1
40
72
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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/10.1111/j.1365-2788.2007.01016.x" target="_blank" rel="noreferrer noopener">http://doi.org/10.1111/j.1365-2788.2007.01016.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
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Melatonin treatment in individuals with intellectual disability and chronic insomnia: a randomized placebo-controlled study
Publisher
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Journal of Intellectual Disability Research
Date
A point or period of time associated with an event in the lifecycle of the resource
2008
Subject
The topic of the resource
Saliva; Middle Aged; Treatment Outcome; Humans; Adolescent; Child Preschool; Double-Blind Method; Time Factors; Central Nervous System Depressants/adverse effects/therapeutic use; Melatonin/adverse effects/therapeutic use; Mental Retardation/epidemiology/psychology; Sleep Initiation and Maintenance Disorders/drug therapy/epidemiology/psychology; Q3 Literature Search; chronic disease; child; female; male; adult; comorbidity; aged; sleep disturbance/disorders; chromosome 18q deletion; MPS III; pharmacologic intervention; melatonin
Creator
An entity primarily responsible for making the resource
Braam W; Didden R; Smits M; Curfs L
Description
An account of the resource
BACKGROUND: While several small-number or open-label studies suggest that melatonin improves sleep in individuals with intellectual disabilities (ID) with chronic sleep disturbance, a larger randomized control trial is necessary to validate these promising results. METHODS: The effectiveness of melatonin for the treatment of chronic sleep disturbance was assessed in a randomized double-blind placebo-controlled trial with 51 individuals with ID. All of these individuals presented with chronic ideopatic sleep disturbance for more than 1 year. The study consisted of a 1-week baseline, followed by 4 weeks of treatment. Parents or other caregivers recorded lights off time, sleep onset time, night waking, wake up time and epileptic seizures. Endogenous melatonin cycle was measured in saliva before and after treatment. RESULTS: Compared with placebo, melatonin significantly advanced mean sleep onset time by 34 min, decreased mean sleep latency by 29 min, increased mean total sleep time by 48 min, reduced the mean number of times the person awoke during the night by 0.4, decreased the mean duration of these night waking periods by 17 min and advanced endogenous melatonin onset at night by an average of 2.01 h. Lights off time, sleep offset time and the number of nights per week with night waking did not change. Only few minor or temporary adverse reactions and no changes in seizure frequency were reported. CONCLUSIONS: Melatonin treatment improves some aspects of chronic sleep disturbance in individuals with ID.
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1111/j.1365-2788.2007.01016.x" target="_blank" rel="noreferrer noopener">10.1111/j.1365-2788.2007.01016.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).
2008
Adolescent
Adult
Aged
Braam W
Central Nervous System Depressants/adverse effects/therapeutic use
Child
Child Preschool
chromosome 18q deletion
Chronic Disease
Comorbidity
Curfs L
Didden R
Double-Blind Method
Female
Humans
Journal Of Intellectual Disability Research
Male
melatonin
Melatonin/adverse effects/therapeutic use
Mental Retardation/epidemiology/psychology
Middle Aged
MPS III
pharmacologic intervention
Q3 Scoping Review Results
Saliva
sleep disturbance/disorders
Sleep Initiation and Maintenance Disorders/drug therapy/epidemiology/psychology
Smits M
Time Factors
Treatment Outcome
-
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
April 2021 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
April 2021 List
URL Address
<a href="http://doi.org/10.1177/0269216319885566" target="_blank" rel="noreferrer noopener">http://doi.org/10.1177/0269216319885566</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
Insights into the perception that research ethics committees are a barrier to research with seriously ill children: A study of committee minutes and correspondence with researchers studying seriously ill children
Publisher
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Palliative Medicine
Date
A point or period of time associated with an event in the lifecycle of the resource
2020
Subject
The topic of the resource
Infant; Adolescent; Child; Female; Humans; Male; Child Preschool; Infant Newborn; Research Design; Terminally Ill; Ethics Committees Research; Human Experimentation/ethics; content analysis; United Kingdom; research; ethics committees
Creator
An entity primarily responsible for making the resource
Butler AE; Vincent K; Bluebond-Langner M
Description
An account of the resource
BACKGROUND: Research ethics committees are commonly perceived as a 'barrier' to research involving seriously ill children. Researchers studying seriously ill children often feel that committees view their applications more harshly compared to applications for research with other populations. Whether or not this is the case in practice is unknown. AIM: The aim of this study was to explore committees' concerns, expectations and decisions for research applications involving seriously ill children submitted for review in the United Kingdom. DESIGN: Content analysis of committee meeting minutes, decision letters and researcher response letters. SETTING/PARTICIPANTS: Chief investigators for National Institute of Health Research portfolio studies involving seriously ill children were contacted for permission to review their study documents. RESULTS: Of the 77 applications included in this study, 57 received requests for revisions at first review. Committee expectations and concerns commonly related to participant information sheets, methodology, consent, recruitment or formatting. Changes were made to 53 of these studies, all of which were subsequently approved. CONCLUSION: Our findings suggest that committees review applications for research involving seriously ill children with the same scrutiny as applications for research with other populations. Yet, the perception that committees act as a barrier to this type of research persists. We suggest that this perception remains due to other factors including, but not limited to, the high levels of formatting or administrative revisions requested by committees or additional study requirements needed for research involving children, such as multiple versions of consent forms or participant information sheets.
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1177/0269216319885566" target="_blank" rel="noreferrer noopener">10.1177/0269216319885566</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).
2020
Adolescent
April 2021 List
Bluebond-Langner M
Butler AE
Child
Child Preschool
Content Analysis
Ethics Committees
Ethics Committees Research
Female
Human Experimentation/ethics
Humans
Infant
Infant Newborn
Male
Palliative Medicine
Research
Research Design
Terminally Ill
United Kingdom
Vincent K
-
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
March 2021 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
March 2021 List
URL Address
<a href="http://doi.org/10.1001/jamanetworkopen.2020.18109" target="_blank" rel="noreferrer noopener">http://doi.org/10.1001/jamanetworkopen.2020.18109</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
Genome Sequencing as a Diagnostic Test in Children With Unexplained Medical Complexity
Publisher
An entity responsible for making the resource available
JAMA Network Open
Date
A point or period of time associated with an event in the lifecycle of the resource
2020
Subject
The topic of the resource
Child; Female; Humans; Male; Reproducibility of Results; Child Preschool; Canada; Prospective Studies; Predictive Value of Tests; Genetic Testing/statistics & numerical data; Somatoform Disorders/diagnosis; Whole Genome Sequencing/statistics & numerical data
Creator
An entity primarily responsible for making the resource
Costain G; Walker S; Marano M; Veenma D; Snell M; Curtis M; Luca S; Buera J; Arje D; Reuter MS; Thiruvahindrapuram B; Trost B; Sung WWL; Yuen RKC; Chitayat D; Mendoza-Londono R; Stavropoulos DJ; Scherer SW; Marshall CR; Cohn RD; Cohen E; Orkin J; Meyn MS; Hayeems RZ
Description
An account of the resource
IMPORTANCE: Children with medical complexity (CMC) represent a growing population in the pediatric health care system, with high resource use and associated health care costs. A genetic diagnosis can inform prognosis, anticipatory care, management, and reproductive planning. Conventional genetic testing strategies for CMC are often costly, time consuming, and ultimately unsuccessful. OBJECTIVE: To evaluate the analytical and clinical validity of genome sequencing as a comprehensive diagnostic genetic test for CMC. DESIGN, SETTING, AND PARTICIPANTS: In this cohort study of the prospective use of genome sequencing and comparison with standard-of-care genetic testing, CMC were recruited from May 1, 2017, to November 30, 2018, from a structured complex care program based at a tertiary care pediatric hospital in Toronto, Canada. Recruited CMC had at least 1 chronic condition, technology dependence (child is dependent at least part of each day on mechanical ventilators, and/or child requires prolonged intravenous administration of nutritional substances or drugs, and/or child is expected to have prolonged dependence on other device-based support), multiple subspecialist involvement, and substantial health care use. Review of the care plans for 545 CMC identified 143 suspected of having an undiagnosed genetic condition. Fifty-four families met inclusion criteria and were interested in participating, and 49 completed the study. Probands, similarly affected siblings, and biological parents were eligible for genome sequencing. EXPOSURES: Genome sequencing was performed using blood-derived DNA from probands and family members using established methods and a bioinformatics pipeline for clinical genome annotation. MAIN OUTCOMES AND MEASURES: The primary study outcome was the diagnostic yield of genome sequencing (proportion of CMC for whom the test result yielded a new diagnosis). RESULTS: Genome sequencing was performed for 138 individuals from 49 families of CMC (29 male and 20 female probands; mean [SD] age, 7.0 [4.5] years). Genome sequencing detected all genomic variation previously identified by conventional genetic testing. A total of 15 probands (30.6%; 95% CI 19.5%-44.6%) received a new primary molecular genetic diagnosis after genome sequencing. Three individuals had novel diseases and an additional 9 had either ultrarare genetic conditions or rare genetic conditions with atypical features. At least 11 families received diagnostic information that had clinical management implications beyond genetic and reproductive counseling. CONCLUSIONS AND RELEVANCE: This study suggests that genome sequencing has high analytical and clinical validity and can result in new diagnoses in CMC even in the setting of extensive prior investigations. This clinical population may be enriched for ultrarare and novel genetic disorders. Genome sequencing is a potentially first-tier genetic test for CMC.
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1001/jamanetworkopen.2020.18109" target="_blank" rel="noreferrer noopener">10.1001/jamanetworkopen.2020.18109</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).
2020
Arje D
Buera J
Canada
Child
Child Preschool
Chitayat D
Cohen E
Cohn RD
Costain G
Curtis M
Female
Genetic Testing/statistics & numerical data
Hayeems RZ
Humans
JAMA Network Open
Luca S
Male
Marano M
March 2021 List
Marshall CR
Mendoza-Londono R
Meyn MS
Orkin J
Predictive Value of Tests
Prospective Studies
Reproducibility of Results
Reuter MS
Scherer SW
Snell M
Somatoform Disorders/diagnosis
Stavropoulos DJ
Sung WWL
Thiruvahindrapuram B
Trost B
Veenma D
Walker S
Whole Genome Sequencing/statistics & numerical data
Yuen RKC
-
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/10.1111/j.1469-8749.2010.03636.x" target="_blank" rel="noreferrer noopener">http://doi.org/10.1111/j.1469-8749.2010.03636.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
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Level of purposeful hand function as a marker of clinical severity in Rett 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
2010
Subject
The topic of the resource
Cross-Sectional Studies; Age Factors; Severity of Illness Index; Young Adult; Child; Humans; Adult; Adolescent; Female; Child Preschool; Phenotype; Video Recording; Mutation; Registries; Genotype; Motor Activity; Databases as Topic; Walking; Rett Syndrome; Methyl-CpG-Binding Protein 2; Hand; tone and motor problems; trajectory; characteristics
Creator
An entity primarily responsible for making the resource
Downs J; Bebbington A; Jacoby P; Williams A; Ghosh S; Kaufmann W E; Leonard H
Description
An account of the resource
AIM: We investigated relationships between hand function and genotype and aspects of phenotype in Rett syndrome. METHOD: Video assessment in naturalistic settings was supplemented by parent-reported data in a cross-sectional study of 144 females with a mean age of 14 years 10 months (SD 7 y 10 mo; range 2 y-31 y 10 mo), 110 of whom had a mutation of the methyl CpG binding protein 2 (MECP2) gene. Ordinal logistic regression was used to assess relationships between hand function and MECP2 mutation, age, a modified Kerr score, Functional Independence Measure for Children (WeeFIM), ambulation level, and frequency of hand stereotypies. RESULTS: Approximately two-thirds of participants demonstrated purposeful hand function, ranging from simple grasping skills to picking up and manipulating small objects. In participants with a confirmed MECP2 mutation, those with the p.R168X mutation had the poorest hand function on multivariate analysis with C-terminal deletion as the baseline (odds ratio [OR] 0.19; 95% confidence interval [CI] 0.04-0.95), whereas those with the p.R133C or p.R294X mutation had better hand function. Participants aged 19 years or older had lower hand function than those aged less than 8 years (OR 0.36; 95% CI 0.14-0.92). Factors that were associated with better hand function were lower Kerr scores for a 1-point increase in score (OR 0.77; 95% CI 0.69-0.86), higher WeeFIM scores for a 1-point increase in score (OR 1.08; 95% CI 1.04-1.12), and greater ambulation than those completely dependent on carers for mobility (OR 22.64; 95% CI 7.02-73.08). The results for participants with a confirmed pathogenic mutation were similar to results obtained when participants without a mutation were also included. INTERPRETATION: Our novel assessment of hand function in Rett syndrome correlated well with known profiles of common MECP2 mutations and overall clinical severity. This promising assessment could measure clinical responses to therapy.
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1111/j.1469-8749.2010.03636.x" target="_blank" rel="noreferrer noopener">10.1111/j.1469-8749.2010.03636.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).
