Epidemiology and Risk Factors for Thromboembolic Complications of Childhood Nephrotic Syndrome: A Midwest Pediatric Nephrology Consortium (MWPNC) Study

2011 ◽  
Vol 2011 ◽  
pp. 194-196
Author(s):  
J.A. Stockman
2021 ◽  
Vol 5 (2) ◽  
pp. 067-076
Author(s):  
Faizan Mohammed K ◽  
McCracken Courtney ◽  
Lieberman Kenneth ◽  
Leong Traci ◽  
Benfield Mark R

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.


2019 ◽  
Vol 12 (1) ◽  
pp. 90-94 ◽  
Author(s):  
Khalid Alfakeekh ◽  
Mohammed Azar ◽  
Banan Al Sowailmi ◽  
Saja Alsulaiman ◽  
Salwa Al Makdob ◽  
...  

1996 ◽  
Vol 10 (6) ◽  
pp. 740-741 ◽  
Author(s):  
Atsushi Takeda ◽  
Hidetomo Matsutani ◽  
Fumio Niimura ◽  
Hiroaki Ohgushi

2013 ◽  
Vol 79 (06) ◽  
pp. 454-462 ◽  
Author(s):  
Melisa Sahin ◽  
Sultan Ozkurt ◽  
Nevbahar Akcar Degirmenci ◽  
Ahmet Musmul ◽  
Gokhan Temiz ◽  
...  

Author(s):  
Anne M. Schijvens ◽  
Lucie van der Weerd ◽  
Joanna A. E. van Wijk ◽  
Antonia H. M. Bouts ◽  
Mandy G. Keijzer-Veen ◽  
...  

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.


2013 ◽  
Vol 18 (5) ◽  
pp. 803-813 ◽  
Author(s):  
Deepti Suri ◽  
Jasmina Ahluwalia ◽  
Akshay K. Saxena ◽  
Kushaljit S. Sodhi ◽  
Paramjeet Singh ◽  
...  

2014 ◽  
Vol 19 (3) ◽  
pp. 506-513 ◽  
Author(s):  
Betul Tavil ◽  
Fehime Kara ◽  
Rezan Topaloglu ◽  
Selin Aytac ◽  
Sule Unal ◽  
...  

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