betaine anhydrous at a glance
Medicine class
Amino-acid derivative and methyl donor
Usual role
Additional treatment for inherited homocystinuria
How it is given
Oral powder dissolved in water, juice, milk, formula or food and taken immediately
Monitoring
Blood homocysteine and methionine measurements guide treatment
What is betaine anhydrous used for?
Betaine anhydrous is licensed as an adjunct to other treatment for homocystinuria caused by:
Cystathionine beta-synthase deficiency
Methylenetetrahydrofolate reductase deficiency
Defects of cobalamin cofactor metabolism
How does betaine anhydrous work?
Betaine donates a methyl group that helps convert homocysteine back into methionine. This lowers blood homocysteine but can raise methionine, particularly in CBS deficiency, so both require monitoring.
How to take betaine anhydrous
Use the supplied measuring spoon specified by the prescriber. Prepare each measured amount immediately before taking it.
Lightly shake the closed bottle before opening.
Level the filled measuring spoon with a flat edge as described in the leaflet.
Mix the powder with water, juice, milk, formula or food until completely dissolved.
Take the mixture immediately after preparation.
betaine anhydrous side effects
Common or expected effects
- Raised blood methionine is very common and is detected through blood tests.
- Uncommon effects include nausea, vomiting, diarrhoea, stomach discomfort, tongue inflammation or reduced appetite.
- Other uncommon effects include agitation, irritability, hair loss, hives, altered skin odour and urinary incontinence.
Get urgent medical advice
- Morning headache with vomiting or visual changes may indicate cerebral oedema and needs immediate medical assessment.
betaine anhydrous in pregnancy
Experience during pregnancy is limited. Betaine anhydrous should be used only when considered clearly necessary. Contact the metabolic team early when pregnant or planning pregnancy because close biochemical, dietary and obstetric monitoring may be needed.
betaine anhydrous while breastfeeding
It is not known whether betaine anhydrous passes into human milk. A specialist should assess treatment and breastfeeding together because evidence is limited.
betaine anhydrous interactions
Formal interaction studies have not been performed. Laboratory data identify potential interactions with the following:
Amino-acid mixtures
Administration may need to be separated as directed by the specialist team.
Vigabatrin
Administration may need to be separated as directed by the specialist team.
GABA analogues
Administration may need to be separated as directed by the specialist team.
Common questions about betaine anhydrous
Answers are fully visible for fast scanning and source review.
Why are blood tests needed?
Tests show whether homocysteine is responding and detect excessive methionine, which can cause serious complications.
Can the powder be mixed with food or drink?
Yes. It can be dissolved in water, juice, milk, formula or food and should be taken immediately after mixing.
Which symptoms need immediate assessment?
Sources
These sources were used to prepare and review this medicine page.
Prepared and reviewed by the iatroX editorial team.