Adult Dosing

Management of homocystinuria

• 6 g/day PO divided bid
• MAX: 20 g/day divided bid

• Measure the dose with the scoop provided (1.7 cc scoop = 1 g betaine anhydrous)
• Dissolve in 120-180 mL water/juice/milk/formula or mix with food for immediate ingestion
• All patients may receive gradual increases in betaine dosage until the concentration of homocysteine in plasma is undetectable or present only in small amounts

Pediatric Dosing

Management of homocystinuria

• <3yrs: Start 100 mg/kg/day PO divided bid; increase 50 mg/kg qwk. Max: 150 mg/kg/day
• >3 yrs: 6 g/day PO divided bid. Max: 20 g/day

• Measure the dose with the scoop provided (1.7 cc scoop = 1 g betaine anhydrous)
• Dissolve in 120-180 mL water/juice/milk/formula or mix with food for immediate ingestion
• All patients may receive gradual increases in betaine dosage until the concentration of homocysteine in plasma is undetectable or present only in small amounts

[Outline]

• Adult Dosing
• Pediatric Dosing

• Adjunctive treatment of homocystinuria, involving deficiencies or defects in:
• Cystathionine beta-synthase (CBS)
• 5,10-methylenetetrahydrofolate reductase (MTHFR)
• Cobalamin cofactor metabolism (cbl)

• Hypersensitivity to any of the ingredients of the product

• N/A

Renal Dose Adjustment

• Renal impairment: Dose adjustments not defined

Hepatic Dose Adjustment

• Hepatic impairment: Dose adjustments not defined

See Supplemental Patient Information

• Use of betaine should be limited to physicians knowledgable in treating patients with homocystinuria
• It has been administered concomitantly with vitamin B6 (pyridoxine), vitamin B₁₂ (cobalamin), and folate
• Cystadane can cause increase methionine concentrations in patients with cystathionine beta-synthase (CBS) deficiency. Monitor plasma concentrations and interrupt treatment if symptoms of cerebral edema. Desired levels <1000 umol/L
• Monitor plasma homocysteine levels. Levels decrease within a week of starting therapy and reach a steady state within a month

Cautions: Use with caution in

• Patients with CBS deficiency

Supplemental Patient Information

• Instruct patients and caregivers to take betaine as directed by their healthcare professional

Pregnancy Category:C

Breastfeeding: Unknown whether drug is excreted in human milk. Manufacturer advises caution.

• CNS: Cerebral edema
• GI: Nausea, gastrointestinal distress, diarrhea, bad taste, caused odor
• MISC: Hypermethioninemia

• Homocystinuria, Treatment Agent; acts as a methyl group donor in the remethylation of homocysteine to methionine in patients with homocystinuria
• Metabolism: Unknown
• Excretion: Unknown
• Half-life: Unknown

US Trade Name(s)

• Cystadane

US Availability

Cystadane

• PWDR: 1 g/1.7 cc scoop

Canadian Trade Name(s)

• Cystadane

Canadian Availability

Cystadane

• PWDR for SOLN: 1 g/1.7 cc scoop

UK Trade Name(s)

• Cystadane

UK Availability

Cystadane (betaine anhydrous)

• PWDR: 180 g

Australian Trade Name(s)

• Cystadane

Australian Availability

betaine trimethyglycenine (generic)

• PWDR: 100% (60 g)

Cystadane (betaine anhydrous)

• PWDR: 180 g

[Outline]

Endocrine/Metabolic

Metabolic Agents

Miscellaneous