Fat and Carbohydrate Metabolism During Exercise in Phosphoglucomutase Type 1 Deficiency

Context: Phosphoglucomutase type 1 (PGM1) deficiency is a rare metabolic myopathy in which symptoms are provoked by exercise. Objective: Because the metabolic block is proximal to the entry of glucose into the glycolytic pathway, we hypothesized that iv glucose could improve the exercise intolerance...

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Veröffentlicht in:	The journal of clinical endocrinology and metabolism 2013-07, Vol.98 (7), p.E1235-E1240
Hauptverfasser:	Preisler, Nicolai, Laforêt, Pascal, Echaniz-Laguna, Andoni, Ørngreen, Mette C, Lonsdorfer-Wolf, Evelyne, Doutreleau, Stephane, Geny, Bernard, Stojkovic, Tanya, Piraud, Monique, Petit, François M, Vissing, John
Format:	Artikel
Sprache:	eng
Schlagworte:	Adult Bicycling Carbohydrate Metabolism Exercise Test Exercise Tolerance Glucose - administration & dosage Glucose - metabolism Glucose - therapeutic use Glycogen Storage Disease - blood Glycogen Storage Disease - metabolism Glycogen Storage Disease - physiopathology Glycogen Storage Disease - therapy Heart Rate Humans Hypoglycemia - etiology Hypoglycemia - prevention & control Infusions, Intravenous Lactic Acid - blood Lactic Acid - metabolism Lipid Metabolism Male Motor Activity Muscle, Skeletal - metabolism Muscular Diseases - etiology Muscular Diseases - physiopathology Oxygen Consumption Severity of Illness Index
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