scholarly journals Practice variations in the management of childhood nephrotic syndrome in the Netherlands

2021 ◽  
Author(s):  
Anne M. Schijvens ◽  
Lucie van der Weerd ◽  
Joanna A. E. van Wijk ◽  
Antonia H. M. Bouts ◽  
Mandy G. Keijzer-Veen ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
The Netherlands ◽  
Kidney Biopsy ◽  
Immunosuppressive Treatment ◽  
Pediatric Nephrology ◽  
Practice Variation ◽  
Childhood Nephrotic Syndrome ◽  
Steroid Dependent ◽  
Practice Variations ◽  
Steroid Sparing

AbstractNephrotic syndrome in childhood is a common entity in the field of pediatric nephrology. The optimal treatment of children with nephrotic syndrome is often debated. Previously conducted studies have shown significant variability in nephrotic syndrome management, especially in the choice of steroid-sparing drugs. In the Netherlands, a practice guideline on the management of childhood nephrotic syndrome has been available since 2010. The aim of this study was to identify practice variations and opportunities to improve clinical practice of childhood nephrotic syndrome in the Netherlands. A digital structured survey among Dutch pediatricians and pediatric nephrologists was performed, including questions regarding the initial treatment, relapse treatment, kidney biopsy, additional immunosuppressive treatment, and supportive care. Among the 51 responses, uniformity was seen in the management of a first presentation and first relapse. Wide variation was found in the tapering of steroids after alternate day dosing. Most pediatricians and pediatric nephrologists (83%) would perform a kidney biopsy in case of steroid-resistant nephrotic syndrome, whereas for frequent relapsing and steroid-dependent nephrotic syndrome this was 22% and 41%, respectively. Variation was reported in the steroid-sparing treatment. Finally, significant differences were present in the supportive treatment of nephrotic syndrome.Conclusion: Substantial variation was present in the management of nephrotic syndrome in the Netherlands. Differences were identified in steroid tapering, use of steroid coverage during stress, choice of steroid-sparing agents, and biopsy practice. To promote guideline adherence and reduce practice variation, factors driving this variation should be assessed and resolved. What is Known:• National and international guidelines are available to guide the management of childhood nephrotic syndrome.• Several aspects of the management of childhood nephrotic syndrome, including the choice of steroid-sparing drugs and biopsy practice, are controversial and often debated among physicians.What is New:• Significant practice variation is present in the management of childhood nephrotic syndrome in the Netherlands, especially in the treatment of FRNS, SDNS, and SRNS.• The recommendation on the steroid treatment of a first episode of nephrotic syndrome in the KDIGO guideline leaves room for interpretation and is likely the cause of substantial differences in steroid-tapering practices among Dutch pediatricians and pediatric nephrologists.

scholarly journals Practice patterns and outcomes of repository corticotropin injection (Acthar® Gel) use in childhood nephrotic syndrome: A study of the North American Pediatric Renal Trials and collaborative studies and the Pediatric Nephrology Research Consortium

2021 ◽  
Vol 5 (2) ◽  
pp. 067-076
Author(s):  
Faizan Mohammed K ◽  
McCracken Courtney ◽  
Lieberman Kenneth ◽  
Leong Traci ◽  
Benfield Mark R
Keyword(s):  
Nephrotic Syndrome ◽  
Demographic Data ◽  
Pediatric Nephrology ◽  
Drug Regimen ◽  
Disease Response ◽  
Childhood Nephrotic Syndrome ◽  
The North ◽  
Set Up ◽  
Steroid Responsiveness ◽  
Active Disease

Objective: We set up a U.S. registry to examine prescription patterns and patient outcomes of repository corticotropin injection (Acthar® Gel) for childhood nephrotic syndrome. Methods: 18 participating U.S. pediatric centers performed retrospective review and prospective observation of patients < 21 years old with nephrotic syndrome treated with Acthar Gel. We captured baseline characteristics, drug regimen and duration, and disease response following treatment. Results: 46 patients, enrolled from 2015 to 2020 were included. 27 (58.7%) were male. 18 patients (39.1%) had a diagnosis of minimal change followed by focal segmental glomerulosclerosis in 16 patients (34.7%). Median age at start of treatment was 12.5 years (IQR 8.5-17.4) compared to 5.3 years at diagnosis (IQR 2.7-10.5 years). 52% were resistant to corticosteroids. The most common Acthar Gel regimen was 80IU twice a week with a median duration of 199 days (IQR 88-365). Among 37 patients with active disease, 18 (49%) were able to achieve partial or complete remission, though all patients that had a positive response were on other immunosuppressants concomitantly. Conclusion: We report the findings of the largest registry cohort of pediatric patients in the U.S. treated with Acthar Gel for clinically challenging cases of nephrotic syndrome. Acthar Gel was successful in inducing remission in approximately half of the patients with active disease at time of treatment. No predictors of response with respect to demographic data, age at start of Acthar Gel therapy, etiology of nephrotic syndrome, presence or absence of comorbidities, or steroid responsiveness was noted.

