Arterial Wall Calcium Load in Pseudoxanthoma Elasticum

Study Overview
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Brief Summary
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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.

Detailed Description
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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.

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

Conditions
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Pseudoxanthoma Elasticum, Type 2 Diabetes, Chronic Kidney Disease

Participation Criteria
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Eligibility Criteria
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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

Ages Eligible for Study
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Minimum Age: 18 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Pseudoxanthoma Elasticum<br>Patients with genetically and clincally proven PXE | |
| chronic kidney disease<br>Type 2 diabetic patients with mediacalcosis and matched to PXE patients for gender and age (+/- 5 yrs). | |
| Diabetes<br>patients with chronic kidney disease and matched to PXE patients for gender and age (+/- 5 yrs). | |

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| calcification score | the level of arterial wall calcification determined by a Ct-scan and computed by the Agatston score | after inclusion | 

Secondary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| site of calcification | the anatomical site of arterial wall calcification determined by a Ct-scan and computed by the Agatston score | after inclusion |