National Jewish Health, Departments of Medicine and Pediatrics; University of Colorado Anschutz Medical Campus, Departments of Medicine and Pediatrics.
National Jewish Health, Clinical Research Services.
J Cyst Fibros. 2024 May;23(3):412-416. doi: 10.1016/j.jcf.2023.10.022. Epub 2023 Nov 11.
Most males with cystic fibrosis (mwCF) are infertile but with CF transmembrane conductance regulator (CFTR) modulator-conferred benefits, more are utilizing assisted reproductive technologies (ART). Administration of normal human doses of modulators in animal reproductive models caused no genotoxicity; no human data exists. Potential health decline following modulator discontinuation makes the decision to withhold therapy during reproduction challenging.
From August-October 2021, international CF clinicians completed an anonymous questionnaire regarding mwCF who used modulators during reproduction.
We received 42 surveys for mwCF with partner pregnancies. Forty of 42 mwCF utilized ART; 35 continued modulators during sperm retrieval and 40/42 during partner pregnancy. One of four males who discontinued modulators experienced clinical deterioration. First trimester miscarriages occurred in 11.9 % of partner pregnancies. No congenital anomalies were reported.
Use of CFTR modulators during reproduction and partner pregnancy in mwCF did not result in a higher-than-expected miscarriage rate nor congenital anomalies.
大多数囊性纤维化（mwCF）男性不育，但 CF 跨膜电导调节剂（CFTR）调节剂带来的益处，更多的人正在利用辅助生殖技术（ART）。在动物生殖模型中给予正常人用剂量的调节剂不会引起遗传毒性；没有人类数据。由于停药后潜在的健康状况下降，使得在生殖过程中决定是否停止治疗具有挑战性。
2021 年 8 月至 10 月，国际 CF 临床医生完成了一项关于在生殖期间使用调节剂的 mwCF 的匿名问卷调查。
我们收到了 42 份关于 mwCF 伴妊娠伴侣的调查。42 名 mwCF 中有 40 名使用了 ART；35 名在精子采集期间继续使用调节剂，40 名在伴侣妊娠期间继续使用调节剂。4 名停止使用调节剂的男性中有 1 名出现临床恶化。伴侣妊娠中，有 11.9%发生了早期流产。没有报告先天性异常。
在 mwCF 中，CFTR 调节剂在生殖和伴侣妊娠期间的使用并未导致流产率或先天性异常高于预期。
