1
40
4
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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
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.36573" target="_blank" rel="noreferrer noopener">http://doi.org/10.1002/ajmg.a.36573</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
Autism traits in children and adolescents with Cornelia de Lange 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
2014
Subject
The topic of the resource
Communication; adolescent; Autism; emotion; age; verbal communication; communication disorder; priority journal; interpersonal communication; skill; intellectual impairment; human; article; child; female; male; adult; diagnosis; clinical article; daily life activity; disease severity; autism; Cornelia de Lange syndrome; phenotype; compulsion; language; stereotypy; adaptive behavior; socialization; Aberrant Behavior Checklist; behavior disorder; Behavioral phenotype; checklist; Childhood Autism Rating Scale; de Lange syndrome; hyperactivity; lethargy; limb; maladjustment; psychological rating scale; rating scale; Repetitive behaviors; rigidity; social cognition; Social cognition; Vineland Adaptive Behaviors Scales; behavioral problems; De Lange syndrome; trajectory; characteristics; irritability
Creator
An entity primarily responsible for making the resource
Srivastava S; Landy-Schmitt C; Clark B; Kline A D; Specht M; Grados M A
Description
An account of the resource
Cornelia de Lange syndrome (CdLS) is a cohesinopathy causing delayed growth and limb deficits. Individuals with CdLS have mild to profound intellectual disability and autistic features. This study characterizes the behavioral phenotype of children with CdLS, focusing on autistic features, maladaptive behaviors, and impact of age. Children with CdLS (5-18 years) were administered normed instruments to characterize autism features (Childhood Autism Rating Scale, CARS), maladaptive behaviors (Aberrant Behavior Checklist), and adaptive skills (Vineland Adaptive Behaviors Scales). CdLS features and severity were rated with Diagnostic Criteria for CdLS. Forty-one children with CdLS (23 females, 18 males) were classified as having "no autism" (n=7; 17.1%), "mild autism" (n=17; 41.4%), and "severe autism" (n=17; 41.4%), using CARS scores. Characteristic items were abnormal emotional response, stereotypies, odd object use, rigidity, lack of verbal communication, and low intellectual functioning. Verbal communication deficits and repetitive behaviors were higher compared to sensory, social cognition, and behavior abnormalities (P<0.0001). Maladaptive behaviors associated with autism traits were stereotypies (P=0.003), hyperactivity (P=0.01), and lethargy (P=0.03). Activities of daily living were significantly affected; socialization adaptive skills were a relative strength. However, with advancing age, both socialization (P<0.0001) and communication (P=0.001) domains declined significantly. CdLS is characterized by autistic features, notably excessive repetitive behaviors and expressive language deficits. While other adaptive skills are impacted, socialization adaptive skills are less affected. Advancing age can worsen communication and socialization deficits relative to neurotypical peers. © 2014 Wiley Periodicals, Inc.
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1002/ajmg.a.36573" target="_blank" rel="noreferrer noopener">10.1002/ajmg.a.36573</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).
