Rationalet for behandling med Kuvan i patienter med BH4 mangel er at erstatte det manglende BH4, hvor ved aktiviteten af fenylalanin hydroksylase genoprettes.
The rationale for administration of Kuvan in patients with BH4 Deficiency is to replace the deficient levels of BH4, thereby restoring the activity of phenylalanine hydroxylase.