[Pregnancy management in patients with hepatolenticular degeneration].

Hepatolenticular degeneration (HLD) is an autosomal recessive genetic disease with a wide range of clinical manifestations. Women of childbearing age often present with irregular or even absent menstruation. Getting pregnant can be difficult without systematic treatment, and even if someone does become pregnant, miscarriages are common. This article reviews the use of medication during pregnancy in patients with hepatolenticular degeneration and also discusses the mode of delivery, anesthetic drug selection, and breastfeeding safety. 肝豆状核变性是一种常染色体隐性遗传病，临床表现差异很大，育龄期女性多表现为月经不规律甚至闭经，未经系统治疗者很难怀孕，即便怀孕也容易流产。现就肝豆状核变性患者孕期用药问题展开综述，同时也讨论分娩方式、麻醉药物的选择以及母乳喂养安全性等问题。.