- AIP attacks are 4 to 5 times more common in women than in men.2
- In women, attacks occur during reproductive years, most commonly in their 30s and often during the luteal phase of the menstrual cycle.1, 2, 3
AIP is caused by a deficiency of the enzyme porphobilinogen (PBG) deaminase in the heme biosynthetic pathway. This enzyme deficiency “predisposes” patients to the effects of precipitating factors that can increase the demand for hepatic heme and lead to a chemical buildup that may cause symptoms to develop.1
The heme biosynthetic pathway can function with half-normal activity of the PBG enzyme. However, with certain precipitating factors, this pathway can become overactivated and can cause an AIP attack. Specialists in the treatment of AIP, Manisha Balwani, MS, MD, Associate Professor and Robert Desnick, Ph.D, MD, Dean for Genetics and Genomic Medicine from the Icahn School of Medicine at Mount Sinai describe the pathogenesis of AIP.Watch the Video
AIP attacks are usually due to the additive effects of several factors,1 including:
In 90 patients diagnosed with AIP in a recent observational study 6:
Reported that medications triggered acute attacks
Reported that weight-loss diets triggered acute attacks
Many drugs that are commonly prescribed to treat signs and symptoms of AIP actually increase the demand for hepatic heme and induce ALA synthase. These drugs may therefore exacerbate attacks and should be avoided.1,7 These drugs include1,5:
Lists of unsafe, possibly unsafe, and safe drugs for individuals with AIP are continuously updated.
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