医学
肝移植
铜代谢
威尔逊病
移植
疾病
表型
肝病
内科学
重症监护医学
基因
生物
有机化学
化学
铜
生物化学
作者
Alberto Ferrarese,Patrizia Burra
标识
DOI:10.1177/15269248211002806
摘要
Liver transplantation is considered an effective therapeutic option for Wilson's disease (WD) patients with hepatic phenotype, since it removes the inherited defects of copper metabolism, and is associated with excellent graft and patient outcomes. The role of liver transplantation in WD patients with mixed hepatic and neuropsychiatric phenotype has remained controversial over time, mainly because of high post-operative complications, reduced survival and a variable, unpredictable rate of neurological improvement. This article critically discusses the recently published data in this field, focussing in more detail on isolated neuropsychiatric phenotype as a potential indication for liver transplantation in WD patients.
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