Haem arginate

Oral and intravenous glucose and haem arginate are the mainstay of treatment. Glucose 10% should be infused intravenously for mild attacks and pending the administration of haem arginate. The recommended daily dose of haem arginate is 3 mg/kg once daily for 4 days. 

Glucose 10% is generally readily available in a hospital environment. Supplies of haem arginate (human hemin), trade name Normosang may be obtained from Orphan Europe during office hours and out of hours from St Thomas Hospital, London. 

Repeat the measurement at regular intervals or if the patient has symptoms in keeping with cm acute attack. If there is an increase in the precursor levels, stop the treatment and consider giving haem arginate for acute attack (see below). 

In the treatment of the acute attack it is rational to use any safe means of depressing the formation of ALA-S5mthase. Haem arginate (human haematin) infusion, by replenishing haem and so removing the stimulus to ALA-synthase, is effective if given early, and may prevent chronic neuropathy. Additionally, attention to nutrition, particularly the supply of carbohydrate, relief of pain (with an opioid), and of hypertension and tachycardia (with a p-adreno-ceptor blocker) are important. Hyponatraemia is a frequent complication, and plasma electrolytes should be monitored. 

Treatment consists of i.v. glucose infusions (2 X 2,000 ml, 20% per day) plus administration of haem arginate (3 mg/kg BW/day on four consecutive days and at varying sites of injection) (s. p. 846), possibly with simultaneous administration of metalloporphyrin (for the inhibition of haemoxygenase) (276, 301) as well as intensive care measures and administration of cimetidine (292) or iron. In cases with peripheral or CNS symptoms, prednisolone (100 mg/day) may be administered in addition. (267, 289, 295, 296, 312, 313, 318)... 

Herrick, A., McLellan, A., Brodie, M.J., McColl, K.E.L., Moore, M.R., Goldberg, A. Effect of haem arginate therapy on porphyrin metabolism and mixed function oxygenase activity in acute hepatic porphyria. Lancet 1987/11 1178—1179... 

Manning, D.J., Gray, T.A. Haem arginate in acute hereditary coproporphyria. Arch. Dis. Child. 1991 66 730-731... 

Mustajoki, R, Nordmann, Y. Early administration of haem arginate for acute porphyric attacks. Arch. Intern. Med. 1993 153 2004-2008... 

Herrick AL, McColl KE, Moore MR, Cook A, Goldberg A. Controlled trial of haem arginate in acute hepatic porphyria. Lancet 1989 l(8650) 1295-7. 

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