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

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.