Abstract
ACUTE intermittent porphyria (AIP) is well known as an inborn error of porphyrin metabolism, clinically characterized by attacks of abdominal pains and frequently associated with the neurological and mental symptoms and signs. The excretion of the two kinds of porphyrin precursors, porphobilinogen (PBG) and delta-amino-laevulinic acid (ALA) in urine, suggesting the presence of a disturbance in the porphyrin metabolism, has been thought essential for the diagnosis of this disease.
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References
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NAKAO, K., WADA, O., KITAMURA, T. et al. Activity of Amino-laevulinic Acid Synthetase in Normal and Porphyric Human Livers. Nature 210, 838–839 (1966). https://doi.org/10.1038/210838b0
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DOI: https://doi.org/10.1038/210838b0
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