暴发性肝衰竭型肝豆状核变性的诊治进展

Progress in diagnosis and treatment of fulminant Wilson’s disease

肝豆状核变性(Wilson’s disease,WD),又名威尔森氏病,是一种铜代谢障碍的常染色体隐性遗传疾病。肝损伤症状是WD的主要临床表现形式,其中暴发性肝衰竭型肝豆状核变性(fulminant Wilson’s disease,FWD)是WD临床上的一种特殊类型,其具有病情进展迅速、早期诊断困难、病死率高等特点,早期的识别并采取积极治疗措施,直接影响患者的预后。

Hepatolenticular degeneration,also known as Wilson’s disease(WD),is an autosomal recessive inherited disorder of copper metabolism.Symptoms of liver injury are the main clinical manifestations of WD.Fulminant Wilson’s disease(FWD)is a special clinical type of WD,which is characterized by rapid progression,difficult early diagnosis and high mortality.Early identification and active treatment directly affect the prognosis of patients.