纯合子家族性高胆固醇血症的肝移植治疗。

IF 4.6 3区医学 Q2 BIOCHEMISTRY & MOLECULAR BIOLOGY

Current opinion in lipidology Pub Date : 2025-08-07 DOI:10.1097/MOL.0000000000001007

Gilbert R Thompson, Shahenaz Walji, Jaimini Cegla

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引用次数: 0

摘要

综述目的：本综述主要关注肝移植在纯合子家族性高胆固醇血症（HoFH）治疗中的作用，很少涉及肝心联合移植。最新发现：该综述收录了过去10年世界范围内发表的报告，描述了儿童和成人HoFH肝移植的基本原理和结果。它还提供了肝脏和心脏移植治疗各种其他疾病的规模信息。摘要：肝移植提供了一种比目前可用的替代方法（如采血和洛米他胺）更有效的降低LDL的方法，但同时也带来了移植后不可接受的高发病率和死亡率风险。这主要是由于终身免疫抑制药物治疗的不良影响，这限制了肝移植的使用，只有那些最佳药物治疗失败的HoFH患者。

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Liver transplantation for homozygous familial hypercholesterolaemia.

Purpose of review: The review focusses on the role of liver transplantation, and rarely combined liver and heart transplantation, in the current management of homozygous familial hypercholesterolaemia (HoFH).

Recent findings: The review features world-wide reports published during the past 10 years describing the rationale and outcomes of liver transplantation for children and adults with HoFH. It also provides information on the scale of liver and heart transplantation for a variety of other disorders.

Summary: Liver transplantation provides a more effective means of lowering LDL than currently available alternatives such as apheresis and lomitapide but carries with it an unacceptably high risk of posttransplant morbidity and mortality. This is mainly due to the adverse effects of life-long immunosuppressive drug therapy, which restricts the use of liver transplantation to those HoFH patients in whom optimal medical therapy has failed.

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来源期刊

Current opinion in lipidology 医学-内分泌学与代谢

CiteScore

6.70

自引率

4.50%

发文量

审稿时长

6-12 weeks

期刊介绍： With its easy-to-digest reviews on important advances in world literature, Current Opinion in Lipidology offers expert evaluation on a wide range of topics from six key disciplines including nutrition and metabolism, genetics and molecular biology, and hyperlipidaemia and cardiovascular disease. Published bimonthly, each issue covers in detail the most pertinent advances in these fields from the previous year. This is supplemented by a section of Bimonthly Updates, which deliver an insight into new developments at the cutting edge of the disciplines covered in the journal.