Liver transplantation for pediatric metabolic disease
Liver transplantation for pediatric metabolic disease
复制标题
DOI：
10.1016/j.ymgme.2014.01.006
复制
发表时间：
2014-04-01
影响因子：
3.8
通讯作者：
Vockley, Jerry
中科院分区：
文献类型：
--
作者：
Mazariegos, George;Shneider, Benjamin;Vockley, Jerry
肝移植（LTx）最初是作为已知与近期死亡率高风险相关的肝脏疾病的治疗而开发的，但基于一组不同的先天性代谢疾病的范例。随着该手术的总体结果得到改善，LTx已发展成为一种有吸引力的方法，用于各种临床情况下越来越多的代谢疾病。该手术不再仅仅是挽救生命，即使不是原发性疾病的所有症状都被消除，也可以提高生活质量。因此，随着适合LTx治疗的潜在疾病清单的增加，风险-收益比的平衡变得更加复杂。本文综述了最近在匹兹堡儿童医院UPMC举行的代谢性肝移植会议上关于肝脏或肝细胞移植在代谢性肝病治疗中的作用的报告。
Liver transplantation (LTx) was initially developed as a therapy for liver diseases known to be associated with a high risk of near-term mortality but is based upon a different set of paradigms for inborn metabolic diseases. As overall outcomes for the procedure have improved, LTx has evolved into an attractive approach for a growing number of metabolic diseases in a variety of clinical situations. No longer simply life-saving, the procedure can lead to a better quality of life even if not all symptoms of the primary disorder are eliminated. Juggling the risk-benefit ratio thus has become more complicated as the list of potential disorders amenable to treatment with LTx has increased. This review summarizes presentations from a recent conference on metabolic liver transplantation held at the Children's Hospital of Pittsburgh of UPMC on the role of liver or hepatocyte transplantation in the treatment of metabolic liver disease.