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Biomedical subjects

J Ninet

Publications and source records attributed to J Ninet.

At least 55 records · Page 3Linked to original sources

Anticardiolipin antibodies and giant cell arteritis: a prospective, multicenter case-control study. Groupe de Recherche sur l'Artérite à Cellules Géantes.

OBJECTIVE: To assess the prevalence and thrombogenic role of anticardiolipin antibodies (aCL) in giant cell arteritis. METHODS: Two hundred eighty-four patients with newly diagnosed temporal arteritis or polymyalgia rheumatica and 210 age- and sex-matched controls randomly selected from the general population were included in a multicenter, prospective case-control study. Blood samples were obtained at the time of diagnosis, data on initial clinical features were collected in a questionnaire, and temporal artery biopsy findings were reviewed by an experienced pathologist. RESULTS: Anticardiolipin antibodies were present in 20.7% of patients compared with 2.9% of controls (P = 1.45 x 10(-9)). The prevalence of aCL was higher in samples found positive for temporal arteritis than in those found negative on biopsy (31.2% versus 16.7%; P = 0.04), and was similarly higher in the biopsy-positive temporal arteritis group compared with the polymyalgia rheumatica and control groups. Although aCL were associated with thrombotic complications in univariate analysis, the positivity of the biopsy findings remained the only predictive variable in stratified analysis. CONCLUSION: In giant cell arteritis, aCL seem to function as reactive antibodies in relation to endothelial lesions.

Aged↗

Giant cell arteritis and cardiovascular risk factors: a multicenter, prospective case-control study. Groupe de Recherche sur l'Artérite à Cellules Géantes.

OBJECTIVE: To assess the role of cardiovascular risk factors, measured at the time of diagnosis, in the pathogenesis of giant cell (temporal) arteritis (GCA). METHODS: Four hundred new patients with GCA or polymyalgia rheumatica (PMR) and population-based, individually age- and sex-matched controls were included in this multicenter, prospective case-control study. Each participant was evaluated by review of the medical history and by clinical and laboratory assessments. RESULTS: Among women, smoking was associated with a 6-fold increase in risk (P = 0.00006, 95% confidence interval [95% CI] 2-17), heavy smoking with a 17-fold increase in risk, and previous atheromatous disease with a 4.5-fold increase in risk (P = 0.0003, 95% CI 2-11) of GCA in both the biopsy-positive and biopsy-negative GCA groups; only smoking appeared to be a risk factor for PMR in women (odds ratio 3.64, 95% CI 1.07-12.40). Among men, no risk factor was found to be significant. CONCLUSION: Smoking and previous arterial disease were independently associated with GCA in women. In order to avoid matching bias, risk factors for diseases with an unbalanced sex distribution should be studied separately in each sex, using a sex-matched, case-control study design.

Aged↗

Acquired hemophilia due to factor VIII inhibitors in 34 patients.

BACKGROUND: Acquired hemophilia is a rare disease caused by the development of auto-antibodies against factor VIII. SUBJECTS AND METHODS: We studied the characteristics and outcomes of 34 patients (19 women and 15 men) with acquired hemophilia from 1980 to 1997. RESULTS: The mean age of the patients was 61 years (range, 22-93 years). An underlying disease was observed in 18 (53%) patients: 5 patients had cancer, 4 an autoimmune disorder, 2 a dermatologic disorder, 3 asthma, 3 were postpartum, and 1 had an adverse reaction to ampicillin. Factor VIII level was <5% in 30 (90%) patients; factor VIII antibodies were elevated (>10 Bethesda units) in 23 (69%) patients. Bleeding requiring transfusions was reported in 25 (75%) patients. Human factor VIII was given to 14 patients and porcine factor VIII to 5. Six patients received prothrombin complex concentrates and one desmopressin. Several immunosuppressive treatments were used, mainly corticosteroids, cyclophosphamide, and intravenous immunoglobulin. Bleeding stopped in all but one patient within 2 weeks. Most patients achieved complete remission, although two relapses were observed subsequently. CONCLUSION: This large study helps to clarify the presentation and clinical course of acquired hemophilia. Prospective studies are needed to determine the efficacy of treatment.

