| U.S. Brand Names |
| Cystadane® |
 |
| Generic Available |
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No |
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| Pharmacological Index |
|
Homocystinuria, Treatment Agent |
|
| Use |
|
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), and cobalamin cofactor metabolism (CBL). |
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| Pregnancy Risk Factor |
|
C |
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| Pregnancy/Breast-Feeding Implications |
|
Clinical effects on the fetus: Animal reproduction studies have not been conducted with betaine. It is also not known whether betaine can cause fetal harm when administered to a pregnant woman or can affect reproductive capacity. Betaine should be given to a pregnant woman only if needed. |
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| Contraindications |
|
Hypersensitivity to betaine or its ingredients |
|
| Adverse Reactions |
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Minimal; have included nausea, GI distress, and diarrhea |
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| Stability |
|
Store at room temperature |
|
| Usual Dosage |
|
Children <3 years: Dosage may be started at 100 mg/kg/day and then increased weekly by 100 mg/kg increments Children greater than or equal to 3 years and Adults: Oral: 6 g/day administered in divided doses of 3 g twice daily. Dosages of up to 20 g/day have been necessary to control homocysteine levels in some patients. Dosage in all patients can be gradually increased until plasma homocysteine is undetectable or present only in small amounts |
|
| Administration |
|
Measure prescribed amount with provided measuring scoop and dissolve in 120-180 mL of water for immediate ingestion |
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| Mental Health: Effects on Mental Status |
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None reported |
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| Mental Health: Effects on Psychiatric Treatment |
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None reported |
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| Dental Health: Local Anesthetic/Vasoconstrictor Precautions |
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No information available to require special precautions |
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| Dental Health: Effects on Dental Treatment |
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No effects or complications reported |
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| Patient Information |
|
One level scoop is equivalent to 1 g of betaine anhydrous powder; use only the scoop provided; shake bottle lightly before opening, close cap tightly after use to protect from moisture; do not use if powder does not completely dissolve or gives a colored solution |
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| Dosage Forms |
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Powder: 1 g/1.7 mL (180 g) |
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| References |
|
Burns SP, Iles RA, Ryalls M, et al "Methylgenesis From Betaine in Cystathionine-Beta-Synthase Deficiency," Biochem Soc Trans, 1993, 21:455S. Kishi T, Kawamura I, Harada Y, et al, "Effect of Betaine on S-Adenosylmethionine Levels in the Cerebrospinal Fluid in a Patient With Methylenetrahydrofolate Reductase Deficiency and Peripheral Neuropathy," J Inherit Metab Dis, 1994, 17:560-5. Smolin LA, Benevenga NJ, and Berlow S, "The Use of Betaine for the Treatment of Homocystinuria," J Pediatr, 1981, 99:467-72. |
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