[Erythropoietic protoporthyria. Clinical aspects and therapy].
After introduction in clinical, histological and ultrastructural features of erythropoietic protoporphyria the therapeutic effect of beta-Carotene is reported in 5 patients with erythropoietic proporphyria and in 1 patient with polymorphous light dermatosis. beta-Carotene was given orally in a daily initial dose of 50-100 mg, continued with 25 mg a day. In all patients the therapeutic effect set on about 2 months after beginning of the treatment and was measured by increased toleration to insolation. During the treatment over a period of four years no side effects were noticed by 127 clinical and biochemical controls beside a dose-dependent "icterus" localized as well in the perioral region as on the hands and feet.
D000328:Adult; D002338:Carotenoids / Q000008:administration & dosage / Q000627:therapeutic use; D002648:Child; D004912:Erythrocytes / Q000378:metabolism; D004920:Erythropoiesis; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D011164:Porphyrias / Q000097:blood* / Q000175:diagnosis / Q000188:drug therapy; D011166:Porphyrins / Q000097:blood*; D011524:Protoporphyrins / Q000097:blood*
D011166:Porphyrins; D011524:Protoporphyrins; D002338:Carotenoids
Erythropoetische Protoporphyrie Klinik und Therapie