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Quantitation of 3‐ethyl‐5‐hydroxy‐4,5‐dimethyl‐Δ 3 ‐pyrrolin‐2‐one in the urine of patients with acute intermittent porphyria
Author(s) -
GRAHAM D. J. M.,
BRODIE M. J.,
McCOLL K. E. L.,
MOORE M. R.,
GOLDBERG A.
Publication year - 1979
Publication title -
european journal of clinical investigation
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.164
H-Index - 107
eISSN - 1365-2362
pISSN - 0014-2972
DOI - 10.1111/j.1365-2362.1979.tb01666.x
Subject(s) - porphobilinogen , acute intermittent porphyria , porphyria , excretion , urine , medicine , urinary system , endocrinology , chemistry , gastroenterology
Abstract. The monopyrrole 3‐ethyl‐5‐hydroxy‐4,5‐dimethyl‐Δ 3 ‐pyrrolin‐2‐one (hydroxyhaemopyrrolin‐2‐one) has been quantitated in the urine of eight patients with acute intermittent porphyria in attack or remission and ten subjects latent for the disorder. Previous qualitative investigations had revealed an association between hydroxyhaemopyrrolin‐2‐one excretion and certain psychiatric disorders including acute porphyria. Urinary excretion of hydroxyhaemopyrrolin‐2‐one was significantly elevated in patients with acute intermittent porphyria in attack or remission and in latent subjects. No significant difference could be found between the excretion in attack or remission and latency. A significant association was found in all subjects between urinary hydroxyhaemopyrrolin‐2‐one concentrations and the urinary excretion of the porphyrin precursors δ‐aminolaevulinic acid and porphobilinogen. There was also found to be a linear relationship between the excretion of δ‐aminolaevulinic acid and of porphobilinogen. Excretion of hydroxyhaemopyrrolin‐2‐one, δ‐aminolaevulinic acid and porphobilinogen was also followed in one subject throughout the duration of an acute attack. The significance of the findings are discussed in relationship to a possible role of hydroxyhaemopyrrolin‐2‐one in the manifestations of acute porphyria.