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Biomedical subjects

Jean-Noël Fiessinger

Publications and source records attributed to Jean-Noël Fiessinger.

16 recordsLinked to original sources
PubMed2006

[Vascular Ehlers-Danlos syndrome].

The vascular type of Ehlers-Danlos syndrome (EDS) is a rare genetic disease transmitted as an autosomal dominant trait. It is distinguished from other forms of EDS by its unstable acrogeric morphotype and by vascular, gastrointestinal, and obstetrical complications. Diagnosis is based on various clinical signs, noninvasive imaging, and on the identification of a mutation of the COL3A1 gene, which provides diagnostic certainty but has a sensitivity of only 61%. When two major diagnostic criteria are present, a genetic test should be proposed, performed and its result presented in a multidisciplinary group. The precautionary principle requires that preventive measures be implemented when the diagnosis is suspected. All artery puncture, surgery, and gastrointestinal and uterine endoscopy are contraindicated, permissible only in life-threatening emergencies. Straining against a closed glottis and all other situations or drugs likely to raise blood pressure must be avoided. Contraception must be discussed to avoid pregnancy during the diagnostic period. Arterial lesions suggestive of the disease include dissecting aneurysms of the internal carotid and iliac arteries and of the anterior visceral branches of the abdominal aorta, fusiform aneurysms of the splenic artery, and early onset nontraumatic direct carotid-cavernous fistulae. Early-onset varicose veins, spontaneous peritonitis or unusually important perineal lesions after giving birth should also attract the physician's attention. Psychological treatment and support of patients and their families is essential, to help them both to live with their disease and to deal with the information and screening issues. The prognosis of Ehlers-Danlos syndrome, vascular type, is grim but there is wide interindividual variability and life expectancy is best among patients receiving regular follow-up. Management by an experienced multidisciplinary team, implementation of drastic prevention measures and, depending on the results of the BBEST study, the possible prescription of beta-blockers should help to reduce the risk of complications and justify hope for a real improvement in prognosis in the near future.

Adult↗
PubMed2006

[Medical treatment of critical leg ischemia: current status and future perspectives of gene and cell therapy].

Critical limb ischemia (CLI) is associated with a high risk of amputation and death. For patients who cannot be surgically revascularized, medical options include prostanoids, spinal cord stimulation and lumbar sympathectomy, but none of these treatments has a demonstrated impact on the amputation rate at six months. Gene and cell therapy, aimed at stimulating angiogenesis, have mainly been tested in phase I and II clinical trials. These approaches appear to be feasible and safe in the short-term, but large randomized studies are necessary to demonstrate their clinical benefits and long-term safety.

Amputation, Surgical↗
PubMed2005

[Management of patients with intermittent claudication].

Management of patients with intermittent claudication must be first the treatment of the atherosclerosis process: cessation of smoking, antiplatelet therapy, betablockers, angiotensin-converting-enzyme inhibitor and cholesterol lowering in order to have a LDL cholesterol < 1 g/L. Treatment of intermittent claudication depends of the arterial lesions. If an endovascular procedure is feasible and safe, iliac stenosis or short iliac occlusion, it permits a spectacular improvement. Most often only medical treatment with physical training is advisable. Surgical revascularisation is rarely justified in patients with intermittent claudication.

Adrenergic beta-Antagonists↗
PubMed2004

Increased carotid wall stress in vascular Ehlers-Danlos syndrome.

BACKGROUND: Vascular Ehlers-Danlos syndrome (vEDS), also known as EDS type IV, an inherited disorder of connective tissue, results from mutations in the gene encoding type III procollagen (COL3A1). Affected patients are at risk for arterial dissection or rupture, the main cause of death. To understand the pathogenesis of the vascular lesions, we used a biomechanical approach and determined steady and pulsatile wall stress. METHODS AND RESULTS: Sixteen patients with vEDS and 16 age-, gender-, and blood pressure-matched control subjects were included in this cross-sectional noninvasive study. Circumferential wall stress was determined under steady and pulsatile conditions at the site of an elastic (common carotid) and a muscular (radial) artery from the measurements of intima-media thickness and internal diameter with high-resolution echo-tracking systems and either mean blood pressure or pulse pressure, respectively. At the site of the carotid artery, steady circumferential wall stress was 43% higher in vEDS patients than in control subjects (68.9+/-14.3 versus 48.2+/-12.1 kPa, P<0.001), and pulsatile circumferential wall stress was 22% higher (28.2+/-7.7 versus 23.1+/-5.7 kPa, P<0.001). Carotid intima-media thickness was 32% lower (408+/-56 versus 598+/-171 microm, P<0.001) in vEDS patients, and internal diameter was not different between groups. Radial artery parameters were not significantly different between groups. CONCLUSIONS: In vEDS patients, an abnormally low intima-media thickness generates a higher wall stress than in control subjects at the site of an elastic artery, which may increase the risk of arterial dissection and rupture.

