Biomedical subjects
Publications and source records attributed to M Mathieu.
A child with a myopathy that started in the pelvic girdle, non-obstructive cardiomyopathy and retinitis pigmentosa is described. There was a progressive neurological deterioration with external ophthalmoplegia and ptosis. The clinical course could be predicted from the appearance of the muscle biopsy.
The authors describe four cases of atypical forms of glycogenosis with alpha-1,4-glucosidase (acid maltase) deficiency. The results of clinical, microscopic, histochemical, enzymological and immunological studies are described. Acid maltase activity has been studied in muscle, leukocytes and fibroblasts. The authors show no difference in the properties of acid maltase; the authors study the purified enzyme from various tissues.
Peritoneoscopy was carried out in 352 cancer patients with clinical suspicion of liver involvement in most cases. Principally because of patient discomfort, adequate liver biopsy was obtained in only 66% of 240 patients who underwent peritoneoscopy under local anesthesia while, under general anesthesia, biopsies could be taken in 90% of 112 patients. When the liver was macroscopically free of disease, the yield of positive peritoneoscopy was minimal regardless of the number of blind deep biopsies. Peritoneoscopy provided histologic demonstration of hepatic invasion in a total of 55 patients. Seven false-negative examinations out of 19 negative peritoneoscopies (36%) were identified by subsequent laparotomy or autopsy within 2 months. These preliminary data, although difficult to interpret in terms of accuracy of the method, point to the possible contributions of peritoneoscopy in detecting liver metastases.
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1. 10,294 hypertensive patients were treated and followed by 2200 general practitioners under the supervision of 130 cardiologists and nephrologists. 2. The treatment groups, randomly allocated, were designated to use three distinct antihypertensive drugs, administered alone, and combined two-by-two. 3. Some 75% of patients had a supine diastolic blood pressure of less than 95 mmHg after 4 months treatment. 4. A total of 12% of patients had dropped out by 4 months from entry; no clear relationship was established between side effects and drop out.
Ten term and eleven preterm newborn infants with appropriate weights for their gestational age were infused for one minute with L-alanine (150 mg/kg) at the age of 29 to 76 hours (mean 48 hours) and circulating levels of glucose, lactate, pyruvate, D-betahydroxybutyrate (D-BOHB), insulin and glucagon were monitored. Plasma glucose concentrations increased from 2.7 +/- 0.16 (mean +/- S.E.M.) to 3.7 +/- 0.2 mmol/l after 50 min (p less than 0.01) in term infants. In preterm infants, after an initial decrease of the glucose level from 3.1 +/- 0.16 to 2.6 +/- 0.16 mmol/l (p less than 0.05), it returned to the baseline level at 50 min: 3.0 +/- 0.2 mmol/l. The blood concentration of D-BOHB decreased in term infants from 192 +/- 37 to 112 + 6 micrometer/l (p less than 0.01) after 40 min. In preterms, its decrease was not significant (p greater than 0.05). Plasma glucagon level rose from 53 +/- 5 to 70 +/- 8 pmol/l after ten minutes (p less than 0.01) in terms infants and from 61 +/- 6 to 75 +/- 9 after 20 min (p less than 0.01) in preterm infants. There were no significant changes in plasma insulin concentrations in either group. Forty minutes after L-alanine infusion, I/G ratios were lower in preterm infants (1.26 +/- 0.14) than in term infants (1.71 +/- 0.25) (p less than 0.01). There was no relationship between the glycemic responses to L-alanine and the basal levels of D-BOHB. The data suggest that the glycemic effect of L-alanine infusion and circulating glucagon depends upon a specific stage in maturation. The antiketogenic effect of L-alanine infusion is observed in term infants as in adults.
A clinical report of a typical case of infantile Gaucher's disease is given. Study of the beta-glucosidase activity in leukocytes and skin fibroblasts confirmed the diagnosis in the patient and established the heterozygote condition of the parents. Two later pregnancies have been monitored by enzymatic study of cultured amniotic fluid cells. Both foetuses were predicted to be healthy, which was confirmed after birth. The report includes a histopathological ultrastructural, chemical and enzymatic study of liver, spleen and brain obtained at autopsy. Possible clues to the physiopathological mechanism of the cerebral lesion are discussed.
Two children, born to related parents, presented since birth a muscular defect rapidly complicated by painful joint stiffness. The oldest child died at 6 months of age, from respiratory complications. The second-14 month old- does not sit without support. The muscle fibres are of unequal calibre and numerous fibres have under-sarcolemmal PAS positive areas contain glycogen, as seen on electron microscopy. In the second patient, the biochemical analysis showed a moderate glycogen accumulation and muscular enzymatic studies demonstrated an isolated and major deficiency in phosphofructokinase activity. Activity was normal in red blood cells and in fibroblasts cultured in vitro. Hence, these cases should be distinguished from formerly reported cases of phosphofructokinase deficiency. This type of P.F.K. deficiency should be looked for in patients with severe congenital muscular dystrophy and early joint involvement.
Various lesions have been described as post-intubation complications: injuries, of usually minor degree, of the pharynx and larynx, oedema of the larynx, ulcerations of the pharynx and larynx with pseudomembranes and bleeding, chondromalacy of the larynx, granulomata, oesophago-tracheal fistula, stenosis of the larynx or trachea, paralysis and synechia of the vocal cords, paralysis of the tongue. Etiologic factors of these complications are mainly chemical, in relation with the material of the tube and with the sterilization agents, or mechanical due to pressure on neighbouring tissues. Post-intubation sore throat seems independent from traumatic laryngoscopy. Although intubation is meant to provide safer ventilation, interference with respiration may occur by compression of the tube or accidental obstruction from various causes. It should not be forgotten that in anesthesia cases, for which intubation is not really required, it may be advantageous to administer the anesthetic by mask.
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The induction of beta-galactosidase and alkaline phosphatase by the nucleoid of Escherichia coli was studied. Only the membrane-associated form was active in the presence of S 30. The induction of beta-galactosidase showed an absolute requirement for the inducer and was enhanced by cyclic AMP and cyclic GMP. Further-more, in our hands, the synthetic activity of the membrane-associated nucleoid proved to be far higher than that of the soluble system described by Zubay. Our results suggest that membrane shield the structure which is necessary for the integrity of the initiation step of both transcription and translation.
In this article we describe and contrast the psychodynamic and directive approaches to the treatment of sexual dysfunction. We have also summarized and critically reviewed published reports with ten or more cases. No firm conclusions can be drawn on the efficacy of the psychodynamic approach with this population because of the absence of controlled studies. Fairly conclusive evidence for the efficacy of the directive approaches is now available, but solely with highly selected populations. Success rates in studies with unselected psychiatric outpatient populations are much less favorable. The effect of characteristics of the patient population on outcome with the directive sex therapies has received dangerously little attention.
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