Arterial Wall Calcium Load in Pseudoxanthoma Elasticum

Arterial Wall Calcium Load and Preferential Sites in Pseudoxanthoma Elasticum Versus Type 2 Diabetes and Chronic Kidney Disease.

Quantification and preferential sites of arterial wall calcification within the coronary and lower legs arteries will be comared between Pseudo-Xanthoma elasticum(PXE) atients and type 2 diabetics and Chronic Kidney disease.

In a previous work we have observed that patients suffering from PXE, a soft-tissue calcifying inherited autosomal disease, exhibited specific structural and functionnal features within the peripheral arteries. We now want to quantify and determine the preferential sites of the arterial wall calcification (Agatston score) between these patients compared to diabetics and patients with chronic renal insufficiency with mediacalcosis.

All variables will be collected from a cohort of PXE monitored in our PXE Health and Care centre at the university hospital of Angers. Quantification of the arterial wall calcium load will be performed using helicoidal CT-scan. These variables will be compared to the same measurements performed in type 2 diabetic and chronic kidney disease patients paired by age and gender.

Inclusion Criteria:

• Patients with phenotypically and genotypically-proven PXE
• Type 2 diabetic patients with mediacalcosis (i.e. Ankle Brachial Index > 1.30)
• Patients with a chronic kidney disease (CKD), before kidney transplantation
• All patients should be affiliated to a Health care system

Exclusion Criteria:

• Patient with unproved PXE
• Patient under the age of 18 yrs
• Patient unwilling to participate to the study
• Pregnant women
• Patient not affiliated to a Health care system