Diagnosis and treatment of acute liver failure in children with Wilson's disease
10.3969/j.issn.1001-5256.2022.02.004
- VernacularTitle:儿童肝豆状核变性急性肝衰竭的诊断与治疗
- Author:
Weiyuan FANG
1
;
Yi LU
1
Author Information
1. Center for Pediatric Liver Diseases, Children's Hospital of Fudan University, Shanghai 201102, China
- Publication Type:Discussions by Experts
- Keywords:
Hepatolenticular Degeneration;
Liver Failure, Acute;
Plasma Exchange;
Liver Transplantation;
Diagnosis;
Therapeutics
- From:
Journal of Clinical Hepatology
2022;38(2):268-272
- CountryChina
- Language:Chinese
-
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.
