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This report describes an infant diagnosed aged twenty-five months as having glutaric aciduria Type 1 (GA 1). Initial presentation was with isolated macrocephaly at four months of age. Severe hypertonia, and dystonia, within 24 hours of minor head injury occurred at nineteen months of age. Serial cranial imaging showed subdural fluid collections, and(More)
OBJECTIVE Magnetic resonance spectroscopy (MRS) provides an exceptional opportunity for the study of in vivo metabolism. MRS is widely used to measure phosphorus metabolites in trained muscle, although there are no published data regarding its reproducibility in this specialized cohort. Thus, the aim of this study was to assess the reproducibility of(More)
We postulated that changes in cardiac high-energy phosphate metabolism may underlie the myocardial dysfunction caused by hypobaric hypoxia. Healthy volunteers (n=14) were studied immediately before, and within 4 d of return from, a 17-d trek to Mt. Everest Base Camp (5300 m). (31)P magnetic resonance (MR) spectroscopy was used to measure cardiac(More)
Intestinal surgery is usually associated with the parenteral administration of sodium and water, sometimes in amounts considerably in excess of excretory capacity. We have studied the effect of this situation on the water content of the gut at and 5 cm from a single-layer end-to-end anastomosis in the rabbit. Water content was measured by desiccation. One(More)
Early weight gain by starving patients managed with total parenteral nutrition has been regarded as spurious - that is, merely an increase in body water. We designed an experiment to mimic the starved state in which glycogen stores are depleted and sodium intake is very low. The subjects were then repleted with a sodium-free, high carbohydrate intake. All(More)
A case of dilated cardiomyopathy in a young boy secondary to type II 3-methylglutaconic aciduria is described. A metabolic cause for his dilated cardiomyopathy was suspected because of the development on the electrocardiogram of an unusual "camel's hump" shape of the T waves, and of progressive thickening with increasing echogenicity of the left ventricular(More)
A nucleic acid hybridization assay has been developed for Listeria spp. in dairy foods and environmental samples. The assay is based on detection of unique Listeria 16S rRNA sequences by using a 32P-labeled synthetic DNA probe. Inclusivity and exclusivity of the probe were confirmed with 139 Listeria isolates representing all known species, and 73(More)
Energy requirements of pediatric intensive-care patients are unknown due to the difficulty of measuring total energy expenditure in free-living conditions. We investigated energy expenditure and body composition in stable pediatric intensive-care patients receiving long-term ventilatory support. Total energy expenditure and total body water were measured in(More)
Non-isotopic colorimetric detection has been applied in a rapid nucleic acid dipstick hybridization assay for detection of Listeria spp. in food and environmental samples. The assay takes approximately 2.5-3 h following a two day broth and plate enrichment. Hybridization occurs between fluorescein labeled detector probes, poly(deoxyadenosine)-tailed capture(More)