scholarly journals Spectrum of Biopsy Proven Glomerular Disease in Children at Kanti Children’s Hospital

2015 ◽  
Vol 34 (3) ◽  
pp. 225-229 ◽  
Author(s):  
N Khatun ◽  
KP Bista ◽  
C Mahaseth
Keyword(s):  
Nephrotic Syndrome ◽  
Lupus Nephritis ◽  
Renal Disease ◽  
Iga Nephropathy ◽  
Kidney Biopsy ◽  
Demographic Data ◽  
Glomerular Disease ◽  
Children's Hospital ◽  
Steroid Dependent ◽  
Children’S Hospital

Introduction: Glomerular disease is one of the most common forms of renal disease and can have many different clinical presentations. However there is variation in the prevalence in the type of glomerular disease according to geographical location and race of population. The aim of the study was to find the overall distribution pattern of glomerular disease based on renal biopsies. Material and Methods: The medical records of all children who underwent kidney biopsy (n=29) between January 2012-june 2014 were analyzed. In this retrospective study we review children from Kanti Children’s Hospital, Nephrology Department. Demographic data including age, sex and indication of kidney biopsy as well as complication of the procedures were recorded. Result: A total number of thirty one biopsies were done. Two children were excluded from the study due to inadequate tissue and artifacts defects in preservative. The remaining twenty nine biopsies were included in the study and analyzed. Renal disease was found nearly equal in both male and female (51.72% vs. 48.27%).Mean age was 10.95±3.30 years. Maximum number of biopsies (75.86%) was performed between 10- 15 years. The most common indication of the kidney biopsy was nephrotic syndrome (steroid resistant nephrotic syndrome and steroid dependent nephrotic syndrome, 31.02% followed by lupus nephritis 27.58%.The most common glomerular disease were focal segmental glomerulosclerosis and lupus nephritis both contribute 27.58%. Among lupus nephritis class III was found more common (44.44%) in children. IgA nephropathy was also contribute (17.24%) in all glomerular disease. Regarding the kidney biopsy complication gross hematuria was observed in 6.89% of children which was self-limited. Conclusion: FSGS and lupus nephritis is becoming the most common glomerular disease in children attending the Kanti Children Hospital. IgA nephropathy was also contribute the glomerular disease in the children. J Nepal Paediatr Soc 2014;34(3):225-229   DOI: http://dx.doi.org/10.3126/jnps.v34i3.11162  

scholarly journals Childhood Nephrotic Syndrome Management and Outcome: A Single Center Retrospective Analysis

2017 ◽  
Vol 2017 ◽  
pp. 1-7 ◽  
Author(s):  
Chia-shi Wang ◽  
Jia Yan ◽  
Robert Palmer ◽  
James Bost ◽  
Mattie Feasel Wolf ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Length Of Stay ◽  
Hospital Length ◽  
Hospital Length Of Stay ◽  
High Morbidity ◽  
Metropolitan Statistical Area ◽  
Childhood Nephrotic Syndrome ◽  
Steroid Dependent ◽  
Increased Risk ◽  
Serious Disease

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. Approximately half of the patients were noted to be nonadherent to medications and urine protein monitoring. The majority (71%) of patients were hospitalized at least once, with a median rate of 0.5 hospitalizations per patient year. Mean hospital length of stay was 4.0 (3.8) days. Fourteen percent of patients experienced at least one serious disease complication. Black race, frequently relapsing/steroid-dependent and steroid-resistant disease, and the first year following diagnosis were associated with higher hospitalization rates. The presence of comorbidities was associated with longer hospital length of stay and increased risk of serious disease complications. Our results highlight the high morbidity and burden of NS and point to particular patient subgroups that may be at increased risk for poor outcome.

scholarly journals Use of Cyclosporine Therapy in Steroid Resistant Nephrotic Syndrome (SRNS): A Review

