title: Steroid-resistant nephrotic syndrome: long-term evolution after sequential therapy.
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.
authors: Nefrología, Hospital Infantil La Paz, Paseo de la Castellana 261, Madrid 28046, Spain. apena.hulp@salud.madrid.org
authors: Antonia
authors: A
authors: Peña
authors: Juan
authors: J
authors: Bravo
authors: Marta
authors: M
authors: Melgosa
authors: Carlota
authors: C
authors: Fernandez
authors: Carmen
authors: C
authors: Meseguer
authors: Laura
authors: L
authors: Espinosa
authors: Angel
authors: A
authors: Alonso
authors: M Luz
authors: ML
authors: Picazo
authors: Mercedes
authors: M
authors: Navarro
mesh_terms: D000284:Administration, Oral; D000893:Anti-Inflammatory Agents / Q000008:administration & dosage* / Q000009:adverse effects; D002648:Child; D002675:Child, Preschool; D003520:Cyclophosphamide / Q000008:administration & dosage* / Q000009:adverse effects; D018450:Disease Progression; D004351:Drug Resistance; D005500:Follow-Up Studies; D015432:Glomerulonephritis, Membranoproliferative / Q000188:drug therapy; D005923:Glomerulosclerosis, Focal Segmental / Q000188:drug therapy; D006801:Humans; D007166:Immunosuppressive Agents / Q000008:administration & dosage* / Q000009:adverse effects; D007223:Infant; D007275:Injections, Intravenous; D008297:Male; D008775:Methylprednisolone / Q000008:administration & dosage* / Q000009:adverse effects; D009402:Nephrosis, Lipoid / Q000188:drug therapy; D009404:Nephrotic Syndrome / Q000188:drug therapy*; D012074:Remission Induction; D012189:Retrospective Studies; D013030:Spain
chemical_list: D000893:Anti-Inflammatory Agents; D007166:Immunosuppressive Agents; D003520:Cyclophosphamide; D008775:Methylprednisolone