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Mécanismes moléculaires du syndrome néphrotique idiopathiqueMolecular basis of idiopathic nephrotic syndrome.
Authors:G Deschênes  L Martinat
Institution:1 Service de néphrologie pédiatrique, hôpital Armand-Trousseau, 26, avenue du Docteur-Arnold-Netter, 75571  Paris cedex 12, France;2 service de pédiatrie générale, centre hospitalier Lucien-Hussel, 38200 Vienne, France
Abstract:Steroid-sensitive idiopathic nephrotic syndrome is a T-cell disorder associated with a functional renal impairment. The molecular mechanisms leading from the stimulation of the immune system to the clinical expression of the renal disease can be analyzed according to five biological events: 1) a Th2 activation of T-cells by interleukin-13; 2) a yet unidentified glomerular permeability factor from immune origin; 3) a molecular disorientation of slit diaphragms or glomerular basement membrane responsible for proteinuria; 4) a podocyte cytoskeleton rearrangement responsible for foot process effacement; and 5) renal avidity for sodium and edema formation resulting from a primary stimulation of tubular Na,K-ATPase and an increase of endothelial permeability.
Keywords:Mots-clé  : syndrome né  phrotique  biologie molé  culaireMots-clé  : nephrotic syndrome  minimal change  molecular biology  review
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