英国囊性纤维化女性的妊娠率和结局：与疾病修正治疗引入前后（2003-17 年）普通人群的比较。
Pregnancy rates and outcomes in women with cystic fibrosis in the UK: comparisons with the general population before and after the introduction of disease-modifying treatment, 2003-17.
机构信息
Department of Public Health, Policy and Systems, University of Liverpool, Liverpool, UK.
Cardiff School of Sport and Health Sciences, Cardiff Metropolitan University, Cardiff, UK.
出版信息
BJOG. 2022 Apr;129(5):743-751. doi: 10.1111/1471-0528.16957. Epub 2021 Nov 8.
OBJECTIVE
To compare pregnancy rates and outcomes for women with cystic fibrosis in the UK with those of the general population and assess the effect of the introduction of disease-modifying treatment.
DESIGN
A population-based longitudinal study, 2003-17.
SETTING
United Kingdom.
POPULATION
Women aged 15-44 years in the UK cystic fibrosis (CF) Registry compared with women in England and Wales.
METHODS
We calculated pregnancy and live-birth rates for the CF population and the general population of England and Wales. For women with CF we compared pregnancy rates before and after ivacaftor was introduced in 2013. We further used CF registry data to assess pregnancy outcomes for mothers with CF, and to assess the relationship between maternal pre-pregnancy lung function and nutritional status and child gestational age.
MAIN OUTCOME MEASURES
Pregnancy and live-birth rates and child gestational age.
RESULTS
Of 3831 women with CF, 661 reported 818 pregnancies. Compared with the general population, the pregnancy rate was 3.3 times lower in the CF population (23.5 versus 77.7 per 1000 woman-years); the live-birth rate was 3.5 times lower (17.4 versus 61.4 per 1000 woman-years) with 70% of pregnancies in CF women resulting in live births; termination of pregnancy rates were also lower (9% versus 22%). Pregnancy rates increased post-ivacaftor for eligible women with CF, from 29.7 to 45.7 per 1000 woman-years. There was no association between pre-pregnancy lung function/nutrition status and gestational age.
CONCLUSIONS
Pregnancy rates in women with CF are about one-third of the rates in the general population with favourable outcomes, and increased for eligible women post-ivacaftor.
TWEETABLE ABSTRACT
Pregnancy rates in women with CF are about a third of the rate in England and Wales with 70% live births. Ivacaftor increases the rate.
目的
比较英国囊性纤维化（CF）女性与普通人群的妊娠率和结局，并评估疾病修正治疗的引入对妊娠率的影响。
设计
一项基于人群的纵向研究，2003-2017 年。
地点
英国。
人群
英国 CF 登记处年龄在 15-44 岁的女性与英格兰和威尔士的女性进行比较。
方法
我们计算了 CF 人群和英格兰及威尔士普通人群的妊娠率和活产率。对于接受伊伐卡托治疗的 CF 女性，我们比较了伊伐卡托于 2013 年引入前后的妊娠率。我们进一步利用 CF 登记处的数据评估 CF 母亲的妊娠结局，并评估母亲孕前肺功能和营养状况与胎儿胎龄之间的关系。
主要观察指标
妊娠率和活产率及胎儿胎龄。
结果
在 3831 名 CF 女性中，661 名报告了 818 次妊娠。与普通人群相比，CF 人群的妊娠率低 3.3 倍（每 1000 名女性年 23.5 次 vs 77.7 次）；活产率低 3.5 倍（每 1000 名女性年 17.4 次 vs 61.4 次），70%的妊娠导致活产；人工流产率也较低（9% vs 22%）。对于符合条件的 CF 女性，伊伐卡托治疗后妊娠率从 29.7 次/1000 名女性年增加至 45.7 次/1000 名女性年。孕前肺功能/营养状况与胎龄无相关性。
结论
CF 女性的妊娠率约为普通人群的三分之一，但结局良好，伊伐卡托治疗后妊娠率增加。
推文摘要
CF 女性的妊娠率约为英格兰和威尔士的三分之一，活产率为 70%。伊伐卡托增加了这一比例。
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