1
40
11
-
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/10.1017/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
A point or period of time associated with an event in the lifecycle of the resource
2005
Subject
The topic of the resource
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
An account of the resource
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
An unambiguous reference to the resource within a given context
<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
-
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/10.1017/s001216220300032x" target="_blank" rel="noreferrer noopener">http://doi.org/10.1017/s001216220300032x</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
Modes and patterns of self-mutilation in persons with Lesch-Nyhan disease
Publisher
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Developmental Medicine and Child Neurology
Date
A point or period of time associated with an event in the lifecycle of the resource
2003
Subject
The topic of the resource
Male; Surveys and Questionnaires; Child; Humans; Adult; Adolescent; Female; Child Preschool; Infant; Anticonvulsants/therapeutic use; Carbamazepine/therapeutic use; Lesch-Nyhan Syndrome/genetics; Chromosomes Human X/genetics; Diazepam/therapeutic use; Self-Injurious Behavior/diagnosis/drug therapy; behavioral problems; Lesch-Nyhan syndrome; trajectory; characteristics; self-injury
Creator
An entity primarily responsible for making the resource
Robey K L; Reck J F; Giacomini K D; Barabas G; Eddey G E
Description
An account of the resource
Lesch-Nyhan disease (LND) is a rare X-linked recessive genetic disorder associated with cognitive impairment, choreoathetosis, hyperuricemia, and the hallmark symptom of severe and involuntary self-mutilation. This study examines data gathered from a survey of 64 families in the USA and abroad regarding the self-injury of their family members who have LND. The individuals with LND ranged in age from 1 to 40 years (mean 16 years 7 months, SD 11 years 2 months) and, with the exception of one, were males. The most common initial mode of self-mutilation, and the most frequently cited past or current behavior, was biting of lips and/or fingers. Other behaviors, in order of frequency, included head banging, extension of arms when being wheeled through doorways, tipping of wheelchairs, eye-poking, fingers in wheelchair spokes, and rubbing behaviors. Hierarchical cluster analysis identified patterns of association among the types of self-mutilation. Modes of self-mutilation in which external surfaces (such as a wheelchair component) served as instruments of self-injury tended to co-occur, as did biting of lips and fingers.
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1017/s001216220300032x" target="_blank" rel="noreferrer noopener">10.1017/s001216220300032x</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).
2003
Adolescent
Adult
Anticonvulsants/therapeutic use
Barabas G
behavioral problems
Carbamazepine/therapeutic use
characteristics
Child
Child Preschool
Chromosomes Human X/genetics
Developmental Medicine and Child Neurology
Diazepam/therapeutic use
Eddey G E
Female
Giacomini K D
Humans
Infant
Lesch-Nyhan syndrome
Lesch-Nyhan Syndrome/genetics
Male
Reck J F
Robey K L
Self-Injurious Behavior/diagnosis/drug therapy
self-injury
Surveys And Questionnaires
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/10.1016/0270-3092(80)90014-4" target="_blank" rel="noreferrer noopener">http://doi.org/10.1016/0270-3092(80)90014-4</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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Serotonergic approaches to the modification of behavior in the Lesch-Nyhan syndrome
Publisher
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Applied Research in Mental Retardation
Date
A point or period of time associated with an event in the lifecycle of the resource
1980
Subject
The topic of the resource
behavioral problems; Lesch-Nyhan syndrome; pharmacologic intervention; 5-hydroxy-tryptophan; self-injurious behavior
Creator
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Nyhan W L; Johnson H G; Kaufman I A; Jones K L
Description
An account of the resource
In a series of patients with the Lesch-Nyhan syndrome a pharmacologic attempt was made to alter the balance of biogenic amines in the central nervous system by increasing the level of serotin. Patients were treated with 5-hydroxytryptophan in combination with a peripheral decarboxylase inhibitor carbidopa and with imipramine. Most patients had a striking alteration in self-mutilative behavior. However, within 1–3 months each became tolerant and this pharmacologic effect could not be produced again even a year later. These observations suggest that the characteristic behavior of the patient with the Lesch-Nyhan syndrome is related to neurotransmitter balance, and that there may be ways to modify it.
Identifier
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<a href="http://doi.org/10.1016/0270-3092(80)90014-4" target="_blank" rel="noreferrer noopener">10.1016/0270-3092(80)90014-4</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).