2010
Adolescent
Adult
Age Factors
Bebbington A
characteristics
Child
Child Preschool
Cross-sectional Studies
Databases as Topic
Developmental Medicine and Child Neurology
Downs J
Female
Genotype
Ghosh S
Hand
Humans
Jacoby P
Kaufmann W E
Leonard H
Methyl-CpG-Binding Protein 2
Motor Activity
Mutation
Phenotype
Registries
Rett syndrome
Severity Of Illness Index
tone and motor problems
Trajectory
Video Recording
Walking
Williams A
Young Adult
-
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
March 2021 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
March 2021 List
URL Address
<a href="http://doi.org/10.1001/jamanetworkopen.2020.29082" target="_blank" rel="noreferrer noopener">http://doi.org/10.1001/jamanetworkopen.2020.29082</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
Parent-Reported Symptoms and Medications Used Among Children With Severe Neurological Impairment
Publisher
An entity responsible for making the resource available
JAMA Network Open
Date
A point or period of time associated with an event in the lifecycle of the resource
2020
Subject
The topic of the resource
Infant; Child; Comorbidity; Female; Male; Child Preschool; Adolescence; Data Analysis Software; Human; Confidence Intervals; Descriptive Statistics; Record Review; Health Resource Utilization; Cross Sectional Studies; Funding Source; Scales; Severity of Illness; Central Nervous System Agents -- Therapeutic Use; Nervous System Diseases -- Epidemiology -- In Adolescence; Nervous System Diseases -- Epidemiology -- In Infancy and Childhood; Nervous System Diseases -- Symptoms; Peripheral Nervous System Agents -- Therapeutic Use; Polypharmacy
Creator
An entity primarily responsible for making the resource
Feinstein JA; Feudtner C; Blackmer AB; Valuck RJ; Fairclough DL; Holstein J; Gregoire LA; Samay S; Kempe A
Description
An account of the resource
Key Points: Question: In children with severe neurological impairment (SNI) who cannot self-report, can comprehensive parent-reported symptom assessments inform medication use? Findings: In this cross-sectional study of 100 children with SNI and polypharmacy, parents reported that children experienced multiple concurrent high-distress symptoms, notably irritability (65.0%), insomnia (55.0%), and pain (54.0%). Although higher symptom burdens were associated with increasing polypharmacy, opportunities existed to optimize pharmacotherapy; for example, among 54.0% of children with pain, only 61.0% were prescribed an analgesic. Meaning: Comprehensive parent-reported symptom data paired with medication data could help clinicians identify targets for personalized symptom management, including underrecognized or undertreated symptoms. This cross-sectional study examines whether higher global symptom scores are associated with use of more medications and assesses associations between specific symptoms and medications among children with severe neurological impairment. Importance: Children with severe neurological impairment (SNI) often take multiple medications to treat problematic symptoms. However, for children who cannot self-report symptoms, no system exists to assess multiple symptoms and their association with medication use. Objectives: To assess the prevalence of 28 distinct symptoms, test whether higher global symptom scores (GSS) were associated with use of more medications, and assess the associations between specific symptoms and medications. Design, Setting, and Participants: This cross-sectional study was conducted between April 1, 2019, and December 31, 2019, using structured parent-reported symptom data paired with clinical and pharmacy data, at a single-center, large, hospital-based special health care needs clinic. Participants included children aged 1 to 18 years with SNI and 5 or more prescribed medications. Data analysis was performed from April to June 2020. Exposure: During routine clinical visits, parent-reported symptoms were collected using the validated 28-symptom Memorial Symptom Assessment Scale (MSAS) and merged with clinical and pharmacy data. Main Outcomes and Measures: Symptom prevalence, counts, and GSS (scored 0-100, with 100 being the worst) were calculated, and the association of GSS with medications was examined. To evaluate associations between symptom-medication pairs, the proportion of patients with a symptom who used a medication class or specific medication was calculated. Results: Of 100 patients, 55.0% were boys, the median (interquartile range [IQR]) age was 9 (5-12) years, 62.0% had 3 or more complex chronic conditions, 76.0% took 10 or more medications, and none were able to complete the MSAS themselves. Parents reported a median (IQR) of 7 (4-10) concurrent active symptoms. The median (IQR) GSS was 12.1 (5.4-20.8) (range, 0.0-41.2) and the GSS was 9.8 points (95% CI, 5.5-14.1 points) higher for those with worse recent health than usual. Irritability (65.0%), insomnia (55.0%), and pain (54.0%) were the most prevalent symptoms. Each 10-point GSS increase was associated with 12% (95% CI, 4%-19%) higher medication counts, adjusted for age and complex chronic condition count. Among the 54.0% of children with reported pain, 61.0% were prescribed an analgesic. Conclusions and Relevance: These findings suggest that children with SNI reportedly experience substantial symptom burdens and that higher symptom scores are associated with increased medication use. Paired symptom-medication data may help clinicians identify targets for personalized symptom management, including underrecognized or undertreated symptoms.
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1001/jamanetworkopen.2020.29082" target="_blank" rel="noreferrer noopener">10.1001/jamanetworkopen.2020.29082</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).
2020
Adolescence
Blackmer AB
Central Nervous System Agents -- Therapeutic Use
Child
Child Preschool
Comorbidity
Confidence Intervals
Cross Sectional Studies
Data Analysis Software
Descriptive Statistics
Fairclough DL
Feinstein JA
Female
Feudtner C
Funding Source
Gregoire LA
Health Resource Utilization
Holstein J
Human
Infant
JAMA Network Open
Kempe A
Male
March 2021 List
Nervous System Diseases -- Epidemiology -- In Adolescence
Nervous System Diseases -- Epidemiology -- In Infancy and Childhood
Nervous System Diseases -- Symptoms
Peripheral Nervous System Agents -- Therapeutic Use
Polypharmacy
Record Review
Samay S
scales
Severity of Illness
Valuck RJ
-
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/10.1136/bmj.e6664" target="_blank" rel="noreferrer noopener">http://doi.org/10.1136/bmj.e6664</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
Melatonin for sleep problems in children with neurodevelopmental disorders: randomised double masked placebo controlled trial
Publisher
An entity responsible for making the resource available
British Medical Journal
Date
A point or period of time associated with an event in the lifecycle of the resource
2012
Subject
The topic of the resource
Male; Treatment Outcome; Severity of Illness Index; Dose-Response Relationship Drug; Child; Humans; Adolescent; Family Health; Female; Child Preschool; Drug Monitoring; Child Behavior/drug effects; Melatonin/administration & dosage/adverse effects; Sleep Wake Disorders/diagnosis/drug therapy/etiology; Central Nervous System Depressants/administration & dosage/adverse effects; Central Nervous System Diseases/complications; Developmental Disabilities/complications; Polysomnography/methods; Sleep/drug effects; sleep disturbance/disorders; unspecified Q3 conditions; Q3 conditions; pharmacologic intervention; melatonin
Creator
An entity primarily responsible for making the resource
Gringras P; Gamble C; Jones A P; Wiggs L; Williamson P R; Sutcliffe A; Montgomery P; Whitehouse W P; Choonara I; Allport T; Edmond A; Appleton R
Description
An account of the resource
OBJECTIVE: To assess the effectiveness and safety of melatonin in treating severe sleep problems in children with neurodevelopmental disorders. DESIGN: 12 week double masked randomised placebo controlled phase III trial. SETTING: 19 hospitals across England and Wales. PARTICIPANTS: 146 children aged 3 years to 15 years 8 months were randomised. They had a range of neurological and developmental disorders and a severe sleep problem that had not responded to a standardised sleep behaviour advice booklet provided to parents four to six weeks before randomisation. A sleep problem was defined as the child not falling asleep within one hour of lights out or having less than six hours' continuous sleep. INTERVENTIONS: Immediate release melatonin or matching placebo capsules administered 45 minutes before the child's bedtime for a period of 12 weeks. All children started with a 0.5 mg capsule, which was increased through 2 mg, 6 mg, and 12 mg depending on their response to treatment. MAIN OUTCOME MEASURES: Total sleep time at night after 12 weeks adjusted for baseline recorded in sleep diaries completed by the parent. Secondary outcomes included sleep onset latency, assessments of child behaviour, family functioning, and adverse events. Sleep was measured with diaries and actigraphy. RESULTS: Melatonin increased total sleep time by 22.4 minutes (95% confidence interval 0.5 to 44.3 minutes) measured by sleep diaries (n=110) and 13.3 (-15.5 to 42.2) measured by actigraphy (n=59). Melatonin reduced sleep onset latency measured by sleep diaries (-37.5 minutes, -55.3 to -19.7 minutes) and actigraphy (-45.3 minutes, -68.8 to -21.9 minutes) and was most effective for children with the longest sleep latency (P=0.009). Melatonin was associated with earlier waking times than placebo (29.9 minutes, 13.6 to 46.3 minutes). Child behaviour and family functioning outcomes showed some improvement and favoured use of melatonin. Adverse events were mild and similar between the two groups. CONCLUSIONS: Children gained little additional sleep on melatonin; though they fell asleep significantly faster, waking times became earlier. Child behaviour and family functioning outcomes did not significantly improve. Melatonin was tolerable over this three month period. Comparisons with slow release melatonin preparations or melatonin analogues are required. TRIAL REGISTRATION: ISRCT No 05534585.
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1136/bmj.e6664" target="_blank" rel="noreferrer noopener">10.1136/bmj.e6664</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).
2012
Adolescent
Allport T
Appleton R
British Medical Journal
Central Nervous System Depressants/administration & dosage/adverse effects
Central Nervous System Diseases/complications
Child
Child Preschool
Child Behavior/drug effects
Choonara I
Developmental Disabilities/complications
Dose-Response Relationship Drug
Drug Monitoring
Edmond A
Family Health
Female
Gamble C
Gringras P
Humans
Jones A P
Male
melatonin
Melatonin/administration & dosage/adverse effects
Montgomery P
pharmacologic intervention
Polysomnography/methods
Q3 conditions
Severity Of Illness Index
sleep disturbance/disorders
Sleep Wake Disorders/diagnosis/drug therapy/etiology
Sleep/drug effects
Sutcliffe A
Treatment Outcome
unspecified Q3 conditions
Whitehouse W P
Wiggs L
Williamson P R
-
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
A name given to the resource
Self-injurious behaviour in young children with Lesch-Nyhan syndrome
Publisher
An entity responsible for making the resource available
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/10.1016/0887-8994(93)90082-n" target="_blank" rel="noreferrer noopener">http://doi.org/10.1016/0887-8994(93)90082-n</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
Prospective study of spinal muscular atrophy before age 6 years. DCN/SMA Group
Publisher
An entity responsible for making the resource available
Pediatric Neurology
Date
A point or period of time associated with an event in the lifecycle of the resource
1993
Subject
The topic of the resource
Male; Child; Humans; Prospective Studies; Female; Child Preschool; Infant; Neurologic Examination; Motor Skills/physiology; Muscle Contraction/physiology; Range of Motion Articular/physiology; Reflex Stretch/physiology; Spinal Muscular Atrophies of Childhood/diagnosis/physiopathology; Sucking Behavior/physiology; tone and motor problems; SMA1; trajectory; characteristics; tongue fasciculation; facial weakness
Creator
An entity primarily responsible for making the resource
Iannaccone S T; Browne R H; Samaha F J; Buncher C R
Description
An account of the resource
Spinal muscular atrophy (SMA) is a common neuromuscular disorder of childhood, associated with a high mortality rate during the first 2 years of life. Most practitioners expect patients with SMA to follow a progressive course with loss of muscle strength and function over 2-10 years. Counselling sessions with parents frequently emphasize the high mortality rate and risk for respiratory failure. The progressive nature of SMA has been attributed to the loss of motor neurons. Fifty-eight children, ages 6 years and younger, were examined between January, 1987, and April, 1992, as part of a large, multicenter collaborative study of SMA. Muscle function was evaluated at regular intervals using a standardized protocol that was demonstrated to be reliable. We determined a prevalence of 56% for tongue fasciculations, a prevalence of 22% for facial weakness, and persistent deep tendon reflexes in one patient. Improved motor function and acquired milestones during the study were documented. This work should contribute toward a better understanding of the natural history of SMA.
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1016/0887-8994(93)90082-n" target="_blank" rel="noreferrer noopener">10.1016/0887-8994(93)90082-n</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).
1993
Browne R H
Buncher C R
characteristics
Child
Child Preschool
facial weakness
Female
Humans
Iannaccone S T
Infant
Male
Motor Skills/physiology
Muscle Contraction/physiology
Neurologic Examination
Pediatric Neurology
Prospective Studies
Range of Motion Articular/physiology
Reflex Stretch/physiology
Samaha F J
SMA1
Spinal Muscular Atrophies of Childhood/diagnosis/physiopathology
Sucking Behavior/physiology
tone and motor problems
tongue fasciculation
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/10.1212/WNL.0b013e318237f649" target="_blank" rel="noreferrer noopener">http://doi.org/10.1212/WNL.0b013e318237f649</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
Quantifying physical decline in juvenile neuronal ceroid lipofuscinosis (Batten disease)
Publisher
An entity responsible for making the resource available
Neurology
Date
A point or period of time associated with an event in the lifecycle of the resource
2011
Subject
The topic of the resource
Cross-Sectional Studies; Disease Progression; Young Adult; Child; Humans; Adult; Prospective Studies; Adolescent; Child Preschool; Regression Analysis; Reproducibility of Results; Analysis of Variance; Neuropsychological Tests; Mutation; Disabled Persons; Genotype; Homozygote; Membrane Glycoproteins; Molecular Chaperones; Neuronal Ceroid-Lipofuscinoses; tone and motor problems; NCL3; tool development; scale development; UBDRS
Creator
An entity primarily responsible for making the resource
Kwon J M; Adams H; Rothberg P G; Augustine E F; Marshall F J; Deblieck E A; Vierhile A; Beck C A; Newhouse N J; Cialone J; Levy E; Ramirez-Montealegre D; Dure L S; Rose K R; Mink J W
Description
An account of the resource
OBJECTIVE: To use the Unified Batten Disease Rating Scale (UBDRS) to measure the rate of decline in physical and functional capability domains in patients with juvenile neuronal ceroid lipofuscinosis (JNCL) or Batten disease, a neurodegenerative lysosomal storage disorder. We have evaluated the UBDRS in subjects with JNCL since 2002; during that time, the scale has been refined to improve reliability and validity. Now that therapies are being proposed to prevent, slow, or reverse the course of JNCL, the UBDRS will play an important role in quantitatively assessing clinical outcomes in research trials. METHODS: We administered the UBDRS to 82 subjects with JNCL genetically confirmed by CLN3 mutational analysis. Forty-four subjects were seen for more than one annual visit. From these data, the rate of physical impairment over time was quantified using multivariate linear regression and repeated-measures analysis. RESULTS: The UBDRS Physical Impairment subscale shows worsening over time that proceeds at a quantifiable linear rate in the years following initial onset of clinical symptoms. This deterioration correlates with functional capability and is not influenced by CLN3 genotype. CONCLUSION: The UBDRS is a reliable and valid instrument that measures clinical progression in JNCL. Our data support the use of the UBDRS to quantify the rate of progression of physical impairment in subjects with JNCL in clinical trials.
