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The lung clearance index (LCI) assesses global ventilation inhomogeneity and is a sensitive biomarker of airway function in cystic fibrosis (CF) lung disease. We examined the association of LCI with the risk of death or lung transplantation (LTX) in individuals with CF. We performed a retrospective analysis in a cohort of individuals with CF aged≥5 years with LCI and FEV measurements performed between 1980 and 2006. The outcome was time until death or LTX. We used the earliest available LCI and FEV values in a Cox proportional hazard regr