Some chronic diseases, notably liver or kidney diseases, are associated with a high prevalence of arterial calcification, the determinants of which are not known. In the most advanced stages of these diseases, characterized by organ fibrosis and then organ failure, liver or kidney transplantation is suggested.Pyrophosphate is a potent...
-
March 31, 2022 (v1)PublicationUploaded on: December 3, 2022
-
June 13, 2024 (v1)Journal article
Pseudoxanthoma elasticum (PXE) is a rare inherited systemic disease responsible for a juvenile peripheral arterial calcification disease. The clinical diagnosis of PXE is only based on a complex multi-organ phenotypic score and/or genetical analysis. Reduced plasma inorganic pyrophosphate concentration [PPi]p has been linked to PXE. In this...
Uploaded on: July 27, 2024 -
2022 (v1)Journal article
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 this study, we used a cohort of 107 PXE...
Uploaded on: December 4, 2022 -
2020 (v1)Journal article
Introduction: Patients on dialysis and kidney transplant recipients (KTR) present the syndrome of mineral and bone disorders (MBD), which share common traits with monogenic calcifying diseases related to disturbances of the purinergic system. Low plasma levels of inorganic pyrophosphate (PPi) and ectopic vascular calcifications belong to these...
Uploaded on: December 3, 2022 -
2022 (v1)Journal article
Liver fibrosis is associated with arterial calcification (AC). Since the liver is a source of inorganic pyrophosphate (PPi), an anti-calcifying compound, we investigated the relationship between plasma PPi ([PPi]pl), liver fibrosis, liver function, AC, and the hepatic expression of genes regulating PPi homeostasis. To that aim, we compared...
Uploaded on: December 4, 2022 -
June 24, 2022 (v1)Journal article
Liver fibrosis is associated with arterial calcification (AC). Since the liver is a source of inorganic pyrophosphate (PPi), an anti-calcifying compound, we investigated the relationship between plasma PPi ([PPi]pl), liver fibrosis, liver function, AC, and the hepatic expression of genes regulating PPi homeostasis. To that aim, we compared...
Uploaded on: March 10, 2024