Excess granulation tissue during etretinate therapy.
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Biomedical subjects
Publications and source records attributed to M David.
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An arab moslem family with members affected by PTA deficiency is described. 3 children were found to have major deficiency, factor XI procoagulant activity being 3, 3 and 4 units/dl. 8 members, including parents, paternal grandparents and 4 siblings, were found to have minor deficiency of factor XI (40 to 68 units/dl). Assays of immunoreactive material in 4 members corresponded to the level of procoagulant activity. In this family, gene expression is autosomal recessive. The only bleeding episode reported was haematuria in the propositus. No other spontaneous, post-trauma or post-operative bleeding was noted. The PTA deficiency was reported until now, mainly in ashkenazi jews. This family is the first case of PTA deficiency ever reported in arab moslems.
In 20 patients with Kaposi's sarcoma (KS) the level of B and T lymphocytes in the peripheral blood, as well as the function of T cells measured by the graft-versus-host reaction (GVHR), were investigated. Analysis of the results regarding the distribution of skin lesions showed that the mean GVHR was within normal range in 13 patients in whom the lesions were confined to the distal portions of the extremities. In 7 patients, who demonstrated generalized distribution of the eruption, the mean GVHR was significantly lower in comparison with the control group. In view of our results we may conclude that cellular immunity is impaired in KS patients with generalized distribution. Testing T cell function is recommended as a possible prognostic indicator.
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Patients with a cancer of the upper airways or upper gastro-intestinal tract present a state of malnutrition as a result of the disease itself and, more importantly, as a result of its localisation. Loco-regional radiotherapy often leads to an aggravation of this state. The protein profile, consisting of nine serum proteins, was determined each week in 54 patients with cancer of the upper respirato-gastro-intestinal tract receiving radiotherapy. During the course of radiotherapy, the already altered nutritional state of these patients deteriorated further, as shown by a regular and significant downturn in the weight curve. The weekly monitoring of the protein profile showed a gradual and significant decrease in the levels of nutritional proteins (prealbumin, retinol binding protein, transferrin) and immunoglobulins (IgM, IgA) and a small variation in the levels of inflammatory proteins (haptoglobin, orosomucoid, C3 complement fraction, alpha 1-antitrypsin). The protein profile, established on the basis of carefully selected proteins, can provide useful information in the monitoring of a patient's nutritional state.
After a recall of surgical indications and of technical points of the lower limbs amputations in arteriosclerosis disease, the authors report their experience of early rehabilitation. They define the aims of the rehabilitation and the means to reach it. Among these, a multipurpose prosthesis made by them, authorized their patients to an early walk training. The results are analysed with 86 patients rehabilitated in 2 years by this method. They have 49 good results/51 with amputation below the knee, 31 good results/35 with femoral amputation. The mean age of the patients is 72.
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The medium chain acyl-CoA deshydrogenase defect: a new inherited metabolic disorder. This enzymatic defect blocks the catabolism of non esterified fatty acids during fasting. Thus, this disease is revealed by a coma due to hypoglycemia in a young child; the presence of dicarboxylic aciduria in such a situation is the main evidence for this diagnosis. Finally, the enzymatic studies performed on skin fibroblasts show a defect in medium chain acyl-CoA deshydrogenase. When a child is investigated away from a coma episode, the ketotic diet induces dicarboxylic aciduria but must be performed in an intensive care unit for its dangers.
Fifty-four percent of a sample of 227 women reported having experienced an orgasmic expulsion of fluid at least one time. The source of the fluid is still not certain.
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Authors report two observations of embolus in the superior mesenteric artery. Patient's age, bad general status of the first, obesity of the second, importance of one emboli and especially affirmative angiography indicate treatment by streptokinase like the usual one in venous pathology. Good clinical result is approved by new control arteriography in two cases.
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Urinary analysis of the pattern of 23 organic acid metabolites derived from fatty acids in three patients with general (medium-chain) acyl-CoA dehydrogenase deficiency was performed. Although there exist quantitative differences in the excreted amounts of the different metabolites in the three patients the qualitative picture was the same. The excretion of adipic, suberic and sebacic acids was substantial, whereas that of dodecanedioic acid was within or just above control limit. The monounsaturated C6-C10-dicarboxylic acid excretion was only marginally or not increased. 5-OH-hexanoic acid and hexanoylglycine were excreted in excessive amounts, whereas 7-OH-octanoic acid, 9-OH-decanoic acid, octanoylglycine and decanoylglycine were excreted in limited amounts. The excreted amounts of 6-OH-hexanoic, 8-OH-octanoic and 10-OH-decanoic acids were not or only marginally elevated compared to controls. In one of the patients the excretion of ethylmalonic and methylsuccinic acids was enhanced, whereas the excretion of these two acids in the two other patients was comparable to that in controls. The urinary excretion of hexanoic, octanoic, decanoic and dodecanoic acids was just a little above the control limit, whereas the esterified hexanoic and octanoic acids were excreted in appreciable amounts. It is argued that the microsomal omega- and omega-1-oxidation systems are involved in the dicarboxylic and omega-1-OH-monocarboxylic acids formation at C10 and C12 level and that the C8-C6-dicarboxylic and omega-1-OH-monocarboxylic acids are formed from higher chained acids by beta-oxidation in both mitochondria and peroxisomes.