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2020年至今,苏格兰成人囊性纤维化治疗中心接受依列卡福托/替扎卡福托/依伐卡托治疗的患者的妊娠结局。

Pregnancy outcomes in patients from a Scottish Adult Cystic Fibrosis Unit taking elexacaftor/tezacaftor/ivacaftor, 2020-present.

作者信息

Valcheva Ivaila, Paterson Iona

机构信息

West of Scotland Adult Cystic Fibrosis Unit, Queen Elizabeth University Hospital, Glasgow, Scotland.

出版信息

Obstet Med. 2025 Feb 26:1753495X251319588. doi: 10.1177/1753495X251319588.

Abstract

BACKGROUND

Elexacaftor/tezacaftor/ivacaftor (ETI) was made available to eligible women in September 2020 by NHS Scotland.

METHODS

Retrospective data collection for the 13 pregnancies in women taking ETI from the West of Scotland Adult Cystic Fibrosis Unit, September 2020-December 2023.

RESULTS

Mean pre-pregnancy FEV1 was 2.26L, 70% predicted (range 1.25-3.19); (38-86% predicted). Mean FEV1 post-pregnancy was 2.29L, 71% predicted (range 1.49-3.40); (45-92% predicted). The mean age at conception (29 years) and mean percentage predicted FEV1 (70%) were higher than in other UK studies. Two pregnancies resulted in miscarriage, the remaining 11 pregnancies resulted in a live birth. Seven women had a pulmonary exacerbation of CF during pregnancy. Three of four women with FEV1 < 60% predicted had uncomplicated pregnancies with no pulmonary exacerbations.

CONCLUSION

We demonstrate that people with CF and varying spectrums of lung disease who take CFTR modulators can have uncomplicated pregnancies with positive lung function outcomes.

摘要

背景

2020年9月,苏格兰国民保健服务体系(NHS Scotland)向符合条件的女性提供了依列卡福妥/替扎卡福妥/依伐卡托(ETI)。

方法

对2020年9月至2023年12月期间从苏格兰西部成人囊性纤维化病房获取的13例服用ETI的女性的妊娠情况进行回顾性数据收集。

结果

妊娠前平均第一秒用力呼气容积(FEV1)为2.26升,预测值的70%(范围1.25 - 3.19升);(预测值的38% - 86%)。妊娠后平均FEV1为2.29升,预测值的71%(范围1.49 - 3.40升);(预测值的45% - 92%)。受孕时的平均年龄(29岁)和预测FEV1的平均百分比(70%)高于英国其他研究。2例妊娠导致流产,其余11例妊娠分娩存活婴儿。7名女性在孕期出现了囊性纤维化肺部加重。4名预测FEV1 < 60%的女性中有3名妊娠过程顺利,无肺部加重情况。

结论

我们证明,服用囊性纤维化跨膜传导调节因子(CFTR)调节剂的不同肺部疾病谱的囊性纤维化患者可以有顺利的妊娠过程以及良好的肺功能结果。

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