1980
5-hydroxy-tryptophan
Applied Research in Mental Retardation
behavioral problems
Johnson H G
Jones K L
Kaufman I A
Lesch-Nyhan syndrome
Nyhan W L
pharmacologic intervention
Self-Injurious behavior
-
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/10.1055/s-0028-1091647" target="_blank" rel="noreferrer noopener">http://doi.org/10.1055/s-0028-1091647</a>
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Title
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Prophylactic effect of L 5 hydroxytryptophan on self mutilation in the Lesch Nyhan syndrome
Publisher
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Neuropadiatrie
Date
A point or period of time associated with an event in the lifecycle of the resource
1975
Subject
The topic of the resource
behavioral problems; tone and motor problems; Lesch-Nyhan syndrome; pharmacologic intervention; L-5-Hydroxytryptophan; L-tryptophan; L-Dopa; self-injury; choreoathetosis
Creator
An entity primarily responsible for making the resource
Mizuno T; Yugari Y
Description
An account of the resource
Four cases of the Lesch Nyhan syndrome found in the Tokyo area were given L 5 hydroxytryptophan orally. L 5 hydroxy tryptophan relieved self mutilation effectively without causing any side effects. However this substance did not alleviate choreoathetoid movement. Self destructive behavior may be caused by an imbalance of cerebral amines, especially of serotonin.
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1055/s-0028-1091647" target="_blank" rel="noreferrer noopener">10.1055/s-0028-1091647</a>
Rights
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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).
1975
behavioral problems
choreoathetosis
L-5-Hydroxytryptophan
L-Dopa
L-tryptophan
Lesch-Nyhan syndrome
Mizuno T
Neuropadiatrie
pharmacologic intervention
self-injury
tone and motor problems
Yugari Y
-
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/10.1016/j.ymgme.2016.04.012" target="_blank" rel="noreferrer noopener">http://doi.org/10.1016/j.ymgme.2016.04.012</a>
Dublin Core
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Title
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A double-blind, placebo-controlled, crossover trial of the selective dopamine D1 receptor antagonist ecopipam in patients with Lesch-Nyhan disease
Publisher
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Molecular Genetics and Metabolism
Date
A point or period of time associated with an event in the lifecycle of the resource
2016
Subject
The topic of the resource
behavioral problems; Lesch-Nyhan syndrome; pharmacologic intervention; ecopipam; self-injury
Creator
An entity primarily responsible for making the resource
Khasnavis T; Torres R J; Sommerfeld B; Puig J G; Chipkin R; Jinnah H A
Description
An account of the resource
Lesch-Nyhan disease (LND) is a genetic disorder that has characteristic metabolic, neurologic, and behavioral features. There are multiple behavioral problems including impulsivity, aggressiveness, and severe recurrent self-injurious behavior (SIB). This last behavior varies considerably across subjects and may encompass self-biting, self-hitting, scratching, head banging, and other injurious actions. Current treatments for SIB involve behavioral extinction, sedatives, physical restraints, and removal of teeth. Because these interventions do not reliably control SIB, better treatments are urgently needed. Animal studies have suggested that D1-dopamine receptor antagonists such as ecopipam may suppress SIB. These observations have led to proposals that such drugs might provide effective treatment for in LND. The current study describes the results of a double-blind, three-period, crossover trial of a single dose of ecopipam in subjects with LND. The study was designed for 20 patients, but it was terminated after recruitment of only 10 patients, because interim analysis revealed unanticipated side effects. These side effects were most likely related to starting with a single large dose without any titration phase. Despite the limited data due to early termination, the drug appeared to reduce SIB in most cases. Subjects who completed the trial were eligible to continue the drug in an open-label extension phase lasting a year, and one patient who elected to continue has maintained a striking reduction in SIB for more than a year with no apparent side effects. These results suggest ecopipam could be a useful treatment for SIB in, but further studies are needed to establish an appropriate dosing regimen.
Identifier
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<a href="http://doi.org/10.1016/j.ymgme.2016.04.012" target="_blank" rel="noreferrer noopener">10.1016/j.ymgme.2016.04.012</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).