Adolescent↗
PubMed2003

P2Y12 H2 haplotype is associated with peripheral arterial disease: a case-control study.

BACKGROUND: We recently described a gain-of-function haplotype, called H2, of the adenosine diphosphate (ADP) receptor P2Y12 gene associated with increased ADP-induced platelet aggregation ex vivo in healthy volunteers. Because platelets play a key role in atherosclerosis and arterial thrombosis, we tested the possible link between the H2 haplotype and the risk of peripheral arterial disease (PAD) in a case-control study. METHODS AND RESULTS: We studied 184 consecutive male patients under 70 years of age with PAD and 330 age-matched control subjects free of symptomatic PAD and with no cardiovascular history. Mean age was 57.1+/-7.2 years (cases) and 56.7+/-7.6 years (control subjects). The H2 haplotype was more frequent in patients with PAD than in control subjects (30% and 21%, respectively; OR, 1.6; CI, 1.1 to 2.5; P=0.02 in univariate analysis). This association with PAD remained significant in multivariate regression analysis (OR, 2.3; CI, 1.4 to 3.9; P=0.002) after adjustment for diabetes, smoking, hypertension, hypercholesterolemia, and other selected platelet receptor gene polymorphisms. CONCLUSIONS: These data point to a role of the H2 haplotype in atherosclerosis and raise the possibility of relative thienopyridine resistance in carriers of the P2Y12 H2 haplotype.

Aged↗
PubMed2003

Cleaved protein S (PS), total PS, free PS, and activated protein C cofactor activity as risk factors for venous thromboembolism.

BACKGROUND: Although hereditary protein S (PS) deficiency is clearly associated with venous thromboembolism (VTE), the importance of low PS concentrations as a risk factor for VTE in other patients is still a matter of debate. To clarify this issue, we designed a case-control study to evaluate the role of different molecular forms of plasma PS. METHODS: We quantified plasma cleaved, total, and free PS and activated protein C (APC) cofactor activity in 87 VTE patients and 174 controls matched for age, sex, and hormonal treatment. Free PS was measured by ELISA or by enzyme-linked ligand sorbent assay (ELSA). Cleaved and total PS were measured by ELISA. RESULTS: In controls, the mean (SD) concentration of circulating cleaved PS was 39 (14) nmol/L, corresponding to 10% (3.5%) of total PS. Concentrations of cleaved PS and total PS were not significantly different in patients with VTE compared with controls. However, in our population, low free PS measured by ELISA or ELSA, as well as APC cofactor activity values were significantly associated with VTE with odds ratios (95% confidence intervals) of 2.9 (1.3-6.3), 2.5 (1.1-5.6), and 2.9 (1.3-6.4), respectively, in multivariate analyses. CONCLUSION: Phenotypic low PS detected by APC cofactor activity assay or by an assay specific for free PS should be considered a risk factor for VTE.

Adult↗
PubMed2003

Association of Takayasu's arteritis and Crohn's disease. Results of a study on 44 Takayasu patients and review of the literature.

Takayasu's arteritis and Crohn's colitis are exceptionally associated and characteristics of patients affected with both diseases have not been fully described. In a group of 44 consecutive Takayasu patients, 4 also had Crohn's disease. This 9% prevalence (95% confidence interval=2.5-21.7%) of Crohn's disease in the group is significantly greater than the highest reported prevalence of the disease in the general population. In these 4 patients, fulfilling 5 or more ACR criteria for Takayasu's disease, Crohn's disease was confirmed with colonoscopic examination and biopsies. Takayasu-Crohn patients were younger at diagnosis and tended to have systemic symptoms more frequently. Other clinical, radiological and biological characteristics were not different between the 2 groups. In the literature, 16 cases of this unusual association have been reported. The diagnosis of Takayasu's disease was simultaneous or posterior to Crohn's disease in 87%. Data on vascular lesions was available in 6 out of 16 patients and in the 4 patients of the present study: the distribution of vascular lesions in Takayasu-Crohn patients did not appear to be different from Takayasu patients. Our results and previous reports suggest that this association of rare diseases is not fortuitous. With respect to the indolent course of Takayasu's arteritis, early diagnosis of vascular lesions in Crohn patients is encouraged.

