AIP

AIP acute intermittent porphyria.

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acute intermittent porphyria (AIP),

an autosomal-dominant, genetically transmitted metabolic hepatic disorder characterized by acute attacks of neurologic dysfunction that can be started by environmental or endogenous factors. Women are affected more frequently than men, and attacks often are precipitated by starvation or severe dieting, alcohol ingestion, bacterial or viral infections, and a wide range of pharmaceutic products. Any part of the nervous system can be affected, and an initial common effect is mild to severe abdominal pain. Other effects can include tachycardia, hypertension, hyponatremia, peripheral neuropathy, and organic brain dysfunction marked by seizures, coma, hallucinations, and respiratory paralysis. A frequent diagnostic factor is a high level of porphyrin precursors in the urine, which usually increases during periods of acute attacks. Treatment is generally symptomatic, with emphasis on respiratory support, beta-blockers, and pain control. Education of the patient focuses on environmental factors, particularly medications such as barbiturates, that are known to cause an onset of symptoms, as well as avoidance of alcohol, sunlight, and skin trauma. A high-carbohydrate diet is reported to reduce the risk of acute attacks because glucose tends to block the induction of hepatic gamma-aminolevulinic acid synthetase, an enzyme involved in the porphyrias. See also porphyria.

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AIP

Acute intermittent porphyria