2014
Aberrant Behavior Checklist
adaptive behavior
Adolescent
Adult
Age
American Journal of Medical Genetics Part A
Article
Autism
behavior disorder
Behavioral phenotype
behavioral problems
characteristics
Checklist
Child
Childhood Autism Rating Scale
Clark B
Clinical Article
Communication
communication disorder
compulsion
Cornelia de Lange syndrome
daily life activity
De Lange syndrome
Diagnosis
Disease Severity
Emotion
Female
Grados M A
Human
hyperactivity
Intellectual Impairment
Interpersonal Communication
Irritability
Kline A D
Landy-Schmitt C
Language
lethargy
limb
maladjustment
Male
Phenotype
Priority Journal
psychological rating scale
Rating Scale
Repetitive behaviors
rigidity
Skill
social cognition
Socialization
Specht M
Srivastava S
stereotypy
Trajectory
Verbal Communication
Vineland Adaptive Behaviors Scales
-
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.brs.2016.07.009" target="_blank" rel="noreferrer noopener">http://doi.org/10.1016/j.brs.2016.07.009</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 Rare Inherited Dystonias
Publisher
An entity responsible for making the resource available
Brain Stimulation
Date
A point or period of time associated with an event in the lifecycle of the resource
2016
Subject
The topic of the resource
adolescent; clinical assessment; disease duration; time to treatment; dystonic disorder/su [Surgery]; priority journal; follow up; school child; outcome assessment; Dystonia; clinical effectiveness; gabapentin/dt [Drug Therapy]; human; article; child; adult; clinical article; aged; surgery; middle aged; disease severity; dystonia; phenotype; rating scale; ataxia telangiectasia/di [Diagnosis]; ataxia telangiectasia/dt [Drug Therapy]; atypical dopa responsive dystonia/di [Diagnosis]; atypical dopa responsive dystonia/dt [Drug Therapy]; baclofen/dt [Drug Therapy]; benzodiazepine derivative/dt [Drug Therapy]; brain depth stimulation; Burke Fahn Marsden Dystonia Rating Scale; cerebellar ataxia/di [Diagnosis]; cerebellar ataxia/dt [Drug Therapy]; chorea/di [Diagnosis]; chorea/dt [Drug Therapy]; clobazam/dt [Drug Therapy]; clonazepam/dt [Drug Therapy]; Deep brain stimulation; diazepam/dt [Drug Therapy]; dystonia/di [Diagnosis]; dystonia/dt [Drug Therapy]; dystonic disorder/th [Therapy]; entacapone/cb [Drug Combination]; entacapone/dt [Drug Therapy]; escitalopram/dt [Drug Therapy]; extrapyramidal syndrome/di [Diagnosis]; extrapyramidal syndrome/dt [Drug Therapy]; haloperidol/dt [Drug Therapy]; Inherited dystonia; levodopa/dt [Drug Therapy]; lorazepam/dt [Drug Therapy]; methylmalonic aciduria/di [Diagnosis]; methylmalonic aciduria/dt [Drug Therapy]; mirtazapine/dt [Drug Therapy]; motor dysfunction assessment; nemaline myopathy/di [Diagnosis]; nemaline myopathy/dt [Drug Therapy]; neuronal ceroid lipofuscinosis/di [Diagnosis]; neuronal ceroid lipofuscinosis/dt [Drug Therapy]; olanzapine/dt [Drug Therapy]; pramipexole/cb [Drug Combination]; pramipexole/dt [Drug Therapy]; preoperative care; risperidone/dt [Drug Therapy]; selegiline/cb [Drug Combination]; selegiline/dt [Drug Therapy]; tetrabenazine/dt [Drug Therapy]; therapy effect; tizanidine/dt [Drug Therapy]; trazodone/dt [Drug Therapy]; Treatment; trihexyphenidyl/cb [Drug Combination]; trihexyphenidyl/dt [Drug Therapy]; trisomy/di [Diagnosis]; trisomy/dt [Drug Therapy]; Wilson disease/di [Diagnosis]; Wilson disease/dt [Drug Therapy]; woodhouse sakati syndrome/di [Diagnosis]; woodhouse sakati syndrome/dt [Drug Therapy]; x trisomy/di [Diagnosis]; x trisomy/dt [Drug Therapy]; tone and motor problems; ataxia telangiectasia; MCM deficiency; NCL; Nemaline myopathy; surgical intervention; Deep Brain Stimulation
Creator
An entity primarily responsible for making the resource
Beaulieu-Boire I; Aquino C C; Fasano A; Poon Y Y; Fallis M; Lang A E; Hodaie M; Kalia S K; Lozano A; Moro E
Description
An account of the resource
Background Rare causes of inherited movement disorders often present with a debilitating phenotype of dystonia, sometimes combined with parkinsonism and other neurological signs. Since these disorders are often resistant to medications, DBS may be considered as a possible treatment. Methods Patients with identified genetic diseases (ataxia-telangiectasia, chorea-achantocytosis, dopa-responsive dystonia, congenital nemaline myopathy, methylmalonic aciduria, neuronal ceroid lipofuscinosis, spinocerebellar ataxia types 2 and 3, Wilson's disease, Woodhouse-Sakati syndrome, methylmalonic aciduria, and X trisomy) and disabling dystonia underwent bilateral GPi DBS (bilateral thalamic Vim nucleus in 1 case). The primary outcome was the difference in the Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS) between baseline, 1 year and last available follow-up. Preoperative factors such as age at surgery, disease duration at surgery, proportion of life lived with dystonia and severity of dystonia were correlated to the primary outcome. Results Eleven patients were operated between February 2003 and December 2013. Age and duration of disease at time of surgery were 30+/-19 and 12.5+/-15.7 years, respectively. DBS effects on dystonia severity were variable but overall marginally effective, with a mean improvement of 7.9% (p=0.39) at 1-year follow-up and 16.7% (p=0.46) at last follow-up (mean 47.3+/-19.9 months after surgery). No preoperative factors were identified to predict the surgical outcome. Conclusion Our findings support the current knowledge that DBS is modestly effective in treating rare inherited dystonias with a combined phenotype. However, the BFMDRS might not be the best tool to measure outcome in these severely affected patients. Copyright © 2016 Elsevier Inc.
