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Biomedical subjects

M Mathieu

Publications and source records attributed to M Mathieu.

At least 127 records · Page 7Linked to original sources
PubMed1981

Immunochemical studies of human acid alpha-1,4-glucosidase in type II glycogenosis.

The results of immunochemical studies performed in 6 cases of type II glycogenosis (1 classical form, Pompe's disease) and 5 atypical forms (2 juvenile, 3 adult) are reported. The use of antiacid alpha-1,4-glucosidase antibodies greatly improved the specificity of the diagnostic tests for type II glycogenosis, particularly in fibroblasts, lymphocytes, and urine. The use of these antibodies permits the precise measurement of lysosomal enzymes and also enables to demonstrate the activating influence of potassium chloride on urinary lysosomal alpha-1,4-glucosidase. The use of such specific antibodies should prove to be an aid in genetic studies and allows a better understanding of the disease mechanism.

Fibroblasts↗
PubMed1981

[Reliability of DNA measurement after simultaneous staining of DNA and RNA by acridine-orange for flow-cytophotometry (author's transl)].

The reliability of DNA cell content measurement by flow cytophotometry was assessed after simultaneous staining of DNA and RNA by acridine-orange. The methods of staining allowing the lowest CV were determined. The specificity of this staining for DNA appeared satisfactory. When the distribution in the various phases of the cell cycle was computerized, a good correlation was found for the S phase with the 3H-thymidine labeling index. The interest of this method for prognosis and monitoring of acute leukemia is underlined.

Acridine Orange↗
PubMed1980

[Clinical, biological, histological, ultrastructural and therapeutic studies in one case (author's transl)].

The diagnosis of glucagonoma was made in a 51 year-old woman who suffered from a polymorphous dermatitis and an insulin-dependent diabetes mellitus. Denutrition was present and there was a previous history of thrombo-embolism. Immunoreactive plasma glucagon was constantly higher than 1 000 pg/ml (N less than 175). Plasma aminoacids were low. After angiographic confirmation, the tumour and part of its hepatic metastases were resected. The dermatitis disappeared soon after. Its recurrence required chemotherapy (successively mithramycin, streptozotocin, DTIC) and good clinical results were obtained. On histological examination, the cutaneous lesions consisted of an epidermal edema, and a bullous intra-epidermic detachment. The pancreatic tumour was of the trabecular type with a very important sclerosis. On electron microscopy, the tumoral cells, some with a syncitial aspect, contained granules of the D1 type. These granules are different from the typical glucagon granules. The clinical and biological features in this case are compared with those of the 41 cases of glucagonoma previously published.

Adenoma, Islet Cell↗
PubMed1980

Beta-glucuronidase deficiency: enzyme studies in an affected family and prenatal diagnosis.

A beta-glucuronidase deficiency found in serum, leukocytes and fibroblasts and an increased [35S]sulphate incorporation in fibroblasts led us to diagnose two cases of type VII mucopolysaccharidosis in one family. In spite of the wide distribution of activities in serum from controls, decreased beta-glucuronidase activity allowed us to demonstrate the heterozygous status of the parents and two other children. Following these studies, and antenatal diagnosis was performed when the mother was pregnant again; amniotic fluid and cultured amniotic cells were used for enzyme activity determination. A heterozygous fetus was suspected and confirmed after birth. The reliability of various biological materials for enzymatic diagnosis and existence of genetic variants in the normal population are discussed.

Amniotic Fluid↗
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↗