Betaine Hcl (betaine HCl)
Oral Tablet
Last reviewed · Source: First Databank (FDB) drug data
Overview
Betaine is a methyl group donor that functions in the normal metabolic cycle of methionine and reduces homocystinuria in patients with inborn errors of methionine metabolism.
Betaine, a methyl group donor that functions in the normal metabolic cycle of methionine, occurs naturally in the body as a metabolite of choline and is present in foods such as beets, spinach, cereals, and seafood. However, natural dietary sources provide betaine in amounts insufficient to be therapeutic in patients with homocystinuria.
In patients with inborn errors of methionine metabolism and resultant homocystinuria, oral administration of betaine at recommended pharmacologic dosages results in reduction of elevated plasma concentrations of homocysteine through the action of betaine functioning as a methyl group donor in the remethylation of homocysteine to methionine. Remethylation of homocysteine to methionine is catalyzed by betaine-homocysteine S-methyltransferase and does not require 5-methyltetrahydrofolate or methylcobalamin cofactors. Because this reaction does not depend on these cofactors, betaine is effective in all 3 primary forms of homocystinuria (i.e., cystathionine beta-synthase (CBS) deficiency, 5,10-methylenetetrahydrofolate reductase (MTHFR) deficiency, and cobalamin cofactor metabolism (cbl) defect).
Betaine also can increase low plasma methionine and S-adenosylmethionine (SAM) concentrations in patients with MTHFR deficiency or cbl defect. Elevations in plasma homocysteine concentrations in conditions other than homocystinuria also have been reported.
Betaine is commercially available as an anhydrous powder, and potency of the drug is expressed on the anhydrous basis.
SumMon(R) (see Users Guide). For additional information on this drug until a more detailed monograph is developed and published, the manufacturer's labeling should be consulted. It is essential that the labeling be consulted for detailed information on the usual cautions, precautions, and contraindications.
Generic name: betaine HCl
Therapeutic class: Alternative Therapy
Reference product & biosimilars
No reference, biosimilar or interchangeable associated with this drug.
Today’s intelligence on Betaine Hcl
Indications
No indications are available for this drug.
Dosing
Dosage of betaine is expressed in terms of anhydrous drug.
The usual dosage of betaine for the management of homocystinuria in adults and children is 6 g daily administered in 2 equally divided doses. Although children ranging in age from several months to years have been treated successfully and without unusual adverse effects with 6 g daily, dosage titration may be preferable in children. Children younger than 3 years of age may receive an initial betaine dosage of 100 mg/kg daily that is subsequently increased at weekly intervals in increments of 100 mg/kg daily.
All patients may receive gradual increases in betaine dosage until the concentration of homocysteine in plasma is undetectable or present only in small amounts. Some patients have required up to 20 g daily to control concentrations of homocystine in plasma.
Betaine is administered orally as a solution prepared from the anhydrous powder. The solution should be prepared immediately before administration by dissolving the appropriate amount of powder in 120-180 mL of water. The scoop provided by the manufacturer delivers 1 g of betaine anhydrous powder per level scoopful, which apportions 1.7 mL. The solution should not be administered if it is colored or the powder does not dissolve completely.
No dosing information is available for this tab.
No dosing information is available for this tab.
Drug interactions
There is no enhanced messaging for this drug.
There are no contraindication interactions for this drug.
There are no severe interactions for this drug.
There are no moderate interactions for this drug.
Contraindications
There is no enhanced messaging for this drug.
There are no contraindication contraindications for this drug.
- Cerebral edema
- Hypermethioninemia
There are no moderate contraindications for this drug.
Adverse reactions
There is no enhanced messaging for this drug.
There are no severe adverse reactions for this drug.
There are no less severe adverse reactions for this drug.
Precautions
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ICD codes
No ICD codes are available for this drug.
Formulary status
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Last reviewed · Source: First Databank (FDB) drug data
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