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https://www.readbyqxmd.com/read/28326197/childhood-nephrotic-syndrome-management-and-outcome-a-single-center-retrospective-analysis
#1
Chia-Shi Wang, Jia Yan, Robert Palmer, James Bost, Mattie Feasel Wolf, Larry A Greenbaum
There is a paucity of information on outpatient management and risk factors for hospitalization and complications in childhood nephrotic syndrome (NS). We described the management, patient adherence, and inpatient and outpatient usage of 87 pediatric NS patients diagnosed between 2006 and 2012 in the Atlanta Metropolitan Statistical Area. Multivariable analyses were performed to examine the associations between patient characteristics and disease outcome. We found that 51% of the patients were treated with two or more immunosuppressants...
2017: International Journal of Nephrology
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
#2
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/28318625/mycophenolate-mofetil-is-inferior-to-tacrolimus-in-sustaining-remission-in-children-with-idiopathic-steroid-resistant-nephrotic-syndrome
#3
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
#4
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
https://www.readbyqxmd.com/read/28314579/keeping-the-customers-stratified-moving-toward-genetics-based-treatment-options-in-childhood-ns
#5
Megan Puckelwartz, H William Schnaper
In this issue, Bierzynska et al. report the results of analyzing a cohort of children with steroid-resistant nephrotic syndrome (SRNS) of childhood, correlating genetic studies with clinical outcomes. This approach has the potential to advance both our research into the causes and treatments of childhood SRNS and our ability to stratify patients into groups that are likely to be responsive to specific treatments. Here, we discuss some of the strengths and limitations of this report.
April 2017: Kidney International
https://www.readbyqxmd.com/read/28303389/variability-of-diagnostic-criteria-and-treatment-of-idiopathic-nephrotic-syndrome-across-european-countries
#6
Georges Deschênes, Marina Vivarelli, Licia Peruzzi
The aim of the surveys conducted by the Idiopathic Nephrotic Syndrome Working Group of the ESPN was to study the possible variability of treatment in Europe at different stages of the disease by means of questionnaires sent to members of the Working Group. Four surveys have been completed: treatment of the first flare, treatment of the first relapse and the issue of steroid dependency, use of rituximab, and the management of steroid-resistant patients. A uniform treatment of the first flare was applied in only three countries, and ten additional centers have adopted one of the three main protocols...
March 16, 2017: European Journal of Pediatrics
https://www.readbyqxmd.com/read/28299461/efficacy-of-higher-dose-levamisole-in-maintaining-remission-in-steroid-dependant-nephrotic-syndrome
#7
Asiri S Abeyagunawardena, Umeshi Karunadasa, Heshan Jayaweera, Shenal Thalgahagoda, Sampath Tennakoon, Shamali Abeyagunawardena
OBJECTIVE: Levamisole (LEV) has been used successfully on an alternate-day regime of 2.5 mg/kg in steroid-dependant nephrotic syndrome (SDNS) to maintain remission. This pilot study was carried out between 2010 and 2015 at a single center in Sri Lanka to evaluate the efficacy of LEV prescribed at 2.5 mg/kg daily, which is double the alternate-day dose. METHODS: Sequential children with SDNS, relapsing more than twice in the preceding 12 months and previously treated with LEV and low-dose alternate-day prednisolone (0...
March 15, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28299339/cytoplasmic-localization-of-wt1-and-decrease-of-mirna-16-1-in-nephrotic-syndrome
#8
Pablo Zapata-Benavides, Mariela Arellano-Rodríguez, Juan José Bollain-Y-Goytia, Moisés Armides Franco-Molina, Gloria Azucena Rangel-Ochoa, Esperanza Avalos-Díaz, Rafael Herrera-Esparza, Cristina Rodríguez-Padilla
Nephrotic syndrome (NS) is a glomerular disease that is defined by the leakage of protein into the urine and is associated with hypoalbuminemia, hyperlipidemia, and edema. Steroid-resistant NS (SRNS) patients do not respond to treatment with corticosteroids and show decreased Wilms tumor 1 (WT1) expression in podocytes. Downregulation of WT1 has been shown to be affected by certain microRNAs (miRNAs). Twenty-one patients with idiopathic NS (68.75% were SSNS and 31.25% SRNS) and 10 healthy controls were enrolled in the study...
2017: BioMed Research International
https://www.readbyqxmd.com/read/28298181/early-onset-of-adck4-glomerulopathy-with-renal-failure-a-case-report
#9
Ksenija Lolin, Benedetta D Chiodini, Elise Hennaut, Brigitte Adams, Karin Dahan, Khalid Ismaili
BACKGROUND: We present a rare early presentation of a ADCK4-related glomerulopathy. This case is of interest as potentially treatable if genetic results are timely obtained. CASE PRESENTATION: We report the case of a 5-year-old boy who was identified with significant proteinuria by a urinary routine screening program for school children. Physical examination revealed dysplastic ears and abnormal folded pinna. Albumin level was 41 g/L (39-53 g/L), and urine proteins/creatinine ratio was 2...
