Recent Update in The Management of Childhood Nephrotic Syndrome (original) (raw)
Related papers
Steroid Sensitive Nephrotic Syndrome in Children
Journal of Nephrology & Therapeutics, 2014
Nephrotic Syndrome (NS) is one of the most frequent glomerular diseases seen in children. Children who go into complete remissin following treatment with corticosteroids are classified as having "steroid sensitive" NS. In developed countries over 80% of children with idiopathic NS have steroid sensitive disease although response to steroids is somewhat tempered in developing countries, especially in black children, the majority of whom have steroid resistant disease. The exact pathogenesis of this condition remains elusive. Podocyte injury and proteinuria are the two main issues in the pathogenesis. Recent studies suggest alterations in both innate and adaptive immune responses. There is release of cytokines by T-cells as well as a strong contribution of B-cell immunity. Genetic studies have reported human leucocyte antigen (HLA) class II antigens DR and DQ associations linked to steroid sensitive NS and in small case studies, single gene mutations e.g. PLCE1 although to date no homozygous mutations in NPHS1 or NPHS2 and WT-1 genes have been reported. Most children with steroid sensitive NS have multiple relapses and a significant percentage also develop steroid dependent NS. These children receive multiples courses of steroids and are at high risk of developing steroid toxicity. Patient with frequent relapses who develop steroid dependency thus require alternative treatment. Steroids sparing agents used include levamisole, cyclophosphamide, mycophenolate mofetil (MMF), calcineurin inhibitors (cyclosporine and tacrolimus), rituximab and vincristine. The steroid-sparing effects of these agents have greatly reduced the adverse effects seen with long-term use of steroids. Despite the wide arsenal of agents available, therapy needs to be individualised to achieve optimal care of each child. More randomised controlled trials are required to evaluate the therapeutic and economic efficacy of this agent, define criteria for selection of patients for use of particular agents and to determine the safety profile of these drugs in children. This article reviews the epidemiology, pathogenesis, clinical presentation, diagnosis, complications, management and long term outcome of children with steroid sensitive NS.
Steroid Sensitive Nephrotic Syndrome in children: What steroid regime for the initial attack?
Galle Medical Journal, 2012
Nephrotic syndrome remains the most common manifestation of glomerular disease in childhood. Minimal change nephropathy is the most common cause of the syndrome in children. Despite its initial high response rate to corticosteroids and its favorable prognosis, relapses are common leading to increased morbidity and cost of treatment. This review seeks to appraise the common triggers of relapse and to highlight the evolving hypotheses about the pathogenesis of the syndrome. Literature search was conducted through PubMed, Google web search and Cochrane Database of Systematic reviews using relevant search terms. Acute respiratory infections and urinary tract infections are the most frequent infectious triggers of relapse. Targeted interventions like initiating corticosteroid or its dose-adjustment during episodes of acute respiratory infection and zinc supplementation are reportedly effective in reducing relapse rates. Hypotheses on pathogenesis of the syndrome have evolved from the concepts of 'immune dysregulation', 'increased glomerular permeability' to 'podocytopathy'. Although development of drugs which can regulate the pathways for podocyte injury offers future hope for effective and targeted treatment, the relapse-specific interventions currently contribute to significant reduction in disease morbidity.
Pediatric Nephrology
Background The aim of the current PodoNet registry analysis was to evaluate the outcome of steroid-resistant nephrotic syndrome (SRNS) in children who were not treated with intensified immunosuppression (IIS), focusing on the potential for spontaneous remission and the role of angiotensin blockade on proteinuria reduction. Methods Ninety-five pediatric patients who did not receive any IIS were identified in the PodoNet Registry. Competing risk analyses were performed on 67 patients with nephrotic-range proteinuria at disease onset to explore the cumulative rates of complete or partial remission or progression to kidney failure, stratified by underlying etiology (genetic vs. non-genetic SRNS). In addition, Cox proportional hazard analysis was performed to identify factors predicting proteinuria remission. Results Eighteen of 31 (58.1%) patients with non-genetic SRNS achieved complete remission without IIS, with a cumulative likelihood of 46.2% at 1 year and 57.7% at 2 years. Remissio...
Management and outcome of steroid-resistant nephrotic syndrome in children
Iranian journal of kidney diseases, 2009
Steroid-resistant nephrotic syndrome (SRNS) is uncommon in children, but often leads to ESRD. We report our experience with SRNS and its treatments and outcomes. We assessed 73 children with SRNS admitted to Ali Asghar Children Hospital in Tehran, Iran. Their clinical presentations, treatment, and disease courses were reviewed. The mean follow-up duration was 6.0 +/- 4.2 years. Moreover, survival times were calculated and the Cox regression method was used to determine variables able to predict survival of the kidneys. Age at the onset of the disease, sex, and hematuria were not predictive of the response to treatment with immunosuppressive drugs in the children with SRNS. The type of resistance (early or late) was associated with the responsiveness to immunosuppressives. Response to any of the immunosuppressive drugs determined the responsiveness to other immunosuppressive drugs. Cyclosporine was more effective than cyclophosphamide as initial therapy. The mean kidney survival time...
Introduction: Nephrotic syndrome is characterized by gross proteinuria, hypo albuminemia, hyperlipidemia, and peripheral edema. Objectives: The main objective of the study is to find the long-term outcome of children with steroid-sensitive idiopathic nephrotic syndrome. Results: The study group comprised of 100 patients 72 (93.5%) were initial steroid resistant and 28 were late non-responders. Gender distribution showed 49 (63.6%) males and 28 (36.4%) females with a ratio of 1.75. Age range of patients was 1-15 years with a mean of 8.11 +3.58 years. Sixty nine (89.6%) patients underwent renal biopsy. Conclusion: Nephrotic syndrome can increase your child's risk of infection and blood clots. It always affects both kidneys and usually appears in the early years of your child's life.
