1
40
2
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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
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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/10.1002/ajmg.a.31169" target="_blank" rel="noreferrer noopener">http://doi.org/10.1002/ajmg.a.31169</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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Behavioral and temperamental features of children with Costello syndrome
Publisher
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American Journal of Medical Genetics Part A
Date
A point or period of time associated with an event in the lifecycle of the resource
2006
Subject
The topic of the resource
Male; Child Behavior; Child; Humans; Female; Child Preschool; Syndrome; Linear Models; Abnormalities Multiple/px [Psychology]; Temperament; Abnormalities Multiple/ge [Genetics]; Abnormalities Multiple/pa [Pathology]; Aggression/px [Psychology]; Developmental Disabilities/pa [Pathology]; Face/ab [Abnormalities]; Growth Disorders/pa [Pathology]; Juvenile Delinquency/px [Psychology]; Shyness; behavioral problems; Costello syndrome; trajectory; characteristics; internalizing problems
Creator
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Galera C; Delrue M A; Goizet C; Etchegoyhen K; Taupiac E; Sigaudy S; Arveiler B; Philip N; Bouvard M; Lacombe D
Description
An account of the resource
Costello syndrome (CS) is a rare genetic condition due to germline mutations in HRAS proto-oncogene and characterized by increased birth weight, postnatal growth retardation, distinctive facial appearance, typical medical problems (including feeding problems in the neonatal period), cutaneous anomalies, and developmental delay. Outgoing personality has often been noted in case reports, but few studies have focused specifically on the behavioral aspects of CS. A preliminary survey described irritability in younger patients with improvement between age 2 and 4, but a standardized psychometric tool was not used. A second study using the Child Behavior Checklist (CBCL) showed relatively high (albeit subclinical) levels of internalizing problems. These descriptive investigations lacked a control group. We describe a comparative survey to evaluate the behavioral and temperamental features of children with CS. We conducted a cross-sectional assessment using the CBCL and the Emotionality, Activity, Shyness, Sociability (EAS) temperament questionnaire to evaluate behavior and temperament in 11 CS children (2 years 5 months to 9 years) comparing them to 33 gender- and age-matched children without disability. The results suggest that the high levels of internalizing problems found before age 4 in CS patients might decrease with age. They also point to possible "hyperemotionality." Further studies using a larger sample size and IQ-matched control groups are needed to more accurately characterize individuals with this rare syndrome.Copyright 2006 Wiley-Liss, Inc.
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1002/ajmg.a.31169" target="_blank" rel="noreferrer noopener">10.1002/ajmg.a.31169</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).
2006
Abnormalities Multiple/ge [Genetics]
Abnormalities Multiple/pa [Pathology]
Abnormalities Multiple/px [Psychology]
Aggression/px [Psychology]
American Journal of Medical Genetics Part A
Arveiler B
behavioral problems
Bouvard M
characteristics
Child
Child Preschool
Child Behavior
costello syndrome
Delrue M A
Developmental Disabilities/pa [Pathology]
Etchegoyhen K
Face/ab [Abnormalities]
Female
Galera C
Goizet C
Growth Disorders/pa [Pathology]
Humans
internalizing problems
Juvenile Delinquency/px [Psychology]
Lacombe D
Linear Models
Male
Philip N
Shyness
Sigaudy S
Syndrome
Taupiac E
Temperament
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/10.1007/bf02172213" target="_blank" rel="noreferrer noopener">http://doi.org/10.1007/bf02172213</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
Self-injury in Lesch-Nyhan disease
Publisher
An entity responsible for making the resource available
Journal of Autism and Developmental Disorders
Date
A point or period of time associated with an event in the lifecycle of the resource
1994
Subject
The topic of the resource
Adolescent; Adult; Aggression/px [Psychology]; Arousal; Child; behavioral problems; Lesch-Nyhan syndrome; trajectory; characteristics; self-injury
Creator
An entity primarily responsible for making the resource
Anderson L T; Ernst M
Description
An account of the resource
UNLABELLED: Parents of 40 patients with Lesch-Nyhan disease completed a questionnaire detailing developmental history, life course, management, medication, factors influencing variability and topography of self-injury. Several conclusions were reached. Characteristics: Biting was the predominant form, perhaps only because of the difficulty of preventing it. There was considerable variability in self-injury which was strongly related to stress rather than to operant influences. Even though patients could not inhibit self-injury they could predict it and request restraints. Aggression against others was as prevalent as self-injury. MANAGEMENT: Stress reduction, teeth extraction, and physical restraint were the most commonly used management techniques. Behavior modification was of limited efficacy. Benzodiazepines were the most commonly used medications for controlling self-injury. OUTCOME: The severity of self-injury did not change over years. Age of onset was a predictor of outcome. The earlier the age of onset the worse the self-injury eventually became. The discussion describes research strategies, suggests dimensions along which self-injury can be classified, and highlights behavior not commonly described in patients with Lesch-Nyhan disease.
Identifier
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<a href="http://doi.org/10.1007/bf02172213" target="_blank" rel="noreferrer noopener">10.1007/bf02172213</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).
1994
Adolescent
Adult
Aggression/px [Psychology]
Anderson L T
Arousal
behavioral problems
characteristics
Child
Ernst M
Journal of Autism and Developmental Disorders
Lesch-Nyhan syndrome
self-injury
Trajectory