Cystadane oral powder

Nazione: Regno Unito

Lingua: inglese

Fonte: MHRA (Medicines & Healthcare Products Regulatory Agency)

Principio attivo:

Betaine

Commercializzato da:

Orphan Europe (UK

Codice ATC:

A16AA06

INN (Nome Internazionale):

Betaine

Dosaggio:

1gram/1gram

Forma farmaceutica:

Powder

Via di somministrazione:

Oral

Classe:

No Controlled Drug Status

Tipo di ricetta:

Valid as a prescribable product

Dettagli prodotto:

BNF: 09080100

Scheda tecnica

OBJECT 1
CYSTADANE 1 G ORAL POWDER
Summary of Product Characteristics Updated 23-Feb-2017 | Orphan Europe
(UK) Limited
1. Name of the medicinal product
Cystadane 1 g oral powder
2. Qualitative and quantitative composition
1 g of powder contains 1 g of betaine anhydrous.
For the full list of excipients, see section 6.1.
3. Pharmaceutical form
Oral powder.
White crystalline free flowing powder.
4. Clinical particulars
4.1 Therapeutic indications
Adjunctive treatment of homocystinuria, involving deficiencies or
defects in:
• cystathionine beta-synthase (CBS),
• 5,10-methylene-tetrahydrofolate reductase (MTHFR),
• cobalamin cofactor metabolism (cbl).
Cystadane should be used as supplement to other therapies such as
vitamin B6 (pyridoxine), vitamin B12
(cobalamin), folate and a specific diet.
4.2 Posology and method of administration
Cystadane treatment should be supervised by a physician experienced in
the treatment of patients with
homocystinuria.
Posology
_Children and Adult _
The recommended total daily dose is 100 mg/kg/day given in 2 doses
daily. However, the dose should be
individually titrated according to plasma levels of homocysteine and
methionine. In some patients doses
above 200 mg/ kg/day were needed to reach therapeutic goals. Caution
should be exercised with up-
titrating doses for patients with CBS deficiency due to the risk for
hypermethioninaemia. Methionine
levels should be closely monitored in these patients.
_Special populations _
_Hepatic or renal impairment_
Experience with betaine anhydrous therapy in patients with renal
insufficiency or non-alcoholic hepatic
steatosis has demonstrated no need to adapt the dose regimen of
Cystadane.
Method of administration
The bottle should be lightly shaken before opening. Three measuring
spoons are provided which dispense
either 100 mg, 150 mg or 1 g of betaine anhydrous. It is recommended
that a heaped measuring spoon is
removed from the bottle and a flat surface e.g. base of a knife is
drawn across the top of the measure. This
will give the followin