Triacylglycerol infusion does not improve hyperlactemia in resting patients with mitochondrial myopathy due to complex I deficiency
| Autor: | Dirk-Jan Reijngoud, Satish C. Kalhan, Kees de Meer, Mark J. Roef, Helma W. H. C. Straver, Ruud Berger, Martina M.J. de Barse |
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| Přispěvatelé: | Faculteit Medische Wetenschappen/UMCG, Life Course Epidemiology (LCE) |
| Rok vydání: | 2002 |
| Předmět: |
Blood Glucose
Respiratory chain Medicine (miscellaneous) CHILDREN glucose infusion chemistry.chemical_compound substrate oxidation Infusion Procedure complex I deficiency NADH NADPH Oxidoreductases PHOSPHORYLATION Infusions Intravenous Nutrition and Dietetics Chemistry Mitochondrial Myopathies Lactic acid Treatment Outcome medicine.anatomical_structure Lactates SKELETAL-MUSCLE Female RESPIRATORY-CHAIN Adult NUTRITIONAL-STATUS medicine.medical_specialty Adolescent Diet therapy GLYCOLYSIS hyperlactemia stable isotopes Calorimetry Carbohydrate metabolism Oxygen Consumption Internal medicine MAGNETIC-RESONANCE SPECTROSCOPY medicine LACTATE KINETICS Humans Triglycerides Electron Transport Complex I mitochondrial myopathy Skeletal muscle ISOTOPIC EQUILIBRATION Metabolism Carbohydrate P-31 magnetic resonance spectroscopy Glucose Endocrinology Case-Control Studies triacylglycerol infusion |
| Zdroj: | American Journal of Clinical Nutrition, 75(2), 228-236. Oxford University Press |
| ISSN: | 0002-9165 |
| DOI: | 10.1093/ajcn/75.2.228 |
| Popis: | Background: A high-fat diet has been recommended for correction of biochemical abnormalities and muscle energy state in patients with complex I (NADH dehydrogenase) deficiency (CID). Objective: This study evaluated the effects of intravenous infusion of isoenergetic amounts of triacylglycerol or glucose on substrate oxidation, glycolytic carbohydrate metabolism, and energy state in patients with CID. Design: Four CID patients and 15 matched control subjects were infused with triacylglycerol (1.85 mg(.)kg(-1.)min(-1)) or glucose (5 mg(.)kg(-1.)min(-1)) while at rest. Respiratory calorimetry was used to evaluate mitochondrial substrate oxidation. Metabolism of glycolytic carbohydrate was determined on the basis of the rates of appearance and concentrations of plasma lactate from dilution of [1-C-13]lactate measurements. In addition, high-energy phosphate metabolism was measured in forearm muscle by P-31 magnetic resonance spectroscopy. Results: Whole-body oxygen consumption rates were higher in the patients than in the control subjects (P |
| Databáze: | OpenAIRE |
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