scholarly journals Pulsed Vincristine Therapy in Steroid-Resistant Nephrotic Syndrome

2017 ◽  
Vol 2017 ◽  
pp. 1-4 ◽  
Author(s):  
Shenal Thalgahagoda ◽  
Shamali Abeyagunawardena ◽  
Heshan Jayaweera ◽  
Umeshi Ishanthika Karunadasa ◽  
Asiri Samantha Abeyagunawardena

Steroid-resistant nephrotic syndrome (SRNS) poses a therapeutic challenge for the paediatric nephrologist. As relentless progression to renal failure occurs with continued proteinuria, such patients will be treated with different cytotoxic medications with variable success rates and side-effects. We present here our findings on administering the anticancer drug vincristine for SRNS patients at a single centre in Sri Lanka. Methods. Between 2002 and 2007, fifty-four children presenting with steroid and cyclophosphamide resistance were treated with vincristine at 1.5 mg/m2 in weekly intravenous pulses for 8 weeks along with a tapering steroid regimen of 6 months. All patients were closely followed up for 5 years. Results. Of the 54 patients 39 were males and 15 were females (age range 3.5–11.6 years, median 6.1 years). At the end of the treatment course, 21 patients achieved complete remission while 7 had partial remission and no response was seen in 26 patients. Sustained remission at 6, 12, 24, and 60 months were 15 (27.78%), 11 (20.37%), 9 (16.67%), and 7 (12.96%), respectively. Most side-effects observed were reversible and no serious side-effects were noted during vincristine therapy. Conclusion. Although its therapeutic mechanisms in nephrotic syndrome are still not elucidated, vincristine appears to be a potent alternative that could be considered for treating SRNS.

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>


2018 ◽  
Vol 3 (1) ◽  

Background: Nephrotic syndrome may be caused by primary (idiopathic) renal disease or by a variety of secondary causes. Patients present with marked edema, proteinuria, hypoalbuminemia, and often hyperlipidemia. Treatment of most patients should include fluid and sodium restriction, oral or intravenous diuretics, and angiotensin-converting enzyme inhibitors. Adults with nephrotic syndrome may benefit from corticosteroid treatment. The treatment of patients with the steroid-resistant nephrotic syndrome (SRNS) and steroid-dependent nephrotic syndrome (SDNS) is challenging. On the basis of suggestions that B lymphocytes are crucial in the pathogenesis of the nephrotic syndrome, rituximab (a monoclonal antibody against CD20 antigen) is used in treatment of these patients. Aim of study: To evaluate the role of rituximaband mycophenolic acid in treatment of patientswith steroid-resistant (SRNS) and steroid-dependent nephrotic syndrome (SDNS), whom not respond or relapse after calcineurin inhibitor (CNI) (tacrolimus or cyclosporine) had been used. Patients and methods: Case series study was done between 2012 - 2015 in AL-Sadder Teaching Hospital Nephrology Center and record 40 patients with different age groups, males and females with different histopathological types (Minimal Change Glomerulonephritis, Focal Segmental Glomerulosclerosis, Mesengeo Prolifrative Glomerulonephritis). These patients were taking prednisilone and\or calcineurin inhibitor (tacrolimus “prograf”) or (cyclosporine “sandimmune”), and they get either Steroid Dependent Nephrotic Syndrome or Steroid Resistant Nephrotic Syndrome with frequent admission more than four time per year. To these patients we start rituximab intravenous infusion monthly for at least six months with the use of steroid and mycophenolate mofetil during these six months. The patients followed up for 3-12 months after initiation of rituximab by different investigations and the patients were classified according to their response into complete, partial and no response. After one year stop rituximab treatment, follow the patients clinically and by investigations for (1-2) years to determine which patients get relapse. Results: Majority (80%) of patients with nephrotic syndrome who had good response to rituximab were younger age group < 15 years. Better response to rituximab associated with Minimal Change Glomerulonephritis. There was significant reduction in blood urea, serum creatinine, urine (protein/creatinine) ratio and serum cholesterol. Serum albumin was significant elevated. Response to rituximab was not significantly associated with gender or steroid response. Majority of patients with good response not relapse and need more time for follow up. Relapsing after stopping rituximab not significantly associated with age, gender, histopathological type and steroid response. Conclusion: Rituximab and mycophenolate mofetil used in steroid-resistant nephrotic syndrome to get ride from side effects of calcineurine inhibitor (tacrolimus or cyclosporine). Rituximab and mycophenolate mofetil used in steroid-dependent nephrotic syndrome after calcineurine inhibitorto get ride from side effects of steroid. Improvement in renal function is result from stopping of calcineurine inhibitor (nephrotoxic drugs) and/or from rituximab and mycophenolate mofetil. Cost of rituximab is less than the cost that needed if the patients had frequent admissions to the hospital or developed renal failure and ended with dialysis.


2019 ◽  
Vol 35 (4) ◽  
pp. 621-623
Author(s):  
Lale Guliyeva ◽  
Yılmaz Tabel ◽  
Ali Düzova ◽  
Nusret Akpolat ◽  
Seza Özen ◽  
...  

Nephron ◽  
2021 ◽  
pp. 1-6
Author(s):  
Suramath Isaranuwatchai ◽  
Ankanee Chanakul ◽  
Chupong Ittiwut ◽  
Chalurmpon Srichomthong ◽  
Vorasuk Shotelersuk ◽  
...  

Chronic kidney disease of unknown etiology (CKDu) has been a problem in renal practice as indefinite diagnosis may lead to inappropriate management. Here, we report a 54-year-old father diagnosed with CKDu at 33 years old and his 8-year-old son with steroid-resistant nephrotic syndrome. Using whole-exome sequencing, both were found to be heterozygous for c.737G>A (p.Arg246Gln) in LMX1B. The diagnosis of LMX1B-associated nephropathy has led to changes in the treatment plan with appropriate genetic counseling. The previously reported cases with this particular mutation were also reviewed. Most children with LMX1B-associated nephropathy had nonnephrotic proteinuria with normal renal function. Interestingly, our pediatric case presented with steroid-resistant nephrotic syndrome at 8 years old and progressed to ESRD requiring peritoneal dialysis at the age of 15 years. Our report emphasized the need of genetic testing in CKDu for definite diagnosis leading to precise management.


2016 ◽  
Vol 31 (suppl_1) ◽  
pp. i353-i353
Author(s):  
Beata S. Lipska-Ziętkiewicz ◽  
Olivia Boyer ◽  
Olivier Gribouval ◽  
Mansoureh Tabatabaei ◽  
Cecile Fourrage ◽  
...  

2017 ◽  
Vol 32 (suppl_3) ◽  
pp. iii110-iii113
Author(s):  
Maddalena Gigante ◽  
Sterpeta Diella ◽  
Luisa Santangelo ◽  
Ottavio Amatruda ◽  
Gianluca Caridi ◽  
...  

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