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Sex differences in morbidity and mortality have been reported in the cystic fibrosis (CF) population worldwide. However, it is unclear why CF women have worse clinical outcomes than men. In this review, we focus on the influence of female sex hormones on CF pulmonary outcomes and summarise data from  in vitro  and  in vivo  experiments on how oestrogen and progesterone might modify mucociliary clearance, immunity and infection in the CF airways. The potential for novel sex hormone-related therapeutic interventions is also discussed.

Sex disparities in cystic fibrosis: review on the effect of female sex hormones on lung pathophysiology and outcomes