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- W2907723480 abstract "Metabolic and genetic factors underlie some forms of cardiomyopathy in childhood. A variety of inborn errors of metabolism can impair mitochondrial energy production, or β-oxidation, in the heart and lead to myocardial dysfunction. L-Carnitine, an essential element of β-oxidation, transports fatty acids across the mitochondrial membrane for energy production. L-Carnitine deficiency syndromes are now well described as secondary to a variety of inborn errors of metabolism and often include cardiomyopathy in the clinical picture. Despite traditional therapies for cardiomyopathy, mortality for this disorder remains at well over 50%. Review of reports of L-carnitine supplementation studies and results from our own trial underscore the importance of its role in cardiac function and demonstrates that there is likely a subpopulation of patients with cardiomyopathy responsive to L-carnitine treatment. (J Child Neurol 1995; 10(Suppl):2S45-2S5 1)." @default.
- W2907723480 created "2019-01-11" @default.
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- W2907723480 date "1995-11-01" @default.
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- W2907723480 title "The Role of L-Carnitine in Pediatric Cardiomyopathy" @default.
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- W2907723480 doi "https://doi.org/10.1177/0883073895010002s07" @default.
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