Efficiency of oxidative work performance of skeletal muscle in patients with cystic fibrosis

• Published in: Thorax Vol. 50; no. 9; pp. 980 - 983
• Main Authors: de Meer, K, Jeneson, J A, Gulmans, V A, van der Laag, J, Berger, R
• Format: Journal Article
• Language: English
• Published: London BMJ 01.09.1995; BMJ Publishing Group LTD
• Subjects: Adolescent; Biological and medical sciences; Case-Control Studies; Child; Cystic Fibrosis - metabolism; Female; Gastroenterology. Liver. Pancreas. Abdomen; Humans; Liver. Biliary tract. Portal circulation. Exocrine pancreas; Magnetic Resonance Spectroscopy; Male; Medical sciences; Mitochondria, Muscle - metabolism; Muscle, Skeletal - metabolism; Other diseases. Semiology; Oxidative Phosphorylation; Physical exercise; Human; Respiratory disease; Nervous system; Exploration; Cystic fibrosis; Striated muscle; Metabolism; Genetic disease; Mitochondria; Oxidative phosphorylation; Digestive diseases; Oxidation
• ISSN: 0040-6376; 1468-3296
• DOI: 10.1136/thx.50.9.980

BACKGROUND--Exercise intolerance in patients with cystic fibrosis is commonly attributed to reduced pulmonary and nutritional status. The possible role of diminished efficiency of mitochondrial oxidative phosphorylation in relation to skeletal muscle performance was investigated in patients with cystic fibrosis. METHODS--In vivo synthesis of ATP in skeletal muscle during submaximal exercise was studied in eight patients with cystic fibrosis aged 12-17 years, and in 19 healthy control subjects aged 8-36 years. The intracellular pH and concentrations of phosphate compounds were calculated at four steady states from phosphorus-31 labelled nuclear magnetic resonance spectroscopy measurements in the forearm muscle during bulb squeezing in an exercise protocol. Normalised power output, expressed as percentage maximal voluntary contraction (Y, in %MVC), was related to the energy force of ATP hydrolysis (X = ln [ATP]/[ADP][Pi]). This relationship provides an in vivo measure of efficiency of oxidative work performance of skeletal muscle. RESULTS--During all workloads (but not at rest) intracellular pH was higher in the patients with cystic fibrosis than in the controls. The linear least square fit for Y = a-bX showed high correlations in both groups; the slope b was 19% lower in the patients than in the controls (11.8% v 14.5% MVC/ln M; 95% confidence interval for difference 0.3 to 5.0). CONCLUSIONS--In patients with cystic fibrosis oxidative work performance of skeletal muscle is reduced. This may be related to secondary pathophysiological changes in skeletal muscle in cystic fibrosis.