title: High-dose mizoribine therapy for childhood-onset frequently relapsing steroid-dependent nephrotic syndrome with cyclosporin nephrotoxicity.
abstract: Cyclosporin A (CsA) is an effective treatment for frequently relapsing steroid-dependent nephrotic syndrome (FR-SDNS), but its use can be complicated by renal toxicity and a high incidence of relapses after withdrawal. We report 9 adolescent patients with childhood-onset FR-SDNS who had been treated with long-term CsA that resulted in moderate-to-severe CsA nephropathy (CsAN). They were treated with high-dose (mean: 10.1 mg/kg per day) mizoribine (MZR) in an attempt to allow weaning of CsA and/or steroid therapy, and reduce the frequency of relapses. Seven out of 9 patients were weaned off CsA by 1-year follow-up, although in the remaining 2 patients, MZR did not show any beneficial effects. Overall, this high-dose MZR therapy results in significant steroid sparing and reduction in relapse rates in our patients. Our experience shows that high-dose MZR therapy in patients with FR-SDNS who are also CsA-dependent appears to be effective in reducing CsA exposure as well as in decreasing the frequency of relapses.
authors.affiliation: Department of Nephrology, Saitama Children's Medical Center, Saitama, Japan. ohtomo_dr@hotmail.com
authors.forename: Yoshiyuki
authors.initials: Y
authors.lastname: Ohtomo
authors.forename: Shu-ichiro
authors.initials: S
authors.lastname: Fujinaga
authors.forename: Masaru
authors.initials: M
authors.lastname: Takada
authors.forename: Hitohiko
authors.initials: H
authors.lastname: Murakami
authors.forename: Shunji
authors.initials: S
authors.lastname: Akashi
authors.forename: Toshiaki
authors.initials: T
authors.lastname: Shimizu
authors.forename: Kazunari
authors.initials: K
authors.lastname: Kaneko
authors.forename: Yuichiro
authors.initials: Y
authors.lastname: Yamashiro
mesh_terms: D000293:Adolescent; D000328:Adult; D017668:Age of Onset; D000894:Anti-Inflammatory Agents, Non-Steroidal / Q000008:administration & dosage*; D002648:Child; D002675:Child, Preschool; D016572:Cyclosporine / Q000008:administration & dosage / Q000009:adverse effects / Q000627:therapeutic use*; D004334:Drug Administration Schedule; D004359:Drug Therapy, Combination; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D007166:Immunosuppressive Agents / Q000008:administration & dosage; D007223:Infant; D007564:Japan / Q000453:epidemiology; D008297:Male; D009402:Nephrosis, Lipoid / Q000188:drug therapy*; D009404:Nephrotic Syndrome / Q000175:diagnosis / Q000188:drug therapy* / Q000453:epidemiology / Q000473:pathology; D012263:Ribonucleosides / Q000008:administration & dosage*; D055502:Secondary Prevention; D013997:Time Factors; D016896:Treatment Outcome
chemical_list: D000894:Anti-Inflammatory Agents, Non-Steroidal; D007166:Immunosuppressive Agents; D012263:Ribonucleosides; C010052:mizoribine; D016572:Cyclosporine