Erythropoietic porphyria. Two cases and the results of metabolic alkalinization.
Two previously unreported cases of erythropoietic porphyria are presented. Therapeutic modalities are discussed along with the results of both short- and long-term metabolic alkalinization. Although there was a substantial increase in urinary coproporphyrin excretion during a six- to 8-day period of alkalinization, three months of alkalinization therapy resulted in no substantial depletion of porphyrin stores and both patients continued to have new skin lesions develop, indicating no clinical improvement.
D000293:Adolescent; D001639:Bicarbonates / Q000627:therapeutic use*; D003306:Coproporphyrins / Q000652:urine; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D011164:Porphyrias / Q000097:blood / Q000188:drug therapy* / Q000378:metabolism / Q000652:urine; D011166:Porphyrins / Q000378:metabolism; D013997:Time Factors
D001639:Bicarbonates; D003306:Coproporphyrins; D011166:Porphyrins