“Normosang® Infusion 250 mg ( human hemin)” for the treatment of acute hepatic porphyria; an uncommon but life-threatening medical emergency.
The porphyrias are a hereditary group of disorders, each of which is related to a deficiency in one of the enzymes involved in the biosynthesis of heme. The acute porphyrias [acute intermittent porphyria (AIP), variegate porphyria (VP) and hereditary coproporphyria (HCP)] are rare, autosomal dominant, and characterised by episodic acute neurovisceral attacks which may be life threatening.
Heme arginate (Normosang) in the treatment of attacks of acute hepatic porphyrias.
Normosang® (human hemin, Orphan Europe) is a concentrated heme solution (250 mg heme per ampoule) in which heme is stabilised as a complex with arginine (267 mg) suspended in a mixture of ethanol (1g) and propyleneglycol (4g) made up to 10 ml with water.
Heme as a lyophilized powder (Panhematin; Abbot Laboratories) is available in the United States where human hemin (Normosang®) does not have FDA approval.
Normosang : Prescription products
Composition : Human hemin 25mg/ml. One ampoule of 10 ml contains 250 mg of human hemin.
Presentation : Concentrate for solution for infusion. Each pack contains 4 ampoules of 250mg of human hemin.
Storage and stability: Store between 2-8C, protected from light. The diluted solution should be used immediately.
Indications : Treatment of acute attacks of hepatic porphyria (acute intermittent porphyria, porphyria variegata, hereditary coproporphyria).
Contraindications : Known hypersensitivity to one of the constituents of Normosang.
Dosage and administration: Adults and elderly, recommended daily dose 3mg/kg once daily for four days, diluted in 100ml of 0.9 per cent sodium chloride in a glass bottle and infused intraveneously over at least 30 minutes into a large brachial vein using an inline filter. The dose should not exceed 250mg (one ampoule) daily. Exceptionally, the course may be repeated under strict biochemical surveillance if there is inadequate response after the first course of treatment.
Children, limited experience in tyrosinaemia suggests that it is safe to use a dose of not more than 3mg/kg daily for four days, administered with the same precautions as for adults. See (SPC) for preparation and administration of solution.