Treatment outcome of late steroid-resistant nephrotic syndrome: a study by the Midwest Pediatric Nephrology Consortium.

Journal Article (Journal Article;Multicenter Study)

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. CONCLUSION: 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.

Full Text

Duke Authors

Cited Authors

  • Straatmann, C; Ayoob, R; Gbadegesin, R; Gibson, K; Rheault, MN; Srivastava, T; Tran, CL; Gipson, DS; Greenbaum, LA; Smoyer, WE; Vehaskari, VM

Published Date

  • August 2013

Published In

Volume / Issue

  • 28 / 8

Start / End Page

  • 1235 - 1241

PubMed ID

  • 23633037

Electronic International Standard Serial Number (EISSN)

  • 1432-198X

Digital Object Identifier (DOI)

  • 10.1007/s00467-013-2483-y


  • eng

Conference Location

  • Germany