1
40
57
-
Dublin Core
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Title
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August 2023 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
August List 2023
URL Address
<a href="http://doi.org/10.1089/pmr.2021.0005" target="_blank" rel="noreferrer noopener"> http://doi.org/10.1089/pmr.2021.0005</a>
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Title
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Care Provider Behaviors That Shape Parent Identity as a "Good Parent" to Their Seriously Ill Child
Publisher
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Palliative Medicine Reports
Date
A point or period of time associated with an event in the lifecycle of the resource
2021
Subject
The topic of the resource
child; United States; article; female; hospital admission; human; male; priority journal; quality of life; palliative therapy; hospice; Europe; parent; interpersonal communication; legal guardian; patient comfort; physician; child parent relation; demographics; care behavior; health belief; voice; nurse; medical decision making; encouragement; reassurance
Creator
An entity primarily responsible for making the resource
Neumann ML; Weaver MS; Lord B; Wiener L; Hinds PS
Description
An account of the resource
Full text linksCite Abstract Background: Parents of medically complex children hold deeply personal definitions of how to be "good parents" that guide their medical decision making and interactions with providers and are impacted by provider behaviors. Objective: This study explored whether and how these beliefs are shaped by interactions with care providers and which provider behaviors foster or impede parents' ability to achieve their "good parent" definitions. Methods: A 63-item web-based survey distributed by an online support network for parents of medically complex children. Responses to closed- and open-ended questions from 67 caregivers based in the United States and Europe were analyzed. Results: Respondents' medical decisions are driven by goals of unselfishly doing what is best for my child (61%) and being my child's voice (18%). Almost half indicated that their personal "good parent" definition was impacted by provider behaviors or interactions with physicians or nurses. Although most parents reported wanting trusted care providers to ask them about their personal "good parent" definition, only 7% had ever been directly asked by members of their care teams about this topic. Provider behaviors such as kind and caring interactions, acknowledging the parents' role in caring for the child, and truly seeing the child as more than a diagnosis were reported as fostering caregivers' ability to achieve their "good parent" beliefs. Conclusions: The findings indicate that trusted provider-initiated conversations about "good parent" beliefs would be well received and are an opportunity to improve family-centered care. Care provider behaviors deemed by parents as supportive facilitate their efforts to achieve their "good parent" beliefs.
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1089/pmr.2021.0005" target="_blank" rel="noreferrer noopener">10.1089/pmr.2021.0005</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).
2021
Article
August List 2041
care behavior
Child
Child Parent Relation
Demographics
encouragement
Europe
Female
Health Belief
Hinds PS
Hospice
Hospital Admission
Human
Interpersonal Communication
legal guardian
Lord B
Male
Medical Decision Making
Neumann ML
Nurse
Palliative Medicine Reports
Palliative Therapy
Parent
Patient Comfort
Physician
Priority Journal
Quality Of Life
reassurance
United States
Voice
Weaver MS
Wiener L
-
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
2020 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 2020 List
URL Address
<a href="http://doi.org/10.1002/pbc.27993" target="_blank" rel="noreferrer noopener">http://doi.org/10.1002/pbc.27993</a>
Dublin Core
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Title
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Helping parents prepare for their child's end of life: A retrospective survey of cancer-bereaved parents
Publisher
An entity responsible for making the resource available
Pediatric Blood and Cancer
Date
A point or period of time associated with an event in the lifecycle of the resource
2020
Subject
The topic of the resource
newborn; social support; child; human; adult; female; major clinical study; male; retrospective study; article; priority journal; aged; Bereavement; adolescent; terminal care; quality of life; emotion; Parent; psychologist; social worker; content analysis; interpersonal communication; quantitative analysis; time of death; infant; caregiver; pediatric patient; nurse; support group; thematic analysis; psycho-oncology; open ended questionnaire; childhood cancer/dm [Disease Management]; pediatric oncologist; health care need; social media; worker
Creator
An entity primarily responsible for making the resource
Wiener L; Tager J; Mack J; Battles H; Bedoya S Z; Gerhardt C A
Description
An account of the resource
Background: Most parents vividly recall the weeks, days, and moments preceding their child's death for years to come. Dissatisfaction with communication about their child's condition and lack of guidance can contribute to stress prior to a child's death. Based on findings from a study assessing the degree of preparation bereaved parents received and our collective clinical experience, the authors provide suggestions on end-of-life communication and guidance for parents.
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1002/pbc.27993" target="_blank" rel="noreferrer noopener">10.1002/pbc.27993</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
Aged
Article
Battles H
Bedoya S Z
Bereavement
Caregiver
Child
childhood cancer/dm [Disease Management]
Content Analysis
Emotion
Female
Gerhardt C A
health care need
Human
Infant
Interpersonal Communication
Mack J
Major Clinical Study
Male
Newborn
Nurse
Oncology 2020 List
open ended questionnaire
Parent
Pediatric Blood and Cancer
pediatric oncologist
pediatric patient
Priority Journal
Psycho-Oncology
Psychologist
Quality Of Life
quantitative analysis
Retrospective Study
social media
Social Support
Social Worker
support group
Tager J
Terminal Care
Thematic Analysis
time of death
Wiener L
worker
-
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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2020 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 2020 List
URL Address
<a href="http://doi.org/10.1002/cncr.32553" target="_blank" rel="noreferrer noopener">http://doi.org/10.1002/cncr.32553</a>
Dublin Core
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Title
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Unrealistic parental expectations for cure in poor-prognosis childhood cancer
Publisher
An entity responsible for making the resource available
Cancer
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; human; patient care; controlled study; female; major clinical study; male; cohort analysis; article; priority journal; childhood cancer; cancer recurrence; quality of life; confidence interval; cancer palliative therapy; interpersonal communication; expectation; clinical decision making; parental behavior; prospective study; cancer prognosis; neuroblastoma; questionnaire; medical record; pediatric patient; cancer risk; theoretical model; high risk population; odds ratio
Creator
An entity primarily responsible for making the resource
Mack J W; Cronin A M; Uno H; Shusterman S; Twist C J; Bagatell R; Rosenberg A; Marachelian A; Granger M M; Glade Bender J; Baker J N; Park J; Cohn S L; Levine A; Taddei S; Diller L R
Description
An account of the resource
Background: Many parents of children with advanced cancer pursue curative goals when cure is no longer possible. To the authors' knowledge, no pediatric studies to date have prospectively evaluated prognosis communication or influences on decision making in poor-prognosis childhood cancer.
Identifier
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<a href="http://doi.org/10.1002/cncr.32553" target="_blank" rel="noreferrer noopener">10.1002/cncr.32553</a>
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Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
2020
Article
Bagatell R
Baker J N
Cancer
Cancer Palliative Therapy
Cancer Prognosis
Cancer Recurrence
cancer risk
Child
Childhood Cancer
Clinical Decision Making
Cohn S L
Cohort Analysis
confidence interval
Controlled Study
Cronin A M
Diller L R
Expectation
Female
Glade Bender J
Granger M M
High Risk Population
Human
Interpersonal Communication
Levine A
Mack J W
Major Clinical Study
Male
Marachelian A
Medical Record
Neuroblastoma
Odds Ratio
Oncology 2020 List
parental behavior
Park J
Patient Care
pediatric patient
Priority Journal
Prospective Study
Quality Of Life
Questionnaire
Rosenberg A
Shusterman S
Taddei S
theoretical model
Twist C J
Uno H
-
Dublin Core
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Title
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2020 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 2020 List
URL Address
<a href="http://doi.org/10.1007/978-3-030-25804-7_15" target="_blank" rel="noreferrer noopener">http://doi.org/10.1007/978-3-030-25804-7_15</a>
Dublin Core
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Title
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Pediatric oncology nursing research in low- and middle-income countries
Publisher
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Pediatric Oncology
Date
A point or period of time associated with an event in the lifecycle of the resource
2020
Subject
The topic of the resource
fatigue; stress; human; palliative therapy; priority journal; cancer survival; health care personnel; pain; quality of life; posttraumatic stress disorder; risk factor; low income country; middle income country; cancer incidence; nutrition; caregiver; sepsis; treatment response; infant mortality; geographic distribution; malnutrition; high income country; methodology; nursing care; pediatric oncology nursing; caregiver burden; gross national income; Burkitt lymphoma; lifestyle; alternative medicine; caregiver burnout; cytotoxicity; economic status; immune status; Impact of Events Scale; Kaposi sarcoma; nurse training; nursing practice; nursing science; Southeast Asian; Southern Europe; traditional medicine
Creator
An entity primarily responsible for making the resource
Challinor J M; Day S W; Afungchwi G M; Alqudimat M R
Description
An account of the resource
Cancer and other non-communicable diseases are a growing public health issue now that infectious disease control (e.g., HIV/AIDS, malaria, and tuberculosis) has made great strides across low- and middle-income countries (L&MIC). The large majority (85%) of children and adolescents with cancer reside in L&MIC where children represent up to 50% of a country's population, and resources are severely limited for the comprehensive cancer care these patients require. Nursing care of these patients and families must be based on research performed in country to account for challenges in access to care and limited resources and opportunities for nursing specialization. Examples of these challenges include cancer stigma, poverty, traditional medicine practices, cultural norms and decision-making hierarchies, limited education opportunities, lack of universal healthcare, and poor transportation infrastructure to access tertiary care. This chapter summarizes the 137 articles in five languages from 2008 to 2018 that communicate nursing research findings pertaining to pediatric oncology issues from L&MIC across all six World Health Organization (WHO) regions. Despite little or no funding, nurses in academic and clinical settings are actively exploring care priorities in their settings, most often (but not limited to) addressing parent coping, nurse and nursing care issues including symptom management (pain and fatigue, especially), and children's quality of life. The nursing research evidence presented here will begin to inform personalized and precision health in L&MIC to ensure that care is culturally acceptable and considers the environment, nursing practice, nursing science, family, lifestyle behaviors, and response to disease and treatment of this large patient population.
Identifier
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<a href="http://doi.org/10.1007/978-3-030-25804-7_15" target="_blank" rel="noreferrer noopener">10.1007/978-3-030-25804-7_15</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
Afungchwi G M
Alqudimat M R
alternative medicine
Burkitt lymphoma
cancer incidence
Cancer Survival
Caregiver
Caregiver Burden
caregiver burnout
Challinor J M
Cytotoxicity
Day S W
Developing World 2020 List
economic status
Fatigue
geographic distribution
gross national income
Health Care Personnel
high income country
Human
immune status
Impact of Events Scale
Infant Mortality
Kaposi sarcoma
lifestyle
low income country
malnutrition
Methodology
middle income country
nurse training
Nursing Care
Nursing Practice
nursing science
Nutrition
Pain
Palliative Therapy
Pediatric Oncology
pediatric oncology nursing
PostTraumatic Stress Disorder
Priority Journal
Quality Of Life
risk factor
Sepsis
Southeast Asian
Southern Europe
Stress
traditional medicine
treatment response
-
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
2019 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 2019 List
URL Address
<a href="http://doi.org/10.1007/s00520-018-4100-x" target="_blank" rel="noreferrer noopener">http://doi.org/10.1007/s00520-018-4100-x</a>
Dublin Core
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Title
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Palliative care in Swiss pediatric oncology settings: a retrospective analysis of medical records
Publisher
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Supportive Care in Cancer
Date
A point or period of time associated with an event in the lifecycle of the resource
2707
Subject
The topic of the resource
adolescent; article; cancer mortality; cancer palliative therapy; central nervous system tumor/th [Therapy]; child; childhood cancer/th [Therapy]; clinical decision making; diagnosis related group; female; human; leukemia/th [Therapy]; major clinical study; male; medical record; priority journal; retrospective study; Switzerland; treatment duration
Creator
An entity primarily responsible for making the resource
Rost M; Acheson E; Kuhne T; Ansari M; Pacurari N; Brazzola P; Niggli F; Elger B S; Wangmo T
Description
An account of the resource
Purpose: This study examined the provision of palliative care and related decision-making in Swiss pediatric oncology settings. The aim was to determine if and when children who died from cancer received palliative care, whether there were differences by cancer diagnosis, and inclusion of children in decision-making regarding palliative care.
Identifier
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<a href="http://doi.org/10.1007/s00520-018-4100-x" target="_blank" rel="noreferrer noopener">10.1007/s00520-018-4100-x</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).
