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Biomedical subjects

M Mathieu

Publications and source records attributed to M Mathieu.

At least 163 records · Page 9Linked to original sources

[Prenatal ultrasonic diagnosis of malformation uropathies: 13 cases].

The systematic prenatal diagnosis of 5 cases of urinary tract malformations out of 2 500 pregnancies in 1982 in a special care maternity, or 2 per 1 000 is compared with the discovery of 10 cases in 28 months in 17 960 foetuses, or 0.55 per 1 000 whereas the overall frequency varies from 0.3 to 5.25 per 1 000 in infants. We studied 13 children whose prenatal abnormalities were 2 unilateral ureterohydronephrosis and 7 bilateral dilatations associated to 2 ureteroceles, 1 multicystic dysplasia and 1 ureteral duplicity. All of these except two were confirmed at birth. The exceptions consisted in one unilateral dilatation and one bilateral ureterohydronephrosis which turned out to be dysplastic kidneys unknown during pregnancy. Three ureteral duplicities were also ignored. The results are the following: Three cases were severe: one therapeutic abortion after 27 weeks, one intra-uterine decompression followed after few days by a preterm delivery and neonatal death and one provocated preterm delivery (36 weeks). All the others underwent decompression during the first hours of their life. Five cases can be considered unsuccessful: 2 abortions, the death of a 22 days old infant with an unilateral multicystic dysplasia and 2 bilateral malformations with renal failure, one of which was associated to an unilateral dysplastic kidney. Reliability of sonography as well as its interest in prognosis and prenatal evaluation of renal function are demonstrated.

Amniotic Fluid

Transferability studies for the AACC reference method and the IFCC method for measurement of alkaline phosphatase activity.

We present the results of measurements of alkaline phosphatase activity from interlaboratory transferability studies conducted by 12 laboratories in five countries. The variability, as demonstrated by within-day precision (CV less than or equal to 2.6%), between-day precision (CV less than or equal to 3.6%), and between-lab precision (CV less than or equal to 6.3%) establishes the transferability of this method for alkaline phosphatase. Some common errors encountered in enzyme measurements that affect the accuracy and precision of the measurements are listed.

Alkaline Phosphatase

In vitro culture of clonogenic leukaemic cells in acute myeloid leukaemia: growth pattern and drug sensitivity.

Bone marrow from 43 of 45 AML patients grew leukaemic colonies in culture with a technique using methyl-cellulose semi-solid medium and stimulation with PHA-leucocyte conditioned medium. Plating efficiency was significantly greater in M4FAB subtypes than in M1 or M2. The presence of Auer rods in cultured cells and the existence of cytogenetic abnormalities in both fresh and cultured blast cells in one patient confirmed the leukaemic origin of these colonies. These clonogenic cells were closely related to the growth fraction, as demonstrated by a high suicide index and a linear correlation between percentage of bone marrow blasts in S phase and plating efficiency. In vitro CFU-L sensitivity to cytosine-arabinoside (ARA-C) and to adriamycin (ADR) was tested in 22 patients treated with these two drugs. In the group sensitive in vitro to ARA-C (10 patients), 70% entered complete remission. In the resistant group (12 patients), only 25% had complete remission while 75% had resistant disease. Eight of 14 patients sensitive to ADR in vitro achieved complete remission, while five were resistant to chemotherapy. On the other hand, six of eight patients resistant in vitro were resistant in vivo. When drug sensitivities to ARA-C and ADR were cumulated, an excellent in vitro to in vivo correlation was found when the patient was sensitive or resistant to both drugs in vitro.

Bone Marrow

[Regular dominance of thumb ankylosis with mental retardation transmitted over 3 generations].

A young girl 12 old, sent to us for obesity, and coxa-epiphysiolysis showed signs of mental retardation and bilateral thumb ankylosis. The fact that the mother was also affected by both of these signs, led to a more detailed genetic research. The latter revealed that not only the daughter, the mother, but also their own mother and may be, the sister, the grand-mother and the great-aunt of the patient had a retardation, a slight dysmorphia, a type A brachydactylia, signs of obesity and an identical ankylosis of both thumbs. This vertical inheritance, affecting apparently females only, but not associated with a high rate of miscarriage, has, it seems, never been reported. The characteristics of this family are being considered and discussed.

Adult

Human placenta as a convenient source of creatine kinase BB.

We demonstrate that human placenta is a convenient source of creatine kinase isoenzyme BB. We compare the physicochemical and catalytic properties with those of other creatine kinase isoenzymes: purified human abdominal muscle MM and brain BB. We also describe a stabilizing medium for creatine kinase BB. Human placental and brain BB have similar catalytic properties, the respective Km values for creatine phosphate being 0.66 and 0.56 mmol/L and for adenosine diphosphate 89 and 70 nmol/L.

