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idiopathic nephrotic syndrome

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https://www.readbyqxmd.com/read/29155175/fcgr2a-single-nucleotide-polymorphism-confers-susceptibility-to-childhood-onset-idiopathic-nephrotic-syndrome
#1
Giovanni M Rossi, Francesco Bonatti, Alessia Adorni, Federico Alberici, Monica Bodria, Alice Bonanni, Gian M Ghiggeri, Davide Martorana, Augusto Vaglio
Childhood-onset idiopathic nephrotic syndrome affects 1.15-3.4 children/100,000 children/year in Western Countries. Immune-mediated mechanisms, particularly T cell-mediated, are thought to play a key pathogenic role. The genetic basis of the disease is still poorly understood. We tested the association between single nucleotide polymorphisms (SNPs) of four genes encoding Fc gamma receptors (FCGR2A, FCGR2B, FCGR3A, FCGR3B) and idiopathic nephrotic syndrome in a case-control study of paediatric patients. Children with idiopathic nephrotic syndrome (aged 1-16 years) were included...
November 15, 2017: Immunology Letters
https://www.readbyqxmd.com/read/29149305/rituximab-for-non-responsive-idiopathic-membranous-nephropathy-in-a-chinese-cohort
#2
Xin Wang, Zhao Cui, Yi-Miao Zhang, Zhen Qu, Fang Wang, Li-Qiang Meng, Xu-Yang Cheng, Gang Liu, Fu-de Zhou, Ming-Hui Zhao
Background: Rituximab had been shown to be effective in inducing remission of nephrotic syndrome in patients with idiopathic membranous nephropathy (iMN). This study applied rituximab therapy for 36 non-responsive iMN patients to investigate its effects and safety. Methods: Thirty-six iMN patients who were non-responsive to prior immunosuppression were enrolled. Rituximab was used for B-cell depletion in patients, with a goal of <5 B cells/mm3 in the circulation...
November 14, 2017: Nephrology, Dialysis, Transplantation
https://www.readbyqxmd.com/read/29120340/mycophenolate-sodium-for-children-with-frequently-relapsing-or-steroid-dependent-nephrotic-syndrome
#3
Kanika Kapoor, Abhijeet Saha, Manpreet Kaur, Nand Kishore Dubey, Ashish Datt Upadhyay
In this retrospective study, patients with idiopathic frequently-relapsing nephrotic syndrome (FRNS) (n=27) and steroid dependent nephrotic syndrome (SDNS) (n=13) who received enteric coated mycophenolate sodium (ECMS) for at least 6 months, were included for analysis. Primary outcome was response to ECMS, which was defined as complete if there were no relapses, partial response if there was 1 relapse and no response if there were 2 or more relapses within 6 months of initiation. The mean (SD) dose of ECMS was 985...
October 15, 2017: Indian Pediatrics
https://www.readbyqxmd.com/read/29081077/effect-of-cesarean-section-on-relapse-of-childhood-idiopathic-nephrotic-syndrome
#4
Takahisa Kimata, Jiro Kino, Sohsaku Yamanouchi, Chikushi Suruda, Shoji Tsuji, Kazunari Kaneko
In parallel with the increase in the prevalence of childhood chronic diseases, the rate of cesarean delivery has risen during the past decades. This study tested the hypothesis that children delivered by cesarean section (CS) have a higher risk of relapse of idiopathic nephrotic syndrome (INS). Fifty-six children with INS were categorized into three groups. Group A consisted of patients with INS who had no relapses after the onset of INS; group B consisted of patients with INS who had infrequent relapse; and group C consisted of patients with INS who had frequent relapse...
October 2017: Pediatrics International: Official Journal of the Japan Pediatric Society
https://www.readbyqxmd.com/read/29079545/thsd7a-associated-membranous-nephropathy-in-a-patient-with-neurofibromatosis-type-1
#5
Fujun Lin, Dan Zhang, Juan Chang, Xuanli Tang, Wenbin Guan, Gengru Jiang, Chun Zhu, Fan Bian
Target antigens in idiopathic membranous nephropathy (MN) include the phospholipase A2 receptor (PLA2R), and in some cases, the thrombospondin type 1 domain-containing 7A (THSD7A). A notable phenomenon is the high rate of cancer (reported to be as high as 20%) in patients with THSD7A-associated MN. Neurofibromatosis type 1 (NF1) is an autosomal dominant disease caused by NF1 gene mutation, and clinically characterized by multiple cutaneous neurofibromas and café-au-lait spots. In this article, we report a patient with NF1 who developed THSD7A-associated MN when the NF1 skin lesions deteriorated...
