1 40 2 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/10.1002/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 A name given to the resource Behavioral and temperamental features of children with Costello syndrome Publisher An entity responsible for making the resource available 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 An entity primarily responsible for making the resource 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 An unambiguous reference to the resource within a given context <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