Betaine is an endogenous substance that converts homocysteine to methionine.

It is used to treat homocystinuria resulting from the genetic defect or deficiency of cystathionine beta-synthase, 5,10-methylene tetrahydrofolate reductase (MTHFR), and cobalamin cofactor metabolism (CBL). Betaine does not cure homocystinuria.

Betaine Dose in Adults

Dose in the treatment of Homocystinuria:

• 3 gms two times a day orally to a maximum dose of 20 gms/day
• The dose is gradually increased till the homocysteine levels are undetectable.

Betaine Dose in Childrens

Dose in the treatment of Homocystinuria:

• Infants and Children less than 3 years:
  • 50 mg/kg/dose two times a day orally
  • The dose is incrementally increased at weekly intervals by 50 mg/kg/day until the plasma levels are undetectable.
  • The maximum dose should not exceed 150 - 200 mg/kg/day.

• Children older than 3 years and Adolescents:
  • 3,000 mg two times a day orally.
  • The dose should be gradually increased until the plasma homocysteine levels are undetectable to a maximum dose of 20 gm/day.

Pregnancy Risk Factor: C

• Its use during pregnancy has not yet been investigated.
• Betaine is an endogenous compound, but it has been shown to have adverse effects in pregnant women.
• Patients should be treated before conception.

Betaine use during breastfeeding:

• Its use during breastfeeding is not well documented.
• It is important to weigh the risks and benefits of betaine during breastfeeding.

Betaine Dose in Renal Disease:

• Adjustment in the dose has not been recommended by the manufacturer in patients with renal disease.

Betaine Dose in Liver Disease:

• Adjustment in the dose has not been recommended by the manufacturer in patients with liver disease.

Side Effects of Betaine Frequency not defined.

• Gastrointestinal:
  • Diarrhea
  • Dysgeusia
  • Gastrointestinal Distress
  • Nausea

Contraindication to Betaine Include:

• The manufacturer has not listed any contraindications.

Warnings and Precautions

• Cystathionine beta–synthase deficiency (CBS).
  • Betaine treatment may increase plasma methionine levels, causing cerebral edema. Patients with CBS deficiencies should not use it.
• Use it in the right way:
  • Betaine anhydrous should not be mixed with betaine hydrochloride.

Monitor:

• Plasma homocysteine levels
• Monitor plasma methionine levels in patients with CBS deficiency.

How to administer Betaine?

Administer betaine without regard to meals orally.

Mechanism of action of Betaine:

• Betaine is an endogenous compound and a metabolite choline. It is a donor of methyl groups and converts homocysteine into methionine.

Homocystinuria refers to a genetic error in metabolism where plasma homocysteine levels rise. Homocysteine elevations can lead to mental retardation, ocular abnormalities and osteoporosis.

International Brands of Betaine:

• Betargin
• Cystadan
• Cystadane
• Reqmed

Betaine Brands in Pakistan:

No brands available in Pakistan