The present study was carried out in five cases of hepatic porphyria, including three of acute intermittent porphyria, one of variegate porphyria, and one of porphyria cutanea tarda in clinical remission. In two cases of acute intermittent porphyria (in relapse), a marked lowering effect on serum and urine porphobilinogen and delta-aminolevulinic acic was observed, together with prompt and gratifying clinical improvement. In a third case, in chemical remission but with longstanding psychoneurosis, no significant effects were noted, nor were any observed in the case of porphyria cutanea tarda. Although clinical improvements occurred in the case of variegate porphyria, the results were inconclusive for reasons given. Hematin was generally well tolerated. Preliminary reference is made to a transitory renal injury, without sequelae, where an excess of hematin was given in relation to time. Limits of tolerance are proposed. In the light of these observations the basic mechanism of the acute attack is diccussed.
ABSTRACT. Transitory renal failure occurred in a patient with acute intermittent porphyria in clinical remission following i.v. administration of 1000 mg hematin. The clinical and biochemical picture suggested “acute tubular necrosis”, which was followed by a prompt and complete return of renal function without any late sequelae. The renal failure is thought to have resulted from the presence of circulating free hematin, formed as a result of rapid administration of such a relatively large amount. Such a complication has not occurred in patients given hematin for acute porphyric relapse, in whom much smaller amounts have been infused.
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