Long-term outcomes following infection and inflammation in early CF lung disease
Author(s)
Frayman, K
Armstrong, D
Grimwood, K
Ranganathan, S
Griffith University Author(s)
Year published
2020
Metadata
Show full item recordAbstract
Introduction/Aim. Infection and inflammation are critical to the pathogenesis of cystic fibrosis (CF) lung disease. In infancy, the detection of pathogenic organisms and neutrophilic inflammation in the lower airways is associated with earlier development of bronchiectasis and lower lung function at age 6‐years. This study explores the impact of early life lower airway infection and inflammation on survival, need for lung transplant and the development of structural and functional lung disease over 25‐years.
Methods. A birth cohort of infants newly diagnosed with CF was established at the Royal Children's Hospital, Melbourne, ...
View more >Introduction/Aim. Infection and inflammation are critical to the pathogenesis of cystic fibrosis (CF) lung disease. In infancy, the detection of pathogenic organisms and neutrophilic inflammation in the lower airways is associated with earlier development of bronchiectasis and lower lung function at age 6‐years. This study explores the impact of early life lower airway infection and inflammation on survival, need for lung transplant and the development of structural and functional lung disease over 25‐years. Methods. A birth cohort of infants newly diagnosed with CF was established at the Royal Children's Hospital, Melbourne, Australia, in 1992, soon after the introduction of newborn screening for CF. Bronchoscopy and bronchoalveolar lavage (BAL) were performed at study entry and approximately annually thereafter. Quantitative microbiological culture was performed and inflammatory markers were measured contemporaneously. Where possible, 16S rRNA gene analysis was performed on stored BAL fluid. Clinical outcome data, including survival, need for lung transplant, rate of decline of FEV1 and bronchiectasis severity, defined by the CF‐CT score, were obtained from participant medical records and the Australian Cystic Fibrosis Data Registry. Results. One hundred infants with CF (50% male), were recruited between 1992‐1999. A total of 255 BALs were performed, median three per participant (range 0‐5). Sixty‐six surviving adults (71%, seven participants lost to follow up), are currently aged 21‐29‐years. Conclusion. To our knowledge, this is the first comprehensively studied birth cohort of infants with CF, diagnosed predominantly via newborn screening and who underwent serial lower airway microbiological assessment, to reach adulthood. This study therefore provides a unique insight into the role of early life lower airway infection and inflammation in the pathogenesis and progression of CF lung disease. Grant Support: Murdoch Childrens Research Institute “65 km for Cystic Fibrosis”; Royal Children's Hospital Cystic Fibrosis Research Trust; Thoracic Society of Australia and New Zealand Vertex Cystic Fibrosis Paediatric Clinical Fellowship; Australian Cystic Fibrosis Research Trust Postgraduate Studentship; Australian National Health and Medical Research Council (NHMRC) postgraduate scholarship; Royal Australasian College of Physicians Paediatrics and Child Health Division NHMRC Award for Excellence (Top‐up); Clifford Family PhD Scholarship
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View more >Introduction/Aim. Infection and inflammation are critical to the pathogenesis of cystic fibrosis (CF) lung disease. In infancy, the detection of pathogenic organisms and neutrophilic inflammation in the lower airways is associated with earlier development of bronchiectasis and lower lung function at age 6‐years. This study explores the impact of early life lower airway infection and inflammation on survival, need for lung transplant and the development of structural and functional lung disease over 25‐years. Methods. A birth cohort of infants newly diagnosed with CF was established at the Royal Children's Hospital, Melbourne, Australia, in 1992, soon after the introduction of newborn screening for CF. Bronchoscopy and bronchoalveolar lavage (BAL) were performed at study entry and approximately annually thereafter. Quantitative microbiological culture was performed and inflammatory markers were measured contemporaneously. Where possible, 16S rRNA gene analysis was performed on stored BAL fluid. Clinical outcome data, including survival, need for lung transplant, rate of decline of FEV1 and bronchiectasis severity, defined by the CF‐CT score, were obtained from participant medical records and the Australian Cystic Fibrosis Data Registry. Results. One hundred infants with CF (50% male), were recruited between 1992‐1999. A total of 255 BALs were performed, median three per participant (range 0‐5). Sixty‐six surviving adults (71%, seven participants lost to follow up), are currently aged 21‐29‐years. Conclusion. To our knowledge, this is the first comprehensively studied birth cohort of infants with CF, diagnosed predominantly via newborn screening and who underwent serial lower airway microbiological assessment, to reach adulthood. This study therefore provides a unique insight into the role of early life lower airway infection and inflammation in the pathogenesis and progression of CF lung disease. Grant Support: Murdoch Childrens Research Institute “65 km for Cystic Fibrosis”; Royal Children's Hospital Cystic Fibrosis Research Trust; Thoracic Society of Australia and New Zealand Vertex Cystic Fibrosis Paediatric Clinical Fellowship; Australian Cystic Fibrosis Research Trust Postgraduate Studentship; Australian National Health and Medical Research Council (NHMRC) postgraduate scholarship; Royal Australasian College of Physicians Paediatrics and Child Health Division NHMRC Award for Excellence (Top‐up); Clifford Family PhD Scholarship
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Conference Title
Respirology
Volume
25
Issue
S1
Publisher URI
Subject
Biomedical and clinical sciences
Science & Technology
Life Sciences & Biomedicine
Respiratory System