A naturally occurring substance called levocarnitine transports long-chain fatty acids in the mitochondria, which leads to the creation of energy.  It is recommended for the following conditions:

• Carnitine deficit in patients with end-stage renal disease who need hemodialysis can be treated and  prevented with certain supplements (intravenous formulation only) as a dietary addition.
• Primary systemic carnitine insufficiency therapy (oral formulation only).
• For the emergency and ongoing management of individuals with secondary
• Carnitine insufficiency brought on by inborn metabolic defects (both oral and intravenous formulations)

Off Label Use of Levocarnitine in Adults include:

• Patients with valproic acid toxicity or overdose resulting in liver failure, elevated ammonia levels, and coma

Risk factors for Carnitine deficiency include:

• Chronic valproic acid therapy
• Young age as in infants and young children who may be lacking the carnitine activating enzyme 
• Neurologic disorders
• Cirrhosis
• Metabolic disorders
• Critical care patients including burns, sepsis, trauma, and those with organ failure
• Renal failure
• Use of multiple antiepileptic drugs and other chemotherapeutic drugs & antinucleoside analogs

Levocarnitine Dose in Adult:

Levocarnitine dose in the treatment of primary or secondary Carnitine deficiency:

• Oral solution of levocarnitine:
  • 1,000 mg/day initially in divided doses administered every 3 to 4 hours.
  • Titrate the dose slowly, based on tolerance and therapeutic response to 1,000 to 3,000 mg/day in divided doses.
• Levocarnitine Tablets:
  • 990 mg twice or thrice daily based on the clinical response.

Levocarnitine dose for treating secondary Carnitine deficiency:

• To a maximum dose of 300 mg/kg/day, 50 mg/kg/day intravenous in divided doses is given every 3 or 4 hours.
• Patients who are experiencing a severe metabolic crisis may get a loading dosage of 50 mg/kg, followed by another 50 mg/kg over the course of  the next 24 hours in divided doses.

Dose in patients with ESRD requiring dialysis with Carnitine deficiency:

• 10–20 mg/kg intravenously given following each dialysate session
• Subsequent dosage should be guided by trough levels of levocarnitine
• The clinical response should be evaluated at 3-months intervals and the lowest effective dose should be used. If no improvement is observed after 9 to 12 months of therapy, therapy should be discontinued.

Note: Levocarnitine use is not routinely indicated in dialysis patients. It may be indicated in patients who are hyporesponsive to erythropoietin based therapy, hypotension during dialysis, Symptomatic cardiomyopathy, fatigue or muscle weakness that is unresponsive to normal medication, heart failure (NYHA functional class III or IV).

Off label use in the treatment of acute Valproic acid toxicity:

• Following a 100 mg/kg intravenous IV bolus, 50 mg/kg up to a maximum dose of 3,000 mg were given as an intermittent infusion over 15 to 30  minutes, three times each day.
• It should be continued until clinical improvement is noted or objective evidence of decreasing ammonia is documented.

Levocarnitine Dose in children:

Levocarnitine dose in the treatment of Carnitine deficiency:

• Infants, Children, and Adolescents:
  • Primary carnitine deficiency:
    • 50 mg/kg/day orally in split doses, up to a target dose of 100 mg/kg/day (and up to a maximum of 400 mg/kg/day), to a maximum cumulative daily dose of 3,000 mg/day (if tablets are given twice or three times daily; every 3 to 4 hours  if an oral solution is used).
  • Secondary carnitine deficiency:
    • 50 mg/kg/day orally in divided doses (twice or thrice daily if tablets are given and every 3 - 4 hours if an oral solution is used) initially to the target dose of 100 mg/kg/day (and up to a maximum of 300 mg/kg/day) to a maximum cumulative daily dose of 3,000 mg/day
  • Intravenous formulation:
    • •  
      • 50 mg/kg intravenous as a single dose. A repeat loading dose 50 mg/kg in severe crisis, followed by the maintenance dose divided every 3 - 6 hours to a maximum daily dose of 300 mg/kg/day.

