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[Treatment of steroid-resistant idiopathic nephrotic syndrome]

AbstractIdiopathic 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.
AuthorsJ Chemli, A Harbi (Affiliation: Service de néphrologie pédiatrique, CHU Sahloul, Sousse 4054, Tunisie. jalel_chemli at yahoo.fr)
JournalArchives de pédiatrie : organe officiel de la Sociéte française de pédiatrie (Arch Pediatr) Vol. 16 Issue 3 Pg. 260-8 (Mar 2009) ISSN: 0929-693X [Print] France
Vernacular TitleTraitement du syndrome néphrotique idiopathique corticorésistant.
PMID19195856 (Publication Type: English Abstract, Journal Article, Review)
Chemical References
  • Adrenal Cortex Hormones
  • Enzyme Inhibitors
  • Immunosuppressive Agents
Topics
  • Adrenal Cortex Hormones (administration & dosage, adverse effects)
  • Drug Resistance
  • Enzyme Inhibitors (therapeutic use)
  • Humans
  • Immunosorbent Techniques
  • Immunosuppressive Agents (therapeutic use)
  • Kidney Transplantation
  • Nephrotic Syndrome (therapy)
  • Plasmapheresis