A systematic review of studies examining the rate of lung function decline in patients with cystic fibrosis.

Abstract:

: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.

journal_name

Paediatr Respir Rev

authors

Harun SN,Wainwright C,Klein K,Hennig S

doi

10.1016/j.prrv.2016.03.002

subject

Has Abstract

pub_date

2016-09-01 00:00:00

pages

55-66

eissn

1526-0542

issn

1526-0550

pii

S1526-0542(16)00029-4

journal_volume

20

pub_type

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