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Review
. 2021 Feb;21(2):66-74.
doi: 10.1016/j.bjae.2020.09.005. Epub 2020 Dec 9.

Acute hepatic porphyria and anaesthesia: a practical approach to the prevention and management of acute neurovisceral attacks

N Wilson-Baig  1   2 , M Badminton  3 , D Schulenburg-Brand  3
Affiliations
Review

Acute hepatic porphyria and anaesthesia: a practical approach to the prevention and management of acute neurovisceral attacks

N Wilson-Baig et al. BJA Educ. 2021 Feb.
No abstract available

Keywords: acute attack; acute hepatic porphyria; anaesthetic risks; perioperative.

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Conflict of interest statement

The authors declare that they have no conflicts of interest.

Figures

Fig 1
Fig 1
Haem biosynthesis pathway. Porphyrias in bold present with acute neurovisceral attacks. Two acute porphyrias, variegate porphyria and hereditary coproporphyria, can present with acute attacks, photosensitivity or both. HMB is a linear tetrapyrrole, made from four porphobilinogen molecules, which forms the first of the cyclic ring structures, uroporphyrinogen III. ALA, aminolaevulinic acid; CoA, coenzyme A; HMB, hydroxymethylbilane; PBG, porphobilinogen. #Gain of function mutations in the ALA synthase 2 gene results in increased synthesis of free protoporphyrin IX in erythroid cells causing acute photosensitivity and not acute attacks.
See this image and copyright information in PMC

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