TY - T1的怀孕在两个囊性纤维化患者接受lumacaftor / ivacaftor JF -欧洲呼吸杂志》乔和J - 10.1183/13993003.国会2019欧元。PA4517六世- 54 - 5 63 SP - PA4517盟Keßler克里斯蒂娜盟——配音演员,Angelika盟——Große-Onnebrink Jorg盟——奥木兰·Heymut Y1 - 2019/09/28 UR - //www.qdcxjkg.com/content/54/suppl_63/PA4517.abstract N2 -简介:雌性生殖道的影响调节器囊性纤维化患者的生育力(CF)是未知的。良好的研究实践禁止参与的患者在临床试验中尝试怀孕,导致缺乏安全数据。我们报告两个怀孕在lumacaftor / ivacaftor纤维化患者。例:例1是29岁,纯合子F508del,身体质量指数(BMI) 20日3 kg / m2, pred FEV1 58岁的6%。她是2016年2月以来lumacaftor / ivacaftor使用激素避孕混合制和医疗建议。CFTR-modulator是停止发现怀孕时妊娠8周。FEV1%pred拒绝45,6在32周,体重只有4公斤。经过反复肺发作需要输血治疗,进行剖腹产在35 + 2周。婴儿需要通气支持由于早产,但健康,出生体重2690克。 After two months FEV1%pred was 48, BMI 20,1 kg/m2.Case 2 is also 29 years old, homozygous for F508del, BMI 20,6 kg/m2, FEV1%pred 39,2 with CF-diabetes. She had unprotected sexual intercourse for over a decade. After treatment with lumacaftor/ivacaftor for two years, a pregnancy was diagnosed at 20 weeks gestation. The patient decided to continue lumacaftor/ivacaftor. FEV1%pred at 36 weeks was 43,9 and weight gain was 9 kg. She gave birth to a healthy girl, birth weight 2440 g via planned caesarean section at 37 weeks. After two months FEV1%pred was 39,2, BMI 20,3 kg/m2.Conclusions: There might be an effect of CFTR-modulators on fecundity and the course and outcome of pregnancy in CF-patients. More data are needed to support the safety or risk for infants of mothers treated with CFTR-modulators.FootnotesCite this article as: European Respiratory Journal 2019; 54: Suppl. 63, PA4517.This is an ERS International Congress abstract. No full-text version is available. Further material to accompany this abstract may be available at www.ers-education.org (ERS member access only). ER -