Adult↗

Long-term clinical and hemodynamic evaluation of porcine valved conduits implanted from the right ventricle to the pulmonary artery.

OBJECTIVE: This retrospective study was initiated to evaluate the long-term results of valved prosthetic conduits implanted in the right ventricular outflow tract in patients with complex ventricular-pulmonary discontinuity. METHODS: A cohort of 103 patients out of 127 (24 early deaths, 19%) operated on between 1973 and 1996 with porcine valved conduits was available for evaluation, with a follow-up ranging from 1 to 21.6 years (mean follow-up 8.4 +/- 6 years). A total of 74 hemodynamic studies were performed after the operation, 50 patients having undergone at least 1 cardiac catheterization during the follow-up period. RESULTS: There were 16 late deaths, and the actuarial survivals, including early mortality, were 72.9% +/- 4% at 5 years, 63.1% +/- 5% at 10 years, and 58.2% +/- 5% at 15 years, at which time 20 patients were still available for review and exposed to the risk of dying. The mean peak systolic gradient across the right ventricular outflow tract was plotted as a function of time, showing a gradual increase and a significant step-up after the eighth year, from 43 +/- 36 to 69 +/- 19 mm Hg (P < .005). Reoperation was required for progressive conduit obstruction between 1.1 and 17.7 years after implantation (mean 7.4 +/- 4.8 years) in 25 patients (24%, 70% CL 15%-33%), with generally very few symptoms, or for residual ventricular septal defect in 3 patients. Freedom from reoperation was 79.5% +/- 5% at 10 years and 65.8% +/- 7% at 15 years. CONCLUSIONS: Porcine conduits may represent a valuable alternative to biologic substitutes with similar long-term results. Given the few symptoms, progressive conduit stenosis after the eighth postoperative year imposes a yearly noninvasive patient evaluation during the follow-up.

Actuarial Analysis↗

Effects of various flow types on maternal hemodynamics during fetal bypass: is there nitric oxide release during pulsatile perfusion?

OBJECTIVE: This study investigates the role of various flow conditions on maternal hemodynamics during fetal cardiopulmonary bypass. METHODS: Normothermic fetal bypass was conducted under pulsatile, or steady flow, for a 60-minute period. Fetal lamb preparations were randomly assigned to 1 of the 3 groups: steady flow (n=7), pulsatile flow (n=7), or pulsatile blocked flow bypass (n=7), where fetuses were perfused with Nomega-nitro-L-arginine after the first 30 minutes of pulsatile flow to assess the potential role of endothelial autacoids. RESULTS: Maternal oximetry and pressures remained unchanged throughout the procedure. Under fetal pulsatile flow, maternal cardiac output increased after 20 minutes of bypass and remained significantly higher than under steady flow at minute 30 (8.8+/-0.7 L x min(-1) vs 5.9+/-0.5 L x min(-1), P=.02). Maternal cardiac output in the pulsatile group also remained higher than in both steady and pulsatile blocked flow groups, reaching respectively 8.7+/-0.9 L x min(-1) vs 5.8+/-0.4 L x min(-1) (P=.02) and 5.9+/-0.3 L min(-1) (P=.01) at minute 60. Maternal systemic vascular resistances were significantly lower under pulsatile than under steady flow after 30 minutes and until the end of bypass (respectively, 9.1+/-0.6 IU vs 12.7+/-1.1 IU, P=.02 and 8.9+/-0.5 IU vs 12.9+/-1.2 IU, P=.01). Infusion of Nomega-nitro-L-arginine was followed by an increase in systemic vascular resistances from 9.3+/-0.7 IU, similar to that of the pulsatile group, to 13.5+/-1 IU at 60 minutes, similar to that of the steady flow group. CONCLUSIONS: Maternal hemodynamic changes observed under fetal pulsatile flow are counteracted after infusion of Nomega-nitro-L-arginine, suggesting nitric oxide release from the fetoplacental unit under pulsatile fetal flow conditions.

