Relationships between Plasma Pyrophosphate, Vascular Calcification and Clinical Severity in Patients Affected by Pseudoxanthoma Elasticum

Relationships between Plasma Pyrophosphate, Vascular Calcification and Clinical Severity in Patients Affected by Pseudoxanthoma Elasticum

Pseudoxanthoma elasticum (PXE; OMIM 264800) is an autosomal recessive metabolic disorder characterized by progressive calcification in the skin, the Bruch’s membrane, and the vasculature. Calcification in PXE results from a low level of circulating pyrophosphate (PPi) caused by ABCC6 deficiency. In...

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Bibliographic Details
Main Authors: Georges Leftheriotis, Nastassia Navasiolava, Laetitia Clotaire, Christophe Duranton, Olivier Le Saux, Saïd Bendahhou, Audrey Laurain, Isabelle Rubera, Ludovic Martin
Format: Article
Language:English
Published: MDPI AG 2022-05-01
Series:Journal of Clinical Medicine
Subjects:
pseudoxanthoma elasticum
inorganic pyrophosphate
ectopic calcification
cardiovascular
PHENODEX score
disease severity
Online Access:https://www.mdpi.com/2077-0383/11/9/2588

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https://www.mdpi.com/2077-0383/11/9/2588

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