What is acute intermittent porphyria (AIP)?

Updated: Jul 08, 2020
  • Author: Thomas G DeLoughery, MD; Chief Editor: Emmanuel C Besa, MD  more...
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Answer

Acute intermittent porphyria (AIP) is one of the porphyrias, a group of hereditary diseases that involve defects in heme metabolism and result in excessive secretion of porphyrins and porphyrin precursors. [1] AIP manifests as episodes of abdominal pain, neuropathies, and constipation, but, unlike most types of porphyria, patients with AIP do not have a rash.

The diagnosis of AIP can be confirmed by finding an elevated level of porphobilinogen (>6 mg/L) on a spot urine test during an acute attack (see Workup). High doses of glucose can inhibit heme synthesis and are useful for treatment of mild attacks. Patients experiencing severe attacks, especially those with severe neurologic symptoms, should be treated with hematin. During attacks, which generally last for several days, patients require symptomatic treatment for pain and other manifestations. (See Treatment and Medication.)

For more information on the porphyrias, see Porphyria Overview.


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