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1212/WNL.0b013e318237f649" target="_blank" rel="noreferrer noopener">10.1212/WNL.0b013e318237f649</a>
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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
Adams H
Adolescent
Adult
Analysis of Variance
Augustine E F
Beck C A
Child
Child Preschool
Cialone J
Cross-sectional Studies
Deblieck E A
Disabled Persons
Disease Progression
Dure L S
Genotype
Homozygote
Humans
Kwon J M
Levy E
Marshall F J
Membrane Glycoproteins
Mink J W
Molecular Chaperones
Mutation
NCL3
Neurology
Neuronal Ceroid-Lipofuscinoses
Neuropsychological Tests
Newhouse N J
Prospective Studies
Ramirez-Montealegre D
Regression Analysis
Reproducibility of Results
Rose K R
Rothberg P G
scale development
tone and motor problems
tool development
UBDRS
Vierhile A
Young Adult
-
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
March 2021 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
March 2021 List
URL Address
<a href="http://doi.org/10.1093/ajcn/nqaa142" target="_blank" rel="noreferrer noopener">http://doi.org/10.1093/ajcn/nqaa142</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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Use of standardized body composition measurements and malnutrition screening tools to detect malnutrition risk and predict clinical outcomes in children with chronic conditions
Publisher
An entity responsible for making the resource available
American Journal of Clinical Nutrition
Date
A point or period of time associated with an event in the lifecycle of the resource
2020
Subject
The topic of the resource
Adolescent; Child; Female; Humans; Male; Risk Factors; Child Preschool; Anthropometry; Chronic Disease; screening; Child Development; pediatric patients; body composition; Body Composition; clinical outcomes; malnutrition; nutritional risk; Child Nutrition Disorders/diagnosis
Creator
An entity primarily responsible for making the resource
Lara-Pompa NE; Hill S; Williams J; Macdonald S; Fawbert K; Valente J; Kennedy K; Shaw V; Wells JC; Fewtrell M
Description
An account of the resource
BACKGROUND: Better tools are needed to diagnose and identify children at risk of clinical malnutrition. OBJECTIVES: We aimed to compare body composition (BC) and malnutrition screening tools (MSTs) for detecting malnutrition on admission; and examine their ability to predict adverse clinical outcomes [increased length of stay (LOS) and complications] in complex pediatric patients. METHODS: This was a prospective study in children 5-18 y old admitted to a tertiary pediatric hospital (n = 152). MSTs [Pediatric Yorkhill Malnutrition Score (PYMS), Screening Tool for the Assessment of Malnutrition in Pediatrics (STAMP), and Screening Tool for Risk of Impaired Nutritional Status and Growth (STRONGkids)] were completed on admission. Weight, height, and BC [fat mass (FM) and lean mass (LM) by DXA] were measured (n = 118). Anthropometry/BC and MSTs were compared with each other and with clinical outcomes. RESULTS: Subjects were significantly shorter with low LM compared to reference data. Depending on the diagnostic criteria used, 3%-17% were classified as malnourished. Agreement between BC/anthropometric parameters and MSTs was poor. STAMP and STRONGkids identified children with low weight, LM, and height. PYMS, and to a lesser degree STRONGkids, identified children with increased LOS, as did LM compared with weight or height. Patients with complications had lower mean ± SD LM SD scores (-1.38 ± 1.03 compared with -0.74 ± 1.40, P < 0.05). In multivariable models, PYMS high risk and low LM were independent predictors of increased LOS (OR: 3.76; 95% CI: 1.36, 10.35 and OR: 3.69; 95% CI: 1.24, 10.98, respectively). BMI did not predict increased LOS or complications. CONCLUSIONS: LM appears better than weight and height for predicting adverse clinical outcomes in this population. BMI was a poor diagnostic parameter. MSTs performed differently in associations to BC/anthropometry and clinical outcomes. PYMS and LM provided complementary information regarding LOS. Studies on specific patient populations may further clarify the use of these tools and measurements.
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1093/ajcn/nqaa142" target="_blank" rel="noreferrer noopener">10.1093/ajcn/nqaa142</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).
2020
Adolescent
American Journal of Clinical Nutrition
Anthropometry
Body Composition
Child
Child Preschool
Child Development
Child Nutrition Disorders/diagnosis
Chronic Disease
Clinical outcomes
Fawbert K
Female
Fewtrell M
Hill S
Humans
Kennedy K
Lara-Pompa NE
Macdonald S
Male
malnutrition
March 2021 List
nutritional risk
pediatric patients
Risk Factors
Screening
Shaw V
Valente J
Wells JC
Williams 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/10.1007/s10803-008-0655-6" target="_blank" rel="noreferrer noopener">http://doi.org/10.1007/s10803-008-0655-6</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
The prevalence and phenomenology of repetitive behavior in genetic syndromes
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
2009
Subject
The topic of the resource
Middle Aged; Male; Young Adult; Surveys and Questionnaires; Child; Humans; Adult; Adolescent; Prevalence; Female; Child Preschool; Phenotype; Syndrome; London; Stereotyped Behavior; Angelman Syndrome/epidemiology/psychology; Cri-du-Chat Syndrome/epidemiology/psychology; De Lange Syndrome/epidemiology/psychology; Fragile X Syndrome/epidemiology/psychology; Intellectual Disability/epidemiology/genetics/psychology; Prader-Willi Syndrome/epidemiology/psychology; behavioral problems; tone and motor problems; cri-du-chat; De Lange syndrome; trajectory; characteristics; repetitive behavior
Creator
An entity primarily responsible for making the resource
Moss J; Oliver C; Arron K; Burbidge C; Berg K
Description
An account of the resource
We investigated the prevalence and phenomenology of repetitive behavior in genetic syndromes to detail profiles of behavior. The Repetitive Behaviour Questionnaire (RBQ) provides fine-grained identification of repetitive behaviors. The RBQ was employed to examine repetitive behavior in Angelman (N = 104), Cornelia de Lange (N = 101), Cri-du-Chat (N = 58), Fragile X (N = 191), Prader-Willi (N = 189), Lowe (N = 56) and Smith-Magenis (N = 42) syndromes and individuals with intellectual disability of heterogeneous aetiology (N = 56). Repetitive behavior was variable across syndromes. Fragile X syndrome scored highly on all subscales. Angelman syndrome demonstrated a significantly lowered probability for most behaviors. Prader-Willi, Cri-du-Chat and Smith-Magenis syndrome evidenced unique profiles of repetitive behavior. There is extreme heterogeneity of repetitive behavior across genetic syndromes, highlighting syndrome specific profiles.
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1007/s10803-008-0655-6" target="_blank" rel="noreferrer noopener">10.1007/s10803-008-0655-6</a>
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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).
2009
Adolescent
Adult
Angelman Syndrome/epidemiology/psychology
Arron K
behavioral problems
Berg K
Burbidge C
characteristics
Child
Child Preschool
Cri-du-chat
Cri-du-Chat Syndrome/epidemiology/psychology
De Lange syndrome
De Lange Syndrome/epidemiology/psychology
Female
Fragile X Syndrome/epidemiology/psychology
Humans
Intellectual Disability/epidemiology/genetics/psychology
Journal of Autism and Developmental Disorders
London
Male
Middle Aged
Moss J
Oliver C
Phenotype
Prader-Willi Syndrome/epidemiology/psychology
Prevalence
repetitive behavior
Stereotyped Behavior
Surveys And Questionnaires
Syndrome
tone and motor problems
Trajectory
Young Adult
-
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
March 2021 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
March 2021 List
URL Address
<a href="http://doi.org/10.1002/ppul.24923" target="_blank" rel="noreferrer noopener">http://doi.org/10.1002/ppul.24923</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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Healthcare utilization and costs of pediatric home mechanical ventilation in Canada
Publisher
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Pediatric Pulmonology
Date
A point or period of time associated with an event in the lifecycle of the resource
2020
Subject
The topic of the resource
Adult; Child; Female; Humans; Male; Child Preschool; Middle Aged; Quality of Life; Longitudinal Studies; Canada; Prospective Studies; pediatrics; Health Care Costs; Health Expenditures; Patient Acceptance of Health Care; Tracheostomy; healthcare costs; healthcare utilization; home mechanical ventilation; long-term mechanical ventilation; ventilator assisted individual; Ambulatory Care/economics; Caregivers/economics; Home Care Services/economics; Respiration Artificial/economics
Creator
An entity primarily responsible for making the resource
Nonoyama ML; Katz SL; Amin R; McKim DA; Guerriere D; Coyte PC; Wasilewski M; Zagorski B; Rose L
Description
An account of the resource
BACKGROUND: Children using home mechanical ventilation (HMV) live at home with better quality of life, despite financial burden for their family. Previous studies of healthcare utilization and costs have not considered public and private expenditures, including family caregiver time. Our objective was to examine public and private healthcare utilization and costs for children using HMV, and variables associated with highest costs. METHODS: Longitudinal, prospective, observational cost analysis study (2012-2014) collecting data on public and private (out-of-pocket, third-party insurance, and caregiving) costs every 2 weeks for 6 months using the Ambulatory Home Care Record. Functional Independence Measure (FIM), WeeFIM, and Caregiving Impact Scale (CIS) were measured at baseline and study completion. Regression modeling examined a priori selected variables associated with monthly costs using Andersen and Newman's framework for healthcare utilization, relevant literature, and clinical expertise. Data are reported in 2015 Canadian dollars ($1CAD = $0.78USD). RESULTS: Forty two children and their caregivers were enrolled. Overall median (interquartile range) monthly healthcare cost was $12 131 ($8159-$15 958) comprising $9929 (89%) family caregiving hours, $996 (9%) publicly funded, and $252 (2%) out-of-pocket (<1% third-party insurance) costs. With higher FIM score (lower dependency), median costs were reduced by 4.5% (95% confidence interval: 8.3%-0.5%), adjusted for age, sex, tracheostomy, and daily ventilation duration. Note: since the three cost categories did not sum to the total statistically derived median cost, the percentage of each category used the sum of median public + caregiver lost time + private out-of-pocket + third-party insurance as the denominator. CONCLUSIONS: For HMV children, most healthcare costs were due to family caregiving costs. More dependent children incur highest costs. The financial burden to family caregivers is substantial and needs to considered in future policy decisions related to pediatric HMV.
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1002/ppul.24923" target="_blank" rel="noreferrer noopener">10.1002/ppul.24923</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).
2020
Adult
Ambulatory Care/economics
Amin R
Canada
Caregivers/economics
Child
Child Preschool
Coyte PC
Female
Guerriere D
Health Care Costs
Health Expenditures
healthcare costs
healthcare utilization
Home Care Services/economics
home mechanical ventilation
Humans
Katz SL
long-term mechanical ventilation
Longitudinal Studies
Male
March 2021 List
McKim DA
Middle Aged
Nonoyama ML
Patient Acceptance of Health Care
Pediatric Pulmonology
Pediatrics
Prospective Studies
Quality Of Life
Respiration Artificial/economics
Rose L
Tracheostomy
ventilator assisted individual
Wasilewski M
Zagorski B
-
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/10.1192/bjp.bp.107.044370" target="_blank" rel="noreferrer noopener">http://doi.org/10.1192/bjp.bp.107.044370</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
Behavioural phenotype of Cornelia de Lange syndrome: case-control study
Publisher
An entity responsible for making the resource available
The British Journal of Psychiatry
Date
A point or period of time associated with an event in the lifecycle of the resource
2008
Subject
The topic of the resource
Male; Case-Control Studies; Young Adult; Surveys and Questionnaires; Child; Humans; Adult; Adolescent; Female; Child Preschool; Phenotype; Autistic Disorder/psychology; Communication Disorders/psychology; Compulsive Behavior/psychology; De Lange Syndrome/psychology; behavioral problems; De Lange syndrome; trajectory; characteristics; global behavior disorder
Creator
An entity primarily responsible for making the resource
Oliver C; Arron K; Sloneem J; Hall S
Description
An account of the resource
BACKGROUND: Cornelia de Lange syndrome is associated with abnormalities on chromosomes 5, 10 and X. AIMS: To delineate the behavioural phenotype of Cornelia de Lange syndrome with specific reference to autistic-spectrum disorder. METHOD: A total of 54 individuals with Cornelia de Lange syndrome (mean age 13.88 years; s.d.=8.58) and 46 comparable individuals with intellectual disability (mean age 13.74 years; s.d.=7.99) were assessed on measures of autistic-spectrum disorder, and adaptive, compulsive and disordered behaviour. RESULTS: There was no difference between the groups in global behaviour disorder. Severe autism was significantly more prevalent in the syndrome group (32.1%) than the comparison group (7.1%). In addition, the syndrome group also evidenced significantly higher levels of compulsive behaviour. CONCLUSIONS: These data suggest that autistic-spectrum disorder is part of the behavioural phenotype of Cornelia de Lange syndrome and that compulsive behaviours are evident. Future research should investigate this behavioural phenotype using contemporary diagnostic algorithms for autism with detailed examination of the phenomenology of compulsive behaviours.
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1192/bjp.bp.107.044370" target="_blank" rel="noreferrer noopener">10.1192/bjp.bp.107.044370</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).
