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Le pseudoxanthome élastique

une cause rare de calcifications vasculaires chez l'enfant

Language FrenchFrench
Book Paperback
Book Le pseudoxanthome élastique Asmaa Dibi
Libristo code: 13709066
Publishers Editions universitaires europeennes EUE, July 2016
Les calcifications vasculaires sont exceptionnelles chez l'enfant, leurs principales causes sont gén... Full description
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Les calcifications vasculaires sont exceptionnelles chez l'enfant, leurs principales causes sont génétiques dont le pseudoxanthome élastique (PXE). C'est une pathologie due ŕ des mutations du gčne ABCC6 et transmit selon un mode autosomique récessif. Le rôle de ABCC6 et la nature de son substrat (s) dans le PXE restent inconnus. L'expression phénotypique de la maladie est caractérisée par la fragmentation et la minéralisation des fibres élastiques dans la paroi de la peau, de la rétine et artérielles. Le PXE représente une maladie métabolique prototypique qui partage certaines des caractéristiques de calcifications vasculaires avec des maladies acquises d'origine métabolique tels que le diabčte de type II et les maladies rénales chroniques. D'un point de vue clinique, l'absence actuelle d'un traitement ciblé efficace nécessite le contrôle et la prise en charge rigoureuse des facteurs de risque cardio-vasculaires associés au PXE, en plus de la limitation des conditions pro-calcifiante. De nouvelles perspectives prometteuses pour des traitements étiologiques sont cependant en cours, et peuvent rapidement donner de nouvelles options de traitement pour les patients atteints de PXE.

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About the book

Full name Le pseudoxanthome élastique
Author Asmaa Dibi
Language French
Binding Book - Paperback
Date of issue 2016
Number of pages 68
EAN 9783639528466
ISBN 3639528468
Libristo code 13709066
Publishers Editions universitaires europeennes EUE
Weight 118
Dimensions 150 x 220 x 4

Categories

Medicine & Nursing > Clinical & internal medicine
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