A Brief Overview of the management of Steroid-Sensitive Nephrotic Syndrome in Children
Journal of the Postgraduate Institute of Medicine
Management of steroid sensitive nephrotic syndrome (SSNS) in the paediatric age group poses many challenges, which are not limited to steroid toxicity and suboptimal control of proteinuria but also include obtaining long term remission and improving quality of life. Though steroids are the mainstay of treatment for SSNS, various other immunosuppressant medications have been used to achieve remission in frequently relapsing and steroid dependent nephrotic syndrome patients. Selection of these medications should be done carefully, weighing their toxicity profiles and relative effectiveness. This clinical update discusses the disease SSNS and the different treatment strategies that have been evaluated along with their therapeutic outcomes
Pediatric Nephrology, 2020
Idiopathic nephrotic syndrome newly affects 1–3 per 100,000 children per year. Approximately 85% of cases show complete remission of proteinuria following glucocorticoid treatment. Patients who do not achieve complete remission within 4–6 weeks of glucocorticoid treatment have steroid-resistant nephrotic syndrome (SRNS). In 10–30% of steroid-resistant patients, mutations in podocyte-associated genes can be detected, whereas an undefined circulating factor of immune origin is assumed in the remaining ones. Diagnosis and management of SRNS is a great challenge due to its heterogeneous etiology, frequent lack of remission by further immunosuppressive treatment, and severe complications including the development of end-stage kidney disease and recurrence after renal transplantation. A team of experts including pediatric nephrologists and renal geneticists from the International Pediatric Nephrology Association (IPNA), a renal pathologist, and an adult nephrologist have now developed com...
Long-Term Outcome of Steroid-Resistant Nephrotic Syndrome in Children
Journal of the American Society of Nephrology : JASN, 2017
We investigated the value of genetic, histopathologic, and early treatment response information in prognosing long-term renal outcome in children with primary steroid-resistant nephrotic syndrome. From the PodoNet Registry, we obtained longitudinal clinical information for 1354 patients (disease onset at >3 months and <20 years of age): 612 had documented responsiveness to intensified immunosuppression (IIS), 1155 had kidney biopsy results, and 212 had an established genetic diagnosis. We assessed risk factors for ESRD using multivariate Cox regression models. Complete and partial remission of proteinuria within 12 months of disease onset occurred in 24.5% and 16.5% of children, respectively, with the highest remission rates achieved with calcineurin inhibitor-based protocols. Ten-year ESRD-free survival rates were 43%, 94%, and 72% in children with IIS resistance, complete remission, and partial remission, respectively; 27% in children with a genetic diagnosis; and 79% and 52...
Management of steroid-resistant nephrotic syndrome in children and adolescents
The Lancet Child & Adolescent Health, 2018
More than 85% of children and adolescents (majority between 1-12 years old) with idiopathic nephrotic syndrome show complete remission of proteinuria following daily treatment with corticosteroids. Patients who do not show remission after 4 weeks' treatment with daily prednisolone are considered to have steroid-resistant nephrotic syndrome (SRNS). Renal histology in most patients shows presence of focal segmental glomerulosclerosis, minimal change disease, and (rarely) mesangioproliferative glomerulonephritis. A third of patients with SRNS show mutations in one of the key podocyte genes. The remaining cases of SRNS are probably caused by an undefined circulating factor. Treatment with calcineurin inhibitors (ciclosporin and tacrolimus) is the standard of care for patients with non-genetic SRNS, and approximately 70% of patients achieve a complete or partial remission and show satisfactory long-term outcome. Additional treatment with drugs that inhibit the renin-angiotensin axis is recommended for hypertension and for reducing remaining proteinuria. Patients with SRNS who do not respond to treatment with calcineurin inhibitors or other immunosuppressive drugs can show declining kidney function and are at risk for endstage renal failure. Approximately a third of those who undergo renal transplantation show recurrent focal segmental glomerulosclerosis in the allograft and often respond to combined treatment with plasma exchange, rituximab, and intensified immunosuppression.
Children with Steroid-resistant Nephrotic Syndrome: a Single-Center Study
DOAJ (DOAJ: Directory of Open Access Journals), 2016
Background Steroid-resistant nephrotic syndrome (SRNS) accounts for 10%-20% of all cases of idiopathic nephrotic syndrome. These patients are at risk of developing end-stage renal disease. The aim of this study was to determine the demographic characteristics, renal biopsy findings, response to immunosuppressive treatment, and prognosis in pediatric patients with SRNS. Materials and Methods This retrospective study included 31 patients diagnosed as primary SRNS. Age at first episode, gender, parental consanguinity, and familial history of nephrotic syndrome were recorded. Demographic characteristics, renal biopsy findings, response to immunosuppressive treatment and prognosis were analyzed, as were the number of and treatment of relapses, extra-renal manifestations and complications of disease and treatment. Data were analyzed using SPSS version 15.0 Results Mean age at first episode of nephrotic syndrome was 4.1±2.9 years. At the end of the first immunosuppressive treatment cycle, 14 (51.8%) patients achieved complete remission, 4 (14.8%) patients achieved partial remission and 9 patients (33.3%) did not achieve remission. Analysis of the final status of the patients showed that 16 patients (51.6%) developed remission, 5 patients (16%) continued to have nephrotic range proteinuria and 10 patients (32%) developed chronic renal failure (CRF). Conclusion The treatment of SRNS remains controversial. Early genetic testing can help the inevitable immunosuppressive treatments which may not be effective and have several side effects. Calcineurin inhibitors and mycophenolate mofetil are known to be effective immunosuppressive drugs for treating steroid resistant nephrotic syndrome.