2008
Adolescent
Adult
Arron K
Autistic Disorder/psychology
behavioral problems
Case-Control Studies
characteristics
Child
Child Preschool
Communication Disorders/psychology
Compulsive Behavior/psychology
De Lange syndrome
De Lange Syndrome/psychology
Female
global behavior disorder
Hall S
Humans
Male
Oliver C
Phenotype
Sloneem J
Surveys And Questionnaires
The British Journal of Psychiatry
Trajectory
Young Adult
-
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
March 2021 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
March 2021 List
URL Address
<a href="http://doi.org/10.1097/pcc.0000000000002417" target="_blank" rel="noreferrer noopener">http://doi.org/10.1097/pcc.0000000000002417</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
Prediction of Acquired Morbidity Using Illness Severity Indices in Pediatric Intensive Care Patients
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
2020
Subject
The topic of the resource
Infant; Adolescent; Child; Humans; Risk Factors; Child Preschool; Severity of Illness Index; Retrospective Studies; Infant Newborn; Hospital Mortality; Singapore/epidemiology; Intensive Care Units Pediatric; Morbidity; Critical Care
Creator
An entity primarily responsible for making the resource
Senna S; Ong C; Wong JJ; Allen JC Jr; Sultana R; Lee JH
Description
An account of the resource
OBJECTIVES: To assess the ability of two illness severity scores, Pediatric Logistic Organ Dysfunction Score 2 and Pediatric Index of Mortality 3, in predicting PICU-acquired morbidity. DESIGN: Retrospective chart review conducted from April 2015 to March 2016. SETTING: Single-center study in a multidisciplinary PICU in a tertiary pediatric hospital in Singapore. PATIENTS: The study included all index admissions of patients 0-18 years old to the PICU during the study period. INTERVENTIONS: None. MEASUREMENTS AND MAIN RESULTS: Three outcomes were assessed at hospital discharge: mortality, survival with new morbidity defined as an increase in the Functional Status Scale score of greater than or equal to 3 points from baseline, and survival without morbidity. Of 577 consecutive admissions, 95 were excluded: 82 readmissions, 10 patients greater than or equal to 18 years old, two patients with missing baseline data, and one transferred to another PICU. Of 482 patients, there were 37 hospital deaths (7.7%) and 39 (8.1%) with acquired new morbidity. Median admission Pediatric Logistic Organ Dysfunction Score 2 and Pediatric Index of Mortality 3 scores differed among the three outcome groups. In addition, differences were found in emergency admission and neurologic diagnosis rates, PICU mechanical ventilation usage rates, and PICU length of stay. The highest proportion of neurologic diagnoses was observed in the new morbidity group. The final model simultaneously predicted risks of mortality, survival with new morbidity and survival without morbidity using admission Pediatric Logistic Organ Dysfunction Score 2 score, admission type, neurologic diagnosis, and preexisting chronic disease. Pediatric Logistic Organ Dysfunction Score 2 was superior to Pediatric Index of Mortality 3 in predicting risks of mortality and new morbidity, as indicated by volume under surface values of 0.483 and 0.362, respectively. CONCLUSIONS: Risk of mortality, survival with new morbidity, and survival without morbidity can be predicted simultaneously using admission Pediatric Logistic Organ Dysfunction Score 2, admission type, admission diagnosis, and preexisting chronic disease. Future independent studies will be required to validate the proposed model before clinical implementation.
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1097/pcc.0000000000002417" target="_blank" rel="noreferrer noopener">10.1097/pcc.0000000000002417</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).
2020
Adolescent
Allen JC Jr
Child
Child Preschool
Critical Care
Hospital Mortality
Humans
Infant
Infant Newborn
Intensive Care Units Pediatric
Lee JH
March 2021 List
Morbidity
Ong C
Pediatric Critical Care Medicine
Retrospective Studies
Risk Factors
Senna S
Severity Of Illness Index
Singapore/epidemiology
Sultana R
Wong JJ
-
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/s11325-010-0432-6" target="_blank" rel="noreferrer noopener">http://doi.org/10.1007/s11325-010-0432-6</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
Respiratory events and obstructive sleep apnea in children with achondroplasia: investigation and treatment outcomes
Publisher
An entity responsible for making the resource available
Sleep and Breathing
Date
A point or period of time associated with an event in the lifecycle of the resource
2011
Subject
The topic of the resource
Cross-Sectional Studies; Age Factors; Male; Treatment Outcome; Comorbidity; Child; Humans; Adolescent; Cohort Studies; Female; Child Preschool; Infant; Polysomnography; Body Mass Index; Achondroplasia/di [Diagnosis]; Achondroplasia/th [Therapy]; Sleep Apnea Central/di [Diagnosis]; Sleep Apnea Central/th [Therapy]; Sleep Apnea Obstructive/di [Diagnosis]; Sleep Apnea Obstructive/th [Therapy]; Achondroplasia/ep [Epidemiology]; Airway Obstruction/di [Diagnosis]; Airway Obstruction/ep [Epidemiology]; Airway Obstruction/th [Therapy]; Sleep Apnea Central/ep [Epidemiology]; Sleep Apnea Obstructive/ep [Epidemiology]; breathing difficulties; achondroplasia; trajectory; characteristics; obstructive sleep apnea
Creator
An entity primarily responsible for making the resource
Afsharpaiman S; Sillence D O; Sheikhvatan M; Ault J E; Waters K
Description
An account of the resource
PURPOSE: We report aspects of sleep-disordered breathing in a cohort of achondroplastic children who attended our hospital. METHODS: A retrospective chart review was conducted for a 15-year period to further evaluate the diagnosis and treatment of sleep-disordered breathing in children with achondroplasia. RESULTS: A review of the medical records was undertaken for 46 children (63%, mean age 3.9 years) with achondroplasia that had overnight polysomnography. Among them, 25 (54.3%) had obstructive sleep apnea (OSA). For 19 out of 46 patients (follow-up rate, 41.3%) with a mean follow-up of 31.3 months (range, 3 month to 11 years), 13 had undergone adenotonsillectomy, while nine were treated with continuous positive airway pressure. CONCLUSIONS: Prospective evaluation of our clinic population confirms a high incidence of SDB in achondroplastic children. OSA has been linked to raise intracranial pressure as well as neurocognitive deficits in children and we hypothesize that associations between neurological and respiratory abnormalities in this disorder are a consequence of the early onset of associated respiratory, rather than the neurological complications.
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1007/s11325-010-0432-6" target="_blank" rel="noreferrer noopener">10.1007/s11325-010-0432-6</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
achondroplasia
Achondroplasia/di [Diagnosis]
Achondroplasia/ep [Epidemiology]
Achondroplasia/th [Therapy]
Adolescent
Afsharpaiman S
Age Factors
Airway Obstruction/di [Diagnosis]
Airway Obstruction/ep [Epidemiology]
Airway Obstruction/th [Therapy]
Ault J E
Body Mass Index
breathing difficulties
characteristics
Child
Child Preschool
Cohort Studies
Comorbidity
Cross-sectional Studies
Female
Humans
Infant
Male
obstructive sleep apnea
Polysomnography
Sheikhvatan M
Sillence D O
Sleep and Breathing
Sleep Apnea Central/di [Diagnosis]
Sleep Apnea Central/ep [Epidemiology]
Sleep Apnea Central/th [Therapy]
Sleep Apnea Obstructive/di [Diagnosis]
Sleep Apnea Obstructive/ep [Epidemiology]
Sleep Apnea Obstructive/th [Therapy]
Trajectory
Treatment Outcome
Waters K
-
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
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1007/bf01058150" target="_blank" rel="noreferrer noopener">10.1007/bf01058150</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).
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
2022 Special Edition 4 - Low Resource Setting 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
2022 Special Edition Low Resource Setting Issue
URL Address
<a href="http://doi.org/10.31557/apjcp.2022.23.9.3029" target="_blank" rel="noreferrer noopener"> http://doi.org/10.31557/apjcp.2022.23.9.3029</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
Home-based Palliative Intervention to Improve Quality of Life in Children with Cancer: A Randomized Controlled Trial
Publisher
An entity responsible for making the resource available
Asian Pacific Journal of Cancer Prevention
Date
A point or period of time associated with an event in the lifecycle of the resource
2022
Subject
The topic of the resource
Adolescent; Humans; Child; palliative; Palliative Care; Neoplasms/therapy; cancer; Child Preschool; Pediatric; Quality of Life; Quality of Life; Pain; Anorexia; home visits
Creator
An entity primarily responsible for making the resource
Andriastuti M; Halim PG; Tunjungsari E; Widodo DP
Description
An account of the resource
OBJECTIVE: Over the past few years, an integrated approach of palliative care (PC) to chronic and/or life-threatening conditions care has been widely used. Home-based PC (HBPC) service is developed to meet the needs of patients at home; however, it has not been used widely. This study is aimed at determining the benefits of integrated HBPC for the quality of life (QoL) and symptoms intensity in Indonesian children with malignancies. METHOD: A randomized controlled trial was carried out to compare the quality of life between patients who were given PC (a three-month home visit) and those who did not receive PC (control group). Each group was constituted of thirty children with cancer and aged 2-18 years old and were consulted by a palliative team. The participants were randomly allocated to two groups. In the first and twelfth weeks of the intervention, all patients were assessed using the Pediatric Quality of Life Inventory (PedsQLTM) questionnaire cancer module 3.0 (report by proxy or self-report). Symptoms intensity (pain, anorexia, sleep disturbance) were scored by using Edmonton Symptoms Assessment Scale (ESAS). The mean score and each dimension score of both groups were compared and analyzed using bivariate analysis. RESULTS: In total, fifty participants were included in the study. A significant difference was found between the two groups in terms of the mean total score in control group 62.39 and intervention group 81.63 (p<0.001). The QoL was improved in the intervention group, while it was declined in the control group as the disease progressed. The main improvements were in the pain and nausea aspects (p<0.001), followed by procedural anxiety (p=0.002), treatment anxiety (p=0.002), and worry (p=0.014). Palliative intervention significantly reduced sleep disturbances (p=0.003) and anorexia (p<0.001). CONCLUSION: Home-based PC improved several aspects of QoL and caused better symptom management in children with malignancies. An early intervention concurrent with the underlying treatment can improve QoL in these children.
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.31557/apjcp.2022.23.9.3029" target="_blank" rel="noreferrer noopener">10.31557/apjcp.2022.23.9.3029</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).
2022
2022 Special Edition Low Resource Setting Issue
Adolescent
Andriastuti M
Anorexia
Asian Pacific Journal Of Cancer Prevention
Cancer
Child
Child Preschool
Halim PG
home visits
Humans
Neoplasms/therapy
Pain
Palliative
Palliative Care
Pediatric
Quality Of Life
Tunjungsari E
Widodo DP
-
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/s10578-007-0065-6" target="_blank" rel="noreferrer noopener">http://doi.org/10.1007/s10578-007-0065-6</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
Social and behavioral problems of children with agenesis of the corpus callosum
Publisher
An entity responsible for making the resource available
Child Psychiatry and Human Development
Date
A point or period of time associated with an event in the lifecycle of the resource
2007
Subject
The topic of the resource
Male; Surveys and Questionnaires; Child; Humans; Female; Child Preschool; Agenesis of Corpus Callosum; Social Behavior; Autistic Disorder/epidemiology; Child Behavior Disorders/epidemiology; alertness; behavioral; sleep disturbance; agenesis of the corpus callosum; trajectory; characteristics; attention; aggression; behavior
Creator
An entity primarily responsible for making the resource
Badaruddin D H; Andrews G L; Bolte S; Schilmoeller K J; Schilmoeller G; Paul L K; Brown W S
Description
An account of the resource
Archival data from a survey of parent observations was used to determine the prevalence of social and behavioral problems in children with agenesis of the corpus callosum (ACC). Parent observations were surveyed using the Child Behavior Checklist (CBCL) for 61 children with ACC who were selected from the archive based on criteria of motor development suggesting a relatively high general level of functioning. Younger children with ACC (ages 2-5) were rated as primarily having problems with sleep. Older children with ACC (ages 6-11) manifested problems in attention, social function, thought, and somatic complaints. The older children with ACC were also compared to CBCL data from 52 children with autism who were selected from a previous study. Children with ACC were generally less impaired than children with autism on nearly all scales, with significantly less severe problems in the areas of attention, anxiety/depression, social function, and unusual thoughts. A further questionnaire related to diagnostic criteria for autism indicated that some children with ACC had traits that are among those that contribute to the diagnosis of autism within the domains of social interaction and social communication, but fewer who manifest repetitive and restricted behaviors.
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1007/s10578-007-0065-6" target="_blank" rel="noreferrer noopener">10.1007/s10578-007-0065-6</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).
2007
Agenesis of Corpus Callosum
agenesis of the corpus callosum
Aggression
alertness
Andrews G L
Attention
Autistic Disorder/epidemiology
Badaruddin D H
Behavior
behavioral
Bolte S
Brown W S
characteristics
Child
Child Preschool
Child Behavior Disorders/epidemiology
Child Psychiatry and Human Development
Female
Humans
Male
Paul L K
Schilmoeller G
Schilmoeller K J
sleep disturbance
Social Behavior
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/10.1002/ajmg.a.37784" target="_blank" rel="noreferrer noopener">http://doi.org/10.1002/ajmg.a.37784</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
Determinants of sleep disturbances in Rett syndrome: Novel findings in relation to genotype
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
2016
Subject
The topic of the resource
Middle Aged; Infant Newborn; Severity of Illness Index; Epilepsy/ge [Genetics]; Young Adult; Child; Humans; Adult; Adolescent; Child Preschool; Infant; Mutation; Epilepsy/di [Diagnosis]; Sleep Wake Disorders/di [Diagnosis]; Phenotype; Genetic Association Studies; Genotype; Rett Syndrome/ge [Genetics]; Rett Syndrome/pp [Physiopathology]; Sleep Wake Disorders/ge [Genetics]; 0 (Methyl-CpG-Binding Protein 2); Methyl-CpG-Binding Protein 2/ge [Genetics]; Rett Syndrome/di [Diagnosis]; sleep disturbance/disorders; Rett syndrome; trajectory; characteristics
Creator
An entity primarily responsible for making the resource
Boban S; Wong K; Epstein A; Anderson B; Murphy N; Downs J; Leonard H
Description
An account of the resource
Rett syndrome is a rare but severe neurological disorder associated with a mutation in the methyl CpG binding protein 2 (MECP2) gene. Sleep problems and epilepsy are two of many comorbidities associated with this disorder. This study investigated the prevalence and determinants of sleep problems in Rett syndrome using an international sample. Families with a child with a confirmed Rett syndrome diagnosis and a MECP2 mutation registered in the International Rett Syndrome Phenotype Database (InterRett) were invited to participate. Questionnaires were returned by 364/461 (78.9%) either in web-based or paper format. Families completed the Sleep Disturbance Scale for Children and provided information on the presence, nature, and frequency of their child's sleep problems. Multivariate multinomial regression was used to investigate the relationships between selected sleep problems, age group, and genotype and linear regression for the relationships between sleep disturbance scales and a range of covariates. Night waking was the most prevalent sleep problem affecting over 80% with nearly half (48.3%) currently waking often at night. Initiating and maintaining sleep was most disturbed for younger children and those with a p.Arg294* mutation. Severe seizure activity was associated with poor sleep after adjusting for age group, mutation type, and mobility. We were surprised to find associations between the p.Arg294* mutation and some sleep disturbances given that other aspects of its phenotype are milder. These findings highlight the complexities of aberrant MECP2 function in Rett syndrome and explain some of the variation in manifestation of sleep disturbances. © 2016 Wiley Periodicals, Inc.
