title: Prospective 5-year follow-up of cyclosporine treatment in children with steroid-resistant nephrosis.
abstract: BACKGROUND
  Cyclosporine has improved remission rates in children with steroid-resistant nephrotic syndrome (SRNS). However, little prospective long-term follow-up data is available.
  
  
  METHODS
  We prospectively followed and analyzed 5-year outcomes of all 35 patients enrolled in our previous prospective multicenter trial with cyclosporine and steroids in children with SRNS. At enrollment, 23 cases were classified as minimal change (MC), five as diffuse mesangial proliferation (DMP), and seven as focal segmental glomerulosclerosis (FSGS).
  
  
  RESULTS
  Renal survival at 5 years (median 7.7 years) was 94.3 %. Patient status was complete remission (CR) in 31 (88.6 %) (MC/DMP, 25; FSGS, 6); partial remission in one (FSGS); and non-remission in three (MC/DMP), including chronic kidney disease and end-stage kidney disease in one each. Among 31 patients with CR, 22 (71.0 %) were receiving treatment with immunosuppressants at 5 years, including cyclosporine in 19, and seven of these 22 continued to show frequent relapse. Response to cyclosporine at 4 months predicted 5-year outcome in 31 of 35 patients.
  
  
  CONCLUSIONS
  Although SRNS treatment with cyclosporine provides high renal survival and remission rates, many children require ongoing immunosuppression. Management has advanced from the prevention of end-stage kidney disease to the long-term maintenance of remission and management of relapse after induction therapy.
authors: Department of Nephrology, Tokyo Metropolitan Children's Medical Center, Fuchu, Japan. yuhamasaki@med.toho-u.ac.jp
authors: Yuko
authors: Y
authors: Hamasaki
authors: Norishige
authors: N
authors: Yoshikawa
authors: Hitoshi
authors: H
authors: Nakazato
authors: Satoshi
authors: S
authors: Sasaki
authors: Kazumoto
authors: K
authors: Iijima
authors: Koichi
authors: K
authors: Nakanishi
authors: Takeshi
authors: T
authors: Matsuyama
authors: Kenji
authors: K
authors: Ishikura
authors: Shuichi
authors: S
authors: Ito
authors: Tetsuji
authors: T
authors: Kaneko
authors: Masataka
authors: M
authors: Honda
mesh_terms: D000293:Adolescent; D002648:Child; D016572:Cyclosporine / Q000009:adverse effects / Q000627:therapeutic use*; D018450:Disease Progression; D004359:Drug Therapy, Combination; D005260:Female; D005500:Follow-Up Studies; D005923:Glomerulosclerosis, Focal Segmental / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy*; D006801:Humans; D007166:Immunosuppressive Agents / Q000009:adverse effects / Q000627:therapeutic use*; D007564:Japan; D007668:Kidney / Q000187:drug effects* / Q000473:pathology; D007676:Kidney Failure, Chronic / Q000209:etiology; D008297:Male; D008775:Methylprednisolone / Q000627:therapeutic use; D009402:Nephrosis, Lipoid / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy*; D009404:Nephrotic Syndrome / Q000150:complications / Q000151:congenital* / Q000175:diagnosis / Q000188:drug therapy; D011446:Prospective Studies; D012008:Recurrence; D012074:Remission Induction; D051436:Renal Insufficiency, Chronic / Q000209:etiology; D013997:Time Factors; D016896:Treatment Outcome; D055815:Young Adult
chemical_list: D007166:Immunosuppressive Agents; D016572:Cyclosporine; D008775:Methylprednisolone