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Biomedical subjects

L Dallaire

Publications and source records attributed to L Dallaire.

At least 55 records · Page 3Linked to original sources
PubMed1988

Total parenteral nutrition in the newborn: amino acids-energy interrelationships.

As part of an ongoing study on the influence of intravenous glucose and fat on nitrogen metabolism we evaluated the relationship between the source of infused energy and plasma amino acid levels. Thirty-two studies were performed in 16 appropriate-for-gestational-age newborn infants (birth weight, 2150 +/- 115 g; means +/- SEM). In a crossover design each patient received two 6-d periods of isocaloric and isonitrogenous infusions, differing only by the source of calories (high or low fat intakes). For an energy intake of 80 kcal.kg-1.d-1 (335 kJ.kg-1.d-1) there was a significant hypoaminoacidemia (2338 +/- 185 vs 2937 +/- 196 mumol/L, high fat vs low fat) under the high-glucose intake. These data suggest that above an energy intake of 60 kcal.kg-1.d-1 (251 kJ.kg-1.d-1) there is a threshold at which changes in plasma amino acid levels are triggered by variations in the source of infused energy. Careful examination of all variables, including energy sources, is essential when aminograms are compared.

Amino Acids↗
PubMed1987

Prenatal detection of intestinal obstructions, aneuploidy syndromes, and cystic fibrosis by microvillar enzyme assays (disaccharidases, alkaline phosphatase, and glutamyltransferase) in amniotic fluid.

Microvillar enzymes (disaccharidases, alkaline phosphatase, and gamma-glutamyltransferase) were assayed in amniotic fluid from pregnancies with normal and abnormal fetuses to determine their specificity and reliability for the prenatal detection of intestinal obstructions and cystic fibrosis. All fetuses with imperforate anus, duodenal atresia, jejuno-ileal atresia, multiple intestinal atresia, or other forms of intestinal obstructions, with or without associated ventral wall defect or aneuploidy syndrome, showed diminished microvillar enzyme activities below the normal range of control amniotic fluid samples. The exclusively intestinal hydrolases maltase, sucrase, palatinase, and alkaline phosphatase were the most reliable and sensitive markers to detect intestinal obstructions whereas more widely distributed trehalase and gamma-glutamyltransferase activities were less sensitive. The combination of intestinal disaccharidase maltase, sucrase or palatinase and ALP assays is more accurate for prenatal diagnosis of CF than a combination of intestinal ALP and GGTF assays.

Alkaline Phosphatase↗
PubMed1987

mRNA characterization of human fetal enamel matrix.

Preliminary characterization of the human enamel matrix at 16-18 weeks in utero was performed. Using an homogenizing buffer, the proteins were extracted and analysed by gel electrophoresis. Total cellular RNA was isolated and the cell-free mRNA translated. The major component was a 68,000 protein with an enamelin-like amino-acid composition. Other translation products included a 55,000 polypeptide and lower mol. wt components of 26,000, 22,000 and 20,000 size of amelogenin size. It is suggested that high mol. wt component in the enamelin range is the most prevalent at the early stage of human tooth development.

Amino Acids↗
PubMed1986

Clinical heterogeneity in cobalamin C variant of combined homocystinuria and methylmalonic aciduria.

We describe two patients with methylmalonic aciduria and homocystinuria (Cbl C). The disorder was not diagnosed in patient 1 until 4 1/2 years of age; he had a history of fatigue, anorexia, delirium, and spasticity. Moderate megaloblastic bone marrow changes were observed, and there was hyperreflexia of the lower limbs. His condition improved clinically with hydroxycobalamin therapy. Patient 2 was hospitalized at 6 weeks of age because of lethargy and poor feeding. She was found to have macrocytosis. Despite an initial good clinical response to hydroxycobalamin, she developed a striking pigmentary retinopathy. Methylmalonic aciduria persisted in both patients, and homocystinuria persisted in patient 1 despite therapy. The diagnosis of Cbl C disease has been confirmed in both patients by biochemical studies of cultured fibroblasts, including complementation studies. The differences in age of onset and clinical findings together with the similar biochemical findings in these two patients demonstrate the heterogeneity of phenotypic expression in patients with apparently identical abnormalities of vitamin B12 metabolism.

Amino Acid Metabolism, Inborn Errors↗
PubMed1985

Comparative behavioral, biochemical and pigmentary effects of MPTP, MPP+ and paraquat in Rana pipiens.