2707
Acheson E
Adolescent
Ansari M
Article
Brazzola P
Cancer Mortality
Cancer Palliative Therapy
central nervous system tumor/th [Therapy]
Child
childhood cancer/th [Therapy]
Clinical Decision Making
diagnosis related group
Elger B S
Female
Human
Kuhne T
leukemia/th [Therapy]
Major Clinical Study
Male
Medical Record
Niggli F
Oncology 2019 List
Pacurari N
Priority Journal
Retrospective Study
Rost M
Supportive Care In Cancer
Switzerland
treatment duration
Wangmo 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
2019 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 2019 List
URL Address
<a href="http://doi.org/10.1016/j.radonc.2019.05.017" target="_blank" rel="noreferrer noopener">http://doi.org/10.1016/j.radonc.2019.05.017</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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Clinical practice and outcomes of palliative radiation therapy in pediatric oncology patients: An international comparison of experiences from two distinct countries and health care systems
Publisher
An entity responsible for making the resource available
Radiotherapy and Oncology
Date
A point or period of time associated with an event in the lifecycle of the resource
2019
Subject
The topic of the resource
anorexia; article; bradycardia; brain metastasis; cancer palliative therapy; cancer radiotherapy; cancer survival; child; childhood cancer/rt [Radiotherapy]; clinical outcome; clinical practice; cohort analysis; follow up; human; leukemia/rt [Radiotherapy]; lymphoma/rt [Radiotherapy]; major clinical study; mucosa inflammation; neuroblastoma/rt [Radiotherapy]; opiate/dt [Drug Therapy]; opiate/pv [Special Situation for Pharmacovigilance]; osteosarcoma/rt [Radiotherapy]; pain/dt [Drug Therapy]; Palliative radiation therapy; Palliative therapy; pediatric patient; Pediatric radiation therapy; preschool child; priority journal; radiation dermatitis; radiation dose fractionation; radiation induced neoplasm; rectum hemorrhage; treatment outcome; treatment response; tumor growth
Creator
An entity primarily responsible for making the resource
Rao A D; Figueiredo M L S; Yegya-Raman N; Sehgal S; Chen Q; Alcorn S R; Chen M J; Ladra M; Villar R; Terezakis S A
Description
An account of the resource
Background and purpose: This study describes clinical outcomes of palliative radiation therapy (RT)for children treated in distinct health-care environments-the US where there is advanced integration of palliative resources and Brazil, a country in the process of developing provisions for pediatric palliative care. Methods and materials: Palliative RT cases of pediatric oncology patients aged <=21-years from 2010 to 2016 in two Brazil-based and one US-based (Johns Hopkins Hospital, JHH)academic centers were reviewed in this study. Result(s): Eighty-eight pediatric patients were treated to 131 lesions with palliative RT. Forty-nine patients from the JHH cohort comprised 84 cases and 39 patients from the Brazil cohort comprised 46 cases. The most common indication for palliative RT was pain (55% overall, 39% Brazil, 63% JHH). Sixty-seven percent of patients experienced a complete (CR)or partial response (PR)to palliative RT, 12% reported stable symptoms (SS), and 22% reported progressive symptoms (PS). The median survival from the end of palliative RT was 3.6 months (95% confidence interval (CI), 2.3-4.8 months). When treated with palliative RT for pain, 83% of patients experience CR/PR, facilitating reduction or discontinuation of opiates in 46% of these patients. Conclusion(s): Despite different practices, the clinical results using palliative RT for pediatric patients treated in two unique healthcare environments demonstrated it is an effective tool for pediatric oncology patients across systems. Copyright © 2019 Elsevier B.V.
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1016/j.radonc.2019.05.017" target="_blank" rel="noreferrer noopener">10.1016/j.radonc.2019.05.017</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).
2019
Alcorn S R
Anorexia
Article
bradycardia
Brain Metastasis
Cancer Palliative Therapy
cancer radiotherapy
Cancer Survival
Chen M J
Chen Q
Child
Childhood Cancer/rt [radiotherapy]
Clinical Outcome
Clinical Practice
Cohort Analysis
Figueiredo M L S
Follow Up
Human
Ladra M
leukemia/rt [Radiotherapy]
lymphoma/rt [Radiotherapy]
Major Clinical Study
mucosa inflammation
neuroblastoma/rt [Radiotherapy]
Oncology 2019 List
Opiate/dt [drug Therapy]
opiate/pv [Special Situation for Pharmacovigilance]
osteosarcoma/rt [Radiotherapy]
Pain/dt [drug Therapy]
Palliative radiation therapy
Palliative Therapy
pediatric patient
Pediatric radiation therapy
Preschool Child
Priority Journal
radiation dermatitis
Radiation Dose Fractionation
radiation induced neoplasm
Radiotherapy and Oncology
Rao A D
rectum hemorrhage
Sehgal S
Terezakis S A
Treatment Outcome
treatment response
tumor growth
Villar R
Yegya-Raman N
-
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
2019 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 2019 List
URL Address
<a href="http://doi.org/10.1016/j.jpedsurg.2018.10.007" target="_blank" rel="noreferrer noopener">http://doi.org/10.1016/j.jpedsurg.2018.10.007</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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Procedural burden experienced by children with cancer during their terminal hospital admission
Publisher
An entity responsible for making the resource available
Journal of Pediatric Surgery
Date
A point or period of time associated with an event in the lifecycle of the resource
2019
Subject
The topic of the resource
acute respiratory failure; adolescent; amputation; article; artificial ventilation; bone marrow biopsy; bone marrow transplantation; brain ventricle peritoneum shunt; cancer palliative therapy; central nervous system tumor; central venous catheter; child; childhood cancer; cohort analysis; craniotomy; debridement; disease burden; excision; fasciotomy; female; heart arrest; heart surgery; hospital admission; human; intestine resection; invasive procedure; laparoscopy; length of stay; leukemia; lobectomy; major clinical study; male; myringotomy; ostomy; pain; priority journal; public health insurance; retrospective study; segmentectomy; sepsis; spine fusion; stem cell transplantation; terminal care; thoracoscopy; thoracotomy; vascular access; ventriculostomy
Creator
An entity primarily responsible for making the resource
Corkum K S; Lautz T B; Hebal F N; Rowell E E
Description
An account of the resource
Background: Children with chronic conditions, including cancer, have been shown to have high-intensity end-of-life care. We assessed the frequency and timing of invasive procedures that children with cancer undergo during their terminal hospital admission (THA).
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1016/j.jpedsurg.2018.10.007" target="_blank" rel="noreferrer noopener">10.1016/j.jpedsurg.2018.10.007</a>
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Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
2019
acute respiratory failure
Adolescent
amputation
Article
Artificial Ventilation
bone marrow biopsy
Bone Marrow Transplantation
brain ventricle peritoneum shunt
Cancer Palliative Therapy
Central Nervous System Tumor
central venous catheter
Child
Childhood Cancer
Cohort Analysis
Corkum K S
craniotomy
debridement
Disease Burden
excision
fasciotomy
Female
Heart Arrest
heart surgery
Hebal F N
Hospital Admission
Human
intestine resection
invasive procedure
Journal Of Pediatric Surgery
laparoscopy
Lautz T B
Length Of Stay
Leukemia
lobectomy
Major Clinical Study
Male
myringotomy
Oncology 2019 List
Ostomy
Pain
Priority Journal
public health insurance
Retrospective Study
Rowell E E
segmentectomy
Sepsis
spine fusion
Stem Cell Transplantation
Terminal Care
thoracoscopy
Thoracotomy
vascular access
ventriculostomy
-
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.c.31497" target="_blank" rel="noreferrer noopener">http://doi.org/10.1002/ajmg.c.31497</a>
Dublin Core
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Title
A name given to the resource
Sleep disorders in Cornelia de Lange syndrome
Publisher
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American Journal of Medical Genetics Part C - Seminars in Medical Genetics
Date
A point or period of time associated with an event in the lifecycle of the resource
2016
Subject
The topic of the resource
adolescent; problem behavior; priority journal; gene mutation; intellectual impairment; human; article; child; female; male; controlled study; adult; clinical article; comorbidity; epilepsy; de Lange syndrome; sleep disorder; body mass; gastroesophageal reflux; gene; HDAC8 gene; histone deacetylase 8; NIPBL gene; RAD21 gene; SMC1A gene; SMC3 gene; sleep disturbance/disorders; trajectory; characteristics
Creator
An entity primarily responsible for making the resource
Zambrelli E;Fossati C;Turner K;Taiana M;Vignoli A;Gervasini C;Russo S;Furia F;Masciadri M;Ajmone P;Kullman G; Canevini M P; Selicorni A
Description
An account of the resource
Cornelia de Lange syndrome (CdLS) is a rare genetic disorder characterized by growth retardation, intellectual disability, limb defects, typical facial dysmorphism, and other systemic involvement. Sleep disturbances have been frequently reported in CdLS, but these have not been completely characterized, and prevalence data are conflicting. The aim of this paper is to characterize and determine the prevalence of sleep disorders in CdLS patients by means of a validated questionnaire. From November 2012 to November 2013, we asked 46 consecutive parents/caregivers of CdLS patients aged more than 3 years old to fill out the sleep disturbances scale for children (SDSC). The subjects were also characterized by the presence of epilepsy, intellectual disability (ID), behavioral problems, CdLS severity score, gastroesophageal reflux disease (GERD), and genetic test results. An abnormal total sleep score was found in 7 patients (15.2%), 26 (56.5%) showed a borderline total score, and 18 (39.1%) had an abnormal score for at least one SDSC factor. In our study sleep disorders were found to be positively associated to presence of epilepsy, GERD, ID, and behavioral disturbances. No correlation was evident with specific mutations of the different genes, BMI, and severity score. Our results confirm that sleep disorders represent a common problem in CdLS, with higher incidence than in the normal population. In these patients sleep disorders seem to be more prevalent in comorbid settings, representing a clinical indicator for different medical and neuropsychiatric disorders. Better knowledge and characterization of typology of sleep disorders in CdLS patients could permit a more specific therapeutic approach. © 2016 Wiley Periodicals, Inc.
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1002/ajmg.c.31497" target="_blank" rel="noreferrer noopener">10.1002/ajmg.c.31497</a>
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Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
2016
Adolescent
Adult
Ajmone P
American Journal of Medical Genetics Part C - Seminars in Medical Genetics
Article
body mass
Canevini M P
characteristics
Child
Clinical Article
Comorbidity
Controlled Study
De Lange syndrome
Epilepsy
Female
Fossati C
Furia F
Gastroesophageal Reflux
gene
gene mutation
Gervasini C
HDAC8 gene
histone deacetylase 8
Human
Intellectual Impairment
Kullman G
Male
Masciadri M
NIPBL gene
Priority Journal
problem behavior
RAD21 gene
Russo S
Selicorni A
sleep disorder
sleep disturbance/disorders
SMC1A gene
SMC3 gene
Taiana M
Trajectory
Turner K
Vignoli A
Zambrelli 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/10.1186/s11689-016-9145-x" target="_blank" rel="noreferrer noopener">http://doi.org/10.1186/s11689-016-9145-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, behavior, and biomarker characterization of Smith-Lemli-Opitz syndrome: An update
Publisher
An entity responsible for making the resource available
Journal of Neurodevelopmental Disorders
Date
A point or period of time associated with an event in the lifecycle of the resource
2016
Subject
The topic of the resource
adolescent; Autism; parent; development; cognition; behavior; Developmental delay; problem behavior; priority journal; case study; interpersonal communication; preschool child; observational study; unclassified drug; childhood; human; article; child; female; male; adult; clinical article; young adult; automutilation; 7 dehydrocholesterol/ec [Endogenous Compound]; 8 dehydrocholesterol/ec [Endogenous Compound]; adaptive behavior; aggression; cerebrospinal fluid level; child rearing; cholesterol/ec [Endogenous Compound]; intelligence quotient; Smith Lemli Opitz syndrome; Smith-Lemli-Opitz syndrome; socialization; Stanford-Binet Intelligence Scale; Sterols; walking; behavior; tone and motor problems; trajectory; characteristics; aggression; development; delayed development
Creator
An entity primarily responsible for making the resource
Thurm A; Tierney E; Farmer C; Albert P; Joseph L; Swedo S; Bianconi S; Bukelis I; Wheeler C; Sarphare G; Lanham D; Wassif C A; Porter F D
Description
An account of the resource
Background: Smith-Lemli-Opitz syndrome (SLOS) is an autosomal recessive inborn error of cholesterol metabolism syndrome with neurocognitive manifestations. SLOS is the result of mutations in the gene encoding the 7-dehydrocholesterol reductase, which results in the elevation of the cholesterol precursor 7-dehydrocholesterol (7-DHC). Previous reports indicate that intellectual disability, behavioral disturbances, and autism symptoms are frequently part of the SLOS behavioral phenotype. In the current study, we characterize the developmental history and current behavior of 33 individuals with SLOS aged 4 to 23 years and report on biomarkers 7-DHC and 8-DHC in relation to cognition and behavior. Methods: This was an observational case series, wherein participants with SLOS underwent extensive behavioral evaluation of cognitive function, adaptive function, autism symptoms, and problem behaviors, in addition to parent report of developmental milestones. Serum and CSF were contemporaneously obtained from the majority of participants. Results: Developmental milestones such as walking, talking, and toileting were uniformly delayed. Overall levels of cognitive and adaptive functioning were low; no participant received adaptive behavior scores in the average range, and the mean level of cognitive functioning in the full sample was in the moderate range of impairment. Aggressive behavior was present in nearly half of participants. Although the majority of participants had elevated scores on the gold standard autism diagnostic instruments, only about half of participants received a clinical diagnosis of autism spectrum disorder. Finally, while CSF cholesterol was not found to correlate with cognitive or adaptive functioning, both serum and CSF 7-DHC and 8-DHC (and their ratios with cholesterol) were moderately and negatively correlated with functioning in this group. Conclusions: A history of developmental delay, followed by intellectual disability, is common in individuals with SLOS. Although autism spectrum disorder appears to be a frequent diagnosis in this population, it is apparent that the low level of functioning observed in SLOS may artificially inflate scores on standard autism assessments. Our findings further support that cholesterol precursors 7-DHC and 8-DHC are important biomarkers of the level of functioning in SLOS, especially regarding cognitive abilities, and thus may be to explore as mediators within the context of treatment trials.
