Inborn errors of metabolism:

Indications for CYSTADANE:

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.

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.

Warnings/Precautions:

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

Pharmacologic Class:

Methylating agent.

Adverse Reactions:

Nausea, GI distress; hypermethioninemia, cerebral edema.

How Supplied:

Bottle—180g (w. measuring scoop)