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Biomedical subjects

J N Fiessinger

Publications and source records attributed to J N Fiessinger.

At least 55 records · Page 3Linked to original sources

Brain involvement in scleroderma: two autopsy cases.

BACKGROUND: Neuropathological data are very scarce in systemic sclerosis and fail to demonstrate primary changes in the brains of such patients. CASE DESCRIPTIONS: A 41-year-old woman with CREST syndrome developed signs of dementia after an episode of severe dehydration and died two months later of septic shock. A 63-year-old woman with CREST syndrome and a history of two unexplained transient ischemic attacks had had balance disorders since age 62. She died of severe pulmonary hypertension. In both cases, the autopsy showed extensive wall calcification of small arteries and arterioles in the brain, primarily in the basal ganglia, and also in the frontal lobes and the cerebellar area in the second case. No known cause of cerebrovascular calcification was found in either patient. CONCLUSION: The neuropathological findings in these two patients suggest that systemic sclerosis may induce primary vascular changes in the brain, of which calcification may be a marker.

Adult↗

Successful treatment of ergotism with Iloprost--a case report.

A 34-year-old woman was hospitalized for severe acute arterial insufficiency of the limbs. Overuse of ergotamine derivative was acknowledged by the patient, who had a long history of migraine headaches. An arteriogram showed diffuse arterial spasm and occlusion of right leg distal arteries. Intravenous infusion of a stable prostacyclin analog (Iloprost) was administered. Rapid and complete improvement of arteriospasm was noted within a few hours, confirmed by a further arteriogram. This appears to be one of the rare case reports of successful limb salvage by use of Iloprost for ergotism.

Adult↗

[Diagnostic and therapeutic stragegies in peripheral obliterative arteriopathy: non-drug treatment].

The therapeutic management of patients with peripheral arterial disease relies initially on the assessment of the severity of arterial insufficiency. At this stage, measurement of ankle systolic pressure plays a particularly important role, and is an essential part of the clinical examination. When the severity of ischaemia jeopardises the survival of a limb, the limitations associated with medical treatment clearly justify all steps being taken to enable the patient to benefit from revascularisation. In this often fragile host environment, endovascular techniques play an important part. As first-line procedures, they have a place within a multidisciplinary management approach, particularly since further surgical procedures, such as distal bypass, often prove necessary. For patients at the intermittent claudication stage, treatment indications become more complex. They include the functional repercussions of peripheral arterial occlusive disease and the cardiovascular prognosis for the patient, which is determined by assessing the extent of the arterial disease. Ultrasonography provides a topographical evaluation of the lesions and their haemodynamic repercussions. This investigation is crucial for screening patients who present with a lesion that may be appropriate for endovascular surgery. Ultrasonography is often programmed at the same time as arteriography. For patients with intermittent claudication, surgical revascularisation is considered only after a minimum 3-month period of medical treatment, for those who have significant functional impairment. In some instances, ultrasonographic evaluation, or even arteriography, may reveal lesions associated with a real risk of deterioration, such as arterial or popliteal aneurysm, and this constitutes the basis of the indication. The development of endovascular techniques has broadened the indications for surgical revascularisation to include patients with intermittent claudication. As a result, there has been a radical change with regard to the management of these patients, limiting the number for whom medical treatment is the only feasible solution.

Arterial Occlusive Diseases↗

Comparison of a once daily with a twice daily subcutaneous low molecular weight heparin regimen in the treatment of deep vein thrombosis. FRAXODI group.

BACKGROUND: Clinical trials have been performed to compare with standard heparin a once or a twice daily regimen of low-molecular-weight heparin but no direct comparison has been done between these two low-molecular-weight heparin regimens in terms of efficacy and safety with a long-term clinical evaluation. METHODS: Patients with proximal deep vein thrombosis, confirmed by venography were randomly assigned to either nadroparin (10,250 AXa IU/ml) twice daily or nadroparin (20,500 AXa IU/ml) once daily for at least 5 days. Regimens were adjusted to bodyweight. Oral anticoagulants were started on day 1 or 2 and continued for 3 months. Patients were followed up for 3 months. The composite outcome of venous thromboembolism and death possibly related to pulmonary embolism was the primary measure of efficacy. Major bleeding was the principal measure of safety. The study was designed to show equivalence between the two regimens. RESULTS: Recurrent thromboembolic events or death possibly related to pulmonary embolism were reported in 13 patients in the once daily group (4.1%) and in 24 patients of the twice daily group (7.2%): (absolute difference 3.1% in favor of the once daily regimen; 95% confidence interval -6.6%, +0.5%). Major bleeding episodes during nadroparin treatment occurred in 4 (1.3%) and 4 patients (1.2%) in the once and twice daily groups, respectively. CONCLUSIONS: A nadroparin regimen of one injection per day is at least as effective and safe as the same total daily dose divided over two injections for the treatment of acute deep vein thrombosis.

