Iterative prophylactic treatment by hematin in two cases of recurrent disabling acute intern1ittent porphyria (AIP)

Abstract

Summary: Hematin is known as a useful drug in the treatment of acute intermittent porphyria (Al P) attacks. We have used it with success as an iterative prophylactic treatment in two cases of AIP with recurrent disabling crises. In one of the two cases, the kinetic study of plasmatic delta amino-fernlinic acid (ALA) has shown an ALA plasma level (40-45 μg/dl) which is considered to be a trigger level for this patient. Cntil now, no side effects due to such a hematin treatment have been observed in either patient. This schedule could represent an improvement in the management of recurrent disabling AI P crises.