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多产妇囊性纤维化的母婴结局。

Maternal and fetal outcomes in multiparous women with Cystic Fibrosis.

机构信息

Pediatric Pulmonary Unit and Cystic Fibrosis Center, Hadassah Medical Center, Jerusalem, Israel; Faculty of Medicine, Hebrew University of Jerusalem, Israel.

Faculty of Medicine, Hebrew University of Jerusalem, Israel.

出版信息

Respir Med. 2024 Jul;228:107654. doi: 10.1016/j.rmed.2024.107654. Epub 2024 May 11.

Abstract

BACKGROUND

Quality of life and survival in Cystic Fibrosis (CF) have improved dramatically, making family planning a feasible option. Maternal and perinatal outcomes in women with CF (wwCF) are similar to those seen in the general population. However, the effect of undergoing multiple pregnancies is unknown.

METHODS

A multinational-multicenter retrospective cohort study. Data was obtained from 18 centers worldwide, anonymously, on wwCF 18-45 years old, including disease severity and outcome, as well as obstetric and newborn complications. Data were analyzed, within each individual patient to compare the outcomes of an initial pregnancy (1st or 2nd) with a multigravid pregnancy (≥3) as well as secondary analysis of grouped data to identify risk factors for disease progression or adverse neonatal outcomes. Three time periods were assessed - before, during, and after pregnancy.

RESULTS

The study population included 141 wwCF of whom 41 (29%) had ≥3 pregnancies, "multiparous". Data were collected on 246 pregnancies, between 1973 and 2020, 69 (28%) were multiparous. A greater decline in ppFEV was seen in multiparous women, primarily in pancreatic insufficient (PI) wwCF and those with two severe (class I-III) mutations. Multigravid pregnancies were shorter, especially in wwCF over 30 years old, who had high rates of prematurity and newborn complications. There was no effect on pulmonary exacerbations or disease-related complications.

CONCLUSIONS

Multiple pregnancies in wwCF are associated with accelerated respiratory deterioration and higher rates of preterm births. Therefore, strict follow-up by a multidisciplinary CF and obstetric team is needed in women who desire to carry multiple pregnancies.

摘要

背景

囊性纤维化 (CF) 的生活质量和生存率有了显著提高,这使得计划生育成为一种可行的选择。CF 女性(wwCF)的母婴和围产期结局与一般人群相似。然而,多次妊娠的影响尚不清楚。

方法

这是一项多国家-多中心的回顾性队列研究。数据来自全球 18 个中心,匿名收集了 18-45 岁的 wwCF 的疾病严重程度和结局,以及产科和新生儿并发症的数据。对每个患者进行分析,比较首次妊娠(1 次或 2 次)和多胎妊娠(≥3 次)的结局,并对分组数据进行二次分析,以确定疾病进展或不良新生儿结局的危险因素。评估了三个时间段:妊娠前、妊娠期间和妊娠后。

结果

研究人群包括 141 名 wwCF,其中 41 名(29%)有≥3 次妊娠,即“多胎妊娠”。共收集了 246 次妊娠的数据,时间为 1973 年至 2020 年,其中 69 次(28%)为多胎妊娠。多胎妊娠的 ppFEV 下降更为明显,主要见于胰腺功能不全(PI)的 wwCF 和有两个严重(I-III 级)突变的患者。多胎妊娠的时间更短,尤其是年龄超过 30 岁的 wwCF,她们有较高的早产率和新生儿并发症。对肺恶化或与疾病相关的并发症没有影响。

结论

wwCF 中的多次妊娠与呼吸功能恶化加速和早产率升高有关。因此,希望多胎妊娠的妇女需要由多学科 CF 和产科团队进行严格的随访。

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