Adolescent↗

[Epidemiology and etiological factors in giant cell arteritis (Horton's disease and Takayasu's disease)].

Temporal arteritis is without a doubt the most frequent vasculitis. Incidence is age related and most patients are over 50. The incidence in Caucasian populations shows a North-South gradient with a clear predominance in northern Europe and in the northern part of the United States. In these populations, annual incidence is estimated at 15 to 30/100,000 persons over 50 years of age. The sex ratio (F/M) ranges from 2 to 4. Data in France, Scotland, Italy and Israel show an annual incidence below 10/100,000 inhabitants. The disease is very uncommon in persons of African or Asian ethnic origin. Histology and immunohistochemistry studies of the temporal artery walls favor a vasculitis triggered by one or more unknown antigens, but the cause of temporal arteritis remains unknown. A link with HLA DR4 was demonstrated early. The epidemiology of Takayasu's disease is less well known except for the very strong female predominance (90% of cases). The disease is frequent in Japan, Asia, India and South America, more uncommon in Europe and North America. The "gradient" is thus inverted compared with temporal arteritis. In Japan, among 300,000 autopsies, only 0.03% cases of Takayasu disease have been observed. The incidence of the disease in one study was 2.6 cases per million inhabitants per year. Unlike temporal arteritis, most cases begin early in the 10 to 30 year age range. Genetic susceptibility linked to HLA BW52 is observed in Asia while in the United States, it would appear, though on small numbers of cases, that the frequencies of MB3 and DR4 are increased. We emphasize the epidemiological relationship with tuberculosis and Takayasu disease.

Adolescent↗

[Comparison of magnetic resonance angiography with injection of gadolinium and conventional arteriography of the ilio-femoral arteries].

Twenty-three patients underwent conventional arteriography and 3D contrast enhanced magnetic resonance angiography explorations. The study was limited to the iliofemoral arteries (13 segments for each patient). Each segment was classified as having 0-49%, 50-99% or 100% stenosis. Overall results were excellent with K = 0.822, sensitivity 92% and specificity 93%. Segment by segment analysis corroborated the overall results except for the internal iliac arteries and the deep femoral arteries, demonstrating the limitations of this technique in this series.

Aged↗

[Arterial lesions of the upper limbs in Horton disease. Surgical revascularization by bilateral carotid-humeral bypass].

BACKGROUND: While external carotid lesions predominate, giant cell arteritis (Horton's disease) involves the arteries of the upper limbs in 7.7 to 16% of the patients. CASE REPORT: We cared for one patient with clinically and biologically proven Horton's disease. The patient was given corticosteroid therapy, but although general health status improved and markers of the inflammatory reaction returned to normal levels, exertion-induced ischemia progressively worsened. Due to the length of the arterial lesions, we performed bilateral carotid-humeral bypass surgery. DISCUSSION: Clinical expression of Horton's disease is often quiescent even in cases with extensive anatomic lesions. When clinical signs do appear, they generally respond well to corticosteroid therapy. In certain cases, as reported here, improvement can only be obtained by surgical revascularization.

Arm↗

[Evaluation of a test for rapid detection of D-dimers for the exclusion of the diagnosis of venous thrombosis].

OBJECTIVES: The SimpliRED whole blood D-dimer assay for exclusion of deep venous thrombosis in symptomatic outpatient appears to be a simple and rapid method; we wanted to confirm its reliability. METHODS: Fifty consecutive outpatients (mean age 57, range 20 to 89) referred to our department between September and December 1996, for clinically suspected deep venous thrombosis (DVT) were included. Hospitalized patients were excluded as well as patients under anticoagulant and pregnant women. DVT was diagnosed with our usual strategy of compression ultrasonography at the levels of the common femoral, the superficial femoral and the popliteal veins including the exploration of sural and saphenous veins. The D-dimer assay was performed, according to the manufacturer recommendation, blindly by a physician unaware of the results of ultrasonography within one hour. RESULTS: Eight of nineteen patients with DVT had a normal D-dimer test result Four had a sural DVT, but four had a proximal DVT. Furthermore four patients with normal D-dimers had superficial venous thrombosis. CONCLUSIONS: Our series does not confirm the high sensitivity and negative predictive value reported previously. To date it is premature to propose this assay as a first line test in the therapeutic management of patients with suspected DVT.

