4.4 Article

Does newborn screening improve early lung function in cystic fibrosis?

Journal

PAEDIATRIC RESPIRATORY REVIEWS
Volume 42, Issue -, Pages 17-22

Publisher

ELSEVIER SCI LTD
DOI: 10.1016/j.prrv.2020.08.005

Keywords

Cystic fibrosis; Infant lung function; Spirometry; Lung clearance index

Funding

  1. UCL Wellcome Institutional Strategic Support Fund 3 [204841/Z/16/Z]

Ask authors/readers for more resources

The impact of newborn screening on pulmonary outcomes in cystic fibrosis remains unclear, despite evidence of improved nutritional outcomes. Comparisons between different diagnostic methods and improvements in clinical management and public health interventions have complicated the evaluation of the impact. Forced expiratory volumes have been used as the main outcome measure, but may not accurately reflect early lung function.
Despite evidence showing an improvement in nutritional outcomes following diagnosis by newborn screening (NBS) for cystic fibrosis (CF), the impact on pulmonary outcomes has been less clear. In this review the approaches to measurement of early lung function and knowledge gained from NBS CF cohorts will be described. Studies which have compared outcomes in those diagnosed by NBS to those diagnosed following symptomatic presentation will be presented. Compiling the evidence base used to evaluate the impact of NBS on pulmonary outcomes has been complicated by improvements in clinical management, infection control practices, as well as public health interventions (such as tobacco smoking bans in public places) that have evolved substantially over recent decades. Forced expiratory volumes have been used as the main outcome but it is important not to draw conclusions for 'early lung function' from tests such as spirometry alone, which lack sensitivity in early lung disease. There is, at present, insufficient evidence to draw firm conclusions about the effect of NBS on early lung function. In an era of highly effective treatments targeting the underlying molecular defect responsible for CF, future opportunities for early initiation of treatment may mean that the impact of NBS on early lung function may yet to be realised. (c) 2020 Elsevier Ltd. All rights reserved.

Authors

I am an author on this paper
Click your name to claim this paper and add it to your profile.

Reviews

Primary Rating

4.4
Not enough ratings

Secondary Ratings

Novelty
-
Significance
-
Scientific rigor
-
Rate this paper

Recommended

No Data Available
No Data Available