Posology : מינונים

4.2 Posology and method of administration
Cystadane treatment should be supervised by a physician experienced in the treatment of patients with homocystinuria.
Posology
Adult
The recommended total daily dose in adult is 6 g per day administered orally in divided doses of 3 g two times per day.
Paediatric population
The recommended total daily dose in paediatric patients over 10 years of age is the same as adult. However, dose titration may be preferable in paediatric patients.
In paediatric patients less than 10 years of age, the usual effective dose regimen is 100 mg/kg/day given in 2 doses daily; increasing the frequency above twice daily and/or the dose above 150 mg/kg/day does not improve the homocysteine-lowering effect.
Special populations
Hepatic or renal impairment
Experience with betaine anhydrous therapy in patients with renal insufficiency or non- alcoholic hepatic steatosis has demonstrated no need to adapt the dose regimen of Cystadane.
Method of administration
The bottle should be lightly shaken before opening.Measuring spoons are provided which dispense 1 g of betaine anhydrous. It is recommended that a heaped measuring spoon is removed from the container and a flat surface e.g. base of a knife is drawn across the top of the measure. This will give 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.
Therapeutic monitoring
The aim of treatment is to keep plasma levels of total homocysteine below 15 μM or as low as possible. The steady-state response usually occurs within a month.