• Open Access

Liver transplantation for acute intermittent porphyria is complicated by a high rate of hepatic artery thrombosis

Authors

  • Joanna K. Dowman,

    Corresponding author
    1. Liver Unit, Queen Elizabeth Hospital Birmingham, Birmingham, United Kingdom
    2. NIHR Biomedical Research Unit and Centre for Liver Research, University of Birmingham, Birmingham, United Kingdom
    • NIHR Biomedical Research Unit and Centre for Liver Research, University of Birmingham, Wolfson Drive, Edgbaston, Birmingham, B15 2TH, UK
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    • Telephone: 0121 4158692; FAX: 0121 4158701

  • Bridget K. Gunson,

    1. Liver Unit, Queen Elizabeth Hospital Birmingham, Birmingham, United Kingdom
    2. NIHR Biomedical Research Unit and Centre for Liver Research, University of Birmingham, Birmingham, United Kingdom
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  • Darius F. Mirza,

    1. Liver Unit, Queen Elizabeth Hospital Birmingham, Birmingham, United Kingdom
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  • Simon R. Bramhall,

    1. Liver Unit, Queen Elizabeth Hospital Birmingham, Birmingham, United Kingdom
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  • Mike N. Badminton,

    1. Department of Infection, Immunity, and Biochemistry, School of Medicine, Cardiff University, Cardiff, United Kingdom
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  • Philip N. Newsome,

    1. Liver Unit, Queen Elizabeth Hospital Birmingham, Birmingham, United Kingdom
    2. NIHR Biomedical Research Unit and Centre for Liver Research, University of Birmingham, Birmingham, United Kingdom
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  • on behalf of the UK Liver Selection and Allocation Working Party

    1. Liver Unit, Queen Elizabeth Hospital Birmingham, Birmingham, United Kingdom
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  • Joanna K. Dowman is a clinical research fellow at the Wellcome Trust and is, therefore, receiving funding from the Wellcome Trust for liver research unrelated to this study.

  • Re-use of this article is permitted in accordance with the Terms and Conditions set out at http://wileyonlinelibrary.com/onlineopen#OnlineOpen_Terms

Abstract

Acute intermittent porphyria (AIP) is an autosomal-dominant condition resulting from a partial deficiency of the ubiquitously expressed enzyme porphobilinogen deaminase. Although its clinical expression is highly variable, a minority of patients suffer recurrent life-threatening neurovisceral attacks despite optimal medical therapy. Because the liver is the major source of excess precursor production, liver transplantation (LT) represents a potentially effective treatment for severely affected patients. Using data from the UK Transplant Registry, we analyzed all transplants performed for AIP in the United Kingdom and Ireland. Between 2002 and 2010, 10 patients underwent LT for AIP. In all cases, the indication for transplantation was recurrent, biochemically proven, medically nonresponsive acute attacks of porphyria resulting in significantly impaired quality of life. Five patients had developed significant neurological morbidities such as paraplegia before transplantation. The median follow-up time was 23.4 months, and there were 2 deaths from multiorgan failure at 98 days and 26 months. Eight recipients were alive for 3.2 to 109 months after transplantation. Complete biochemical and symptomatic resolution was observed in all patients after transplantation. However, there was a high rate of hepatic artery thrombosis (HAT; 4/10), with 1 patient requiring regrafting. The effects of previous neuronal damage such as joint contractures were not improved by transplantation. Thus, impaired quality of life in the surviving patients was usually a result of preoperative complications. Refractory AIP is an excellent indication for LT, and long-term outcomes for carefully selected patients are good. There is, however, an increased incidence of HAT in these patients, and we recommend routine antiplatelet therapy after transplantation. Liver Transpl 18:195–200, 2012. © 2011 AASLD.

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