妊娠合并肝豆状核变性的孕期管理  

Pregnancy management of hepatolenticular degeneration in pregnancy

作　　者：杨桦[1] 韩肖燕[1] 张丽丽[1] Yang Hua;Han Xiaoyan;Zhang Lili(Department of Obstetrics and Gynecology,Beijing Friendship Hospital,Capital Medical University,Beijing 100050,China)

机构地区：[1]首都医科大学附属北京友谊医院妇产科,北京100050

出　　处：《中国综合临床》2022年第6期574-576,共3页Clinical Medicine of China

摘　　要：肝豆状核变性(hepatolenticular degeneration,HLD)即Wilson病,是基因异常导致肝细胞铜转运障碍的常染色体隐性遗传病。HLD患者怀孕是安全的,妊娠期间应继续驱铜治疗,根据病情减少用药剂量,做好定期监测。铜控制满意的HLD患者比未经治疗的HLD患者有更好的妊娠结局。妊娠期间的治疗应个体化,需要产科、肝病科、肝移植科、遗传咨询、药剂科、麻醉科、儿科等多学科协同进行围产期保健。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.

关 键 词：肝豆状核变性 常染色体隐性遗传病 孕期管理 

分 类 号：R714.25[医药卫生—妇产科学]