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Clinical characteristics of people with cystic fibrosis and frequent fungal infection
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  • Thomas Poore,
  • Maxene Meier,
  • Elin Towler,
  • John Brinton,
  • Stacey Martiniano,
  • Scott Sagel,
  • Emily DeBoer,
  • Brandie Wagner,
  • Edith Zemanick
Thomas Poore
University of Colorado Denver School of Medicine
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Maxene Meier
University of Colorado Denver - Anschutz Medical Campus
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Elin Towler
University of Colorado Denver - Anschutz Medical Campus
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John Brinton
University of Colorado Denver - Anschutz Medical Campus
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Stacey Martiniano
University of Colorado at Denver - Anschutz Medical Campus
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Scott Sagel
University of Colorado Health Sciences Center
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Emily DeBoer
University of Colorado Anschutz Medical Campus
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Brandie Wagner
University of Colorado Denver - Anschutz Medical Campus
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Edith Zemanick
University of Colorado School of Medicine
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Abstract

Background: Individuals with cystic fibrosis (CF) and fungal airway infection may present with fungal bronchitis, allergic bronchopulmonary aspergillosis (ABPA) or may appear unaffected despite fungal detection. We sought to characterize people with CF with frequent detection of fungi from airway samples and determine clinical outcomes. Methods: This retrospective study included individuals with CF with ≥ 4 lower airway cultures over a 2-year baseline period and ≥ 2 years of follow-up. We defined two groups: ≤ 1 positive fungus culture (rare) or ≥ 2 positive cultures during baseline (frequent). Clinical characteristics and outcomes were determined. Results: Between 2004-2016, 294 individuals met inclusion with 62% classified as rare and 38% as frequent fungi during baseline. Median follow-up was 6 years (range 2-9 years). Aspergillus fumigatus was the most common fungal species detected. Individuals with frequent fungi were older (13.7 vs. 11.7 yrs, p = 0.02) and more likely to have Stenotrophomonas maltophilia (35% vs 17%, p < 0.001) at baseline, but did not differ in lung function or ABPA diagnosis. During follow-up, those with frequent fungi were more likely to have chronic P. aeruginosa and S. maltophilia. Individuals with ABPA and frequent fungi had the highest rates of co-infection and co-morbidities, and a trend towards more rapid lung function decline. Discussion: Fungal infection in CF was associated with frequent P. aeruginosa and S. maltophilia co-infection even in those without ABPA. Individuals with frequent fungi and ABPA had worse outcomes, highlighting the potential contribution of fungi to CF pulmonary disease.

Peer review status:IN REVISION

05 Jun 2021Submitted to Pediatric Pulmonology
18 Jun 2021Assigned to Editor
18 Jun 2021Submission Checks Completed
21 Jun 2021Reviewer(s) Assigned
05 Aug 2021Review(s) Completed, Editorial Evaluation Pending
08 Aug 2021Editorial Decision: Revise Major