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1186/s11689-016-9145-x" target="_blank" rel="noreferrer noopener">10.1186/s11689-016-9145-x</a>
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Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
2016
7 dehydrocholesterol/ec [Endogenous Compound]
8 dehydrocholesterol/ec [Endogenous Compound]
adaptive behavior
Adolescent
Adult
Aggression
Albert P
Article
Autism
automutilation
Behavior
Bianconi S
Bukelis I
Case Study
cerebrospinal fluid level
characteristics
Child
Child Rearing
childhood
cholesterol/ec [Endogenous Compound]
Clinical Article
Cognition
delayed development
development
Developmental delay
Farmer C
Female
Human
Intelligence Quotient
Interpersonal Communication
Joseph L
Journal of Neurodevelopmental Disorders
Lanham D
Male
Observational Study
Parent
Porter F D
Preschool Child
Priority Journal
problem behavior
Sarphare G
Smith Lemli Opitz syndrome
Smith-Lemli-Opitz syndrome
Socialization
Stanford-Binet Intelligence Scale
Sterols
Swedo S
Thurm A
Tierney E
tone and motor problems
Trajectory
Unclassified Drug
Walking
Wassif C A
Wheeler C
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/j.ijporl.2014.06.027" target="_blank" rel="noreferrer noopener">http://doi.org/10.1016/j.ijporl.2014.06.027</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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Comparison of treatment modalities in syndromic children with Obstructive Sleep Apnea-A randomized cohort study
Publisher
An entity responsible for making the resource available
International Journal of Pediatric Otorhinolaryngology
Date
A point or period of time associated with an event in the lifecycle of the resource
2014
Subject
The topic of the resource
quality of life; major clinical study; priority journal; follow up; randomized controlled trial; mucopolysaccharidosis; outcome assessment; clinical effectiveness; human; article; child; female; male; controlled study; adenotonsillectomy; apnea hypopnea index; Continuous Positive Airway Pressure; CPAP device; Down syndrome; Epworth sleepiness scale; ess-c; Mucopolysaccharidoses; osa-18; Pediatric obstructive sleep apnea; positive end expiratory pressure; radiofrequency ablation device; sleep disordered breathing/su [Surgery]; sleep disordered breathing/th [Therapy]; treatment outcome; breathing difficulties; MPSI; MPSII; MPSIII; MPSIIIA; MPSIIIB; MPSVI; MPSVII; surgical interventions; physical interventions; adenotonsillectomy
Creator
An entity primarily responsible for making the resource
Sudarsan S S; Paramasivan V K; Arumugam S V; Murali S; Kameswaran M
Description
An account of the resource
Introduction: Obstructive Sleep Apnea (OSA) is a common medical problem in adults that is becoming increasingly recognized in children. It occurs in the pediatric age group, from newborns to teens. More recently, many specialists have estimated OSA prevalence to be between 5 and 6%. However, in syndromic children, the prevalence of OSA can be from 50 to 100%, having a significant effect on their Quality-of-Life. As they are a challenging population for management, it is essential to evaluate them thoroughly before planning appropriate intervention. Objective: To compare the efficacy of Adenotonsillectomy (T&A) and Continuous Positive Airway Pressure (CPAP) in syndromic children [Down syndrome (DS) and Mucopolysaccharidoses (MPS)] with Obstructive Sleep Apnea (OSA). Materials and methods: In a prospective, randomized, cohort comparative study, 124 syndromic children (DS and MPS) aged between 6 and 12 years were recruited from a private MPS support group and the Down Syndrome Society, Chennai. A standard assessment was performed on all children who entered the study including a full overnight Polysomnogram (PSG), Epworth Sleepiness Scale-Children (ESS-C) and Quality-of-Life (QOL) tool OSA-18. The children with positive PSG who consented for the study (n= 80) were randomly distributed to two groups, T&A group & CPAP group. The children were followed up with repeat PSG, clinical evaluation, ESS-C and Quality-of-Life (QOL) tool OSA-18 for a period of 1 year. Observation and results: Follow-up was available for 73 syndromic children. Both the groups, T&A group and CPAP group, showed statistically significant (p<. 0.05) improvement in Apnea-Hypoapnea Index (AHI), ESS-C, QOL from the intervention. In our study, T&A showed equal outcome compared to CPAP. The contrasting feature between the two groups was that CPAP use gave immediate sustained improvement while T&A gave gradual progressive improvement of symptoms over a period of 1 year. Conclusion: On average, T&A gives equal outcomes as CPAP and it can be suggested as a first-line treatment in this group of syndromic children. © 2014.
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1016/j.ijporl.2014.06.027" target="_blank" rel="noreferrer noopener">10.1016/j.ijporl.2014.06.027</a>
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Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
2014
adenotonsillectomy
apnea hypopnea index
Article
Arumugam S V
breathing difficulties
Child
Clinical Effectiveness
Continuous Positive Airway Pressure
Controlled Study
CPAP device
Down Syndrome
Epworth sleepiness scale
ess-c
Female
Follow Up
Human
International Journal of Pediatric Otorhinolaryngology
Kameswaran M
Major Clinical Study
Male
MPSI
MPSII
MPSIII
MPSIIIA
MPSIIIB
MPSVI
MPSVII
Mucopolysaccharidoses
Mucopolysaccharidosis
Murali S
osa-18
outcome assessment
Paramasivan V K
Pediatric obstructive sleep apnea
physical interventions
positive end expiratory pressure
Priority Journal
Quality Of Life
radiofrequency ablation device
Randomized Controlled Trial
sleep disordered breathing/su [Surgery]
sleep disordered breathing/th [Therapy]
Sudarsan S S
surgical interventions
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
Treatment of Symptoms in Children with Q3 Conditions Scoping Review Results
Text
A resource consisting primarily of words for reading. Examples include books, letters, dissertations, poems, newspapers, articles, archives of mailing lists. Note that facsimiles or images of texts are still of the genre Text.
URL Address
<a href="http://doi.org/10.1016/j.rasd.2013.07.010" target="_blank" rel="noreferrer noopener">http://doi.org/10.1016/j.rasd.2013.07.010</a>
Dublin Core
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Title
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Promoting adaptive behaviors by two girls with Rett syndrome through a microswitch-based program
Publisher
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Research in Autism Spectrum Disorders
Date
A point or period of time associated with an event in the lifecycle of the resource
2013
Subject
The topic of the resource
Assistive technology; Indices of happiness; Locomotor behavior; Microswitches; Rett syndrome; adaptive behavior; adolescent; article; case report; child behavior; female; hand washing; happiness; human; locomotion; priority journal; school child; stereotypy; tone and motor problems; Rett syndrome; physical intervention; Wobble microswitch; optic sensors; stereotyped movements
Creator
An entity primarily responsible for making the resource
Stasolla F; Caffo A O
Description
An account of the resource
We assessed a microswitch-based program to improve self-determination to access to preferred stimuli and to foster locomotor behavior by two girls with Rett syndrome and multiple disabilities. To enhance the first behavior (access to preferred stimuli) a wobble microswitch (sensitive touch sensor) was used while for the second behavior (step responses) optic sensors were applied. A second aim of the study was to monitor indices of happiness as consequence of the use of assistive technology. Finally, a third objective of the study was the reduction of hand washing and body rocking related stereotypies. The study was carried out according to a multiple probe design across behaviors for both participants, where the two behaviors were first learned independently, then combined together. Results showed an increasing of performance and of indices of happiness and a decreasing of stereotyped behaviors for both participants during intervention phases. Practical, psychological and clinical implications of the findings are discussed. © 2013 Elsevier Ltd. All rights reserved.
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1016/j.rasd.2013.07.010" target="_blank" rel="noreferrer noopener">10.1016/j.rasd.2013.07.010</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).
2013
adaptive behavior
Adolescent
Article
Assistive technology
Caffo A O
Case Report
Child Behavior
Female
hand washing
Happiness
Human
Indices of happiness
locomotion
Locomotor behavior
Microswitches
optic sensors
physical intervention
Priority Journal
Research In Autism Spectrum Disorders
Rett syndrome
School Child
Stasolla F
stereotyped movements
stereotypy
tone and motor problems
Wobble microswitch
-
Dublin Core
The Dublin Core metadata element set is common to all Omeka records, including items, files, and collections. For more information see, http://dublincore.org/documents/dces/.
Title
A name given to the resource
Treatment of Symptoms in Children with Q3 Conditions Scoping Review Results
Text
A resource consisting primarily of words for reading. Examples include books, letters, dissertations, poems, newspapers, articles, archives of mailing lists. Note that facsimiles or images of texts are still of the genre Text.
URL Address
<a href="http://doi.org/10.1002/ajmg.a.36573" target="_blank" rel="noreferrer noopener">http://doi.org/10.1002/ajmg.a.36573</a>
Dublin Core
The Dublin Core metadata element set is common to all Omeka records, including items, files, and collections. For more information see, http://dublincore.org/documents/dces/.
Title
A name given to the resource
Autism traits in children and adolescents with Cornelia de Lange syndrome
Publisher
An entity responsible for making the resource available
American Journal of Medical Genetics Part A
Date
A point or period of time associated with an event in the lifecycle of the resource
2014
Subject
The topic of the resource
Communication; adolescent; Autism; emotion; age; verbal communication; communication disorder; priority journal; interpersonal communication; skill; intellectual impairment; human; article; child; female; male; adult; diagnosis; clinical article; daily life activity; disease severity; autism; Cornelia de Lange syndrome; phenotype; compulsion; language; stereotypy; adaptive behavior; socialization; Aberrant Behavior Checklist; behavior disorder; Behavioral phenotype; checklist; Childhood Autism Rating Scale; de Lange syndrome; hyperactivity; lethargy; limb; maladjustment; psychological rating scale; rating scale; Repetitive behaviors; rigidity; social cognition; Social cognition; Vineland Adaptive Behaviors Scales; behavioral problems; De Lange syndrome; trajectory; characteristics; irritability
Creator
An entity primarily responsible for making the resource
Srivastava S; Landy-Schmitt C; Clark B; Kline A D; Specht M; Grados M A
Description
An account of the resource
Cornelia de Lange syndrome (CdLS) is a cohesinopathy causing delayed growth and limb deficits. Individuals with CdLS have mild to profound intellectual disability and autistic features. This study characterizes the behavioral phenotype of children with CdLS, focusing on autistic features, maladaptive behaviors, and impact of age. Children with CdLS (5-18 years) were administered normed instruments to characterize autism features (Childhood Autism Rating Scale, CARS), maladaptive behaviors (Aberrant Behavior Checklist), and adaptive skills (Vineland Adaptive Behaviors Scales). CdLS features and severity were rated with Diagnostic Criteria for CdLS. Forty-one children with CdLS (23 females, 18 males) were classified as having "no autism" (n=7; 17.1%), "mild autism" (n=17; 41.4%), and "severe autism" (n=17; 41.4%), using CARS scores. Characteristic items were abnormal emotional response, stereotypies, odd object use, rigidity, lack of verbal communication, and low intellectual functioning. Verbal communication deficits and repetitive behaviors were higher compared to sensory, social cognition, and behavior abnormalities (P<0.0001). Maladaptive behaviors associated with autism traits were stereotypies (P=0.003), hyperactivity (P=0.01), and lethargy (P=0.03). Activities of daily living were significantly affected; socialization adaptive skills were a relative strength. However, with advancing age, both socialization (P<0.0001) and communication (P=0.001) domains declined significantly. CdLS is characterized by autistic features, notably excessive repetitive behaviors and expressive language deficits. While other adaptive skills are impacted, socialization adaptive skills are less affected. Advancing age can worsen communication and socialization deficits relative to neurotypical peers. © 2014 Wiley Periodicals, Inc.
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1002/ajmg.a.36573" target="_blank" rel="noreferrer noopener">10.1002/ajmg.a.36573</a>
Rights
Information about rights held in and over the resource
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
2014
Aberrant Behavior Checklist
adaptive behavior
Adolescent
Adult
Age
American Journal of Medical Genetics Part A
Article
Autism
behavior disorder
Behavioral phenotype
behavioral problems
characteristics
Checklist
Child
Childhood Autism Rating Scale
Clark B
Clinical Article
Communication
communication disorder
compulsion
Cornelia de Lange syndrome
daily life activity
De Lange syndrome
Diagnosis
Disease Severity
Emotion
Female
Grados M A
Human
hyperactivity
Intellectual Impairment
Interpersonal Communication
Irritability
Kline A D
Landy-Schmitt C
Language
lethargy
limb
maladjustment
Male
Phenotype
Priority Journal
psychological rating scale
Rating Scale
Repetitive behaviors
rigidity
Skill
social cognition
Socialization
Specht M
Srivastava S
stereotypy
Trajectory
Verbal Communication
Vineland Adaptive Behaviors Scales
-
Dublin Core
The Dublin Core metadata element set is common to all Omeka records, including items, files, and collections. For more information see, http://dublincore.org/documents/dces/.
Title
A name given to the resource
Treatment of Symptoms in Children with Q3 Conditions Scoping Review Results
Text
A resource consisting primarily of words for reading. Examples include books, letters, dissertations, poems, newspapers, articles, archives of mailing lists. Note that facsimiles or images of texts are still of the genre Text.
URL Address
<a href="http://doi.org/10.1016/j.ijporl.2015.11.009" target="_blank" rel="noreferrer noopener">http://doi.org/10.1016/j.ijporl.2015.11.009</a>
Dublin Core
The Dublin Core metadata element set is common to all Omeka records, including items, files, and collections. For more information see, http://dublincore.org/documents/dces/.
