Pregnancy management of hepatolenticular degeneration in pregnancy / 中国综合临床

ABSTRACT

Hepatolenticular degeneration (HLD) is Wilson's disease, which is an autosomal recessive inherited disease caused by gene abnormality and copper transport disorder in liver cells. The pregnancy of HLD patients is safe. During pregnancy, the treatment of eliminating copper should be continued, the dosage of drugs should be reduced according to the condition, and regular monitoring should be done. HLD patients with satisfactory copper control had better pregnancy outcomes than untreated HLD patients. The treatment during pregnancy should be individualized, and perinatal care should be coordinated by obstetrics, hepatology, liver transplantation, genetic counseling, pharmacy, anesthesiology, pediatrics and other disciplines.