Are there concerns about using nitisinone in special populations?
The effects on the pharmacokinetics of nitisinone have not been studied in the following special populations: • Geriatric: No studies/ no patients • Gender: Not studied • Race: Not studied • Renal Insufficiency: Not studied • Hepatic Dysfunction: Not studied 26. What is porphobilinogen synthase (PBG)?- Porphobilinogen synthase (PBG) is one of the 8 requisite enzymes of heme biosynthesis. Abnormally elevated porphyrin levels and their precursors including PBG results in a group of disorders known as porphyrias. Deficiencies in PBG result in porphyria-like neurologic symptoms. Porphobilinogen synthase is formed in the heme biosynthesis pathway by the conversion of aminolevulinic acid (ALA) into PBG. Succinylacetoacetone is a potent inhibitor of ALA thus a resulting in a deficiency of PBG.
