Metabolic changes in patients with mitochondrial myopathies and effects of coenzyme Q10 therapy |
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Authors: | Andrew Chan H Reichmann Anke Kögel Andreas Beck R Gold |
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Institution: | Neurologische Universit?tsklinik, Josef-Schneider-Strasse 11, D-97080 Würzburg Tel.: +49-931-2015755 Fax: +49-931-2013488,
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Abstract: | We used a standardized bicycle ergometry protocol with a stepwise increasing workload (30–100 W) to evaluate various metabolic
factors for the diagnosis and metabolic monitoring of mitochondrial encephalomyopathies. All patients (n = 9) showed pathological venous lactate/pyruvate (L/P) ratios, which normalized in three patients after 6 months of coenzyme
Q10 (CoQ) therapy. Thus, the L/P ratio proved to be the clinically most useful parameter in the evaluation and monitoring of
mitochondrial diseases, showing higher sensitivity than lactate measurements only. CoQ may exert a favourable effect in
some patients with mitochondrial diseases.
Received: 15 October 1997 Received in revised form: 6 February 1998 Accepted: 20 March 1998 |
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Keywords: | Bicycle ergometry Coenzyme Q10 Lactate Lactate/ pyruvate ratio Mitochondrial encephalomyopathies |
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