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1002/ajmg.a.37784" target="_blank" rel="noreferrer noopener">10.1002/ajmg.a.37784</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).
0 (Methyl-CpG-Binding Protein 2)
2016
Adolescent
Adult
American Journal of Medical Genetics Part A
Anderson B
Boban S
characteristics
Child
Child Preschool
Downs J
Epilepsy/di [diagnosis]
Epilepsy/ge [Genetics]
Epstein A
Genetic Association Studies
Genotype
Humans
Infant
Infant Newborn
Leonard H
Methyl-CpG-Binding Protein 2/ge [Genetics]
Middle Aged
Murphy N
Mutation
Phenotype
Rett syndrome
Rett Syndrome/di [Diagnosis]
Rett Syndrome/ge [Genetics]
Rett Syndrome/pp [Physiopathology]
Severity Of Illness Index
sleep disturbance/disorders
Sleep Wake Disorders/di [Diagnosis]
Sleep Wake Disorders/ge [Genetics]
Trajectory
Wong K
Young Adult
-
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/10.1016/0387-7604(94)00118-h" target="_blank" rel="noreferrer noopener">http://doi.org/10.1016/0387-7604(94)00118-h</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
Sleep disorders in tuberous sclerosis: a polysomnographic study
Publisher
An entity responsible for making the resource available
Brain and Development
Date
A point or period of time associated with an event in the lifecycle of the resource
1995
Subject
The topic of the resource
Circadian Rhythm; Male; Child; Humans; Adolescent; Female; Child Preschool; Epilepsy; Tuberous Sclerosis; Polysomnography; Sleep Wake Disorders; sleep disturbance/disorders; trajectory; characteristics
Creator
An entity primarily responsible for making the resource
Bruni O; Cortesi F; Giannotti F; Curatolo P
Description
An account of the resource
Overnight polysomnography was performed in 10 subjects with tuberous sclerosis (TS) and partial epilepsy in order to investigate the relationships between sleep organization, sleep disorders and epilepsy. Sleep architecture abnormalities were observed in 9 cases. Compared with ten healthy age-matched controls, the TS group showed a shorter total sleep time, a reduced sleep efficiency, a higher number of awakenings and stage transitions, an increased wake after sleep onset and stage 1 and a decreased REM sleep. Children with seizures showed a more disrupted sleep architecture compared with seizure-free children. Sleep disorders in TS were mainly due to sleep-related epileptic events and were more evident in children who showed large bifrontal or temporal tubers on MRI.
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1016/0387-7604(94)00118-h" target="_blank" rel="noreferrer noopener">10.1016/0387-7604(94)00118-h</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).
1995
Adolescent
Brain and Development
Bruni O
characteristics
Child
Child Preschool
Circadian Rhythm
Cortesi F
Curatolo P
Epilepsy
Female
Giannotti F
Humans
Male
Polysomnography
sleep disturbance/disorders
Sleep Wake Disorders
Trajectory
Tuberous Sclerosis
-
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/10.1177/088307389601100414" target="_blank" rel="noreferrer noopener">http://doi.org/10.1177/088307389601100414</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
Melatonin appears ineffective in children with intellectual deficits and fragmented sleep: six "N of 1" trials
Publisher
An entity responsible for making the resource available
Journal of Child Neurology
Date
A point or period of time associated with an event in the lifecycle of the resource
1996
Subject
The topic of the resource
Male; Child; Humans; Adolescent; Female; Child Preschool; Double-Blind Method; Intellectual Disability/complications; Melatonin/therapeutic use; Sleep Wake Disorders/drug therapy/etiology; sleep disturbance/disorders; undefined neurodevelopmental disorders; neurodevelopmental disorder; pharmacologic intervention; melatonin
Creator
An entity primarily responsible for making the resource
Camfield P; Gordon K; Dooley J; Camfield C
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1177/088307389601100414" target="_blank" rel="noreferrer noopener">10.1177/088307389601100414</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).
1996
Adolescent
Camfield C
Camfield P
Child
Child Preschool
Dooley J
Double-Blind Method
Female
Gordon K
Humans
Intellectual Disability/complications
Journal of Child Neurology
Male
melatonin
Melatonin/therapeutic use
neurodevelopmental disorder
pharmacologic intervention
sleep disturbance/disorders
Sleep Wake Disorders/drug therapy/etiology
undefined neurodevelopmental disorders
-
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
April 2019 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
April 2019 List
URL Address
<a href="http://doi.org/10.1089/jpm.2010.0315" target="_blank" rel="noreferrer noopener">http://doi.o rg/10.1089/jpm.2010.0315</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
Perceptions of discrimination among Mexican American families of seriously ill children
Publisher
An entity responsible for making the resource available
Journal of Palliative Medicine
Date
A point or period of time associated with an event in the lifecycle of the resource
2011
Subject
The topic of the resource
Middle Aged; Male; Young Adult; Child; Humans; Adult; Adolescent; Female; Child Preschool; Infant; Retrospective Studies; Critical Illness; California; Mexican Americans/px [Psychology]; Prejudice
Creator
An entity primarily responsible for making the resource
Davies B; Larson J; Contro N; Cabrera AP
Description
An account of the resource
This paper describes Mexican American family members' descriptions of perceived discrimination by pediatric health care providers (HCPs) and the families' reactions to the HCPs' discriminatory conduct. A retrospective, grounded theory design guided the overall study. Content analysis of interviews with 13 participants from 11 families who were recruited from two children's hospitals in Northern California resulted in numerous codes and revealed that participants perceived discrimination when they were treated differently from other, usually white, families. They believed they were treated differently because they were Mexican, because they were poor, because of language barriers, or because of their physical appearance.
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1089/jpm.2010.0315" target="_blank" rel="noreferrer noopener">10.1089/jpm.2010.0315</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
April 2019 List
Cabrera AP
California
Child
Child Preschool
Contro N
Critical Illness
Davies B
Female
Humans
Infant
Journal of Palliative Medicine
Larson J
Male
Mexican Americans/px [Psychology]
Middle Aged
Prejudice
Retrospective Studies
Young Adult
-
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/10.1111/j.1469-8749.1997.tb07377.x" target="_blank" rel="noreferrer noopener">http://doi.org/10.1111/j.1469-8749.1997.tb07377.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
Correlates of maladaptive behavior in individuals with 5p- (cri du chat) syndrome
Publisher
An entity responsible for making the resource available
Developmental Medicine and Child Neurology
Date
A point or period of time associated with an event in the lifecycle of the resource
1997
Subject
The topic of the resource
Male; Surveys and Questionnaires; Child; Humans; Adult; Adolescent; Female; Child Preschool; Analysis of Variance; Risk Factors; Psychiatric Status Rating Scales; Adjustment Disorders/etiology; Child Behavior Disorders/etiology; Cri-du-Chat Syndrome/genetics/psychology; Gene Deletion; Intellectual Disability/psychology; Mental Disorders/etiology; Translocation Genetic; behavioral problems; Cri-du-chat; trajectory; characteristics; self-injury; aggression; pain behaviors; mood; low mood; hyperactivity; impulsivity; repetitive language use
Creator
An entity primarily responsible for making the resource
Dykens E M; Clarke D J
Description
An account of the resource
This study examined the range, distinctiveness, and correlates of maladaptive behavior in 146 subjects with 5p- (cri du chat) syndrome using the Aberrant Behavior Checklist as a standardized measure. Hyperactivity was the most significant and frequent problem in the sample. Subjects with 5p- syndrome also showed aggression, tantrums, self-injurious behavior, and stereotypies; some of these problems were more pronounced in individuals with lower cognitive-adaptive levels, as well as in those with histories of previous medication trials. Autistic-like features and social withdrawal were more characteristic of individuals with translocations as opposed to deletions, even when controlling for the lower adaptive level of the translocation group. These findings encourage further research on the behavior of individuals with 5p- syndrome.
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1111/j.1469-8749.1997.tb07377.x" target="_blank" rel="noreferrer noopener">10.1111/j.1469-8749.1997.tb07377.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).
1997
Adjustment Disorders/etiology
Adolescent
Adult
Aggression
Analysis of Variance
behavioral problems
characteristics
Child
Child Preschool
Child Behavior Disorders/etiology
Clarke D J
Cri-du-chat
Cri-du-Chat Syndrome/genetics/psychology
Developmental Medicine and Child Neurology
Dykens E M
Female
Gene Deletion
Humans
hyperactivity
impulsivity
Intellectual Disability/psychology
low mood
Male
Mental Disorders/etiology
Mood
pain behaviors
Psychiatric Status Rating Scales
repetitive language use
Risk Factors
self-injury
Surveys And Questionnaires
Trajectory
Translocation Genetic
-
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.37502" target="_blank" rel="noreferrer noopener">http://doi.org/10.1002/ajmg.a.37502</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
Sleep disturbance in Mowat-Wilson 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
2016
Subject
The topic of the resource
Middle Aged; Male; Severity of Illness Index; Surveys and Questionnaires; Child; Humans; Adult; Adolescent; Female; Child Preschool; Australia; Mutation; Facies; Hirschsprung Disease/di [Diagnosis]; Intellectual Disability/di [Diagnosis]; Microcephaly/di [Diagnosis]; Sleep Initiation and Maintenance Disorders/di [Diagnosis]; 0 (Homeodomain Proteins); 0 (Repressor Proteins); 0 (ZEB2 protein human); Behavior Rating Scale; Gene Expression; Hirschsprung Disease/co [Complications]; Hirschsprung Disease/ge [Genetics]; Hirschsprung Disease/pp [Physiopathology]; Homeodomain Proteins/ge [Genetics]; Intellectual Disability/co [Complications]; Intellectual Disability/ge [Genetics]; Intellectual Disability/pp [Physiopathology]; Microcephaly/co [Complications]; Microcephaly/ge [Genetics]; Microcephaly/pp [Physiopathology]; Mowat-Wilson syndrome; Repressor Proteins/ge [Genetics]; Sleep Initiation and Maintenance Disorders/co [Complications]; Sleep Initiation and Maintenance Disorders/ge [Genetics]; Sleep Initiation and Maintenance Disorders/pp [Physiopathology]; sleep disturbance/disorders; Mowat-Wilson syndrome; trajectory; characteristics
Creator
An entity primarily responsible for making the resource
Evans E; Mowat D; Wilson M; Einfeld S
Description
An account of the resource
Mowat-Wilson syndrome (MWS) is a multiple congenital anomaly syndrome caused by a heterozygous mutation or deletion of the ZEB2 gene. It is characterized by a distinctive facial appearance in association with intellectual disability (ID) and variable other features including agenesis of the corpus callosum, seizures, congenital heart defects, microcephaly, short stature, hypotonia, and Hirschsprung disease. The current study investigated sleep disturbance in people with MWS. In a series of unstructured interviews focused on development and behaviors in MWS, family members frequently reported sleep disturbance, particularly early-morning waking and frequent night waking. The Sleep Disturbance Scale for Children (SDSC) was therefore administered to a sample of 35 individuals with MWS, along with the Developmental Behaviour Checklist (DBC) to measure behavioral and emotional disturbance. A high level of sleep disturbance was found in the MWS sample, with 53% scoring in the borderline range and 44% in the clinical disorder range for at least one subscale of the SDSC. Scores were highest for the Sleep-wake transition disorders subscale, with 91% of participants reaching at least the borderline disorder range. A significant positive association was found between total scores on the SDSC and the DBC Total Behaviour Problem Score. These results suggest that sleep disorders should be screened for in people with MWS, and where appropriate, referrals to sleep specialists made for management of sleep problems.Copyright © 2015 Wiley Periodicals, Inc.
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1002/ajmg.a.37502" target="_blank" rel="noreferrer noopener">10.1002/ajmg.a.37502</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).
