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囊性纤维化患者的妊娠——过去、现在与未来

Pregnancy in Cystic Fibrosis-Past, Present, and Future.

作者信息

Gur Michal, Pollak Mordechai, Bar-Yoseph Ronen, Bentur Lea

机构信息

Pediatric Pulmonary Institute and CF Center, Rappaport Children's Hospital, Rambam Health Care Campus, Haifa 3109601, Israel.

Rappaport Faculty of Medicine, Technion-Israel Institute of Technology, Haifa 3525422, Israel.

出版信息

J Clin Med. 2023 Feb 12;12(4):1468. doi: 10.3390/jcm12041468.

Abstract

The introduction of mutation-specific therapy led to a revolution in cystic fibrosis (CF) care. These advances in CF therapies have changed the disease profile from a severe incurable disease with limited survival to a treatable disease with improved quality of life and survival into adulthood. CF patients are now able to plan their future, including marriage and parenthood. Side by side with the optimism, new issues and concerns are arising, including fertility and preparation for pregnancy, maternal and fetal care during pregnancy, and post-partum care. While cystic fibrosis transmembrane regulator (CFTR) modulators show promising results for improving CF lung disease, data on their safety in pregnancy are still limited. We performed a literature review on pregnancy in CF from the past, with the first described pregnancy in 1960, through the current fascinating changes in the era of CFTR modulators, to ongoing studies and future directions. Current advances in knowledge give hope for improved outcomes of pregnancy, towards the best possible prognosis for the mother and for the baby.

摘要

突变特异性疗法的引入引发了囊性纤维化(CF)治疗的一场革命。CF治疗的这些进展已将疾病状况从一种生存有限的严重不治之症转变为一种可治疗的疾病,患者的生活质量得到改善,生存期延长至成年期。CF患者现在能够规划自己的未来,包括结婚和生育。在充满乐观情绪的同时,新的问题和担忧也随之出现,包括生育能力和妊娠准备、孕期母婴护理以及产后护理。虽然囊性纤维化跨膜传导调节因子(CFTR)调节剂在改善CF肺部疾病方面显示出有前景的结果,但它们在孕期安全性的数据仍然有限。我们对过去CF患者妊娠情况进行了文献综述,从1960年首次描述的妊娠,到CFTR调节剂时代当前令人着迷的变化,再到正在进行的研究和未来方向。当前知识的进步为改善妊娠结局带来了希望,朝着为母亲和婴儿实现尽可能最佳的预后发展。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/1007/9963833/10cac652f439/jcm-12-01468-g001.jpg

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