Treatment of THB deficiencies consists of THB supplementation (2–20 mg/kg per day) or diet to control blood phenylalanine concentration and replacement therapy with neurotransmitters precursors (L-DOPA and 5-HTP) and supplements of folinic acid in DHPR deficiency.<unk>  is available as a tablet for oral administration in the form of<unk>  dihydrochloride (BH4*2HCL). BH4*2HCL is FDA approved under the trade name Kuvan. The typical cost of treating a patient with Kuvan is $100,000 per year. BioMarin holds the patent for Kuvan until at least 2024, but Par Pharmaceutical has a right to produce a generic version by 2020. BH4*2HCL is indicated at least in<unk>  caused by GTPCH deficiency or PTPS deficiency.
Tetrahydrobiopterin deficiency
Cerebral folate deficiency
Cytochrome b5 deficiency
Adenine phosphoribosyltransferase deficiency
Molybdenum cofactor deficiency
Methylenetetrahydrofolate reductase deficiency
Dihydropyrimidine dehydrogenase deficiency
Biotin deficiency