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 P-glycoprotein and/or Histone Deacetylase 2 Regulates Steroid Responsiveness in Childhood Nephrotic Syndrome

2020 ◽  
Vol 15 (1) ◽  
pp. 5
Author(s):  
Vikas Agarwal ◽  
Harshit Singh ◽  
Narayan Prasad ◽  
DurgaPrasanna Misra ◽  
AkhileshKumar Jaiswal
Keyword(s):  
Nephrotic Syndrome ◽  
Histone Deacetylase ◽  
Histone Deacetylase 2 ◽  
Childhood Nephrotic Syndrome ◽  
P Glycoprotein ◽  
Steroid Responsiveness

scholarly journals SAT-468 Can steroid responsiveness be regulated epigenetically in childhood nephrotic syndrome?

2020 ◽  
Vol 5 (3) ◽  
pp. S196
Author(s):  
H. SINGH PhD ◽  
N. Prasad ◽  
A. Jaiswal ◽  
D. Misra ◽  
V. Agarwal
Keyword(s):  
Nephrotic Syndrome ◽  
Childhood Nephrotic Syndrome ◽  
Steroid Responsiveness

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.

The Rotterdam Initiative in Reducing Waste Discharges at Source

1991 ◽  
Vol 24 (10) ◽  
pp. 171-177
Author(s):  
T. Vellinga ◽  
J. P. J. Nijssen
Keyword(s):  
Public Relations ◽  
Action Plan ◽  
Detailed Balance ◽  
River Rhine ◽  
The North ◽  
Complementary Role ◽  
Action Programme ◽  
The North Sea ◽  
Set Up

Much of the material dredged from the port of Rotterdam is contaminated to such a degree that it must be placed in specially constructed sites. The aim of Rotterdam is to ensure that the dredged material will once again be clean. This will entail the thorough cleansing of the sources of the contamination of the sediment in the harbours and in the River Rhine. The Rotterdam Rhine Research Project (RRP) is one of the means to achieve this based on: technical research, legal research, public relations and dialogues with dischargers. The programme for five selected heavy metals is almost complete. For many heavy metal discharge points between Rotterdam and Rheinfelden, a specially devised independent load assessment has been carried out four times. Balance studies were used to determine the relative contributions of the point discharges to the total. Currently the results are being used in an attempt to negotiate agreements with a selected number of the major dischargers. At present, more detailed balance studies are being set up and exploratory measurements carried out for organic micropollutants. It may be concluded that the research is progressing successfully and methods and techniques developed seem satisfactory and broadly applicable. The Rhine Action Programme encompasses an international effort to improve the quality of the Rhine water. Although the RRP plays a modest complementary role to the Rhine Action Plan, there is no doubt of the value of this Rotterdam initiative. The mode of work followed in the RRP contains elements that can be of use in combatting the contamination of the North Sea by rivers other than the Rhine.

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