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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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Sleep disordered breathing in young boys with Duchenne muscular dystrophy

Hemant Sawnani1, Lunliya Thampratankul2, Rhonda D Szczesniak3

  • 1Division of Pulmonary Medicine, Cincinnati Children's Hospital Medical Center, Cincinnati, OH.

The Journal of Pediatrics
|February 28, 2015
PubMed

Insights

Sleep-disordered breathing (SDB) is common in boys with Duchenne muscular dystrophy (DMD) on steroid treatment. Obstructive sleep apnea during REM sleep is frequent and linked to body mass index (BMI).

Area of Science:

  • Pediatric Pulmonology
  • Neuromuscular Disorders
  • Sleep Medicine

Background:

  • Duchenne muscular dystrophy (DMD) is a progressive genetic disorder affecting muscle strength.
  • Steroid treatment is common in DMD but can impact respiratory function and sleep.
  • Sleep-disordered breathing (SDB) is a potential complication in DMD patients.

Purpose of the Study:

  • To characterize SDB in young boys with DMD receiving steroid therapy.
  • To investigate the association between SDB and pulmonary function tests (PFTs) in this population.

Main Methods:

  • Retrospective analysis of polysomnogram and PFT data from 110 boys with DMD under 18 years old on steroids.
  • Evaluation of spirometry, respiratory muscle strength, BMI, sleep architecture, and SDB indices.
  • Logistic regression used to determine the risk of respiratory events associated with PFT measures.

Main Results:

  • SDB was prevalent, with 63.6% experiencing obstructive sleep apnea, 33.6% central sleep apnea, and 17% hypoventilation.
  • Obstructive sleep apnea during rapid eye movement (REM) sleep correlated with higher BMI, BMI z-score, and age.
  • Reduced forced vital capacity was linked to an increased risk of hypoventilation (OR 0.8, P = .001).

Conclusions:

  • SDB is a frequent comorbidity in young boys with DMD on steroid treatment.
  • Obstructive sleep apnea during REM sleep is a significant manifestation, often severe and influenced by BMI.
  • Pulmonary function, particularly forced vital capacity, is a predictor of respiratory complications like hypoventilation.
Abstract

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