Title
A name given to the resource
Otorhinolaryngological, Audiovestibular and swallowing manifestations of patients with Niemann-Pick disease Type C
Publisher
An entity responsible for making the resource available
International Journal of Pediatric Otorhinolaryngology
Date
A point or period of time associated with an event in the lifecycle of the resource
2016
Subject
The topic of the resource
adolescent; hearing; dysphagia; priority journal; school child; preschool child; prospective study; human; article; child; female; male; controlled study; clinical article; auditory response; Balance disorders; body equilibrium; endoscopy; Flexible endoscopy; hearing impairment; Hearing problems; Niemann Pick disease/di [Diagnosis]; Niemann-Pick disease; otorhinolaryngology; pure tone audiometry; stabilography; swallowing; Swallowing disorders; vestibular system; feeding difficulties; tone and motor problems; NPC; penetration; aspiration scale; trajectory; characteristics; postural imbalance; dysphagia
Creator
An entity primarily responsible for making the resource
Senirli R T; Kuscu O; Akyol U; Topcu M; Yigit O; Aksoy S; Demir N
Description
An account of the resource
Objectives: The aim of this study is to evaluate audiovestibular and swallowing impairment of patients with NPC. Methods: Audiovestibular and swallowing evaluation were performed on patients with Niemann-Pick disease type C (NPC) at Hacettepe University between 20013 and 2015 prospectively. Pure-tone audiometry (PTA), Auditory Brain stem response (ABR), Flexible endoscopic evaluation of swallowing (FEES) test and posturography were done. Hearing, swallowing and balance states were measured. Results: There were 16 patients (5 male and 11 female, with a median age of 6.5 years old). The most common ABR abnormalities observed were absent waves I and III (%70 absent I waves, %43.75 absent III waves). Twelve of sixteen patients (%75) had an ABR abnormality in at least one ear, of these, four patients had normal hearing and three of them had periferal hearing loss. 12 (75%) patients had complaint of postural imbalance. 11(69%) of patients had peripheral and one (6%) patient had central impairment. Nine of sixteen patients (56.25%) show some degree of dysphagia (either penetration or aspiration). Two patients (12.5%) showed aspiration both liquid and viscous nutrition. Three patients (18.75%) showed aspiration primarily in liquids and two of them had penetration with viscous nutrition. Three patients (18.75%) had penetration with no aspiration neither liquid nor viscous nutrition (PEN-ASP score was 3, 3, 5, respectively). Conclusion: There is no curative treatment for this devastating and fatal disorder and hearing impairment, balance and swallowing disorders can be seen especially late onset form of disease.
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1016/j.ijporl.2015.11.009" target="_blank" rel="noreferrer noopener">10.1016/j.ijporl.2015.11.009</a>
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Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
2016
Adolescent
Aksoy S
Akyol U
Article
aspiration scale
auditory response
Balance disorders
body equilibrium
characteristics
Child
Clinical Article
Controlled Study
Demir N
Dysphagia
Endoscopy
feeding difficulties
Female
Flexible endoscopy
hearing
hearing impairment
Hearing problems
Human
International Journal of Pediatric Otorhinolaryngology
Kuscu O
Male
Niemann Pick disease/di [Diagnosis]
Niemann-Pick Disease
NPC
Otorhinolaryngology
penetration
postural imbalance
Preschool Child
Priority Journal
Prospective Study
pure tone audiometry
School Child
Senirli R T
stabilography
Swallowing
Swallowing disorders
tone and motor problems
Topcu M
Trajectory
vestibular system
Yigit O
-
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.1365-2869.1995.tb00216.x" target="_blank" rel="noreferrer noopener">http://doi.org/10.1111/j.1365-2869.1995.tb00216.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
Polysomnographic recordings of respiratory disturbances in Rett syndrome
Publisher
An entity responsible for making the resource available
Journal of Sleep Research
Date
A point or period of time associated with an event in the lifecycle of the resource
1995
Subject
The topic of the resource
adolescent; respiratory tract disease; priority journal; school child; human; female; case report; conference paper; Disorder of respiratory control; Hyperventilation; Polygraphic recording; polysomnography; Rett syndrome; Rett syndrome/di [Diagnosis]; breathing difficulties; trajectory; characteristics; hypocapnia; apnea
Creator
An entity primarily responsible for making the resource
Schluter B; Aguigah G; Buschatz D; Trowitzsch E; Aksu F
Description
An account of the resource
Polygraphic recordings in the awake state and during sleep were performed in two girls with Rett syndrome, aged 9 y and 17 y, in order to characterize the clinically manifest breathing disorder of these patients. Primary hyperventilation was detected, leading to hypocapnia and compensatory apnoea. Hypoxaemia occurred as the consequence of prolonged apnoea. In the younger patient these disturbances were observed only in the awake state, whereas in the older patient they were found in the awake state and during sleep.
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1111/j.1365-2869.1995.tb00216.x" target="_blank" rel="noreferrer noopener">10.1111/j.1365-2869.1995.tb00216.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).
1995
Adolescent
Aguigah G
Aksu F
apnea
breathing difficulties
Buschatz D
Case Report
characteristics
Conference Paper
Disorder of respiratory control
Female
Human
hyperventilation
hypocapnia
Journal of Sleep Research
Polygraphic recording
Polysomnography
Priority Journal
Respiratory Tract Disease
Rett syndrome
Rett Syndrome/di [Diagnosis]
Schluter B
School Child
Trajectory
Trowitzsch 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/10.1177/0883073817741054" target="_blank" rel="noreferrer noopener">http://doi.org/10.1177/0883073817741054</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
Gait, Balance, and Coordination Impairments in Niemann Pick Disease, Type C1
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
2018
Subject
The topic of the resource
adolescent; retrospective study; priority journal; scoring system; school child; outcome assessment; preschool child; intervention study; human; article; child; female; male; adult; clinical article; young adult; comparative study; body equilibrium; stabilography; performance; neurologic examination; balance impairment; coordination disorder; gait disorder; Niemann Pick disease; 2 hydroxypropyl beta cyclodextrin; 94035-02-6 (2 hydroxypropyl beta cyclodextrin); case control study; clinical evaluation; disease severity assessment; human development; molecular stability; motion analysis system; NIH NPC Neurologic Severity Scale; research; spatiotemporal analysis; upper limb; vts 270; tone and motor problems; NPC; trajectory; characteristics
Creator
An entity primarily responsible for making the resource
Sansare A; Zampieri C; Alter K; Stanley C; Farhat N; Keener L A; Porter F
Description
An account of the resource
This is the first study to objectively measure gait, balance, and upper limb coordination in a group of patients with NPC1 and compare the results to age and gender matched controls. This is also the first study to report effect sizes in these measures. Spatiotemporal gait analysis, static and dynamic posturography, and upper limb reaching motion analysis were performed. The findings showed that the NPC1 subjects had statistically significant deficits on 12 out of the 16 parameters investigated compared to controls, and large effect sizes for all but 1 parameter. When ranking the variables in terms of the effect sizes, the top 5 included at least 1 parameter from each of the 3 motor domains investigated. These results can provide insight to clinical researchers on the selection of outcome measures for longitudinal and interventional studies.
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1177/0883073817741054" target="_blank" rel="noreferrer noopener">10.1177/0883073817741054</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).
2 hydroxypropyl beta cyclodextrin
2018
94035-02-6 (2 hydroxypropyl beta cyclodextrin)
Adolescent
Adult
Alter K
Article
balance impairment
body equilibrium
case control study
characteristics
Child
Clinical Article
clinical evaluation
Comparative Study
coordination disorder
disease severity assessment
Farhat N
Female
gait disorder
Human
Human Development
intervention study
Journal of Child Neurology
Keener L A
Male
molecular stability
motion analysis system
Neurologic Examination
Niemann Pick disease
NIH NPC Neurologic Severity Scale
NPC
outcome assessment
performance
Porter F
Preschool Child
Priority Journal
Research
Retrospective Study
Sansare A
School Child
scoring system
spatiotemporal analysis
stabilography
Stanley C
tone and motor problems
Trajectory
Upper limb
vts 270
Young Adult
Zampieri C
-
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/10.1177/0883073812450750" target="_blank" rel="noreferrer noopener">http://doi.org/10.1177/0883073812450750</a>
Dublin Core
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Title
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Fatigue and depression in children with demyelinating disorders
Publisher
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Journal of Child Neurology
Date
A point or period of time associated with an event in the lifecycle of the resource
2013
Subject
The topic of the resource
adolescent; Canada; epidemiology; child behavior; disease duration; priority journal; school child; health survey; self report; cohort analysis; cross-sectional study; psychological aspect; human; article; child; female; male; controlled study; clinical article; comorbidity; childhood disease; mood; sleep; therapy; acute disseminated encephalomyelitis; demyelinating disease; depression; fatigue; multiple sclerosis; acute disseminated encephalomyelitis; demyelinating disorders; multiple sclerosis; acquired demyelinating syndromes; trajectory; characteristics; alertness
Creator
An entity primarily responsible for making the resource
Parrish J B;Weinstock-Guttman B;Smerbeck A;Benedict R H B;Yeh E A
Description
An account of the resource
Fatigue and depression have been shown to be significant problems in children with multiple sclerosis. The rate at which these conditions occur in children with other acquired demyelinating syndromes is unknown. In this cross-sectional study, the authors evaluated 49 children with demyelinating disorders (multiple sclerosis and acute disseminated encephalomyelitis) and 92 healthy controls for depression and/or fatigue using the Behavior Assessment System for Children, Second Edition behavior and mood rating scale and Varni PedsQL Multidimensional Fatigue Scale. The parents of acquired demyelinating syndrome patients were more likely to report elevated depressive symptoms (30.8% vs 10.8%, P =.008). Elevated parent and self-reported total fatigue (25% vs 0%, P <.001, 26.7% vs 8.6%, P =.024) was seen in the patient cohort. The authors conclude that fatigue and depression are far more common in children with acquired demyelinating syndromes than in controls. Clinical attention to and implementation of effective therapies oriented toward these conditions in children with acquired demyelinating syndromes is needed. © The Author(s) 2012.
Identifier
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<a href="http://doi.org/10.1177/0883073812450750" target="_blank" rel="noreferrer noopener">10.1177/0883073812450750</a>
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Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
2013
acquired demyelinating syndromes
acute disseminated encephalomyelitis
Adolescent
alertness
Article
Benedict R H B
Canada
characteristics
Child
Child Behavior
Childhood Disease
Clinical Article
Cohort Analysis
Comorbidity
Controlled Study
Cross-sectional Study
demyelinating disease
demyelinating disorders
Depression
disease duration
Epidemiology
Fatigue
Female
Health Survey
Human
Journal of Child Neurology
Male
Mood
Multiple Sclerosis
Parrish J B
Priority Journal
psychological aspect
School Child
Self Report
Sleep
Smerbeck A
Therapy
Trajectory
Weinstock-Guttman B
Yeh E 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
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/10.2217/nmt-2017-0019" target="_blank" rel="noreferrer noopener">http://doi.org/10.2217/nmt-2017-0019</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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A survey-based study identifies common but unrecognized symptoms in a large series of juvenile Huntington's disease
Publisher
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Neurodegenerative Disease Management
Date
A point or period of time associated with an event in the lifecycle of the resource
2017
Subject
The topic of the resource
adolescent; priority journal; health survey; human; pain; article; child; female; male; controlled study; adult; clinical article; sleep disorder; Huntington chorea; pruritus; psychosis; tic; auditory hallucination; CAG repeat; juvenile; leg pain; skin irritation; visual hallucination; sleep disturbance/disorders; Juvenile Huntington disease; trajectory; characteristics
Creator
An entity primarily responsible for making the resource
Moser A D; Epping E; Espe-Pfeifer P; Martin E; Zhorne L; Mathews K; Nance M; Hudgell D; Quarrell O; Nopoulos P
Description
An account of the resource
Aim: The symptoms of Huntington's disease are well known, yet the symptoms of juvenile Huntington's disease (JHD) are less established due to its rarity. The study examined a cluster of symptoms considered to be common, but under-recognized in JHD: pain, itching, sleeping difficulties, psychosis and tics. Materials & methods: A symptom survey was constructed using the online tool Qualtrics and dispersed to JHD caregivers through websites. Results: A total of 33 surveys were completed. Disrupted sleep was the most prevalent symptom (87%), followed by tics (78%), pain (69%), itching (60%) and psychosis (39%). Conclusion: Despite limitations, the study supports that there are symptoms in the JHD population that are not considered classic, however, are common and significant for patients and caregivers.
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.2217/nmt-2017-0019" target="_blank" rel="noreferrer noopener">10.2217/nmt-2017-0019</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).
