肝豆状核变性（Wilson病，WD）是一种以铜代谢障碍为特征的常染色体隐性遗传病。它是由ATP7B基因突变引起，导致铜从胆汁排泄障碍，进而在肝脏和脑等部位病理性沉积。早期诊断、早期治疗可以显著改善患者预后。但目前关于妊娠期WD的治疗和管理仍未达成明确共识。现对妊娠期WD的临床管理进行总结，以供临床医生参考。“,”Hepatolenticular degeneration (Wilson’s disease, WD) is a kind of autosomal recessive genetic disease characterized by disorders of copper metabolism. It is caused by mutations in the ATP7B gene, resulting in impaired excretion of copper into the bile, and then pathological deposition in the liver, brain, and other organs. Early diagnosis and treatment can significantly improve the prognosis of patients with WD. However, there is still no clear consensus on the treatment and management of WD during pregnancy. Herein, the clinical management of WD during pregnancy is summarized for clinicians\' reference.