Primary Carnitine Deficiency

• PO (Adults ): Tablets: 990 mg 2–3 times daily. Oral solution: 1000 mg/day in divided doses (given every 3–4 hr throughout the day); titrate slowly as needed up to 3000 mg/day in divided doses (given every 3–4 hr throughout the day) based on tolerance and therapeutic response.
• PO (Children ): Tablets or solution: 50–100 mg/kg/day in divided doses (given 2–4 times daily); may titrate slowly as needed based on serum carnitine concentrations to 100–400 mg/kg/day (max dose = 3000 mg/day).

Secondary Carnitine Deficiency

• PO (Adults ): Tablets: 990 mg 2–3 times daily. Oral solution: 1000 mg/day in divided doses (given every 3–4 hr); titrate slowly as needed up to 3000 mg/day in divided doses (given every 3–4 hr) based on tolerance and therapeutic response.
• PO (Children ): Tablets or solution: 50–100 mg/kg/day in divided doses (given 2–4 times daily); may titrate slowly as needed based on serum carnitine concentrations to 100–400 mg/kg/day (max dose = 3000 mg/day).
• IV (Adults and Children ): 50 mg/kg/day in divided doses (given every 3–6 hr); subsequent doses of 50 mg/kg/day may be given if clinically indicated.

Carnitine Deficiency in Patients With End-Stage Renal Disease Requiring Hemodialysis.

• IV (Adults and Children ): 10–20 mg/kg (using dry body weight) after each dialysis session; guide dosage adjustments by trough (predialysis) levocarnitine concentrations. Evaluate clinical response every 3 mo and titrate to the lowest effective dose; therapy should be discontinued if no improvement is seen after 9–12 mo of therapy.

Acute Valproic Acid Overdose/Toxicity (Off Label)

• IV (Adults ): 100 mg/kg initially; then 50 mg/kg (max dose = 3,000 mg) every 8 hr until serum ammonia concentrations begin to ↓ and clinical improvement occurs.
• IV (Children ): No evidence of hepatotoxicity: 25 mg/kg every 6 hr (max dose = 3000 mg/day ) until serum ammonia and valproic acid concentrations begin to ↓ and clinical improvement occurs. Symptomatic hyperammonemia or hepatotoxicity: 100 mg/kg (max dose = 6000 mg) initially; then 15 mg/kg every 4–6 hr until serum ammonia concentrations begin to ↓ and clinical improvement occurs.