2016
behavioral problems
Chipkin R
ecopipam
Jinnah H A
Khasnavis T
Lesch-Nyhan syndrome
Molecular Genetics and Metabolism
pharmacologic intervention
Puig J G
self-injury
Sommerfeld B
Torres R J
-
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/10.1017/s0012162201001360" target="_blank" rel="noreferrer noopener">http://doi.org/10.1017/s0012162201001360</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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Self-injurious behaviour in young children with Lesch-Nyhan syndrome
Publisher
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Developmental Medicine and Child Neurology
Date
A point or period of time associated with an event in the lifecycle of the resource
2001
Subject
The topic of the resource
Male; Severity of Illness Index; Humans; Child Preschool; Environment; Extinction Psychological; Lesch-Nyhan Syndrome/diagnosis/prevention & control; Self-Injurious Behavior/prevention & control; Sign Language; behavioral problems; Lesch-Nyhan syndrome; trajectory; characteristics; self-injury
Creator
An entity primarily responsible for making the resource
Hall S; Oliver C; Murphy G
Description
An account of the resource
The early development of self-injurious behaviour in three young boys (aged 17, 25, and 30 months at start of study) with Lesch-Nyhan syndrome was examined by means of parental interviews and by direct observations completed at 3 to 4 monthly intervals over an 18-month period. Results suggest that the self-injury began in a different way from that of other young children with autism and/or developmental disabilities in that, from the start, self-injurious responses were sudden and violent, rather than emerging gradually over time. Drastic measures, such as removal of the teeth or provision of tooth guards, were often taken to prevent further tissue damage. Direct observations showed that the boys' self-injury occurred at lower rates, but their carers were highly concerned about the behaviour. Sequential analysis of the observational data indicated that on some occasions the children were more likely to self-injure during periods of low social interaction, suggesting that their self-injury may have been influenced by environmental factors. The theoretical and practical implications of these findings are discussed.
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1017/s0012162201001360" target="_blank" rel="noreferrer noopener">10.1017/s0012162201001360</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).
2001
behavioral problems
characteristics
Child Preschool
Developmental Medicine and Child Neurology
Environment
Extinction Psychological
Hall S
Humans
Lesch-Nyhan syndrome
Lesch-Nyhan Syndrome/diagnosis/prevention & control
Male
Murphy G
Oliver C
Self-Injurious Behavior/prevention & control
self-injury
Severity Of Illness Index
Sign Language
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/10.1002/mds.23478" target="_blank" rel="noreferrer noopener">http://doi.org/10.1002/mds.23478</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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Levodopa is not a useful treatment for Lesch-Nyhan disease
Publisher
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Movement Disorders
Date
A point or period of time associated with an event in the lifecycle of the resource
2011
Subject
The topic of the resource
behavior problems; tone and motor problems; Lesch-Nyhan syndrome; pharmacologic intervention; levodopa; levodopa-dopa; carbidopa
Creator
An entity primarily responsible for making the resource
Bloem B R; Schretlen D J; Jinnah H A; Visser J E
Description
An account of the resource
Lesch-Nyhan disease (LND) is characterized by dystonia, cognitive abnormalities, and self-injurious behavior. No effective therapies are available. LND is associated with a presynaptic dopaminergic deficit, but the reported effects of dopamine replacement therapy are conflicting. The current prospective open-label study assesses the effects of levodopa on both neurological and behavioral features of LND. All 6 study participants discontinued levodopa early, due to lack of effect and sometimes worsening of motor function. The results provide important clues for pathophysiological mechanisms and suggestions for future treatment options.Copyright © 2011 Movement Disorder Society.
Identifier
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<a href="http://doi.org/10.1002/mds.23478" target="_blank" rel="noreferrer noopener">10.1002/mds.23478</a>
Rights
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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).
