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Published on: May 6, 2018
Treatment of steroid sensitive nephrotic syndrome
1Department of Pediatrics, Faculty of Medicine, University of Peradeniya, Sri Lanka. asiri26@hotmail.com
Insights
Childhood idiopathic nephrotic syndrome (NS) management aims for remission without adverse effects. While corticosteroids are effective for minimal change disease (MCD), alternative therapies are explored for frequent relapses to minimize steroid toxicity.
Area of Science:
- Pediatric Nephrology
- Glomerular Disorders
- Chronic Kidney Disease Management
Background:
- Childhood idiopathic nephrotic syndrome (NS) is a chronic glomerular disorder with risks of infection, thromboembolism, and malnutrition.
- Minimal change disease (MCD) accounts for over 90% of childhood NS cases, often responding to corticosteroids.
- Steroid-sensitive NS is generally benign, but relapsing courses necessitate effective management to prevent complications and minimize steroid toxicity.
Purpose of the Study:
- To outline the management goals for childhood idiopathic nephrotic syndrome (NS).
- To discuss therapeutic strategies for maintaining remission and minimizing adverse effects in children with NS.
- To review the use and associated side-effects of steroid-sparing agents in NS treatment.
Main Methods:
- Literature review of therapeutic regimens for childhood idiopathic nephrotic syndrome (NS).
- Analysis of corticosteroid efficacy in minimal change disease (MCD).
- Evaluation of steroid-sparing agents including alkylating agents, calcineurin inhibitors, and immunomodulatory drugs.
Main Results:
- Corticosteroids are effective in a majority of childhood NS cases, particularly MCD.
- Steroid-sparing agents like cyclophosphamide, cyclosporin A, and levamisole have shown variable success.
- These alternative agents are associated with specific side-effects that require careful consideration.
Conclusions:
- Effective management of childhood NS requires balancing treatment benefits against risks.
- Therapeutic strategies must address frequent relapses and minimize long-term steroid toxicity.
- Careful weighing of benefits and risks is crucial when considering steroid-sparing agents for NS treatment.
Abstract:
Childhood idiopathic nephrotic syndrome (NS) is a chronic glomerular disorder, and if untreated, is associated with increased risk of life-threatening infections, thromboembolism, lipid abnormalities, and malnutrition. The aim of the management of NS in children is to induce and maintain complete remission with resolution of proteinuria and edema without encountering serious adverse effects of therapy. Over 90% of cases in children are due to minimal change disease (MCD) and a majority of them will respond to corticosteroid therapy. Steroid sensitive NS is considered to be a relatively benign condition; progression to end stage renal failure is extremely rare and over 80% achieve spontaneous remission in later childhood. The early disease is characterized by a relapsing course, placing the child at risk of acute complications. The occurrence of frequent relapses necessitates clear therapeutic strategies in order to maintain sustained remission and minimize steroid toxicity. Numerous therapeutic regimens have been proposed utilizing steroid sparing agents such as alkylating agents, principally, cyclophosphamide and chlorambucil, calcineurin inhibitors namely cyclosporin A and immunomodulatory drug levamisole with variable success and associated side-effects. It is therefore important that the benefits and risks of these agents are weighed before considering their use in the treatment of patients with NS.
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