Abstract
Four patients with hereditary coproporphyria have been investigated, two during an acute attack and two during remission. In all four, urinary and fæcal coproporphyrin levels were significantly raised. In one of the patients studied during an attack, hepatic δ-aminolævulinic acid (A.L.A.) synthetase activity was high, but it was normal in a patient in remission at a time when urinary A.L.A. and porphobilinogen levels were also normal. Blood A.L.A. dehydrase activity was also elevated intwo patients who were studied during an attack. In both of these patients excessive urinary excretion of 17–oxosteroid was also found, while in the two patients in remission urinary 17–oxosteroid excretion was within the normal range.
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