We demonstrate that injections of 1-methyl-4-phenyl-1,2,3,6-tetra-hydropyridine (MPTP), 1-methyl-4-phenyl-pyridinium ion (MPP+) and Paraquat (PQ+) produce in Rana Pipiens different behavioral, biochemical and skin pigmentation changes. MPTP causes in frogs the main symptoms of Parkinsonism (rigidity, akinesia and tremor) and it darkens the skin of animals. It also decreases brain and, less so, adrenal medulla dopamine. These effects are blocked by Pargyline. MPP+ causes the same symptoms but more rapidly. In contrast, skin pigmentation is clearly lightened. Brain and particularly adrenal dopamine reserves are nearly abolished. Pargyline increases these effects. Paraquat, in a cumulative fashion, eventually causes the same behavioral changes and a slight increase in pigmentation. It initially produces an increase in brain and adrenal dopamine concentrations, but later a significant dopamine concentration decrease. Pargyline potentiates these long term effects, blocks the dopamine increase, but reverses the PQ+ effect upon melanin, producing the same depigmentation as MPP+ alone.

1-Methyl-4-phenyl-1,2,3,6-tetrahydropyridine↗
PubMed1985

New amphibian models for the study of 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP).

We report the development of two animal models in amphibians (frogs and salamanders) in whom 1-methyl-4-phenyl-1,2,3,6-tetrahydropyridine (MPTP) produces the behavioral (neurological) and biochemical equivalents of the human disease and, in addition, a measurable modification in at least one form of pigment-bearing cell from the neural crest, the skin melanocyte. We propose that this new approach can become an inexpensive, easily quantifiable model for the study of the effect of MPTP on the central and peripheral nervous systems. We also demonstrate that the toxic effect of MPTP can be completely abolished in vivo by treatment with a monoamine oxidase inhibitor and potentiated by an inhibitor of catechol-O-methyltransferase. MPTP is catabolised by oxidation into toxic metabolites, but 1-methyl-4-phenylpyridinium ion (MPP+), the proposed end-metabolite, is even more toxic than MPTP in this model, possibly through a different mechanism.

1-Methyl-4-phenyl-1,2,3,6-tetrahydropyridine↗
PubMed1985

[Comparison of the metabolic response of the premature child fed parenterally, and then with mother's milk of prematurity].

The nitrogen retention and plasma amino acid concentration were determined in eight preterm infants (mean birth weight 970 +/- 130 g; mean gestational age 28 +/- 1 weeks) receiving successively total parenteral nutrition and their own mother's milk. The nitrogen retention during both regimens was comparable to the fetal accretion rate. Plasma amino acid concentrations were lower during the enteral phase of the study than during parenteral nutrition. The metabolic response of small preterm infants is related to the quality of amino acids as well as to the route of intake.

Amino Acids↗
PubMed1985

Effect of amino acid composition of parenteral solutions on nitrogen retention and metabolic response in very-low-birth weight infants.

To evaluate the influence of amino acid preparations on the metabolic response of parenterally fed immature newborn infants, nitrogen retention and plasma amino acid concentrations were compared in very-low-birth-weight infants given two parenteral regimens differing only by the composition of the infused amino acids (Travasol 10% blend B and Vamin 7%). The intakes of fluid, nitrogen, and calories were comparable. The nitrogen retention was 72% +/- 7% with Vamin and 65% +/- 6% with Travasol. The differences in plasma amino acid concentrations were consistent with the composition of the amino acid solutions. During the infusion of Vamin the increased intake of aromatic amino acids resulted in high plasma levels of tyrosine (256 +/- 233 mumol/L, range 67 to 894 mumol/L). The infusion of Travasol resulted in high plasma levels of methionine (114 +/- 39 mumol/L, range 53 to 260 mumol/L) and an elevated load of glycine, which was accompanied by an abnormally high urinary loss of this amino acid. Despite these metabolic imbalances, the growth rate over the whole study was adequate. These results emphasize the importance of the composition of amino acid solutions on the metabolic response of the very immature preterm infant.

Amino Acids↗
PubMed1985

Oral and dental development in X chromosome aneuploidy.

