Country: Australia
Language: English
Source: Department of Health (Therapeutic Goods Administration)
betaine, Quantity: 1 g/g
Recordati Rare Diseases Australia Pty Ltd
Betaine
Powder, oral
Excipient Ingredients:
Oral
180g
Not scheduled. Not considered by committee
As an adjunct in the treatment of homocystinuria.
Visual Identification: A white, granular powder; Container Type: Bottle; Container Life Time: 4 Years; Container Temperature: Store below 25 degrees Celsius
Licence status A
1996-09-30
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 Read the complete document
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 Read the complete document