Sleep-related breathing disorder in Duchenne muscular dystrophy: Disease spectrum in the paediatric population

Suresh, S., Wales, P., Dakin, C., Harris, M. A. and Cooper, D. M. (2005) Sleep-related breathing disorder in Duchenne muscular dystrophy: Disease spectrum in the paediatric population. Journal of Paediatrics And Child Health, 41 9-10: 500-503. doi:10.1111/j.1440-1754.2005.00691.x

Author Suresh, S.
Wales, P.
Dakin, C.
Harris, M. A.
Cooper, D. M.
Title Sleep-related breathing disorder in Duchenne muscular dystrophy: Disease spectrum in the paediatric population
Journal name Journal of Paediatrics And Child Health   Check publisher's open access policy
ISSN 1034-4810
Publication date 2005-09
Sub-type Article (original research)
DOI 10.1111/j.1440-1754.2005.00691.x
Open Access Status
Volume 41
Issue 9-10
Start page 500
End page 503
Total pages 4
Place of publication Australia
Publisher Blackwell Publishing Asia
Collection year 2005
Language eng
Subject C1
321019 Paediatrics
321027 Respiratory Diseases
730204 Child health
730110 Respiratory system and diseases (incl. asthma)
Abstract Duchenne muscular dystrophy (DMD) is a progressive neuromuscular disease with death usually occurring because of respiratory failure. Signs of early respiratory insufficiency are usually first detectable in sleep. Objective: To study the presentation of sleep-related breathing disorder (SRBD) in patients with DMD. Method:> A retrospective review of patients with DMD attending a tertiary paediatric sleep disorder clinic over a 5-year period. Symptoms, lung function and polysomnographic indices were reviewed. Results: A total of 34 patients with DMD were referred for respiratory assessment (1-15 years). Twenty-two (64%) reported sleep-related symptomatology. Forced vital capacity (FVC) was between 12 and 107% predicted (n = 29). Thirty-two progressed to have polysomnography of which 15 were normal studies (median age: 10 years) and 10 (31%) were diagnostic of obstructive sleep apnoea (OSA) (median age: 8 years). A total of 11 patients (32%) showed hypoventilation (median age: 13 years) during the 5-year period and non-invasive ventilation (NIV) was offered to them. The median FVC of this group was 27% predicted. There was a significant improvement in the apnoea/hypopnoea index (AHI) (mean difference = 11.31, 95% CI = 5.91-16.70, P = 0.001) following the institution of NIV. Conclusions: The prevalence of SRBD in DMD is significant. There is a bimodal presentation of SRBD, with OSA found in the first decade and hypoventilation more commonly seen at the beginning of the second decade. Polysomnography is recommended in children with symptoms of OSA, or at the stage of becoming wheelchair-bound. In patients with the early stages of respiratory failure, assessment with polysomnography-identified sleep hypoventilation and assisted in initiating NIV.
Keyword Pediatrics
Duchenne Muscular Dystrophy
Non-invasive Ventilation
Obstructive Sleep Apnoea
Sleep-related Breathing Disorder
Positive-pressure Ventilation
Neuromuscular Disease
Nasal Ventilation
Noninvasive Ventilation
Daytime Predictors
Q-Index Code C1
Q-Index Status Provisional Code
Institutional Status Non-UQ

Document type: Journal Article
Sub-type: Article (original research)
Collections: Excellence in Research Australia (ERA) - Collection
2006 Higher Education Research Data Collection
School of Medicine Publications
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Citation counts: TR Web of Science Citation Count  Cited 51 times in Thomson Reuters Web of Science Article | Citations
Scopus Citation Count Cited 73 times in Scopus Article | Citations
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Created: Wed, 15 Aug 2007, 05:57:03 EST