关键词: ART CBAVD CF Phe508del Pubertal development Sexual reproductive health

来  源:   DOI:10.1016/j.jcf.2024.01.009

Abstract:
This systematic review summarizes the impact of cystic fibrosis (CF) on sexual and reproductive health (SRH) in males and females, covering pubertal development, hormonal function, family planning, and fertility. Included articles featured historical CF diagnostic criteria, preclinical or clinical data (retrospective cohorts or open label trials), while excluded articles lacked full text availability, explicit methodology, or comparisons between CF and non-CF patients. Genotype differences in CFTR mutations influenced symptom severity. Males with CF experienced delayed puberty, hypogonadism, infertility from obstructive azoospermia, and semen parameter issues. Female CF patients showed decreased fertility, possibly linked to disrupted ionic balance and ovarian cystic disease. Assistive reproductive technologies addressed fertility issues, but success varied based on disease severity and genotype. CFTR modulators aided pulmonary function and sexual health but require further assessment for fertility benefits.
摘要:
本系统综述总结了囊性纤维化(CF)对男性和女性性健康和生殖健康(SRH)的影响,涵盖青春期发育,荷尔蒙功能,计划生育,和生育能力。收录的文章介绍了历史CF诊断标准,临床前或临床数据(回顾性队列或开放标签试验),虽然被排除的文章缺乏全文可用性,明确的方法论,或CF和非CF患者之间的比较。CFTR突变的基因型差异影响症状严重程度。患有CF的男性经历了青春期延迟,性腺功能减退,阻塞性无精子症导致的不孕症,和精液参数问题。女性CF患者生育能力下降,可能与离子平衡破坏和卵巢囊性疾病有关。辅助生殖技术解决了生育问题,但是成功取决于疾病的严重程度和基因型。CFTR调节剂有助于肺功能和性健康,但需要进一步评估生育益处。
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