Abstract
IntroductionChildren with steroid resistant nephrotic syndrome usually require treatment with second-line agents and calcineurin inhibitors such as cyclosporine are now recommended as initial therapy. These agents only recently become available in our environment and their impact on care is unknown. We reviewed the short-term treatment outcomes of their use in comparison with previous outcomes.MethodsMedical records of children managed for idiopathic steroid resistant nephrotic syndrome over a 5 year period were reviewed. Remission rates and improvement in renal function following use of various agents were compared.ResultsOf 103 children with idiopathic nephrotic syndrome, 25(24.3%) were steroid resistant, of whom 17 received additional medications. Full remission rate for cyclosporine was 70% (7/10). Remission rates prior to the availability of cyclosporine were 40% (2/5) for cyclophosphamide and 66% (2/3), (partial remission only) with enalapril, an angiotensin converting enzyme inhibitor used in combination with alternate day prednisolone. One child with cyclophosphamide resistance subsequently achieved remission with cyclosporine. Remission was not related to sex (p=0.96), age (p=0.54), serum albumin (p=0.37) or hypertension (p=0.43) but to serum cholesterol (p= 0.02). The estimated glomerular filteration rate (eGFR) among children treated with cyclosporine ranged from 30-167 ml/min/1.73m2 as follows: >90 (5); 60-89 (3); 30-59 (2) while the mean pre and post treatment eGFR in those with eGFR <90 were 60 and 104ml/min/1.73m2 respectively (p=0.03). Mortality rate was 10% (1/10) in children treated with cyclosporine compared with 28.6% (2/7) in those treated with other medications (p=0.54).ConclusionCyclosporine resulted in improved treatment outcomes in children with idiopathic steroid resistant nephrotic syndrome.
Highlights
Children with steroid resistant nephrotic syndrome usually require treatment with second-line agents and calcineurin inhibitors such as cyclosporine are recommended as initial therapy
Median age was significantly higher in children with idiopathic SRNS compared with the steroid sensitive form (9.1 vs 5years respectively, p=0.008) Two children were referred to other centers on request, one died from complications of acute kidney injury before treatment for SRNS could be commenced while five defaulted from follow-up
Three children were treated with enalapril and prednisolone of whom two achieved partial remission while the third progressed to End-stage Kidney Disease (ESKD) within a few months
Summary
Children with steroid resistant nephrotic syndrome usually require treatment with second-line agents and calcineurin inhibitors such as cyclosporine are recommended as initial therapy. Efforts to improve the outcome of children with idiopathic steroid resistant nephrotic syndrome (iSRNS) have resulted in the emergence of various steroid sparing agents with considerations for sustained remission and reduced side effects These include alkylating agents such as cyclophosphamide (CYC), calcineurin inhibitors such as cyclosporine (CsA) and tacrolimus, mycophenolate mofetil (MMF), a T and B cell proliferation inhibitor and rituximab, a monoclonal antibody generally reserved for multi-therapy resistant cases. In order to standardize care, the Kidney Disease: Improving Global Outcomes (KDIGO) group [7] in 2012, made several recommendations on the treatment of childhood idiopathic SRNS These include the use of calcineurin inhibitors as the first line drugs for the management of steroid resistant nephrotic syndrome which we have since adopted at our centre. The objective of this study was to determine the short-term outcome of the treatment of childhood iSRNS with cyclosporine, and compare this with outcome prior to adoption of the KDIGO guidelines
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