title: Unfavorable response to cyclophosphamide in steroid-dependent nephrotic syndrome.
abstract: Development of steroid dependency represents a significant therapeutic challenge in steroid-sensitive nephrotic syndrome. Previous studies have shown conflicting results concerning the benefit of a 12-week treatment with cyclophosphamide (CPO), with 24%-67% of patients achieving long-term remission. We therefore analyzed the clinical response of 20 consecutive children with steroid-dependent nephrotic syndrome (SDNS) (12 male, median age at start of treatment 5.9 years, range 3.2-14.7 years) treated at our institution with CPO (2 mg/kg per day) for 12 weeks since 1989. Median duration of follow-up was 5.8 (range 1.1-9.25) years. Only 6 of 20 children (30%) showed a long-term remission of >2 years, while 14 of 20 (70%) developed relapses again. Of these, 12 patients (86%) again developed steroid dependency, requiring further alternative treatment. Our data show that a 12-week course of CPO leads to unfavorable results in the majority of patients with SDNS. We therefore conclude that there is a need for further optimization of therapy in SDNS.
authors.affiliation: Department of Pediatric Nephrology, University Children's Hospital, Hamburg, Germany. kemper@uke.uni-hamburg.de
authors.forename: M J
authors.initials: MJ
authors.lastname: Kemper
authors.forename: H
authors.initials: H
authors.lastname: Altrogge
authors.forename: K
authors.initials: K
authors.lastname: Ludwig
authors.forename: K
authors.initials: K
authors.lastname: Timmermann
authors.forename: D E
authors.initials: DE
authors.lastname: Müller-Wiefel
mesh_terms: D002648:Child; D003520:Cyclophosphamide / Q000009:adverse effects / Q000627:therapeutic use*; D005260:Female; D015809:HLA-DR7 Antigen / Q000032:analysis; D006801:Humans; D008297:Male; D009404:Nephrotic Syndrome / Q000188:drug therapy* / Q000503:physiopathology; D011241:Prednisone / Q000627:therapeutic use*; D012008:Recurrence; D013256:Steroids / Q000627:therapeutic use*
chemical_list: D015809:HLA-DR7 Antigen; D013256:Steroids; D003520:Cyclophosphamide; D011241:Prednisone