Adult↗

[Lines of conduct for training of physicians in echography and vascular Doppler and realization of vascular ultrasonic tests].

Vascular tests with duplex scanning have an important lace in the diagnostic and therapeutic strategy in vascular medicine; they are integrated by the physician within the strategy of pathology support. The realization and interpretation of vascular duplex scanning are subject to recommendations. The training of physicians takes into account the clinical context due to the fact that all aspects of these examinations are inseparable. Besides the theoretical education, it is important to be thorough in practical training. The realization of a minimal number of 250 examinations is advised, distributed among different sectors of anatomy and pathology. The acquiring and preservation of technical skills and the ability to provise solutions, requires the realization of additional 250 examinations. This total amount of 500 examinations is integrated into a global 2 year educational program. The preservation of skills requires 500 annual examinations. The training center is placed under the responsibility of a clinical physician, recognized in vascular medicine. This center must perform more than 2,000 examinations a year. Various centers can associate their abilities in order to ensure a complete training program.

Blood Vessels↗

[Distal gangrene and cryoglobulinemia related to hepatitis C virus infection with presence of anticardiolipin antibodies].

We report the case of a 63-year old women with toe gangrene, peripheral polyneuropathy, polyarthritis, histologically proven necrotizing vasculitis, in association with type III mixed cryoglobulinemia and hepatitis C virus (HCV) infection. Raised anticardiolipin antibodies (aCL) were found, without beta 2-glycoprotein I. HCV infection is associated with mixed cryoglobulinemia which can cause a vasculitis affecting various organs. The pathogenesis of production and clinical significance of aCL could be associated in this case with HCV infection.

Antibodies, Anticardiolipin↗

Interdonor variability of platelet response to thrombin receptor activation: influence of PlA2 polymorphism.

Considering that platelet response to thrombin receptor activation might be critical for the development of arterial thrombosis, we measured the dense granule release under stimulation by the thrombin receptor activating peptide (TRAP) in a series of 102 healthy volunteers. The threshold TRAP concentration which initiated a secretion ranged from 3 to 20 microM. A good concordance (79%, k=0.677) between two tests performed at a 1 month interval indicated that platelet response to thrombin receptor activation was characteristic of each individual donor. Since the threshold concentration required to initiate secretion corresponded to the threshold concentration which induced a biphasic aggregation, all volunteers were genotyped for the PlA2 polymorphism, the Pro33 variant of GPIIIa. Platelets from subjects with the PlA2 polymorphism required higher TRAP concentrations to aggregate than those from subjects with no PlA2 allele (P=0.0012). However, they also required a higher ADP concentration to aggregate. In order to exclude any influence of GPIIIa polymorphism on TRAP-induced secretion, we studied the variability of platelet response to TRAP among the 77 individuals with no PlA2 allele, and found the same interdonor variability with the same distribution of threshold TRAP concentrations as for the 102 individuals. The results suggest that (i) platelet secretion in response to thrombin receptor activation could be a genetically controlled phenotype independent of the GPIIIa polymorphism; (ii) the PlA2 polymorphism is associated with platelet hypoaggregability.

Blood Platelets↗

Clinical features in 36 patients homozygous for the ARG 506-->GLN factor V mutation.

We analyzed the clinical features of 36 patients homozygous for the Arg 506 to Gln factor V mutation and found a circumstantial event at risk for thrombosis in 29 of the 31 patients with thrombosis. The most frequent predisposing factors were the post-partum period and the use of oral contraceptives in women, and surgery in both sexes. Venous thrombosis recurred in 48% of the patients. One patient had a myocardial infarction at age 33 years, and also had an antiphospholipid syndrome. Homozygous Gln 506 mutation leads to far less severe thrombotic complications than homozygous protein C and protein S deficiencies and does not seem to predispose patients to arterial thrombosis.

Adult↗