2015 ◽  
Vol 8 (4) ◽  
pp. 136 ◽  
Author(s):  
Syed Raza Shah ◽  
Areeba Altaf ◽  
Mohammad Hussham Arshad ◽  
Anum Mari ◽  
Sahir Noorani ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Side Effects ◽  
Immunosuppressive Treatment ◽  
Pediatric Nephrology ◽  
Calcineurin Inhibitors ◽  
Symptomatic Treatment ◽  
Steroid Resistant ◽  
Steroid Resistant Nephrotic Syndrome ◽  
Cyclosporine Therapy ◽  
Difficult Challenge

<p>A chronic, progressive disorder Steroid Resistant Nephrotic Syndrome (SRNS) accounts for 10-20% of all children with Nephrotic Syndrome. It is a heterogeneous disorder comprised of persistent edema, proteinuria, hypoalbuminemia and hyperlipidemia. Treatment for steroid-resistant nephrotic syndrome (SRNS) is challenging and children who suffer from SRNS require aggressive treatment to achieve remission. Calcineurin inhibitors have been used more in an empirical manner than on the basis of clear rationale. It was in 1984 when cyclosporine was first considered for the treatment of steroid resistant nephrotic syndrome. Cyclosporin is a calcineurin inhibitor that suppresses immune response by downregulating the transcription of various cytokine genes. Till now many studies have been conducted to determine dosages, duration of therapy, side effects and advantages of cyclosporine. Treatment of SRNS remains a difficult challenge in pediatric nephrology.  Treatment should be individualized according to the underlying histopathology, and clinical and environmental conditions of the children. There is an urgent need to distinguish as soon as possible those patients who may benefit from prolonged immunosuppressive treatment from those who will not benefit from such treatment and who will just suffer from its major side effects. The emerging evidence that the majority of genetic forms of SRNS should receive symptomatic treatment only, should also be clinically tested and studies baring its significance should be evaluated in the future.</p>

scholarly journals Factors influencing practice variation in the management of nephrotic syndrome: a qualitative study of pediatric nephrology care providers

CMAJ Open ◽  
2017 ◽  
Vol 5 (2) ◽  
pp. E424-E430 ◽  
Author(s):  
Susan M. Samuel ◽  
Rachel Flynn ◽  
Michael Zappitelli ◽  
Allison Dart ◽  
Rulan Parekh ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Qualitative Study ◽  
Pediatric Nephrology ◽  
Practice Variation ◽  
Care Providers ◽  
Factors Influencing ◽  
Nephrology Care

Steroid sparing effect of a herbal preparation in steroid-dependent nephrotic syndrome

2006 ◽  
Vol 21 (7) ◽  
pp. 1031-1033 ◽  
Author(s):  
Arpana Iyengar ◽  
Hrishikesh Damle ◽  
Chanda Kulkarni ◽  
Latha Damle ◽  
Kishore Phadke
Keyword(s):  
Nephrotic Syndrome ◽  
Herbal Preparation ◽  
Steroid Dependent ◽  
Sparing Effect ◽  
Steroid Sparing ◽  
Steroid Dependent Nephrotic Syndrome

scholarly journals Long term tapering versus standard prednisolone treatment for first episode of childhood nephrotic syndrome: phase III randomised controlled trial and economic evaluation

BMJ ◽  
2019 ◽  
pp. l1800 ◽  
Author(s):  
Nicholas J A Webb ◽  
Rebecca L Woolley ◽  
Tosin Lambe ◽  
Emma Frew ◽  
Elizabeth A Brettell ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Confidence Interval ◽  
Immunosuppressive Treatment ◽  
Phase Iii ◽  
Prednisolone Treatment ◽  
Steroid Dependent ◽  
Life Years ◽  
Steroid Sensitive Nephrotic Syndrome ◽  
Steroid Sensitive ◽  
Extended Course

