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Updated: Jul 16, 2025

Measurements of Motor Function and Other Clinical Outcome Parameters in Ambulant Children with Duchenne Muscular Dystrophy
09:18

Measurements of Motor Function and Other Clinical Outcome Parameters in Ambulant Children with Duchenne Muscular Dystrophy

Published on: January 12, 2019

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Can postural changes in spirometry in children with Duchenne muscular dystrophy predict sleep hypoventilation?

C Pandit1, B Kennedy2, K Waters1

  • 1Department of Respiratory Medicine, The Children's Hospital at Westmead, Sydney, NSW, Australia; Discipline of Child and Adolescent Health, Sydney Medical School, University of Sydney, Sydney, NSW, Australia.

Paediatric Respiratory Reviews
|September 11, 2023
PubMed

Insights

Children with Duchenne muscular dystrophy (DMD) experience greater lung function changes when moving from sitting to lying down. However, reduced supine lung function does not predict sleep hypoventilation in these patients.

Area of Science:

  • Pediatric Pulmonology
  • Neuromuscular Disorders
  • Sleep Medicine

Background:

  • Duchenne muscular dystrophy (DMD) is a progressive neuromuscular disorder affecting respiratory muscles.
  • Postural changes can significantly impact lung function, particularly in individuals with muscle weakness.
  • Polysomnography (PSG) is used to assess sleep-related breathing disorders.

Purpose of the Study:

  • To investigate the relationship between postural changes in lung function and polysomnography (PSG) findings in children with DMD.
  • To determine if spirometry measurements in the supine position correlate with sleep hypoventilation in DMD patients.

Main Methods:

  • A prospective cross-sectional study involving children with DMD and a control group of healthy children.
  • Spirometry was performed in both sitting and supine positions for all participants.
  • Polysomnography (PSG) was conducted within six months of spirometry for DMD patients.

Main Results:

  • Children with DMD exhibited significantly greater changes in forced vital capacity (FVC) between sitting and supine positions compared to healthy controls (9% vs. 4%, P < 0.001).
  • Of 17 DMD children, 14 (84%) had acceptable supine spirometry; mean FEV1 and FVC were 77% and 74% predicted, respectively.
  • The change in FVC (ΔFVC(sit-sup)) showed a poor correlation with polysomnography parameters, including total apnea-hypopnea index (AHI) and REM AHI.

Conclusions:

  • Children with DMD and mild restrictive lung disease demonstrate more pronounced postural effects on spirometry than healthy peers.
  • Lower supine spirometry results in DMD patients were not found to be predictive of sleep hypoventilation detected by PSG.
Abstract

Keywords:
Duchenne muscular dystrophyPolysomnographySupine spirometry

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