1
40
4
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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
A name given to the resource
Treatment of Symptoms in Children with Q3 Conditions Scoping Review Results
Text
A resource consisting primarily of words for reading. Examples include books, letters, dissertations, poems, newspapers, articles, archives of mailing lists. Note that facsimiles or images of texts are still of the genre Text.
URL Address
<a href="http://doi.org/10.1007/s10545-012-9572-0" target="_blank" rel="noreferrer noopener">http://doi.org/10.1007/s10545-012-9572-0</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
A name given to the resource
Behavioural phenotypes of the mucopolysaccharide disorders: a systematic literature review of cognitive, motor, social, linguistic and behavioural presentation in the MPS disorders
Publisher
An entity responsible for making the resource available
Journal of Inherited Metabolic Disease
Date
A point or period of time associated with an event in the lifecycle of the resource
2013
Subject
The topic of the resource
children; Medicine; Endocrinology & Metabolism; Genetics & Heredity; Research & Experimental; syndrome; mental-retardation; annotation; clinical variability; hunters; mild; sanfilippo b-disease; behavior; tone and motor problems; MPSI; MPSII; MPSIIIA; MPSIIIB; trajectory; characteristics; sleep disturbance; challenging behavior
Creator
An entity primarily responsible for making the resource
Cross E M; Hare D J
Description
An account of the resource
The mucopolysaccharide disorders (MPS) are a group of recessively inherited metabolic disorders resulting in progressive physical and cognitive decline. MEDLINE, PsycINFO and Embase databases were searched, alongside manual screening, to identify relevant literature. Papers were included in the review if they were published in a peer reviewed journal and conducted empirical research into cognitive, motor, social or linguistic development or behaviour in one or more MPS disorders. Twenty-five papers were reviewed. Two papers used methodology of a sufficiently high standard to demonstrate a behavioural phenotype; both found sleep disturbance to be part of the phenotype of MPS III. Fearfulness and sleep disturbance were frequently observed in people with MPS I and II. Cognitive and motor impairment and decline, and challenging behaviour were highly prevalent in the severe form of MPS II. Cognitive decline and severe behavioural problems relating to aggression, hyperactivity, orality, unusual affect and temper tantrums were seen in MPS III. Sleep disturbance is part of the behavioural phenotype of MPS III, and challenging behaviour is highly prevalent in MPS II and MPS III, therefore the efficacy of behavioural interventions for these populations should be investigated. Further research into the behaviour and adaptive skills of children with MPS III and MPS IV is required.
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1007/s10545-012-9572-0" target="_blank" rel="noreferrer noopener">10.1007/s10545-012-9572-0</a>
Rights
Information about rights held in and over the resource
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).
2013
annotation
Behavior
challenging behavior
characteristics
Children
clinical variability
Cross E M
Endocrinology & Metabolism
Genetics & Heredity
Hare D J
hunters
Journal Of Inherited Metabolic Disease
Medicine
mental-retardation
mild
MPSI
MPSII
MPSIIIA
MPSIIIB
Research & Experimental
sanfilippo b-disease
sleep disturbance
Syndrome
tone and motor problems
Trajectory
-
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
A name given to the resource
Treatment of Symptoms in Children with Q3 Conditions Scoping Review Results
Text
A resource consisting primarily of words for reading. Examples include books, letters, dissertations, poems, newspapers, articles, archives of mailing lists. Note that facsimiles or images of texts are still of the genre Text.
URL Address
<a href="http://doi.org/10.1186/1866-1955-6-46" target="_blank" rel="noreferrer noopener">http://doi.org/10.1186/1866-1955-6-46</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
A name given to the resource
An investigation of the middle and latebehavioural phenotypes of Mucopolysaccharidosis Type-III
Publisher
An entity responsible for making the resource available
Journal of Neurodevelopmental Disorders
Date
A point or period of time associated with an event in the lifecycle of the resource
2014
Subject
The topic of the resource
health sciences; digital collections; electronic information; information dissemination; life sciences; medical research; scientific information; behavioral problems; MPS III; trajectory; characteristics; challenging behaviors
Creator
An entity primarily responsible for making the resource
Cross E M; Grant S; Jones S; Bigger B W; Wraith J E; Mahon L V; Lomax M; Hare D J; Canada Government of Canada National Research Council
Description
An account of the resource
PubMed Central Canada (PMC Canada) provides free access to a stable and permanent online digital archive of full-text, peer-reviewed health and life sciences research publications. It builds on PubMed Central (PMC), the U.S. National Institutes of Health (NIH) free digital archive of biomedical and life sciences journal literature and is a member of the broader PMC International (PMCI) network of e-repositories.
