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https://www.readbyqxmd.com/read/29143203/relapse-of-nephrotic-syndrome-triggered-by-kawasaki-disease
#1
Ryo Maeda, Yukihiko Kawasaki, Shigeo Suzuki, Shinichiro Ohara, Suyama Kazuhide, Mitsuaki Hosoya
Minor infections, allergies, insect bites, and bee stings are commonly reported causes of nephrotic syndrome (NS). Herein, we report, to the best of our knowledge, the first case of NS relapse due to Kawasaki disease (KD). An 8-year-old boy presented with high fever of 4-day duration. He had developed steroid-dependent NS at the age of 4 years and remained in remission after steroid and mizonbin therapy. Renal biopsy, performed at the age of four, showed minimal change (MC) disease. Upon examination, the patient fulfilled 5 of 6 criteria for KD under the Japanese diagnostic guidelines, with positive proteinuria...
November 15, 2017: CEN Case Reports
https://www.readbyqxmd.com/read/29141309/-interpretation-of-guidelines-for-the-diagnosis-and-treatment-of-steroid-resistant-nephrotic-syndrome
#2
C L Gao, Z K Xia
No abstract text is available yet for this article.
November 2, 2017: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
https://www.readbyqxmd.com/read/29141308/-guidelines-for-the-diagnosis-and-treatment-of-steroid-resistant-nephrotic-syndrome
#3
(no author information available yet)
No abstract text is available yet for this article.
November 2, 2017: Zhonghua Er Ke za Zhi. Chinese Journal of Pediatrics
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/29098400/histone-deacetylase-2-expression-and-activity-in-children-with-nephrotic-syndrome-with-different-glucocorticoid-response
#7
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/29094388/peptide-gam-immunoadsorption-therapy-in-primary-membranous-nephropathy-prism-phase-ii-trial-investigating-the-safety-and-feasibility-of-peptide-gam-immunoadsorption-in-anti-pla2-r-positive-primary-membranous-nephropathy
#8
Patrick Hamilton, Durga Kanigicherla, Prasanna Hanumapura, Lars Walz, Dieter Kramer, Moritz Fischer, Paul Brenchley, Sandip Mitra
INTRODUCTION: Membranous nephropathy (MN) is among the most common causes of nephrotic syndrome in adults worldwide. Most patients have primary MN (PMN), an autoimmune condition associated with the IgG anti-PLA2 R autoantibody. For patients with severe disease, standard of care continues to be a 6-month regime of rotating high dose steroids and immunosuppression that comes with a significant side-effect profile. Immunoadsorption is a relatively safe procedure for the extracorporeal removal of specific immunoglobulins without the need for medications...
November 2, 2017: Journal of Clinical Apheresis
https://www.readbyqxmd.com/read/29080118/immunosuppressive-therapy-for-steroid-resistant-nephrotic-syndrome-a-bayesian-network-meta-analysis-of-randomized-controlled-studies
#9
Xinxin Jiang, Wei Shen, Xiujun Xu, Xiaogang Shen, Yiwen Li, Qiang He
BACKGROUND: The purpose of this study was to conduct a meta-analysis examining the efficacy of cyclophosphamide, cyclosporin, and tacrolimus in treating steroid resistant nephrotic syndrome. METHODS: Medline, Cochrane, EMBASE, and Google Scholar were searched until May 02, 2017 using the keywords: immunosuppressive therapy, steroid-resistant nephrotic syndrome, cyclophosphamide, cyclosporine A, and tacrolimus. Inclusion criteria were randomized controlled trials (RCTs) including patients with SRNS treated with an immunosuppressive therapy or placebo...
October 27, 2017: Clinical and Experimental Nephrology
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
#10
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/29058690/advillin-acts-upstream-of-phospholipase-c-%C3%AF%C2%B51-in-steroid-resistant-nephrotic-syndrome
#11
Jia Rao, Shazia Ashraf, Weizhen Tan, Amelie T van der Ven, Heon Yung Gee, Daniela A Braun, Krisztina Fehér, Sudeep P George, Amin Esmaeilniakooshkghazi, Won-Il Choi, Tilman Jobst-Schwan, Ronen Schneider, Johanna Magdalena Schmidt, Eugen Widmeier, Jillian K Warejko, Tobias Hermle, David Schapiro, Svjetlana Lovric, Shirlee Shril, Ankana Daga, Ahmet Nayir, Mohan Shenoy, Yincent Tse, Martin Bald, Udo Helmchen, Sevgi Mir, Afig Berdeli, Jameela A Kari, Sherif El Desoky, Neveen A Soliman, Arvind Bagga, Shrikant Mane, Mohamad A Jairajpuri, Richard P Lifton, Seema Khurana, Jose C Martins, Friedhelm Hildebrandt
Steroid-resistant nephrotic syndrome (SRNS) is a frequent cause of chronic kidney disease. Here, we identified recessive mutations in the gene encoding the actin-binding protein advillin (AVIL) in 3 unrelated families with SRNS. While all AVIL mutations resulted in a marked loss of its actin-bundling ability, truncation of AVIL also disrupted colocalization with F-actin, thereby leading to impaired actin binding and severing. Additionally, AVIL colocalized and interacted with the phospholipase enzyme PLCE1 and with the ARP2/3 actin-modulating complex...
