A patient with a hereditary disorder of carnitine metabolism may be unable to transport long-chain fatty acids across membranes. A number of interventions may help reduce the impact of this metabolic defect.
Dietary management may include:
(1) avoidance of long-chain fatty acids in the diet
(2) inclusion of short and medium chain fatty acids in the diet (do not require carnitine to enter into mitochondria)
(3) diet high in carbohydrates
Some types of hereditary defects in carnitine metabolism may benefit from administration of carnitine, sometimes requiring high doses.
The patient should avoid anything that promotes fatty acid oxidation such as:
(1) prolonged fasting
(2) excessive exercise
(3) cold exposure
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