2017
Adolescent
Adult
Article
auditory hallucination
CAG repeat
characteristics
Child
Clinical Article
Controlled Study
Epping E
Espe-Pfeifer P
Female
Health Survey
Hudgell D
Human
Huntington chorea
juvenile
Juvenile Huntington disease
leg pain
Male
Martin E
Mathews K
Moser A D
Nance M
Neurodegenerative Disease Management
Nopoulos P
Pain
Priority Journal
pruritus
psychosis
Quarrell O
skin irritation
sleep disorder
sleep disturbance/disorders
tic
Trajectory
visual hallucination
Zhorne L
-
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/10.1097/MPG.0000000000001543" target="_blank" rel="noreferrer noopener">http://doi.org/10.1097/MPG.0000000000001543</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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Feed-induced Dystonias in Children with Severe Central Nervous System Disorders
Publisher
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Journal of Pediatric Gastroenterology and Nutrition
Date
A point or period of time associated with an event in the lifecycle of the resource
2017
Subject
The topic of the resource
infant; adolescent; cerebral palsy; percutaneous endoscopic gastrostomy; retrospective study; priority journal; practice guideline; case study; school child; preschool child; intestine obstruction; human; article; child; female; male; clinical article; Rett syndrome; gastroesophageal reflux; infantile spasm; intestinal dysmotility; baclofen/dt [Drug Therapy]; dystonia/dt [Drug Therapy]; motor dysfunction; onset age; central nervous system disease; dystonia/dt [Drug Therapy]; feed induced dystonia; 1134-47-0 (baclofen); acquired brain injury; baclofen/tl [Intrathecal Drug Administration]; feed induced dystonia/dt [Drug Therapy]; gastroscopy; hydrocephalus; hyperglycinemia; intestine motility; microcephaly; peristalsis; reflux esophagitis; Sanfilippo syndrome; total parenteral nutrition; constipation; feeding difficulties; tone and motor problems; MPSIII; West syndrome; pharmacologic intervention; parenteral nutrition; dystonia; GERD; intestinal dysmotility
Creator
An entity primarily responsible for making the resource
Mordekar S R; Velayudhan M; Campbell D I
Description
An account of the resource
Dystonias can arise from any painful stimuli in neurologically disabled children. Classically, feed-induced dystonias from mediastinal pain due to severe gastroesophageal reflux disease are described as Sandifer spasm. We report a case series of 12 severely neurologically impaired children with enteral feed-induced dystonias. Intestinal dysmotility was demonstrated in several. Improvements are seen with jejunal feeds or gut rest with total parenteral nutrition. Use of parenteral nutrition in children with severe neurodisability requires thorough discussion with patient groups and commissioners to give clinicians guidelines to standardize care.
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1097/MPG.0000000000001543" target="_blank" rel="noreferrer noopener">10.1097/MPG.0000000000001543</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).
1134-47-0 (baclofen)
2017
acquired brain injury
Adolescent
Article
Baclofen/dt [drug Therapy]
baclofen/tl [Intrathecal Drug Administration]
Campbell D I
Case Study
central nervous system disease
Cerebral Palsy
Child
Clinical Article
Constipation
Dystonia
dystonia/dt [Drug Therapy]
feed induced dystonia
feed induced dystonia/dt [Drug Therapy]
feeding difficulties
Female
Gastroesophageal Reflux
Gastroscopy
GERD
Human
Hydrocephalus
Hyperglycinemia
Infant
infantile spasm
intestinal dysmotility
intestine motility
Intestine Obstruction
Journal Of Pediatric Gastroenterology And Nutrition
Male
microcephaly
Mordekar S R
motor dysfunction
MPSIII
onset age
Parenteral Nutrition
Percutaneous Endoscopic Gastrostomy
peristalsis
pharmacologic intervention
Practice Guideline
Preschool Child
Priority Journal
reflux esophagitis
Retrospective Study
Rett syndrome
Sanfilippo syndrome
School Child
tone and motor problems
total parenteral nutrition
Velayudhan M
West syndrome
-
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/10.1186/s11689-017-9196-7" target="_blank" rel="noreferrer noopener">http://doi.org/10.1186/s11689-017-9196-7</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
Autonomic breathing abnormalities in Rett syndrome: Caregiver perspectives in an international database study
Publisher
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Journal of Neurodevelopmental Disorders
Date
A point or period of time associated with an event in the lifecycle of the resource
2017
Subject
The topic of the resource
Genotype; caregiver; Developmental disability; priority journal; data base; school child; autonomic dysfunction; noninvasive ventilation; oxygen; human; article; child; female; male; adult; questionnaire; Rett syndrome; breathing disorder; hyperventilation; mecp2; bloating; breath holding; Breathing disorders; breathing pattern; International database; mutation; onset age; Rare disorder; rebreathing; breathing difficulties; trajectory; characteristics; breath-holding; hyperventilation; abdominal bloating
Creator
An entity primarily responsible for making the resource
MacKay J; Downs J; Wong K; Heyworth J; Epstein A; Leonard H
Description
An account of the resource
Background: Rett syndrome is a severe neurodevelopmental disorder associated with mutations in the MECP2 gene. Irregular breathing patterns and abdominal bloating are prominent but poorly understood features. Our aims were to characterize the abnormal breathing patterns and abdominal bloating, investigate the distribution of these by age and mutation type and examine their impact and management from a caregiver perspective. Methods: We invited previously recruited families from the International Rett Syndrome Study to complete a web-based questionnaire concerning their family member with Rett syndrome aged between 2 and 57 years. We used logistic regression to investigate presence, frequency and impact of breath-holding, hyperventilation, or abdominal bloating by age group and mutation type. Age of onset for both breathing abnormalities was investigated using time-to-onset analysis, and the Kaplan-Meier method was used to estimate the failure function for the study sample. Descriptive statistics were used to characterize the management of irregular breathing. Results: Questionnaires were returned by 413/482 (85.7%) families. Breath-holding was reported for 68.8%, hyperventilation for 46.4% and abdominal bloating for 42.4%. Hyperventilation was more prevalent and frequent in those younger than 7 years of age and abdominal bloating in those aged over 20 years. Onset of breathing irregularities usually occurred during early childhood. Caregivers perceived that daily life was considerably impacted for almost half (44.1%) of those with abdominal bloating and in just over than a third of those with breath-holding (35.8%) or hyperventilation (35.1%). Although perceived impact was broadly comparable between age and mutation groups for breath-holding, hyperventilation and abdominal bloating, girls and women with a p.Arg294*mutation were considered to be more affected by all three conditions. Only 31 individuals had received medically prescribed treatments including 12 different medications, added oxygen, rebreathing apparatus or non-invasive ventilation. Conclusions: Autonomic disturbances are prevalent and burdensome in Rett syndrome. This information may guide the design of inclusion criteria and outcome measures for clinical intervention trials targeting autonomic abnormalities. Further investigation of available treatments is necessary to delineate evidence-based management pathways. Copyright © 2017 The Author(s).
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1186/s11689-017-9196-7" target="_blank" rel="noreferrer noopener">10.1186/s11689-017-9196-7</a>
Rights
Information about rights held in and over the resource
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
2017
abdominal bloating
Adult
Article
autonomic dysfunction
bloating
breath holding
breath-holding
breathing difficulties
breathing disorder
Breathing disorders
breathing pattern
Caregiver
characteristics
Child
Data Base
Developmental Disability
Downs J
Epstein A
Female
Genotype
Heyworth J
Human
hyperventilation
International database
Journal of Neurodevelopmental Disorders
Leonard H
MacKay J
Male
mecp2
Mutation
Noninvasive Ventilation
onset age
oxygen
Priority Journal
Questionnaire
Rare disorder
rebreathing
Rett syndrome
School Child
Trajectory
Wong 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/10.1016/j.ijporl.2017.07.039" target="_blank" rel="noreferrer noopener">http://doi.org/10.1016/j.ijporl.2017.07.039</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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Pediatric leukodystrophies: The role of the otolaryngologist
Publisher
An entity responsible for making the resource available
International Journal of Pediatric Otorhinolaryngology
Date
A point or period of time associated with an event in the lifecycle of the resource
2017
Subject
The topic of the resource
adolescent; dysphagia; priority journal; cohort analysis; physician attitude; nose feeding; human; article; child; adult; clinical article; comorbidity; hearing impairment; anamnesis; physical examination; leukodystrophy/dt [Drug Therapy]; otolaryngologist; 1309378-01-5 (botulinum toxin A); 1638949-86-6 (botulinum toxin A); 1800016-51-6 (botulinum toxin A); 93384-43-1 (botulinum toxin A); aspiration pneumonia; botulinum toxin A/dt [Drug Therapy]; Drool; Quality of Life; Assessment; Questionnaire; head and neck disease; hypersalivation/su [Surgery]; leukodystrophy/dt [Drug Therapy]; quality of life assessment; stomach tube; breathing difficulties; feeding difficulties; sleep disturbance; Aicardi-Goutières syndrome; Krabbe disease; leukodystrophy; Pelizaeus-Merzbacher disease; x-linked adreno-leukodystrophy; trajectory; characteristics; drooling
Creator
An entity primarily responsible for making the resource
Kay-Rivest E; Khendek L; Bernard G; Daniel S J
Description
An account of the resource
Background Leukodystrophies consist of degenerative neurogenetic diseases often associated with comorbidities that extend beyond the neurological system. Despite their impacts on patients' quality of life and risks of complications, head and neck symptomology is poorly reported in the literature. The objective of this study was to identify and quantify the main head and neck complaints among a cohort of patients diagnosed with leukodystrophies and define the role of the otolaryngologist as part of a multidisciplinary team for treating these patients. Methods During the First Canadian National Conference on Leukodystrophies held at the Montreal's Children Hospital, a cohort of 12 patients diagnosed with leukodystrophies were recruited and evaluated by a multidisciplinary team. An otolaryngology-focused assessment was done through history and physical examination, and included a screening questionnaire for 23 common otolaryngology issues. If families reported a history of sialorrhea, a validated questionnaire (Drool Quality of Life Assessment Questionnaire (DroolQoL)) was subsequently distributed. Results from the questionnaires were then compiled and analyzed. Results Of the 12 recruited patients, 83% (10/12) were known to an otolaryngologist. Drooling affected 67% (8/12) of patients although only 37.5% (3/8) of patients had undergone medical or surgical therapies for this issue. Four patients experienced at least one aspiration pneumonia. 58% (7/12) of the patients had dysphagia, of whom 43% (3/12) were fed exclusively via gastrostomy tube and 28% (2/7) required thickening of feeds. Two patients, despite suspicion of dysphagia and aspiration, had never undergone evaluation. As for otologic issues, it was noted that 25% (3/12) of patients had a history of pressure equalizing tubes (PETs) and one patient had a history of hearing loss. Conclusion Head and neck comorbidities affect children with leukodystrophies. Therefore, the otolaryngologist should be part of the multidisciplinary team, specifically for the management of dysphagia and sialorrhea.
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1016/j.ijporl.2017.07.039" target="_blank" rel="noreferrer noopener">10.1016/j.ijporl.2017.07.039</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).
1309378-01-5 (botulinum toxin A)
1638949-86-6 (botulinum toxin A)
1800016-51-6 (botulinum toxin A)
2017
93384-43-1 (botulinum toxin A)
Adolescent
Adult
Aicardi-Goutières syndrome
anamnesis
Article
aspiration pneumonia
Assessment
Bernard G
botulinum toxin A/dt [Drug Therapy]
breathing difficulties
characteristics
Child
Clinical Article
Cohort Analysis
Comorbidity
Daniel S J
Drool
drooling
Dysphagia
feeding difficulties
head and neck disease
hearing impairment
Human
hypersalivation/su [Surgery]
International Journal of Pediatric Otorhinolaryngology
Kay-Rivest E
Khendek L
Krabbe disease
Leukodystrophy
leukodystrophy/dt [Drug Therapy]
nose feeding
otolaryngologist
Pelizaeus-Merzbacher disease
physical examination
Physician Attitude
Priority Journal
Quality Of Life
quality of life assessment
Questionnaire
sleep disturbance
stomach tube
Trajectory
x-linked adreno-leukodystrophy
-
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.33902" target="_blank" rel="noreferrer noopener">http://doi.org/10.1002/ajmg.a.33902</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 abnormalities in untreated patients with mucopolysaccharidosis type VI
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
2011
Subject
The topic of the resource
clinical feature; priority journal; preschool child; cross-sectional study; prospective study; human; article; child; female; male; controlled study; clinical article; prevalence; disease severity; pulmonary hypertension; polysomnography; disease association; anamnesis; apnea; Doppler echocardiography; Glycosaminoglycans; Lysosomal storage diseases; lysosome storage disease; macroglossia; Maroteaux Lamy syndrome; Morquio syndrome; Mucopolysaccharidosis; oxygen saturation; oxygen/ec [Endogenous Compound]; physical examination; pigeon thorax; Sleep apnea; sleep apnea syndrome/di [Diagnosis]; sleep disorder; snoring; breathing difficulties; MPSVI; trajectory; characteristics; witnessed apnea
Creator
An entity primarily responsible for making the resource
John A; Fagondes S; Schwartz I; Azevedo A C; Barrios P; Dalcin P; Menna-Barreto S; Giugliani R
Description
An account of the resource
Mucopolysaccharidosis type VI (MPS VI) is a lysosomal storage disease that affects an enzyme responsible for the degradation of glycosaminoglycans (GAGs). Partially degraded GAGs accumulate in several tissues, such as the upper airways (UA), which leads to the development of obstructive sleep apnea (OSA). Our objective was to determine the prevalence of OSA in a group of untreated patients with MPS VI and the association of OSA with clinical and echocardiographic findings. Patients aged 4 years or older with a biochemical diagnosis of MPS VI were included. Data about clinical history, physical examination, Doppler echocardiogram, and overnight polysomnography (PSG) were collected. Our results showed that of the 28 participants, 14 were boys; mean age was 98.5 months, and mean age at MPS VI diagnosis was 48.4 months. Snoring, witnessed apnea, pectus carinatum, and macroglossia were the main clinical findings. PSG results showed that 23:27 patients (85.1%) had OSA which was mild in 4, moderate in 5, and severe in 14 patients. Echocardiograms showed evidence of pulmonary hypertension (PH) in 14 patients. Lower (P=0.037) and nadir SpO<inf>2</inf> (P=0.007) were positively associated with PH. Clinical signs suggestive of respiratory abnormalities during sleep were not significantly correlated with the results of PSG. We conclude that the prevalence of OSA in patients with MPS VI was high, and the level of desaturation was positively correlated with PH. Symptoms during sleep were not associated with PSG findings, which suggests that this population should undergo routine PSG as earlier as possible. This study provides baseline data to estimate the potential impact of specific treatments in the sleep abnormalities presented by patients with MPS VI. © 2011 Wiley-Liss, Inc.
