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Pediatric Nephrology

, Volume 22, Issue 11, pp 1875–1880 | Cite as

Steroid-resistant nephrotic syndrome: long-term evolution after sequential therapy

  • Antonia PeñaEmail author
  • Juan Bravo
  • Marta Melgosa
  • Carlota Fernandez
  • Carmen Meseguer
  • Laura Espinosa
  • Angel Alonso
  • M. Luz Picazo
  • Mercedes Navarro
Original Article

Abstract

We present a retrospective study of 30 children of mean age 3.02 ± 1.81 years with steroid-resistant nephrotic syndrome (SRNS) treated with intravenous injection of methylprednisolone plus orally administered prednisone; 24 children also received cyclophosphamide (CP). Sixteen were resistant to steroids from the beginning, and 14 after a mean of 11.26 ± 16.61 months. The initial histological diagnosis was: 18 minimal change disease (MCD), 11 focal segmental glomerulosclerosis (FSGS) and one diffuse mesangial proliferative glomerulonephritis (DMPG). Total remission was achieved in 22 patients (73.3%), partial response in three (10%) and no response in five (16.6%), two of whom were brothers carrying an NPHS2 gene double mutation. There was no difference in response between the MCD and FSGS patients; the only patient with DMPG did not respond. Only initial resistance was a sign of bad prognosis. At follow-up (6.4 ± 3.6 years from last pulse), 21/22 were still in remission, 14/21 were without treatment. Six patients required cyclosporine or mycophenolate mofetil because of steroid dependence. Two non-responders developed end-stage renal failure (ESRF); the remaining patients maintained normal glomerular filtration. The treatment was well tolerated. In conclusion, most of the patients treated with sequential therapy consisting of methylprednisolone (MP) (100%) and CP (80%) showed remission and preserved renal function, but 20% developed steroid dependence.

Keywords

Steroid-resistant nephritic syndrome Pulses Methylprednisolone Cyclosporine Tacrolimus Mycophenolate mofetil Children 

Notes

Acknowledgements

We would like to give special thanks to Elia Pérez Fernández from the Statistics Department of La Paz Hospital for her help with the statistical analysis of the data.

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Copyright information

© IPNA 2007

Authors and Affiliations

  • Antonia Peña
    • 1
    Email author
  • Juan Bravo
    • 1
  • Marta Melgosa
    • 1
  • Carlota Fernandez
    • 1
  • Carmen Meseguer
    • 1
  • Laura Espinosa
    • 1
  • Angel Alonso
    • 1
  • M. Luz Picazo
    • 2
  • Mercedes Navarro
    • 1
  1. 1.NefrologíaHospital Infantil La PazMadridSpain
  2. 2.Department of PathologyHospital Universitario La PazMadridSpain

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