α-1抗胰蛋白酶缺乏症：肝移植后的结局
Alpha-1-antitrypsin deficiency: outcomes after liver transplantation.
作者信息
Kemmer N, Kaiser T, Zacharias V, Neff G W
机构信息
Department of Internal Medicine, University of Cincinnati, Cincinnati, Ohio 45267, USA.
出版信息
Transplant Proc. 2008 Jun;40(5):1492-4. doi: 10.1016/j.transproceed.2008.02.075.
Alpha-1-antitrypsin deficiency (AAT) is the most common inherited metabolic disease leading to liver transplantation (LT) in children and adults. The aim of the study was to determine transplantation trends and survival of LT recipients with AAT. Using the UNOS (United Network for Organ Sharing) database, we identified 567 patients who underwent LT and 3 who received lung and LT from 1995 to 2004. AAT accounted for 1.06% of all adult LTs and 3.51% for pediatric LT. The 1-, 3-, and 5-year patient survival was 89%, 85%, and 83%, respectively, for adults versus 92%, 90%, and 90% for pediatric patients (P = .04), and graft survival was 83%, 79%, and 77% for adults versus 84%, 81%, and 78% for pediatric patients (P = .51). By regression analysis, age was the only predictor for patient survival (P = .04). In conclusion, adult and pediatric LT recipients with AAT are predominantly of Caucasian ethnicity and have an excellent post-LT survival.
α-1抗胰蛋白酶缺乏症（AAT）是导致儿童和成人进行肝移植（LT）的最常见遗传性代谢疾病。本研究的目的是确定AAT肝移植受者的移植趋势和生存率。利用器官共享联合网络（UNOS）数据库，我们确定了1995年至2004年间接受肝移植的567例患者以及3例接受肺移植和肝移植的患者。AAT占所有成人肝移植的1.06%，占儿童肝移植的3.51%。成人患者1年、3年和5年的生存率分别为89%、85%和83%，而儿童患者分别为92%、90%和90%（P = 0.04）；成人移植物生存率分别为83%、79%和77%，儿童患者分别为84%、81%和78%（P = 0.51）。通过回归分析，年龄是患者生存的唯一预测因素（P = 0.04）。总之，患有AAT的成人和儿童肝移植受者主要为白种人，肝移植后生存率很高。
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