title: Three year outcome of childhood idiopathic nephrotic syndrome under a unified immunosuppressive protocol.
abstract: BACKGROUND
  This retrospective study was performed to assess the 3 year outcome of a unified protocol for childhood idiopathic nephrotic syndrome.
  
  
  METHODS
  Cyclosporine A (CsA) or CsA plus mycophenolate mofetil (MMF) were used in patients without remission on high-dose steroid therapy. CsA was maintained at an area under the whole blood concentration-time curve up to 4 h after dose (AUC0-4 ) of 1500 and 2000 ng·h/mL in steroid-dependent nephrotic syndrome (SDNS) and steroid-resistant nephrotic syndrome (SRNS), respectively. Ninety-one children were enrolled in the study (SDNS, n = 64; SRNS, n = 18). Patients were divided into minimal change (MC) and focal segmental glomerulosclerosis (FSGS) groups. Three year outcome was evaluated using clinical severity defined as degree of dependence on immunosuppressive therapy for maintenance of remission.
  
  
  RESULTS
  In the SDNS group, the numbers of MC and no biopsy were 51 and 13, respectively. No patient had FSGS. Twelve SRNS patients had FSGS and six had MC. In SDNS, 15/64 patients (23%) received no medication. CsA was effective as steroid-sparing agent in 31/38 patients (82%). MMF was effective in all eight patients for whom CsA was unsuccessful. Remission rate in the SRNS group was 14/18 (78%; eight with CsA, and six with a combination of CsA + MMF). Five of the 14 SRNS remission patients received methylprednisolone pulse therapy. Four were resistant to therapy, and had impaired renal function. The clinical severity of MC and FSGS overlapped.
  
  
  CONCLUSIONS
  Treatment with CsA and combination of CsA plus MMF was useful for SDNS and for remission induction in SRNS.
authors.affiliation: Pediatric Nephrology Aichi Children's Health and Medical Center, Aichi, Japan.
authors.forename: Satoshi
authors.initials: S
authors.lastname: Hibino
authors.forename: Osamu
authors.initials: O
authors.lastname: Uemura
authors.forename: Takuhito
authors.initials: T
authors.lastname: Nagai
authors.forename: Satoshi
authors.initials: S
authors.lastname: Yamakawa
authors.forename: Naoyuki
authors.initials: N
authors.lastname: Iwata
authors.forename: Hidekazu
authors.initials: H
authors.lastname: Ito
authors.forename: Masaru
authors.initials: M
authors.lastname: Nakano
authors.forename: Kazuki
authors.initials: K
authors.lastname: Tanaka
mesh_terms: D002675:Child, Preschool; D016572:Cyclosporine / Q000008:administration & dosage*; D004305:Dose-Response Relationship, Drug; D004359:Drug Therapy, Combination; D005260:Female; D005500:Follow-Up Studies; D005938:Glucocorticoids / Q000008:administration & dosage*; D006801:Humans; D007166:Immunosuppressive Agents / Q000008:administration & dosage; D008297:Male; D009173:Mycophenolic Acid / Q000008:administration & dosage*; D009404:Nephrotic Syndrome / Q000188:drug therapy*; D012074:Remission Induction; D012189:Retrospective Studies; D013997:Time Factors; D016896:Treatment Outcome
chemical_list: D005938:Glucocorticoids; D007166:Immunosuppressive Agents; D016572:Cyclosporine; D009173:Mycophenolic Acid
keywords: childhood; cyclosporine A; mycophenolate mofetil; steroid-dependent nephrotic syndrome; steroid-resistant nephrotic syndrome