[Familial dysbetalipoproteinemia].
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Biomedical subjects
Publications and source records attributed to Y Levy.
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The authors report a case of combined mesenteric and retroperitoneal fibrosis resulting in vascular and digestive compressions. Diagnosis was established by laparoscopy and biopsy. No etiologic factors were found. Such an association is exceptional and suggests that the disease is systemic. The possibility of fibrosis arising far from the mesenterium (mediastinum, extrahepatic biliary tract, thyroid, orbit) has led some authors to suggest that this condition be termed Idiopathic Systemic Fibrosis. An immunologic origin has been speculated in the literature.
The cardiovascular effects of lithium have been indexed in literature since 1900, most frequently in cases of intoxication. We recently studied the exceptional case of a patient showing a serious sinus node dysfunction during lithium treatment. The serum lithium levels were always within the therapeutic range. After two endocardial explorations and withdrawal of lithium, we found that the sinus node dysfunction was due to lithium and reversible after withdrawal of it. Some ignored dysfunctions could exist with a regularly controlled treatment.
We studied an exceptional case of a patient showing serious sinus node dysfunction during lithium treatment with levels constantly within the therapeutic range. After two endocardial explorations we found that the sinus node dysfunction was due to lithium and reversible after its withdrawal.
Plasma lipids and lipoproteins were studied in 21 patients with benign monoclonal gammopathy, 21 patients with multiple myeloma and seven patients with Waldenström's macroglobulinaemia. Results were compared with those of a control group, age and sex matched. Low plasma cholesterol levels in all three patient groups were associated with low HDL-cholesterol concentrations. Apo A-I, but not apo B, was significantly reduced. Sixty per cent of the patients exhibited an extra lipid band on plasma lipoprotein electrophoresis, which could be an immunoglobulin-lipid complex. In these patients plasma and LDL-cholesterol levels were significantly lower than in those patients in whom this band was absent. No correlation was found between the severity of the disease and plasma lipid pattern.
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Fourteen patients with established hypertension followed a double-blind crossover-styled trial to study the effects of 100 mg/day atenolol compared to placebo. Atenolol was found to be an effective antihypertensive agent, reducing both systolic and diastolic blood pressure. Hypertensive patients appear to have increased in vitro platelet adhesion and aggregation. Atenolol significantly reduced platelet adhesion, but had little effect on aggregation. This may be important in contributing towards the now-recognised cardio-protective effect of the beta-adrenoceptor blocking agents. Blood chemistry and haematological parameters were unchanged; but whereas plasma cholesterol and plasma triglyceride levels remained normal, there was a significant fall in plasma high-density lipoprotein cholesterol levels. Side effects were very few.
The mechanisms by which dietary fat influences fasting plasma lipid concentrations have been investigated in hyperlipidaemic subjects. The synthetic and fractional catabolic (FCR) rates of the apoprotein B (apo B) of very-low density (VLDL) and low-density (LDL) lipoproteins were measured using radioiodinated autologous lipoproteins. Reductions of LDL concentration in eight subjects during low-fat (25% of energy) diets were largely explained by diminished synthesis (-20%, P less than 0.02), and possibly also by an increased FCR (+15%, P = 0.05) of LDL, compared with observations made during a high-fat (45% of energy) diet of similar fatty acid composition. VLDL apo B synthesis and FCR were not significantly altered. When a diet rich in polyunsaturated fatty acids was exchanged for one high in saturated fatty acids (fat providing 45% of energy on both occasions) in four subjects, the synthetic rates of both VLDL apo B (-31%, P less than 0.02) and LDL apo B (-23%, P less than 0.10) were reduced while their FCRs were unchanged.
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Seven patients, 4 women and 3 men afflicted with severe progressive systemic sclerosis (PSS) were treated with Plasma Exchange after failure of different other treatment. All patients presented Raynaud phenomenon and arthritis, 6 patients presented extensive skin lesions, 5 of them digestive manifestations, 3 pulmonary fibrosis. In one case PSS was associated with polymyositis, one patient presented bilateral recurrent cornea ulcerations, (Sjögren Syndrome++) and one patient numerous skin ulcerations. In 5 patients adjuvant corticosteroid therapy was given during the course of PE. In 3 patients PE must be stopped after one or two sessions because of insufficient venous access. Among the 4 other patients 8 to 20 PE were performed: the patient with cornea ulcerations became blind during the treatment, skin ulcerations and severe Raynaud phenomenon did not improved in two other patients. Benefit of PE was noted in only one patient with regressive myositis, and improvement of articular and cutaneous symptoms. Therefore, PE are not useful in most patients afflicted with PSS, they are difficult to realize in numerous patients and did not improve clinical symptoms in most cases.
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Two patients with splenic abscess successfully diagnosed and treated are described. The first case, a diabetic, developed a pyogenic abscess caused by klebsiella, while the other suffered a splenic infarct complicated by splenic abscess. although relatively easy to treat, solitary abscess of the spleen is a potentially lethal condition due to diagnostic difficulties. In both present instances the final diagnosis was easily achieved before surgery by the aid of computerized tomography (CT). This investigation seems to be more accurate than 99Tcm-sulphur colloid scan and ultrasound scan, which failed in both cases to diagnose splenic abscess clearly. The use of this investigation is suggested in every suspected case and will, no doubt, confirm the diagnosis at an early stage of the disease. Splenectomy with antibiotic cover will usually result in cure.
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