Animals↗

Mechanical versus biological isolated aortic valvular replacement after the age of 70: equivalent long-term results.

OBJECTIVE: In order to evaluate the long-term outcome of valvular substitutes in the elderly, we retrospectively analyzed two comparative groups of patients consecutively operated on by the same team for an isolated valvular aortic replacement using either a mechanical or a pericardial prosthesis. METHODS: From 1982 to 1996, 206 patients over 70 years (mean 76.5+/-4.4) underwent an isolated aortic valvular replacement using either a St. Jude Medical (Group I, n = 93) or a Mitroflow (Group II, n= 113) prosthesis depending on the surgeon's preference at the time of surgery. Both groups matched for the following pre-operative variables: sex ratio, type of aortic valve disease, NYHA status, cardiac rhythm, mean pulmonary arterial pressure, left ventricular end-diastolic pressure, LV-AO gradient, cardiac index and ejection fraction. RESULTS: Given an early mortality rate of 6.4% in Group I and 4.4% in Group II (NS), follow-up (mean 4.4+/-3.7 years in Group I and 5.3+/-3.1 years in Group II) was 100% complete. Actuarial survival was 69.9+/-6 and 70.2+/-4.6% at 5 years for Group I and Group II, respectively, and 49.6+/-7.7 vs. 51.4+/-6.3% at 10 years (NS). Freedom from valve-related death was 86.5+/-4.8% in Group I vs. 82.7+/-4% in Group II at 5 years (NS) and 66.7+/-8.7 vs. 66.3+/-7% at 10 years (NS). There were no anticoagulant-related deaths or severe accidents in Group 1. A secondary valvular replacement was necessary in 4 patients in Group II vs. none in Group I. CONCLUSION: The study shows a similar late survival in both groups, with a strikingly low incidence of anticoagulant-related deaths in this population. Given a higher rate of reoperation after biological valve replacement, the use of mechanical valve in this aging population seems to be a valid option.

Aged↗

Disseminated aseptic abscesses associated with Crohn's disease: a new entity?

Our purpose is to describe seven cases of disseminated aseptic abscesses with regard to clinical, biological, radiological, and histological information, treatment, and outcome. Data were collected on seven Caucasian patients who had proven sterile deep abscesses diagnosed in French university hospitals. The onset of the disease related to abscesses began at times from June 1988 to August 1994. Follow-up periods were 1 year, 7 months to 8 years, 2 months. The age of the patients ranged from 15 to 26 years old. At onset, all had fever and six had abdominal pain. Abscesses involved spleen and abdominal lymph nodes in six cases; liver in three; pancreas, brain, and chest in one. All had polymorphonuclear leukocytosis. Pathological examination showed granulomatous abscesses. Direct and indirect investigations failed to identify any causal microorganism. On six occasions, Crohn's disease was revealed 1 to 41 months later and in one case, it preceded the onset of abscesses. One subsequently developed Sweet's syndrome. Various antibiotic regimes were inefficient. Steroids, associated in three cases with immunosuppressive agents, resulted in a rapid improvement in six patients. In one case, splenectomy followed by 5-ASA therapy was used successfully. The dramatic effectiveness of steroids and immunosuppressive agents as well as follow-up suggest that disseminated aseptic abscesses might be an extraintestinal manifestation of Crohn's disease. Although the pathogenesis of this condition remains unknown, this entity may be related to neutrophilic dermatosis in which sterile deep abscesses have been reported.

Abdominal Abscess↗

[Horton's disease, polymyalgia rheumatica, and cardiovascular risk factors. A case-control, prospective multicenter study].

