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Biomedical subjects

M Mathieu

Publications and source records attributed to M Mathieu.

At least 91 records · Page 5Linked to original sources
PubMed1985

[Teratogenic risk during treatment of Wilson disease].

Untreated Wilson's disease usually causes infertility or abortion, as a result of increased intrauterine copper level. Therefore, a chelation treatment is necessary during the whole pregnancy. The most used is D-Penicillamine whose teratogenic risks such as cutis laxa, dermatopathy or complex mesenchyme abnormalities are paradoxically rare in the new borns of treated Wilson's disease mothers, perhaps owing to hypercupremia that protects the foetus from excessive copper deficiency. Yet, it's wise to reduce chelation treatment about a quarter fold and to add 50 mg vitamin B6 weekly as we did in our case whose child was born normal.

Adult↗
PubMed1979

Effect of intravenous hydrocortisone administration on glucose homeostasis in small for gestational age infants.

The effects of I.V. hydrocortisone (H) (10 mg/kg) on glucose homeostasis were evaluated at 25 to 85 hours of age in 14 infants who were small for gestational age (SGA) in comparison to 17 control SGA infants. Three hours after H administration, higher levels of plasma glucose than in controls were detected (mean +/- S.E.M.): 4.78 +/- 0.2 vs. 2.88 +/- 0.2 mmol/l (p less than 0.01), while lower levels were found for blood pyruvate (38 +/- 7 vs. 89 +/- 12 mumol/l--p less than 0.01), plasma insulin (6.4 +/- 0.5 vs. 12 +/- 0.8 muIU/ml--p less than 0.05) and plasma glucagon (62.25 +/- 6.6 vs. 81.6 +/- 6.6 pmol/l--p less than 0.05). Three hours after H administration, I.V. injection of L-alanine (150 mg/kg) produced a significant rise over baseline of plasma glucose concentration from 4.78 +/- 0.2 to 5.94 +/- 0.2 mmol/l at 50 min (p less than 0.05), whereas no significant change was observed in controls. There was no significant change in plasma glucagon and insulin concentrations after L-alanine injection in either group. These results show that in SGA infants primed with H, the rise of plasma glucose concentration after L-alanine administration is observed with low plasma insulin levels and without stimulation of glucagon secretion. They suggest that H induced a reduced peripheral utilization of glucose by lowering the plasma levels of insulin and a production of glucose from alanine through gluconeogenesis.

Alanine↗
PubMed1979

The directive sex therapies in psychiatric outpatient settings.

The treatment techniques and associated outcome studies of the directive sex therapies (DST) are briefly reviewed and data on the treatment of 12 couples are presented. The directive sex therapies have achieved rather impressive results but only with highly selected populations. Studies, such as the present, with a range of patients more typical of psychiatric outpatient settings have consistently achieved lower success rates. The important effect of patient selection on outcome with DST has, to date, received little attention. The present authors discuss selection criteria for DST in the light of the literature and the outcome in their own cases.

Adult↗
PubMed1979

[Oculocraniosomatic neuromuscular disease].

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.

Adolescent↗
PubMed1978

[Heterogeneity of glycogenosis with alpha-1,4-glucosidase deficiency: enzymatic studies in three families (author's transl)].

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.

Antibodies↗
PubMed1978

The use of peritoneoscopy in the detection of liver metastases.

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.

Biopsy, Needle↗
PubMed1978

Results of a national therapeutic trial conducted in 10,000 hypertensive patients by 2000 general practitioners.

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.

Adult↗
PubMed1978

Effect of intravenous L-alanine administration on plasma glucose, insulin and glucagon, blood pyruvate, lactate and beta-hydroxybutyrate concentrations in newborn infants. Study in term and preterm newborn infants.

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.

Alanine↗
PubMed1978

[Infantile form of Gaucher's disease. Clinical and biological studies in 1 case. Prenatal diagnosis in 2 further normal pregnancies].

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.

Amniotic Fluid↗
PubMed1978

[Familial congenital muscular dystrophy caused by phosphofructokinase deficiency].

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.

Erythrocytes↗