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1016/j.brs.2016.07.009" target="_blank" rel="noreferrer noopener">10.1016/j.brs.2016.07.009</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).
2016
Adolescent
Adult
Aged
Aquino C C
Article
ataxia telangiectasia
ataxia telangiectasia/di [Diagnosis]
ataxia telangiectasia/dt [Drug Therapy]
atypical dopa responsive dystonia/di [Diagnosis]
atypical dopa responsive dystonia/dt [Drug Therapy]
Baclofen/dt [drug Therapy]
Beaulieu-Boire I
benzodiazepine derivative/dt [Drug Therapy]
Brain Depth Stimulation
Brain Stimulation
Burke Fahn Marsden Dystonia Rating Scale
cerebellar ataxia/di [Diagnosis]
cerebellar ataxia/dt [Drug Therapy]
Child
chorea/di [Diagnosis]
chorea/dt [Drug Therapy]
Clinical Article
clinical assessment
Clinical Effectiveness
clobazam/dt [Drug Therapy]
clonazepam/dt [Drug Therapy]
deep brain stimulation
diazepam/dt [Drug Therapy]
disease duration
Disease Severity
Dystonia
dystonia/di [Diagnosis]
dystonia/dt [Drug Therapy]
Dystonic Disorder/su [surgery]
dystonic disorder/th [Therapy]
entacapone/cb [Drug Combination]
entacapone/dt [Drug Therapy]
escitalopram/dt [Drug Therapy]
extrapyramidal syndrome/di [Diagnosis]
extrapyramidal syndrome/dt [Drug Therapy]
Fallis M
Fasano A
Follow Up
gabapentin/dt [Drug Therapy]
haloperidol/dt [Drug Therapy]
Hodaie M
Human
Inherited dystonia
Kalia S K
Lang A E
levodopa/dt [Drug Therapy]
lorazepam/dt [Drug Therapy]
Lozano A
MCM deficiency
methylmalonic aciduria/di [Diagnosis]
methylmalonic aciduria/dt [Drug Therapy]
Middle Aged
mirtazapine/dt [Drug Therapy]
Moro E
motor dysfunction assessment
NCL
Nemaline myopathy
nemaline myopathy/di [Diagnosis]
nemaline myopathy/dt [Drug Therapy]
neuronal ceroid lipofuscinosis/di [Diagnosis]
neuronal ceroid lipofuscinosis/dt [Drug Therapy]
olanzapine/dt [Drug Therapy]
outcome assessment
Phenotype
Poon Y Y
pramipexole/cb [Drug Combination]
pramipexole/dt [Drug Therapy]
Preoperative Care
Priority Journal
Rating Scale
risperidone/dt [Drug Therapy]
School Child
selegiline/cb [Drug Combination]
selegiline/dt [Drug Therapy]
Surgery
surgical intervention
tetrabenazine/dt [Drug Therapy]
therapy effect
time to treatment
tizanidine/dt [Drug Therapy]
tone and motor problems
trazodone/dt [Drug Therapy]
Treatment
trihexyphenidyl/cb [Drug Combination]
trihexyphenidyl/dt [Drug Therapy]
Trisomy/di [diagnosis]
trisomy/dt [Drug Therapy]
Wilson disease/di [Diagnosis]
Wilson disease/dt [Drug Therapy]
woodhouse sakati syndrome/di [Diagnosis]
woodhouse sakati syndrome/dt [Drug Therapy]
x trisomy/di [Diagnosis]
x trisomy/dt [Drug Therapy]
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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.
