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脊髓性肌萎缩症女性的妊娠结局:综述。

Pregnancy outcomes in women with spinal muscular atrophy: A review.

机构信息

Dino Ferrari Centre, Neuroscience Section, Department of Pathophysiology and Transplantation (DEPT), University of Milan, Neurology Unit, IRCCS Foundation Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.

Dino Ferrari Centre, Neuroscience Section, Department of Pathophysiology and Transplantation (DEPT), University of Milan, Neurology Unit, IRCCS Foundation Ca' Granda Ospedale Maggiore Policlinico, Milan, Italy.

出版信息

J Neurol Sci. 2018 May 15;388:50-60. doi: 10.1016/j.jns.2018.03.001. Epub 2018 Mar 2.

DOI:10.1016/j.jns.2018.03.001
PMID:29627031
Abstract

Spinal muscular atrophy (SMA) is an autosomal recessive disease characterized by muscle weakness and atrophy resulting from progressive degeneration and loss of the anterior horn cells in the spinal cord and brain stem nuclei. The onset of weakness ranges from prenatal age to young adulthood. Thus, many female patients reach fertile age and may consider getting pregnant. However, only little information is available about outcomes and complications of pregnancy in women with SMA. In this review, we compared different studies on the subject, then we analyzed outcomes in the different stages of the pregnancy (preconceptional period, embryonal period, fetal period, delivery and post partum), with a special focus on maternal and fetal complications, prematurity, mode of delivery, anesthesiological risk, respiratory function and influence of pregnancy on the disease course. This is the first review focused exclusively on pregnancy in women affected by SMA. Our aim is to help clinicians who wish to understand the risks connected with pregnancy in SMA patients and to manage pregnancy course and delivery in an evidence-based and patient-oriented manner.

摘要

脊髓性肌萎缩症(SMA)是一种常染色体隐性疾病,其特征是肌肉无力和萎缩,这是由于脊髓和脑干核中的前角细胞进行性退化和丧失所致。发病的起始年龄从胎儿期到青年期不等。因此,许多女性患者达到生育年龄,可能会考虑怀孕。但是,关于 SMA 女性怀孕的结局和并发症的信息却很少。在这篇综述中,我们比较了关于该主题的不同研究,然后分析了妊娠不同阶段(孕前、胚胎期、胎儿期、分娩和产后)的结局,特别关注母婴并发症、早产、分娩方式、麻醉风险、呼吸功能以及妊娠对疾病进程的影响。这是第一篇专门针对 SMA 女性怀孕的综述。我们的目的是帮助希望了解 SMA 患者妊娠相关风险并以循证和以患者为中心的方式管理妊娠过程和分娩的临床医生。

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