[Acute attacks of hepatic porphyria: specific treatment with heme arginate].
Severe hepatic porphyrias (acute intermittent porphyria, variegate porphyria and hereditary coproporphyria) are hereditary diseases. Each type of porphyria is the result of a specific decrease in the activity of one of the enzymes of heme biosynthesis. Acute attacks are very serious: the abdominal pains are severe and the neurological manifestations can lead to death or incomplete recovery with irreversible sequelae (usually paralysis). Since 1985, the prognosis of acute attacks has been greatly improved by the introduction of heme-arginate. The 69 acute attacks (30 patients, 4 men and 26 women) that we treated with heme-arginate between 1988 and 1991 are described in this report. All patients were infused with 250 mg/d of heme-arginate for 4 days: the mean duration of abdominal pain was 2.5 days (SD 0.72). For 95 p. 100 of the attacks, the total hospitalization time was 5 days or less; side effects were very minor. In every case, a favorable response was dependent upon the early initiation of heme therapy.
Nordmann
Y
Y
Centre Français des Porphyries, Hôpital Louis-Mourier, Colombes.
Deybach
J C
JC
D000208:Acute Disease; D001120:Arginine / Q000008:administration & dosage / Q000627:therapeutic use*; D005260:Female; D006418:Heme / Q000008:administration & dosage / Q000627:therapeutic use*; D006760:Hospitalization; D006801:Humans; D007262:Infusions, Intravenous; D008297:Male; D017094:Porphyrias, Hepatic / Q000188:drug therapy*; D013997:Time Factors
D006418:Heme; D001120:Arginine; C048849:heme arginate
Les crises aiguës des porphyries hépatiques: leur traitement spécifique par l'hème-arginate.