CLINICAL STUDIES ON ACUTE INTERMITTENT PORPHYRIA

概要

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Two cases with acute intermittent porphyia were investigated clinically, and the following results were obtained. 1) Typical gastro-intestinal, neurological and cardiovascular symptomes as acute intermittent porphyria were observed during the clinical course of these two cases. Chemical analyse of urine from both cases revealed an increased excretion of porphobilinogen and δ-amino laevulinic acid. 2) Generalized muscular atrophy was one of the prominent features of acute phase in any of these cases. Creatinuria was detected in one case with severe muscular athrophy in acute phase of the disease. But excretion of creatinine was not increased significantly. 3) No remarkable changes were observed in activity of creatine-phosphokinase and Aldolase of serum. 4) Analysis of electromyogram revealed that muscular atrophy observed in both cases resulted from both myogenic and neurogenic disturbances. 5) Atrophy of whole muscular fibers was observed in histological sections of M. quadriceps femoris obtained by biopsy. But no changes in interstitial tissue could be found. 6) Creatin excreted to urine was decreased to normal level, after the recovery from muscular atrophy. No abnormalities were detected in electromyogram after the complete recovery from muscular atrophy.
北関東医学会の論文