肝移植治疗家族性高胆固醇血症的临床研究进展  

Clinical research advances of liver transplantation for patients with familial hypercholesterolemia

作　　者：张磊[1] 宋少华 傅志仁 Zhang Lei;Song Shaohua;Fu Zhiren(Department of General Surgery,Affiliated Ruijin Hospital,Shanghai Jiao Tong University,School of Medicine,Shanghai 200025,China)

机构地区：[1]上海交通大学医学院附属瑞金医院普外科,上海200025

出　　处：《中华器官移植杂志》2023年第2期112-116,共5页Chinese Journal of Organ Transplantation

基　　金：国家自然科学基金(82270681、81970563)。

摘　　要：家族性高胆固醇血症(familial hypercholestemlemia,FH)是一种常染色体显性遗传病,纯合子型患者在青少年阶段即可出现严重的动脉硬化性心血管疾病,多于20~30岁前死亡。肝移植可以纠正肝细胞上低密度脂蛋白受体的缺失或功能障碍,恢复正常的脂蛋白代谢,对于延缓疾病进展、改善患者预后有确切效果。然而,由于肝移植治疗FH病例数稀少,移植中心往往缺乏临床实践经验。本文将对FH患者接受肝移植治疗的手术适应证、手术时机、供体来源、临床疗效、移植并发症、病肝再利用等重要问题进行分析、总结,以期为该病临床治疗决策提供参考。Familial hypercholesterolemia(FH)is an autosomal dominant disease.Homozygous FH patients tend to have a high incidence of severe arteriosclerotic cardiovascular disease during adolescence and die at ages of 20-30.Liver transplantation(LT)may correct the dysfunction of LDL receptor on hepatocytes,restore normal lipoprotein metabolism,arrest disease progression and improving patient outcomes.However,due to a great rarity of LT for FH,domestic transplantation centers lack the relevant clinical experiences.This review summarized some important issues of FH patients undergoing LT,such as indications,timing,donor sources,efficacies,complications and reusing diseased liver.The goal was to provide practical references for managing FH.

关 键 词：家族性高胆固醇血症 肝移植 治疗 

分 类 号：R657.3[医药卫生—外科学] R589.2[医药卫生—临床医学]