Intravenous methylprednisolone and oral alkylating agent therapy of prednisone-resistant pediatric focal segmental glomerulosclerosis: a long-term follow-up.

摘要: Prednisone-resistant nephrotic syndrome (NS) due to focal segmental glomerulosclerosis (FSGS), the most common acquired disease requiring chronic dialysis and transplantation in children, has a low likelihood of response alkylating agent therapy. This report summarizes results 0.75-12.5 (average 6.33) year follow-up 32 pediatric cases prednisone-resistant FSGS treated with regimen high-dose intravenous methylprednisolone (M-P) alternate-day prednisone, plus an 25/32. On last followup: 21/32 were remission [urine protein-to-creatinine ratios (Pr u /Cr ) ≤0.2]; 3/32 had mild proteinuria >0.2-0.5); 2/32 moderate >0.5-1.9); 6/32 remained ≥2.0). Of incomplete or nonresponders: 3/11 progressed end-stage renal failure; 5/11 decreased creatinine clearances (CrCl); persistent normal CrCl. All persistently but none complete responders, developed responders able stop treatment; four relapsed responded well retreatment. Conclusions: without agent, is effeciive achieving sustained remissions preserving function great majority children NS