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1186/1866-1955-6-46" target="_blank" rel="noreferrer noopener">10.1186/1866-1955-6-46</a>
Rights
Information about rights held in and over the resource
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).
2014
behavioral problems
Bigger B W
Canada Government of Canada National Research Council
challenging behaviors
characteristics
Cross E M
digital collections
electronic information
Grant S
Hare D J
Health Sciences
Information Dissemination
Jones S
Journal of Neurodevelopmental Disorders
Life Sciences
Lomax M
Mahon L V
Medical Research
MPS III
scientific information
Trajectory
Wraith J E
-
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
A name given to the resource
Treatment of Symptoms in Children with Q3 Conditions Scoping Review Results
Text
A resource consisting primarily of words for reading. Examples include books, letters, dissertations, poems, newspapers, articles, archives of mailing lists. Note that facsimiles or images of texts are still of the genre Text.
URL Address
<a href="http://doi.org/10.1371/journal.pone.0084128" target="_blank" rel="noreferrer noopener">http://doi.org/10.1371/journal.pone.0084128</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
A name given to the resource
Assessment of sleep in children with mucopolysaccharidosis type III
Publisher
An entity responsible for making the resource available
PLoS ONE
Date
A point or period of time associated with an event in the lifecycle of the resource
2014
Subject
The topic of the resource
Actigraphy; Adolescent; Case-Control Studies; Child; Preschool; Female; Humans; Male; Melatonin/me [Metabolism]; Mucopolysaccharidosis III/pp [Physiopathology]; Sleep/ph [Physiology]; Time Factors; JL5DK93RCL (Melatonin); sleep disturbance/disorders; MPSIIIA; MPSIIIB; trajectory; characteristics
Creator
An entity primarily responsible for making the resource
Mahon L V; Lomax M; Grant S; Cross E; Hare D J; Wraith J E; Jones S; Bigger B; Langford-Smith K; Canal M
Description
An account of the resource
Sleep disturbances are prevalent in mucopolysaccharidosis Type III (MPS III), yet there is a lack of objective, ecologically valid evidence detailing sleep quantity, quality or circadian system. Eight children with MPS III and eight age-matched typically developing children wore an actigraph for 7-10 days/nights. Saliva samples were collected at three time-points on two separate days, to permit analysis of endogenous melatonin levels. Parents completed a sleep questionnaire and a daily sleep diary. Actigraphic data revealed that children with MPS III had significantly longer sleep onset latencies and greater daytime sleep compared to controls, but night-time sleep duration did not differ between groups. In the MPS III group, sleep efficiency declined, and sleep onset latency increased, with age. Questionnaire responses showed that MPS III patients had significantly more sleep difficulties in all domains compared to controls. Melatonin concentrations showed an alteration in the circadian system in MPS III, which suggests that treatment for sleep problems should attempt to synchronise the sleep-wake cycle to a more regular pattern. Actigraphy was tolerated by children and this monitoring device can be recommended as a measure of treatment success in research and clinical practice.
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1371/journal.pone.0084128" target="_blank" rel="noreferrer noopener">10.1371/journal.pone.0084128</a>
Rights
Information about rights held in and over the resource
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).
2014
Actigraphy
Adolescent
Bigger B
Canal M
Case-Control Studies
characteristics
Child
Cross E
Female
Grant S
Hare D J
Humans
JL5DK93RCL (Melatonin)
Jones S
Langford-Smith K
Lomax M
Mahon L V
Male
Melatonin/me [Metabolism]
MPSIIIA
MPSIIIB
Mucopolysaccharidosis III/pp [Physiopathology]
PLoS One
Preschool
sleep disturbance/disorders
Sleep/ph [Physiology]
Time Factors
Trajectory
Wraith J E
-
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
A name given to the resource
Treatment of Symptoms in Children with Q3 Conditions Scoping Review Results
Text
A resource consisting primarily of words for reading. Examples include books, letters, dissertations, poems, newspapers, articles, archives of mailing lists. Note that facsimiles or images of texts are still of the genre Text.
URL Address
<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
A name given to the resource
Actigraphic investigation of circadian rhythm functioning and activity levels in children with mucopolysaccharidosis type III (Sanfilippo syndrome)
Publisher
An entity responsible for making the resource available
Journal of Neurodevelopmental Disorders
Date
A point or period of time associated with an event in the lifecycle of the resource
2015
Subject
The topic of the resource
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
Creator
An entity primarily responsible for making the resource
Mumford R A; Mahon L V; Jones S; Bigger B; Canal M; Hare D J
Description
An account of the resource
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.
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1186/s11689-015-9126-5" target="_blank" rel="noreferrer noopener">10.1186/s11689-015-9126-5</a>
Rights
Information about rights held in and over the resource
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