October 24, 2017: European Journal of Medical Genetics
https://www.readbyqxmd.com/read/29079480/structure-of-human-m-type-phospholipase-a2-receptor-revealed-by-cryo-electron-microscopy
#6
Yue Dong, Longxing Cao, Hua Tang, Xiangyi Shi, Yongning He
M-type phospholipase A2 receptor (M-PLA2R) is a member of the mannose receptor family and known as the receptor of secretory phospholipase A2s (sPLA2s). It has also been identified as the major autoantigen of idiopathic membranous nephropathy, one of the most common causes for nephrotic syndrome in adults. Here we determine the structure of human M-PLA2R ectodomain by cryo-electron microscopy. The results show that the ectodomain has high internal flexibility and forms a compact dual-ring shaped conformation at acidic pH, and adopts extended conformations at basic pH...
October 24, 2017: Journal of Molecular Biology
https://www.readbyqxmd.com/read/29055396/role-of-gut-microbiota-in-idiopathic-nephrotic-syndrome-in-children
#7
Kazunari Kaneko, Shoji Tsuji, Takahisa Kimata
Nephrotic syndrome characterized by heavy proteinuria and edema is the most common chronic kidney disease in children. It is classified into three categories, of which the idiopathic type accounts for the vast majority of cases. As indicated by the name, the etiology of idiopathic nephrotic syndrome remains unknown though it has been suggested that impaired T cell function is involved. Recently, evidence has mounted to suggest that dysfunction in regulatory T cells plays an important role in the development of allergic disease, a recognized comorbid condition for children with idiopathic nephrotic syndrome...
October 2017: Medical Hypotheses
https://www.readbyqxmd.com/read/29054532/a-randomized-clinical-trial-indicates-that-levamisole-increases-the-time-to-relapse-in-children-with-steroid-sensitive-idiopathic-nephrotic-syndrome
#8
Mariken P Gruppen, Antonia H Bouts, Marijke C Jansen-van der Weide, Maruschka P Merkus, Aleksandra Zurowska, Michal Maternik, Laura Massella, Francesco Emma, Patrick Niaudet, Elisabeth A M Cornelissen, Thierry Schurmans, Ann Raes, Johan van de Walle, Mieke van Dyck, Ashima Gulati, Arvind Bagga, Jean-Claude Davin
Levamisole has been considered the least toxic and least expensive steroid-sparing drug for preventing relapses of steroid-sensitive idiopathic nephrotic syndrome (SSINS). However, evidence for this is limited as previous randomized clinical trials were found to have methodological limitations. Therefore, we conducted an international multicenter, placebo-controlled, double-blind, randomized clinical trial to reassess its usefulness in prevention of relapses in children with SSINS. The efficacy and safety of one year of levamisole treatment in children with SSINS and frequent relapses were evaluated...
October 17, 2017: Kidney International
https://www.readbyqxmd.com/read/29031489/-pediatric%C3%A2-nephrology-what%C3%A2-should-an%C3%A2-adult%C3%A2-nephrologist-know-about%C3%A2-these%C3%A2-diseases
#9
Sandrine Lemoine, Pierre Cochat, Aurélia Bertholet-Thomas, Charlène Levi, Catherine Bonnefoy, Anne-Laure Sellier-Leclerc, Justine Bacchetta
In nephrology, some diseases begin specifically during childhood; however, they must be known by adult nephrologists so that to ensure continuity and homogeneity for their management. The aim of this review is therefore to propose a brief overview of the main pediatric diseases, for which a specific knowledge is warranted, and notably pediatric idiopathic nephrotic syndrome, cystinosis, primary hyperoxaluria and hereditaries tubulopathies.
