Homosistinex® is given to 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.

It is indicated for the adjunctive (additional) treatment of homocystinuria involving the following deficiencies or disorders:
• Cysteine 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 aged three years and over and adults is 6 g per day, divided into 2 doses of 3 g per day.
The recommended starting dose for children aged three years and under is 50 mg/kg twice daily for a total of 100 mg/kg/day, and the maintenance dose is 25 mg/kg twice daily for a total of 50 mg/kg/day.
The bottle should be shaken gently before opening.
The powder dose taken is mixed with water or fruit juice until it is completely dissolved and drunk immediately, or, if the calculated dose is suitable for the patient's diet, it can be mixed with milk, formula or food according to the doctor's recommendation.

Oral white crystalline powder