Steroid-resistant nephrotic syndrome: long-term evolution after sequential therapy
| Autor: | A. Peña, Carmen Garcia Meseguer, Angel Alonso, M. Luz Picazo, Mercedes Navarro, Juan Bravo, L. Espinosa, C. Fernández, Marta Melgosa |
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| Rok vydání: | 2007 |
| Předmět: |
Male
medicine.medical_specialty Nephrotic Syndrome Glomerulonephritis Membranoproliferative Anti-Inflammatory Agents Drug Resistance Administration Oral Methylprednisolone Gastroenterology Focal segmental glomerulosclerosis Prednisone Internal medicine medicine Humans Minimal change disease Child Cyclophosphamide Retrospective Studies Glomerulosclerosis Focal Segmental business.industry Nephrosis Lipoid Remission Induction Infant Glomerulonephritis medicine.disease Steroid-resistant nephrotic syndrome Endocrinology Spain Nephrology Child Preschool Injections Intravenous Pediatrics Perinatology and Child Health Disease Progression Mesangial proliferative glomerulonephritis business Nephrotic syndrome Immunosuppressive Agents Follow-Up Studies medicine.drug |
| Zdroj: | Pediatric Nephrology. 22:1875-1880 |
| ISSN: | 1432-198X 0931-041X |
| DOI: | 10.1007/s00467-007-0567-2 |
| Popis: | 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. |
| Databáze: | OpenAIRE |
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