Liver transplantation for acute intermittent porphyria is complicated by a high rate of hepatic artery thrombosis.
Liver transplantation for acute intermittent porphyria is complicated by a high rate of hepatic artery thrombosis.
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DOI：
10.1002/lt.22345
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发表时间：
2012-02
影响因子：
4.6
通讯作者：
Newsome, Philip N.
中科院分区：
文献类型：
--
作者：
Dowman, Joanna K.;Gunson, Bridget K.;Mirza, Darius F.;Bramhall, Simon R.;Badminton, Mike N.;Newsome, Philip N.
急性间歇性卟啉症（AIP）是一种常染色体显性疾病，由普遍表达的卟胆色素原脱氨酶部分缺乏引起。虽然其临床表现是高度可变的，少数患者遭受复发危及生命的神经内脏发作，尽管最佳的药物治疗。由于肝脏是过量前体产生的主要来源，肝移植（LT）代表了严重影响患者的潜在有效治疗方法。使用来自英国移植登记处的数据，我们分析了在英国和爱尔兰为AIP进行的所有移植。2002年至2010年间，10例AIP患者接受了肝移植。在所有病例中，移植的指征都是复发性的、经生物化学证明的、医学上无反应的急性卟啉症发作，导致生活质量严重受损。5例患者在移植前出现了严重的神经系统疾病，如截瘫。中位随访时间为23.4个月，其中98天和26个月有2例多器官衰竭死亡。8名受者在移植后存活了3.2至109个月。移植后所有患者的生化和症状均完全缓解。然而，肝动脉血栓形成率很高（HAT; 4/10）， 1例患者需要再次移植。先前的神经损伤如关节挛缩的影响并没有通过移植得到改善。因此，存活患者的生活质量受损通常是术前并发症的结果。难治性AIP是LT的一个很好的适应症，精心挑选的患者长期预后良好。然而，在这些患者中，HAT的发生率增加，我们建议移植后常规抗血小板治疗。中华肝病杂志，2012,18(6):518 - 518。©2011 aasld。
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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