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Coexistence of hereditary coproporphyria with acute intermittent porphyria.
The results obtained suggest that deficiency of porphobilinogen deaminase and coproporphyrinogen oxidase can be inherited independently. Expand
Porphyric polyneuropathy and its pathogenesis in the light of electrophysiological investigations
Patients with acute intermittent porphyria were examined by means of routine electromyographic tests, measurements of motor and sensory conduction in peripheral nerves and stimulation fatigue tests, finding that the pathogenesis of porphyric polyneuropathy may be dependent upon a block of cholinergic endings. Expand
[Evaluation of porphyrogenic action of selected drugs].
The function and morphology of the liver in porphyria cutanea tarda.
The aminopyrine breath test, postprandial serum bile acids, and routine liver tests were assessed as indicators of liver dysfunction in 38 patients with porphyria cutanea tarda and there was no evident relationship between morphological and functional liver changes. Expand
Abnormalities in liver function and morphology and impaired aminopyrine metabolism in hereditary hepatic porphyrias.
It is possible that the defective heme biosynthesis, perhaps related to the reduced aminopyrine demethylation, could form the basis for the ultrastructural hepatic changes in porphyria, and could be the cause of the elevated postprandial serum bile acids levels. Expand
Increased activity of porphobilinogen deaminase in erythrocytes during attacks of acute intermittent porphyria.
It was found that in most cases the activity of porphobilinogen deaminase was higher than 24.3 nmoles/ml erythrocytes/hour, a value considered as characteristic for acute intermittent porphyria, and that it decreased during convalescence and remission. Expand
Heme arginate (Normosang) in the treatment of attacks of acute hepatic porphyrias.
The treatment with heme arginate of 47 attacks of acute hepatic porphyrias are presented. Distribution of patients into three groups of severity has shown that the best clinical response is obtainedExpand
Increased protoporphyrin in erythrocytes in a child with acute intermittent porphyria.
A child is described with acute intermittent porphyria but having an erythropoietic component, with disordered metabolism of porphyrins in bone marrow.