Journal
PAEDIATRIC RESPIRATORY REVIEWS
Volume 20, Issue -, Pages 55-66Publisher
ELSEVIER SCI LTD
DOI: 10.1016/j.prrv.2016.03.002
Keywords
Cystic fibrosis; lung function; disease progression; risk factors; nonlinear decline
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A systematic review was performed (i) to describe the reported overall rate of progression of CF lung disease quantified as FEV1%predicted decline with age, (ii) to summarise identified influencing risk factors and (iii) to review methods used to analyse CF lung disease progression data. A search of publications providing FEV1%predicted values over age was conducted in PUBMED and EMBASE. Baseline and rate of FEV1%predicted decline were summarised overall and by identified risk factors. Thirty-nine studies were included and reported variable linear rates of lung function decline in patients with CF. The overall weighted mean FEV1%predicted over age was graphically summarised and showed a nonlinear, time-variant decline of lung function. Compared to their peers, Pseudomonas aeruginosa infection and pancreatic insufficiency were most commonly associated with lower baseline and more rapid FEV1%predicted declines respectively. Considering nonlinear models and drop-out in lung disease progression, analysis is lacking and more studies are warranted. (C) 2016 Elsevier Ltd. All rights reserved.
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