Liver transplantation for inherited metabolic liver diseases

Shen Conghuan; Wang Zhengxin

doi:10.3969/j.issn.1674-7445.2023212

Volume 15 Issue 2

Mar. 2024

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Shen Conghuan, Wang Zhengxin. Liver transplantation for inherited metabolic liver diseases[J]. ORGAN TRANSPLANTATION, 2024, 15(2): 178-184. doi: 10.3969/j.issn.1674-7445.2023212

Citation:

Shen Conghuan, Wang Zhengxin. Liver transplantation for inherited metabolic liver diseases[J]. ORGAN TRANSPLANTATION, 2024, 15(2): 178-184. doi: 10.3969/j.issn.1674-7445.2023212

Citation:

Shen Conghuan, Wang Zhengxin. Liver transplantation for inherited metabolic liver diseases[J]. ORGAN TRANSPLANTATION, 2024, 15(2): 178-184. doi: 10.3969/j.issn.1674-7445.2023212

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Liver transplantation for inherited metabolic liver diseases

doi: 10.3969/j.issn.1674-7445.2023212

Shen Conghuan,
Wang Zhengxin^,

Liver Transplantation Center, General Surgery, Huashan Hospital, Fudan University, Shanghai 200040, China

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Author Bio:
沈丛欢，复旦大学附属华山医院副主任医师，普外科肝脏移植中心副主任。参与肝脏移植手术1 500余例，其中成人肝移植1 000余例，儿童肝移植500余例。擅长活体肝移植的肝动脉显微重建及胆管重建。兼任中国医疗保健国际交流促进会肝脏移植分会委员、海峡两岸医药卫生交流协会器官移植分会委员、中国研究型医院学会加速康复外科专业委员会肝脏移植加速康复学组委员、上海市医学会肝病分会遗传代谢肝病学组委员、中国抗癌协会胆道肿瘤专业委员会青年委员、上海岩华公益基金会理事。兼任《器官移植》杂志通讯编委。作为第一作者发表核心期刊及SCI论文20余篇。获2015年上海市科技进步奖一等奖和2021年上海市医学科技奖二等奖
Corresponding author: Wang Zhengxin, Email: wangzhengxin@huashan.org.cn
Received Date: 2023-11-15
Accepted Date: 2024-01-12

Available Online: 2024-01-18

Publish Date: 2024-03-15

Abstract

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.
- Inherited metabolic liver disease,
- Tyrosinemia,
- Glycogen storage disease,
- Hepatolenticular degeneration,
- Hyperoxaluria,
- Split liver transplantation,
- Domino liver transplantation,
- Auxiliary liver transplantation

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References(48)

References

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Liver transplantation for inherited metabolic liver diseases

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Liver transplantation for inherited metabolic liver diseases

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