0 (Homeodomain Proteins)
0 (Repressor Proteins)
0 (ZEB2 protein human)
2016
Adolescent
Adult
American Journal of Medical Genetics Part A
Australia
Behavior Rating Scale
characteristics
Child
Child Preschool
Einfeld S
Evans E
Facies
Female
Gene Expression
Hirschsprung Disease/co [Complications]
Hirschsprung Disease/di [Diagnosis]
Hirschsprung Disease/ge [Genetics]
Hirschsprung Disease/pp [Physiopathology]
Homeodomain Proteins/ge [Genetics]
Humans
Intellectual Disability/co [Complications]
Intellectual Disability/di [Diagnosis]
Intellectual Disability/ge [Genetics]
Intellectual Disability/pp [Physiopathology]
Male
Microcephaly/co [Complications]
Microcephaly/di [Diagnosis]
Microcephaly/ge [Genetics]
Microcephaly/pp [Physiopathology]
Middle Aged
Mowat D
Mowat-Wilson syndrome
Mutation
Repressor Proteins/ge [Genetics]
Severity Of Illness Index
sleep disturbance/disorders
Sleep Initiation and Maintenance Disorders/co [Complications]
Sleep Initiation and Maintenance Disorders/di [Diagnosis]
Sleep Initiation and Maintenance Disorders/ge [Genetics]
Sleep Initiation and Maintenance Disorders/pp [Physiopathology]
Surveys And Questionnaires
Trajectory
Wilson M
-
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.870050303" target="_blank" rel="noreferrer noopener">http://doi.org/10.1002/mds.870050303</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
Rett syndrome and associated movement disorders
Publisher
An entity responsible for making the resource available
Movement Disorders
Date
A point or period of time associated with an event in the lifecycle of the resource
1990
Subject
The topic of the resource
Child; Humans; Adult; Adolescent; Female; Child Preschool; Infant; Movement Disorders/etiology/physiopathology; Rett Syndrome/complications/physiopathology; Stereotyped Behavior; tone and motor problems; Rett syndrome; trajectory; characteristics; stereotypy; gait disturbance
Creator
An entity primarily responsible for making the resource
FitzGerald P M; Jankovic J; Percy A K
Description
An account of the resource
Rett syndrome, a progressive neurodegenerative disorder described only in female subjects, is manifested by a wide spectrum of behavioral and motor abnormalities. We studied 32 patients with this disorder, ages 30 months to 28 years old, and characterized their extrapyramidal disturbance. The most common motor abnormalities were stereotyped movements and gait disturbance, seen in all patients. Bruxism, oculogyric crises, parkinsonism, and dystonia were also common, but myoclonus and choreoathetosis were seen only infrequently. The hyperkinetic movement disorders tended to dominate in younger patients, while bradykinetic disorders were more evident in the older patients. This study provides evidence that movement disorders seen in Rett syndrome reflect age-related neurodegenerative changes in the basal ganglia.
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1002/mds.870050303" target="_blank" rel="noreferrer noopener">10.1002/mds.870050303</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).
1990
Adolescent
Adult
characteristics
Child
Child Preschool
Female
FitzGerald P M
gait disturbance
Humans
Infant
Jankovic J
Movement Disorders
Movement Disorders/etiology/physiopathology
Percy A K
Rett syndrome
Rett Syndrome/complications/physiopathology
Stereotyped Behavior
stereotypy
tone and motor problems
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/10.1136/adc.2004.065482" target="_blank" rel="noreferrer noopener">http://doi.org/10.1136/adc.2004.065482</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
Sleep disturbance in Sanfilippo syndrome: a parental questionnaire study
Publisher
An entity responsible for making the resource available
Archives of Disease in Childhood
Date
A point or period of time associated with an event in the lifecycle of the resource
2005
Subject
The topic of the resource
Treatment Outcome; Infant Newborn; Humans; Adolescent; Child Preschool; Infant; Questionnaires; Age of Onset; Incidence; Behavior Therapy; Melatonin/therapeutic use; Mucopolysaccharidosis III/psychology; Sleep Disorders/epidemiology/etiology/therapy; Q3 Literature Search; child; adult; Adolescent; Adult; Age of Onset; Behavior Therapy; Child; Preschool; Humans; Incidence; Infant; sleep disturbance/disorders; MPS III; trajectory; characteristics; melatonin; benzodiazepines
Creator
An entity primarily responsible for making the resource
Fraser J; Gason A A; Wraith J E; Delatycki M B
Description
An account of the resource
AIMS: To determine the incidence, manifestations, and best management of sleep disturbance in Sanfilippo syndrome (mucopolysaccharidosis (MPS) type III). METHODS: Families were ascertained through the MPS societies of Australasia, the UK, and the USA. Questionnaires were sent by mail and were answered anonymously. Identical questions regarding sleep disturbance were asked about unaffected siblings to provide control data. Sleep disturbance was quantified by a total sleep disturbance score. RESULTS: A total of 141 responses were received; 91.5% of children with Sanfilippo syndrome had sleep disturbance and this was significantly higher than for their unaffected sibs; 77.5% of parents had used medication for this problem, with melatonin and antihistamines being most commonly used. Melatonin and benzodiazepines were reported as the most efficacious. Many different environmental modifications had been employed for this problem and some parents reported success with behavioural therapies. CONCLUSIONS: Sleep disturbance is common, severe, and difficult to manage in Sanfilippo syndrome. Based on the parental responses and its safety profile, melatonin is the first line drug that should be tried. Behavioural therapy should be tried in all with Sanfilippo syndrome and sleep disturbance.
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1136/adc.2004.065482" target="_blank" rel="noreferrer noopener">10.1136/adc.2004.065482</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).
2005
Adolescent
Adult
Age of Onset
Archives of Disease in Childhood
Behavior Therapy
Benzodiazepines
characteristics
Child
Child Preschool
Delatycki M B
Fraser J
Gason A A
Humans
Incidence
Infant
Infant Newborn
melatonin
Melatonin/therapeutic use
MPS III
Mucopolysaccharidosis III/psychology
Preschool
Q3 Scoping Review Results
Questionnaires
Sleep Disorders/epidemiology/etiology/therapy
sleep disturbance/disorders
Trajectory
Treatment Outcome
Wraith 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/10.1002/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
January 2021 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 2021 List
URL Address
<a href="http://doi.org/10.1016/j.ctim.2020.102402" target="_blank" rel="noreferrer noopener">http://doi.org/10.1016/j.ctim.2020.102402</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
Safety and pharmacokinetics of medical cannabis preparation in a monocentric series of young patients with drug resistant epilepsy
Publisher
An entity responsible for making the resource available
Complementary Therapies in Medicine
Date
A point or period of time associated with an event in the lifecycle of the resource
2020
Subject
The topic of the resource
Adolescent; Adult; Child; Female; Humans; Male; Child Preschool; Dose-Response Relationship Drug; Young Adult; Prospective Studies; Cannabidiol (CBD); Childhood; Delta-9-tetrahydrocannabinol (THC); Drug-resistant epilepsy; Medical cannabis galenic preparation; Drug Resistant Epilepsy/drug therapy; Medical Marijuana/administration & dosage/adverse effects/pharmacokinetics
Creator
An entity primarily responsible for making the resource
Gherzi M; Milano G; Fucile C; Calevo MG; Mancardi MM; Nobili L; Astuni P; Marini V; Barco S; Cangemi G; Manfredini L; Mattioli F; De Grandis E
Description
An account of the resource
OBJECTIVES: To evaluate safety and pharmacokinetic parameters (PK) of medical cannabis in add-on for children and young adults with drug-resistant epilepsy. DESIGN, SETTING: Ten patients (4 females, 6 males, age 2.5-23.2 years) were enrolled in a prospective open trial with a galenic preparation (decoction) of Italian cannabis (FM2, ratio THC:CBD = 3:5, range THC 5.2-7.2 %; CBD 8.2-11.1 %). Patients received the first dose in Hospital, progressively augmented by CBD dose titration (from 1 to 4 mg/kg/day). OUTCOME MEASURES: In order to assess safety, blood parameters, heart rates and electrocardiograms (ECGs) were evaluated before the enrollment and during the follow up. The PK study was performed measuring THC and CBD concentrations by UHPLC-MS/MS in plasma samples collected during the first administration and at each follow-up visit. RESULTS: Two out of ten patients stopped the treatment for adverse events (detected in 6/10: gastroenteric, sleep or behavioral disorders) and difficulties in drug supply. We observed minor ECG alterations in two patients and asymptomatic transient reductions of fibrinogen after 6 months of therapy. The PK study during follow-up revealed statistically significant correlations between THC-CBD blood concentrations and: volumes of decoction, FM2 and THC-CBD daily dosages. CONCLUSIONS: The present study, although with some limitations, shows a good safety profile of medical cannabis in children and young patients with drug-resistant epilepsy and encourages the possibility of further studies with oral cannabis-based drugs. The correlations between THC-CBD plasma concentrations and their administered dosages underline the need of a therapeutic drug monitoring for cannabinoids therapy.
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1016/j.ctim.2020.102402" target="_blank" rel="noreferrer noopener">10.1016/j.ctim.2020.102402</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).
2020
Adolescent
Adult
Astuni P
Barco S
Calevo MG
Cangemi G
Cannabidiol (CBD)
Child
Child Preschool
childhood
Complementary Therapies in Medicine
De Grandis E
Delta-9-tetrahydrocannabinol (THC)
Dose-Response Relationship Drug
Drug Resistant Epilepsy/drug therapy
Drug-resistant epilepsy
Female
Fucile C
Gherzi M
Humans
January 2021 List
Male
Mancardi MM
Manfredini L
Marini V
Mattioli F
Medical cannabis galenic preparation
Medical Marijuana/administration & dosage/adverse effects/pharmacokinetics
Milano G
Nobili L
Prospective Studies
Young Adult
-
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/10.1177/08830738050200010301" target="_blank" rel="noreferrer noopener">http://doi.org/10.1177/08830738050200010301</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
Melatonin excretion in normal children and in tuberous sclerosis complex with sleep disorder responsive to melatonin
Publisher
An entity responsible for making the resource available
Journal of 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
Circadian Rhythm; Male; Case-Control Studies; Child; Humans; Adolescent; Female; Child Preschool; Administration Oral; Reference Values; Tuberous Sclerosis; Antioxidants; Melatonin; Sleep Wake Disorders; Antioxidants/pk [Pharmacokinetics]; Circadian Rhythm; Melatonin/aa [Analogs & Derivatives]; Melatonin/pk [Pharmacokinetics]; Sleep Wake Disorders/co [Complications]; Tuberous Sclerosis/co [Complications]; 0 (Antioxidants); 2208-40-4 (6-sulfatoxymelatonin); Antioxidants/ad [Administration & Dosage]; JL5DK93RCL (Melatonin); Melatonin/ad [Administration & Dosage]; Melatonin/ur [Urine]; Sleep Wake Disorders/pp [Physiopathology]; Tuberous Sclerosis/pp [Physiopathology]; sleep disturbance/disorders; trajectory; characteristics; melatonin
Creator
An entity primarily responsible for making the resource
Hancock E; O'Callaghan F; English J; Osborne J P
Description
An account of the resource
To determine normal melatonin excretion patterns in healthy children without sleep disorder and to compare these with those of patients with tuberous sclerosis complex and sleep disorder responsive to exogenous melatonin, we measured 6-sulfatoxymelatonin excretion in 21 healthy children and in 7 patients with tuberous sclerosis complex and sleep disorder responsive to melatonin (a 5 mg oral dose increasing total sleep time). Total excretion, cosinor percentage, and acrophase time of 6-sulfatoxymelatonin excretion were estimated. In normal children, total 6-sulfatoxymelatonin excretion was range 11.1 to 40.2 microg (mean 19.0 microg, SD 7.4 microg); cosinor percentage rhythm range was 52.9% to 100% (mean 87%, median 94%); and acrophase time range was 23 hours, 54 minutes to 10 hours, 42 minutes (mean 5 hours, 54 minutes; median 4 hours, 12 minutes). Fifth and 95th percentiles were 11.1 to 29.0 microg, 57.8% to 99.9%, and 2 hours, 1 minute to 10 hours, 4 minutes. In tuberous sclerosis, normal patterns of melatonin excretion were seen in responders. Circadian patterns of melatonin excretion were similar in children and adults. We propose that exogenous melatonin can act by a simple sedative action.
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1177/08830738050200010301" target="_blank" rel="noreferrer noopener">10.1177/08830738050200010301</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).
0 (Antioxidants)
2005
2208-40-4 (6-sulfatoxymelatonin)
Administration Oral
Adolescent
Antioxidants
Antioxidants/ad [Administration & Dosage]
Antioxidants/pk [Pharmacokinetics]
Case-Control Studies
characteristics
Child
Child Preschool
Circadian Rhythm
English J
Female
Hancock E
Humans
JL5DK93RCL (Melatonin)
Journal of Child Neurology
Male
melatonin
Melatonin/aa [Analogs & Derivatives]
Melatonin/ad [Administration & Dosage]
Melatonin/pk [Pharmacokinetics]
Melatonin/ur [Urine]
O'Callaghan F
Osborne J P
Reference Values
sleep disturbance/disorders
Sleep Wake Disorders
Sleep Wake Disorders/co [Complications]
Sleep Wake Disorders/pp [Physiopathology]
Trajectory
Tuberous Sclerosis
Tuberous Sclerosis/co [Complications]
Tuberous Sclerosis/pp [Physiopathology]
-
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/10.1177/08830738050200011302" target="_blank" rel="noreferrer noopener">http://doi.org/10.1177/08830738050200011302</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
Effect of melatonin dosage on sleep disorder in tuberous sclerosis complex
Publisher
An entity responsible for making the resource available
Journal of 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
Male; Treatment Outcome; Dose-Response Relationship Drug; Child; Humans; Adult; Cross-Over Studies; Female; Child Preschool; Infant; Administration Oral; Double-Blind Method; Tuberous Sclerosis/complications; Antioxidants/pharmacology/therapeutic use; Melatonin/pharmacology/therapeutic use; Sleep Wake Disorders/drug therapy/etiology; sleep disturbance/disorders; tuberous sclerosis; pharmacologic intervention; melatonin
Creator
An entity primarily responsible for making the resource
Hancock E; O'Callaghan F; Osborne J P
Description
An account of the resource
We report a randomized, double-blind, controlled, crossover trial investigating the response to oral melatonin using two dose regimens in patients with sleep disorders associated with tuberous sclerosis complex. Eight outpatients with tuberous sclerosis complex and sleep disorder received either 5 or 10 mg of melatonin. Sleep latency, total sleep time, number of awakenings, and seizure frequency were recorded in sleep and seizure diaries. No evidence of a dose effect between 5 and 10 mg was seen with respect to any outcome measure. (The 5 mg results are given first: sleep latency, 86 and 76 minutes; total sleep time, 8 hours, 57 minutes and 9 hours, 4 minutes; and sleep fragmentation, 0.8 and 1.0). This study might have missed a small beneficial effect of 10 mg melatonin. We propose that an initial trial of 5 mg melatonin is worth considering in patients with tuberous sclerosis complex and sleep disorder.