Citation List Month
Backlog
URL Address
<a href="http://doi.org/10.1046/j.1526-4637.2001.01041.x" target="_blank" rel="noreferrer">http://doi.org/10.1046/j.1526-4637.2001.01041.x</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
Designing pain research from the patient's perspective: What trial end points are important to patients with chronic pain?
Publisher
An entity responsible for making the resource available
Pain Medicine
Date
A point or period of time associated with an event in the lifecycle of the resource
2001
Subject
The topic of the resource
Female; Male; Adult; Aged; Perception; Demography; Human; Sleep; Interview; Evaluation; Chronic Pain/dt [Drug Therapy]; Outcomes Research; Article; Clinical Article; Clinical Study; Controlled Study; Disease Severity; Dose Response; Empiricism; Health Center; Medical Decision Making; Medical Information; Medical Research; Methodology; Opiate/do [Drug Dose]; Opiate/dt [Drug Therapy]; Pain Assessment; Pain Clinic; qualitative analysis; Rating Scale; Urban Area
Creator
An entity primarily responsible for making the resource
Casarett D; Karlawish J; Sankar P; Hirschman K; Asch DA
Description
An account of the resource
Objectives. The goals of this study were to define the endpoints of pain research that are important to patients with chronic pain and to identify clinical and demographic variables that are associated with patients' choices of endpoints. Patients & Setting. Interviews were completed with 40 patients seen at the anesthesia pain clinic of an urban tertiary care medical center. Design. Each patient was presented with 4 brief (3-4 sentences) fixed information vignettes describing studies in which new medications would be evaluated. For each, patients were asked to describe how the medication being studied might offer an improvement over their current therapy. Outcome measures. Measures included structured qualitative analysis of responses, the Brief Pain Inventory, and Global Distress Index of the Memorial Symptom Assessment Scale. Results. Patients described a total of 20 endpoints. Individually, patients cited between 2 and 9 end-points each (mean 4.9, standard deviation 1.7). Of these, the most commonly cited were decrease pain, decrease opioid dose, decrease frequency of scheduled dose, increased ability to function, decrease frequency of breakthrough dose, and improve sleep. Patients with severe pain cited more endpoints than did those with mild or moderate pain (mean 5.5 vs. 4.3; Rank sum test p = 0.01). Conclusions. These data suggest that empirical research can provide data to guide the choice of endpoints in clinical studies of pain interventions.
2001
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1046/j.1526-4637.2001.01041.x" target="_blank" rel="noreferrer">10.1046/j.1526-4637.2001.01041.x</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).
Type
The nature or genre of the resource
Journal Article
2001
Adult
Aged
Article
Asch DA
Backlog
Casarett D
Chronic Pain/dt [Drug Therapy]
Clinical Article
Clinical Study
Controlled Study
Demography
Disease Severity
Dose Response
Empiricism
Evaluation
Female
Health Center
Hirschman K
Human
Interview
Journal Article
Karlawish J
Male
Medical Decision Making
Medical Information
Medical Research
Methodology
Opiate/do [Drug Dose]
Opiate/dt [drug Therapy]
Outcomes Research
Pain Assessment
Pain Clinic
Pain Medicine
Perception
Qualitative Analysis
Rating Scale
Sankar P
Sleep
Urban Area
-
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
January 2018 List
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.
