What's UP Doc? Is a monthly column where we feature a patient question along with a response from a member of the UPA Scientific Advisory Board.
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Thank you so much for the wonderful follow up question. UPA Scientific Advisory Board member Dr. Herbert L. Bonkovsky provided his response below.
“Most patients with AIP have normal or near-normal livers and they do not suffer from progressive liver disease or its complications. However, there is a modestly increased risk of their developing chronic liver disease and primary liver cancer [hepatocellular carcinoma, HCC], especially with advancing age. The risks of these disorders are likely greater among patients with biochemically active AIP: those who chronically over produce and over-excrete delta-aminolevulinic acid [ALA] and porphobilinogen [PBG]. It seems likely that the chief potential toxic intermediate is ALA, which causes increased oxidative stress.
We recommend that patients with AIP take good care of their livers: they should receive vaccinations to protect them from developing hepatitis A and B, for which there are safe and effective vaccines; they should not drink alcohol to excess—for women this means not more than one drink per day; for men, not more than two. They should avoid obesity and hyperlipidemia, both of which are associated with fatty liver disease, as is excess alcohol. Drugs and herbals and dietary supplements, especially concentrated extracts of green tea, are also hepatotoxic in some susceptible persons, so we recommend caution in their use and only for clearly indicated medical reasons, not for purposes of 'general health' or for 'weight loss.' Keeping other approved vaccinations complete and up-to-date, such as DPT, HPV, yearly flu, Covid-19 with boosters, etc, is strongly recommended.
Acute porphyric attacks should be treated promptly and vigorously, as recommended in our general guidelines:
It is good medical practice for AIP patients to have yearly tests of hepatic and kidney status with comprehensive metabolic panels. If there are abnormalities detected, further assessments by hepatologists and/or nephrologists with knowledge of AIP are likely indicated. Beginning at age 50 years, we recommend regular and ongoing screening for the development of HCC with liver ultrasound, CT, or MRI every 6 months. Fortunately, if HCC develop in patients with AIP and it, they are detected early, before they have become very extensive or have spread to other organs, they generally can be removed surgically with good long-term survivals.