Abstract Objective To determine whether extending initial prednisolone treatment from eight to 16 weeks in children with idiopathic steroid sensitive nephrotic syndrome improves the pattern of disease relapse. Design Double blind, parallel group, phase III randomised placebo controlled trial, including a cost effectiveness analysis. Setting 125 UK National Health Service district general hospitals and tertiary paediatric nephrology centres. Participants 237 children aged 1-14 years with a first episode of steroid sensitive nephrotic syndrome. Interventions Children were randomised to receive an extended 16 week course of prednisolone (total dose 3150 mg/m 2 ) or a standard eight week course of prednisolone (total dose 2240 mg/m 2 ). The drug was supplied as 5 mg tablets alongside matching placebo so that participants in both groups received the same number of tablets at any time point in the study. A minimisation algorithm ensured balanced treatment allocation by ethnicity (South Asian, white, or other) and age (5 years or less, 6 years or more). Main outcome measures The primary outcome measure was time to first relapse over a minimum follow-up of 24 months. Secondary outcome measures were relapse rate, incidence of frequently relapsing nephrotic syndrome and steroid dependent nephrotic syndrome, use of alternative immunosuppressive treatment, rates of adverse events, behavioural change using the Achenbach child behaviour checklist, quality adjusted life years, and cost effectiveness from a healthcare perspective. Analysis was by intention to treat. Results No significant difference was found in time to first relapse (hazard ratio 0.87, 95% confidence interval 0.65 to 1.17, log rank P=0.28) or in the incidence of frequently relapsing nephrotic syndrome (extended course 60/114 (53%) v standard course 55/109 (50%), P=0.75), steroid dependent nephrotic syndrome (48/114 (42%) v 48/109 (44%), P=0.77), or requirement for alternative immunosuppressive treatment (62/114 (54%) v 61/109 (56%), P=0.81). Total prednisolone dose after completion of the trial drug was 6674 mg for the extended course versus 5475 mg for the standard course (P=0.07). There were no statistically significant differences in serious adverse event rates (extended course 19/114 (17%) v standard course 27/109 (25%), P=0.13) or adverse event rates, with the exception of behaviour, which was poorer in the standard course group. Scores on the Achenbach child behaviour checklist did not, however, differ. Extended course treatment was associated with a mean increase in generic quality of life (0.0162 additional quality adjusted life years, 95% confidence interval −0.005 to 0.037) and cost savings (difference −£1673 ($2160; €1930), 95% confidence interval −£3455 to £109). Conclusions Clinical outcomes did not improve when the initial course of prednisolone treatment was extended from eight to 16 weeks in UK children with steroid sensitive nephrotic syndrome. However, evidence was found of a short term health economic benefit through reduced resource use and increased quality of life. Trial registration ISRCTN16645249; EudraCT 2010-022489-29.

scholarly journals Relative safety of steroid sparing agents in frequently relapsing, steroid dependent and steroid resistant nephrotic syndrome in pediatric patients

2020 ◽  
Vol 7 (8) ◽  
pp. 1762
Author(s):  
Sabnam Ara Begum ◽  
Santanu Kumar Tripathi ◽  
Mousumi Nandy ◽  
Sanat Kumar Ghosh ◽  
Shatavisa Mukherjee ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Adverse Events ◽  
Tertiary Care ◽  
Serious Adverse Events ◽  
Steroid Resistant ◽  
Steroid Resistant Nephrotic Syndrome ◽  
Steroid Dependent ◽  
Relative Safety ◽  
Tertiary Care Hospitals ◽  
Steroid Sparing

Background: In patients with frequently relapsing nephrotic syndrome (FRNS), steroid-dependent nephrotic syndrome (SDNS) and steroid resistant nephrotic syndrome (SRNS) steroids are either used for prolonged period of time or ineffective. To reduce the degree of steroid dependency and avoid steroid toxicity, several immunosuppressive steroid sparing agents (SPAs) have been proposed to treat these children. The present study tried to study the relative safety of most commonly steroid sparing agent in such children.Methods: A multi-centred, prospective observational study was conducted in paediatric nephrology OPD of two tertiary care hospitals in Kolkata over a period of 24 months. All consecutive children with diagnosed FRNS, SDNS and SRNS who were started on steroid sparing agents were enrolled and followed up for at least 6 months. Records of clinical examination, laboratory tests were collected and measured at the baseline and regular intervals. Safety parameters were noted and statistically analysed.Results: A total 110 patients were screened, examined and enrolled. Levamisole, cyclophosphamide and MMF were commonly used SPAs. Of the two tertiary care hospitals, all the patients of FRNS and SDNS were started with levamisole and SRNS with cyclophosphamide in one set-up, while in the other hospital some SDNS patients were started with cyclophosphamide and SRNS with MMF but without clinically significant outcomes. In comparison with few minor adverse events in MMF group, some serious adverse events were documented in the both cyclophosphamide and levamisole groups.Conclusions: Levamisole being a very efficacious, safe and easily affordable drug, should be used as an initial first line SPA in treating FRNS and SDNS children. The side effect profiles of levamisole and MMF are much more patient friendly.

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