Clinical disorders of porphyrin metabolism: Part 1

digital file Black & White Sound 1972 40:31

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Summary: This is the first part of a talk by Professor Abe Goldberg from the University of Glasgow. A summary accompanying the cassette says: Haems and porphyrins are present in every living cell of the body and disorders of their metabolism can take place in a number of clinical states. Acute intermittent porphyria is the most important of the porphyria disorders, due to a defect of an hepatic enzyme and presenting with neuro-psychiatric manifestations, hypertension and severe abdominal pain. The disease can be provoked by drugs such as barbiturates, and an endogenous abnormality of 17-oxosteroid metabolism has been identified in a majority of cases. The cutaneous porphyrias present with skin photosensitivity, due to a defect in the liver, sometimes associated with alcoholism or hepatic cirrhosis and sometimes with excessive porphyrin production in erythrocyte precursors of the bone marrow. Abnormalities of porphyrin metabolism also occur in lead poisoning, a number of anaemias, including the secondary anaemias of infectin and neoplasm, and the sideroachrestic anaemias.