EFFECT OF HEMATIN ADMINISTRATION TO PATIENTS WITH PROTOPORPHYRIA AND LIVER-DISEASE

Hepatic damage in protoporphyria appears to be caused by a toxic effect of excess protoporphyrin. Therapy which reduces the formation of excess protoporphyrin may, therefore, be helpful. The effects of hematin administered i.v. to 2 patients with protoporphyria and decompensated cirrhosis are examined. Neither patient had side effects from the compound or manifested signs of toxicity. The vascular disappearance of hematin in 1 patient was similar to that in patients with porphyria who do not have structural liver disease. In both patients, biochemical changes occurred that were compatible with a reduced rate of protoporphyrin formation. Thus, hematin administration may be useful in treating patients with protoporphyria who develop liver disease.