Carnitine Deficiency

Rare (1:40,000 in Japan)

Hypoglycemia triggered by fasting

Massive rhabdomyolysis and cardiac arrest described following GA and succinylcholine. (The response may be confused with malignant hyperthermia.)

No evidence that susceptibility to malignant hyperthermia is associated with the carnitine palmitoyltransferase enzyme system

Periop hypoglycemia: Avoid prolonged fasting; IV glucose should be administered

Neurologic and cardiopulmonary status: Determine if a cardiomyopathy is present

CACT and CPT2 are essential cofactors in enzymatic transport of long-chain fatty acids into mitochondria, in which they are oxidized.

When carnitine is deficient, peripheral tissues cannot use fatty acids for energy production, and the liver cannot adequately make ketone bodies as an alternative substrate.

The tissues become glucose dependent, and their metabolism exceeds the liver’s capacity for glucose production.

This glucose dependency can lead to severe liver failure (increased hepatic enzymes, lactic acidosis, and encephalopathy) and hypoketotic hypoglycemia.