Medical Definition of Intermittent acute porphyria
A group of rare inherited metabolic disorders that result from a disturbance in porphyrin metabolism, causing increased formation and excretion of porphyrin or its precursors.
It is caused by hepatic overproduction of d-aminolevulinic acid, which has greatly increased urinary excretion and of porphobilinogen, and some increase of uroporphyrin, due to a deficiency of porphobilinogen deaminase.
Clinical features: intermittent acute attacks of hypertension, abdominal colic, psychosis, and polyneuropathy, but with no photosensitivity.
It is exacerbated by the ingestion of certain drugs such as; barbiturates).
Inheritance: autosomal dominant.
(20 Sep 2002)
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