Though frequently noted, orofacial anomalies in poly X syndromes have been seldom reviewed. In a study of patients with an X chromosome aneuploidy (XXXXX, XXXX, XXXXY, XXXY) the following facial and dental defects were found to be characteristic features of these syndromes: mainly bifid uvula and macroglossia, enamel defects, dental taurodontism and abnormal roots. These observations are discussed in the light of the possible role of X-linked genes.

Adolescent↗
PubMed1984

Friedreich's ataxia: malic enzyme activity in cellular fractions of cultured skin fibroblasts.

We have measured the activity of malic enzyme NADP+ dependent in the nuclear, mitochondrial, lysosomal and cytosolic fractions of cultured skin fibroblasts from twelve patients with Friedreich's ataxia and nine control subjects. Hexosaminidase, cytochrome-C-oxidase, lactate dehydrogenase and malic enzyme NAD+ dependent were used as marker enzymes. The activity of malic enzyme NADP+ dependent was not significantly reduced in the mitochondrial fraction of patients with Friedreich's ataxia as compared with controls. When corrected for possible contamination between mitochondrial and cytosolic fractions, malic enzyme NADP+ dependent activity was still not significantly reduced in patients with Friedreich's ataxia. Unless critical methodological differences were overlooked in this or previously published studies, we conclude that mitochondrial malic enzyme deficiency is not the primary genetic defect underlying Friedreich's ataxia.

Adolescent↗
PubMed1984

The composition of preterm milk in relation to the degree of prematurity.

Fifty-one samples of 24-h milk collections obtained during the 1st month of lactation from mothers who delivered after gestations of 26 to 31 wk (VPT) contained higher concentrations of nitrogen (297 +/- 11 mg/dl), total fatty acids (4.46 +/- 0.17 g/dl), percentage medium chain fatty acids (10.8 +/- 0.7), and energy (78.3 +/- 2.0 kcal/dl) than either or both those from 32 to 36 wk (PT) and term (T) gestations. PT collections did not differ from T milk with regard to nitrogen (250 +/- 13 versus 259 +/- 13), total fatty acids (3.94 +/- 0.20 versus 3.20 +/- 0.30), percentage medium chain fatty acids (9.1 +/- 0.5 versus 8.1 +/- 0.7) and energy (69.0 +/- 2.7 versus 66.6 +/- 2.4). Although postpartum age (5 to 10 versus 11 to 30 days) did not change the nutrient and energy content of VPT, PT, and T collections, it is only in 11 to 30 day VPT milk that nitrogen and energy content became higher (p less than 0.05) than either or both PT and T milk. We conclude that the differences in macronutrient composition of PT milk are limited to VPT milk and the data from repeated milk collections in the same mother (28 wk) suggest that there is considerable variability in its composition.

Amino Acids↗
PubMed1984

Date of conception and prevention of neural tube defects.

The authors studied the time of conception of 280 fetuses and liveborns with anencephaly or spina bifida seen in a French Canadian population during a period of six years. There is an evident seasonal variation in the conception of NTD fetuses. Using a wave statistical model applied to data recorded over a six year period the authors have rejected the "no variation between the six two-month intervals" hypothesis at a P less than or equal to 0.005 level. It is postulated that, in any vitamin intake or environmental program to prevent NTD in families at risk, one must take into account the annual and seasonal variations, ethnic groups identity and the date of conception in the choice of control mothers.

Canada↗
PubMed1984

Differential stabilities of fetal intestinal disaccharidases determine their relative amounts released into amniotic fluid.

Comparison of relative disaccharidase activities in jejunal mucosa, amniotic fluid, and meconium samples taken from the ileum, proximal and distal colon of 5 human fetuses, indicates that trehalase and lactase are more sensitive to the denaturation conditions existing in the fetal intestinal lumen than maltase, sucrase, palatinase and turanase . Relative activities of sensitive disaccharidases (trehalase and lactase) were also low in the amniotic fluid of these fetuses as compared to the jejunal mucosa. These results suggest that the in vivo stability of disaccharidases, exposed to proteolytic digestion and other denaturing conditions in the fetal intestinal lumen, governs the relative activities of these enzymes released into the amniotic fluid.

Amniotic Fluid↗
PubMed1984

[Role of the genes in odontogenesis].

The authors suggest a genetic model of dental development. This model is based on chromosomal mapping of genes involved in odontogenesis. The role of X and Y chromosomes is updated.

Chromosome Aberrations↗