2011
behavior problems
Bloem B R
carbidopa
Jinnah H A
Lesch-Nyhan syndrome
levodopa
levodopa-dopa
Movement Disorders
pharmacologic intervention
Schretlen D J
tone and motor problems
Visser 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/10.1037/0022-006X.46.3.529" target="_blank" rel="noreferrer noopener">http://doi.org/10.1037/0022-006X.46.3.529</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 contingencies and self-mutilation in Lesch-Nyhan disease
Publisher
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Journal of Consulting and Clinical Psychology
Date
A point or period of time associated with an event in the lifecycle of the resource
1978
Subject
The topic of the resource
Adolescent; Behavior Therapy/mt [Methods]; Child; Preschool; Humans; Lesch-Nyhan Syndrome/px [Psychology]; Lesch-Nyhan Syndrome/th [Therapy]; Male Punishment; Self Mutilation/th [Therapy]; behavioral problems; Lesch-Nyhan syndrome; psychological intervention; aversive stimulation electric shock; positive reinforcement; time-out; self-injury; self-mutilation
Creator
An entity primarily responsible for making the resource
Anderson L; Dancis J; Alpert M
Description
An account of the resource
Lesch-Nyhan syndrome is a rare, sex-linked, recessive disease that is accompanied by severe self-mutilation, especially finger biting. Evidence is presented suggesting that parental response patterns may contribute to the genesis of the self-injurious behavior (SIB). The therapeutic effectiveness of punishment, positive reinforcement of either SIB or non-SIB, and time-out learning paradigms were evaluated in 5 Ss aged 3-13 yrs. Electric skin shock failed to suppress the behavior. Positive reinforcement of non-self-injury and time-out from social reinforcement were consistently and rapidly effective, indicating a complex interaction of genetic and environmental factors in the production of SIB. Elimination or major reductions in incidence of SIB was maintained during follow-up periods of 2 yrs. (19 ref)
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1037/0022-006X.46.3.529" target="_blank" rel="noreferrer noopener">10.1037/0022-006X.46.3.529</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).
1978
Adolescent
Alpert M
Anderson L
aversive stimulation electric shock
Behavior Therapy/mt [Methods]
behavioral problems
Child
Dancis J
Humans
Journal Of Consulting And Clinical Psychology
Lesch-Nyhan syndrome
Lesch-Nyhan Syndrome/px [Psychology]
Lesch-Nyhan Syndrome/th [Therapy]
Male Punishment
positive reinforcement
Preschool
psychological intervention
Self Mutilation/th [Therapy]
self-injury
self-mutilation
time-out
-
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/bf01058150" target="_blank" rel="noreferrer noopener">http://doi.org/10.1007/bf01058150</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
Cognitive abilities of patients with 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
1992
Subject
The topic of the resource
Male; Child; Humans; Adult; Adolescent; Female; Child Preschool; Social Environment; Combined Modality Therapy; Social Adjustment; Social Behavior; Behavior Therapy; Awareness; Intelligence; Neuropsychological Tests; Achievement; Lesch-Nyhan Syndrome/di [Diagnosis]; Intellectual Disability/di [Diagnosis]; Intellectual Disability/px [Psychology]; Intellectual Disability/th [Therapy]; Language Development Disorders/di [Diagnosis]; Language Development Disorders/px [Psychology]; Language Development Disorders/th [Therapy]; Lesch-Nyhan Syndrome/px [Psychology]; Lesch-Nyhan Syndrome/th [Therapy]; Self-Injurious Behavior/di [Diagnosis]; Self-Injurious Behavior/px [Psychology]; Self-Injurious Behavior/th [Therapy]; Thinking; alertness; behavioral; Lesch-Nyhan syndrome; trajectory; characteristics; attempted suicide; suicide
Creator
An entity primarily responsible for making the resource
Anderson L T; Ernst M; Davis S V
Description
An account of the resource
Parents of 42 patients with Lesch-Nyhan disease completed a questionnaire systematizing caregiver observations of the subject's behavior during a wide variety of daily events. Responses were grouped in nine categories reflecting different aspects of cognitive skills. Only 1 boy appears to have any significant generalized cognitive impairment. The patients' memory for both recent and past events is excellent, their emotional life has a normal range of reactions and is appropriate; they have good concentration, are capable of abstract reasoning, have good self-awareness, and are highly social. However, they are behind in academic ability, with only 15% at grade level for math and reading. Implications for designing educational activities, parenting or caregiver strategies, and research methodology are discussed.
Identifier
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<a href="http://doi.org/10.1007/bf01058150" target="_blank" rel="noreferrer noopener">10.1007/bf01058150</a>
Rights
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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).
