CYSTADANE- betaine powder, for solution

Држава: Сједињене Америчке Државе

Језик: Енглески

Извор: NLM (National Library of Medicine)

Активни састојак:

BETAINE (UNII: 3SCV180C9W) (BETAINE - UNII:3SCV180C9W)

Доступно од:

Recordati Rare Diseases

INN (Међународно име):

BETAINE

Састав:

BETAINE 1 g in 1 g

Пут администрације:

ORAL

Тип рецептора:

PRESCRIPTION DRUG

Терапеутске индикације:

CYSTADANE ® is indicated for the treatment of homocystinuria to decrease elevated homocysteine blood concentrations in pediatric and adult patients. Included within the category of homocystinuria are: - Cystathionine beta-synthase (CBS) deficiency - 5,10-methylenetetrahydrofolate reductase (MTHFR) deficiency - Cobalamin cofactor metabolism (cbl) defect None. Risk Summary Available data from a limited number of published case reports and postmarketing experience with CYSTADANE use in pregnancy have not identified any drug associated risks for major birth defects, miscarriage, or adverse maternal or fetal outcomes. Animal reproduction studies have not been conducted with betaine. The estimated background risk of major birth defects and miscarriage for the indicated population is unknown. All pregnancies have a background risk of birth defect, loss, or other adverse outcomes. In the U.S. general population, the estimated background risk of major birth defects and miscarriage in clinically recognized pregnanc

Резиме производа:

CYSTADANE is available in plastic bottles containing 180 grams of betaine anhydrous as a white, granular, hygroscopic powder. Each bottle is equipped with a plastic child-resistant cap and is supplied with a polypropylene measuring scoop. One level scoop (1.7 mL) is equal to 1 gram of betaine anhydrous powder. NDC 52276-400-01 180 g/bottle Store at room temperature, 15 to 30 ˚C (59 to 86 ˚F). Protect from moisture.

Статус ауторизације:

New Drug Application

Карактеристике производа

CYSTADANE- BETAINE POWDER, FOR SOLUTION
RECORDATI RARE DISEASES
----------
HIGHLIGHTS OF PRESCRIBING INFORMATION
THESE HIGHLIGHTS DO NOT INCLUDE ALL THE INFORMATION NEEDED TO USE
CYSTADANE SAFELY AND
EFFECTIVELY. SEE FULL PRESCRIBING INFORMATION FOR CYSTADANE.
CYSTADANE (BETAINE ANHYDROUS FOR ORAL SOLUTION) POWDER
INITIAL U.S. APPROVAL: 1996
INDICATIONS AND USAGE
CYSTADANE is a methylating agent indicated in pediatric and adult
patients for the treatment of
homocystinuria to decrease elevated homocysteine blood concentrations.
Included within the category of
homocystinuria are ( 1):
Cystathionine beta-synthase (CBS) deficiency
5,10-methylenetetrahydrofolate reductase (MTHFR) deficiency
Cobalamin cofactor metabolism (cbl) defect
DOSAGE AND ADMINISTRATION
Adults and Pediatric Patients 3 Years of Age and Older
The recommended dosage is 6 grams per day, administered orally in
divided doses of 3 grams twice
daily. ( 2.1)
Pediatric Patients Less than 3 Years of Age
The recommended starting dosage is 100 mg/kg/day, administered orally
in divided doses of 50 mg/kg
twice daily, and then increased weekly by 50 mg/kg increments. ( 2.1)
Monitor patient response by plasma homocysteine concentrations. ( 2.1)
Increase the dosage gradually until the plasma total homocysteine
concentration is undetectable or
present only in small amounts. ( 2.1)
Preparation and Adminstration Instructions
Prescribed amount of CYSTADANE should be measured with the measuring
scoop provided and then
dissolved in 4 to 6 ounces of water, juice, milk, or formula until
completely dissolved, or mixed with food
for immediate ingestion. ( 2.2)
DOSAGE FORMS AND STRENGTHS
For oral solution: in bottles containing 180 grams of betaine
anhydrous. ( 3)
CONTRAINDICATIONS
None ( 4)
WARNINGS AND PRECAUTIONS
Hypermethioninemia in Patients with CBS Deficiency:
CYSTADANE may worsen elevated plasma methionine concentrations and
cerebral edema has been
reported. Monitor plasma methionine concentrations in patients with
CBS deficiency. Keep plasma
methionine concentration