October 11, 2017: Néphrologie & Thérapeutique
https://www.readbyqxmd.com/read/29022104/deriving-and-understanding-the-risk-of-post-transplant-recurrence-of-nephrotic-syndrome-in-the-light-of-current-molecular-and-genetic-advances
#10
REVIEW
Agnieszka Bierzynska, Moin A Saleem
After renal transplantation, recurrence of the original disease is the second most common cause of graft loss, after rejection. The most dramatic manifestation of this phenomenon is in patients with nephrotic syndrome (NS). NS is a descriptive term describing a clinical picture centred on proteinuria arising from damage to the glomerular filtration barrier (GFB). There are many different drivers of that damage, ranging from immune dysregulation to genetic disorders and chronic disease/infections. The main categories in childhood are "idiopathic" (presumed immune mediated) and genetic NS, with further stratification of the idiopathic group according to steroid responses...
October 11, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28979773/efficacy-of-mycophenolate-treatment-in-adults-with-steroid-dependent-frequently-relapsing-idiopathic-nephrotic-syndrome
#11
Diego Sandoval, Rafael Poveda, Juliana Draibe, Laureà Pérez-Oller, Montserrat Díaz, José Ballarín, Anna Saurina, Helena Marco, Josep Bonet, Xoana Barros, Xavier Fulladosa, Joan Torras, Josep M Cruzado
BACKGROUND: This study assessed the efficacy of therapy with mycophenolate (MF) and reduced doses of steroids in adults with steroid-dependent/frequently relapsing idiopathic nephrotic syndrome (SD/FR-INS). METHODS: Twenty-nine nephrotic patients (including 16 males and 13 females; mean age: 40 years, range: 18-74) were treated. Starting doses of MF were 2000 mg/day for mofetil MF (1500 mg/day in one patient) or 1440 mg/day for sodium MF. The initial prednisone (PDN) dose was 10 mg/day in 14 patients, 5 mg/day in two patients and no steroids in one patient...
October 2017: Clinical Kidney Journal
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
#12
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/28975099/predictive-risk-factors-of-steroid-dependent-nephrotic-syndrome-in-children
#13
Maher Ahmed Abdel-Hafez, Nagy Mohamed Abou-El-Hana, Adel Ali Erfan, Mohamed El-Gamasy, Hend Abdel-Nabi
BACKGROUND: Development of steroid dependency is one of the difficult problems in the management of children with idiopathic nephrotic syndrome, leading to increased morbidity, complications and cost of treatment. Thus, predicting early in the disease course will be useful in counseling parents and may improve treatment strategy. OBJECTIVES: To determine the clinical characteristics that can predict the development of steroid dependency early in the initial episodes of steroid sensitive nephrotic syndrome (SSNS)...
July 2017: Journal of Nephropathology
https://www.readbyqxmd.com/read/28965242/uteroglobin-gene-polymorphism-g38a-may-be-a-risk-factor-in-childhood-idiopathic-nephrotic-syndrome
#14
Beltinge Demircioglu Kılıc, Mithat Buyukcelik, Sibel Oguzkan Balcı, Sacide Pehlivan, Seval Kul, Nilgun Col, Ayse Balat
BACKGROUND: Uteroglobin (UG) is a multifunctional protein with anti-inflammatory properties. The aim of this study was to first evaluate the role of UG gene G38A polymorphism in childhood idiopathic nephrotic syndrome (INS), and determine whether this variation may be related to the occurrence of INS or a steroid response. METHODS: One hundred and thirty-six children diagnosed with INS in Gaziantep University, Department of Pediatric Nephrology, and 70 healthy volunteers were included...
September 30, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28963877/t-lymphocyte-expressing-inflammatory-cytokines-underlie-persistence-of-proteinuria-in-children-with-idiopathic-nephrotic-syndrome
#15
Fábio Tadeu Lourenço Guimarães, Gustavo Eustáquio Brito Alvim de Melo, Thiago Macedo Cordeiro, Victor Feracin, Etel Rocha Vieira, Wagner de Fátima Pereira, Sérgio Veloso Brant Pinheiro, Aline Silva Miranda, Ana Cristina Simões E Silva
OBJECTIVE: There is evidence of an important role of immune system changes in the triggering and maintenance of idiopathic nephrotic syndrome (INS). The aim of this study was to investigate the expression of cytokines in lymphocyte populations of patients with INS in comparison to healthy individuals, according to proteinuria. METHODS: This cross-sectional study included 44 patients with INS and eight healthy children, matched for age and sex (controls). Patients were subdivided according to proteinuria: persistent proteinuria or partial remission (PP≥300mg/24h, n=17) and low proteinuria or complete remission (LP<300mg/24h, n=27)...
