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.
Hypermethioninemia. Monitor plasma methionine concentrations in patients with CBS deficiency. Pregnancy. Nursing mothers.
Nausea, GI distress; hypermethioninemia, cerebral edema.
Generic Drug Availability:
Bottle—180g (w. measuring scoop)