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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.
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References

    1. Bissell D.M., Anderson K.E., Bonkovsky H.L. Porphyria. N Engl J Med. 2017;377:862–872. - PubMed
    1. Findley H., Philips A., Cole D., Nair A. Porphyrias: implications for anaesthesia, critical care, and pain medicine. Contin Educ Anaesth Crit Care Pain. 2012;12:128–133.
    1. Stein P.E., Badminton M.N., Rees D.C. Update review of the acute porphyrias. Br J Haematol. 2017;176:527–538. - PubMed
    1. Elder G., Harper P., Badminton M., Sandberg S., Deybach J.C. The incidence of inherited porphyrias in Europe. J Inherit Metab Dis. 2013;36:849–857. - PubMed
    1. Chen B., Solis-Villa C., Hakenberg J. Acute intermittent porphyria: predicted pathogenicity of HMBS variants indicates extremely low penetrance of the autosomal dominant disease. Hum Mutat. 2016;37:1215–1222. - PMC - PubMed