BACKGROUND: Although the lung clearance index (LCI) is a sensitive marker of small airway disease in individuals with cystic fibrosis (CF), less is known about longitudinal changes in LCI during routine clinical surveillance. Here, our objectives were to describe the longitudinal course of LCI in children with CF during routine clinical surveillance and assess influencing factors. METHODS: Children with CF aged 3-18 years performed LCI measurements every 3 months as part of routine clinical care between 2011 and 2018. We recorded clinical data at every visit. We used a multilevel mixed effect model to determine changes in LCI over time and identify clinical factors that influence LCI course. RESULTS: We collected LCI measurements from 1204 visits (3603 trials) in 78 participants, of which 907 visits had acceptable LCI data. The average unadjusted increase in LCI for the entire population was 0.29 (95% CI 0.20-0.38) LCI units·year-1. The increase in LCI was more pronounced in adolescence (0.41 (95% CI 0.27-0.54) LCI units·year-1). Colonisation with either Pseudomonas aeruginosa or Aspergillus fumigatus, pulmonary exacerbations, CF-related diabetes and bronchopulmonary aspergillosis were associated with a higher increase in LCI over time. Adjusting for clinical risk factors reduced the increase in LCI over time to 0.24 (95% CI 0.16-0.33) LCI units·year-1. CONCLUSIONS: LCI measured during routine clinical surveillance is associated with underlying disease progression in children with CF. An increased change in LCI over time should prompt further diagnostic intervention.
BACKGROUND: Although the lung clearance index (LCI) is a sensitive marker of small airway disease in individuals with cystic fibrosis (CF), less is known about longitudinal changes in LCI during routine clinical surveillance. Here, our objectives were to describe the longitudinal course of LCI in children with CF during routine clinical surveillance and assess influencing factors. METHODS:Children with CF aged 3-18 years performed LCI measurements every 3 months as part of routine clinical care between 2011 and 2018. We recorded clinical data at every visit. We used a multilevel mixed effect model to determine changes in LCI over time and identify clinical factors that influence LCI course. RESULTS: We collected LCI measurements from 1204 visits (3603 trials) in 78 participants, of which 907 visits had acceptable LCI data. The average unadjusted increase in LCI for the entire population was 0.29 (95% CI 0.20-0.38) LCI units·year-1. The increase in LCI was more pronounced in adolescence (0.41 (95% CI 0.27-0.54) LCI units·year-1). Colonisation with either Pseudomonas aeruginosa or Aspergillus fumigatus, pulmonary exacerbations, CF-related diabetes and bronchopulmonary aspergillosis were associated with a higher increase in LCI over time. Adjusting for clinical risk factors reduced the increase in LCI over time to 0.24 (95% CI 0.16-0.33) LCI units·year-1. CONCLUSIONS:LCI measured during routine clinical surveillance is associated with underlying disease progression in children with CF. An increased change in LCI over time should prompt further diagnostic intervention.
Authors: Sotirios Fouzas; Anne-Christianne Kentgens; Olga Lagiou; Bettina Sarah Frauchiger; Florian Wyler; Ilias Theodorakopoulos; Sophie Yammine; Philipp Latzin Journal: ERJ Open Res Date: 2022-03-14
Authors: Edith T Zemanick; Jennifer L Taylor-Cousar; Jane Davies; Ronald L Gibson; Marcus A Mall; Edward F McKone; Paul McNally; Bonnie W Ramsey; Jonathan H Rayment; Steven M Rowe; Elizabeth Tullis; Neil Ahluwalia; Chenghao Chu; Thang Ho; Samuel M Moskowitz; Sabrina Noel; Simon Tian; David Waltz; Tanya G Weinstock; Fengjuan Xuan; Claire E Wainwright; Susanna A McColley Journal: Am J Respir Crit Care Med Date: 2021-06-15 Impact factor: 21.405
Authors: Alex R Horsley; John Belcher; Katie Bayfield; Brooke Bianco; Steve Cunningham; Catherine Fullwood; Andrew Jones; Anna Shawcross; Jaclyn A Smith; Anirban Maitra; Francis J Gilchrist Journal: Thorax Date: 2021-07-22 Impact factor: 9.139