• Inborn errors of metabolism

Cystadane Generic Name & Formulations

General Description

Betaine anhydrous 1g; per scoopful; pwd for oral soln after reconstitution.

Pharmacological Class

Methylating agent.

How Supplied

Bottle—180g (w. measuring scoop)

Generic Availability


Cystadane Indications


Treatment of homocystinuria (eg, cystathionine beta-synthase [CBS] deficiency, 5,10-methylenetetrahydrofolate reductase [MTHFR] deficiency, cobalamin cofactor metabolism [cbl] defect) to decrease elevated homocysteine blood levels.

Cystadane Dosage and Administration

Adults and Children

Dissolve one scoop (1g betaine anhydrous pwd) in 4–6 ounces of water, juice, milk, formula, or mixed with food for immediate ingestion. <3yrs: initially 100mg/kg/day divided in twice daily doses, may increase weekly by 50mg/kg increments. ≥3yrs: 3g twice daily. Increase gradually until plasma total homocysteine is undetectable or present only in small amounts. Up to 20g/day has been used.

Cystadane Contraindications

Not Applicable

Cystadane Boxed Warnings

Not Applicable

Cystadane Warnings/Precautions


Hypermethioninemia. Monitor plasma methionine concentrations in patients with CBS deficiency. Pregnancy. Nursing mothers.

Cystadane Pharmacokinetics

See Literature

Cystadane Interactions

Cystadane Adverse Reactions

Adverse Reactions

Nausea, GI distress; hypermethioninemia, cerebral edema.

Cystadane Clinical Trials

See Literature

Cystadane Note

Not Applicable

Cystadane Patient Counseling

See Literature