Changes in physiological, functional and structural markers of cystic fibrosis lung disease with treatment of a pulmonary exacerbation

Alexander Horsley; Alex R. Horsley; Jane C. Davies; Robert D. Gray; Kenneth A. MacLeod; Jackie Donovan; Zelena A. Aziz; Nicholas J. Bell; Margaret Rainer; Shahrul Mt-Isa; Nia Voase; Maria H. Dewar; Clare Saunders; James S. Gibson; Javier Parra-Leiton; Mia D. Larsen; Sarah Jeswiet; Samia Soussi; Yusura Bakar; Mark G. Meister; Philippa Tyler; Ann Doherty; David M. Hansell; Deborah Ashby; Stephen C. Hyde; Deborah R. Gill; Andrew P. Greening; David J. Porteous; J. Alastair Innes; A. Christopher Boyd; Uta Griesenbach; Steve Cunningham; Eric W F W Alton

doi:10.1136/thoraxjnl-2012-202538

Changes in physiological, functional and structural markers of cystic fibrosis lung disease with treatment of a pulmonary exacerbation

Alexander Horsley, Alex R. Horsley, Jane C. Davies, Robert D. Gray, Kenneth A. MacLeod, Jackie Donovan, Zelena A. Aziz, Nicholas J. Bell, Margaret Rainer, Shahrul Mt-Isa, Nia Voase, Maria H. Dewar, Clare Saunders, James S. Gibson, Javier Parra-Leiton, Mia D. Larsen, Sarah Jeswiet, Samia Soussi, Yusura Bakar, Mark G. MeisterPhilippa Tyler, Ann Doherty, David M. Hansell, Deborah Ashby, Stephen C. Hyde, Deborah R. Gill, Andrew P. Greening, David J. Porteous, J. Alastair Innes, A. Christopher Boyd, Uta Griesenbach, Steve Cunningham, Eric W F W Alton

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Abstract

Background: Clinical trials in cystic fibrosis (CF) have been hindered by the paucity of well characterised and clinically relevant outcome measures. Aim: To evaluate a range of conventional and novel biomarkers of CF lung disease in a multicentre setting as a contributing study in selecting outcome assays for a clinical trial of CFTR gene therapy. Methods: A multicentre observational study of adult and paediatric patients with CF (>10 years) treated for a physician-defined exacerbation of CF pulmonary symptoms. Measurements were performed at commencement and immediately after a course of intravenous antibiotics. Disease activity was assessed using 46 assays across five key domains: symptoms, lung physiology, structural changes on CT, pulmonary and systemic inflammatory markers. Results: Statistically significant improvements were seen in forced expiratory volume in 1 s (p

Original language	English
Pages (from-to)	532-539
Number of pages	7
Journal	Thorax
Volume	68
Issue number	6
DOIs	https://doi.org/10.1136/thoraxjnl-2012-202538
Publication status	Published - Jun 2013

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http://thorax.bmj.com/content/68/6/532.full.pdf+html

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Horsley, A., Horsley, A. R., Davies, J. C., Gray, R. D., MacLeod, K. A., Donovan, J., Aziz, Z. A., Bell, N. J., Rainer, M., Mt-Isa, S., Voase, N., Dewar, M. H., Saunders, C., Gibson, J. S., Parra-Leiton, J., Larsen, M. D., Jeswiet, S., Soussi, S., Bakar, Y., ... Alton, E. W. F. W. (2013). Changes in physiological, functional and structural markers of cystic fibrosis lung disease with treatment of a pulmonary exacerbation. Thorax, 68(6), 532-539. https://doi.org/10.1136/thoraxjnl-2012-202538

@article{ec996ddc67aa48f6bcd8de141e2b7ef5,

title = "Changes in physiological, functional and structural markers of cystic fibrosis lung disease with treatment of a pulmonary exacerbation",

abstract = "Background: Clinical trials in cystic fibrosis (CF) have been hindered by the paucity of well characterised and clinically relevant outcome measures. Aim: To evaluate a range of conventional and novel biomarkers of CF lung disease in a multicentre setting as a contributing study in selecting outcome assays for a clinical trial of CFTR gene therapy. Methods: A multicentre observational study of adult and paediatric patients with CF (>10 years) treated for a physician-defined exacerbation of CF pulmonary symptoms. Measurements were performed at commencement and immediately after a course of intravenous antibiotics. Disease activity was assessed using 46 assays across five key domains: symptoms, lung physiology, structural changes on CT, pulmonary and systemic inflammatory markers. Results: Statistically significant improvements were seen in forced expiratory volume in 1 s (p",

