Liver transplantation for inherited metabolic liver diseases
10.3969/j.issn.1674-7445.2023212
- VernacularTitle:遗传代谢性肝病的肝移植治疗
- Author:
Conghuan SHEN
1
;
Zhengxin WANG
1
Author Information
1. Liver Transplantation Center, General Surgery, Huashan Hospital, Fudan University, Shanghai 200040, China.
- Publication Type:ExpertForum
- Keywords:
Inherited metabolic liver disease;
Tyrosinemia;
Glycogen storage disease;
Hepatolenticular degeneration;
Hyperoxaluria;
Split liver transplantation;
Domino liver transplantation;
Auxiliary liver transplantation
- From:
Organ Transplantation
2024;15(2):178-184
- CountryChina
- Language:Chinese
-
Abstract:
Inherited metabolic liver disease (IMLD) is a category of liver metabolic diseases caused by genetic disorders. The pathogenesis of IMLD is complex, which primarily comprises the accumulation of harmful metabolic substrates or products caused by specific enzyme defects and energy defects or abnormal deposition caused by abnormal metabolism of glucose, fat and other substances. In recent years, liver transplantation has played an increasingly critical role in the treatment of IMLD with the development of liver transplantation. At present, IMLD has become the second most important indication after biliary atresia in pediatric liver transplantation. Currently, IMLD patients receiving liver transplantation can be divided into two categories: the first category is IMLD complicated with liver disease; Category 2 patients have a normal liver structure but are deficient in related metabolic enzymes. It can not only replace the liver with abnormal structure and function, but also provide normal enzymes required for patients' metabolism, which may improve their quality of life and even save their lives. In this article, common feasible liver transplantation for IMLD, clinical prognosis and surgical procedures of liver transplantation for IMLD were reviewed, aiming to provide reference for liver transplantation for IMLD.
