scholarly journals Rituximab in steroid resistant nephrotic syndrome

2019 ◽  
Vol 59 (4) ◽  
pp. 175-82
Author(s):  
Agomoni Chaki ◽  
Farhana Rahman ◽  
Jahanara Arju ◽  
Abdullah- Al Mamun ◽  
Tahmina Jesmin ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Pediatric Nephrology ◽  
Relapse Free Survival ◽  
Steroid Resistant ◽  
Steroid Resistant Nephrotic Syndrome ◽  
Free Survival ◽  
Pharmaceutical Management ◽  
Steroid Dependent ◽  
Before And After ◽  
Medical University

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.

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>

Treatment outcome of late steroid-resistant nephrotic syndrome: a study by the Midwest Pediatric Nephrology Consortium

2013 ◽  
Vol 28 (8) ◽  
pp. 1235-1241 ◽  
Author(s):  
Caroline Straatmann ◽  
Rose Ayoob ◽  
Rasheed Gbadegesin ◽  
Keisha Gibson ◽  
Michelle N. Rheault ◽  
...  
Keyword(s):  
Nephrotic Syndrome ◽  
Treatment Outcome ◽  
Pediatric Nephrology ◽  
Steroid Resistant ◽  
Steroid Resistant Nephrotic Syndrome

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.

scholarly journals The Spectrum of Histopathological Lesions in Children Presenting with Steroid-Resistant Nephrotic Syndrome at a Single Center in Pakistan

2012 ◽  
Vol 2012 ◽  
pp. 1-5 ◽  
Author(s):  
Muhammed Mubarak ◽  
Javed I. Kazi ◽  
Shaheera Shakeel ◽  
Ali Lanewala ◽  
Seema Hashmi
Keyword(s):  
Nephrotic Syndrome ◽  
Clinical Laboratory ◽  
Pediatric Nephrology ◽  
Female Ratio ◽  
Igm Nephropathy ◽  
Steroid Resistant ◽  
Steroid Resistant Nephrotic Syndrome ◽  
Histopathological Lesions ◽  
Renal Biopsies ◽  
Male To Female

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.

scholarly journals Blood Pressure Differences in Nephrotic Syndrome Patients with Steroid Resistant Nephrotic Syndrome Patients and Steroid Sensitive Nephrotic Syndrome

2021 ◽  
Vol 9 (T3) ◽  
pp. 349-352
Author(s):  
Muhammad Akbar ◽  
Oke Rina Ramayani ◽  
Gema Nazri Yanni
Keyword(s):  
Blood Pressure ◽  
Nephrotic Syndrome ◽  
Kidney Disease ◽  
Systolic Blood Pressure ◽  
Diastolic Blood Pressure ◽  
Pediatric Nephrology ◽  
Disease Classification ◽  
Steroid Resistant ◽  
Steroid Resistant Nephrotic Syndrome ◽  
Steroid Sensitive

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.

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