Research progress of liver transplantation in the treatment of hepatolenticular degeneration
10.3760/cma.j.cn115396-20240708-00214
- VernacularTitle:肝移植治疗肝豆状核变性的研究进展
- Author:
Xinghua HUANG
1
;
Jianyong LIU
;
Huanzhang HU
;
Lizhi LYU
;
Yi JIANG
;
Qiucheng CAI
Author Information
1. 福建医科大学福总临床医学院 联勤保障部队第九〇〇医院肝胆胰外科暨肝移植中心，福州 350025
- Keywords:
Liver transplantation;
Hepatolenticular degeneration;
Metabolic diseases;
Treatment outcome
- From:
International Journal of Surgery
2024;51(8):566-570
- CountryChina
- Language:Chinese
-
Abstract:
Hepatolenticular degeneration is kind of an autosomal recessive genetic disease with diverse, complex and non-specific clinical manifestations, high misdiagnosis rate, rapid disease progression, poor drug treatment effect, and high mortality. It is one of the rare several genetic metabolic diseases in clinic that could be cured by liver transplantation method. Liver transplantation provides healthy P-type ATP enzyme through the donor liver, which can correct its genetic defects, improve copper metabolism disorders, relieve clinical symptoms, improve the quality of life, and improve the survival rate of patients. Liver transplantation is playing an increasingly important role as an important means to treat hepatolenticular degeneration. With the rapid development of partial living donor liver transplantation, auxiliary liver transplantation, domino-assisted liver transplantation and cross-assisted domino liver transplantation, a new way has been provided for patients with hepatolenticular degeneration, alleviating the problem of donor liver shortage and shortening the waiting time of recipients, which has certain clinical value and development prospects. In this paper, a review of the research progress in the treatment of hepatolenticular degeneration with liver transplantation was made with reference to the relevant literature at home and abroad.
