Carnitine deficiency
First line
Levocarnitine is the first-line drug for primary systemic carnitine deficiency (OCTN2 transporter genetic defect) and secondary deficiency on long-term haemodialysis, valproate or pivampicillin therapy, and in preterm infants. The approved Carnitor for this indication in 1985. AEMPS includes levocarnitine in the standard for metabolic disorders of fatty-acid oxidation.