September 27, 2017: Jornal de Pediatria
https://www.readbyqxmd.com/read/28963315/reducing-steroids-in-relapsing-nephrotic-syndrome-the-restern-study-protocol-of-a-national-double-blind-randomised-placebo-controlled-non-inferiority-intervention-study
#16
A M Schijvens, E M Dorresteijn, N Roeleveld, R Ter Heine, J A E van Wijk, A H M Bouts, M G Keijzer-Veen, N C A J van de Kar, L P W J van den Heuvel, M F Schreuder
INTRODUCTION: Oral corticosteroids are the first-line treatment for idiopathic childhood nephrotic syndrome. Most children experience several relapses, needing repeated courses of corticosteroid therapy. This exposes them to side effects and long-term complications. For most patients, long-term prognosis is for complete resolution of the disease over time and maintenance of normal kidney function. Therefore, it is vital to focus on minimising adverse events of the disease and its therapy...
September 27, 2017: BMJ Open
https://www.readbyqxmd.com/read/28949280/childhood-nephrotic-syndrome-in-tropical-africa-then-and-now
#17
Wasiu A Olowu, Adebowale Ademola, Adebukola B Ajite, Yauba M Saad
This descriptive and comparative review examines the changing epidemiology, treatment, renal and patient outcome of childhood nephrotic syndrome (NS) in tropical Africa (TpAfr). In the 1960s to 1980s, corticosteroid-resistant non-minimal change disease (nMCD) including quartan malaria nephropathy (QMN) was the dominant renal histopathology type. The overall incidence of NS was 0.35-1.34% of hospital admissions. Median age at onset of NS ranged between 4.0 and 12.0 years while the mean (SD) age range was 5.8 (3...
September 26, 2017: Paediatrics and International Child Health
https://www.readbyqxmd.com/read/28941627/-childhood-steroid-dependent-idiopathic-nephrotic-syndrome-predictive-factors-for-the-need-of-immunosuppressive-treatment
#18
M Golay, A Douillard, N Nagot, M Fila, L Ichay, F Dalla Vale, J Tenenbaum, D Morin
BACKGROUND AND AIMS: More than half of the children with idiopathic nephrotic syndrome become steroid-dependent (or frequent relapsers) and will later require the use of complementary treatment aiming to reduce steroids' side effects and to limit the number of proteinuria relapses. It appears important to identify these children as early as possible in order to adapt their treatment. The aim of this study was to analyze the population of children, under 18 years of age, diagnosed between 1/01/2000 and 31/05/2015 with an idiopathic nephrotic syndrome and followed at the Montpellier University Hospital to search for criteria predictive of steroid-sparing agent use...
November 2017: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
https://www.readbyqxmd.com/read/28914167/nephrotic-syndrome-in-infants-and-children-pathophysiology-and-management
#19
Mallory L Downie, Claire Gallibois, Rulan S Parekh, Damien G Noone
Nephrotic syndrome is defined by nephrotic-range proteinuria (≥40 mg/m(2)/hour or urine protein/creatinine ratio ≥200 mg/mL or 3+ protein on urine dipstick), hypoalbuminaemia (<25 g/L) and oedema. This review focuses on the classification, epidemiology, pathophysiology, management strategies and prognosis of idiopathic nephrotic syndrome of childhood, and includes a brief overview of the congenital forms.
September 15, 2017: Paediatrics and International Child Health
https://www.readbyqxmd.com/read/28898290/comparative-effectiveness-and-tolerance-of-immunosuppressive-treatments-for-idiopathic-membranous-nephropathy-a-network-meta-analysis
#20
COMPARATIVE STUDY
Song Ren, Ying Wang, Li Xian, Tadashi Toyama, Meg Jardine, Guisen Li, Vlado Perkovic, Daqing Hong
BACKGROUND: Immunosuppressive agents in general are shown to prevent renal progression and all-cause mortality in idiopathic membranous nephropathy (IMN) patients with nephrotic syndrome. However, the efficacy and safety of different immunosuppressive treatments have not been systematic assessed and compared. A network meta-analysis was performed to compare different immunosuppressive treatment in IMN. METHODS: Cochrane library, MEDLINE, EMBASE and trial register system were searched for randomized controlled trials reporting the treatments for IMN to May 3, 2016...
2017: PloS One
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