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01-07-2013 | Review

An update on the pathomechanisms and future therapies of Alport syndrome

Authors: Damien Noone, Christoph Licht

Published in: Pediatric Nephrology | Issue 7/2013

Abstract

Alport Syndrome (AS) is an inherited progressive disease that is caused by mutations of the genes encoding the key collagen chains, α3, α4, and α5, which are necessary for the composition of collagen type IV to form a robust glomerular basement membrane (GBM), capable of withstanding the significant biomechanical strain to which the glomerulus is subjected. Progressive loss of the filtration barrier allows excessive proteinuria, which ultimately leads to end-stage kidney disease (ESKD). The evidence for a beneficial renoprotective effect of renin-angiotensin-aldosterone system (RAAS) blockade by angiotensin-converting enzyme (ACE) inhibition and/or angiotensin receptor blockers (ARBs) is well established in AS and recent evidence has shown that it can significantly delay the time to onset of renal replacement therapy and ESKD. Future potential treatments of AS disease progression are evaluated in this review.

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Title: An update on the pathomechanisms and future therapies of Alport syndrome
Authors: Damien Noone
Christoph Licht
Publication date: 01-07-2013
Publisher: Springer-Verlag
Published in: Pediatric Nephrology / Issue 7/2013
Print ISSN: 0931-041X
Electronic ISSN: 1432-198X
DOI: https://doi.org/10.1007/s00467-012-2272-z

At a glance: The STEP trials

Springer Medicine

An update on the pathomechanisms and future therapies of Alport syndrome

Abstract

At a glance: The STEP trials

Springer Medicine

Abstract

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