1
40
1
-
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