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[马方综合征与妊娠。附病例报告]

[Marfan's disease and pregnancy. Apropos of a case].

作者信息

Criscuolo J L, Chansiguad J P, Ducroz B, Marechaud M, Magnin G, de Tourris H

机构信息

Service de Gynécologie-Obstétrique, Hôpital la Milétrie, CHU Poitiers.

出版信息

J Gynecol Obstet Biol Reprod (Paris). 1987;16(8):1039-44.

PMID:3330098
Abstract

Marfan's syndrome is a dominant autosome transmitted disease with different degrees of seriousness. When it is associated with pregnancy there is a considerable increase in the rate of cardio-vascular complications which are responsible for a serious rate of maternal mortality due to aortic dissection. It is therefore very important that the patient, before starting a pregnancy, should be examined very carefully clinically and with ultrasound. A woman with dilatation of the aorta less then 40 mm as measured ultrasonically and who has no major clinical signs can start a pregnancy but even then she should be considered as a high risk case. In other cases therapeutic termination of pregnancy or prophylactic surgical treatment of the condition should be considered. Apart from the serious genetic risks (50%) the risks for the fetus are dominated by prematurity and by fetal death resulting from maternal death.

摘要

马凡氏综合征是一种常染色体显性遗传病,病情严重程度不一。当它与妊娠相关时,心血管并发症的发生率会显著增加,这些并发症是导致因主动脉夹层引起的孕产妇严重死亡率的原因。因此,非常重要的是,患者在怀孕前应进行非常仔细的临床检查和超声检查。超声测量主动脉直径小于40毫米且无重大临床症状的女性可以怀孕,但即便如此,她仍应被视为高危病例。在其他情况下,应考虑治疗性终止妊娠或对该病症进行预防性手术治疗。除了严重的遗传风险(50%)外,胎儿的风险主要是早产和因母亲死亡导致的胎儿死亡。

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