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Is cyclophosphamide effective in patients with IgM-positive minimal change disease?

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Abstract

Background

We analyzed the impact of immunoglobulin M (IgM) positivity on the relapse-free interval post completed course of cyclophosphamide (CYC) treatment in patients with steroid-dependent nephrotic syndrome (SDNS) and minimal change disease (MCD).

Methods

This was a retrospective chart review of all children who received CYC for SDNS and MCD between 1988 and 2009. Patients were divided into three groups based on kidney biopsy: MCD without immunoglobulin M (IgM) positivity (IgM−), MCD with IgM-positive immunofluorescence (IF) only (IgM+), and MCD with IgM-positive IF and electron-dense deposits on electron microscopy (IgM++). The relapse-free time interval to the first relapse post-CYC therapy or up to 48 months of follow-up (if no relapse occurred) was used for survival analysis.

Results

Forty children aged 1.5–12.3 years (15 were IgM−, 16 were IgM+, 9 were IgM++) received a cumulative CYC dose of 175 ± 30 mg/kg. The overall relapse-free survival time was 75 % at 12 months, 64 % at 24 months, 59 % at 36 months, and 56 % at 48 months, with no significant differences between the IgM groups (p = 0.80).

Conclusions

Based on our results, we conclude that more than 50% of our SDNS patients with MCD remained relapse-free 4 years post-CYC treatment. No significant difference in the response to CYC was observed between patients with or without IgM positivity.

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Authors and Affiliations

  1. Division of Nephrology, Department of Pediatrics, Children’s Hospital of Eastern Ontario, 401 Smyth Road, Ottawa, Ontario, K1H 8L1, Canada

    Pavel Geier, Amani Roushdi, Sylva Skálová, Jennifer Vethamuthu, Gabrielle Weiler & Janusz Feber

  2. Department of Pediatrics, University Hospital, Hradec Králové, Czech Republic

    Sylva Skálová

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  1. Pavel Geier
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  2. Amani Roushdi
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  3. Sylva Skálová
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  4. Jennifer Vethamuthu
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  5. Gabrielle Weiler
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  6. Janusz Feber
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Correspondence to Janusz Feber.

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Geier, P., Roushdi, A., Skálová, S. et al. Is cyclophosphamide effective in patients with IgM-positive minimal change disease?. Pediatr Nephrol 27, 2227–2231 (2012). https://doi.org/10.1007/s00467-012-2234-5

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  • DOI: https://doi.org/10.1007/s00467-012-2234-5

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