Betaine Dosage

Medically reviewed by Drugs.com. Last updated on Nov 13, 2023.

Usual Adult Dose for Hyperhomocysteinemia

Initial dose: 3 grams orally twice a day

• Increase the dose gradually until plasma total homocysteine is undetectable or only present in small amounts

Maximum dose: Some patients have required 20 grams/day

Comments:

• One in vitro pharmacokinetic/pharmacodynamic study showed minimal benefit from more than twice daily dosing and 150 mg/kg/day betaine.
• An initial plasma homocysteine response takes several days; steady state is reached within a month.

Uses: To decreases elevated blood homocysteine levels in the treatment of homocystinuria from:

• Cystathionine beta-synthase (CBS) deficiency
• 5,10-methylenetetrahydrofolate reductase (MTHFR) deficiency

or

• Cobalamin cofactor metabolism (cbl) defect

Usual Pediatric Dose for Hyperhomocysteinemia

3 years and older:
Initial dose: 3 grams orally twice a day

• Increase the dose gradually until plasma total homocysteine is undetectable or only present in small amounts

Under 3 years:
Initial dose: 100 mg/kg/day divided into 2 daily doses

• Increase weekly by 50 mg/kg increments until plasma total homocysteine is undetectable or only present in small amounts

Comments:

• Some patients have required 20 grams/day.
• One in vitro pharmacokinetic/pharmacodynamic study showed minimal benefit from more than twice daily dosing and 150 mg/kg/day betaine.
• An initial plasma homocysteine response takes several days; steady state is reached within a month.

Uses: To decreases elevated blood homocysteine levels in the treatment of homocystinuria from:

• Cystathionine beta-synthase (CBS) deficiency
• 5,10-methylenetetrahydrofolate reductase (MTHFR) deficiency

or

• Cobalamin cofactor metabolism (cbl) defect

Renal Dose Adjustments

Data not available

Liver Dose Adjustments

Data not available

Precautions

CONTRAINDICATIONS: None

Consult WARNINGS section for additional precautions.

Dialysis

Data not available

Other Comments

Storage requirements:

• Store at room temperature; protect from moisture.

Reconstitution/preparation techniques:

• Shake bottle lightly before removing cap.
• Measure the dose using the provided scoop; 1 level scoop (1.7 mL) equals 1 gram betaine.
• Mix with 4 to 6 ounces water, juice, milk, or formula until completely dissolved, or mix with food, ingest immediately.
• Replace cap tightly.

Monitoring:

• Monitor plasma homocysteine concentrations for patient response to therapy.
• Monitor plasma methionine in cystathionine beta-synthase (CBS) deficiency.

Further information

Always consult your healthcare provider to ensure the information displayed on this page applies to your personal circumstances.

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