To evaluate sex disparities in cystic fibrosis (CF) outcomes before and after the introduction of highly effective modulator therapy.
Key Findings:
Female patients with CF experience worse pulmonary morbidity and earlier mortality compared to male patients.
Female sex remains an independent risk factor for death even after adjusting for comorbidities and chronic airway infection.
Female patients have more frequent pulmonary exacerbations, longer treatment courses, and are less likely to recover baseline lung function post-exacerbation.
Common airway pathogens are acquired earlier by female patients, leading to accelerated lung function decline.
Estrogen may negatively impact CF lung disease through various airway-level effects.
Interpretation:
Limitations:
Many findings are based on mechanistic, in vitro, animal, or observational studies, which may not fully reflect clinical physiology.
Existing studies on the effects of oral contraceptives and hormonal states on CF outcomes show inconsistent results.
“High-dose intravenous vitamin C administered early after injury did not reduce organ dysfunction or [mortality] and should not be a treatment in severe burns.”
