Aspergillus ever cultured from BAL during first 5 years of life associated with worse CT PRAGMA score in same year, following year, and at the end of the study at 5 or 6 years old (adjusted for pancreatic insufficiency, age, gender, CFTR genotype, and other infections; and baseline CT score for 1 year progression results)Aspergillus positive BAL significantly associated with BAL markers of neutrophil inflammation (p < 0.001)Aspergillus positive BAL significantly associated respiratory admissions risk in the subsequent year (p = 0.008). Here, CFTR is linked to exocrine pancreatic insufficiency.