夏科-马里-图思病及相关疾病患者的妊娠与分娩
Pregnancy and delivery in patients with Charcot-Marie-Tooth disease and related disorders.
作者信息
Skorupinska Mariola, Ramdharry Gita, Byrne Bridgette, Laurá Matilde, Reilly Mary M
机构信息
Centre for Neuromuscular Diseases, UCL Queen Square Institute of Neurology, University College London, London, UK.
Coombe Women and Infants University Hospital, Dublin, Ireland.
出版信息
Obstet Med. 2023 Jun;16(2):83-87. doi: 10.1177/1753495X221107328. Epub 2022 Jun 16.
BACKGROUND
Charcot-Marie-Tooth disease is the most common inherited peripheral neuropathy and many patients with Charcot-Marie-Tooth are women of childbearing age. Guidelines for managing pregnancy in Charcot-Marie-Tooth are lacking.
AIMS
To assess the impact of pregnancy on Charcot-Marie-Tooth and how Charcot-Marie-Tooth affects pregnancy, delivery and postnatal care.
METHODS
A retrospective questionnaire exploring disease course during pregnancy, delivery, pregnancy complications, anaesthetic management and puerperium was administered to 92 patients with Charcot-Marie-Tooth and related disorders.
RESULTS
Worsening of Charcot-Marie-Tooth symptoms were reported in 37% of pregnant patients which resolved after delivery in half of the patients. No significant increase in pregnancy, delivery and anaesthetic complications were observed and the type of delivery did not significantly differ from the normal population.
CONCLUSIONS
While these results are reassuring, ideally an international prospective study should be done to confirm these results and to develop practice guidelines on the management of pregnancy in Charcot-Marie-Tooth.
背景
夏科-马里-图思病是最常见的遗传性周围神经病，许多夏科-马里-图思病患者为育龄女性。目前缺乏关于夏科-马里-图思病患者孕期管理的指南。
目的
评估妊娠对夏科-马里-图思病的影响以及夏科-马里-图思病如何影响妊娠、分娩及产后护理。
方法
对92例患有夏科-马里-图思病及相关疾病的患者进行回顾性问卷调查，内容包括孕期、分娩期、妊娠并发症、麻醉管理及产褥期的疾病进程。
结果
37%的妊娠患者报告夏科-马里-图思病症状加重，其中半数患者产后症状缓解。未观察到妊娠、分娩及麻醉并发症显著增加，分娩方式与正常人群无显著差异。
结论
虽然这些结果令人安心，但理想情况下应开展一项国际前瞻性研究以证实这些结果，并制定关于夏科-马里-图思病患者孕期管理的实践指南。
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