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Homocystinuria (HCU) is an inherited disorder of amino acid metabolism, caused by an enzymatic defect in a metabolic pathway that leads to increased levels of methionine.
Individuals with homocystinuria are treated with the drug betaine (Cystadane). This is a powder dissolved in water, juice, or milk and drunk usually twice a day with meals. This drug is not normally used simply to lower high levels of homocysteine in the absence of congenital disease.