PubMed Health⌕ Search

Biomedical subjects

Pierre Julia

Publications and source records attributed to Pierre Julia.

8 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↗
PubMed2005

Reduced immunoregulatory CD31+ T cells in patients with atherosclerotic abdominal aortic aneurysm.

BACKGROUND: Cell-mediated immunity is considered to contribute to the pathogenesis of abdominal aortic aneurysms (AAA). In particular, infiltrating macrophages and CD8+ T lymphocytes participate in the destruction of the aortic wall extracellular matrix and smooth muscle cells. We surmise that these pathological events are controlled by circulating regulatory lymphocytes. METHODS AND RESULTS: Circulating CD4+/CD31+ cells were reduced in AAA patients (n=80, 8.9+/-0.6%) as compared with controls (n=69, 13.7+/-0.8%; P<0.001) and inversely proportional to AAA size. Exclusion of the aneurysm by an endoprothesis did not affect CD31+ T cell values. Reduction of blood CD4+/CD31+ cells was not attributable to their enrichment in AAA tissue. In contrast, CD8+/CD31+ cells were slightly reduced in the blood while increased in the aneurysmal tissue (29.2+/-0.5 versus 20.2+/-4.7% in blood, n=6; P<0.05). Remarkably, high percentages of CD4+/CD31+ cells were able to regulate proliferation and cytokine production of CD8+ lymphocytes, as well as CD8+ cell-mediated cytotoxicity of aortic smooth muscle cells (P<0.01). Finally, CD4+/CD31+ cells reduced the production and activity of metalloproteinase-9 by lipopolysaccharide-stimulated macrophages. CONCLUSIONS: Circulating CD4+/CD31+ T cells regulate macrophage and CD8+ T cell activation and effector function in the arterial wall. Their reduction might promote the development of AAA.

Aged↗
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↗
PubMed2005

[Management of a patient with critical limb ischemia].

The severity of the prognostic of critical limb ischemia imposes a multidisciplinary approach, done by a highly specialised centre. A delay in managing this condition increases significantly the risk of amputation. For most patients, a revascularisation procedure, surgical or endovascular, can be considered, since the aim of the treatment is to restore an arterial flux to permit cicatrisation and relieve pain. When the choice is possible, endovascular techniques are preferred, because more simple, done without anaesthesia and charged of less complications rate. The choice between two techniques is never simple and imposes the intervention of a multidisciplinary staff composed by vascular specialist, vascular surgeons and interventional radiologists. The aim of the medical treatment, waiting for the revascularisation, is to control the pain and the infection, prevent progression of the lesions and to optimise respiratory and cardiac functions.

Angiography↗
PubMed2003

Intravenous immunoglobulins and transplantation for patients with anti-HLA antibodies.

Transplantation for patients possessing allo-antibodies against HLA antigens can be delayed for years, and, once the graft has been transplanted, its survival is significantly reduced. We and others have shown that administration of intravenous immunoglobulins (IVIgs) can induce a profound and sustained decrease in the titres of the anti-HLA antibodies, thus greatly enhancing the chances of those patients to obtain a transplant. In a number of cases, pre-treatment sera contained anti-donor antibodies that disappeared after IVIg administration. A similar approach, combining plasmapheresis and low-dose IVIgs, has shown similar results and has been successfully applied to ABO-incompatible transplantations. Patient and graft survival are excellent, despite a rather high rate of rejections, most notably humoral ones. These protocols thus demonstrate that the presence of anti-donor antibody, once an absolute contra-indication to transplantation, can, nowadays, be considered as an immunological hurdle that can be managed through appropriate immunological manipulation.

Graft Rejection↗
PubMed2003

Radial nerve compression by a large cephalic vein aneurysm: case report.

A 43-year-old man had weakness of the extensor muscles in the right forearm and could not extend the right wrist. The apparent disorder was caused by radial nerve compression by a large aneurysm in the cephalic vein in the antecubital fossa. Surgical resection of the aneurysm resolved the symptoms. This exceedingly rare complication of venous aneurysm is discussed. Venous aneurysm should be included in the differential diagnosis of a subcutaneous mass, and diagnosis is best made with Duplex ultrasound scanning. Surgical excision is the appropriate treatment.

Adult↗
PubMed2002

Desensitization and subsequent kidney transplantation of patients using intravenous immunoglobulins (IVIg).

Transplantation of patients possessing antibodies against allo-HLA antigens can be delayed for years. We have shown that administration of intravenous immunoglobulins (IVIg) can induce a profound and sustained decrease in the titers of anti-HLA antibodies. We report here the first series of patients desensitized, then transplanted using IVIg therapy. Fifteen patients have been included and treated with IVIg, given as 3 monthly courses of 2g/kg body weight. Thirteen of those 15 patients (87%) were effectively desensitized and underwent immediate transplantation. Eleven were transplanted with a cadaveric donor, and two with a living donor against which the pretreatment cross-match was positive. One graft was lost from thrombosis and one from rejection. All other patients had uneventful courses, without any episodes of rejection, with a follow-up of more than 1 year. Thus, IVIg therapy allows safe and prompt kidney transplantation of immunized patients.

Adolescent↗