Use in the treatment of ESRD patients on hemodialysis:

• Children and Adults:
  • 10 to 20 mg/kg intravenous after each dialysis session.
  • The clinical response should be evaluated at 3-months intervals and the lowest effective dose should be used. If no improvement is observed after 9 to 12 months of therapy, therapy should be discontinued.

Note: Levocarnitine use is not routinely indicated in dialysis patients. It may be indicated in patients who are hyporesponsive to erythropoietin based therapy, hypotension during dialysis, Heart failure, symptomatic cardiomyopathy, muscle weakness, or fatigue not responsive to standard therapy.

Use in the treatment of acute Valproic acid toxicity:

• Children and Adolescents:
  • No hepatotoxicity:
    • 100 mg/kg/day intravenous in four divided doses until clinical improvement is observed or the serum ammonia and valproic acid concentrations begin to decrease
    • The maximum daily dose should not exceed 3,000 mg/day.
  • Symptomatic hepatotoxicity or hyperammonemia:
    • Intravenous Loading dose:
      • 100 mg/kg to a maximum loading dose of 6 gms followed by 50 mg/kg/dose thrice daily to a maximum dose of 3,000 mg/dose or
      • Until clinical improvement is noticed or the serum ammonia levels start to drop, 15 mg/kg/dose given every 4 hours.

Use in the prevention of Cyclic vomiting syndrome:

• Children and Adults:
  • 50 - 100 mg/kg/day orally 2 or 3 divided doses to a maximum daily dose of 1,000 mg/dose.

Pregnancy Risk Factor B

• It is a naturally occurring substance. It has not been shown to have teratogenic properties in animal studies.

Levocarnitine use during breastfeeding:

• When breastfeeding, be careful. Consider the risks and benefits for both mother and baby.

Levocarnitine Dose in Renal:

• Patients with renal insufficiency have not had oral carnitine's safety and effectiveness assessed.
• Additionally, long-term treatment in patients with substantially impaired renal function may cause the buildup of potentially harmful metabolites.
• The manufacturer has not recommended any dose adjustment on patients on intravenous therapy, however, the dose should be adjusted based on serum carnitine levels and clinical response.

Note: Levocarnitine use is not routinely indicated in dialysis patients. It may be indicated in patients who are hyporesponsive to erythropoietin based therapy, hypotension during dialysis, Heart failure (NYHA functional class III or IV), symptomatic cardiomyopathy, muscle weakness, or fatigue not responsive to standard therapy.

Levocarnitine Dose in liver disease:

• The manufacturer has not recommended any dose adjustment in patients with liver disease.

Common Side Effects Of Levocarnitine Include:

• Cardiovascular:
  • Chest pain
  • Hypertension
• Central nervous system:
  • Dizziness
  • Headache
  • Paresthesia
• Endocrine & metabolic:
  • Hypercalcemia
• Gastrointestinal:
  • Vomiting
  • Diarrhea
  • Abdominal pain
  • Nausea
• Hematologic & oncologic:
  • Anemia
• Infection:
  • Infection
• Neuromuscular & skeletal:
  • Weakness
• Respiratory:
  • Cough
  • Rhinitis
• Miscellaneous:
  • Accidental injury
  • Fever

Less Common Side Effects Of Levocarnitine Include:

• Cardiovascular:
  • Tachycardia
  • Palpitations
  • Vascular disease
  • Peripheral edema
  • ECG abnormality
  • Atrial fibrillation
• Central nervous system:
  • Drug dependence
  • Vertigo
  • Depression
• Dermatologic:
  • Skin rash
• Endocrine & metabolic:
  • Weight loss
  • Parathyroid disorder
  • Weight gain
• Gastrointestinal:
  • Dysgeusia
  • Melena
  • Anorexia
  • Gastrointestinal disorder
• Hematologic & oncologic:
  • Hemorrhage
• Hypersensitivity:
  • Hypersensitivity reaction
• Ophthalmic:
  • Amblyopia
  • Eye disease
• Renal:
  • Renal failure
• Respiratory:
  • Bronchitis

• Gastrointestinal:
  • Gastritis (intravenous)
• Miscellaneous:
  • Body odor

Contraindication to Levocarnitine include:

