CYSTADANE betaine 1g/g oral powder bottle

Country: Australia

Language: English

Source: Department of Health (Therapeutic Goods Administration)

Active ingredient:

betaine, Quantity: 1 g/g

Available from:

Recordati Rare Diseases Australia Pty Ltd

INN (International Name):

Betaine

Pharmaceutical form:

Powder, oral

Composition:

Excipient Ingredients:

Administration route:

Oral

Units in package:

180g

Prescription type:

Not scheduled. Not considered by committee

Therapeutic indications:

As an adjunct in the treatment of homocystinuria.

Product summary:

Visual Identification: A white, granular powder; Container Type: Bottle; Container Life Time: 4 Years; Container Temperature: Store below 25 degrees Celsius

Authorization status:

Licence status A

Authorization date:

1996-09-30

Patient Information leaflet

Cystadane AU CMI 3.1
1
CYSTADANE
®
Betaine anhydrous powder for oral administration
Consumer Medicine Information
WHAT IS IN THIS LEAFLET
This leaflet answers some of the
more common questions about
Cystadane.
It does not contain all of the
available information. It does
not take the place of talking to
your doctor or pharmacist.
All medicines have risks and
benefits. Your doctor has
weighed the risks of you taking
Cystadane against the benefits
he/she expects it will have for
you.
IF YOU HAVE ANY CONCERNS
ABOUT TAKING THIS MEDICINE,
ASK YOUR DOCTOR OR
PHARMACIST.
KEEP THIS LEAFLET WITH YOUR
MEDICINE.
You may need to read this again.
WHAT CYSTADANE
IS USED FOR
Cystadane is used to decrease
high levels of homocysteine in
the blood, referred to as
homocystinuria. Homocysteine
is a breakdown product of the
amino acid methionine that
forms part of proteins present in
food. The high levels of
homocysteine in the blood may
be caused by the various
deficiencies in enzymes that
break down proteins,
specifically called cystathionine
beta synthase (CBS) or from 5,
10- methylenetetrahydrofolate
reductase deficiency
(MTHFR
deficiency) or cobalamin
cofactor metabolism defect
(CBL defect).
High levels of homocysteine in
the blood can be toxic and may
cause problems in the skeleton
and the eyes, as well as mental
retardation and cardiovascular
disease.
Some people are born with
inherited diseases that cause
some of the body’s natural
enzymes of metabolism to be
missing or defective. CBS
deficiency, MTHFR deficiency
and CBL defects are all rare
disorders that result in patients
not being able to perform the
conversion of methionine to
cysteine.
This disorder presents
at early childhood and persists
for life, so therefore this
treatment is lifelong.
Cystadane together with other
treatments such as vitamin B6,
vitamin B12 and folate helps the
body effectively process
homocysteine by triggering the
enzymes required to convert it to
methionine. This results in a
decrease of plasma
homocysteine levels.
ASK YOUR DOCTOR IF YOU HAVE

Summary of Product characteristics

Version 2.4
AUSTRALIAN PRODUCT INFORMATION - CYSTADANE® (BETAINE ANHYDROUS
POWDER)
1.
NAME OF THE MEDICINE
Betaine anhydrous
2.
QUALITATIVE AND QUANTITATIVE COMPOSITION
1 g of powder contains 1 g of betaine anhydrous.
It contains no ingredient other than anhydrous betaine. Betaine
anhydrous powder is soluble in
water, methanol and ethanol. It is sparingly soluble in ether.
3.
PHARMACEUTICAL FORM
Cystadane® (betaine anhydrous powder) for oral administration is a
white, granular powder.
4.
CLINICAL PARTICULARS
4.1
THERAPEUTIC INDICATIONS
Cystadane is indicated as an adjunct in the treatment of
homocystinuria.
Cystadane is also indicated to decrease elevated homocysteine blood
levels in patients of all age
groups with:
1.
cystathionine beta-synthase (CBS deficiency) type of homocystinuria,
or
2.
5, 10- methylenetetrahydrofolate reductase deficiency (MTHFR
deficiency), or
3.
cobalamin cofactor metabolism defect (cbl defect) type of
homocystinuria.
Cystadane is also indicated to increase methionine and
S-adenosylmethionine blood levels in
patients with 5, 10- methylenetetrahydrofolate reductase deficiency
(MTHFR deficiency) and
cobalamin cofactor metabolism defect (cbl defect) types of
homocystinuria.
Patient response to Cystadane can be monitored by homocysteine plasma
levels (see 4.2 DOSE AND
METHOD OF ADMINISTRATION). Response usually occurs within a week and
steady state within a
month.
Methionine blood levels may become greatly elevated in CBS deficiency
type patients. However.
monitoring of patients with high methionine blood levels for many
years has not revealed any
toxicities or other clinical problems.
Cystadane can be administered along with folate, vitamin B
6
, and vitamin B
12
(cobalamin).
4.2 DOSE AND METHOD OF ADMINISTRATION
The usual dose used in adults and paediatric patients is 6 grams per
day administered orally in
divided doses of 3 grams two times per day. Dosages of up to 20 grams
per day have been necessary
to control homocysteine levels in some patients. In paediatric
patients less than 3 ye