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Nephrotic syndrome children

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https://www.readbyqxmd.com/read/29155846/soluble-cd40-ligand-directly-alters-glomerular-permeability-and-may-act-as-a-circulating-permeability-factor-in-fsgs
#1
Sophie Doublier, Cristina Zennaro, Luca Musante, Tiziana Spatola, Giovanni Candiano, Maurizio Bruschi, Luca Besso, Massimo Cedrino, Michele Carraro, Gian Marco Ghiggeri, Giovanni Camussi, Enrico Lupia
CD40/CD40 ligand (CD40L) dyad, a co-stimulatory bi-molecular complex involved in the adaptive immune response, has also potent pro-inflammatory actions in haematopoietic and non-haematopoietic cells. We describe here a novel role for soluble CD40L (sCD40L) as modifier of glomerular permselectivity directly acting on glomerular epithelial cells (GECs). We found that stimulation of CD40, constitutively expressed on GEC cell membrane, by the sCD40L rapidly induced redistribution and loss of nephrin in GECs, and increased albumin permeability in isolated rat glomeruli...
2017: PloS One
https://www.readbyqxmd.com/read/29155175/fcgr2a-single-nucleotide-polymorphism-confers-susceptibility-to-childhood-onset-idiopathic-nephrotic-syndrome
#2
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/29146214/-treatments-of-steroid-dependent-nephrotic-syndrome-in-children
#3
A Couderc, E Bérard, V Guigonis, I Vrillon, J Hogan, V Audard, V Baudouin, C Dossier, O Boyer
Primary nephrotic syndrome (NS) is the most common glomerular disease in children. It is characterized by massive proteinuria and hypoalbuminemia. It typically has a sudden onset and more than 70% of patients will experience at least one relapse. An immunological origin has long been postulated, although the precise molecular mechanisms underlying the disease remain debated. Steroids are the first-line therapy with cumulative dose and duration of initial treatment varying among countries. Steroid-sparing agents may be indicated in case of steroid-dependency or frequent relapses...
November 13, 2017: Archives de Pédiatrie: Organe Officiel de la Sociéte Française de Pédiatrie
https://www.readbyqxmd.com/read/29130775/results-of-native-and-transplant-kidney-biopsies-of-children-in-a-single-center-over-a-15-years-period
#4
Emel Isiyel, Kibriya Fidan, Bahar Buyukkaragoz, Meltem Akcaboy, Yasar Kandur, Ipek Isik Gonul, Necla Buyan, Sevcan Bakkaloglu, Oguz Soylemezoglu
Renal biopsy is an important diagnostic procedure in pediatric nephrology. This study retrospectively investigates the indications, results and complications in a single tertiary children's hospital in Turkey. We evaluated the native and transplant kidney biopsies in Gazi University Pediatric Nephrology Department between 2001 and 2015. A total of 196 biopsies (144 natives and 52 transplants) were included into the study. The mean age of the patients was respectively 10.8 ± 3.5, 13.9 ± 1.5 years...
November 2017: Renal Failure
https://www.readbyqxmd.com/read/29127259/whole-exome-sequencing-of-patients-with-steroid-resistant-nephrotic-syndrome
#5
Jillian K Warejko, Weizhen Tan, Ankana Daga, David Schapiro, Jennifer A Lawson, Shirlee Shril, Svjetlana Lovric, Shazia Ashraf, Jia Rao, Tobias Hermle, Tilman Jobst-Schwan, Eugen Widmeier, Amar J Majmundar, Ronen Schneider, Heon Yung Gee, J Magdalena Schmidt, Asaf Vivante, Amelie T van der Ven, Hadas Ityel, Jing Chen, Carolin E Sadowski, Stefan Kohl, Werner L Pabst, Makiko Nakayama, Michael J G Somers, Nancy M Rodig, Ghaleb Daouk, Michelle Baum, Deborah R Stein, Michael A Ferguson, Avram Z Traum, Neveen A Soliman, Jameela A Kari, Sherif El Desoky, Hanan Fathy, Martin Zenker, Sevcan A Bakkaloglu, Dominik Müller, Aytul Noyan, Fatih Ozaltin, Melissa A Cadnapaphornchai, Seema Hashmi, Jeffrey Hopcian, Jeffrey B Kopp, Nadine Benador, Detlef Bockenhauer, Radovan Bogdanovic, Nataša Stajić, Gil Chernin, Robert Ettenger, Henry Fehrenbach, Markus Kemper, Reyner Loza Munarriz, Ludmila Podracka, Rainer Büscher, Erkin Serdaroglu, Velibor Tasic, Shrikant Mane, Richard P Lifton, Daniela A Braun, Friedhelm Hildebrandt
BACKGROUND AND OBJECTIVES: Steroid-resistant nephrotic syndrome overwhelmingly progresses to ESRD. More than 30 monogenic genes have been identified to cause steroid-resistant nephrotic syndrome. We previously detected causative mutations using targeted panel sequencing in 30% of patients with steroid-resistant nephrotic syndrome. Panel sequencing has a number of limitations when compared with whole exome sequencing. We employed whole exome sequencing to detect monogenic causes of steroid-resistant nephrotic syndrome in an international cohort of 300 families...