OBJECTIVES: To assess the potential pathogenetic role of cardiovascular risk factors measured at diagnosis in temporal arteritis. METHODS: Four hundred newly diagnosed patients and their age- and sex-matched controls randomly selected from the general population were included in a prospective multicentric cas-control study. RESULTS: In female patients, smoking increased the disease risk by a factor of 6 (95% CI: 2-17, p = 0.00006), and by a factor of 17 in case of > 10 pack-years. Pre-existing peripheral arterial disease increased the risk by a 4.5-fold factor (95% CI: 2-11, p = 0.0003), both in patients with a positive and those with a negative biopsy. Smoking alone appeared as a risk factor for polymyalgia rheumatica (OR: 3,64, 95% CI: 1.07-12.40). In male patients, none of the cardiovascular risk factors studied was positivity associated with the disease. CONCLUSION: Smoking and a pre-existing peripheral arterial disease are independently associated with temporal arteritis in women. Risk factors for disease with an imbalanced sex ratio should be studied separately for each sex in matched case-control studies in order to avoid matching bias.

Aged↗

Giant lambl excrescences. An unusual source of cerebral embolism.

BACKGROUND: A possible association of giant Lambl excrescences (LEs) with stroke has been suggested. However, the treatment of giant LEs is controversial because minimal data are available. OBJECTIVE: To clarify the management of giant LEs through a clinicopathologic study. CASE SERIES: Three young patients (2 women and 1 man) who experienced ischemic stroke were studied. Results of general examinations were normal, as were chest x-ray films, electrocardiograms, ultrasonograms of the neck, and cerebral angiograms. Extensive serological and blood testing failed to show any coagulopathies or systemic disorders that favored a stroke in these patients. Transesophageal echocardiography showed a mitral valve lesion (width, > 1 mm). Two patients (cases 1 and 3) were discharged on a regimen of anticoagulant therapy and sequential transesophageal echocardiographic monitoring was planned, whereas 1 patient (case 2) was promptly scheduled for surgery. A second stroke occurred in patients 1 and 3 at 3 and 6 months, respectively, thus leading to surgery in these 2 patients. Findings from histopathologic studies were consistent with the diagnosis of giant LEs. The patients' outcomes were uneventful after surgery, and none had a recurrence of a stroke. CONCLUSIONS: A relationship between giant LEs and stroke may be suggested. In patients who have transesophageal echocardiographic findings that are consistent with this diagnosis and recurrent stroke despite antithrombotic therapy and without an alternative explanation for the ischemic symptoms, surgery should be considered in view of these findings.

Adult↗

Systemic capillary leak syndrome: report on 13 patients with special focus on course and treatment.

BACKGROUND: Systemic capillary leak syndrome (SCLS) is a rare condition characterized by unexplained episodic capillary hyperpermeability due to a shift of fluid and protein from the intravascular to the interstitial space. This results in diffuse swelling, weight gain, and renal shut-down. From the first publication in 1960, only 34 cases have been reported. OBJECTIVE: To collate enough patients to observe the natural history of the disease and evaluate the efficacy of empiric treatments. DESIGN: Multicentric retrospective study. RESULTS: Thirteen patients (6 women and 7 men) were collated with a mean follow-up of 6.4 years. Eight patients are still alive after a mean of 5.6 years (range 1 to 15). Three patients out of the 11 who were not lost to follow-up died; 1 during an attack and 2 because of a progression towards multiple myeloma. CONCLUSIONS: Our series shows an improvement in the prognosis of SCLS due most likely to improved management during attacks. Some patients' disease could evolve into a multiple myeloma. Treatment is still empiric and no prophylactic therapy, including terbutaline associated with aminophylline, has clearly proven its efficacy.

Acute Disease↗

Flow-induced release of endothelium-derived relaxing factor during pulsatile bypass: experimental study in the fetal lamb.

