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妊娠期肝内胆汁淤积症

Intrahepatic cholestasis of pregnancy.

作者信息

Pusl Thomas, Beuers Ulrich

机构信息

Department of Gastroenterology & Hepatology, AMC, University of Amsterdam, The Netherlands.

出版信息

Orphanet J Rare Dis. 2007 May 29;2:26. doi: 10.1186/1750-1172-2-26.

Abstract

Intrahepatic cholestasis of pregnancy (ICP) is a cholestatic disorder characterized by (i) pruritus with onset in the second or third trimester of pregnancy, (ii) elevated serum aminotransferases and bile acid levels, and (iii) spontaneous relief of signs and symptoms within two to three weeks after delivery. ICP is observed in 0.4-1% of pregnancies in most areas of Central and Western Europe and North America, while in Chile and Bolivia as well as Scandinavia and the Baltic states roughly 5-15% and 1-2%, respectively, of pregnancies are associated with ICP. Genetic and hormonal factors, but also environmental factors may contribute to the pathogenesis of ICP. Intrahepatic cholestasis of pregnancy increases the risk of preterm delivery (19-60%), meconium staining of amniotic fluid (27%), fetal bradycardia (14%), fetal distress (22-41%), and fetal loss (0.4-4.1%), particularly when associated with fasting serum bile acid levels > 40 micromol/L. The hydrophilic bile acid ursodeoxycholic acid (10-20 mg/kg/d) is today regarded as the first line treatment for intrahepatic cholestasis of pregnancy. Delivery has been recommended in the 38th week when lung maturity has been established.

摘要

妊娠肝内胆汁淤积症(ICP)是一种胆汁淤积性疾病,其特征为:(i)瘙痒在妊娠中晚期出现;(ii)血清转氨酶和胆汁酸水平升高;(iii)分娩后两到三周内症状和体征自行缓解。在中欧、西欧和北美的大部分地区,0.4%-1%的妊娠会出现ICP,而在智利、玻利维亚以及斯堪的纳维亚和波罗的海国家,分别约有5%-15%和1%-2%的妊娠与ICP有关。遗传因素、激素因素以及环境因素都可能导致ICP的发病机制。妊娠肝内胆汁淤积症会增加早产(发生率为19%-60%)、羊水粪染(27%)、胎儿心动过缓(14%)、胎儿窘迫(22%-41%)和胎儿死亡(0.4%-4.1%)的风险,尤其是当空腹血清胆汁酸水平>40微摩尔/升时。亲水性胆汁酸熊去氧胆酸(10-20毫克/千克/天)如今被视为妊娠肝内胆汁淤积症的一线治疗药物。当确定肺成熟时,建议在第38周分娩。

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