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Tetrahydrobiopterin (BH4, THB), also known as sapropterin (INN), is a cofactor of the three aromatic amino acid hydroxylase enzymes, used in the metabolism of amino acid phenylalanine and in the biosynthesis of the neurotransmitters serotonin (5-hydroxytryptamine, 5-HT), melatonin, dopamine, norepinephrine (noradrenaline), epinephrine (adrenaline), and is a cofactor for the production of nitric oxide (NO) by the nitric oxide synthases. Chemically, its structure is that of a (dihydropteridine reductase) reduced pteridine derivative (quinonoid dihydrobiopterin).[citation needed]
Read the full article on WikipediaHyperphenylalaninaemia Clinical criteria: Treatment Phase: Initial treatment Treatment criteria: Must be treated by a metabolic physician. Patient must have hyperphenylalaninaemia (HPA) due to tetrahydrobiopterin (BH4) deficiency. Patient must have documented tetrahydrobiopterin (BH4) deficiency using tests for BH4 loading and/or urine pterin metabolites, blood spot dihydropteridine reductase (DHPR) and have cerebrospinal fluid neurotransmitter metabolites measured.
Hyperphenylalaninaemia (HPA) due to phenylketonuria (PKU) Clinical criteria: Treatment Phase: Initial treatment - responsiveness testing Treatment criteria: Must be treated by a metabolic physician. Patient must be untreated with this drug; OR Patient must have completed prior responsiveness testing on only 1 occasion - this occurred when the patient was less than 1 month of age, but this benefit is for a second attempt at responsiveness testing in a patient aged at least 1 month old, AND Patient must have a baseline blood phenylalanine level above 360 micromole per L and be less than one month of age; OR Patient must have a baseline blood phenylalanine level above 600 micromole per L and be more than one month of age, AND The treatment must be for the purpose of initial responsiveness testing for a period of 24 hours in a patient less than one month of age; OR The treatment must be for the purpose of initial responsiveness testing for a period of 7 days in a patient aged more than one month. Dietary phenylalanine intake must be maintained at a constant level. Patients or their parent/guardian should be assessed for their ability to comply with the sapropterin protocol and PKU diet prior to conducting initial responsiveness testing.
Hyperphenylalaninaemia (HPA) due to tetrahydrobiopterin (BH4) deficiency Clinical criteria: Treatment Phase: Continuing treatment Treatment criteria: Must be treated by a metabolic physician; OR Must be treated by a nurse practitioner experienced in the treatment of phenylketonuria in consultation with a metabolic physician. Patient must have hyperphenylalaninaemia (HPA) due to tetrahydrobiopterin (BH4) deficiency, AND Patient must have previously received PBS-subsidised treatment with this drug for this condition. Patient must have documented tetrahydrobiopterin (BH4) deficiency using tests for BH4 loading and/or urine pterin metabolites, blood spot dihydropteridine reductase (DHPR) and have cerebrospinal fluid neurotransmitter metabolites measured.
Maternal hyperphenylalaninaemia (HPA) due to phenylketonuria (PKU) Clinical criteria: Treatment Phase: Pre-conception through to when pregnancy first becomes known Patient must have demonstrated an adequate response to treatment with this drug at least once in a lifetime, with an adequate response defined as a reduction in phenylalanine levels from baseline during initial responsiveness testing of no less than 30%. Treatment criteria: Must be treated by a metabolic physician; OR Must be treated by a nurse practitioner experienced in the treatment of phenylketonuria in consultation with a metabolic physician, AND Patient must not be undergoing treatment with this drug under this Treatment phase, following completion of this authority application, for more than 13 cumulative months (assuming 1 month consists of 30 days), AND Patient must not be undergoing simultaneous treatment with this drug under another non-maternal PBS-listing (apply under either listing type, but not both simultaneously). Population criteria: Patient must be actively trying to conceive.
“4 hours (healthy adults)6–7 hours (PKU patients)”
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