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1177/08830738050200011302" target="_blank" rel="noreferrer noopener">10.1177/08830738050200011302</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).
2005
Administration Oral
Adult
Antioxidants/pharmacology/therapeutic use
Child
Child Preschool
Cross-Over Studies
Dose-Response Relationship Drug
Double-Blind Method
Female
Hancock E
Humans
Infant
Journal of Child Neurology
Male
melatonin
Melatonin/pharmacology/therapeutic use
O'Callaghan F
Osborne J P
pharmacologic intervention
sleep disturbance/disorders
Sleep Wake Disorders/drug therapy/etiology
Treatment Outcome
Tuberous Sclerosis
Tuberous Sclerosis/complications
-
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 2021 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 2021 List
URL Address
<a href="http://doi.org/10.1213/ane.0000000000004585" target="_blank" rel="noreferrer noopener">http://doi.org/10.1213/ane.0000000000004585</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
Adverse Events During Intrahospital Transport of Critically Ill Children: A Systematic Review
Publisher
An entity responsible for making the resource available
Anesthesia & Analgesia
Date
A point or period of time associated with an event in the lifecycle of the resource
2020
Subject
The topic of the resource
Infant; Adolescent; Child; Humans; Child Preschool; Pediatrics; Infant Newborn; Critical Illness/therapy; Critical Care/methods; Transportation of Patients
Creator
An entity primarily responsible for making the resource
Haydar B; Baetzel A; Elliott A; MacEachern M; Kamal A; Christensen R
Description
An account of the resource
Intrahospital transport of a critically ill patient is often required to achieve a diagnostic and/or therapeutic objective. However, clinicians who recommend a procedure that requires transport are often not fully aware of the risks of transport. Clinicians involved in the care of critically ill children may therefore benefit from a clear enumeration of adverse events that have occurred during transport, risk factors for those events, and guidance for event prevention. The objective of this review was to collect all published harm and adverse events that occurred in critically ill children in the context of transport within a medical center, as well as the incidence of each type of event. A secondary objective was to identify what interventions have been previously studied that reduce events and to collect recommendations for harm prevention from study authors. Ovid MEDLINE, Cochrane Central Register of Controlled Trials, Embase, and CINAHL were searched in January 2018 and again in December 2018. Terms indicating pediatric patients, intrahospital transport, critical illness, and adverse events were used. Titles and abstracts were screened and full text was reviewed for any article meeting inclusion criteria. If articles included both children and adults, incidence data were collected only if the number of pediatric patients could be ascertained. Of 471 full-text articles reviewed, 40 met inclusion criteria, of which 24 included only children, totaling 4104 patient transports. Heterogeneity was high, owing to a wide range of populations, settings, data collection methods, and outcomes. The incidence of adverse events varied widely between studies. Examples of harm included emergent tracheostomy, pneumothorax, and cardiac arrest requiring chest compressions. Respiratory and airway events were the most common type of adverse event. Hypothermia was common in infants. One transport-associated death was reported. When causation was assessed, most events were judged to have been preventable or potentially mitigated by improved double-checks and usage of checklists. Prospective studies demonstrated the superiority of mechanical ventilation over manual ventilation for intubated patients. Risk of adverse events during critical care transport appears to relate to the patient's underlying illness and degree of respiratory support. Recommendations for reducing these adverse events have frequently included the use of checklists. Other recommendations include optimization of the patient's physiological status before transport, training with transport equipment, double-checking of equipment before transport, and having experienced clinicians accompany the patient. All available recommendations for reducing transport-associated adverse events in included articles were collated and included.
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1213/ane.0000000000004585" target="_blank" rel="noreferrer noopener">10.1213/ane.0000000000004585</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).
2020
Adolescent
Anesthesia & Analgesia
Baetzel A
Child
Child Preschool
Christensen R
Critical Care/methods
Critical Illness/therapy
Elliott A
Haydar B
Humans
Infant
Infant Newborn
January 2021 List
Kamal A
MacEachern M
Pediatrics
Transportation of Patients
-
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.1320410215" target="_blank" rel="noreferrer noopener">http://doi.org/10.1002/ajmg.1320410215</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 and motor skills in achondroplastic infants: neurologic and respiratory correlates
Publisher
An entity responsible for making the resource available
American Journal of Medical Genetics
Date
A point or period of time associated with an event in the lifecycle of the resource
1991
Subject
The topic of the resource
Brain/pathology; Male; Electroencephalography; Humans; Prospective Studies; Female; Child Preschool; Infant; Neuropsychological Tests; Psychomotor Performance; Intelligence; Atrophy; Achondroplasia/complications/physiopathology/psychology; Evoked Potentials Somatosensory; Intellectual Disability/etiology; Respiration Disorders/etiology/physiopathology; Sleep Apnea Syndromes/etiology/physiopathology; sleep disturbance; tone and motor problems; achondroplasia; trajectory; characteristics; respiratory dysfunction
Creator
An entity primarily responsible for making the resource
Hecht J T; Thompson N M; Weir T; Patchell L; Horton W A
Description
An account of the resource
Thirteen infants with achondroplasia underwent psychometric testing as part of a comprehensive neurologic assessment. As a group, mental development was average and motor development was delayed, although a wide range of scores was obtained. Foramen magnum measurements were correlated with respiratory dysfunction, abnormal somatosensory evoked potentials, and delayed motor development. Abnormal polysomnogram outcome was associated with reduced mental capacity. In light of the reported increased frequency of respiratory dysfunction in achondroplasia, these findings warrant careful attention and further study.
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1002/ajmg.1320410215" target="_blank" rel="noreferrer noopener">10.1002/ajmg.1320410215</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).
1991
achondroplasia
Achondroplasia/complications/physiopathology/psychology
American Journal Of Medical Genetics
Atrophy
Brain/pathology
characteristics
Child Preschool
Electroencephalography
Evoked Potentials Somatosensory
Female
Hecht J T
Horton W A
Humans
Infant
Intellectual Disability/etiology
Intelligence
Male
Neuropsychological Tests
Patchell L
Prospective Studies
Psychomotor Performance
Respiration Disorders/etiology/physiopathology
respiratory dysfunction
Sleep Apnea Syndromes/etiology/physiopathology
sleep disturbance
Thompson N M
tone and motor problems
Trajectory
Weir T
-
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
2018 Oncology 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
Oncology 2018 List
URL Address
<a href="http://doi.org/10.1080/0284186X.2016.1275780" target="_blank" rel="noreferrer noopener">http://doi.o
rg/10.1080/0284186X.2016.1275780</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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Sick leave among parents of children with cancer - a national cohort study
Publisher
An entity responsible for making the resource available
Acta Oncologica
Date
A point or period of time associated with an event in the lifecycle of the resource
2017
Subject
The topic of the resource
Male; Infant Newborn; Follow-Up Studies; Prognosis; Parents/psychology; Child; Humans; Adult; Adolescent; Cohort Studies; Female; Child Preschool; Infant; Sweden/epidemiology; Only Child; Neoplasms/diagnosis/epidemiology/psychology; Registries; Sick Leave; Sick Leave/statistics & numerical data
Creator
An entity primarily responsible for making the resource
Hjelmstedt S; Lindahl NA; Montgomery S; Hed MI; Hoven E
Description
An account of the resource
BACKGROUND: Due to psychological distress and an increased care burden, parents of children diagnosed with cancer may face a higher risk of sickness absence from work. The objective of this study was to examine the association of childhood cancer with parents' sick leave. MATERIAL AND METHODS: The sample comprised 3626 parents of 1899 children diagnosed with cancer in Sweden during 2004-2009, and a matched control group of parents (n = 34 874). Sick leave was measured as number of days with sickness benefit, retrieved from national registries. Logistic and negative binomial regression models were used to compare outcomes with parents from the control cohort. RESULTS: The risk of sick leave was statistically significantly higher up to six years following a child's cancer diagnosis. The increase in number of days with sickness benefit was most pronounced the year after diagnosis. Although mothers' sick leave prevalence was higher, the increase in risk relative to control parents was similar for mothers and fathers. Bereavement was associated with a heightened risk of sick leave, especially on the year of the child's death. CONCLUSIONS: Findings confirm that mothers and fathers of children diagnosed with cancer are at higher risk of sickness absence from work, with particularly pronounced risk among bereaved parents. Acquisition of further knowledge is warranted regarding possibilities and constraints of parents trying to combine their work life with caring for both their child and themselves.
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/%2010.1080/0284186X.2016.1275780" target="_blank" rel="noreferrer noopener">10.1080/0284186X.2016.1275780</a>
2017
Acta Oncologica
Adolescent
Adult
Child
Child Preschool
Cohort Studies
Female
Follow-up Studies
Hed MI
Hjelmstedt S
Hoven E
Humans
Infant
Infant Newborn
Lindahl NA
Male
Montgomery S
Neoplasms/diagnosis/epidemiology/psychology
Oncology 2018 List
Only Child
Parents/psychology
Prognosis
Registries
Sick Leave
Sick Leave/statistics & numerical data
Sweden/epidemiology
-
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
February 2021 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
February 2021 List
URL Address
<a href="http://doi.org/10.1097/mlr.0000000000001388" target="_blank" rel="noreferrer noopener">http://doi.org/10.1097/mlr.0000000000001388</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
A Tertiary Care-based Complex Care Program: Improving Care for Children With Medical Complexity
Publisher
An entity responsible for making the resource available
Medical Care
Date
A point or period of time associated with an event in the lifecycle of the resource
2020
Subject
The topic of the resource
Infant; Child; Humans; Child Preschool; Infant Newborn; Length of Stay; Caregivers/psychology; Patient Care Team/organization & administration; Patient Acceptance of Health Care/statistics & numerical data; Hospitalization/statistics & numerical data; Emergency Service Hospital/statistics & numerical data; Patient Satisfaction; Quality Improvement/organization & administration; Case Management/organization & administration; Multiple Chronic Conditions/therapy; Tertiary Healthcare/economics/organization & administration
Creator
An entity primarily responsible for making the resource
Hogan AK; Galligan MM; Stack NJ; Leach KF; Aredas BL; English R; Dye M; Rubin D
Description
An account of the resource
OBJECTIVE: Children with medical complexity (CMC) have significant health care costs, but they also experience substantial unmet health care needs, hospitalizations, and medical errors. Their parents often report psychosocial stressors and poor care satisfaction. Complex care programs can improve the care for CMC. At our tertiary care institution, we developed a consultative complex care program to improve the quality and cost of care for CMC and to improve the experience of care for patients and families. METHODS: To address the needs of CMC at our institution, we developed the Compass Care Program, a consultative complex care program across inpatient and outpatient settings. Utilization data [hospital admissions per patient month; length of stay per admission; hospital days per patient month; emergency department (ED) visits per patient month; and institutional charges per patient month] and caregiver satisfaction data (obtained via paper survey at outpatient visits) were tracked over the period of participation in the program and compared preenrollment and postenrollment for program participants. RESULTS: Participants had significant decreases in hospital admissions per patient month, length of stay per admission, hospital days per patient month, and charges per patient month following enrollment (P<0.01) without a tandem increase in readmissions within 7 days of discharge. There was no statistically significant difference in ED visits. Caregiver satisfaction scores improved in all domains. CONCLUSION: Participation in a consultative complex care program can improve utilization patterns and cost of care for CMC, as well as experience of care for patients and families.
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1097/mlr.0000000000001388" target="_blank" rel="noreferrer noopener">10.1097/mlr.0000000000001388</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).
2020
Aredas BL
Caregivers/psychology
Case Management/organization & administration
Child
Child Preschool
Dye M
Emergency Service Hospital/statistics & numerical data
English R
February 2021 List
Galligan MM
Hogan AK
Hospitalization/statistics & numerical data
Humans
Infant
Infant Newborn
Leach KF
Length Of Stay
Medical Care
Multiple Chronic Conditions/therapy
Patient Acceptance Of Health Care/statistics & Numerical Data
Patient Care Team/organization & administration
Patient Satisfaction
Quality Improvement/organization & administration
Rubin D
Stack NJ
Tertiary Healthcare/economics/organization & administration
-
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/10.1111/j.1365-2788.1993.tb00868.x" target="_blank" rel="noreferrer noopener">http://doi.org/10.1111/j.1365-2788.1993.tb00868.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
Development, behaviour and seizures in 300 cases of tuberous sclerosis
Publisher
An entity responsible for making the resource available
Journal of Intellectual Disability Research
Date
A point or period of time associated with an event in the lifecycle of the resource
1993
Subject
The topic of the resource
Middle Aged; Family; Male; Aged; Child; Humans; Adult; Adolescent; Female; Child Preschool; Infant; Psychiatric Status Rating Scales; Aggression; Developmental Disabilities/complications/diagnosis; Learning Disorders/complications; Seizures/complications; Sleep Wake Disorders/complications; Social Behavior Disorders/complications; Tuberous Sclerosis/complications; behavioral problems; sleep disturbance; tuberous sclerosis; trajectory; characteristics; hyperactive behavior
Creator
An entity primarily responsible for making the resource
Hunt A
Description
An account of the resource
Three hundred cases of tuberous sclerosis were surveyed by postal questionnaire, including 240 cases with learning difficulties. People who have tuberous sclerosis and severe learning disabilities also have a higher frequency of epileptic seizures than people with learning difficulties from all causes. Pervasive developmental disorder and hyperactive behaviour are behavioural features of the disorder. Sleep problems and the level of vigilant care required because of seizures or behaviour have implications on the level of service needed to support people with tuberous sclerosis in the community.
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1111/j.1365-2788.1993.tb00868.x" target="_blank" rel="noreferrer noopener">10.1111/j.1365-2788.1993.tb00868.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).
