Interventions for idiopathic steroid-resistant nephrotic syndrome in children

Hodson, Elisabeth M; Habashy, Doaa; Craig, Jonathan C.

doi:10.1002/14651858.cd003594.pub3

Cited by 34 publications

(38 citation statements)

References 30 publications

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“…Response to cyclosporine was significantly better, with 88% achieving complete or partial remission. A favorable response to cyclosporine in the treatment of steroid-dependent or steroid-resistant nephrotic syndrome has been previously noted in several clinical studies [28][29][30][31][32]. However, most of these studies did not include children with IgM nephropathy [28,29].…”

Section: Discussionmentioning

confidence: 99%

Minimal change disease with IgM+ immunofluorescence: a subtype of nephrotic syndrome

et al. 2009

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Immunoglobulin (Ig) M nephropathy is defined by electron-dense mesangial deposits and mesangial IgM visible by immunofluorescence (IF) without other histopathologic and immunofluorescent microscopic abnormalities. Certain patients have only immuno-positive (IgM+) IF. Children presenting with steroid-dependent or steroid-resistant nephrotic syndrome have a high prevalence of IgM+ IF with or without electron-dense deposits. We reviewed the clinical course of children with steroid-dependent or steroid-resistant nephrotic syndrome who underwent renal biopsy at Texas Children's Hospital from 1989 to 2006 to further characterize IgM+ IF in children with nephrotic syndrome. Of the 55 children with steroid-resistant or -dependent minimal change disease (MCD), 23 had IgM+ IF. Of these 23 children, 61% had microscopic hematuria at presentation, 48% (11/23) were steroid-dependent, and 48% (11/23) steroid-resistant (one underwent biopsy prior to steroid therapy). We compared the efficacy of adjuvant treatment with cyclophosphamide and cyclosporine: 18% initially treated with cyclophosphamide obtained remission, while 55% had no response; 83% obtained subsequent remission with cyclosporine. Of those initially treated with cyclosporine, 88% obtained complete or partial remission. IgM+ IF may be surrogate marker for the severity of MCD. Based on our results, children with MCD and IgM+ IF have a better response to cyclosporine than cyclophosphamide.

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Section: Discussionmentioning

confidence: 99%

Minimal change disease with IgM+ immunofluorescence: a subtype of nephrotic syndrome

et al. 2009

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“…The RCTs comparing oral alkylating agents with prednisone or indomethacin in children with SRNS have found no significant difference in the number of children with complete remission (three RCTs, 114 children; RR 1.00; 95% CI 0.78-1.29) or complete or partial remission (one RCT, 53 children; RR 1.17; 95% CI 0.64-2.13) [23,26]. In the two trials comparing cyclophosphamide with prednisone [27, 28], 35% of 34 children achieved remission with prednisone alone.…”

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confidence: 96%

“…A systematic review updated in 2006 identified only 11 RCTs (280 children) that had evaluated therapies for SRNS [23]. Most of these trials included children with SRNS due to MCD [20,24].…”

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confidence: 99%

“…One trial [23,30] has compared intravenous cyclophosphamide with oral cyclophosphamide in which both patient groups received oral prednisone. All seven children treated with intravenous cyclophosphamide achieved complete remission compared with only one of four children treated with oral cyclophosphamide; however, due to small numbers there was no significant difference between therapies (RR 0.09; 95% CI 0.01-1.39).…”

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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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“…Overall, nearly two thirds were steroid-resistant, and 30% of the FSGS patients progressed to ESRD. Data on the responsiveness of FSGS to therapy in the pediatric population is problematic in and of itself, as there have been very few controlled treatment trials, and most case series contain small numbers of patients [58,[62][63][64]. While the frequency of progression to ESRD appears to be very high in C1q nephropathy patients with FSGS, this is not substantially different from that seen in the adult population, or in more recent reports of long-term follow up of FSGS in children [65,66].…”

Section: Clinical Correlationsmentioning

confidence: 99%

C1q nephropathy in the pediatric population: pathology and pathogenesis

Wenderfer

Swinford²,

Braun

2010

Pediatr Nephrol

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C1q nephropathy was originally described nearly 25 years ago by Jennette and Hipp. Since that time there have been a limited number of publications on C1q nephropathy, most of them in the pediatric literature. Despite reported incidences as high as 16% in some pediatric biopsy series, a consensus definition on the diagnosis of C1q nephropathy is lacking and its existence as a distinct clinical disease entity remains controversial. The purpose of this review is to discuss the biology of C1q in the context of mechanisms of C1q deposition, and to provide a detailed analysis of the published pediatric case series with a focus on the pathological criteria used to establish the diagnosis of C1q nephropathy as well as long-term outcomes in children.

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Interventions for idiopathic steroid-resistant nephrotic syndrome in children

Cited by 34 publications

References 30 publications

Minimal change disease with IgM+ immunofluorescence: a subtype of nephrotic syndrome

Minimal change disease with IgM+ immunofluorescence: a subtype of nephrotic syndrome

Therapies for steroid-resistant nephrotic syndrome

C1q nephropathy in the pediatric population: pathology and pathogenesis

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