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Dear Readers! I started this blog when my daughter was in the NICU. She had ventriculitis. Her CSF report grew E.coli. But, she was injected Teicoplanin directly into her brain. The doctors made two errors here:

Teicoplanin is for gram-positive bacteria only. It does not treat E.coli.
Teicoplanin is not for intraventricular use. The manufacturer strongly discourage injecting Teicoplanin into the brain.

My daughter bled into the brain. She lived for another two years and ultimately died.

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Betaine for the treatment of homocystinuria

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.