Allergy reactions to levocarnitine and any component of the formulation

Warnings & Precautions

• Gastrointestinal effects:
  • Side effects may occur if oral carnitine is consumed too quickly.
• Hypersensitivity reactions
  • After oral administration, allergic reactions such as rash, urticaria and facial edema were reported. Patients on dialysis have reported anaphylaxis, laryngeal swelling, and bronchospasm after intravenous administration.
  • End-stage renal disease patients may experience reactions to IV use within minutes.
  • After severe allergic reactions, it is best to stop taking any medication.
• Renal impairment
  • Patients with renal impairment have not been able to evaluate the safety and efficacy oral carnitine.
  • Chronic therapy for severely impaired renal function may lead to the accumulation of potentially toxic substances.
• Seizure disorder:
  • Patients suffering from a seizure disorder and neurologic diseases should be cautious when taking the drug.

Carnitine supplements (Levocarnitine): Drug Interaction

Note: Drug Interaction Categories:

• Risk Factor C: Monitor When Using Combination
• Risk Factor D: Consider Treatment Modification
• Risk Factor X: Avoid Concomitant Use

Monitoring parameters:

• Monitor serum Carnitine levels at baseline, weekly, and monthly thereafter in patients on intravenous therapy.
• Monitor blood chemistry, vital signs, and plasma carnitine levels (maintain levocarnitine levels between 35 to 60 μmol/L).
• In ESRD patients on dialysis, if clinical improvement is not noted after 9 - 12 months of therapy, it should be discontinued.

Levocarnitine in Valproic acid toxicity:

• Every 4 to 6 hours, check valproic acid levels until a declining trend is seen.
• Keep track of your body's electrolytes, blood gases, mental state, liver function, serum lactate levels, ammonia concentrations,  and platelet counts.

How to administer Levocarnitine?

Administering Oral solution:

• Levocarnitine oral solution can be taken either on its own or combined with liquid food or drink.
• After meals, it should be consumed carefully, in small dosages, every three to four hours throughout the day.

Intravenous administration:

• Administer the drug via an infusion or as a bolus dose over 2 to 3 minutes.
• It should be administered every 3 or 4 hours, but never less than every 6 hours.

Administration in patients on Hemodialysis:

• After each dialysis session, administer as a bolus dosage over 2 to 3 minutes into the venous return line.

Mechanism of action of Levocarnitine:

• Carnitine, a naturally occurring compound, acts as a carrier molecule to transport long-chain fatty acid within the mitochondria.
• This facilitates the production of energy. Carnitine deficiency can lead to excess acyl CoA ester accumulation and disruption of intermediary metabolism.

It doesn't bind to proteins, and it is notMetabolizedTrimethylamine N-oxide (TMAO) and Trimethylamine Acetate (TMA), are produced by the liver. It also has an oral component.bioavailability15% and moreEliminating half-life17.4 hours The time it took topeak plasma concentrationIt takes approximately 3.3 hours. It isexcretedPrimarily via urine.

International brands of Levocarnitine:

• Carnitor
• Carnitor SF
• G-levOCARNitine S/F
• McCarnitine
• Abedine
• Agvantar
• Albicar
• Bio-Carnitine
• Biocarn
• Branigen
• Cardispan
• Carnicor
• Carnil
• Carnin
• Carnitene
• Carnitina
• Carnitine
• Carnitol
• Carnitor
• Carnivit
• Carnivita
• Carry
• FA
• Cartan
• Cartin
• Cartine
• Chudex
• Disocor
• Efe-Carn
• Elcarnitol
• Entomin
• Fitmax L-Karnityna
• Hardbody L-Karnityna
• LCadul
• L-Carn
• L-Carnitin
• L-Cartin FF
• L-Karnityna
• Lecarna
• Levocarnil
• Levohistam
• Monocamin
• Nefrocarnit
• Neurex
• Ocarnix
• Secabiol
• Trian

Levocarnitine Brands in pakistan:

Carneel (Acetyl L-carnitine) - High Q Pharma