1993
Adolescent
Adult
Aged
Aggression
behavioral problems
characteristics
Child
Child Preschool
Developmental Disabilities/complications/diagnosis
Family
Female
Humans
Hunt A
hyperactive behavior
Infant
Journal Of Intellectual Disability Research
Learning Disorders/complications
Male
Middle Aged
Psychiatric Status Rating Scales
Seizures/complications
sleep disturbance
Sleep Wake Disorders/complications
Social Behavior Disorders/complications
Trajectory
Tuberous Sclerosis
Tuberous Sclerosis/complications
-
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/10.1111/j.1469-8749.1994.tb11819.x" target="_blank" rel="noreferrer noopener">http://doi.org/10.1111/j.1469-8749.1994.tb11819.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
Sleep disorder and epilepsy in children with tuberous sclerosis: a questionnaire-based study
Publisher
An entity responsible for making the resource available
Developmental Medicine and Child Neurology
Date
A point or period of time associated with an event in the lifecycle of the resource
1994
Subject
The topic of the resource
Stress Psychological/etiology; Male; Surveys and Questionnaires; Child; Humans; Adolescent; Female; Child Preschool; Parents/psychology; Developmental Disabilities/complications; Epilepsy/etiology; Learning Disorders/complications; Sleep Wake Disorders/etiology/psychology; Tuberous Sclerosis/complications/psychology; behavioral problems; sleep disturbance; tuberous sclerosis; trajectory; characteristics
Creator
An entity primarily responsible for making the resource
Hunt A; Stores G
Description
An account of the resource
Sleep disorders were investigated in 40 children with tuberous sclerosis (TS) and compared with those found in non-disabled children and those reported in a mixed group with learning disabilities. Significantly higher levels of sleep disturbance were found in the TS group compared with both other groups. Within the TS group, current epilepsy and a high level of daytime behavioural disturbance were significantly associated with sleep disturbance, but pervasive developmental delay and high parental stress levels were not. Detailed study of the relationship between seizure activity and sleep disturbance in tuberous sclerosis is needed.
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1111/j.1469-8749.1994.tb11819.x" target="_blank" rel="noreferrer noopener">10.1111/j.1469-8749.1994.tb11819.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).
1994
Adolescent
behavioral problems
characteristics
Child
Child Preschool
Developmental Disabilities/complications
Developmental Medicine and Child Neurology
Epilepsy/etiology
Female
Humans
Hunt A
Learning Disorders/complications
Male
Parents/psychology
sleep disturbance
Sleep Wake Disorders/etiology/psychology
Stores G
Stress Psychological/etiology
Surveys And Questionnaires
Trajectory
Tuberous Sclerosis
Tuberous Sclerosis/complications/psychology
-
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/10.1352/0895-8017(2002)107" target="_blank" rel="noreferrer noopener">http://doi.org/10.1352/0895-8017(2002)107<0146:SIBSRA>2.0.CO;2</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-injurious behavior, self-restraint, and compulsive behaviors in Cornelia de Lange syndrome
Publisher
An entity responsible for making the resource available
American Journal of Mental Retardation
Date
A point or period of time associated with an event in the lifecycle of the resource
2002
Subject
The topic of the resource
Male; Child; Humans; Adult; Adolescent; Female; Child Preschool; Infant; Compulsive Behavior; De Lange Syndrome/px [Psychology]; Restraint Physical/px [Psychology]; Self-Injurious Behavior/px [Psychology]; behavioral problems; De Lange syndrome; trajectory; characteristics; self-injurious behavior; self-restraint
Creator
An entity primarily responsible for making the resource
Hyman P; Oliver C; Hall S
Description
An account of the resource
Researchers have argued that individuals who show self-restraint are more likely to show self-injurious behavior (SIB) that is compulsive. Self-injurious behavior, self-restraint, and compulsive behaviors have been described as features of Cornelia de Lange syndrome. We examined whether individuals with this syndrome displaying SIB and self-restraint exhibit more compulsive behaviors than do those without SIB and self-restraint. Main caregivers of individuals with the syndrome completed questionnaires. A significant association was found between SIB and self-restraint, and those displaying both behaviors displayed significantly more compulsions than did those not exhibiting them. Findings extend the compulsive behavior theory and highlight areas for further research.
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1352/0895-8017(2002)107" target="_blank" rel="noreferrer noopener">10.1352/0895-8017(2002)107<0146:SIBSRA>2.0.CO;2</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).
2002
Adolescent
Adult
American Journal of Mental Retardation
behavioral problems
characteristics
Child
Child Preschool
Compulsive Behavior
De Lange syndrome
De Lange Syndrome/px [Psychology]
Female
Hall S
Humans
Hyman P
Infant
Male
Oliver C
Restraint Physical/px [Psychology]
Self-Injurious behavior
Self-Injurious Behavior/px [Psychology]
self-restraint
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
2018 Developing World 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
Developing World 2018 List
URL Address
<a href="http://doi.org/10.1089/jpm.2017.0632" target="_blank" rel="noreferrer noopener">http://doi.o
rg/10.1089/jpm.2017.0632</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
End-of-Life Treatments in Pediatric Patients at a Government Tertiary Cancer Center in India
Publisher
An entity responsible for making the resource available
Journal of Palliative Medicine
Date
A point or period of time associated with an event in the lifecycle of the resource
2018
Subject
The topic of the resource
Hospital Mortality; Infant Newborn; Patient Admission; Human; Child; Referral and Consultation; Palliative Care; Child Preschool; Survival; Infant; Hospitals Pediatric; Adolescence; India; Inpatients; Retrospective Design; Record Review; Analgesics Opioid -- Therapeutic Use; Antianxiety Agents Benzodiazepine -- Therapeutic Use; Cancer Care Facilities -- India; Childhood Neoplasms -- Diagnosis; Childhood Neoplasms -- Drug Therapy; Childhood Neoplasms -- Mortality; Childhood Neoplasms -- Prognosis; Delirium -- Symptoms; Dyspnea -- Symptoms; Hematologic Neoplasms -- Diagnosis; Hemorrhage -- Symptoms; Pain -- Symptoms; Seizures -- Symptoms; Terminal Care -- In Infancy and Childhood; Tertiary Health Care
Creator
An entity primarily responsible for making the resource
Jacob J; Matharu Jaskirt K; Palat G; Sinha S; Brun Eva; Wiebe T; Segerlantz M
Identifier
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<a href="http://doi.org/10.1089/jpm.2017.0632" target="_blank" rel="noreferrer noopener">10.1089/jpm.2017.0632</a>
2018
Adolescence
Analgesics Opioid -- Therapeutic Use
Antianxiety Agents Benzodiazepine -- Therapeutic Use
Brun Eva
Cancer Care Facilities -- India
Child
Child Preschool
Childhood Neoplasms -- Diagnosis
Childhood Neoplasms -- Drug Therapy
Childhood Neoplasms -- Mortality
Childhood Neoplasms -- Prognosis
Delirium -- Symptoms
Developing World 2018 List
Dyspnea -- Symptoms
Hematologic Neoplasms -- Diagnosis
Hemorrhage -- Symptoms
Hospital Mortality
Hospitals Pediatric
Human
India
Infant
Infant Newborn
Inpatients
Jacob J
Journal of Palliative Medicine
Matharu Jaskirt K
Pain -- Symptoms
Palat G
Palliative Care
Patient Admission
Record Review
Referral And Consultation
Retrospective Design
Segerlantz M
Seizures -- Symptoms
Sinha S
Survival
Terminal Care -- In Infancy and Childhood
tertiary health care
Wiebe T
-
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/10.1007/s10545-015-9887-8" target="_blank" rel="noreferrer noopener">http://doi.org/10.1007/s10545-015-9887-8</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
Behavioural and emotional problems, intellectual impairment and health-related quality of life in patients with organic acidurias and urea cycle disorders
Publisher
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Journal of Inherited Metabolic Disease
Date
A point or period of time associated with an event in the lifecycle of the resource
2016
Subject
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Male; Humans; Female; Child Preschool; Emotions; Quality of Life; Intellectual Disability/etiology/metabolism; Mental Disorders/etiology/metabolism; Metabolic Diseases/complications/metabolism; Metabolism Inborn Errors/complications/metabolism; Ornithine Carbamoyltransferase Deficiency Disease/complications/metabolism; Urea Cycle Disorders Inborn/complications/metabolism; alertness; behavioral problems; bowel incontinence; feeding difficulties; sleep disturbance; urinary incontinence; trajectory; characteristics; emotional problems
Creator
An entity primarily responsible for making the resource
Jamiolkowski D; Kolker S; Glahn E M; Baric I; Zeman J; Baumgartner M R; Muhlhausen C; Garcia-Cazorla A; Gleich F; Haege G; Burgard P
Description
An account of the resource
BACKGROUND: Organic acidurias (OADs) and urea cycle disorders (UCDs) are inborn metabolic disorders with a risk for acute and chronic metabolic decompensation resulting in impairments of the central nervous system and other organ systems. So far, there is no systematic study of intellectual functioning, behavioural/emotional problems and health-related quality of life (HRQoL), and how these domains are connected. METHODS: Data of 152 patients with OADs (n = 100) and UCDs (n = 52) from the European Registry and Network of intoxication type Metabolic Diseases (E-IMD) using standardized instruments were compared with normative data. RESULTS: Behavioural/emotional problems are increased in OADs or UCDs patients by a factor of 2.5 (3.0), in female asymptomatic carriers of X-linked inherited UCD ornithine transcarbamylase deficiency (fasOTCD) by a factor of 1.5. All groups show similar patterns of behavioural/emotional problems, not different from epidemiological data. Mental disability (IQ </= 70) was found in 31% of OAD, 43% of UCD, but not in fasOTCD subjects. HRQoL was decreased in the physical domain, but in the normal range. Behavioural/emotional problems were significantly associated with intellectual functioning (OR = 6.24, 95%CI: 1.39-27.99), but HRQoL was independent from both variables. CONCLUSIONS: Patients with OADs and UCDs show increased frequencies of mental disability and behavioural/emotional problems. Profiles of behavioural/emotional problems were similar to epidemiological data. Intellectual disability and behavioural/emotional problems were strongly associated. Patients' HRQoL was in the normal range, possibly compensated by coping strategies of their families. Diagnostics and clinical care of OAD/UCD patients should be improved regarding behavioural/emotional, intellectual and quality of life aspects.
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1007/s10545-015-9887-8" target="_blank" rel="noreferrer noopener">10.1007/s10545-015-9887-8</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
alertness
Baric I
Baumgartner M R
behavioral problems
bowel incontinence
Burgard P
characteristics
Child Preschool
emotional problems
Emotions
feeding difficulties
Female
Garcia-Cazorla A
Glahn E M
Gleich F
Haege G
Humans
Intellectual Disability/etiology/metabolism
Jamiolkowski D
Journal Of Inherited Metabolic Disease
Kolker S
Male
Mental Disorders/etiology/metabolism
Metabolic Diseases/complications/metabolism
Metabolism Inborn Errors/complications/metabolism
Muhlhausen C
Ornithine Carbamoyltransferase Deficiency Disease/complications/metabolism
Quality Of Life
sleep disturbance
Trajectory
Urea Cycle Disorders Inborn/complications/metabolism
urinary incontinence
Zeman 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/10.1002/ajmg.a.10236" target="_blank" rel="noreferrer noopener">http://doi.org/10.1002/ajmg.a.10236</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
Further delineation of the behavioral and neurologic features in Costello syndrome
Publisher
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American Journal of Medical Genetics Part A
Date
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2003
Subject
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Male; Child; Humans; Adolescent; Female; Child Preschool; Infant; Abnormalities Multiple/physiopathology/psychology; Intellectual Disability/physiopathology/psychology; behavioral problems; feeding difficulties; Costello syndrome; trajectory; characteristics; irritability; sleep disturbance
Creator
An entity primarily responsible for making the resource
Kawame H; Matsui M; Kurosawa K; Matsuo M; Masuno M; Ohashi H; Fueki N; Aoyama K; Miyatsuka Y; Suzuki K; Akatsuka A; Ochiai Y; Fukushima Y
Description
An account of the resource
To describe clinical and neurodevelopmental phenotypes of Costello syndrome, we performed a retrospective review of the clinical records and findings in 10 children with Costello syndrome. All patients showed significant postnatal growth retardation and severe feeding difficulties leading to failure to thrive from early infancy. All required tube feeding and some needed high-calorie formulas for variable periods. Developmental quotients/IQs in seven children were 50 or less, and three were in the mildly retarded range. Five had seizures. Remarkable manifestations not previously reported were the characteristic behavior in infancy. Although happy and sociable personality was always emphasized in the genetic literature, all children showed significant irritability, including hypersensitivity to sound and tactile stimuli, sleep disturbance, and excess shyness with strangers in infancy. Those symptoms usually disappeared around age 2-4 years. Other clinical signs included cardiac abnormalities (8), musculoskeletal abnormalities (10), ophthalmological manifestations (5), increased urinary vanillymandelic acid (VMA) and homovanillic acid (HVA) (3), rhabdomyosarcoma (1), laryngomalacia (1), and cryptorchidism (1). Only three girls had papillomata. Family histories were negative for Costello syndrome. In conclusion, we confirm the wide spectrum of mental function in patients with Costello syndrome, which ranges from severe to mild. During infancy Costello syndrome showed remarkable irritability with severe feeding problems, which attributes significant difficulties to the parents of affected children.
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1002/ajmg.a.10236" target="_blank" rel="noreferrer noopener">10.1002/ajmg.a.10236</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).
2003
Abnormalities Multiple/physiopathology/psychology
Adolescent
Akatsuka A
American Journal of Medical Genetics Part A
Aoyama K
behavioral problems
characteristics
Child
Child Preschool
costello syndrome
feeding difficulties
Female
Fueki N
Fukushima Y
Humans
Infant
Intellectual Disability/physiopathology/psychology
Irritability
Kawame H
Kurosawa K
Male
Masuno M
Matsui M
Matsuo M
Miyatsuka Y
Ochiai Y
Ohashi H
sleep disturbance
Suzuki K
Trajectory