title: Treatment outcome of late steroid-resistant nephrotic syndrome: a study by the Midwest Pediatric Nephrology Consortium.
abstract: BACKGROUND
  Idiopathic nephrotic syndrome (NS) in children is classified as steroid sensitive or steroid resistant. Steroid sensitivity typically portends a low risk of permanent renal failure. However, some initially steroid-sensitive patients later develop steroid resistance. These patients with late steroid resistance (LSR) are often treated with immunosuppressant medications, but the effect of these additional drugs on the long-term prognosis of LSR is still unknown.
  
  
  METHODS
  A retrospective chart review was performed on patients diagnosed with idiopathic NS and subsequent LSR during the 8-year study period from 2002 up to and including 2009, with a minimum of 2 years of follow-up. Primary outcome measures were proteinuria and renal function.
  
  
  RESULTS
  A total of 29 patients were classified as having LSRNS. The majority of patients received treatment with calcineurin inhibitors and/or mycophenolate mofetil. Seven patients received three or more non-steroid immunosuppressant medications. Sustained complete or partial remission was achieved in 69 % of patients. Three developed end-stage renal disease, and all others maintained normal renal function. There were 13 episodes of serious adverse events, none of which were fatal or irreversible.
  
  
  CONCLUSIONS
  Most patients with LSRNS responded to immunosuppressive therapy by reduction or resolution of proteinuria and preservation of renal function. The results suggest that immunosuppressive treatment is a viable option in NS patients who develop LSR.
authors: Department of Pediatrics, Louisiana State University Health Sciences Center, 200 Henry Clay Avenue, New Orleans, LA 70118, USA. carolinestraatmann@gmail.com
authors: Caroline
authors: C
authors: Straatmann
authors: Rose
authors: R
authors: Ayoob
authors: Rasheed
authors: R
authors: Gbadegesin
authors: Keisha
authors: K
authors: Gibson
authors: Michelle N
authors: MN
authors: Rheault
authors: Tarak
authors: T
authors: Srivastava
authors: Cheryl L
authors: CL
authors: Tran
authors: Debbie S
authors: DS
authors: Gipson
authors: Larry A
authors: LA
authors: Greenbaum
authors: William E
authors: WE
authors: Smoyer
authors: V Matti
authors: VM
authors: Vehaskari
mesh_terms: D000704:Analysis of Variance; D002648:Child; D002675:Child, Preschool; D018450:Disease Progression; D004359:Drug Therapy, Combination; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000009:adverse effects / Q000627:therapeutic use*; D007223:Infant; D007668:Kidney / Q000187:drug effects* / Q000473:pathology / Q000503:physiopathology; D007676:Kidney Failure, Chronic / Q000209:etiology; D008297:Male; D015146:Midwestern United States; D009404:Nephrotic Syndrome / Q000150:complications / Q000151:congenital* / Q000175:diagnosis / Q000188:drug therapy / Q000503:physiopathology; D011507:Proteinuria / Q000188:drug therapy / Q000209:etiology; D012074:Remission Induction; D012189:Retrospective Studies; D013997:Time Factors; D016896:Treatment Outcome
chemical_list: D007166:Immunosuppressive Agents
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