October 23, 2017: Journal of Clinical Investigation
https://www.readbyqxmd.com/read/29058154/clinical-and-genetic-heterogeneity-in-familial-steroid-sensitive-nephrotic-syndrome
#12
Guillaume Dorval, Olivier Gribouval, Vanesa Martinez-Barquero, Eduardo Machuca, Marie-Josèphe Tête, Véronique Baudouin, Stéphane Benoit, Imen Chabchoub, Gérard Champion, Dominique Chauveau, Hassib Chehade, Chokri Chouchane, Sylvie Cloarec, Pierre Cochat, Karin Dahan, Jacques Dantal, Yahsou Delmas, Georges Deschênes, Phillippe Dolhem, Dominique Durand, Zelal Ekinci, Khalil El Karoui, Michel Fischbach, Jean-Pierre Grunfeld, Vincent Guigonis, Mongia Hachicha, Julien Hogan, Maryvonne Hourmant, Aurélie Hummel, Nassim Kamar, Thierry Krummel, Didier Lacombe, Brigitte Llanas, Laurent Mesnard, Nabil Mohsin, Patrick Niaudet, Hubert Nivet, Paloma Parvex, Christine Pietrement, Loic de Pontual, Claire Pouteil Noble, David Ribes, Pierre Ronco, Eric Rondeau, Marion Sallee, Michel Tsimaratos, Tim Ulinski, Rémi Salomon, Corinne Antignac, Olivia Boyer
BACKGROUND: Familial steroid-sensitive nephrotic syndrome (SSNS) is a rare condition. The disease pathophysiology remains elusive. However, bi-allelic mutations in the EMP2 gene were identified, and specific variations in HLA-DQA1 were linked to a high risk of developing the disease. METHODS: Clinical data were analyzed in 59 SSNS families. EMP2 gene was sequenced in families with a potential autosomal recessive (AR) inheritance. Exome sequencing was performed in a subset of 13 families with potential AR inheritance...
October 23, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/29056249/nephrutix-a-randomized-double-blind-placebo-vs-rituximab-controlled-trial-assessing-t-cell-subset-changes-in-minimal-change-nephrotic-syndrome
#13
Ahmed Boumediene, Pauline Vachin, Kelhia Sendeyo, Julie Oniszczuk, Shao-Yu Zhang, Carole Henique-Greciet, Andre Pawlak, Vincent Audard, Mario Ollero, Vincent Guigonis, Djillali Sahali
Minimal-change nephrotic syndrome (MCNS) is an immune-mediated glomerular disease. We have analyzed the modifications on T-cell subsets in twenty-three patients who were highly steroid/calcineurin inhibitor and/or mycophenolate mofetil-dependent for frequently relapsing nephrotic syndrome (FRNS) and who were enrolled in a multicenter, double-blind, randomized, placebo vs Rituximab-controlled trial. Patients with FRNS entered the trial at remission and were randomly assigned to receive either Rituximab or placebo...
October 19, 2017: Journal of Autoimmunity
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
#14
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
#15
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
#16
(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/29022104/deriving-and-understanding-the-risk-of-post-transplant-recurrence-of-nephrotic-syndrome-in-the-light-of-current-molecular-and-genetic-advances
#17
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/28992850/association-of-single-nucleotide-polymorphism-in-the-fkbp5-gene-with-response-to-steroids-in-pediatric-patients-with-primary-nephrotic-syndrome%C3%A2
#18
Ni Du, Fang Yang, Liangzhi Li, Xiaoxiao Liu, Liangzhong Sun, Sui Zhang, Xianhong He, Yuewu Tang, Jihong Shi, Chunqiu Liu, Xianxiang Zhang
AIM: This study aimed to assess the association of single-nucleotide polymorphism (SNP) of FKBP5 with response to steroids in children with primary nephrotic syndrome (NS). MATERIALS AND METHODS: A total of 66 primary NS patients (cases) and 68 healthy individuals (controls) were enrolled in this study. The FKBP5 polymorphism rs4713916 (T/C) was analyzed by polymerase chain reaction (PCR) and sequencing after amplification of regions that potentially contain the SNP...
October 10, 2017: Clinical Nephrology
https://www.readbyqxmd.com/read/28990100/low-molecular-weight-heparin-may-benefit-nephrotic-remission-in-steroid%C3%A2-sensitive-nephrotic-syndrome-via-inhibiting-elastase
#19
Songhui Zhai, Lijuan Hu, Lin Zhong, Yuhong Tao, Zheng Wang
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)...
December 2017: Molecular Medicine Reports
https://www.readbyqxmd.com/read/28979773/efficacy-of-mycophenolate-treatment-in-adults-with-steroid-dependent-frequently-relapsing-idiopathic-nephrotic-syndrome
#20
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
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