Central sleep-disordered breathing and the effects of oxygen therapy in infants with Prader-Willi syndrome

Urquhart, D. S., Gulliver, T., Williams, G., Harris, M. A., Nyunt, O. and Suresh, S. (2013) Central sleep-disordered breathing and the effects of oxygen therapy in infants with Prader-Willi syndrome. Archives of Disease in Childhood, 98 8: 592-595. doi:10.1136/archdischild-2012-303441

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Author Urquhart, D. S.
Gulliver, T.
Williams, G.
Harris, M. A.
Nyunt, O.
Suresh, S.
Title Central sleep-disordered breathing and the effects of oxygen therapy in infants with Prader-Willi syndrome
Journal name Archives of Disease in Childhood   Check publisher's open access policy
ISSN 0003-9888
Publication date 2013-08
Sub-type Article (original research)
DOI 10.1136/archdischild-2012-303441
Open Access Status
Volume 98
Issue 8
Start page 592
End page 595
Total pages 4
Place of publication United Kingdom
Publisher B M J Group
Collection year 2014
Language eng
Formatted abstract
Objectives To describe breathing patterns in infants with Prader-Willi Syndrome (PWS), as well as the effects of supplemental oxygen (O2) on breathing patterns. Children with PWS commonly have sleep-disordered breathing, including hypersomnolence and obstructive sleep apnoea, as well as central sleep breathing abnormalities that are present from infancy.
Design Retrospective cohort study.
Patients Infants with a diagnosis of PWS.
Setting Tertiary children's hospital.
Interventions Infants with PWS underwent full polysomnography, and in those with frequent desaturations associated with central events, supplemental O2 during sleep was started and followed with regular split-night studies (periods in both air and O2).
Results Thirty split-night studies on 10 infants (8 female) aged 0.06–1.79 (median 0.68, IQR 0.45, 1.07) years were undertaken. At baseline (ie, air), children with PWS had a median (IQR) central apnoea index (CAI) of 4.7 (1.9, 10.6) per hour, with accompanying falls in oxygen saturation (SpO2). O2 therapy led to statistically significant reductions in CAI to 2.5/hour (p=0.002), as well as a reduced central event index (CEI) and improved SpO2. No change in the number of obstructive events was noted. Central events were more prevalent in rapid-eye movement/active sleep.
Conclusions It is concluded that infants with PWS may have central sleep-disordered breathing, which, in some children, may cause frequent desaturations. Improvements in CAI and CEI as well as oxygenation were noted with O2 therapy. Longitudinal work with this patient group would help to establish the timing of onset of obstructive symptoms.

What is known about this topic
▸ Children with Prader-Willi syndrome (PWS) are known to have sleep-disordered breathing.
▸ Sleep disorders associated with PWS include both obstructive and central sleep apnoea.
What this study adds
▸ This study demonstrates that central apnoeas are commonly seen in infants with Prader-Willi syndrome (PWS)
▸ Treatment with oxygen therapy reduces the number of observed central events in infants with PWS.
▸ The frequency of central events in PWS improves over time.
Keyword Sleep
Q-Index Code C1
Q-Index Status Confirmed Code
Institutional Status UQ

Document type: Journal Article
Sub-type: Article (original research)
Collections: Official 2014 Collection
School of Medicine Publications
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Citation counts: TR Web of Science Citation Count  Cited 7 times in Thomson Reuters Web of Science Article | Citations
Scopus Citation Count Cited 9 times in Scopus Article | Citations
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