Vascular calcification is a complex and dynamic process occurring in various physiological conditions such as aging and exercise or in acquired metabolic disorders like diabetes or chronic renal insufficiency. Arterial calcifications are also observed in several genetic diseases revealing the important role of unbalanced or defective anti- or pro-calcifying factors. Pseudoxanthoma elasticum (PXE) is an inherited disease (OMIM 264800) characterized by elastic fiber fragmentation and calcification in various soft conjunctive tissues including the skin, eyes, and arterial media. The PXE disease results from mutations in the ABCC6 gene, encoding an ATP-binding cassette transporter primarily expressed in the liver, kidneys suggesting that it is a prototypic metabolic soft-tissue calcifying disease of genetic origin. The clinical expression of the PXE arterial disease is characterized by an increased risk for coronary (myocardial infarction), cerebral (aneurysm and stroke), and lower limb peripheral artery disease. However, the structural and functional changes in the arterial wall induced by PXE are still unexplained. The use of a recombinant mouse model inactivated for the Abcc6 gene is an important tool for the understanding of the PXE pathophysiology although the vascular impact in this model remains limited to date. Overlapping of the PXE phenotype with other inherited calcifying diseases could bring important informations to our comprehension of the PXE disease.

These results suggest that cortical parameters are more sensitive for early diagnosis of ARD than kidney size. Cortical atrophy should be a useful marker for guidance for revascularization.

Peripartum cardiomyopathy (PPCM) is a rare cause of heart failure. Only half of the patients recover normal cardiac function. We assessed the usefulness of magnetic resonance imaging (MRI) and late enhancement imaging to detect myocardial fibrosis in order to predict cardiac function recovery in patients with peripartum cardiomyopathy. Among a consecutive series of 1,037 patients referred for heart failure treatment or prognostic evaluation between 1999 and 2006, eight women had confirmed PPCM. They all underwent echocardiography and cardiac MRI for assessment of left ventricular anatomy, systolic function and detection of myocardial fibrosis through late enhancement imaging. Mean (+/- SD) baseline left ventricular ejection fraction (LVEF) was 28 +/- 4%. After a follow-up of 50 +/- 9 months, half the patients recovered normal cardiac function (LVEF = 58 +/- 4%) and four did not (LVEF = 35 +/- 6%). None of the eight patients exhibited abnormal myocardial late enhancement. No difference in MRI characteristics was observed between the two groups. Patients with PPCM do not exhibit a specific cardiac MRI pattern and particularly no myocardial late enhancement. It suggests that myocardial fibrosis does not play a major role in the limitation of cardiac function recovery after PPCM.

Knowledge of precise cartography of PSCV is essential to therapeutic decisions. MDCT is the best way to understand and describe the different types of PSCV.

The aim of this study was to provide quantitative data on the origin and trajectory of the main renal arteries using spiral CT angiography and arteriography. Normal renal artery anatomy was assessed on spiral CT angiography (axial transverse sections and shaded-surface-display reconstructions) in 100 patients referred for renal arteriography who had no significant renal artery stenosis. Two hundred major renal arteries were studied. The vast majority of right (88 %) and left (87 %) renal arteries originated between the lower third of the first lumbar vertebra and the lower border of the second lumbar vertebra. In 50 patients both ostia were at the same level; in the remaining 50 patients, the right ostium was located above the left in 37 patients. On the right, the angle of origin varied from -10 to + 55 degrees (mean + 24 degrees ). On the left, the angle of origin varied from + 30 to -55 degrees (mean -11 degrees ). Spiral CT angiography provides additional anatomic data, notably regarding the angle of origin of the renal arteries, that is potentially useful for planning interventional procedures.