OBJECTIVE: This study was initiated to test the hypothesis that fetal hemodynamic changes observed under pulsatile flow bypass might be related to the release of endothelium-derived relaxing factor through oscillating shear stress. METHODS: Normothermic bypass was instituted in utero in 21 preterm fetal lambs for a 1-hour period through the right atrium and main pulmonary artery. Ultrasonic flowmeters were positioned around the descending aorta and the umbilical artery. The circuit consisted of an oxygenator and a pump set to either continuous flow (n = 7) or pulsatile flow (n = 7) and adjusted to maintain a fetal main arterial pressure of 50 mm Hg. In seven other animals, endothelium-derived relaxing factor was blocked by a continuous infusion of N omega-nitro-L-arginine after 30 minutes of pulsatile flow. RESULTS: During the first 30 minutes of bypass, pump flows were significantly lower in the continuous-flow group than in the pulsatile-flow or blocked-flow groups (respectively, 612 +/- 144, 907 +/- 153 and 987 +/- 228 ml/min), with similar changes in aortic and umbilical flows. Systemic vascular resistances were significantly lower in the pulsatile-flow and blocked-flow groups than in the continuous-flow group (550 +/- 106 vs 821 +/- 212 dynes/sec/cm-5). However, after blockade of endothelium-derived relaxing factor, resistances increased gradually in the blocked-flow group to reach the level of that of the continuous-flow group at the end of bypass (943 +/- 77 vs 556 +/- 143 dynes/sec/cm-5 in the pulsatile-flow group). CONCLUSIONS: Blockade of endothelium-derived relaxing factor after 30 minutes of pulsatile flow returns fetal hemodynamics to continuous flow conditions. The specific inhibitor of endothelium-derived relaxing factor used in this experiment suggests that nitric oxide may be released by fetal endothelium during pulsatile bypass.

Animals↗

[Current data on the treatment of venous thromboembolic disease].

Venous thromboembolism disease is frequently seen in medical practice. Its morbidity, mortality and long-term sequels, as well as its haemorrhagic iatrogenic complications, represent a major problem of public health. For therapeutic management, we can usually use in medical practice non-fractionated heparin or low molecular weight heparin, sometimes substituted by antivitamin K therapy. Vena cava filter, surgical thrombectomy or thrombolytic therapy are rarely used. Elastic contention should by systematically prescribed. Therapeutic guidelines have been published on the recommendation furnished by randomised controlled trials. Low molecular weight heparin and early substitution by antivitamin K permitted an ambulatory treatment for deep vein thrombosis. The optimal duration of anticoagulant therapy is still controversial. Adequate biological survey causes decreasing incidence of haemorrhagic complications.

Humans↗

[Severe digital ischemia disclosing Basedow's disease: 2 cases].

We report two observations of young women with concomitant diagnosis of acute arterial occlusion of the upper limb and Graves' disease. The occlusion was probably related to arterial embolism on paroxysmal atrial fibrillation, without underlying cardiopathy. Dosage of thyroid hormones should be performed in case of arterial occlusion in young subjects without cardiovascular risk factors.

Acute Disease↗

Mechanical valve in aortic position is a valid option in children and adolescents.

OBJECTIVE: The choice of a valve substitute remains a challenge in young patients, with numerous reports of early degeneration and calcification of biological valves in this age group. Therefore an assessment of the long-term results after mechanical aortic valve replacement in children was initiated. METHODS: A retrospective study was conducted in 54 consecutive patients aged 1.1 to 17 years (mean 12.8 +/- 4 years) operated on between 1975 and 1993. Aetiology was congenital in 34 patients, rheumatic in 13, infectious in 5, and dystrophic in 2. Concomitant surgery included mitral valve replacement (10), aortic annulus enlargement (9), correction of truncus arteriosus (7), Bentall operation (2), coarctation repair (2), tricuspid valvuloplasty (2), correction of double outlet right ventricle (1), and replacement of a right ventricle to pulmonary artery conduit (1). A Bjork-Shiley valve was implanted in 14 patients, and a St Jude Medical valve in 40. All patients were given Warfarin with a monthly INR control. Follow-up was completed through questionnaires mailed to referring physicians and direct clinical examination. RESULTS: Overall early mortality was 13% (7 cases), and 6% (2 cases) in the 32 patients operated on after 1984. Follow-up was complete in 45 survivors (2 lost to follow-up), with a total follow-up of 261 patient-years. There were 6 late deaths, 4 being cardiac and due to persistent LV dysfunction, and 2 valve-related, due respectively to major gastro-intestinal bleeding and massive thromboembolism. Linearized rates of valve thrombosis and anticoagulant-related hemorrhage were both 0.3% per patient-year. Actuarial survival rate was respectively 84.5% at 5 years and 70.2% at 10 years. Reoperation was necessary in 3 patients for recurrent LV outflow tract obstruction. One patient with severe LV dysfunction is awaiting a heart transplant. CONCLUSION: We conclude that the longterm outcome after mechanical aortic valve replacement in children and adolescents is satisfactory and comparable to currently available reports on biological substitutes. The mandatory anticoagulant therapy is well tolerated in this age group.

