Pulmonary function tests for evaluating the severity of Duchenne muscular dystrophy disease

Hagit Levine*, Itai Goldfarb*, Julia Katz, Moriah Carmeli, Tzippy Shochat, Huda Mussaffi, Sharon Aharoni, Dario Prais, Yoram Nevo

*Corresponding author for this work

Research output: Contribution to journalArticlepeer-review

5 Scopus citations

Abstract

Aim: In Duchenne muscular dystrophy (DMD), lung disease contributes significantly to morbidity and mortality. This study aimed to assess the usefulness of various pulmonary function tests in evaluating DMD severity. Methods: This retrospective study analysed lung function tests of patients with DMD-treated in the multidisciplinary respiratory neuromuscular clinic at Schneiders' Children Medical Center of Israel. Data were analysed according to age, ambulatory status and glucocorticoid treatment. Results: Among 90 patients with DMD, 40/63 (63.5%) ambulatory patients and 22/27 (81.5%) nonambulatory patients successfully performed spirometry. Significant annual declines were demonstrated among nonambulatory patients, in percentile predicted forced vital capacity (3.8%) and in total lung capacity (5.5%) per year. The decline correlated with age and loss of ambulation but not with steroid treatment. Peak cough flow values were randomly distributed and did not correlate with age, ambulation or treatment. In nonambulatory patients, transcutaneous carbon dioxide measurement correlated significantly with age (r = 0.55, p = 0.02). Conclusion: Forced vital capacity, total lung capacity and transcutaneous carbon dioxide correlated with the clinical severity of disease in children with DMD. These measures may be useful in follow-up and clinical trials. A comparable correlation was not found for peak cough flow.

Original languageEnglish
Pages (from-to)854-860
Number of pages7
JournalActa Paediatrica, International Journal of Paediatrics
Volume112
Issue number4
DOIs
StatePublished - Apr 2023

Keywords

  • Duchenne muscular dystrophy
  • forced vital capacity
  • lung function
  • peak cough flow
  • restrictive lung disease

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