Finished Products
시스타단 산
Cystadane® Powder
Classification
Metabolic Agents

 

 

Indication/Usage


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


Dosage/Administration

 

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. 

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 following doses: small measure 100 mg, middle size
measure 150 mg and large measure 1 g of betaine anhydrous. 
The powder should be mixed with water, juice, milk, formula or food until completely dissolved and
ingested immediately after mixing.

 

 


 

 

Development Marketing Dept(Product information)
+82-2-566-7199