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Pronunciation |
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(BAY
tayne an HY
drus) |
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U.S. Brand
Names |
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Cystadane® |
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Generic
Available |
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No |
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Pharmacological Index |
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Homocystinuria, Treatment Agent |
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Use |
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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 |
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C |
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Pregnancy/Breast-Feeding
Implications |
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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 |
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Hypersensitivity to betaine or its ingredients |
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Adverse
Reactions |
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Minimal; have included nausea, GI distress, and diarrhea |
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Stability |
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Store at room temperature |
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Usual Dosage |
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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 |
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Administration |
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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 |
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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 |
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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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