Adenosine Diphosphate

PHA-induced blast colonies and kinetic parameters in acute myeloid leukemia.

The in vitro leukemic colony-forming ability of 30 acute myeloid leukemias tested after PHA stimulation was analyzed by comparing the CFU-GM growth pattern, the myeloblast [3H]thymidine index and the response to treatment. PHA-induced leukemic colonies were obtained in 15 out of 30 cases. No correlation was found between the FAB classification, the CFU-GM growth pattern and the PHA-induced colony growth. A significant correlation was observed between PHA-induced colony growth and [3H]thymidine labeling index (p less than 0.001). A correction was also noted between the presence of leukemic growth and the failure of the induction treatment (p less than 0.02). The proliferation of the clonogenic blastic subpopulation selected by this technique seems to be linked to a poor prognosis.

Cell Division

Theophylline kinetics and ventilatory flow in bronchial asthma and chronic airflow obstruction: influence of erythromycin.

The kinetics and the effects on the ventilatory function peak expiratory flow rate (PEFR) of a single 600-mg oral dose of theophylline were investigated in 46 adult patients with bronchial asthma (BA) and in 16 adult patients with chronic airflow obstruction (CAO). In the former, theophylline induced an early and potent bronchodilatation (60% rise in PEFR), the kinetics of which correlated with plasma concentration. Theophylline was also effective in patients with CAO, but the magnitude of its bronchodilator effect was less than in those with BA: this was despite plasma concentrations of much the same order. In adult patients with BA (but not with CAO) theophylline plasma levels and bioavailability are higher after simultaneous erythromycin dosing.

Adult

An evaluation of three approaches to the teaching of a behavioral therapy.

Evaluated the relative efficacy of three approaches to teaching mental health professionals how to do social skill training (SST). All three conditions received a minimal of a common reading package and videotaped modeling of competent SST. Ss in a structured learning format (SLF) as well receive instructions, rehearsal and feedback for a total of 20 hours (N = 39). Therapists in a seminar format (SF) condition discussed topics associated with SST raised by the readings and films for 20 hours. In addition to modeling and the readings, Ss in the workshop format (SF) condition received concise instructions on how to carry out SST during 6 hours of training. All three conditions showed equivalent and significant pre- to posttraining improvement on a questionnaire measure of familiarity with SST; however, only the SLF and CW conditions improved significantly on a test of the therapists' evaluative skills. Finally, on a measure of therapist behavior during a simulated SST session, the three groups showed roughly equivalent improvement. The results were interpreted in terms of the particular ingredients present in each condition, and directions for future research were discussed.

Adult

Effect of oral administration of lipids with 67% medium chain triglycerides on glucose homeostasis in preterm neonates.

Since hypoglycemic responses to medium chain triglycerides (MCT) have been reported in adults we studied the effect of an acute oral load of lipids (2,8 g/kg) with 67% MCT on glucose homeostasis in 21 preterm infants in comparison to 14 age-matched control preterm infants. A hyperglycemic response from (mean +/- SEM) 57 +/- 1.1 to 74 +/- 2.5 at 30 min (p less than 0.01) and to 80.5 +/- 2.5 mg/dl at 60 min (p less than 0.01) was observed after administration of the lipids whereas no change in plasma glucose concentration was observed in the control group. After administration of the lipids there was no change in the concentration of insulin and glucagon in plasma. An intravenous glucose tolerance test (1 g/kg) was similar in the control group and 60 min after administration of the lipids. After administration of the lipids free fatty acid concentration remained unchanged while a significant decrease from 304 +/- 56 to 199 +/- 28 muEq/l was observed in 60 min in the control group. At 60 min beta-hydroxybutyrate concentration was higher after lipid administration (630 +/- 86 mumol/l) than in the control group (436 +/- 66 mumol/l) (p less than 0.05). A more rapid decrease in blood lactate concentration was found after lipid administration than in the control group while no change in plasma alanine concentration was observed in either groups. In five additional preterm infants, plasma glucose concentration increased from 56 +/- 0.6 to 75 +/- 0.9 mg/dl (p less than 0.01) 60 minutes after gastric administration of glycerol (0.3 g/kg). These data show that in preterm infants, a lipid load with 67% TCM produces a hyperglycemic response through gluconeogenesis without changing the peripheral rate of glucose disappearance.

3-Hydroxybutyric Acid

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

[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

[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

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