[Carnitine: physiologic role, primary and secondary deficiency].

L-carnitine is essential in fatty acids metabolism, it is synthetized endogenically and supplied by nurishment as well. Ninety eight percent of it is stored in skeletal muscles. Its importance in primary deficiency, either systemic encephalomyopathies or isolated myopathies, is out of discussion. In deficiency states, life itself may be threatened. Numerous investigations point to secondary carnitine deficiencies. Favorable substitution effects are published, first of all, in organic acidurias in children, in ischemic heart diseases of adults, in hyperlipoproteinemias, in peripheral vascular disease, in patients on hemodialysis, and in muscle dystrophy. Favorable results are published also in total parenteral nourishment of newborns and adults. L-carnitine may prevent toxic effects of valoproates and antracyclins. The substance is not toxic and is well tolerated. LD50 values are almost like those of amino acids. Meanwhile, inspite of many reports on favorable results in secondary carnitne deficiencies the additional controlled clinical trials should be done, especially in children.