title: The role of L-carnitine in pediatric cardiomyopathy.
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.
authors.affiliation: Valley Children's Hospital, Fresno, CA. 93703, USA.
authors.forename: S
authors.initials: S
authors.lastname: Winter
authors.forename: K
authors.initials: K
authors.lastname: Jue
authors.forename: J
authors.initials: J
authors.lastname: Prochazka
authors.forename: P
authors.initials: P
authors.lastname: Francis
authors.forename: W
authors.initials: W
authors.lastname: Hamilton
authors.forename: L
authors.initials: L
authors.lastname: Linn
authors.forename: E
authors.initials: E
authors.lastname: Helton
mesh_terms: D009202:Cardiomyopathies / Q000097:blood / Q000378:metabolism*; D002331:Carnitine / Q000097:blood / Q000378:metabolism*; D002648:Child; D002675:Child, Preschool; D005260:Female; D006801:Humans; D007223:Infant; D008297:Male; D012189:Retrospective Studies; D014804:Vitamin B Deficiency / Q000097:blood
chemical_list: D002331:Carnitine