author = "Alexander Horsley and Horsley, {Alex R.} and Davies, {Jane C.} and Gray, {Robert D.} and MacLeod, {Kenneth A.} and Jackie Donovan and Aziz, {Zelena A.} and Bell, {Nicholas J.} and Margaret Rainer and Shahrul Mt-Isa and Nia Voase and Dewar, {Maria H.} and Clare Saunders and Gibson, {James S.} and Javier Parra-Leiton and Larsen, {Mia D.} and Sarah Jeswiet and Samia Soussi and Yusura Bakar and Meister, {Mark G.} and Philippa Tyler and Ann Doherty and Hansell, {David M.} and Deborah Ashby and Hyde, {Stephen C.} and Gill, {Deborah R.} and Greening, {Andrew P.} and Porteous, {David J.} and {Alastair Innes}, J. and {Christopher Boyd}, A. and Uta Griesenbach and Steve Cunningham and Alton, {Eric W F W}",

year = "2013",

month = jun,

doi = "10.1136/thoraxjnl-2012-202538",

language = "English",

volume = "68",

pages = "532--539",

journal = "Thorax",

issn = "0040-6376",

publisher = "BMJ ",

number = "6",

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Horsley, A, Horsley, AR, Davies, JC, Gray, RD, MacLeod, KA, Donovan, J, Aziz, ZA, Bell, NJ, Rainer, M, Mt-Isa, S, Voase, N, Dewar, MH, Saunders, C, Gibson, JS, Parra-Leiton, J, Larsen, MD, Jeswiet, S, Soussi, S, Bakar, Y, Meister, MG, Tyler, P, Doherty, A, Hansell, DM, Ashby, D, Hyde, SC, Gill, DR, Greening, AP, Porteous, DJ, Alastair Innes, J, Christopher Boyd, A, Griesenbach, U, Cunningham, S & Alton, EWFW 2013, 'Changes in physiological, functional and structural markers of cystic fibrosis lung disease with treatment of a pulmonary exacerbation', Thorax, vol. 68, no. 6, pp. 532-539. https://doi.org/10.1136/thoraxjnl-2012-202538

TY - JOUR

T1 - Changes in physiological, functional and structural markers of cystic fibrosis lung disease with treatment of a pulmonary exacerbation

AU - Horsley, Alexander

AU - Horsley, Alex R.

AU - Davies, Jane C.

AU - Gray, Robert D.

AU - MacLeod, Kenneth A.

AU - Donovan, Jackie

AU - Aziz, Zelena A.

AU - Bell, Nicholas J.

AU - Rainer, Margaret

AU - Mt-Isa, Shahrul

AU - Voase, Nia

AU - Dewar, Maria H.

AU - Saunders, Clare

AU - Gibson, James S.

AU - Parra-Leiton, Javier

AU - Larsen, Mia D.

AU - Jeswiet, Sarah

AU - Soussi, Samia

AU - Bakar, Yusura

AU - Meister, Mark G.

AU - Tyler, Philippa

AU - Doherty, Ann

AU - Hansell, David M.

AU - Ashby, Deborah

AU - Hyde, Stephen C.

AU - Gill, Deborah R.

AU - Greening, Andrew P.

AU - Porteous, David J.

AU - Alastair Innes, J.

AU - Christopher Boyd, A.

AU - Griesenbach, Uta

AU - Cunningham, Steve

AU - Alton, Eric W F W

PY - 2013/6

Y1 - 2013/6

N2 - Background: Clinical trials in cystic fibrosis (CF) have been hindered by the paucity of well characterised and clinically relevant outcome measures. Aim: To evaluate a range of conventional and novel biomarkers of CF lung disease in a multicentre setting as a contributing study in selecting outcome assays for a clinical trial of CFTR gene therapy. Methods: A multicentre observational study of adult and paediatric patients with CF (>10 years) treated for a physician-defined exacerbation of CF pulmonary symptoms. Measurements were performed at commencement and immediately after a course of intravenous antibiotics. Disease activity was assessed using 46 assays across five key domains: symptoms, lung physiology, structural changes on CT, pulmonary and systemic inflammatory markers. Results: Statistically significant improvements were seen in forced expiratory volume in 1 s (p

AB - Background: Clinical trials in cystic fibrosis (CF) have been hindered by the paucity of well characterised and clinically relevant outcome measures. Aim: To evaluate a range of conventional and novel biomarkers of CF lung disease in a multicentre setting as a contributing study in selecting outcome assays for a clinical trial of CFTR gene therapy. Methods: A multicentre observational study of adult and paediatric patients with CF (>10 years) treated for a physician-defined exacerbation of CF pulmonary symptoms. Measurements were performed at commencement and immediately after a course of intravenous antibiotics. Disease activity was assessed using 46 assays across five key domains: symptoms, lung physiology, structural changes on CT, pulmonary and systemic inflammatory markers. Results: Statistically significant improvements were seen in forced expiratory volume in 1 s (p

U2 - 10.1136/thoraxjnl-2012-202538

DO - 10.1136/thoraxjnl-2012-202538

M3 - Article

SN - 0040-6376

VL - 68

SP - 532

EP - 539

JO - Thorax

JF - Thorax

IS - 6

ER -

Changes in physiological, functional and structural markers of cystic fibrosis lung disease with treatment of a pulmonary exacerbation

Abstract

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