LT-11-056 liver transplantation for acute intermittent porphyria is complicated by a high rate of hepatic artery thrombosis.

Joanna Dowman, Bridget Gunson, DF Mirza, Simon Bramhall, MN Badminton, Philip Newsome

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Acute intermittent porphyria (AIP) is an autosomal dominantly inherited condition which results from partial deficiency of the ubiquitously expressed enzyme porphobilinogen (PBG) deaminase. Although clinical expression is highly variable, a minority of patients suffer recurrent life-threatening neurovisceral attacks despite optimal medical therapy. As the liver is the major source of excess precursor production, liver transplantation (LT) represents a potentially effective treatment for severely affected patients. We analysed all transplants performed for AIP in the UK and Ireland using data from the UK Transplant Registry. Between 2002-2010, 10 patients underwent LT for AIP. In all cases indication for transplant was recurrent, biochemically proven, medically non-responsive acute attacks of porphyria resulting in significantly impaired quality of life. Five patients had developed significant neurological morbidity such as paraplegia before transplant. Median follow-up was 23.4 months, with two deaths at 98 days and 36 months from multiorgan failure. Eight recipients are alive at 3.2-109 months after transplant. Complete biochemical and symptomatic resolution was observed in all patients following transplantation. However, there was a high rate of hepatic artery thrombosis (4/10), with one patient requiring re-grafting. Consequences of previous neuronal damage, such as joint contractures, were not improved by transplantation. Thus in surviving patients impaired quality of life was usually a consequence of pre-operative complications. Refractory AIP is an excellent indication for LT with good long-term outcomes in carefully selected patients. There is however, an increased incidence of hepatic artery thrombosis in such patients, and we would recommend routine antiplatelet therapy post-transplantation. © 2011 American Association for the Study of Liver Diseases.
Original languageEnglish
JournalLiver Transplantation
Publication statusPublished - 26 May 2011


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