The Role of L-Carnitine in Pediatric Cardiomyopathy
| Autor: | Paul Francis, Edward D. Helton, Wade Hamilton, James V. Prochazka, Susan Winter, Kenneth L. Jue, Lawrence S. Linn |
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| Rok vydání: | 1995 |
| Předmět: |
Cardiac function curve
Pediatric cardiomyopathy business.industry Cardiomyopathy Bioinformatics medicine.disease Carnitine metabolism 03 medical and health sciences 0302 clinical medicine 030225 pediatrics Pediatrics Perinatology and Child Health medicine Neurology (clinical) Carnitine business Inner mitochondrial membrane 030217 neurology & neurosurgery medicine.drug |
| Zdroj: | Journal of Child Neurology. 10:2S45-2S51 |
| ISSN: | 1708-8283 0883-0738 |
| Popis: | 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). |
| Databáze: | OpenAIRE |
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