Adolescent↗
PubMed2003

Polyarteritis nodosa presenting as acute leg ischemia.

We describe a 33-year-old hypertensive woman with otherwise apparently good health, in whom acute leg ischemia by acute occlusion of the 3 infrapopliteal arteries led to the diagnosis of polyarteritis nodosa (PAN). Angiography and ultrasound imaging showed multiple microaneurysmal and thrombotic lesions, notably in the kidneys and the 4 extremities. Cutaneous biopsy showed necrotizing angiitis. She was treated with corticosteroids. Ischemia improved progressively, with normal walking after several months. Three points were remarkable in this case of PAN: (1) its unusual presentation; (2) the absence of constitutional symptoms despite the diffuse distribution of vascular lesions; (3) the good visualization of peripheral microaneurysms by ultrasonography (US), suggesting a potential diagnostic interest for US examination of the limbs in PAN.

Acute Disease↗
PubMed2002

Extended venous thromboembolism prophylaxis after total hip replacement: a comparison of low-molecular-weight heparin with oral anticoagulant.

BACKGROUND: Oral anticoagulants and low-molecular-weight heparin are both recommended for venous thromboembolism prophylaxis after total hip replacement. To date, these regimens have not been compared by means of clinical end points in the extended prophylaxis setting. METHODS: We randomly assigned 1279 patients 3 days after total hip replacement surgery to fixed-dose subcutaneous low-molecular-weight heparin (reviparin sodium, 4200 anti-Xa IU) or adjusted-dose oral anticoagulant (international normalized ratio, 2-3; acenocoumarol) for a 6-week period. The primary end point was the failure rate, defined as the combined clinical events of a confirmed symptomatic thromboembolic event, a major hemorrhage, or death. All patients were followed up throughout the study interval. The primary objective was to compare the observed cumulative failure rate in the low-molecular-weight heparin vs oral anticoagulant group. RESULTS: In the intent-to-treat population, objectively documented symptomatic thromboembolic events occurred in 15 (2.3%) of 643 patients vs 21 (3.3%) of 636 patients receiving low-molecular-weight heparin or oral anticoagulants, respectively (P =.30; 95% confidence interval for the difference, -0.8% to 2.8%). Major bleeding occurred in 9 (1.4%) of 643 patients vs 35 (5.5%) of 636 patients receiving low-molecular-weight heparin or oral anticoagulants, respectively (P =.001). The failure rate was 24 (3.7%) of 643 patients compared with 53 (8.3%) of 636 patients who received low-molecular-weight heparin or oral anticoagulants (P =.001). CONCLUSIONS: A significantly higher benefit-risk ratio was observed for patients undergoing elective hip replacement who received extended out-of-hospital prophylaxis with low-molecular-weight heparin vs acenocoumarol. Low-molecular-weight heparin prophylaxis was at least as effective as oral anticoagulants, but with a marked improvement in safety.

Acenocoumarol↗
PubMed2002

[Inflammatory and infectious aortitis].

Takayasu's disease is the most characteristic example of aortitis. Non-specific aortoarteritis is the only one with stenotic lesions of the aorta. Ischemic symptoms depend of the arterial lesions. Diagnosis is funded on topographic of arterial involvement. CT scan and ultrasound are the method of choice for the diagnosis and follow-up of patients. Compared to controls, patients with giant cell arteritis are 17 times more likely to develop thoracic aortic aneurysm. The risk of this location could justify a systematic research of an aortic involvement during the follow up of patients with temporal arteritis. Saccular aneurysms are an unusual manifestation of Behçet's disease but as in Horton's disease the poor prognosis impose a systematic evaluation. Syphilitic aortic is actually very rare, staphylococcus and salmonella are the main causes of mycotic aneurysms.

Aortic Aneurysm↗