RELATION BETWEEN UROPORPHYRIN EXCRETION, ACUTE ATTACKS OF HEREDITARY COPROPORPHYRIA AND SUCCESSFUL TREATMENT WITH HEME ARGINATE

1. The increased urinary excretion of porphyrins as well as of their p recursors was studied in a patient with hereditary coproporphyria duri ng two acute attacks in which symptoms differed markedly in character and severity. 2. The increase in urinary coproporphyrin was similar in the 'mild' and in the 'severe' attack, indicating a lack of correlati on between coproporphyrin level and clinical symptoms. 3. Aminolaevuli nic acid, porphobilinogen and uroporphyrin exhibited significantly hig her values during the 'severe' attack than during the 'mild' attack. D uring the severe attack these three compounds were increased 18-, 14- and 46-fold, respectively, compared with increases of 3-, 3- and 8-fol d, respectively, during the mild attack. 4. The striking rise in the f ormation of uroporphyrin was reflected in the plasma porphyrin profile , which revealed predominance of uroporphyrin. In accordance with this finding, an increase in erythrocyte porphobilinogen deaminase of 130% was recorded. 5. The fluorescence emission spectra of saline-diluted plasma (excitation of 405 nm) showed a distinct peak at 618 nm during the 'severe' episode and a small peak during the 'mild' attack, pointi ng to the possibility of diagnosing an attack simply by following the fluorometric screen of plasma. 6. The 'severe' attack of coproporphyri a was treated with daily infusions of haem arginate, 3 mg/kg, every da y for 4 days, at the end of which period a dramatic clinical response was observed. The relief of symptoms was found to be clearly related t o the moderate decrease in uroporphyrin excretion observed rather than to the steep decline in the precursors.