November 10, 2017: Clinical Journal of the American Society of Nephrology: CJASN
https://www.readbyqxmd.com/read/29120340/mycophenolate-sodium-for-children-with-frequently-relapsing-or-steroid-dependent-nephrotic-syndrome
#6
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/29119640/il-23-actived-%C3%AE-%C3%AE-t-cells-affect-th17-cells-and-regulatory-t-cells-by-secreting-il-21-in-children-with-primary-nephrotic-syndrome
#7
Li Zhang, Junli Yan, Baohui Yang, Gaofu Zhang, Mo Wang, Shifang Dong, Wei Liu, Haiping Yang, Qiu Li
OBJECTIVE: This study (1) analyzed the percentage of γδ T cells, γδ T cell subsets, Th17 cells, and regulatory T cells (Treg cells), and (2) determined the role of IL-23 in primary nephrotic syndrome (PNS) patients with active disease and in remission. MATERIALS AND METHODS: Eighty-four PNS patients and 51 healthy age-matched controls were included in this study. The percentage of γδ T cells, γδ T cell subsets, Th17 cells, and Treg cells in peripheral blood mononuclear cells (PBMCs) were analyzed by fluorescence-activated cell sorting...
November 9, 2017: Scandinavian Journal of Immunology
https://www.readbyqxmd.com/read/29098400/histone-deacetylase-2-expression-and-activity-in-children-with-nephrotic-syndrome-with-different-glucocorticoid-response
#8
Feng-Jun Guan, Qian-Qian Peng, Lin-Li Wang, Xue-Bo Yan, Chen Dong, Xiao-Hua Jiang
BACKGROUND: Glucocorticosteroid (GC) is one of the most effective drugs available for the treatment of primary nephrotic syndrome (PNS) in children. However, some patients show little or no response to GC. The purpose of our research was to observe and describe the different levels of histone deacetylase-2 (HDAC2) expression in peripheral blood lymphocytes in children with PNS compared with various responses to the GC treatment, with the primary aim to assess the correlation between HDAC2 and GC resistance in PNS children...
November 2, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29094445/kidney-transplantation-in-a-child-with-pierson-syndrome
#9
Sanem Guler, Sertac Cimen, Phillip Acott, Kathy Whelan, Michele Molinari
Congenital nephrotic syndrome is commonly associated with mutations in genes that encode podocyte and slit diaphragm proteins or the structural and regulatory proteins of the GBM. These mutations lead to the formation of dysfunctional proteins, which account for the resistance of the renal manifestations to conventional treatment methods. Consequently, patients become renal replacement therapy dependent. Mutation of the LAMB2 gene is associated with Pierson syndrome, which is an autosomal recessive disorder characterized by congenital nephrotic syndrome and ocular abnormalities...
November 1, 2017: Pediatric Transplantation
https://www.readbyqxmd.com/read/29081077/effect-of-cesarean-section-on-relapse-of-childhood-idiopathic-nephrotic-syndrome
#10
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/29081073/urinary-excretion-of-sphingomyelinase-phosphodiesterase-acid-like-3b-in-children-with-intractable-nephrotic-syndrome
#11
Shojiro Watanabe, Koji Tsugawa, Kazushi Tsuruga, Tadaatsu Imaizumi, Hiroshi Tanaka
Rituximab (RTX), a specific antibody to human CD20, has been successfully used to treat intractable nephrotic syndrome (NS). Recent studies have suggested a direct effect of RTX on podocytes by targeting sphingomyelinase phosphodiesterase acid-like 3b (SMPDL-3b). Thus, we examined the urinary excretion of SMPDL-3b as well as its immunoreactivity in biopsy specimens from children with intractable NS. Urine samples from six patients (five with minimal-change NS and one with focal segmental glomerulosclerosis) and from four healthy adults were examined...
