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[Treatment of steroid-resistant idiopathic nephrotic syndrome]. | LitMetric

[Treatment of steroid-resistant idiopathic nephrotic syndrome].

Arch Pediatr

Service de néphrologie pédiatrique, CHU Sahloul, Sousse 4054, Tunisie.

Published: March 2009

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.

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Source
http://dx.doi.org/10.1016/j.arcped.2008.11.018DOI Listing

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