Energy substrate metabolism in pyruvate dehydrogenase complex deficiency

Journal of Pediatric Endocrinology and Metabolism

Volumn 27, Issue 11-12, 2014, Pages 1059-1064

  Stenlid, Maria Halldin ^a   Ahlsson, Fredrik ^a   Forslund, Anders ^a   Von Döbeln, Ulrika ^b   Gustafsson, Jan ^a  

^a UPPSALA UNIVERSITY   (Sweden)

^b KAROLINSKA UNIVERSITY HOSPITAL   (Sweden)

Author keywords

glucose production; ketogenic diet; lipolysis; pyruvate dehydrogenase deficiency; respiratory quotient; resting energy expenditure; stable isotope

Indexed keywords

3 HYDROXYBUTYRIC ACID; CARBON DIOXIDE; COCARBOXYLASE; DICHLOROACETIC ACID; DIHYDROLIPOAMIDE DEHYDROGENASE; FATTY ACID; GLUCOSE; GLYCEROL; INSULIN; LACTIC ACID; MALIC ACID; PYRUVATE DEHYDROGENASE COMPLEX; PYRUVIC ACID;

ARTICLE; CALORIMETRY; CASE REPORT; DIET RESTRICTION; ENERGY YIELD; ENZYME ACTIVITY; EXON; FATIGUE; FEEDING DIFFICULTY; GENETIC ANALYSIS; GLUCONEOGENESIS; GLUCOSE BLOOD LEVEL; GLUCOSE INFUSION; GLUCOSE INTAKE; GLUCOSE OXIDATION; GLUCOSE TRANSPORT; HEART RATE; HUMAN; HUMAN TISSUE; INFANT; INSULIN BLOOD LEVEL; INSULIN RELEASE; INSULIN SENSITIVITY; KETOGENIC DIET; LACTATE BLOOD LEVEL; LIPOLYSIS; MALE; METABOLIC ACIDOSIS; MUSCLE BIOPSY; MUSCLE HYPOTONIA; OXYGEN CONSUMPTION; PALLOR; PYRUVATE DEHYDROGENASE COMPLEX DEFICIENCY; RESPIRATORY CHAIN; RESPIRATORY QUOTIENT; RESTING ENERGY EXPENDITURE; SEPTICEMIA; TACHYPNEA;

EID: 84964252320     PISSN: 0334018X     EISSN: 21910251     Source Type: Journal    
DOI: 10.1515/jpem-2013-0423     Document Type: Article

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