Advice

following a second resubmission

betaine anhydrous (Cystadane) is accepted  for restricted use within NHS Scotland.

Indication under review:
adjunctive treatment of homocystinuria involving deficiencies or defects in cystathionine beta-synthase (CBS), 5,10-methylene-tetrahydrofolate reductase (MTHFR) or cobalamin cofactor metabolism (cbl).

SMC restriction: patients who are not responsive to vitamin B6 treatment.

Limited clinical data confirmed the effectiveness of betaine anhydrous in homocystinuria, There remains some uncertainty about the cost-effectiveness of betaine anhydrous even in the restricted patient group described above, but given the orphan nature of the condition the economic case for use was accepted.

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Medicine details

Medicine name:
betaine anhydrous (Cystadane)
SMC ID:
407/07
Indication:
Adjunctive treatment of homocystinuria involving deficiencies or defects in cystathionine beta-synthase (CBS), 5,10-methylene-tetrahydrofolate reductase (MTHFR) or cobalamin cofactor metabolism (cbl).
Pharmaceutical company
Orphan Europe (UK) Ltd
BNF chapter
Nutrition and blood
Submission type
Resubmission
Status
Restricted
Date advice published
09 August 2010