Nephrotic Syndrome in Children: From Bench to Treatment

Idiopathic nephrotic syndrome (INS) is the most frequent form of NS in children. INS is defined by the association of the clinical features of NS with renal biopsy findings of minimal changes, focal segmental glomerulosclerosis (FSGS), or mesangial proliferation (MP) on light microscopy and effaceme...

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Bibliographic Details
Main Authors: J.-C. Davin, N. W. Rutjes
Format: Article
Language:English
Published: Hindawi Limited 2011-01-01
Series:International Journal of Nephrology
Online Access:http://dx.doi.org/10.4061/2011/372304
Description
Summary:Idiopathic nephrotic syndrome (INS) is the most frequent form of NS in children. INS is defined by the association of the clinical features of NS with renal biopsy findings of minimal changes, focal segmental glomerulosclerosis (FSGS), or mesangial proliferation (MP) on light microscopy and effacement of foot processes on electron microscopy. Actually the podocyte has become the favourite candidate for constituting the main part of the glomerular filtration barrier. Most cases are steroid sensitive (SSINS). Fifty percents of the latter recur frequently and necessitate a prevention of relapses by nonsteroid drugs. On the contrary to SSINS, steroid resistant nephrotic syndrome (SRINS) leads often to end-stage renal failure. Thirty to forty percents of the latter are associated with mutations of genes coding for podocyte proteins. The rest is due to one or several different circulating factors. New strategies are in development to antagonize the effect of the latter.
ISSN:2090-214X
2090-2158