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1002/ajmg.a.33902" target="_blank" rel="noreferrer noopener">10.1002/ajmg.a.33902</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
American Journal of Medical Genetics Part A
anamnesis
apnea
Article
Azevedo A C
Barrios P
breathing difficulties
characteristics
Child
Clinical Article
Clinical Feature
Controlled Study
Cross-sectional Study
Dalcin P
disease association
Disease Severity
Doppler echocardiography
Fagondes S
Female
Giugliani R
Glycosaminoglycans
Human
John A
Lysosomal storage diseases
lysosome storage disease
macroglossia
Male
Maroteaux Lamy syndrome
Menna-Barreto S
Morquio syndrome
MPSVI
Mucopolysaccharidosis
oxygen saturation
oxygen/ec [Endogenous Compound]
physical examination
pigeon thorax
Polysomnography
Preschool Child
Prevalence
Priority Journal
Prospective Study
pulmonary hypertension
Schwartz I
Sleep apnea
sleep apnea syndrome/di [Diagnosis]
sleep disorder
snoring
Trajectory
witnessed apnea
-
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/cjn.2016.8" target="_blank" rel="noreferrer noopener">http://doi.org/10.1017/cjn.2016.8</a>
Dublin Core
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Title
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The Incidence and Evolution of Parkinsonian Rigidity in Rett Syndrome: A Pilot Study
Publisher
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Canadian Journal of Neurological Sciences
Date
A point or period of time associated with an event in the lifecycle of the resource
2016
Subject
The topic of the resource
adolescent; age; pharmacokinetics; neck; major clinical study; priority journal; scoring system; pilot study; quantitative study; cohort analysis; cross-sectional study; dopamine; prospective study; human; article; child; female; adult; disease severity; speech; dystonia; Rett syndrome; methyl CpG binding protein 2; methyl CpG binding protein 2/ec [Endogenous Compound]; mecp2; rigidity; 5 hydroxyindoleacetic acid; ankle; cerebrospinal fluid; Dopamine; genetic susceptibility; homovanillic acid; homovanillic acid/ec [Endogenous Compound]; hva; incidence; missense mutation; mobilization; muscle rigidity; muscle tone; musculoskeletal disease assessment; parkinsonism; Rett syndrome rigidity distribution score; walking difficulty; tone and motor problems; trajectory; characteristics; rigidity
Creator
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Humphreys P; Barrowman N
Description
An account of the resource
Background: Patients with Rett syndrome (RTT) may demonstrate parkinsonian features. Here, we report a preliminary cross-sectional and prospective evaluation of the evolution, regional distribution, and eventual incidence of rigid tone in a cohort of MECP2 mutation-positive patients. Methods: In 51 participants, muscle tone rigidity in extremity regions and neck plus hypomimia were quantified using an RTT rigidity distribution (RTTRD) score with a range of 0 to 15. RTTRD scores were correlated with age, ability to walk and speak, mutation type, and, in a small subgroup (n=9), cerebrospinal fluid (CSF) homovanillic acid (HVA) and 5-hydroxyindole-acetic acid levels. Results: Participant ages ranged from 2 years and 5 months, to 54 years. Rigidity was found in 43/51 (84.3%); it appeared as early as age 3, increased in extent with age, and was present in all participants aged >13. Ankle region rigidity appeared first, followed by proximal legs, arms, neck, and face. Ambulatory participants (n=21) had lower RTTRD scores than nonambulatory (n=30; p=0.003). We found a trend to lower scores in participants with retained speech (n=13) versus those with none (n=38; p=0.074), and no difference in scores for those with truncating (n=25) versus missense mutations (n=22; p=0.387). RTTRD scores correlated negatively with CSF HVA levels (R=-0.83; p=0.005), but not with 5-hydroxyindole-acetic acid levels (R=-0.45; p=0.22). Conclusions: Although assessment of muscle tone is somewhat subjective and the RTTRD has not been validated, this study nevertheless suggests that parkinsonian rigidity in RTT is common and frequently increases in extent with age; its severity correlates directly with impaired ambulation and inversely with CSF HVA levels. Copyright © The Canadian Journal of Neurological Sciences Inc. 2016.
Identifier
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<a href="http://doi.org/10.1017/cjn.2016.8" target="_blank" rel="noreferrer noopener">10.1017/cjn.2016.8</a>
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Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
2016
5 hydroxyindoleacetic acid
Adolescent
Adult
Age
ankle
Article
Barrowman N
Canadian Journal of Neurological Sciences
Cerebrospinal Fluid
characteristics
Child
Cohort Analysis
Cross-sectional Study
Disease Severity
Dopamine
Dystonia
Female
genetic susceptibility
homovanillic acid
homovanillic acid/ec [Endogenous Compound]
Human
Humphreys P
hva
Incidence
Major Clinical Study
mecp2
methyl CpG binding protein 2
methyl CpG binding protein 2/ec [Endogenous Compound]
missense mutation
mobilization
muscle rigidity
muscle tone
musculoskeletal disease assessment
neck
parkinsonism
Pharmacokinetics
Pilot Study
Priority Journal
Prospective Study
Quantitative Study
Rett syndrome
Rett syndrome rigidity distribution score
rigidity
scoring system
Speech
tone and motor problems
Trajectory
walking difficulty
-
Dublin Core
The Dublin Core metadata element set is common to all Omeka records, including items, files, and collections. For more information see, http://dublincore.org/documents/dces/.
Title
A name given to the resource
Treatment of Symptoms in Children with Q3 Conditions Scoping Review Results
Text
A resource consisting primarily of words for reading. Examples include books, letters, dissertations, poems, newspapers, articles, archives of mailing lists. Note that facsimiles or images of texts are still of the genre Text.
URL Address
<a href="http://doi.org/10.1016/j.ejpn.2017.09.003" target="_blank" rel="noreferrer noopener">http://doi.org/10.1016/j.ejpn.2017.09.003</a>
Dublin Core
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Title
A name given to the resource
Intrathecal baclofen treatment an option in X-linked adrenoleukodystrophy
Publisher
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European Journal of Paediatric Neurology
Date
A point or period of time associated with an event in the lifecycle of the resource
2018
Subject
The topic of the resource
quality of life; pain; limited mobility; priority journal; school child; spasticity; fatigue; clinical examination; human; article; child; male; clinical article; dystonia; case report; 1309378-01-5 (botulinum toxin A); 1638949-86-6 (botulinum toxin A); 1800016-51-6 (botulinum toxin A); 93384-43-1 (botulinum toxin A); 1134-47-0 (baclofen); adrenoleukodystrophy/dt [Drug Therapy]; adrenoleukodystrophy/su [Surgery]; baclofen/dt [Drug Therapy]; baclofen/po [Oral Drug Administration]; baclofen/tl [Intrathecal Drug Administration]; X chromosome linked disorder/dt [Drug Therapy]; X chromosome linked disorder/su [Surgery]; Addison disease; adrenoleukodystrophy/dt [Drug Therapy]; balance disorder; behavior change; bladder dysfunction; botulinum toxin A; clonus; diplopia; drug dose increase; hearing disorder; hyperpigmentation; intrathecal pump; leukodystrophy; range of motion; strabismus; urinary catheter; visual disorder; X chromosome linked disorder/dt [Drug Therapy]; tone and motor problems; X-linked adrenoleukodystrophy; pharmacologic interventions; intrathecal baclofen; baclofen
Creator
An entity primarily responsible for making the resource
Hjartarson H T; Ehrstedt C; Tedroff K
Description
An account of the resource
Background X-linked adrenoleukodystrophy (X-ALD) is a genetic peroxisomal disorder associated with tissue accumulation of very long chain fatty acids (VLCFAs). In approximately one third of affected males, this causes progressive and irreversible damage to the brain white matter. Progress is often rapid with upper motor neuron damage leading to severe spasticity and dystonia. The increased muscle tone is frequently difficult to alleviate with oral drugs. Here, we describe two patients with X-ALD who have received treatment with intrathecal baclofen pumps (ITB). Case study Both boys had a rapidly progressive cerebral form of the disorder resulting, among other things, in escalating spasticity and dystonia causing severe pain, dramatically reducing their quality of life. Both were treated with a variety of oral medications without adequate relief. Both patients tolerated ITB surgery without complications and the positive clinical effects of treatment with ITB became clear in the following weeks and months, with significantly reduced muscle tone, less pain and better sleep. Moreover, general caretaking became easier. Conclusion The treatment of spasticity and dystonia in these patients is difficult partly due to the relentless nature of this progressive disorder. In our two patients, ITB has been effective from both a symptomatic and palliative perspective. We recommend that such treatment be considered as an early option for increased muscle tone in boys with the cerebral form of X-ALD.
Identifier
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<a href="http://doi.org/10.1016/j.ejpn.2017.09.003" target="_blank" rel="noreferrer noopener">10.1016/j.ejpn.2017.09.003</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).
1134-47-0 (baclofen)
1309378-01-5 (botulinum toxin A)
1638949-86-6 (botulinum toxin A)
1800016-51-6 (botulinum toxin A)
2018
93384-43-1 (botulinum toxin A)
Addison disease
adrenoleukodystrophy/dt [Drug Therapy]
adrenoleukodystrophy/su [Surgery]
Article
baclofen
Baclofen/dt [drug Therapy]
baclofen/po [Oral Drug Administration]
baclofen/tl [Intrathecal Drug Administration]
balance disorder
behavior change
bladder dysfunction
botulinum toxin A
Case Report
Child
Clinical Article
clinical examination
clonus
diplopia
drug dose increase
Dystonia
Ehrstedt C
European Journal of Paediatric Neurology
Fatigue
hearing disorder
Hjartarson H T
Human
hyperpigmentation
intrathecal baclofen
intrathecal pump
Leukodystrophy
limited mobility
Male
Pain
pharmacologic interventions
Priority Journal
Quality Of Life
Range of Motion
School Child
Spasticity
strabismus
Tedroff K
tone and motor problems
urinary catheter
visual disorder
X chromosome linked disorder/dt [Drug Therapy]
X chromosome linked disorder/su [Surgery]
X-linked adrenoleukodystrophy
-
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/dote.12503" target="_blank" rel="noreferrer noopener">http://doi.org/10.1111/dote.12503</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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Balloon dilatation in esophageal strictures in epidermolysis bullosa and the role of anesthesia
Publisher
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Diseases of the Esophagus
Date
A point or period of time associated with an event in the lifecycle of the resource
2017
Subject
The topic of the resource
adolescent; dysphagia; retrospective study; priority journal; school child; preschool child; clinical effectiveness; patient safety; human; article; child; female; male; adult; clinical article; young adult; colon interposition; gastrostomy; anesthesia; balloon dilatation; epidermolysis bullosa; esophagus stenosis/co [Complication]; esophagus stenosis/th [Therapy]; digestive endoscope; esophagus balloon; esophagus perforation; feeding difficulties; surgical intervention; endoscopic balloon dilatation
Creator
An entity primarily responsible for making the resource
Gollu G; Ergun E; Ates U; Can O S; Dindar H
Description
An account of the resource
Esophageal involvement, which causes stricture, is a complication in epidermolysis bullosa. This causes dysphagia and malnutrition and leads to deterioration of skin lesions in these patients. The charts of 11 patients with epidermolysis bullosa and esophageal stricture who were included into dilatation program between 2003 and 2015 were retrospectively reviewed. Seven of the patients were female and four were male. The median age was 14 (2-32) years. The mean body weight of patients was 27.8 (9-51) kg. The location and number of strictured parts of the esophagus were previously evaluated with upper gastrointestinal contrast study and after that flexible endoscopy was used for dilatation. Eight patients had middle esophageal, three patients had proximal esophageal and one of them had both proximal and middle esophageal strictures. The strictures were dilated 56 times in total (mean 5 times). One patient underwent gastrostomy and was medically followed-up after a perforation occurrence during the dilatation procedure. In a 32-year-old female patient, colon interposition was performed after four dilatations since optimal nutritional and developmental status could not be achieved. The dilatation program of nine patients is still in progress. Seven of them can easily swallow solid food but two of them have some difficulties in swallowing between dilatations. One patient rejected the program and quitted, while one patient refused colon interposition and died because of complications related to amyloidosis during the dilatation program. After resolution of the swallowing problem, skin lesions were observed to heal quickly. Epidermolysis bullosa is a rare cause of dysphagia. Esophageal balloon dilatation with flexible endoscopy is a safe and efficient method in patients with this condition.