October 2017: Pediatrics International: Official Journal of the Japan Pediatric Society
https://www.readbyqxmd.com/read/29069848/efficacy-and-safety-of-immunosuppressive-medications-for-steroid-resistant-nephrotic-syndrome-in-children-a-systematic-review-and-network-meta-analysis
#12
Shaojun Li, Haiping Yang, Pengfei Guo, Xiaoxiao Ao, Junli Wan, Qiu Li, Liping Tan
BACKGROUND: Conventional meta-analyses and randomized controlled trials have shown inconsistent results regarding the efficacy of immunosuppressants for pediatric steroid-resistant nephrotic syndrome (SRNS). OBJECTIVE: To conduct a network meta-analysis aimed at evaluating the efficacy and safety of available immunosuppressive agents in pediatric patients with SRNS. STUDY METHODS: MEDLINE, the Cochrane Central Register of Controlled Trials, and EMBASE were searched on January 2017...
September 22, 2017: Oncotarget
https://www.readbyqxmd.com/read/29063524/on-the-relationship-between-proteinuria-and-plasma-phosphate
#13
Sophie de Seigneux, Alexandra Wilhelm-Bals, Marie Courbebaisse
Albuminuria is strongly associated with renal and cardiovascular outcomes independently of renal function level. However, the pathophysiology of these associations is debated. In chronic kidney disease (CKD), phosphate retention participates in cardiovascular events and increased cardiovascular mortality. We hypothesised that albuminuria may modulate tubular phosphate handling by the kidney. To verify this hypothesis, we first studied the association between phosphataemia and albuminuria in children with nephrotic syndrome and in adults with CKD...
October 24, 2017: Swiss Medical Weekly
https://www.readbyqxmd.com/read/29055396/role-of-gut-microbiota-in-idiopathic-nephrotic-syndrome-in-children
#14
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
#15
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/29050110/-interpretation-of-evidence-based-guideline-on-diagnosis-and-treatment-of-steroid-sensitive-relasping-steroid-dependent-nephrotic-syndrome-in-children-2016
#16
F Yang, X Y Jiang
No abstract text is available yet for this article.
October 2, 2017: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/29050108/-evidence-based-guideline-on-diagnosis-and-treatment-of-steroid-sensitive-relapsing-steroid-dependent-nephrotic-syndrome-in-children-2016
#17
(no author information available yet)
No abstract text is available yet for this article.
October 2, 2017: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/29045960/pharmacokinetics-of-once-daily-prolonged-release-formulation-of-tacrolimus-in-children-with-primary-nephrotic-syndrome
#18
Y Han, S Q DU, H J Xiao, Y Zhou, J Ding, J J Ding, Y M Cui
OBJECTIVE: Tacrolimus prolonged-release(PR) formulation is a new once-daily formulation of the calcineurin inhibitor tacrolimus, which is currently used in adult liver or kidney transplant patients,and is also gradually widely used in children with nephrotic syndrome.The present study was undertaken to preliminarily investigate the pharmacokinetic characteristics of tacrolimus PR in pediatric nephrotic syndrome recipients. METHODS: This single-center open-label prospective study was performed in pediatric nephrotic syndrome recipients...
October 18, 2017: Beijing da Xue Xue Bao. Yi Xue Ban, Journal of Peking University. Health Sciences
https://www.readbyqxmd.com/read/29045958/-clinical-characteristics-of-glucocorticoid-induced-eye-adverse-reactions-in-children-with-primary-nephrotic-syndrome
#19
Y Zhao, B G Su, H J Xiao, H W Zhang, X Y Liu, F Wang, J Ding
OBJECTIVE: To investigate the clinical features and side effects, with regard to glucocorticoid-induced ocular hypertension, glaucoma or cataract in children with primary nephrotic syndrome. METHODS: Clinical data were collected and analyzed from 71 cases of primary nephrotic syndrome with glucocorticoid-induced ocular hypertension, glaucoma or cataract from Jun. 2014 to Jun. 2016. These children were hospitalized in Peking University First Hospital. RESULTS: Totally 1 580 children with primary nephrotic syndrome were collected, glucocorticoid-induced complications in eyes were found in 71 cases, and the incidence was 4...
October 18, 2017: Beijing da Xue Xue Bao. Yi Xue Ban, Journal of Peking University. Health Sciences
https://www.readbyqxmd.com/read/29034405/immunohistochemical-and-serological-characterization-of-membranous-nephropathy-in-children-and-adolescents
#20
Anne K Dettmar, Thorsten Wiech, Markus J Kemper, Armin Soave, Michael Rink, Jun Oh, Rolf A K Stahl, Elion Hoxha
BACKGROUND: Membranous nephropathy (MN) is a common cause of nephrotic syndrome in adults, but is less frequent in children. Antibodies against four antigens leading to MN have been described in children: phospholipase A2 receptor 1 (PLA2R1), thrombospondin type-1 domain-containing 7A (THSD7A), neutral endopeptidase (NEP), and cationic bovine serum albumin (BSA). METHODS: Twelve children with MN were included in this study. Sera of all patients were analyzed for antibodies against PLA2R1, THSD7A, NEP, and BSA...
October 15, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
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