Authors: Antonia Peña Juan Bravo Marta Melgosa Carlota Fernandez Carmen Meseguer Laura Espinosa Angel Alonso M Luz Picazo Mercedes Navarro
Publish Date: 2007/09/18
Volume: 22, Issue: 11, Pages: 1875-1880
Abstract
We present a retrospective study of 30 children of mean age 302 ± 181 years with steroidresistant 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 1126 ± 1661 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 733 partial response in three 10 and no response in five 166 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 followup 64 ± 36 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 nonresponders developed endstage 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
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