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Presse Medicale 1986-Oct

[Acute attacks of hepatic porphyria. Treatment with hematin. 5 cases].

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J F Devars du Mayne
J C Deybach
L Phung
J Likforman
R Tenhunen
M Cerf
Y Nordmann

Ključne riječi

Sažetak

The effectiveness of 2 hematins administered by intravenous infusion was compared in acute intermittent porphyria. Judging from subjective symptoms (abdominal pain), clinical improvement was complete and constant. There was a rapid decrease in urinary excretion of porphyrins precursors, with a clearer response of delta-aminolevulinic acid than of porphobilinogen. Urine levels of uroporphyrin often returned to normal after two infusions. The drugs were very well tolerated. Provided it is administered early, before neurological complications develop, treatment with hematin completely relieves abdominal symptoms and suppresses most of the biochemical changes associated with hepatic porphyria.

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