Liver Transplantation From Donors With Acute Intermittent Porphyria
Authors: Joanna K. Dowman, Bridget K. Gunson, Simon Bramhall, Mike N. Badminton, Philip N. Newsome
Journal: Annals of Internal Medicine (2011)

Letters19 April 2011Liver Transplantation From Donors With Acute Intermittent PorphyriaJoanna K. Dowman, MBChB(Hons), Bridget K. Gunson, Simon Bramhall, MD, Philip N. Newsome, PhD, and Mike N. Badminton, MBChB, PhDJoanna K. Dowman, MBChB(Hons)From Queen Elizabeth Hospital Birmingham, Edgbaston, Birmingham B15 2TH, United Kingdom, and Cardiff University School of Medicine, Cardiff CF14 4XN, United Kingdom., Bridget K. GunsonFrom Queen Elizabeth Hospital Birmingham, Edgbaston, Birmingham B15 2TH, United Kingdom, and Cardiff University School of Medicine, Cardiff CF14 4XN, United Kingdom., Simon Bramhall, MDFrom Queen Elizabeth Hospital Birmingham, Edgbaston, Birmingham B15 2TH, United Kingdom, and Cardiff University School of Medicine, Cardiff CF14 4XN, United Kingdom., Philip N. Newsome, PhDFrom Queen Elizabeth Hospital Birmingham, Edgbaston, Birmingham B15 2TH, United Kingdom, and Cardiff University School of Medicine, Cardiff CF14 4XN, United Kingdom., and Mike N. Badminton, MBChB, PhDFrom Queen Elizabeth Hospital Birmingham, Edgbaston, Birmingham B15 2TH, United Kingdom, and Cardiff University School of Medicine, Cardiff CF14 4XN, United Kingdom.Author, Article, and Disclosure Informationhttps://doi.org/10.7326/0003-4819-154-8-201104190-00015 SectionsAboutFull TextPDF ToolsAdd to favoritesDownload CitationsTrack CitationsPermissions ShareFacebookTwitterLinkedInRedditEmail Background: Acute intermittent porphyria (AIP) results from partial deficiency of the enzyme porphobilinogen deaminase (1). The clinical disease includes life-threatening neurovisceral attacks from neuronal damage. The cause of these attacks has not been established but involves the heme precursor δ-aminolevulinic acid (ALA) (2). The liver is the major source of excess ALA production, and transplanting a normal liver into a patient with AIP effectively resolves this disease (3).Because clinical expression of AIP is more common in premenopausal women, we hypothesized that a liver removed from a premenopausal woman who is receiving a normal liver allograft could safely be transplanted ...References1. Puy H, Gouya L, Deybach JC. Porphyrias. Lancet. 2010;375:924-37. [PMID: 20226990] CrossrefMedlineGoogle Scholar2. Meyer UA, Schuurmans MM, Lindberg RL. Acute porphyrias: pathogenesis of neurological manifestations. Semin Liver Dis. 1998;18:43-52. [PMID: 9516677] CrossrefMedlineGoogle Scholar3. Soonawalla ZF, Orug T, Badminton MN, Elder GH, Rhodes JM, Bramhall SR, et al. Liver transplantation as a cure for acute intermittent porphyria. Lancet. 2004;363:705-6. [PMID: 15001330] CrossrefMedlineGoogle Scholar4. Wilczek HE, Larsson M, Yamamoto S, Ericzon BG. Domino liver transplantation. J Hepatobiliary Pancreat Surg. 2008;15:139-48. [PMID: 18392706] CrossrefMedlineGoogle Scholar5. Unzu C, Sampedro A, Maulen I, Vanrell L, Dubrot J, de Salamanca RE, et al. Porphobilinogen deaminase over-expression in hepatocytes, but not in erythrocytes, prevents accumulation of toxic porphyrin precursors in a mouse model of acute intermittent porphyria. J Hepatol. 2010;52:417-24. [PMID: 19815305] CrossrefMedlineGoogle Scholar Author, Article, and Disclosure InformationAffiliations: From Queen Elizabeth Hospital Birmingham, Edgbaston, Birmingham B15 2TH, United Kingdom, and Cardiff University School of Medicine, Cardiff CF14 4XN, United Kingdom.Disclosures: Dr. Dowman: Grant received: Wellcome Trust. Grant received (money to institution): Wellcome Trust. Grant pending: Wellcome Trust. 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