Non-corticosteroid treatment for nephrotic syndrome in children

Durkan, Anne M.; Hodson, EM; Ns, Willis; Craig, Jonathan C.

doi:10.1002/14651858.cd002290.pub2

Cited by 23 publications

(10 citation statements)

References 41 publications

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“…The vast majority (92%) of these patients will respond well to corticosteroids. However, as many as 70% of children with nephrotic syndrome experience at least one relapse, and 30% will develop a more complicated course with frequent relapses (≥2 relapses/ 6 months) with or without steroid dependency (relapse during tapering or within 2 weeks after discontinuation of corticosteroids) [2,3]. A short course of cyclophosphamide (2-3 months 2-3 mg/kg per day) will lead to prolonged remission in 25-60% of children with frequently relapsing nephrotic syndrome [4,5].…”

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confidence: 99%

Mycophenolate mofetil versus cyclosporine for remission maintenance in nephrotic syndrome

et al. 2008

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We performed a multi-centre randomized controlled trial to compare the efficacy of mycophenolate mofetil (MMF) to that of cyclosporine A (CsA) in treating children with frequently relapsing nephrotic syndrome and biopsy-proven minimal change disease. Of the 31 randomized initially selected patients, seven were excluded. The remaining 24 children received either MMF 1200 mg/m 2 per day (n=12) or CsA 4-5 mg/kg per day (n=12) during a 12-month period. Of the 12 patients in the MMF group, two discontinued the study medication. Evaluation of the changes from the baseline glomerular filtration rate showed an overall significant difference in favour of MMF over the treatment period (p=0.03). Seven of the 12 patients in the MMF group and 11 of the 12 patients in the CsA group remained in complete remission during the entire study period. Relapse rate in the MMF group was 0.83/year compared to 0.08/year in the CsA group (p=0.08). None of the patients reported diarrhea. Pharmacokinetic profiles of mycophenolic acid were performed in seven patients. The patient with the lowest area under the curve had three relapses within 6 months. In children with frequently relapsing minimal change nephrotic syndrome, MMF has a favourable side effect profile compared to CsA; however, there is a tendency towards a higher relapse risk in patients treated with MMF.

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Section: Introductionmentioning

confidence: 99%

Mycophenolate mofetil versus cyclosporine for remission maintenance in nephrotic syndrome

et al. 2008

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“…18 However, it is difficult to attribute these effects to any single agent or treatment modality, as multimodal therapy is employed in almost all childhood cancers. 4 The use of certain chemotherapeutic agents, including the immunosuppressant cyclophosphamide, has been expanded to treat benign conditions in children such as nephrotic syndrome, 19 juvenile rheumatoid arthritis, 20 and systemic lupus erythematosus. 21 Cyclophosphamide is an alkylating nitrogen-mustard derivative capable of cross-linking intra-and interstrand DNA and DNA proteins, thereby inhibiting DNA replication and inducing apoptosis.…”

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Association of cyclophosphamide use with dental developmental defects and salivary gland dysfunction in recipients of childhood antineoplastic therapy

Hsieh

Hibbert

Shaw

et al. 2010

Cancer

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BACKGROUND:The aim of this study was to examine the effect of antineoplastic therapy on dental development and saliva function in recipients of childhood antineoplastic therapy. METHODS: Patients attending the long-term follow-up clinic at Children's Hospital at Westmead, NSW, Australia, were included if they had received treatment prior to 16 years of age and were in remission for more than 5 years. A dental examination and saliva test were performed for each participant. Holtta's Defect Index (HDI) was used to assess tooth aplasia, microdontia, and root-crown ratio on an orthopantomogram (OPG). Multivariable-adjusted regression analyses were used to estimate the association of patient characteristics and treatment modalities with dental outcomes. RESULTS: One hundred six participants (61% male) were recruited (response rate ¼ 88%). The mean HDI score was 24.7 AE 17.8. A cumulative dose of cyclophosphamide >7500 mg/m 2 increased the HDI score by 13.06 (P ¼ .01). Recipients of cyclophosphamide also had significantly increased odds of exhibiting very low saliva flow (<0.7 mL/min) (odds ratio ¼ 12.43; 95% confidence interval, 2.08-74.35; P ¼ .006). CONCLUSIONS: Children and adolescents who received high doses of cyclophosphamide were at increased risk of dental disturbances. Cyclophosphamide recipients were also at greater risk of exhibiting very low saliva flow. This study applied the HDI to patients receiving all forms of antineoplastic treatment and highlights the dose-dependent relation between cumulative dose of cyclophosphamide and dental disturbances.

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“…In a retrospective case series of 51 children with FSGS without NPHS2 homozygous or compound heterozygous mutations, 77% achieved remission with cyclosporin and prednisone with or without pulse methylprednisolone [10]. Adverse effects are common; in RCTs comparing cyclosporin with alkylating agents in children with SSNS and normal renal function, about 30% of the children developed hypertension and 9% had reduced renal function [25].…”

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Therapies for steroid-resistant nephrotic syndrome

Hodson

Craig

2008

Pediatr Nephrol

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Between 10 and 20% of children with primary nephrotic syndrome are steroid-resistant (SRNS). From earlier studies in children with SRNS, we know that cyclosporin (with or without alternate-day prednisone) and cyclophosphamide (with pulse intravenous corticosteroids) result in comparable complete or partial remission rates of about 60%. An evaluation of the relative effectiveness of cyclophosphamide and cyclosporin has not been possible because of the absence of a head-to-head randomised trial. The Arbeitsgemeinschaft für Pädiatrische Nephrologie trial, published in this issue of Pediatric Nephrology, has filled this gap in our evidence base. Although there was no difference in the number of patients achieving complete remission, those patients receiving cyclosporin treatment were significantly more likely to achieve partial remission than those receiving intravenous cyclophosphamide. This result suggests that cyclosporin rather than cyclophosphamide should be used as first line therapy for children with SRNS.

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Non-corticosteroid treatment for nephrotic syndrome in children

Cited by 23 publications

References 41 publications

Mycophenolate mofetil versus cyclosporine for remission maintenance in nephrotic syndrome

Mycophenolate mofetil versus cyclosporine for remission maintenance in nephrotic syndrome

Association of cyclophosphamide use with dental developmental defects and salivary gland dysfunction in recipients of childhood antineoplastic therapy

Therapies for steroid-resistant nephrotic syndrome

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