Management of Nephrotic Syndrome in Children

Brodehl, J.

doi:10.1007/bf03259081

Cited by 15 publications

(13 citation statements)

References 73 publications

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“…Unlike cyclophosphamide or chlorambucil, relapse rates are high when the drug is discontinued, and 52% needed reintroduction of cyclosporin A. More stable remission with cyclosporin A, when introduced after initial withdrawal, has been reported with continuous therapy for 5 years [29]. However, the use of cyclosporin A necessitates regular monitoring of blood levels and renal function because of its potential nephrotoxicity, and renal biopsy should be considered after 2 or 3 years of cyclosporin A therapy, even if serum creatinine is normal, since previous studies have shown a lack of correlation between structural damage and renal function [9,29].…”

Section: Discussionmentioning

confidence: 92%

The use of steroid-sparing agents in steroid-sensitive nephrotic syndrome

Abeyagunawardena¹,

Dillon²,

Rees³

et al. 2003

Pediatr Nephrol

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Childhood nephrotic syndrome (NS) is frequently characterized by a relapsing course. There is no uniform agreement about the precise stage at which a steroid-sparing agent should be introduced to control the disease. In order to evaluate the treatment strategies and outcome of steroid-sensitive NS over the last 2 decades, a retrospective notes review was undertaken in a cohort of children treated at Great Ormond Street Children's Hospital between 1980 and 2000. From a population of 863 children with NS referred, 509 had frequently relapsing or steroid-dependent disease and 261 children received at least one steroid-sparing agent. Cyclophosphamide was the first choice in 178 patients and in 114 no further steroid-sparing agent was needed. Levamisole was prescribed as the first steroid-sparing agent for 65 children and disease control was achieved in 30%. Cyclosporin A was prescribed in 61 children and sustained remission was induced in 69%. It is concluded that cyclophosphamide is a potent agent in inducing sustained remission in steroid-sensitive NS. Levamisole and cyclosporin A have emerged as attractive steroid-sparing agents. Complications and major side effects of treatment are infrequent but occasionally fatal.

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Section: Discussionmentioning

confidence: 92%

The use of steroid-sparing agents in steroid-sensitive nephrotic syndrome

Abeyagunawardena¹,

Dillon²,

Rees³

et al. 2003

Pediatr Nephrol

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“…Repeated use of corticosteroids in children with INS leads to steroid toxicity, which often requires the use of potentially toxic drugs such as CP, chlorambucil, and cyclosporin A to achieve long-term remission [19]. Of these, the best results to date are with CP.…”

Section: Discussionmentioning

confidence: 95%

Do glutathione-S-transferase polymorphisms influence response to intravenous cyclophosphamide therapy in idiopathic nephrotic syndrome?

et al. 2008

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The response to cyclophosphamide (CP) is variable and difficult to predict in children with idiopathic nephrotic syndrome (INS). The polymorphic expression of glutathione-S-transferase (GST) may affect the remission rate after CP therapy. In this study, we evaluated the correlation of GST polymorphism and response to CP in INS. We studied GST polymorphism in 74 children with steroid-sensitive (44) and steroid-resistant (30) INS receiving intravenous cyclophosphamide (IVCP) therapy. We correlated GSTM1, GSTT1, and GSTP1 genotypes with response to IVCP. Thirty-seven (50%) out of 74 children responded to CP therapy. A synergistic effect of three genotypic combinations showed significant correlation with remission in the steroid-sensitive group. These combinations were GSTP1 and GSTM1 null genotype (p = 0.013) and GSTP1 together with GSTM1 and GSTT1 null genotypes (p = 0.026). Further, a significant difference was observed with a combination of GSTM1 and GSTT1 null genotypes and Val105 polymorphism. No association was observed among steroid-resistant patients. Our results indicate that among children with steroid-sensitive NS, there is an association with response to IVCP therapy and combination of GSTP1 Val105 polymorphism and the null genotypes of GSTT1 and GSTM1. GST polymorphism may be of significance in the management of children with INS receiving CP therapy.

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“…In patients with frequently relapsing nephrotic syndrome, steroid toxicity may develop with hypertension, cataract, growth failure and obesity; hence these children may require alternative therapy 6 . Cyclophosphamide is the most commonly used drug as an alternative treatment.…”

Section: Discussionmentioning

confidence: 99%