Fatty acid oxidation disorders and cardiomyopathy / 中国实用儿科杂志

ABSTRACT

myocardium. Fatty acid oxidation disorders(FAODs)can give rise to insufficiency of mitochondrial energy production and accumulation of metabolic intermediates in cardiomyocytes,such as lipid and long-chain acyl carnitine,leading to myocardial lesions.FAODs include carnitine-dependent fatty acid transport disorders and mitochondrial fatty acid beta-oxidation disorders,with any enzyme or transporter defect in fatty acid oxidation process resulting in this disease. Although FAODs are one of the rare causes of pediatric cardiomyopathy,some patients with FAODs are expected to have cardiomyopathy alleviated and quality of life improved by early correction of fatty acid metabolic disorders.