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Dublin Core
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Title
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Treatment of Symptoms in Children with Q3 Conditions Scoping Review Results
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<a href="http://doi.org/10.1186/s11689-015-9126-5" target="_blank" rel="noreferrer noopener">http://doi.org/10.1186/s11689-015-9126-5</a>
Dublin Core
The Dublin Core metadata element set is common to all Omeka records, including items, files, and collections. For more information see, http://dublincore.org/documents/dces/.
Title
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Actigraphic investigation of circadian rhythm functioning and activity levels in children with mucopolysaccharidosis type III (Sanfilippo syndrome)
Publisher
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Journal of Neurodevelopmental Disorders
Date
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2015
Subject
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Actigraphy; Sleep; rare disease; Neurosciences & Neurology; controlled-trial; intellectual disability; disturbance; melatonin; autistic spectrum disorders; Circadian rhythms; light; Mucopolysaccharidosis type III; multiple disabilities; Sanfilippo; sleep problems; smith-magenis-syndrome; therapy; sleep disturbance/disorders; MPSIIIA; MPSIIIB; trajectory; characteristics
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Mumford R A; Mahon L V; Jones S; Bigger B; Canal M; Hare D J
Description
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Background: Sleep disturbance is part of the behavioural phenotype of the rare genetic condition mucopolysaccharidosis (MPS) type III. A growing body of evidence suggests that underlying disturbance in circadian rhythm functioning may explain sleep problems within the MPS III population. Methods: Actigraphic data were recorded in eight children with MPS III over 7-10 days and compared to age-matched typically developing controls. Parameters of circadian rhythmicity and activity levels across a 24-h period were analysed. Results: Statistically and clinically significant differences between the two groups were noted. Analysis indicated that children with MPS III showed significantly increased fragmentation of circadian rhythm and reduced stability with external cues (zeitgebers), compared to controls. Average times of activity onset and offset were indicative of a phase delayed sleep-wake cycle for some children in the MPS III group. Children with MPS III had significantly higher activity levels during the early morning hours (midnight-6 am) compared to controls. Conclusions: Results are consistent with previous research into MPS III and suggest that there is an impairment in circadian rhythm functioning in children with this condition. Implications for clinical practice and the management of sleep difficulties are discussed.
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<a href="http://doi.org/10.1186/s11689-015-9126-5" target="_blank" rel="noreferrer noopener">10.1186/s11689-015-9126-5</a>
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Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
2015
Actigraphy
autistic spectrum disorders
Bigger B
Canal M
characteristics
circadian rhythms
CONTROLLED-TRIAL
disturbance
Hare D J
Intellectual Disability
Jones S
Journal of Neurodevelopmental Disorders
Light
Mahon L V
melatonin
MPSIIIA
MPSIIIB
Mucopolysaccharidosis type III
Multiple disabilities
Mumford R A
Neurosciences & Neurology
Rare Disease
Sanfilippo
Sleep
sleep disturbance/disorders
sleep problems
smith-magenis-syndrome
Therapy
Trajectory