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Background: Cystic fibrosis (CF) is a common life-shortening genetic disease in which women have been described to have worse outcomes than males, particularly in response to respiratory infections with Pseudomonas aeruginosa. However, as advancements in therapies have improved life expectancy, this gender disparity has been challenged. The objective of this study is to examine whether a gender-based survival difference still exists in this population and determine the impact of common CF respiratory infections on outcomes in males versus females with CF.

Methods: We conducted a retrospective cohort analysis of 32,766 patients from the United States Cystic Fibrosis Foundation Patient Registry over a 13-year period. Kaplan-Meier and Cox proportional hazards models were used to compare overall mortality and pathogen based survival rates in males and females.

Results: Females demonstrated a …