cystadane- betaine, anhydrous powder, for solution

Country: United States

Language: English

Source: NLM (National Library of Medicine)

Active ingredient:

betaine, anhydrous (UNII: 3SCV180C9W) (betaine, anhydrous - UNII:3SCV180C9W)

Available from:

Jazz Pharmaceuticals, Inc.

INN (International Name):

betaine, anhydrous

Pharmaceutical form:

POWDER, FOR SOLUTION

Composition:

180 g in 1 g

Administration route:

ORAL

Prescription type:

PRESCRIPTION DRUG

Therapeutic indications:

Cystadane (betaine anhydrous for oral solution) is indicated for the treatment of homocystinuria to decrease elevated homocysteine blood levels. Included within the category of homocystinuria are deficiencies or defects in: - cystathionine beta-synthase (CBS), - 5,10-methylenetetrahydrofolate reductase (MTHFR), - cobalamin cofactor metabolism (cbl ). Patient response to Cystadane can be monitored by homocysteine plasma levels (see DOSAGE AND ADMINISTRATION). Response usually occurs within a week and steady state within a month. Cystadane has been administered concomitantly with vitamin B6 (pyridoxine), vitamin B12 (cobalamin), and folate.

Product summary:

Cystadane is available in plastic bottles containing 180 grams of betaine anhydrous. Each bottle is equipped with a plastic child-resistant cap and is supplied with a polystyrene measuring scoop. One level scoop (1.7 cc) is equal to 1 gram of betaine anhydrous powder. NDC 68727-400-01 180g/bottle DIN 02238526 Store at room temperature, 15° - 30°C (59° - 86°F). For questions of a medical nature in the U.S. or Canada, call 1-888-867-7426. Cystadane® can be ordered by calling Jazz Pharmaceuticals, Inc. Customer Service at 1-800-359-4304 or by contacting your local wholesaler. Distributed in the U.S. by: Jazz Pharmaceuticals, Inc. Palo Alto, CA 94304 Distributed in Canada by: Jazz Pharmaceuticals, Inc. Scarborough, Ontario M1H 2W4 Revision Date: August 2006 Part No. CYS PI-8585 i  Matthews A, Johnson TN, Rostami-Hodjegan A, Chakrapani A, et al. An indirect response model of homocysteine suppression by betaine: optimizing the dosage regimen of betaine in homocystinuria. Br J Clin Pharmacol 2002; 54:140-146.

Summary of Product characteristics

CYSTADANE- BETAINE, ANHYDROUS POWDER, FOR SOLUTION
JAZZ PHARMACEUTICALS, INC.
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CYSTADANE® (BETAINE ANHYDROUS FOR ORAL SOLUTION)
R only
DESCRIPTION
Cystadane
(betaine anhydrous for oral solution) is an agent for the treatment of
homocystinuria.
Cystadane is a white, granular, hygroscopic powder. It contains no
ingredients other than anhydrous
betaine. Betaine anhydrous powder is very soluble in water, soluble in
methanol and ethanol, and
sparingly soluble in ether.
The chemical name of betaine anhydrous powder is trimethylglycine. It
has a molecular weight of
117.15. The structural formula is:
CLINICAL PHARMACOLOGY
When administered in recommended oral dosage to children or adults,
Cystadane acts as a methyl group
donor in the remethylation of homocysteine to methionine in patients
with homocystinuria. As a result,
toxic blood levels of homocysteine are reduced in these patients,
usually to 20-30 percent or less of
pre-treatment levels.
Elevated homocysteine blood levels are associated with clinical
problems such as a cardiovascular
thrombosis, osteoporosis, skeletal abnormalities, and optic lens
dislocation. Plasma levels of
homocysteine were decreased in nearly all patients treated with
betaine. In observational studies without
concurrent controls, clinical improvement was reported by the treating
physicians in about three-fourths
of patients taking betaine. Many of these patients were also taking
other therapies such as vitamin B
(pyridoxine), vitamin B
(cobalamin), and folate with variable biochemical responses. In most
cases
studied, adding betaine resulted in a further reduction of
homocysteine.
Betaine was observed to lower plasma homocysteine levels in the three
types of homocystinuria, i.e.,
cystathionine beta-synthase (CBS) deficiency;
5,10-methylenetetrahydrofolate reductase (MTHFR)
x
®
6
12
deficiency; and cobalamin cofactor metabolism (_cbl_) defect.
Betaine has also been demonstrated to increase low plasma methionine
and S-adenosylmethionine (SAM)
levels in patients with MTHFR deficiency