March 16, 2017: BMC Medical Genetics
https://www.readbyqxmd.com/read/28288230/-somatotropic-axis-and-molecular-markers-of-mineral-metabolism-in-children-undergoing-chronic-peritoneal-dialysis
#10
María Luisa Ceballos Osorio, Francisco Cano Schuffeneger
Growth failure is one of the most relevant complications in children with chronic kidney disease (CKD). Among others, growth hormone (GH) resistance and bone mineral disorders have been identified as the most important causes of growth retardation. OBJECTIVES: 1. To characterize bone mineral metabolism and growth hormone bio-markers in CKD children treated with chronic peritoneal dialysis (PD). 2. To evaluate height change with rhGH treatment. PATIENTS AND METHOD: A longitudinal 12-month follow-up in prepuberal PD children...
February 2017: Revista Chilena de Pediatría
https://www.readbyqxmd.com/read/28261934/steroid-induced-impairment-of-glucose-tolerance-prevalence-among-pediatric-patients-on-long-term-steroid-use-in-nigeria
#11
U N Chikani, M U Ibekwe, T Oguonu, L Mungai, A I Bisi-Onyemaechi, O M Ugege, I F Ogbonna, Carine de Beaufort
INTRODUCTION: Glucocorticoid (referred to from here on as simply steroid) is used for effective treatment of various inflammatory disorders since its discovery in 1940s. However, these useful drugs cause important side effects, such as impairment of glucose tolerance. We sought to determine the prevalence of steroid-induced impairment of glucose tolerance in pediatric patients on long-term steroid use. MATERIALS AND METHODS: A cross-sectional, descriptive and hospital-based study...
March 6, 2017: Pediatric Diabetes
https://www.readbyqxmd.com/read/28257282/wt1-mutation-associated-nephropathy-a%C3%A2-single-center-experience%C3%A2
#12
Zhihui Yue, Haiyan Wang, Hongrong Lin, Juan Yang, Ting Liu, Yulin Liu, Huamu Chen, Liangzhong Sun
This study explored Wilms' tumor 1 (WT1) mutations in children with, or suspected of having, steroid-resistant nephrotic syndrome (SRNS), referred to or treated in our hospital in the past 6 years as well as the correlation between genotype and phenotype in WT1 mutation-associated nephropathy in Chinese patients. In total, 76 patients participated in the study. WT1 mutations were identified in 15 patients, 5 of whom harbored splice-site mutations in intron 9. Four of these 5 patients exhibited early onset of nephropathy and rapid deterioration of renal function...
March 3, 2017: Clinical Nephrology
https://www.readbyqxmd.com/read/28250989/apolipoprotein-c-i-levels-are-associated-with-the-urinary-protein-urinary-creatinine-ratio-in-pediatric-idiopathic-steroid-sensitive-nephrotic-syndrome-a-case-control-study
#13
Jun Odaka, Takahiro Kanai, Takane Ito, Takashi Saito, Jun Aoyagi, Hiroyuki Betsui, Takanori Yamagata
Humoral factors may cause idiopathic steroid-sensitive nephrotic syndrome (ISSNS). In the present study, we analyzed serum proteins using mass spectrometry (MS) to identify proteins associated with the pathophysiology of pediatric ISSNS. We collected serial serum samples from 33 children during each ISSNS phase; Phase A1 is the acute phase prior to steroid treatment (STx), Phase A2 represents the remission period with STx, and Phase A3 represents the remission period after completion of STx. Children with normal urinalyses (Group B) and children with a nephrotic syndrome other than ISSNS (Group C) served as controls...
2017: International Journal of Nephrology
https://www.readbyqxmd.com/read/28213687/ofatumumab-for-the-treatment-of-childhood-nephrotic-syndrome
#14
Chia-Shi Wang, Rochelle Schmidt Liverman, Rouba Garro, Roshan Punnoose George, Anastacia Glumova, Alana Karp, Stephanie Jernigan, Barry Warshaw
BACKGROUND: Ofatumumab is a humanized anti-CD20 monoclonal antibody that has recently garnered interest as a potential therapeutic agent for nephrotic syndrome. We report our center's experience in administering ofatumumab to five pediatric patients with idiopathic nephrotic syndrome. METHODS: Between March 2015 and November 2016, five patients were treated with ofatumumab. One patient had post-transplant recurrent focal segmental glomerulosclerosis (FSGS) which had been resistant to plasmapheresis and numerous immunosuppressive agents...
