儿童肝豆状核变性急性肝衰竭的诊断与治疗

方微园; 陆怡

doi:10.3969/j.issn.1001-5256.2022.02.004

儿童肝豆状核变性急性肝衰竭的诊断与治疗

DOI: 10.3969/j.issn.1001-5256.2022.02.004

方微园,
陆怡^,

复旦大学附属儿科医院儿童肝病中心, 上海 201102

利益冲突声明：所有作者均声明不存在利益冲突。

作者贡献声明：方微园负责拟定写作思路、收集整理文献以及撰写修改论文；陆怡负责指导撰写论文并审核。

详细信息

通信作者:
陆怡, luyi@fudan.edu.cn

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- 被引次数: 0
出版历程
- 收稿日期: 2021-10-25
- 录用日期: 2021-12-18
- 出版日期: 2022-02-20

Diagnosis and treatment of acute liver failure in children with Wilson's disease

Weiyuan FANG,
Yi LU^,

Center for Pediatric Liver Diseases, Children's Hospital of Fudan University, Shanghai 201102, China

More Information

Corresponding author: LU Yi, luyi@fudan.edu.cn

摘要

摘要: 急性肝衰竭(ALF)是儿童肝豆状核变性(WD)一种特殊的临床表现形式, 相对罕见但极为严重；其特征为进行性加重的黄疸和显著的凝血功能障碍, 伴急性血管内溶血, 易并发肝性脑病、急性肾衰竭等严重并发症, 一旦起病, 进展迅速, 病死率高。目前, 表现为ALF的WD缺乏单一的快速诊断指标, 早期诊断困难。既往多认为肝移植是其唯一治疗方法, 现发现非肝移植的内科治疗可使部分儿童WD-ALF获得自体肝缓解和恢复。
- 肝豆状核变性 /
- 肝功能衰竭, 急性 /
- 血浆置换 /
- 肝移植 /
- 诊断 /
- 治疗学
Abstract: Acute liver failure (ALF) is a rare and extremely severe clinical form of Wilson's disease (WD), characterized by progressive aggravation of jaundice and significant coagulation disorder with acute intravascular hemolysis. There is a high risk of severe complications such as hepatic encephalopathy and acute renal failure, and the disease progresses rapidly after onset and has a high mortality rate. At present, it is difficult to diagnose WD presenting as ALF in the early stage due to a lack of unified indicators for rapid diagnosis. Liver transplantation was considered the only effective treatment method for this disease in the past; however, recent studies have shown that medical treatment without liver transplantation can achieve autologous liver relief and recovery in some patients with WD-ALF.
- Hepatolenticular Degeneration /
- Liver Failure, Acute /
- Plasma Exchange /
- Liver Transplantation /
- Diagnosis /
- Therapeutics

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参考文献(43)

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