Imperial College London
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ProfessorAndrewBush

Faculty of Medicine, National Heart & Lung Institute

Professor of Paediatric Respirology
 
 
 
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Contact

 

+44 (0)20 7352 8121 ext 2255a.bush

 
 
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Location

 

Chelsea WingRoyal Brompton Campus

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Summary

 

Publications

Citation

BibTex format

@article{Pifferi:2017:10.1016/j.rmed.2017.02.001,
author = {Pifferi, M and Bush, A and Caramella, D and Metelli, MR and Di, Cicco M and Piras, M and Gherarducci, G and Capristo, C and Maggi, F and Peroni, D and Boner, AL},
doi = {10.1016/j.rmed.2017.02.001},
journal = {Respiratory Medicine},
pages = {49--56},
title = {Matrix metalloproteinases and airway remodeling and function in primary ciliary dyskinesia},
url = {http://dx.doi.org/10.1016/j.rmed.2017.02.001},
volume = {124},
year = {2017}
}
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RIS format (EndNote, RefMan)

TY  - JOUR
AB  - Background: The balance between matrix metalloproteinases (MMPs) and their tissue inhibitors (TIMPs)is important in the regulation of airway damage.Objective: To evaluate whether they are important in the pathophysiology of primary and secondaryciliary dyskinesia (PCD, SCD).Methods: We measured sputum bacteriology, lung CT changes, MMPs, TIMPs and lung function in 86patients (51 PCD, 35 SCD) in a cross-sectional study; the 10 controls studied did not have HRCT or sputumcultures. MMPs, TIMPs and lung function were evaluated longitudinally for up to one year in 38 PCDpatients.Results: At baseline, there were no differences in MMPs, TIMPs and MMPs/TIMPs, between PCD and SCDbut lower levels were found in controls. There was an association between poorer lung function withincreasing levels of MMPs in PCD, while in SCD only MMP-9/TIMP-1 values correlated with FRC z-scores.Levels of MMPs and TIMPs significantly correlated with severity HRCT changes. Longitudinally, therewere significant correlations between slope of changes in spirometric parameters and slope of change insputum MMPs in PCD patients.Conclusions: In conclusion, we report for the first time that increased MMPs are associated with worseairway damage in PCD and SCD, and thus are potential therapeutic targets
AU  - Pifferi,M
AU  - Bush,A
AU  - Caramella,D
AU  - Metelli,MR
AU  - Di,Cicco M
AU  - Piras,M
AU  - Gherarducci,G
AU  - Capristo,C
AU  - Maggi,F
AU  - Peroni,D
AU  - Boner,AL
DO  - 10.1016/j.rmed.2017.02.001
EP  - 56
PY  - 2017///
SN  - 0954-6111
SP  - 49
TI  - Matrix metalloproteinases and airway remodeling and function in primary ciliary dyskinesia
T2  - Respiratory Medicine
UR  - http://dx.doi.org/10.1016/j.rmed.2017.02.001
UR  - http://gateway.webofknowledge.com/gateway/Gateway.cgi?GWVersion=2&SrcApp=PARTNER_APP&SrcAuth=LinksAMR&KeyUT=WOS:000398647500008&DestLinkType=FullRecord&DestApp=ALL_WOS&UsrCustomerID=1ba7043ffcc86c417c072aa74d649202
UR  - http://hdl.handle.net/10044/1/48988
VL  - 124
ER  -
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