TY - JOUR
T1 - Lung function in children with cystic fibrosis in the USA and UK
T2 - A comparative longitudinal analysis of national registry data
AU - Schluter, Daniela K.
AU - Ostrenga, Josh S.
AU - Carr, Siobhan B.
AU - Fink, Aliza K.
AU - Faro, Albert
AU - Szczesniak, Rhonda D.
AU - Keogh, Ruth H.
AU - Charman, Susan C.
AU - Marshall, Bruce C.
AU - Goss, Christopher H.
AU - Taylor-Robinson, David
N1 - Publisher Copyright:
©
PY - 2022/2/1
Y1 - 2022/2/1
N2 - Rationale A previous analysis found significantly higher lung function in the US paediatric cystic fibrosis (CF) population compared with the UK with this difference apparently decreasing in adolescence and adulthood. However, the cross-sectional nature of the study makes it hard to interpret these results. Objectives To compare longitudinal trajectories of lung function in children with CF between the USA and UK and to explore reasons for any differences. Methods We used mixed effects regression analysis to model lung function trajectories in the study populations. Using descriptive statistics, we compared early growth and nutrition (height, weight, body mass index), infections (Pseudomonas aeruginosa, Staphylococcus aureus) and treatments (rhDnase, hypertonic saline, inhaled antibiotics). Results We included 9463 children from the USA and 3055 children from the UK with homozygous F508del genotype. Lung function was higher in the USA than in the UK when first measured at age six and remained higher throughout childhood. We did not find important differences in early growth and nutrition, or P.aeruginosa infection. Prescription of rhDNase and hypertonic saline was more common in the USA. Inhaled antibiotics were prescribed at similar levels in both countries, but Tobramycin was prescribed more in the USA and colistin in the UK. S. aureus infection was more common in the USA than the UK. Conclusions Children with CF and homozygous F508del genotype in the USA had better lung function than UK children. These differences do not appear to be explained by early growth or nutrition, but differences in the use of early treatments need further investigation.
AB - Rationale A previous analysis found significantly higher lung function in the US paediatric cystic fibrosis (CF) population compared with the UK with this difference apparently decreasing in adolescence and adulthood. However, the cross-sectional nature of the study makes it hard to interpret these results. Objectives To compare longitudinal trajectories of lung function in children with CF between the USA and UK and to explore reasons for any differences. Methods We used mixed effects regression analysis to model lung function trajectories in the study populations. Using descriptive statistics, we compared early growth and nutrition (height, weight, body mass index), infections (Pseudomonas aeruginosa, Staphylococcus aureus) and treatments (rhDnase, hypertonic saline, inhaled antibiotics). Results We included 9463 children from the USA and 3055 children from the UK with homozygous F508del genotype. Lung function was higher in the USA than in the UK when first measured at age six and remained higher throughout childhood. We did not find important differences in early growth and nutrition, or P.aeruginosa infection. Prescription of rhDNase and hypertonic saline was more common in the USA. Inhaled antibiotics were prescribed at similar levels in both countries, but Tobramycin was prescribed more in the USA and colistin in the UK. S. aureus infection was more common in the USA than the UK. Conclusions Children with CF and homozygous F508del genotype in the USA had better lung function than UK children. These differences do not appear to be explained by early growth or nutrition, but differences in the use of early treatments need further investigation.
KW - clinical epidemiology
KW - cystic fibrosis
KW - paediatric lung disaese
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U2 - 10.1136/thoraxjnl-2021-216849
DO - 10.1136/thoraxjnl-2021-216849
M3 - Article
C2 - 33975926
AN - SCOPUS:85106059175
SN - 0040-6376
VL - 77
SP - 136
EP - 142
JO - Thorax
JF - Thorax
IS - 2
ER -