1992
achievement
Adolescent
Adult
alertness
Anderson L T
attempted suicide
Awareness
Behavior Therapy
behavioral
characteristics
Child
Child Preschool
Combined Modality Therapy
Davis S V
Ernst M
Female
Humans
Intellectual Disability/di [Diagnosis]
Intellectual Disability/px [Psychology]
Intellectual Disability/th [Therapy]
Intelligence
Journal of Autism and Developmental Disorders
Language Development Disorders/di [Diagnosis]
Language Development Disorders/px [Psychology]
Language Development Disorders/th [Therapy]
Lesch-Nyhan syndrome
Lesch-Nyhan Syndrome/di [Diagnosis]
Lesch-Nyhan Syndrome/px [Psychology]
Lesch-Nyhan Syndrome/th [Therapy]
Male
Neuropsychological Tests
Self-Injurious Behavior/di [Diagnosis]
Self-Injurious Behavior/px [Psychology]
Self-Injurious Behavior/th [Therapy]
Social Adjustment
Social Behavior
Social Environment
Suicide
thinking
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
-
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/10.3171/2011.8.PEDS11153" target="_blank" rel="noreferrer noopener">http://doi.org/10.3171/2011.8.PEDS11153</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
Deep brain stimulation in children: experience and technical pearls
Publisher
An entity responsible for making the resource available
Journal of Neurosurgery - Pediatrics
Date
A point or period of time associated with an event in the lifecycle of the resource
2011
Subject
The topic of the resource
Adolescent; Adult; Age; Factors Brain/pa [Pathology]; Brain/ra [Radiography]; Child; Preschool; Deep Brain Stimulation/ae [Adverse Effects]; Deep Brain Stimulation/mt [Methods]; Dystonia/pp [Physiopathology]; Dystonia/th [Therapy]; Female; Follow-Up Studies; Humans; Magnetic Resonance Imaging; Male; Movement; Retrospective Studies; Tomography; X-Ray Computed Treatment Outcome; tone and motor problems; Glutaric acidemia type I; Lesch-Nyhan syndrome; deep brain stimulation; secondary dystonia
Creator
An entity primarily responsible for making the resource
Air E L; Ostrem J L; Sanger T D; Starr P A
Description
An account of the resource
OBJECT: Deep brain stimulation (DBS) is an established technique for the treatment of several movement disorders in adults. However, the technical approach, complications, and results of DBS in children have not been well documented. METHODS: A database of DBS implantations performed at a single institution, prospectively established in 1998, was reviewed for patients who received DBS prior to the age of 18. Diagnoses, surgical technique, and complications were noted. Outcomes were assessed using standard rating scales of neurological function. RESULTS: Of 815 patients undergoing DBS implantation over a 12-year period, 31 were children (mean age at surgery 13.2 years old, range 4-17 years old). Diagnoses included the following: DYT1 primary dystonia (autosomal dominant, Tor1ADELTAGAG mutation, 10 cases), non-DYT1 primary dystonia (3 cases), secondary dystonia (11 cases), neurodegeneration with brain iron accumulation (NBIA, 3 cases), levodopa-responsive parkinsonism (2 cases), Lesch-Nyhan disease (1 case), and glutaric aciduria Type 1 (1 case). Six children ages 15-17 years old underwent awake microelectrode-guided surgery. For 25 children operated under general anesthesia, the surgical technique evolved from microelectrode-guided surgery to image-guided surgeries using real-time intraoperative MR imaging or CT for lead location confirmation. Complications included 5 hardware infections, all in children younger than 10 years old. At 1 year after implantation, patients with DYT1 dystonia had a mean improvement in the Burke-Fahn-Marsden Dystonia Rating Scale movement subscore of 75%, while those with secondary dystonia had only small improvements. Outcomes in the 3 children with NBIA were disappointing. CONCLUSIONS: Results of DBS in children with primary and secondary dystonias were similar to those in adults, with excellent results for DYT1 dystonia in children without fixed orthopedic deformity and much more modest results in secondary dystonia. In contrast to reported experience in adults with NBIA, these results in children with NBIA were poor. Infection risk was highest in the youngest patients.
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.3171/2011.8.PEDS11153" target="_blank" rel="noreferrer noopener">10.3171/2011.8.PEDS11153</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).
2011
Adolescent
Adult
Age
Air E L
Brain/ra [Radiography]
Child
deep brain stimulation
Deep Brain Stimulation/ae [Adverse Effects]
Deep Brain Stimulation/mt [Methods]
Dystonia/pp [Physiopathology]
Dystonia/th [Therapy]
Factors Brain/pa [Pathology]
Female
Follow-up Studies
Glutaric acidemia type I
Humans
Journal of Neurosurgery - Pediatrics
Lesch-Nyhan syndrome
Magnetic Resonance Imaging
Male
Movement
Ostrem J L
Preschool
Retrospective Studies
Sanger T D
secondary dystonia
Starr P A
Tomography
tone and motor problems
X-Ray Computed Treatment Outcome