Drug reference
Adjunctive treatment of homocystinuria involving deficiencies or defects in cystathionine beta-synthase, 5,10-methylene-tetrahydrofolate reductase, or cobalamin cofactor metabolism (specialist use only) ▶BY MOUTH ▶Adult: 3 g twice daily (max. per dose 10 g), adjusted according to response; maximum 20 g per day
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Betaine should be used in conjunction with dietary restrictions and may be given with supplements of Vitamin B12, pyridoxine, Blood and nutrition and folate under specialist advice.
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Scottish Medicines Consortium (SMC) decisions SMC No. 407/07 The Scottish Medicines Consortium has advised (August 2010) that betaine anhydrous (Cystadane ®) is accepted for restricted use within NHS Scotland for the adjunctive treatment of homocystinuria involving deficiencies or defects in cystathionine beta-synthase, 5,10-methylene- tetrahydrofolate reductase, or cobalamin cofactor metabolism in patients who are not responsive to pyridoxine treatment.
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▶Uncommon Abdominal discomfort. agitation. alopecia. appetite decreased. brain oedema. diarrhoea. glossitis. irritability. nausea. skin reactions. urinary incontinence. vomiting
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Manufacturer advises avoid unless essential—limited information available.
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Manufacturer advises caution—no information available.
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Monitor plasma-methionine concentration before and during treatment—interrupt treatment if symptoms of cerebral oedema occur.
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Powder should be mixed with water, juice, milk, formula, or food until completely dissolved and taken immediately; measuring spoons are provided to measure 1 g, 150 mg, and 100 mg of Cystadane ® powder.
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There can be variation in the licensing of different medicines containing the same drug. Forms available from special-order manufacturers include: tablet, oral solution Powder ▶Cystadane (Recordati Rare Diseases UK Ltd) Betaine 1 gram per 1 gram Cystadane oral powder | 180 gram P £347.00 DT = £347.00 2.7 Hypophosphatasia ENZYMES
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3 g twice daily (max. per dose 10 g), adjusted
First-line fragment. Read the complete Indications and Dose section above.
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