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Early NIV is associated with accelerated lung function decline in Duchenne muscular dystrophy treated with glucocorticosteroids

BACKGROUND: Use of non-invasive ventilation (NIV) in adolescents with Duchenne muscular dystrophy (DMD) has increased with concomitant extended survival. AIM: To describe lung function (LF) changes with NIV in adolescents with DMD and to assess differences between Steroid Users and Steroid Naïve sub...

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Detalles Bibliográficos
Autores principales: Angliss, Megan E, Sclip, Kiara D, Gauld, Leanne
Formato: Online Artículo Texto
Lenguaje:English
Publicado: BMJ Publishing Group 2020
Materias:
Acceso en línea:https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7047482/
https://www.ncbi.nlm.nih.gov/pubmed/32079608
http://dx.doi.org/10.1136/bmjresp-2019-000517
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author Angliss, Megan E
Sclip, Kiara D
Gauld, Leanne
author_facet Angliss, Megan E
Sclip, Kiara D
Gauld, Leanne
author_sort Angliss, Megan E
collection PubMed
description BACKGROUND: Use of non-invasive ventilation (NIV) in adolescents with Duchenne muscular dystrophy (DMD) has increased with concomitant extended survival. AIM: To describe lung function (LF) changes with NIV in adolescents with DMD and to assess differences between Steroid Users and Steroid Naïve subjects. METHOD: A retrospective cohort of adolescents with DMD initiating NIV over 10 years was conducted. Serial LF before and after NIV initiation was collated. Use of systemic glucocorticosteroids, adherence to NIV and presence of cardiac disease were assessed. RESULTS: Twenty-nine men started NIV, median age 14.66 years (IQR 2.35, 10.47–17.96). Nine were Steroid Users and eight were Steroid Naïve. Indications for NIV were apnoea–hypopnoea index >5 and/or nocturnal hypoventilation. LF is better (forced vital capacity (FVC) z-score −3.26 vs −5.41, p < 0.02) and decline slower (FVC z-score −0.58 per annum (pa) vs −0.68 pa, p<0.001) in Steroid Users compared with Steroid Naïve subjects. Following NIV initiation, FVC z-score decline slowed for the whole (−0.72 pa (95% CI −0.79 to 0.64) to −0.46 pa (95% CI −0.54 to 0.38) p < 0.001) and Steroid Naïve groups (−0.74 (95% CI −0.85 to 0.63) to −0.44 pa (95% CI −0.56 to 0.32) p < 0.001) but accelerated in the Steroid User group (−0.56 (95% CI −0.70 to 0.42) to −0.75 pa (95% CI −0.89 to 0.61) p < 0.001). Adherence to NIV and cardiac disease did not impact decline. CONCLUSION: Overall, LF decline is reduced on NIV. Steroid Naïve patients have lower LF and faster decline, which slows following NIV initiation. An accelerated LF decline was seen on NIV in Steroid Users which requires further prospective research.
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spelling pubmed-70474822020-03-09 Early NIV is associated with accelerated lung function decline in Duchenne muscular dystrophy treated with glucocorticosteroids Angliss, Megan E Sclip, Kiara D Gauld, Leanne BMJ Open Respir Res Non-Invasive Ventilation BACKGROUND: Use of non-invasive ventilation (NIV) in adolescents with Duchenne muscular dystrophy (DMD) has increased with concomitant extended survival. AIM: To describe lung function (LF) changes with NIV in adolescents with DMD and to assess differences between Steroid Users and Steroid Naïve subjects. METHOD: A retrospective cohort of adolescents with DMD initiating NIV over 10 years was conducted. Serial LF before and after NIV initiation was collated. Use of systemic glucocorticosteroids, adherence to NIV and presence of cardiac disease were assessed. RESULTS: Twenty-nine men started NIV, median age 14.66 years (IQR 2.35, 10.47–17.96). Nine were Steroid Users and eight were Steroid Naïve. Indications for NIV were apnoea–hypopnoea index >5 and/or nocturnal hypoventilation. LF is better (forced vital capacity (FVC) z-score −3.26 vs −5.41, p < 0.02) and decline slower (FVC z-score −0.58 per annum (pa) vs −0.68 pa, p<0.001) in Steroid Users compared with Steroid Naïve subjects. Following NIV initiation, FVC z-score decline slowed for the whole (−0.72 pa (95% CI −0.79 to 0.64) to −0.46 pa (95% CI −0.54 to 0.38) p < 0.001) and Steroid Naïve groups (−0.74 (95% CI −0.85 to 0.63) to −0.44 pa (95% CI −0.56 to 0.32) p < 0.001) but accelerated in the Steroid User group (−0.56 (95% CI −0.70 to 0.42) to −0.75 pa (95% CI −0.89 to 0.61) p < 0.001). Adherence to NIV and cardiac disease did not impact decline. CONCLUSION: Overall, LF decline is reduced on NIV. Steroid Naïve patients have lower LF and faster decline, which slows following NIV initiation. An accelerated LF decline was seen on NIV in Steroid Users which requires further prospective research. BMJ Publishing Group 2020-02-19 /pmc/articles/PMC7047482/ /pubmed/32079608 http://dx.doi.org/10.1136/bmjresp-2019-000517 Text en © Author(s) (or their employer(s)) 2020. Re-use permitted under CC BY-NC. No commercial re-use. See rights and permissions. Published by BMJ. http://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/.
spellingShingle Non-Invasive Ventilation
Angliss, Megan E
Sclip, Kiara D
Gauld, Leanne
Early NIV is associated with accelerated lung function decline in Duchenne muscular dystrophy treated with glucocorticosteroids
title Early NIV is associated with accelerated lung function decline in Duchenne muscular dystrophy treated with glucocorticosteroids
title_full Early NIV is associated with accelerated lung function decline in Duchenne muscular dystrophy treated with glucocorticosteroids
title_fullStr Early NIV is associated with accelerated lung function decline in Duchenne muscular dystrophy treated with glucocorticosteroids
title_full_unstemmed Early NIV is associated with accelerated lung function decline in Duchenne muscular dystrophy treated with glucocorticosteroids
title_short Early NIV is associated with accelerated lung function decline in Duchenne muscular dystrophy treated with glucocorticosteroids
title_sort early niv is associated with accelerated lung function decline in duchenne muscular dystrophy treated with glucocorticosteroids
topic Non-Invasive Ventilation
url https://www.ncbi.nlm.nih.gov/pmc/articles/PMC7047482/
https://www.ncbi.nlm.nih.gov/pubmed/32079608
http://dx.doi.org/10.1136/bmjresp-2019-000517
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