• BMJ Open Respir Res · Feb 2020

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

    • Megan E Angliss, Kiara D Sclip, and Leanne Gauld.
    • Respiratory Medicine, Children's Health Queensland Hospital and Health Service, South Birsbane, Queensland, Australia.
    • BMJ Open Respir Res. 2020 Feb 1; 7 (1).

    BackgroundUse of non-invasive ventilation (NIV) in adolescents with Duchenne muscular dystrophy (DMD) has increased with concomitant extended survival.AimTo describe lung function (LF) changes with NIV in adolescents with DMD and to assess differences between Steroid Users and Steroid Naïve subjects.MethodA 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.ResultsTwenty-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.ConclusionOverall, 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.© 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.

      Pubmed     Free full text   Copy Citation     Plaintext  

      Add institutional full text...

    Notes

     
    Knowledge, pearl, summary or comment to share?
    300 characters remaining
    help        
    You can also include formatting, links, images and footnotes in your notes
    • Simple formatting can be added to notes, such as *italics*, _underline_ or **bold**.
    • Superscript can be denoted by <sup>text</sup> and subscript <sub>text</sub>.
    • Numbered or bulleted lists can be created using either numbered lines 1. 2. 3., hyphens - or asterisks *.
    • Links can be included with: [my link to pubmed](http://pubmed.com)
    • Images can be included with: ![alt text](https://bestmedicaljournal.com/study_graph.jpg "Image Title Text")
    • For footnotes use [^1](This is a footnote.) inline.
    • Or use an inline reference [^1] to refer to a longer footnote elseweher in the document [^1]: This is a long footnote..

    hide…

What will the 'Medical Journal of You' look like?

Start your free 21 day trial now.

We guarantee your privacy. Your email address will not be shared.