scholarly journals Recommendations from the International Pediatric Nephrology Association for the Diagnosis and Management of Children with Steroid-Resistant Nephrotic Syndrome (SRNS)

KIDNEYS ◽  
2021 ◽  
Vol 10 (1) ◽  
pp. 26-28
Author(s):  
No Authors

No abstract

2015 ◽  
Vol 8 (4) ◽  
pp. 136 ◽  
Author(s):  
Syed Raza Shah ◽  
Areeba Altaf ◽  
Mohammad Hussham Arshad ◽  
Anum Mari ◽  
Sahir Noorani ◽  
...  

<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>


2013 ◽  
Vol 28 (8) ◽  
pp. 1235-1241 ◽  
Author(s):  
Caroline Straatmann ◽  
Rose Ayoob ◽  
Rasheed Gbadegesin ◽  
Keisha Gibson ◽  
Michelle N. Rheault ◽  
...  

2012 ◽  
Vol 2012 ◽  
pp. 1-5 ◽  
Author(s):  
Muhammed Mubarak ◽  
Javed I. Kazi ◽  
Shaheera Shakeel ◽  
Ali Lanewala ◽  
Seema Hashmi

Steroid-resistant nephrotic syndrome (SRNS) is a common problem in pediatric nephrology practice. There is currently little information in the literature on the spectrum of histopathologic lesions in children presenting with SRNS in Pakistan. This study was designed to determine the histopathologic lesions in children presenting with SRNS at our center. The study was conducted at the Histopathology Department, Sindh Institute of Urology and Transplantation (SIUT) from January 2009 to August 2011. All children (≤16 years) presenting with SRNS, in whom renal biopsies were performed, were included. Their demographic, clinical, laboratory, and histopathological data were retrieved from files and original renal biopsy forms. The results were analyzed by SPSS version 10.0. A total of 147 children were included. Of these, 91 (61.9%) were males and 56 (38.1%) females, with male-to-female ratio of 1.6 : 1. The mean age was 7.03 ± 4.0 years (range: 6 months–16 years). The histopathological lesions seen on renal biopsies comprised of focal segmental glomerulosclerosis (FSGS) (38.5%), followed by minimal change disease (MCD) (23.2%), IgM nephropathy (IgMN) (13.6%), idiopathic mesangial proliferative GN (10.2%), membranous GN (8.2%), and mesangiocapillary GN (4.8%). Our results indicate that FSGS is the predominant lesion in children with SRNS, followed by MCD and IgMN.


2019 ◽  
Vol 59 (4) ◽  
pp. 175-82
Author(s):  
Agomoni Chaki ◽  
Farhana Rahman ◽  
Jahanara Arju ◽  
Abdullah- Al Mamun ◽  
Tahmina Jesmin ◽  
...  

Background Nephrotic syndrome (NS) is one of the most common glomerular disease in children, characterized by massive proteinuria, hypoalbuminemia, dyslipidemia and edema. Steroid-resistant nephrotic syndrome (SRNS) and steroid-dependent nephrotic syndrome (SDNS) present challenges in pharmaceutical management. Patient need several immunosuppressant for optimal control, each of which has significant side effect and difficult to get  desired results. Rituximab (RTX) is a monoclonal antibody that targets B cells and has been shown to be effective for patients with SRNS and SDNS. Objective To see efficacy of RTX in pediatric patients with SRNS. Method This retrospective study took place in Pediatric Nephrology Department of Bangabandhu Sheikh Mujib Medical University from July 2017 to June 2019. Patients diagnosed with SRNS who were treated with RTX and followed up for 6 months were enrolled in this study. Primary endpoint was achievement of remission after rituximab infusion; secondary endpoint was relapse-free survival rate in 6 months period following rituximab infusion. Results Total  7 patients were recruited in this study. Among them 4 were male. Clinical and lab parameters of all patients before and after RTX  were compared. Complete remission achieved in 4/7 patients, partial remission in 2/7 patients and no response in 1/7 patient. Mean number of relapse in 3 patients before RTX infusion was 3.67 (SD 0.57) and after 0.33 (SD 0.00) (P=0.038). Conclusion RTX is a biological agent that is effective and promising drug in children with SRNS. Rituximab is useful to induce and maintain remission.


2021 ◽  
Vol 9 (T3) ◽  
pp. 349-352
Author(s):  
Muhammad Akbar ◽  
Oke Rina Ramayani ◽  
Gema Nazri Yanni

BACKGROUND: Syndrome nephrotic is the most common kidney disease found in pediatric kidney disease, classification based on clinical response to steroids or histopathological characteristics. Increased blood pressure in steroid-resistant nephrotic syndrome (NS) is still a complication to be aware of in cases of NS. AIM: The aim of the study was to determine the differences in systolic and diastolic blood pressure in patients with steroid-sensitive NS and steroid-resistant NS. METHODS: Analytical correctional study in 50 children with NS divided into 25 Steroid Resistant NS (SRNS) groups and 25 steroid sensitive NS (SSNS) people who met inclusion and exclusion criteria to assess systolic and diastolic blood pressure in each group in pediatric nephrology division of the general hospital of Haji Adam Malik Medan. RESULTS: There is a difference in systolic blood pressure in the SRNS and SSNS groups which mean p = 0.024 and there is no difference in diastolic blood pressure in the SRNS group with SSNS p = 0.358. If linked levels of proteinuria to blood pressure, systolic and diastolic in both groups found no significant link p>0.05 high blood pressure with the degree of proteinuria in both group. CONCLUSION: There are differences in systolic blood pressure in the SRNS and SSNS groups.


2020 ◽  
Vol 60 (6) ◽  
pp. 316-20
Author(s):  
Sudung Oloan Pardede ◽  
Andini Striratnaputri ◽  
Muzal Kadim

Background The mechanisms of pathogenesis of steroid-resistant nephrotic syndrome (SRNS) and steroid-sensitive nephrotic syndrome (SSNS) are not well understood. Antioxidants, such as glutathione peroxidase enzyme (GPx) and its cofactor, selenium, are thought to slow the progress of nephrotic syndrome (NS). Objective To compare selenium levels in SRNS and SSNS pediatric patients. Methods This cross-sectional study was conducted in 51 SRNS and 30 SSNS patients, aged 2 to 18 years, who visited the Pediatric Nephrology Outpatient Clinic at Cipto Mangunkusumo Hospital, Jakarta. Subjects were included by consecutive sampling. Selenium was measured on venous blood using GC tools MS ™ (Agilent technologies, inc.). Results Median selenium levels in SRNS patients were 92 (range 42-154) μg/L and in SSNS patients were 93 (range 69-193) μg/L. Conclusion Selenium levels in SRNS and SSNS patients were not significantly different.


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