SAMOH Pharm CO., LTD. & SAMOH Pharmachem CO., LTD.

The Treatment for hyperphenylalaninaemia in adults and paediatric patients

1. Phenylketonuria

2. Tetrahydrobiopterin (BH4) deficiency

Tretment with Kuvalla must be initiated and supervised by a physician experienced in the treatment PKU and BH4 deficiency.

Active management of dietary phenylalanine and overall protein intake while taking this medicinal product is required to ensure adequate control of blood phenylalanine levels and nutritional balance.

As HPA due to either PKU or BH4 deficiency is a chronic condition, once responsiveness is demonstrated, Kuvan is intended for long-term use. However, there are limited data regarding the long-term use of this medicinal product.

Kuvalla is provided as 100 mg tablets. The calculated daily dose based on body weight should be rounded to the nearest multiple of 100. For instance, a calculated dose of 401 to 450 mg should be rounded down to 400 mg corresponding to 4 tablets. A calculated dose of 451 mg to 499 mg should be rounded up to 500 mg corresponding to 5 tablets

1) Phenylketonuria

The starting dose of Kuvan in adult and paediatric patients with Phenylketonuria is 10 mg/kg body weight once daily. The dose is adjusted, usually between 5 and 20 mg/kg/day, to achieve and maintain adequate blood phenylalanine levels as defined by the physician.

2) Tetrahydrobiopterin (BH4) deficiency

The starting dose of Kuvalla in adult and paediatric patients with Tetrahydrobiopterin(BH4)  deficiency is 2 to 5 mg/kg body weight once daily dose. Doses may be adjusted up to a total of 20 mg/kg per day. It may be necessary to divide the total daily dose into 2 or 3 administration, distributed over the day, to optimize the therapeutic effect.

3) Paediatric population

The posology is the same in adults and children.