Which dietary modifications are used in the treatment of carnitine deficiency?

Updated: Dec 13, 2019
  • Author: Fernando Scaglia, MD, FACMG; Chief Editor: Maria Descartes, MD  more...
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Patients with primary carnitine deficiency requires no special diet as long as they are taking carnitine supplementation and are not faced with situations of stress and starvation. Actively avoid periods of fasting in these patients.

Patients with fatty acid oxidation disorders require a high-carbohydrate fat-restricted diet (30% calories from fat) and must eat frequently.

Prescribe medium-chain triglyceride supplementation in patients with long-chain fatty acid disorders.

Advise use of uncooked cornstarch at bedtime to prevent early morning hypoglycemia after the overnight fast.

Supplementation of essential fatty acids (ie, linoleic acids, linolenic acids) prevents the growth restriction and dermatitis that are associated with fatty acid deficiency.

Consider specific protein-restricted diets in patients with aminoacidopathies and organic acidemias associated with secondary carnitine deficiency.

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