Publications by authors named "Pannier-Moreau I"

Fibromuscular dysplasia is a non-atherosclerotic, non-inflammatory vascular disease that primary involves medium-sized and small arteries, most commonly the renal and carotid arteries. Dysplasic stenoses can be classified by angiography into three main subtypes, multifocal (multiple contiguous stenoses with the "string of beads" appearance), unifocal (single stenosis in a given renal artery), or tubular. The multifocal subtype is the most frequent and is usually associated with medial dysplasia, whereas unifocal and tubular stenoses are associated with intimal and perimedial dysplasia, respectively.

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The authors report an original case of the association of three pathologies: pheochromocytoma, hyperthyroidism and cardiomyopathy with left ventricular outflow tract obstruction. This type of cardiac disease has occasionally been described in cases of pheochromocytoma and are usually induced by the endocrine disturbance because they regress with treatment of the pheochromocytoma. The associated hyperthyroidism observed in this case is very rare and may have increased the left ventricular pressure gradient.

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The aim of this study was to conduct a formal pedigree analysis of the involvement of the elastin gene in families. From 140 subjects with renal FMD documented on angiography, family cases with documented renal artery fibromuscular dysplasia (FMD) and to test pedigrees were constructed and familial cases defined by angiographic evidence of FMD in at least one sibling. Familial screening was made either by echodoppler for asymptomatic subjects or by digital intravenous angiography for hypertensive subjects.

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Objective: To describe phenotypes and estimate the prevalence of familial cases of renal artery fibromuscular dysplasia (FMD).

Patients And Setting: One hundred and four unrelated hypertensive patients (94 women) with renal artery fibromuscular dysplasia documented on angiography and classified as having multifocal or unifocal lesions. Familial cases were defined as those with angiographic evidence of renal artery FMD in at least one sibling.

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Management of patients with renal artery stenoses is aimed at normalizing or reducing blood pressure and correcting or preventing reduced glomerular filtration. The results of renal revascularization have been documented mainly in retrospective, uncontrolled reports in which blood pressure improvement was overestimated due to the placebo effect and optimization of drug treatment, the latter being frequently required despite adequate revascularization. In an overview of 10 series reporting blood pressure outcome following percutaneous angioplasty, cure rates were 50% in patients with fibrodysplastic stenosis but only 19% in those with atherosclerotic stenosis.

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