Clinical and Experimental Nephrology

, Volume 23, Issue 2, pp 244–250 | Cite as

Positive effects of single-daily high-dose mizoribine therapy after cyclophosphamide in young children with steroid-dependent nephrotic syndrome

  • Akira Mizutani
  • Shuichiro FujinagaEmail author
  • Koji Sakuraya
  • Daishi Hirano
  • Toshiaki Shimizu
Original article



Mizoribine (MZR) therapy after cyclophosphamide (CPM) therapy may be an attractive option in patients with steroid-dependent nephrotic syndrome (SDNS) for the purpose of maintaining remission. This is because CPM is administered only once due to its severe side effects such as gonadal toxicity. However, the long-term prognosis after the treatment regimen remains unknown.


We retrospectively analyzed the clinical course (median follow-up, 5.9 years) of 54 young children with SDNS (43 boys; age < 10 years) who had undergone 12-week CPM therapy. The patients were classified into two groups: group A, undergoing MZR therapy for > 12 months for maintaining remission after CPM therapy (N = 36), and group B, undergoing CPM monotherapy (N = 18).


For 2 years after CPM therapy, 21 of the 36 group A patients were in sustained remission, whereas only 4 of the 18 group B patients had maintained remission (58% vs. 22%, p < 0.05). Furthermore, the rate of regression to SDNS after CPM was significantly lower in group A than in group B (6% vs. 39%, p < 0.05). At the last follow-up (mean age, 10.9 years), 27 of the 36 group A patients (75%) had not received any steroid-sparing agent after the treatment regimen.


Single daily high-dose MZR therapy after CPM therapy may have positive outcomes in young children with SDNS in the long term.


Mizoribine Cyclophosphamide Steroid-dependent nephrotic syndrome Young children 



SF received clinical research funding B at Saitama Children’s Medical Center.

Compliance with ethical standards

Conflict of interest

The authors declare no conflicts of interest.

Ethical standards

All procedures performed in studies involving human participants were in accordance with the ethical standards of the Institutional Research Committee and/or National Research Committee at which the study was conducted (approval number 2018-02-19) with the 1964 Helsinki Declaration and its later amendments or comparable ethical standards.

Informed consent

Informed consent was obtained from all participants included in this study.


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Copyright information

© Japanese Society of Nephrology 2018

Authors and Affiliations

  1. 1.Division of NephrologySaitama Children’s Medical CenterSaitama-cityJapan
  2. 2.Department of Pediatrics and Adolescent MedicineJuntendo University Graduate School of MedicineTokyoJapan
  3. 3.Department of PediatricsThe Jikei University School of MedicineTokyoJapan

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