[Treatment of steroid-resistant idiopathic nephrotic syndrome].
Idiopathic nephrotic syndrome (INS) is the most frequent glomerular nephropathy in children. The response to corticoids distinguishes steroid-sensitive nephrotic syndrome (SSINS), by far the most frequent (90% of cases), from steroid-resistant nephrotic syndrome (SRINS). The steroid resistance of nephrotic syndrome is defined by the absence of remission after a full dose of oral corticosteroid therapy for 1 month followed by 3 pulses of intravenous methylprednisolone. Actually, INS constitutes a heterogeneous nosologic entity. Currently, within the SRINS, there are 2 forms that vary greatly in their physiopathology and prognostics: immunologic or sporadic forms, which can be improved by immunosuppressive agents and the genetic or familial forms, which do not respond to any immunosuppressive therapy and usually evolve to end-stage renal insufficiency. In these genetic forms, renal transplantation is the only therapeutic alternative. The aim of this article is to review treatment of SRINS and to propose a management strategy.
Chemli
J
J
Service de néphrologie pédiatrique, CHU Sahloul, Sousse 4054, Tunisie. jalel_chemli@yahoo.fr
Harbi
A
A
D000305:Adrenal Cortex Hormones / Q000008:administration & dosage / Q000009:adverse effects*; D004351:Drug Resistance; D004791:Enzyme Inhibitors / Q000627:therapeutic use; D006801:Humans; D007163:Immunosorbent Techniques; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D016030:Kidney Transplantation; D009404:Nephrotic Syndrome / Q000628:therapy*; D010956:Plasmapheresis
D000305:Adrenal Cortex Hormones; D004791:Enzyme Inhibitors; D007166:Immunosuppressive Agents