title: Cyclosporine and steroid therapy in children with steroid-resistant nephrotic syndrome.
abstract: We conducted a prospective, multicenter trial to evaluate the efficacy and safety of a 12-month course of cyclosporine in children with steroid-resistant nephrotic syndrome (SRNS). Thirty-five patients were enrolled, of whom 28 had minimal change or diffuse mesangial proliferation (MC/DMP), and seven had focal segmental glomerulosclerosis (FSGS). All patients received cyclosporine and prednisolone; patients with FSGS additionally received methylprednisolone pulse therapy (MPT). The dose of cyclosporine was adjusted to maintain a trough level of 120-150 ng/ml during the initial 3 months of treatment, followed by 80-100 ng/ml during months 4-12. The primary end point was the remission rate at month 12. Remission was achieved in 23 of 28 (82.1%) patients in the MC/DMP group and in six of the seven (85.7%) patients in the FSGS group. Follow-up renal biopsies were performed in 26 patients (nine at month 12, 17 at month 24), and cyclosporine-related nephrotoxicity was detected in one (3.8%). Major adverse events comprised severe bacterial infections (two patients) and posterior reversible encephalopathy syndrome (one patient). In conclusion, a high remission rate was achieved in our patient cohort using a combined cyclosporine/ prednisolone treatment regimen in children with SRNS who had MC/DMP and a combined cyclosporine/prednisolone plus MPT regimen in children who had FSGS.
authors.affiliation: Department of Pediatric Nephrology, Tokyo Metropolitan Kiyose Children's Hospital, Kiyose-city, Japan. yu.hama@chp-kiyose-tokyo.jp
authors.forename: Yuko
authors.initials: Y
authors.lastname: Hamasaki
authors.forename: Norishige
authors.initials: N
authors.lastname: Yoshikawa
authors.forename: Shinzaburo
authors.initials: S
authors.lastname: Hattori
authors.forename: Satoshi
authors.initials: S
authors.lastname: Sasaki
authors.forename: Kazumoto
authors.initials: K
authors.lastname: Iijima
authors.forename: Koichi
authors.initials: K
authors.lastname: Nakanishi
authors.forename: Takeshi
authors.initials: T
authors.lastname: Matsuyama
authors.forename: Kenji
authors.initials: K
authors.lastname: Ishikura
authors.forename: Nahoko
authors.initials: N
authors.lastname: Yata
authors.forename: Tetsuji
authors.initials: T
authors.lastname: Kaneko
authors.forename: Masataka
authors.initials: M
authors.lastname: Honda
mesh_terms: D000284:Administration, Oral; D001706:Biopsy; D002648:Child; D002675:Child, Preschool; D016572:Cyclosporine / Q000627:therapeutic use*; D018450:Disease Progression; D004305:Dose-Response Relationship, Drug; D004334:Drug Administration Schedule; D004351:Drug Resistance; D004359:Drug Therapy, Combination; D005260:Female; D005500:Follow-Up Studies; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D007262:Infusions, Intravenous; D007564:Japan; D008297:Male; D008775:Methylprednisolone / Q000627:therapeutic use; D009404:Nephrotic Syndrome / Q000188:drug therapy* / Q000473:pathology; D011239:Prednisolone / Q000627:therapeutic use; D011446:Prospective Studies; D016032:Randomized Controlled Trials as Topic; D012074:Remission Induction; D013256:Steroids / Q000378:metabolism*; D013997:Time Factors; D016896:Treatment Outcome
chemical_list: D005938:Glucocorticoids; D007166:Immunosuppressive Agents; D013256:Steroids; D016572:Cyclosporine; D011239:Prednisolone; D008775:Methylprednisolone