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Routine lung volume recruitment in boys with Duchenne muscular dystrophy: a randomised clinical trial

BACKGROUND: Impaired cough results in airway secretion retention, atelectasis and pneumonia in individuals with Duchenne muscular dystrophy (DMD). Lung volume recruitment (LVR) stacks breaths to inflate the lungs to greater volumes than spontaneous effort. LVR is recommended in DMD clinical care gui...

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Autores principales: Katz, Sherri L, Mah, Jean K, McMillan, Hugh J, Campbell, Craig, Bijelić, Vid, Barrowman, Nick, Momoli, Franco, Blinder, Henrietta, Aaron, Shawn D, McAdam, Laura C, Nguyen, The Thanh Diem, Tarnopolsky, Mark, Wensley, David F, Zielinski, David, Rose, Louise, Sheers, Nicole, Berlowitz, David J, Wolfe, Lisa, McKim, Doug
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BMJ Publishing Group 2022
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9340020/
https://www.ncbi.nlm.nih.gov/pubmed/35236763
http://dx.doi.org/10.1136/thoraxjnl-2021-218196
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author Katz, Sherri L
Mah, Jean K
McMillan, Hugh J
Campbell, Craig
Bijelić, Vid
Barrowman, Nick
Momoli, Franco
Blinder, Henrietta
Aaron, Shawn D
McAdam, Laura C
Nguyen, The Thanh Diem
Tarnopolsky, Mark
Wensley, David F
Zielinski, David
Rose, Louise
Sheers, Nicole
Berlowitz, David J
Wolfe, Lisa
McKim, Doug
author_facet Katz, Sherri L
Mah, Jean K
McMillan, Hugh J
Campbell, Craig
Bijelić, Vid
Barrowman, Nick
Momoli, Franco
Blinder, Henrietta
Aaron, Shawn D
McAdam, Laura C
Nguyen, The Thanh Diem
Tarnopolsky, Mark
Wensley, David F
Zielinski, David
Rose, Louise
Sheers, Nicole
Berlowitz, David J
Wolfe, Lisa
McKim, Doug
author_sort Katz, Sherri L
collection PubMed
description BACKGROUND: Impaired cough results in airway secretion retention, atelectasis and pneumonia in individuals with Duchenne muscular dystrophy (DMD). Lung volume recruitment (LVR) stacks breaths to inflate the lungs to greater volumes than spontaneous effort. LVR is recommended in DMD clinical care guidelines but is not well studied. We aimed to determine whether twice-daily LVR, compared with standard of care alone, attenuates the decline in FVC at 2 years in boys with DMD. METHODS: In this multicentre, assessor-blinded, randomised controlled trial, boys with DMD, aged 6–16 years with FVC >30% predicted, were randomised to receive conventional treatment or conventional treatment plus manual LVR twice daily for 2 years. The primary outcome was FVC % predicted at 2 years, adjusted for baseline FVC % predicted, age and ambulatory status. Secondary outcomes included change in chest wall distensibility (maximal insufflation capacity minus FVC) and peak cough flow. RESULTS: Sixty-six boys (36 in LVR group, 30 in control) were evaluated (median age (IQR): 11.5 years (9.5–13.5), median baseline FVC (IQR): 85% predicted (73–96)). Adjusted mean difference in FVC between groups at 2 years was 1.9% predicted (95% CI −6.9% to 10.7%; p=0.68) in the direction of treatment benefit. We found no differences in secondary outcomes. CONCLUSION: There was no difference in decline in FVC % predicted with use of twice-daily LVR for boys with DMD and relatively normal lung function. The burden associated with routine LVR may outweigh the benefit. Benefits of LVR to maintain lung health in boys with worse baseline lung function still need to be clarified. TRIAL REGISTRATION NUMBER: NCT01999075.