Citation List Month
January 2018 List
URL Address
<a href="http://doi.org/10.1097/PCC.0000000000001219" target="_blank" rel="noreferrer">http://doi.org/10.1097/PCC.0000000000001219</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
Moral distress in PICU and neonatal ICU practitioners: A cross-sectional evaluation
Publisher
An entity responsible for making the resource available
Pediatric Critical Care Medicine
Date
A point or period of time associated with an event in the lifecycle of the resource
2017
Subject
The topic of the resource
distress syndrome; health practitioner; morality; neonatal intensive care unit; pediatric intensive care unit; Adult; Article; burnout; correlation analysis; cross-sectional study; Demography; Female; Hospital care; Human; Intensive Care; linear regression analysis; major clinical study; Male; Maslach Burnout Inventory Depersonalization Subscale; Middle Aged; Mishel Parent Perception of Uncertainty Scale; nurse; outcome assessment; physician; priority journal; Prognosis; Questionnaire; Rating Scale; Revised Moral Distress Scale; Terminal Care; tertiary care center; Uncertainty; work; Young Adult
Creator
An entity primarily responsible for making the resource
Larson CP; Dryden-Palmer KD; Gibbons C; Parshuram CS
Description
An account of the resource
Objectives: To measure the level of moral distress in PICU and neonatal ICU health practitioners, and to describe the relationship of moral distress with demographic factors, burnout, and uncertainty. Design: Cross-sectional survey. Setting: A large pediatric tertiary care center. Subjects: Neonatal ICU and PICU health practitioners with at least 3 months of ICU experience. Interventions: A 41-item questionnaire examining moral distress, burnout, and uncertainty. Measurements and Main Results: The main outcome was moral distress measured with the Revised Moral Distress Scale. Secondary outcomes were frequency and intensity Revised Moral Distress Scale subscores, burnout measured with the Maslach Burnout Inventory depersonalization subscale, and uncertainty measured with questions adapted from Mishel's Parent Perception of Uncertainty Scale. Linear regression models were used to examine associations between participant characteristics and the measures of moral distress, burnout, and uncertainty. Two-hundred six analyzable surveys were returned. The median Revised Moral Distress Scale score was 96.5 (interquartile range, 69-133), and 58% of respondents reported significant work-related moral distress. Revised Moral Distress Scale items involving end-of-life care and communication scored highest. Moral distress was positively associated with burnout (r<sup>2</sup> = 0.27; p < 0.001) and uncertainty (r<sup>2</sup> = 0.04; p = 0.008) and inversely associated with perceived hospital supportiveness (r<sup>2</sup> = 0.18; p < 0.001). Nurses reported higher moral distress intensity than physicians (Revised Moral Distress Scale intensity subscores: 57.3 vs 44.7; p = 0.002). In nurses only, moral distress was positively associated with increasing years of ICU experience (p = 0.02) and uncertainty about whether their care was of benefit (r<sup>2</sup> = 0.11; p < 0.001) and inversely associated with uncertainty about a child's prognosis (r<sup>2</sup> = 0.03; p = 0.03). Conclusions: In this single-center, cross-sectional study, we found that moral distress is present in PICU and neonatal ICU health practitioners and is correlated with burnout, uncertainty, and feeling unsupported.
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1097/PCC.0000000000001219" target="_blank" rel="noreferrer">10.1097/PCC.0000000000001219</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).
2017
Adult
Article
Burnout
Correlation Analysis
Cross-sectional Study
Demography
Distress Syndrome
Dryden-Palmer KD
Female
Gibbons C
health practitioner
Hospital care
Human
Intensive Care
January 2018 List
Larson CP
linear regression analysis
Major Clinical Study
Male
Maslach Burnout Inventory Depersonalization Subscale
Middle Aged
Mishel Parent Perception of Uncertainty Scale
Morality
Neonatal Intensive Care Unit
Nurse
outcome assessment
Parshuram CS
Pediatric Critical Care Medicine
Pediatric Intensive Care Unit
Physician
Priority Journal
Prognosis
Questionnaire
Rating Scale
Revised Moral Distress Scale
Terminal Care
tertiary care center
Uncertainty
Work
Young Adult