J Child Neurol. 1995 Nov;10 Suppl 2:S45-51.

The role of L-carnitine in pediatric cardiomyopathy.

Winter S, Jue K, Prochazka J, Francis P, Hamilton W, Linn L, Helton E.

Source

Valley Children's Hospital, Fresno, CA. 93703, USA.

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 beta-oxidation, in the heart and lead to myocardial dysfunction. L-Carnitine, an essential element of beta-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.

PMID:: 8576569; [PubMed - indexed for MEDLINE]

Publication Types, MeSH Terms, Substances

Publication Types

Research Support, Non-U.S. Gov't

MeSH Terms

Substances

Carnitine

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Cardiomyopathy - MedlinePlus Health Information

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L-CARNITINE - HSDB

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Chemical compound information

Carnitine

MW: 161.19 g/mol

MF: C₇H₁₅NO₃

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