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spelling pubmed-93400202022-08-16 Routine lung volume recruitment in boys with Duchenne muscular dystrophy: a randomised clinical trial Katz, Sherri L Mah, Jean K McMillan, Hugh J Campbell, Craig Bijelić, Vid Barrowman, Nick Momoli, Franco Blinder, Henrietta Aaron, Shawn D McAdam, Laura C Nguyen, The Thanh Diem Tarnopolsky, Mark Wensley, David F Zielinski, David Rose, Louise Sheers, Nicole Berlowitz, David J Wolfe, Lisa McKim, Doug Thorax Paediatric Lung Disease BACKGROUND: Impaired cough results in airway secretion retention, atelectasis and pneumonia in individuals with Duchenne muscular dystrophy (DMD). Lung volume recruitment (LVR) stacks breaths to inflate the lungs to greater volumes than spontaneous effort. LVR is recommended in DMD clinical care guidelines but is not well studied. We aimed to determine whether twice-daily LVR, compared with standard of care alone, attenuates the decline in FVC at 2 years in boys with DMD. METHODS: In this multicentre, assessor-blinded, randomised controlled trial, boys with DMD, aged 6–16 years with FVC >30% predicted, were randomised to receive conventional treatment or conventional treatment plus manual LVR twice daily for 2 years. The primary outcome was FVC % predicted at 2 years, adjusted for baseline FVC % predicted, age and ambulatory status. Secondary outcomes included change in chest wall distensibility (maximal insufflation capacity minus FVC) and peak cough flow. RESULTS: Sixty-six boys (36 in LVR group, 30 in control) were evaluated (median age (IQR): 11.5 years (9.5–13.5), median baseline FVC (IQR): 85% predicted (73–96)). Adjusted mean difference in FVC between groups at 2 years was 1.9% predicted (95% CI −6.9% to 10.7%; p=0.68) in the direction of treatment benefit. We found no differences in secondary outcomes. CONCLUSION: There was no difference in decline in FVC % predicted with use of twice-daily LVR for boys with DMD and relatively normal lung function. The burden associated with routine LVR may outweigh the benefit. Benefits of LVR to maintain lung health in boys with worse baseline lung function still need to be clarified. TRIAL REGISTRATION NUMBER: NCT01999075. BMJ Publishing Group 2022-08 2022-03-02 /pmc/articles/PMC9340020/ /pubmed/35236763 http://dx.doi.org/10.1136/thoraxjnl-2021-218196 Text en © Author(s) (or their employer(s)) 2022. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ. https://creativecommons.org/licenses/by-nc/4.0/This is an open access article distributed in accordance with the Creative Commons Attribution Non Commercial (CC BY-NC 4.0) license, which permits others to distribute, remix, adapt, build upon this work non-commercially, and license their derivative works on different terms, provided the original work is properly cited, appropriate credit is given, any changes made indicated, and the use is non-commercial. See: http://creativecommons.org/licenses/by-nc/4.0/ (https://creativecommons.org/licenses/by-nc/4.0/) .
spellingShingle Paediatric Lung Disease
Katz, Sherri L
Mah, Jean K
McMillan, Hugh J
Campbell, Craig
Bijelić, Vid
Barrowman, Nick
Momoli, Franco
Blinder, Henrietta
Aaron, Shawn D
McAdam, Laura C
Nguyen, The Thanh Diem
Tarnopolsky, Mark
Wensley, David F
Zielinski, David
Rose, Louise
Sheers, Nicole
Berlowitz, David J
Wolfe, Lisa
McKim, Doug
Routine lung volume recruitment in boys with Duchenne muscular dystrophy: a randomised clinical trial
title Routine lung volume recruitment in boys with Duchenne muscular dystrophy: a randomised clinical trial
title_full Routine lung volume recruitment in boys with Duchenne muscular dystrophy: a randomised clinical trial
title_fullStr Routine lung volume recruitment in boys with Duchenne muscular dystrophy: a randomised clinical trial
title_full_unstemmed Routine lung volume recruitment in boys with Duchenne muscular dystrophy: a randomised clinical trial
title_short Routine lung volume recruitment in boys with Duchenne muscular dystrophy: a randomised clinical trial
title_sort routine lung volume recruitment in boys with duchenne muscular dystrophy: a randomised clinical trial
topic Paediatric Lung Disease
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC9340020/
https://www.ncbi.nlm.nih.gov/pubmed/35236763
http://dx.doi.org/10.1136/thoraxjnl-2021-218196
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