February 17, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28213606/parental-health-literacy-and-outcomes-of-childhood-nephrotic-syndrome
#15
Karlota Borges, Cathryn Sibbald, Neesha Hussain-Shamsy, Jovanka Vasilevska-Ristovska, Tonny Banh, Viral Patel, Josefina Brooke, Monica Piekut, Michele Reddon, Kimberly Aitken-Menezes, Ashley McNaughton, Rachel J Pearl, Valerie Langlois, Seetha Radhakrishnan, Christoph P B Licht, Tino D Piscione, Leo Levin, Damien Noone, Diane Hebert, Rulan S Parekh
OBJECTIVE: Determine the association of parental health literacy with treatment response among children with nephrotic syndrome. METHODS: This was a cohort study of children aged 1-18 with nephrotic syndrome and their parent. Health literacy was measured using the validated Short Test of Functional Health Literacy in Adults assessing reading comprehension and numeracy. Outcomes included initial relapse-free period, frequently relapsing disease, relapse rate, second-line medication use, and complete remission after therapy...
February 17, 2017: Pediatrics
https://www.readbyqxmd.com/read/28210837/toll-like-receptor-3-tlr-3-tlr-4-and-cd80-expression-in-peripheral-blood-mononuclear-cells-and-urinary-cd80-levels-in-children-with-idiopathic-nephrotic-syndrome
#16
Om P Mishra, Ravindra Kumar, Gopeshwar Narayan, Pradeep Srivastava, Abhishek Abhinay, Rajniti Prasad, Ankur Singh, Vineeta V Batra
BACKGROUND: The aims of this study were (1) to detect toll-like receptor (TLR)-3, TLR-4 and CD80 expression in peripheral blood mononuclear cells (PBMCs) and estimate urinary CD80 levels in children with idiopathic nephrotic syndrome and (2) to investigate the utility of these markers to differentiate between biopsy-proven minimal change disease (MCD) and focal segmental glomeruloscelerosis (FSGS). METHODS: The study included 70 patients with idiopathic nephrotic syndrome (NS), of whom 40 had steroid-sensitive NS (SSNS; 25 with active NS, 15 in remission) and 30 had steroid-resistant NS (SRNS) patients, and 23 healthy controls...
February 16, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28204946/clinical-outcomes-in-children-with-henoch-sch%C3%A3-nlein-purpura-nephritis-without-crescents
#17
Jean Daniel Delbet, Julien Hogan, Bilal Aoun, Iulia Stoica, Rémi Salomon, Stéphane Decramer, Isabelle Brocheriou, Georges Deschênes, Tim Ulinski
BACKGROUND: Henoch-Schönlein purpura is the most common vasculitis in children. Its long-term prognosis depends on renal involvement. The management of Henoch-Schönlein purpura nephritis (HSPN) remains controversial. This study reports the prognosis of children with HSPN presenting with class 2 International Study of Kidney Disease in Children (ISKDC) nephritis. METHODS: All children with HSPN class 2 diagnosed between 1995 and 2015 in four pediatric nephrology centers were included, and clinical and biological data were collected from the medical files...
February 15, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
https://www.readbyqxmd.com/read/28204945/spectrum-of-mutations-in-chinese-children-with-steroid-resistant-nephrotic-syndrome
#18
Fang Wang, Yanqin Zhang, Jianhua Mao, Zihua Yu, Zhuwen Yi, Li Yu, Jun Sun, Xiuxiu Wei, Fangrui Ding, Hongwen Zhang, Huijie Xiao, Yong Yao, Weizhen Tan, Svjetlana Lovric, Jie Ding, Friedhelm Hildebrandt
BACKGROUND: The aim of this study was to elucidate whether genetic screening test results of pediatric patients with steroid-resistant nephrotic syndrome (SRNS) vary with ethnicity. METHODS: Using high-throughput DNA sequencing, 28 nephrotic syndrome-related genes were analyzed in 110 chil-dren affected by SRNS and 10 children with isolated proteinuria enrolled by 5 centers in China (67 boys, 53 girls). Their age at disease onset ranged from 1 day to 208 months (median, 48...
February 15, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
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
#19
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/28175986/does-zika-virus-infection-induce-prolonged-remissions-in-children-with-idiopathic-nephrotic-syndrome
#20
Carolina Peralta-Aros, Víctor García-Nieto
BACKGROUND: Zika is an emerging mosquito-borne flavivirus. We report two pediatric patients diagnosed with idiopathic nephrotic syndrome who achieved complete remission of the disease after suffering Zika virus (ZIKV) infection. CASE DIAGNOSIS/TREATMENT: The first patient was a young girl aged 2.5 years with steroid-dependent nephrotic syndrome who was subsequently diagnosed with ZIKV infection. Following the infection, the steroid dose could be reduced until complete withdrawal...
February 7, 2017: Pediatric Nephrology: Journal of the International Pediatric Nephrology Association
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