Uses of Carnoact Plus (Acetyl-L-Carnitine)
Carnoact Plus is primarily indicated for the treatment of primary systemic carnitine deficiency and secondary carnitine deficiency resulting from inborn errors of metabolism. 1
Primary Indications
Primary systemic carnitine deficiency: Characterized by:
- Recurrent episodes of Reye-like encephalopathy
- Hypoketotic hypoglycemia
- Cardiomyopathy
- Associated symptoms: hypotonia, muscle weakness, failure to thrive 1
Secondary carnitine deficiency: Resulting from inborn errors of metabolism, including:
- Glutaric aciduria II
- Methyl malonic aciduria
- Propionic acidemia
- Medium chain fatty acylCoA dehydrogenase deficiency 1
Mechanism of Action
L-carnitine facilitates long-chain fatty acid entry into cellular mitochondria, delivering substrate for oxidation and subsequent energy production. It promotes the excretion of excess organic or fatty acids in patients with defects in fatty acid metabolism and/or specific organic acidopathies that bioaccumulate acylCoA esters. 1, 2
Specific Clinical Applications
Dialysis Patients
- May be considered in selected dialysis patients with documented carnitine deficiency for:
- Erythropoietin-resistant anemia
- Intradialytic muscle cramps
- Hypotension during dialysis
- Malaise and asthenia
- Muscle weakness 3
Liver Disease
- May be associated with:
- Lowering of blood ammonia levels
- Lower rates of muscle loss
- Reversal of existing sarcopenia
- Increased physical activity 3
Premature Infants
- Especially indicated in premature infants on total parenteral nutrition for more than 4 weeks
- May be recommended for neonates with metabolic acidosis and elevated lactate levels 3
Dosage Considerations
- Hemodialysis patients: 20 mg/kg IV after each hemodialysis session or 2-4g orally daily in divided doses 3
- Neonates: 20-30 mg/kg/day, increasing to 50-100 mg/kg/day in severe cases 3
- General supplementation: 2-5 mg/kg/day, aligning with physiological requirements 3
Important Clinical Considerations
- Diagnosis of primary carnitine deficiency requires that serum, red cell and/or tissue carnitine levels be low and that the patient does not have a primary defect in fatty acid or organic acid oxidation 1
- Carnitine deficiency is confirmed by an acyl-to-free carnitine ratio >0.4 (normal ratio ≤0.25) and total serum carnitine <40 μmol/L 3
- The bioavailability of pharmacological doses of L-carnitine (1-6g) is only 5-18%, whereas dietary L-carnitine may have bioavailability as high as 75% 2
Monitoring and Side Effects
- Monitor clinical symptoms, hemoglobin/hematocrit if treating anemia, and plasma carnitine levels (free and total) 3
- High-dose L-carnitine therapy (≥3g/day) may cause:
- Gastrointestinal symptoms (nausea, vomiting, abdominal cramps, diarrhea)
- "Uremic breath" due to accumulation of trimethylamine metabolites 3