Drug Detail:Betaine (Betaine [ bet-aine ])
Drug Class: Nutraceutical products
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
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
- 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
- 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.
