What is the role of medications in carnitine deficiency treatment?

Updated: Dec 13, 2019
  • Author: Fernando Scaglia, MD, FACMG; Chief Editor: Maria Descartes, MD  more...
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Answer

Use of L-carnitine in primary carnitine deficiency restores plasma carnitine levels to nearly normal, but muscle carnitine levels rise slightly. Muscle function can be normalized in patients with carnitine deficiency when muscle carnitine levels remain less than 10% of controls. Cardiomyopathy often responds well to carnitine supplementation. Carnitine supplementation in fatty acid oxidation disorders and other organic acidurias is to correct carnitine deficiency and to allow removal of toxic intermediates. The other goal of therapy is to restore CoA levels. Carnitine therapy for long-chain fatty acid oxidation defects has become questionable because it promotes formation of long-chain acylcarnitines that may cause arrhythmogenesis and membrane dysfunction. Carnitine supplementation in total parenteral nutrition (TPN) prevents secondary carnitine deficiency in preterm newborns.


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