Adolescent↗

Malignant neoplasms following cardiac transplantation.

OBJECTIVE: Malignancies have long been recognized as a complication of long lasting immunosuppressive therapy. We reviewed our experience to investigate the incidence and the spectrum of non cutaneous de novo malignant neoplasms. METHODS: Between March 1987 and March 1996, 296 patients underwent 303 cardiac transplantation in our service. The population at risk consists of all patients surviving more than 1 month after transplantation, leading to a total of 267 patients. A triple-immunosuppressive therapy was employed. Moderate doses of antilymphocyte globulin was used as an induction immunotherapy. RESULTS: Neoplasms developed in 18 (6.7%) of the 267 patients at risk. Seventeen patients were male. Mean age was 56 +/- 7 years. Fourteen patients (78%) reported a significant smoking history. Mean interval between transplantation and clinical diagnosis was 36 months. Lung neoplasms (especially adenocarcinoma) were the most commonly encountered tumors (11 of 268 patients, 4.1%). Three Non-Hodgkins' Lymphoma (NHL) were identified (1.1%). No Kaposi's sarcoma were diagnosed. Mean survival after a diagnostic of tumor was 11.7 months. CONCLUSIONS: The incidence of NHL is low in our transplant recipients. Conversely, we observed a high incidence of lung neoplasms (especially adenocarcinoma) which can be correlated with a heavy cigarette use in the study population.

Adenocarcinoma↗

[Medium-term results of non-invasive follow-up after cardiac transplantation in childhood and in adolescence].

Between December 1984 and September 1996, 43 cardiac transplantations were carried out in 40 patients aged 2 days to 21 years (one third under 10 years of age) for cardiomyopathy (21 cases), congenital heart disease (19 cases) or retransplantation (3 cases). The average waiting time for transplantation was 80 days: this delay increased by a factor of five in 2 years (from 1 month, before 1994, to 5 months at present). Twelve patients dies, including 6 before the 8th day. The 28 survivors were prescribed triple immunosuppressive therapy: the average follow-up was 4.4 years (range 3 months to 11 years). Monitoring rejection was carried out by non-invasive methods based on clinical, electrocardiographic and Doppler echocardiographic observations. Any suspicion of acute rejection led to endomyocardial biopsy for confirmation and therapeutic guidance. There was a total of 47 episodes of acute rejection (0.3 per patient), mainly in the first 3 months: acute rejection was less common in the younger children. Graft function was normal in 71% of cases. Five children have a pacemaker implanted during the first month. Despite continuous steroid therapy, 82% of patients had normal staturo-ponderal growth. The myocardial mass of the graft increased in parallel with the body surface area. Nephrotoxicity of ciclosparine was responsible for significant renal failure in 19% of patient and seemed more common in the young children. Psychological disturbances were commonest in adolescence and could result in poor treatment compliance (4 cases, with 1 death and 2 retransplantations). Despite satisfactory medium-term results, nephrotoxicity of ciclosporine, long-term graft function and psychological difficulties of adolescents remain unresolved so that transplantation is reserved for terminal cardiac disease resistant to all other forms of treatment.

Adolescent↗