Impaired cardiac reserve and severely diminished skeletal muscle O₂ utilization mediate exercise intolerance in Barth syndrome

Barth syndrome (BTHS) is a mitochondrial myopathy characterized by reports of exercise intolerance. We sought to determine if 1) BTHS leads to abnormalities of skeletal muscle O(2) extraction/utilization and 2) exercise intolerance in BTHS is related to impaired O(2) extraction/utilization, impaired...

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Veröffentlicht in:	American journal of physiology. Heart and circulatory physiology 2011-11, Vol.301 (5), p.H2122-H2129
Hauptverfasser:	Spencer, Carolyn T, Byrne, Barry J, Bryant, Randall M, Margossian, Renee, Maisenbacher, Melissa, Breitenger, Petar, Benni, Paul B, Redfearn, Sharon, Marcus, Edward, Cade, W Todd
Format:	Artikel
Sprache:	eng ; jpn
Schlagworte:	Adolescent Adrenergic beta-Antagonists - therapeutic use Analysis of Variance Barth Syndrome - complications Barth Syndrome - diagnosis Barth Syndrome - metabolism Barth Syndrome - physiopathology Biomarkers - blood Blood Pressure Cardiomyopathy, Dilated - diagnosis Cardiomyopathy, Dilated - drug therapy Cardiomyopathy, Dilated - etiology Cardiomyopathy, Dilated - metabolism Cardiomyopathy, Dilated - physiopathology Case-Control Studies Child Cross-Sectional Studies Echocardiography Electrocardiography Exercise Test Exercise Tolerance Heart Rate Hemoglobins - metabolism Humans Male Muscle Contraction Oxygen - blood Oxygen - metabolism Oxygen Consumption Quadriceps Muscle - metabolism Quadriceps Muscle - physiopathology Respiratory Mechanics Spectroscopy, Near-Infrared Ventricular Function, Left Young Adult
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