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重症肌无力患者妊娠:来自德国一家大型三级护理中心的母婴结局回顾性分析。

Pregnancy in myasthenia gravis: a retrospective analysis of maternal and neonatal outcome from a large tertiary care centre in Germany.

机构信息

Department of Neurology, Neuroscience Clinical Research Center (NCRC) and Integrated Myasthenia Gravis Center, Charité - Universitätsmedizin, Corporate Member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Charitéplatz 1, 10117, Berlin, Germany.

Center for Stroke Research Berlin, Charité - Universitätsmedizin Berlin, Charitéplatz 1, 10117, Berlin, Germany.

出版信息

Arch Gynecol Obstet. 2024 Jul;310(1):277-284. doi: 10.1007/s00404-024-07436-y. Epub 2024 Mar 16.

Abstract

PURPOSE

Myasthenia gravis (MG) is a rare, potentially life-threatening autoimmune disease with fluctuating muscle weakness frequently affecting women of childbearing age. MG can affect maternal as well as neonatal outcome with risk of worsening of myasthenic symptoms in the mothers and risk of transient neonatal myasthenia gravis (TNMG) and arthrogryposis multiplex congenita (AMC) or foetal acetylcholine receptor antibody-associated disorders (FARAD) in the neonates.

METHODS

Retrospective analysis of maternal and neonatal outcome in a cohort of pregnant MG patients treated at a tertiary care centre in Germany.

RESULTS

Overall, 66 pregnancies were analysed. During 40 (63%) pregnancies, women experienced a worsening of myasthenic symptoms, of whom 10 patients (15.7%) needed acute therapy with IVIg or plasma exchange. There was no case of myasthenic crisis. Rate of caesarean section was comparable to the overall C-section rate at our centre (38% vs. 40%). However, there was a slightly higher rate for operative vaginal delivery (15% vs. 10%) as potential indicator for fatiguing striated musculature in MG patients during the expulsion stage. Rate of TNMG as well as AMC was 3% (two cases each).

CONCLUSIONS

Maternal and neonatal outcome in our cohort was favourable with a low rate of myasthenic exacerbations requiring acute therapies and a low rate of TNMG and AMC/FARAD. Our data might help neurologists and obstetricians to advice MG patients with desire to have children.

摘要

目的

重症肌无力(MG)是一种罕见的、潜在危及生命的自身免疫性疾病,常伴有波动性肌肉无力,好发于育龄期女性。MG 可影响母婴结局,母亲可能出现肌无力症状恶化,新生儿可能出现短暂性新生儿重症肌无力(TNMG)、先天性多发关节弯曲症(AMC)或胎儿乙酰胆碱受体抗体相关疾病(FARAD)。

方法

对德国一家三级医疗中心治疗的一组妊娠 MG 患者的母婴结局进行回顾性分析。

结果

共分析了 66 例妊娠。40 例(63%)妊娠期间,患者肌无力症状恶化,其中 10 例(15.7%)需要 IVIg 或血浆置换急性治疗。无肌无力危象病例。剖宫产率与我院中心的总体剖宫产率相当(38%比 40%)。然而,由于 MG 患者在分娩阶段的横纹肌容易疲劳,产道手术分娩率(15%比 10%)略高。TNMG 和 AMC 的发生率均为 3%(各两例)。

结论

本研究队列的母婴结局良好,肌无力恶化需要急性治疗的比例较低,TNMG 和 AMC/FARAD 的发生率较低。我们的数据可能有助于神经科医生和妇产科医生为有生育意愿的 MG 患者提供建议。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/ee42/11168978/f94d31489c29/404_2024_7436_Fig1_HTML.jpg

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