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
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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