Despite improvements in survival from cystic fibrosis (CF) over successive decades,
many infants with CF have diminished lung function within months of being diagnosed
on newborn screening [
[1]
,
[2]
]. Early childhood has been identified as a crucial period in the establishment of
future lung disease. However, the age of onset and progression of CF lung disease
is highly variable, creating uncertainty as to how proactively children should be
monitored and treated. Reducing the burden of daily treatments is a priority for the
CF community [
[3]
], especially as the use of highly effective modulator therapies becomes widespread.
There is therefore a need to identify children at high risk of irreversible lung damage
who would benefit most from intensive management strategies. In this issue of the
Journal, research groups from both the UK and Australia report prospective observational
data investigating early life factors associated with loss of lung function at different
periods in childhood.- Bayfield K.J.
- Douglas T.A.
- Rosenow T.
- Davies J.C.
- Elborn S.J.
- Mall M.
- Paproki A.
- Ratjen F.
- Sly P.D.
- Smyth A.R.
- Stick S.
- Wainwright C.E.
- Robinson P.D.
Time to get serious about the detection and monitoring of early lung disease in cystic
fibrosis.
ThoraxThorax. 2021; ([Internet] BMJ Publishing Group Ltd[cited 2021 Apr 30];: thoraxjnl-2020-216085Available
from)
Abbreviations:
LCFC (London Cystic Fibrosis Collaboration), ACFBAL (Australasian Cystic Fibrosis Bronchoalveolar Lavage), BAL (bronchoalveolar lavage), PsA (Pseudomonas aeruginosa), AREST-CF (Australian Respiratory Early Surveillance Team for Cystic Fibrosis)To read this article in full you will need to make a payment
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References
- Lung function in infants with cystic fibrosis diagnosed by newborn screening.Am J Respir Crit Care Med. 2008; 178: 1238-1244
- Lung function is abnormal in 3-month-old infants with cystic fibrosis diagnosed by newborn screening.Thorax. 2012; 67 ([Internet]Available from): 874-881
- Time to get serious about the detection and monitoring of early lung disease in cystic fibrosis.ThoraxThorax. 2021; ([Internet] BMJ Publishing Group Ltd[cited 2021 Apr 30];: thoraxjnl-2020-216085Available from)
- Is chest CT useful in newborn screened infants with cystic fibrosis at 1 year of age?.Thorax. 2014; 69 ([Internet] BMJ Publishing Group[cited 2022 Jul 21]Available from: /pmc/articles/PMC3963531/): 320-327
- Evolution of lung function during the first year of life in newborn screened cystic fibrosis infants.Thorax. 2014; 69 ([Internet] BMJ Publishing Group[cited 2019 May 8]Available from): 910-917
- Early Pseudomonas aeruginosa predicts poorer pulmonary function in preschool children with cystic fibrosis.J Cyst Fibros. 2022; ([Internet] J Cyst Fibros[cited 2022 Aug 15]; Available from)
- Progression of lung disease in preschool patients with cystic fibrosis.Am J Respir Crit Care Med. 2017; 195 ([Internet] American Thoracic Society[cited 2019 Mar 28]Available from): 1216-1225
- Factors in childhood associated with lung function decline to adolescence in cystic fibrosis.J Cyst Fibros. 2022; ([Internet] J Cyst Fibros[cited 2022 Aug 15]; Available from)
- Sputum biomarkers of inflammation and lung function decline in children with cystic fibrosis.Am J Respir Crit Care Med. 2012; 186 ([Internet][cited 2022 Aug 24]Available from): 857-865
- Early respiratory infection is associated with reduced spirometry in children with cystic fibrosis.Am J Respir Crit Care Med. 2014; 190 ([Internet] American Thoracic Society[cited 2020 Sep 8]Available from): 1111-1116
- Early lung disease in infants and preschool children with cystic fibrosis: what have we learned and what should we do about it?.Am J Respir Crit Care Med Am Thoracic Soc. 2017; : 1567-1575
- Socioeconomic status, smoke exposure, and health outcomes in young children with cystic fibrosis.Pediatrics. 2017; ([Internet] Pediatrics[cited 2022 Aug 9]Available from): 139
- Quantification of the relative contribution of environmental and genetic factors to variation in cystic fibrosis lung function.J Pediatr. 2010; ([Internet] J Pediatr[cited 2022 Aug 9]; 157Available from)
- Clinical outcomes in cystic fibrosis at 6 years of age with Tricare insurance coverage.J Cyst Fibros. 2022; (Elsevier)
- Effect of bronchoalveolar lavage-directed therapy on Pseudomonas aeruginosa infection and structural lung injury in children with cystic fibrosis: a randomized trial.JAMA - J Am Med Assoc. 2011; 306: 163-171
Article Info
Publication History
Published online: August 29, 2022
Accepted:
August 25,
2022
Received:
August 24,
2022
Identification
Copyright
© 2022 European Cystic Fibrosis Society. Published by Elsevier B.V. All rights reserved.
