HOMOSISTINEX® is indicated for patients diagnosed with homocystinuria due to enzyme deficiencies or metabolic disorders. Patients with this rare disease have high homocysteine levels. HOMOSISTINEX® reduces plasma homocysteine levels.
HOMOSISTINEX® is indicated for the adjunctive (additional) treatment of homocystinuria involving deficiencies or disorders of Cystation beta-synthase (CBS), 5,10-methylene-tetrahydrofolate reductase (MTHFR), Cobalamin cofactor metabolism (cbl).
HOMOSISTINEX® should be used in addition to other treatments such as Vitamin B6 (pyridoxine), Vitamin B12 (cobalamin), folic acid and a special diet.
The recommended dose for children and adults aged 3 years and over is 6 g per day, divided into 2 doses of 3 g per day.
The recommended starting dose for children aged three years and younger is 50 mg/kg twice a day for a total of 100 mg/kg/day, while the maintenance dose is 25 mg/kg twice a day for a total of 50 mg/kg/day.
The bottle should be shaken gently before opening.
The powder should be mixed with water, juice, milk, formula or food until completely dissolved and ingested immediatelyafter mixing.
Oral White Crystalline Powder