Fibromuscular dysplasia.

Fibromuscular dysplasia (FMD), formerly called fibromuscular fibroplasia, is a group of nonatherosclerotic, noninflammatory arterial diseases that most commonly involve the renal and carotid arteries. The prevalence of symptomatic renal artery FMD is about 4/1000 and the prevalence of cervicocranial FMD is probably half that. Histological classification discriminates three main subtypes, intimal, medial and perimedial, which may be associated in a single patient. Angiographic classification includes the multifocal type, with multiple stenoses and the 'string-of-beads' appearance that is related to medial FMD, and tubular and focal types, which are not clearly related to specific histological lesions. Renovascular hypertension is the most common manifestation of renal artery FMD. Multifocal stenoses with the 'string-of-beads' appearance are observed at angiography in more than 80% of cases, mostly in women aged between 30 and 50 years; they generally involve the middle and distal two-thirds of the main renal artery and in some case also renal artery branches. Cervicocranial FMD can be complicated by dissection with headache, Horner's syndrome or stroke, or can be associated with intracerebral aneurysms with a risk of subarachnoid or intracerebral hemorrhage. The etiology of FMD is unknown, although various hormonal and mechanical factors have been suggested. Subclinical lesions are found at arterial sites distant from the stenotic arteries, and this suggests that FMD is a systemic arterial disease. It appears to be familial in 10% of cases. Noninvasive diagnostic tests include, in increasing order of accuracy, ultrasonography, magnetic resonance angiography and computed tomography angiography. The gold standard for diagnosing FMD is catheter angiography, but this invasive procedure is only used for patients in whom it is clinically pertinent to proceed with revascularization during the same procedure. Differential diagnosis include atherosclerotic stenoses and stenoses associated with vascular Ehlers-Danlos and Williams' syndromes, and type 1 neurofibromatosis. Management of cases with renovascular hypertension includes antihypertensive therapy, percutaneous angioplasty of severe stenoses, and reconstructive surgery in cases with complex FMD that extends to segmental arteries. The therapeutic options for securing ruptured intracerebral aneurysms are microvascular neurosurgical clipping and endovascular coiling. Stenosis progression in renal artery FMD is slow and rarely leads to ischemic renal failure.

[Fibromuscular dysplasia]. / Dysplasie fibromusculaire artérielle.

Fibromuscular dysplasia is an idiopathic, segmental, nonatherosclerotic and noninflammatory disease of the muscle layer of arterial walls that leads to stenosis of small- and medium-sized arteries. Fibromuscular dysplasia preferentially affects young women. Although it can affect every arterial tree, it most often touches the renal and internal carotid arteries. Renal fibromuscular dysplasia can cause hypertension by stenosis of the renal artery, most often seen on angiography as resembling a "pearl necklace". Cerebrovascular fibromuscular dysplasia becomes symptomatic when the arterial stenosis is tight and causes hypoperfusion, embolism, or thrombosis or when arterial dissection or rupture of the associated aneurysm occurs.

Family study of the relationship between height and cardiovascular risk factors in the STANISLAS cohort.

BACKGROUND: Short stature is a risk factor for coronary heart disease and is associated with an adverse cardiovascular profile. Mechanisms responsible for this association remain unknown. A genetic contribution to this association would imply a familial clustering between height and cardiovascular risk factors. METHODS: This study investigated whether lipids and blood pressure (BP) levels shared a common familial component with height. The sample included 865 nuclear families from the French STANISLAS cohort volunteering for a free health examination between 1993 and 1994. Within-individual correlations and familial intra-trait and cross-trait correlations were estimated using the Estimating Equation technique extended to a bivariate phenotype. RESULTS: Height negatively correlated to total and low density lipoprotein cholesterol (LDL-C) and triglycerides in both parents and offspring, and positively correlated to high density lipoprotein cholesterol (HDL-C) in parents only. In offspring, the correlation between height and HDL-C markedly increased with sexual maturation to reach after puberty the same value as in parents. The correlation of height with systolic BP was negative in fathers and positive in sons, whereas it was non-significant in mothers and daughters. The pattern of cross-trait familial correlations between height and LDL-C was compatible with the existence of a weak transmissible component explaining the relationship between these two traits. By contrast, the pattern observed for HDL-C and triglycerides was rather compatible with the influence of shared environmental factors. No familial clustering between height and BP levels was detected. CONCLUSIONS: The association between short stature and increased LDL-C might be partly of familial origin.

Diabetes as a marker of pheochromocytoma in hypertensive patients.

OBJECTIVE: To assess the prevalence of diabetes in patients with pheochromocytoma and the probability of pheochromocytoma occurring in hypertensive patients with or without diabetes. SETTING: A tertiary, referral hypertension department. PATIENTS AND METHODS: We compared age, body mass index and the frequency of diabetes in 191 patients with pheochromocytoma and a random sample of 880 patients with essential hypertension. Diabetes was defined as current antihyperglycemic treatment or two fasting blood glucose concentrations >or= 7 mmol/l. For patients with pheochromocytoma, we also recorded plasma catecholamine concentrations, the urinary excretion of metanephrines, and tumor characteristics. RESULTS: Diabetes was present in 68 (35.6%) patients with pheochromocytoma and 192 (21.8%) patients with essential hypertension (P < 0.001). Pheochromocytoma patients with or without diabetes did not differ in body mass index, plasma noradrenaline concentration, metanephrine excretion or tumor characteristics. Age, duration of hypertension and plasma adrenaline concentration were significantly and independently associated with diabetes in patients with pheochromocytoma. They were younger, more likely to be female and had a lower body mass index than those with essential hypertension (P < 0.01). After adjustment for these three variables, the odds ratio for pheochromocytoma in hypertensive patients with diabetes was 5.5 (95% confidence interval, 3.5-8.7). For patients younger than the age of 51 years with a body mass index < 25 kg/m2, the odds ratio was 18.9 (95% confidence interval, 5.9-58.8). CONCLUSION: Diabetes is present in one in three patients with pheochromocytoma. In young patients with hypertension and normal body weight, the presence of diabetes is a clinical clue to the diagnosis of pheochromocytoma.

Familial aggregation of fetal growth restriction in a French cohort of 7,822 term births between 1971 and 1985.

An association between fetal growth restriction and increased rates of metabolic and cardiovascular diseases in adulthood has been reported. This study evaluated familial aggregation of fetal growth restriction in term births. The population consisted of 3,505 sibships comprised of 7,822 full-term singleton infants born between 1971 and 1985 in Haguenau, France, and selected from a regional register of births. Sib-sib odds ratios were estimated for being born small for gestational age (SGA), defined as having a birth weight below the 10th percentile of the sex-specific curve of birth weight by week of gestation. SGA births were further stratified according to ponderal index (birth weight/length(3)). After adjustment for maternal factors, the sib-sib odds ratios were 4.8 (95% confidence interval (CI): 3.7, 6.3) for all SGA births, 7.7 (95% CI: 4.1, 14.7) for SGA births with a low ponderal index (<10th percentile), and 4.4 (95% CI: 2.3, 8.2) for SGA births with a normal ponderal index (25th-75th percentile). None of the maternal factors investigated significantly influenced the magnitude of these odds ratios. This strong residual sib-sib aggregation suggests a role for genetic and/or shared environmental factors in the etiology of fetal growth restriction, especially when associated with a low ponderal index.