Orthotopic liver transplantation for hepatic-based metabolic disorders
Orthotopic liver transplantation for hepatic-based metabolic disorders
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原位肝移植治疗肝脏代谢性疾病
DOI：
10.1111/j.1432-2277.1995.tb01704.x
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发表时间：
1995
影响因子：
3.1
通讯作者：
K. Tan
中科院分区：
文献类型：
--
作者：
M. Rela;P. Muiesan;N. Heaton;M. Corbally;H. Hajj;A. Mowat;R. Williams;K. Tan
1989年1月至1993年6月，国王学院医院共进行了470例肝移植手术。在34例以肝为基础的代谢性疾病患者中进行了37例移植。有16名女性和18名男性，中位年龄为19岁（范围1个月至62岁）。有14名16岁以下的患者。肝移植的适应症为Wilson病（n=16）、α 1-抗胰蛋白酶缺乏症（n=10）、酪氨酸血症（n=2）、原发性高尿酸1型（PH 1; n=2）、先天性血色病（n=1）、家族性淀粉样多发性神经病（FAP; n=1，家族性高胆固醇血症）（n=1）和Crigler-Najjar综合征I型（CNS 1; n=1）。其中包括两名分别因家族性高胆固醇血症和FAP接受联合心脏-肝脏移植的患者。两名患者接受了肝肾联合移植治疗PH 1。有四人死亡：败血症（n=2）、左外侧段移植物中的急性肝静脉阻塞（n=1）和门静脉血栓形成伴肝坏死（n=1）。3例患者再次移植，1例慢性排斥反应，2例肝动脉血栓形成，平均随访34个月（范围10-64个月），移植物总存活率为81%，患者存活率为88%（30/34）。
Between January 1989 and June 1993, a total of 470 liver transplantations were performed at King's College Hospital. Thirty-seven transplantations were performed in 34 patients with liver-based metabolic disease. There were 16 females and 18 males with a median age of 19 years (range 1 month to 62 years). There were 14 patients under 16 years of age. The indications for liver transplantation were Wilson's disease (n=16), alpha 1-antitrypsin deficiency (n=10), tyrosinaemia (n=2), primary hyperoxaluria type 1 (PH1; n=2), congenital haemochromatosis (n=1), familial amyloidotic polyneuropathy (FAP; n=1, familial hypercholesterolaemia) (n=1) and Crigler-Najjar syndrome type I (CNS1; n=1). These included two patients who received combined heart-liver grafts for familial hypercholesterolaemia and FAP, respectively. Two patients received combined liver-kidney transplants for PH1. There were four deaths: from sepsis (n=2), acute hepatic vein obstruction in a left lateral segment graft (n=1) and portal vein thrombosis with liver necrosis (n=1). Three patients were retransplanted, one for chronic rejection and two for hepatic artery thrombosis, giving an overall graft survival of 81% and patient survival of 88% (30/34), at a mean follow-up of 34 months (range 10–64 months).