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Pulmonary function deficits in newborn screened infants with cystic fibrosis managed with standard UK care are mild and transient

Davies, G., Stocks, J., Thia, L.P. , Hoo, A-F., Bush, A., Aurora, P., Brennan, L., Lee, S., Lum, S., Cottam, P., Miles, J., Chudleigh, J. H., Kirkby, J., Balfour-Lynn, I. M., Carr, S. B., Wallis, C., Wyatt, H., Wade, A. & Collabora, L. C. F. (2017). Pulmonary function deficits in newborn screened infants with cystic fibrosis managed with standard UK care are mild and transient. European Respiratory Journal, 50(5), 1700326. doi: 10.1183/13993003.00326-2017


With the advent of novel designer molecules for cystic fibrosis (CF) treatment, there is huge need for early-life clinical trial outcomes, such as infant lung function (ILF). We investigated the degree and tracking of ILF abnormality during the first 2 years of life in CF newborn screened infants.

Forced expiratory volume in 0.5 s (FEV₀.₅), lung clearance index (LCI) and plethysmographic functional residual capacity were measured at ∼3 months, 1 year and 2 years in 62 infants with CF and 34 controls.

By 2 years there was no significant difference in FEV₀.₅ z-score between CF and controls, whereas mean LCI z-score was 0.81 (95% CI 0.45–1.17) higher in CF. However, there was no significant association between LCI z-score at 2 years with either 3-month or 1-year results. Despite minimal average group changes in any ILF outcome during the second year of life, marked within-subject changes occurred. No child had abnormal LCI or FEV₀.₅ on all test occasions, precluding the ability to identify “high-risk” infants in early life.

In conclusion, changes in lung function are mild and transient during the first 2 years of life in newborn screened infants with CF when managed according to a standardised UK treatment protocol. Their potential role in tracking disease to later childhood will be ascertained by ongoing follow-up.

Publication Type: Article
Additional Information: This is an author-submitted, peer-reviewed version of an article that has been accepted for publication in the European Respiratory Journal, prior to copy-editing, formatting and typesetting. This version of the article may not be duplicated or reproduced without prior permission from the copyright owner, the European Respiratory Society. The publisher is not responsible or liable for any errors or omissions in this version of the article or in any version derived from it by any other parties. The final, copy-edited, published article, which is the version of record, is available online from the European Respiratory Journal without a subscription 18 months after the date of issue publication.
Publisher Keywords: cystic fibrosis, newborn screening for cystic fibrosis, infant pulmonary function testing, lung clearance index, plethysmography, raised volume technique
Departments: School of Health & Psychological Sciences > Nursing
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