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Porphyrin precursors in blood, urine and cerebrospinal fluid in acute porphyria

Percy V.A. ; Shanley B.C. (1977)

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Four patients suffering from variegata porphyria were investigated during acute attacks. Porphyrin and porphyrin precursor concentrations were determined in the urine, serum, cerebrospinal fluid (CSF) and stools. Levels of delta-aminolaevulinic acid (ALA) and porphobilinogen were found to be very much lower in CSF than in serum sampled concurrently, and were well below levels at which these substances have been shown to exert effects on neural tissue in vitro. These findings cast doubt on the possibility that ALA or porphobilinogen is responsible for the production of the neural manifestations of acute porphyria.

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