mroczkowska, Magdalena Okarska-Napierała, Elżbieta Górska, Anna Stelmaszczyk-Emmel, Urszula Demkow, Małgorzata Pańczyk-Tomaszewska
Studies suggest that renalase, a renal catecholamine-inactivating enzyme, plays a major role in the pathogenesis of kidney and cardiovascular diseases in adults. This study seeks to determine the role of renalase in children with glomerular kidney diseases. We evaluated the serum renalase, arterial stiffness, intima-media thickness, blood pressure, and clinical and biochemical parameters in 78 children (11.9 ± 4.6 years of age) with glomerulopathies such as idiopathic nephrotic syndrome (40 cases), IgA nephropathy (12 cases), Henoch-Schönlein nephropathy (12 cases), and other glomerulopathies (14 cases)...
April 13, 2017: Advances in Experimental Medicine and Biology
https://www.readbyqxmd.com/read/28391348/membranous-nephropathy-thinking-through-the-therapeutic-options
#14
Daniel Cattran, Paul Brenchley
Idiopathic membranous nephropathy (IMN) remains the most common cause of the nephrotic syndrome in adults and one of the leading identifiable causes of end-stage kidney disease. Prior to considering the best approach to treatment, three important components need to be considered. First, the natural history of the typical membranous patient today; second, the importance of identifying the causative factors; and third, the integration of the current data on the known autoantibody/antigen systems involved in IMN into the diagnosis and management of the patient...
January 1, 2017: Nephrology, Dialysis, Transplantation
https://www.readbyqxmd.com/read/28391338/what-are-we-missing-in-the-clinical-trials-of-focal-segmental-glomerulosclerosis
#15
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/28352006/assay-of-urinary-protein-carbonyl-content-can-predict-the-steroid-dependence-and-resistance-in-children-with-idiopathic-nephrotic-syndrome
#16
Niranjan Gopal, Bidhan Chandra Koner, Atanu Bhattacharjee, Vishnu Bhat, Sathish Babu Murugaiyan, Prakash H Muddegowda
Nephrotic syndrome in pediatric age is mostly idiopathic. Idiopathic nephrotic syndrome (INS) by default is treated with steroids from the very beginning. Some do not respond to steroids and are grouped later as either steroid-resistant (SR) or steroid-dependent (SD) cases. The protein selectivity index often fails to predict the SR and SD cases. The SD and SR cases of INS exhibit higher degrees of oxidative stress compared to steroid responders. Proteins get carbonylated when they are exposed to free radicals...
March 2017: Saudi Journal of Kidney Diseases and Transplantation
https://www.readbyqxmd.com/read/28347297/factors-affecting-the-long-term-outcomes-of-idiopathic-membranous-nephropathy
#17
Hyuk Huh, Hajeong Lee, Jung Pyo Lee, Dong Ki Kim, Sohee Oh, Yun Kyu Oh, Yon Su Kim, Chun Soo Lim
BACKGROUND: We attempted to describe the clinical features and determine the factors associated with renal survival in idiopathic membranous nephropathy (iMN) patients with nephrotic syndrome (NS) and to determine the factors associated with spontaneous complete remission (sCR) and progression to NS in iMN patients with subnephrotic proteinuria. METHODS: This retrospective study involved 166 iMN patients with NS and 65 patients with subnephrotic proteinuria. The primary end point was a doubling of serum creatinine or initiation of dialysis...
March 27, 2017: BMC Nephrology
https://www.readbyqxmd.com/read/28341877/short-courses-of-daily-prednisolone-during-upper-respiratory-tract-infections-reduce-relapse-frequency-in-childhood-nephrotic-syndrome
#18
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/28318625/mycophenolate-mofetil-is-inferior-to-tacrolimus-in-sustaining-remission-in-children-with-idiopathic-steroid-resistant-nephrotic-syndrome
#19
Aditi Sinha, Aarti Gupta, Mani Kalaivani, Pankaj Hari, Amit K Dinda, Arvind Bagga
Studies of nephrotic syndrome show that substitution of calcineurin inhibitors by mycophenolate mofetil (MMF) enables sustained remission and corticosteroid sparing and avoids therapy associated adverse effects. However, controlled studies in patients with steroid resistance are lacking. Here we examined the effect of switching from therapy with tacrolimus to MMF on disease course in an open-label, one-to-one randomized, controlled trial on children (one to 18 years old), recently diagnosed with steroid-resistant nephrotic syndrome, at a referral center in India...
March 15, 2017: Kidney International
https://www.readbyqxmd.com/read/28314744/randomised-controlled-trial-comparing-ofatumumab-to-rituximab-in-children-with-steroid-dependent-and-calcineurin-inhibitor-dependent-idiopathic-nephrotic-syndrome-study-protocol
#20
Pietro Ravani, Alice Bonanni, Gian Marco Ghiggeri
INTRODUCTION: Oral steroids induce remission in about 90% of children with idiopathic nephrotic syndrome (INS), which is characterised by severe proteinuria and hypoalbuminaemia. Some children become steroid-dependent (SD) and require addition of calcineurin inhibitors (CNI) to maintain remission. Since these oral agents are toxic, alternative interventions are needed for long-term treatment. The anti-CD20 antibody rituximab has shown promising steroid-sparing properties in clinical trials, but benefits are less convincing in complicated forms of SD-INS...
March 17, 2017: BMJ Open
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