Identifier
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<a href="http://doi.org/10.1111/dote.12503" target="_blank" rel="noreferrer noopener">10.1111/dote.12503</a>
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Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
2017
Adolescent
Adult
Anesthesia
Article
Ates U
Balloon dilatation
Can O S
Child
Clinical Article
Clinical Effectiveness
colon interposition
digestive endoscope
Dindar H
Diseases of the Esophagus
Dysphagia
endoscopic balloon dilatation
epidermolysis bullosa
Ergun E
esophagus balloon
esophagus perforation
esophagus stenosis/co [Complication]
esophagus stenosis/th [Therapy]
feeding difficulties
Female
Gastrostomy
Gollu G
Human
Male
Patient Safety
Preschool Child
Priority Journal
Retrospective Study
School Child
surgical intervention
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/10.1002/ana.410210410" target="_blank" rel="noreferrer noopener">http://doi.org/10.1002/ana.410210410</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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Rett's syndrome: Characterization of respiratory patterns and sleep
Publisher
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Annals of Neurology
Date
A point or period of time associated with an event in the lifecycle of the resource
1987
Subject
The topic of the resource
etiology; ataxia; major clinical study; central nervous system; priority journal; seizure; human; child; diagnosis; Rett syndrome; autism; dementia; heredity; hyperpnea; respiratory system; sleep; breathing difficulties; sleep disturbance; trajectory; characteristics; abnormal respiratory problems; abnormal sleep patterns
Creator
An entity primarily responsible for making the resource
Glaze D G; Frost Jr J D; Zoghbi H Y; Percy A K
Description
An account of the resource
Rett's syndrome is a progressive disorder that occurs in females and is characterized by autistic behavior, dementia, ataxia, loss of purposeful use of the hands, and seizures. Patients with Rett's syndrome have been observed to have stereotyped hand movements (hand-washing) and to exhibit intermittent hyperventilation. To characterize more precisely the sleep and respiratory patterns associated with this disorder, polygraphic studies were made during sleep and wakefulness in 11 patients with this syndrome. These studies showed abnormal respiratory patterns during wakefulness, and abnormal sleep and electroencephalographic characteristics. The patients had decreased percentages of rapid-eye-movement sleep, and during wakefulness, a pattern of disorganized breathing was observed in all 11 patients and consisted of hypoxia followed by a period of increased respiratory rate and effort. The occurrence of disorganized breathing and compensatory hyperpnea during wakefulness with regular, continuous breathing during sleep is characteristic of Rett's syndrome and suggests an altered or impaired voluntary/behavioral respiratory control system.
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1002/ana.410210410" target="_blank" rel="noreferrer noopener">10.1002/ana.410210410</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).
1987
abnormal respiratory problems
abnormal sleep patterns
Annals Of Neurology
ataxia
Autism
breathing difficulties
Central Nervous System
characteristics
Child
Dementia
Diagnosis
etiology
Frost Jr J D
Glaze D G
heredity
Human
hyperpnea
Major Clinical Study
Percy A K
Priority Journal
Respiratory System
Rett syndrome
Seizure
Sleep
sleep disturbance
Trajectory
Zoghbi H Y
-
Dublin Core
The Dublin Core metadata element set is common to all Omeka records, including items, files, and collections. For more information see, http://dublincore.org/documents/dces/.
Title
A name given to the resource
Treatment of Symptoms in Children with Q3 Conditions Scoping Review Results
Text
A resource consisting primarily of words for reading. Examples include books, letters, dissertations, poems, newspapers, articles, archives of mailing lists. Note that facsimiles or images of texts are still of the genre Text.
URL Address
<a href="http://doi.org/10.1016/j.pediatrneurol.2017.01.019" target="_blank" rel="noreferrer noopener">http://doi.org/10.1016/j.pediatrneurol.2017.01.019</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
Preliminary Study of Neurodevelopmental Outcomes and Parenting Stress in Pediatric Mitochondrial Disease
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
2017
Subject
The topic of the resource
emotion; child behavior; priority journal; intellectual impairment/dm [Disease Management]; prognosis; preschool child; parental stress; human; article; child; female; male; quality of life; clinical article; daily life activity; disease severity; aggression; intelligence quotient; childhood disease/dm [Disease Management]; comorbidity; Leigh disease/dm [Disease Management]; MELAS syndrome/dm [Disease Management]; nervous system development; problem behavior/dm [Disease Management]; anxiety disorder/dm [Disease Management]; attention disturbance/dm [Disease Management]; brain atrophy/dm [Disease Management]; delinquency; depression/dm [Disease Management]; drug resistant epilepsy/dm [Disease Management]; drug resistant epilepsy/dr [Drug Resistance]; intelligence; lactic acidosis/dm [Disease Management]; muscle disease/dm [Disease Management]; neuroimaging; nuclear magnetic resonance imaging; postnatal depression/dm [Disease Management]; psychomotor development; sleep disorder/dm [Disease Management]; social problem; somatization/dm [Disease Management]; thinking impairment/dm [Disease Management]; behavioral problems; Leigh syndrome; mitochondrial disorders; MELAS syndrome; trajectory; characteristics
Creator
An entity primarily responsible for making the resource
Eom S; Lee Y M
Description
An account of the resource
Background Little is known regarding the neuropsychological profiles of pediatric patients with mitochondrial diseases or their parents, information that is crucial for improving the quality of life (QOL) for both patients and parents. We aimed to delineate neurodevelopment and psychological comorbidity in children with mitochondrial diseases in the preliminary investigation of adequate intervention methods, better prognoses, and improved QOL for both patients and parents. Methods Seventy children diagnosed with mitochondrial diseases were neuropsychologically evaluated. Neurocognitive (development, intelligence) and psychological (behavior, daily living function, maternal depression, parenting stress) functions were analyzed. Clinical variables, including the first symptom, epileptic classification, organ involvement, lactic acidosis, brain magnetic resonance imaging findings, muscle pathology, biochemical enzyme assay results, and syndromic diagnosis of mitochondrial diseases, were also reviewed. Results Prediagnostic assessments indicated that cognitive and psychomotor developments were significantly delayed. Group mean full scale intelligence quotient (IQ) scores indicated mild levels of intellectual disability, borderline levels of verbal IQ impairment, and mild levels of intellectual disability on performance IQ. Many children exhibited clinically significant levels of behavioral problems, whereas mothers of children with mitochondrial diseases exhibited significant increases in parenting stress relative to mothers of healthy children. Furthermore, 65% of mothers exhibited significant levels of depression. Early onset of the first symptoms, diffuse brain atrophy, and drug-resistant epilepsy negatively influenced neurodevelopmental and adaptive functions. Conclusion Better understanding of the functional levels and profiles of neurodevelopment and psychological comorbidity in children with mitochondrial diseases in the prediagnostic period is essential for adequate support and QOL of children with mitochondrial diseases and their parents.
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1016/j.pediatrneurol.2017.01.019" target="_blank" rel="noreferrer noopener">10.1016/j.pediatrneurol.2017.01.019</a>
Rights
Information about rights held in and over the resource
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
2017
Aggression
anxiety disorder/dm [Disease Management]
Article
attention disturbance/dm [Disease Management]
behavioral problems
brain atrophy/dm [Disease Management]
characteristics
Child
Child Behavior
childhood disease/dm [Disease Management]
Clinical Article
Comorbidity
daily life activity
delinquency
depression/dm [Disease Management]
Disease Severity
drug resistant epilepsy/dm [Disease Management]
drug resistant epilepsy/dr [Drug Resistance]
Emotion
Eom S
Female
Human
intellectual impairment/dm [Disease Management]
Intelligence
Intelligence Quotient
lactic acidosis/dm [Disease Management]
Lee Y M
Leigh disease/dm [Disease Management]
Leigh syndrome
Male
MELAS syndrome
MELAS syndrome/dm [Disease Management]
mitochondrial disorders
muscle disease/dm [Disease Management]
nervous system development
neuroimaging
nuclear magnetic resonance imaging
Parental Stress
Pediatric Neurology
postnatal depression/dm [Disease Management]
Preschool Child
Priority Journal
problem behavior/dm [Disease Management]
Prognosis
psychomotor development
Quality Of Life
sleep disorder/dm [Disease Management]
social problem
somatization/dm [Disease Management]
thinking impairment/dm [Disease Management]
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/mus.25524" target="_blank" rel="noreferrer noopener">http://doi.org/10.1002/mus.25524</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
Mitochondrial capacity, muscle endurance, and low energy in friedreich ataxia
Publisher
An entity responsible for making the resource available
Muscle and Nerve
Date
A point or period of time associated with an event in the lifecycle of the resource
2017
Subject
The topic of the resource
adolescent; priority journal; scoring system; school child; cross-sectional study; fatigue; human; article; child; female; male; controlled study; adult; clinical article; comparative study; disease severity; Friedreich ataxia; cellular subcellular and molecular biological phenomena and functions; energy; physical energy; mitochondrial capacity; muscle exercise; electrostimulation; Godin Leisure Time Physical Activity Score; mechanomyography; myography; near infrared spectroscopy; oxygen consumption; skeletal muscle; trajectory; characteristics; endurance; muscle endurance; alertness
Creator
An entity primarily responsible for making the resource
Bossie H M; Willingham T B; Schoick R A V; O'Connor P J; McCully K K
Description
An account of the resource
Introduction: In this study we noninvasively evaluated skeletal muscle mitochondrial capacity, muscle-specific endurance, and energy/fatigue feelings in persons with Friedreich ataxia (FRDA) and able-bodied controls (AB). Methods: Forearm mitochondrial capacity was measured in FRDA (n = 16) and AB (n = 10) study participants using the rate of recovery of oxygen consumption after electrical stimulation with near-infrared spectroscopy. Mechanomyography (MMG) assessed muscle endurance after electrical stimulation for 3 minutes at 2 Hz, 4 Hz, and 6 Hz. Validated scales assessed disease severity and energy/fatigue feelings. Results: Groups did not differ in mitochondrial capacity (FRDA and AB: 1.8 +/- 0.3 L/min). The difference in muscle endurance at 6 Hz was lower by 19.2% in the FRDA group (group effect: P < 0.001). Feelings of physical energy were 34% lower in FRDA group. In FDRA muscle, endurance was positively related to mitochondrial capacity (r = 0.59, P = 0.03), and disease severity was negatively related to mitochondrial capacity (r = -0.55, P = 0.04) and muscle endurance (r = -0.60, P = 0.01). Conclusion: Non-invasive measures of skeletal muscle mitochondrial capacity and muscle-specific endurance are useful in monitoring FRDA. Muscle Nerve 56: 773-779, 2017.
Identifier
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<a href="http://doi.org/10.1002/mus.25524" target="_blank" rel="noreferrer noopener">10.1002/mus.25524</a>
Rights
Information about rights held in and over the resource
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
2017
Adolescent
Adult
alertness
Article
Bossie H M
cellular subcellular and molecular biological phenomena and functions
characteristics
Child
Clinical Article
Comparative Study
Controlled Study
Cross-sectional Study
Disease Severity
electrostimulation
endurance
energy
Fatigue
Female
Friedreich ataxia
Godin Leisure Time Physical Activity Score
Human
Male
McCully K K
mechanomyography
mitochondrial capacity
Muscle and Nerve
muscle endurance
muscle exercise
myography
near infrared spectroscopy
O'Connor P J
oxygen consumption
physical energy
Priority Journal
Schoick R A V
School Child
scoring system
skeletal muscle
Trajectory
Willingham T 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/10.1016/j.brs.2016.07.009" target="_blank" rel="noreferrer noopener">http://doi.org/10.1016/j.brs.2016.07.009</a>
Dublin Core
The Dublin Core metadata element set is common to all Omeka records, including items, files, and collections. For more information see, http://dublincore.org/documents/dces/.
Title
A name given to the resource
Deep Brain Stimulation in Rare Inherited Dystonias
Publisher
An entity responsible for making the resource available
Brain Stimulation
Date
A point or period of time associated with an event in the lifecycle of the resource
2016
Subject
The topic of the resource
adolescent; clinical assessment; disease duration; time to treatment; dystonic disorder/su [Surgery]; priority journal; follow up; school child; outcome assessment; Dystonia; clinical effectiveness; gabapentin/dt [Drug Therapy]; human; article; child; adult; clinical article; aged; surgery; middle aged; disease severity; dystonia; phenotype; rating scale; ataxia telangiectasia/di [Diagnosis]; ataxia telangiectasia/dt [Drug Therapy]; atypical dopa responsive dystonia/di [Diagnosis]; atypical dopa responsive dystonia/dt [Drug Therapy]; baclofen/dt [Drug Therapy]; benzodiazepine derivative/dt [Drug Therapy]; brain depth stimulation; Burke Fahn Marsden Dystonia Rating Scale; cerebellar ataxia/di [Diagnosis]; cerebellar ataxia/dt [Drug Therapy]; chorea/di [Diagnosis]; chorea/dt [Drug Therapy]; clobazam/dt [Drug Therapy]; clonazepam/dt [Drug Therapy]; Deep brain stimulation; diazepam/dt [Drug Therapy]; dystonia/di [Diagnosis]; dystonia/dt [Drug Therapy]; dystonic disorder/th [Therapy]; entacapone/cb [Drug Combination]; entacapone/dt [Drug Therapy]; escitalopram/dt [Drug Therapy]; extrapyramidal syndrome/di [Diagnosis]; extrapyramidal syndrome/dt [Drug Therapy]; haloperidol/dt [Drug Therapy]; Inherited dystonia; levodopa/dt [Drug Therapy]; lorazepam/dt [Drug Therapy]; methylmalonic aciduria/di [Diagnosis]; methylmalonic aciduria/dt [Drug Therapy]; mirtazapine/dt [Drug Therapy]; motor dysfunction assessment; nemaline myopathy/di [Diagnosis]; nemaline myopathy/dt [Drug Therapy]; neuronal ceroid lipofuscinosis/di [Diagnosis]; neuronal ceroid lipofuscinosis/dt [Drug Therapy]; olanzapine/dt [Drug Therapy]; pramipexole/cb [Drug Combination]; pramipexole/dt [Drug Therapy]; preoperative care; risperidone/dt [Drug Therapy]; selegiline/cb [Drug Combination]; selegiline/dt [Drug Therapy]; tetrabenazine/dt [Drug Therapy]; therapy effect; tizanidine/dt [Drug Therapy]; trazodone/dt [Drug Therapy]; Treatment; trihexyphenidyl/cb [Drug Combination]; trihexyphenidyl/dt [Drug Therapy]; trisomy/di [Diagnosis]; trisomy/dt [Drug Therapy]; Wilson disease/di [Diagnosis]; Wilson disease/dt [Drug Therapy]; woodhouse sakati syndrome/di [Diagnosis]; woodhouse sakati syndrome/dt [Drug Therapy]; x trisomy/di [Diagnosis]; x trisomy/dt [Drug Therapy]; tone and motor problems; ataxia telangiectasia; MCM deficiency; NCL; Nemaline myopathy; surgical intervention; Deep Brain Stimulation
Creator
An entity primarily responsible for making the resource
Beaulieu-Boire I; Aquino C C; Fasano A; Poon Y Y; Fallis M; Lang A E; Hodaie M; Kalia S K; Lozano A; Moro E
Description
An account of the resource
Background Rare causes of inherited movement disorders often present with a debilitating phenotype of dystonia, sometimes combined with parkinsonism and other neurological signs. Since these disorders are often resistant to medications, DBS may be considered as a possible treatment. Methods Patients with identified genetic diseases (ataxia-telangiectasia, chorea-achantocytosis, dopa-responsive dystonia, congenital nemaline myopathy, methylmalonic aciduria, neuronal ceroid lipofuscinosis, spinocerebellar ataxia types 2 and 3, Wilson's disease, Woodhouse-Sakati syndrome, methylmalonic aciduria, and X trisomy) and disabling dystonia underwent bilateral GPi DBS (bilateral thalamic Vim nucleus in 1 case). The primary outcome was the difference in the Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS) between baseline, 1 year and last available follow-up. Preoperative factors such as age at surgery, disease duration at surgery, proportion of life lived with dystonia and severity of dystonia were correlated to the primary outcome. Results Eleven patients were operated between February 2003 and December 2013. Age and duration of disease at time of surgery were 30+/-19 and 12.5+/-15.7 years, respectively. DBS effects on dystonia severity were variable but overall marginally effective, with a mean improvement of 7.9% (p=0.39) at 1-year follow-up and 16.7% (p=0.46) at last follow-up (mean 47.3+/-19.9 months after surgery). No preoperative factors were identified to predict the surgical outcome. Conclusion Our findings support the current knowledge that DBS is modestly effective in treating rare inherited dystonias with a combined phenotype. However, the BFMDRS might not be the best tool to measure outcome in these severely affected patients. Copyright © 2016 Elsevier Inc.
