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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
Text
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URL Address
<a href="http://doi.org/10.1017/S0012162205001374" target="_blank" rel="noreferrer noopener">http://doi.org/10.1017/S0012162205001374</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 aspects of Lesch-Nyhan disease and its variants
Publisher
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Developmental Medicine and Child Neurology
Date
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2005
Subject
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Adolescent; Adult; Aggression; Anxiety; Child; Depression; Female; Humans; Lesch-Nyhan Syndrome/px [Psychology]; Male; Self-Injurious Behavior; self-injury; Severity of Illness Index; alertness; behavioral problems; Lesch-Nyhan syndrome; trajectory; characteristics; distractibility
Creator
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Schretlen D J; Ward J; Meyer S M; Yun J; Puig J G; Nyhan W L; Jinnah H A; Harris J C
Description
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Self-injury is a defining feature of lesch-nyhan disease (LND) but does not occur in the less severely affected Lesch-Nyhan variants (LNV). The aim of this study was to quantify behavioral and emotional abnormalities in LND and LNV. Thirty-nine informants rated 22 patients with LND (21 males, 1 female), 11 males with LNV, and 11 healthy controls (HC; 10 males, 1 female) using two well-validated rating scales. The age of patients with LND ranged from 12 years 7 months to 38 years 3 months (mean 22 y 11 mo; sd 7 y 8 mo), whereas the age range of those with LNV was 12 years 9 months to 65 years (mean 30 y 7 mo; sd 15 y 2 mo), and the healthy controls were aged 12 years 4 months to 31 years 3 months (mean 17 y 10 mo; sd 5 y 7 mo). Behavioral ratings were based on the Child Behavior Checklist and the American Association On Mental Retardation's Adaptive Behavior Scale--Residential And Community, 2nd edition. Statistical analyses revealed that patients with LND showed severe self-injury together with problematic aggression, anxious-depressed symptoms, distractibility, motor stereotypes, and disturbing interpersonal behaviors. Patients with LNV were rated as being intermediate between the HC and LND groups on all behavior scales. Although the LNV group did not differ from hcs on most scales, their reported attention problems were as severe as those found in LND. We conclude that self-injurious and aggressive behaviors are nearly universal and that other behavioral abnormalities are common in LND. Although patients with LNV typically do not self-injure or display severe aggression, attention problems are common and a few patients demonstrate other behavioral anomalies.
Identifier
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<a href="http://doi.org/10.1017/S0012162205001374" target="_blank" rel="noreferrer noopener">10.1017/S0012162205001374</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).
2005
Adolescent
Adult
Aggression
alertness
anxiety
behavioral problems
characteristics
Child
Depression
Developmental Medicine and Child Neurology
distractibility
Female
Harris J C
Humans
Jinnah H A
Lesch-Nyhan syndrome
Lesch-Nyhan Syndrome/px [Psychology]
Male
Meyer S M
Nyhan W L
Puig J G
Schretlen D J
Self-Injurious behavior
self-injury
Severity Of Illness Index
Trajectory
Ward J
Yun J