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1016/j.brs.2016.07.009" target="_blank" rel="noreferrer noopener">10.1016/j.brs.2016.07.009</a>
Rights
Information about rights held in and over the resource
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
2016
Adolescent
Adult
Aged
Aquino C C
Article
ataxia telangiectasia
ataxia telangiectasia/di [Diagnosis]
ataxia telangiectasia/dt [Drug Therapy]
atypical dopa responsive dystonia/di [Diagnosis]
atypical dopa responsive dystonia/dt [Drug Therapy]
Baclofen/dt [drug Therapy]
Beaulieu-Boire I
benzodiazepine derivative/dt [Drug Therapy]
Brain Depth Stimulation
Brain Stimulation
Burke Fahn Marsden Dystonia Rating Scale
cerebellar ataxia/di [Diagnosis]
cerebellar ataxia/dt [Drug Therapy]
Child
chorea/di [Diagnosis]
chorea/dt [Drug Therapy]
Clinical Article
clinical assessment
Clinical Effectiveness
clobazam/dt [Drug Therapy]
clonazepam/dt [Drug Therapy]
deep brain stimulation
diazepam/dt [Drug Therapy]
disease duration
Disease Severity
Dystonia
dystonia/di [Diagnosis]
dystonia/dt [Drug Therapy]
Dystonic Disorder/su [surgery]
dystonic disorder/th [Therapy]
entacapone/cb [Drug Combination]
entacapone/dt [Drug Therapy]
escitalopram/dt [Drug Therapy]
extrapyramidal syndrome/di [Diagnosis]
extrapyramidal syndrome/dt [Drug Therapy]
Fallis M
Fasano A
Follow Up
gabapentin/dt [Drug Therapy]
haloperidol/dt [Drug Therapy]
Hodaie M
Human
Inherited dystonia
Kalia S K
Lang A E
levodopa/dt [Drug Therapy]
lorazepam/dt [Drug Therapy]
Lozano A
MCM deficiency
methylmalonic aciduria/di [Diagnosis]
methylmalonic aciduria/dt [Drug Therapy]
Middle Aged
mirtazapine/dt [Drug Therapy]
Moro E
motor dysfunction assessment
NCL
Nemaline myopathy
nemaline myopathy/di [Diagnosis]
nemaline myopathy/dt [Drug Therapy]
neuronal ceroid lipofuscinosis/di [Diagnosis]
neuronal ceroid lipofuscinosis/dt [Drug Therapy]
olanzapine/dt [Drug Therapy]
outcome assessment
Phenotype
Poon Y Y
pramipexole/cb [Drug Combination]
pramipexole/dt [Drug Therapy]
Preoperative Care
Priority Journal
Rating Scale
risperidone/dt [Drug Therapy]
School Child
selegiline/cb [Drug Combination]
selegiline/dt [Drug Therapy]
Surgery
surgical intervention
tetrabenazine/dt [Drug Therapy]
therapy effect
time to treatment
tizanidine/dt [Drug Therapy]
tone and motor problems
trazodone/dt [Drug Therapy]
Treatment
trihexyphenidyl/cb [Drug Combination]
trihexyphenidyl/dt [Drug Therapy]
Trisomy/di [diagnosis]
trisomy/dt [Drug Therapy]
Wilson disease/di [Diagnosis]
Wilson disease/dt [Drug Therapy]
woodhouse sakati syndrome/di [Diagnosis]
woodhouse sakati syndrome/dt [Drug Therapy]
x trisomy/di [Diagnosis]
x trisomy/dt [Drug Therapy]
-
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/S0016-5107%2803%2902379-4" target="_blank" rel="noreferrer noopener">http://doi.org/10.1016/S0016-5107%2803%2902379-4</a>
Dublin Core
The Dublin Core metadata element set is common to all Omeka records, including items, files, and collections. For more information see, http://dublincore.org/documents/dces/.
Title
A name given to the resource
Efficacy and safety of endoscopic dilation of esophageal strictures in epidermolysis bullosa
Publisher
An entity responsible for making the resource available
Gastrointestinal Endoscopy
Date
A point or period of time associated with an event in the lifecycle of the resource
2004
Subject
The topic of the resource
adolescent; dysphagia; major clinical study; priority journal; follow up; scoring system; school child; human; article; female; male; adult; morbidity; treatment outcome; balloon dilatation; barium; body weight; contrast enhancement; endoscopic therapy; epidermolysis bullosa/th [Therapy]; epidermolysis bullosa; esophagus dilatation; esophagus stricture/th [Therapy]; nutritional status; propofol; feeding difficulties; surgical intervention; endoscopic balloon dilatation
Creator
An entity primarily responsible for making the resource
Anderson S H C; Meenan J; Williams K N; Eady R A J; Prinja H; Chappiti U; Doig L; Thompson R P H
Description
An account of the resource
Background: Epidermolysis bullosa is a rare genetically determined disorder of the stratified squamous epithelium. Patients with the most severe forms develop scarring of the esophagus after ingestion of food. This results in dysphagia, which severely compromises the ability to eat. Maintenance of adequate nutritional intake is a central aim, but the most appropriate method is unknown. Methods: The results of endoscopic through-the-scope balloon dilation under propofol anesthesia in 53 patients with epidermolysis bullosa and esophageal strictures are reported. Results: Seventy-five percent of patients had a single stricture (range 1 to 6 strictures), most often in the proximal esophagus (median 20 cm from incisors). A total of 182 dilations were performed (median two per patient) over a median follow-up period of 3.5 years. For all but 3 patients, there was an improvement in the dysphagia score. There was a mean increase in weight after the procedure of 2.9 kg: 95% CI[2.0, 3.8]; p < 0.001, over a median 29 days. There was no significant post-procedure morbidity. Conclusions: Endoscopic balloon dilation is a safe and effective treatment for the esophageal strictures of epidermolysis bullosa. In the majority of patients, dilation relieves dysphagia and improves nutritional status.
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1016/S0016-5107%2803%2902379-4" target="_blank" rel="noreferrer noopener">10.1016/S0016-5107%2803%2902379-4</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).
2004
Adolescent
Adult
Anderson S H C
Article
Balloon dilatation
barium
Body Weight
Chappiti U
contrast enhancement
Doig L
Dysphagia
Eady R A J
endoscopic balloon dilatation
endoscopic therapy
epidermolysis bullosa
epidermolysis bullosa/th [Therapy]
esophagus dilatation
esophagus stricture/th [Therapy]
feeding difficulties
Female
Follow Up
Gastrointestinal Endoscopy
Human
Major Clinical Study
Male
Meenan J
Morbidity
Nutritional Status
Prinja H
Priority Journal
Propofol
School Child
scoring system
surgical intervention
Thompson R P H
Treatment Outcome
Williams K N
-
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
December 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
December 2019 List
URL Address
<a href="http://doi.org/10.1136/bmjpo-2019-000547" target="_blank" rel="noreferrer noopener">http://doi.org/10.1136/bmjpo-2019-000547</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
Access to paediatric palliative care in children and adolescents with complex chronic conditions: A retrospective hospital-based study in Brussels, Belgium
Publisher
An entity responsible for making the resource available
BMJ Paediatrics Open
Date
A point or period of time associated with an event in the lifecycle of the resource
2019
Subject
The topic of the resource
adolescent; article; Belgium; cardiovascular disease; child; chronic disease; complex chronic condition; congenital disorder; data base; female; gastrointestinal disease; genetic disorder; groups by age; health care access; hematologic disease; hospital; hospital admission; hospitalized adolescent; hospitalized child; human; Icd-9; immune deficiency; infant; length of stay; major clinical study; male; malignant neoplasm; metabolic disorder; morbidity; neurologic disease; newborn; palliative therapy; patient care; patient referral; pediatric patient; pediatric ward; priority journal; respiratory tract disease; retrospective study; urinary tract disease
Creator
An entity primarily responsible for making the resource
Friedel M; Gilson A; Bouckenaere D; Brichard B; Fonteyne C; Wojcik T; De Clercq E; Guillet A; Mahboub A; Lahaye M; Aujoulat I
Description
An account of the resource
BACKGROUND: Paediatric complex chronic conditions (CCCs) are life-limiting conditions requiring paediatric palliative care, which, in Belgium, is provided through paediatric liaison teams (PLTs). Like the number of children and adolescents with these conditions in Belgium, their referral to PLTs is unknown. OBJECTIVES: The aim of the study was to identify, over a 5-year period (2010-2014), the number of children and adolescents (0-19 years) living with a CCC, and also their referral to PLTs. METHODS: International Classification of Disease codes (ICD-9) corresponding to a CCC, as described by Feudtner et al, and national registration numbers were extracted from the databases of all hospitals (n=8) and PLTs (n=2) based in the Brussels region. Aggregated data and pseudonymised national registration number were transmitted to the research team by a Trusted Third Party (eHealth). Ages and diagnostic categories were calculated using descriptive statistics. RESULTS: Over 5 years (2010-2014) in the Brussels region, a total of 22 721 children/adolescents aged 0-19 years were diagnosed with a CCC. Of this number, 22 533 were identified through hospital registries and 572 through PLT registries. By comparing the registries, we found that of the 22 533 children/adolescents admitted to hospital, only 384 (1.7%) were also referred to a PLT. CONCLUSION: In Belgium, there may be too few referrals of children and adolescents with CCC to PLTs that ensure continuity of care.
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1136/bmjpo-2019-000547" target="_blank" rel="noreferrer noopener">10.1136/bmjpo-2019-000547</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).
2019
Adolescent
Article
Aujoulat I
Belgium
BMJ Paediatrics Open
Bouckenaere D
Brichard B
Cardiovascular Disease
Child
Chronic Disease
complex chronic condition
congenital disorder
Data Base
De Clercq E
December 2019 List
Female
Fonteyne C
Friedel M
gastrointestinal disease
Genetic Disorder
Gilson A
groups by age
Guillet A
Health Care Access
hematologic disease
Hospital
Hospital Admission
hospitalized adolescent
Hospitalized Child
Human
Icd-9
immune deficiency
Infant
Lahaye M
Length Of Stay
Mahboub A
Major Clinical Study
Male
Malignant Neoplasm
Metabolic Disorder
Morbidity
Neurologic Disease
Newborn
Palliative Therapy
Patient Care
Patient Referral
pediatric patient
pediatric ward
Priority Journal
Respiratory Tract Disease
Retrospective Study
urinary tract disease
Wojcik 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
October 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
October 2019 List
URL Address
<a href="http://doi.org/10.1136/bmjpo-2019-000467" target="_blank" rel="noreferrer noopener">http://doi.org/10.1136/bmjpo-2019-000467</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
Gabapentin for the treatment of pain manifestations in children with severe neurological impairment: A single-centre retrospective review
Publisher
An entity responsible for making the resource available
BMJ Paediatrics Open
Date
A point or period of time associated with an event in the lifecycle of the resource
2019
Subject
The topic of the resource
child; human; retrospective study; clinical article; palliative care; pain; priority journal; review; alopecia/si [Side Effect]; disease severity; drug efficacy; drug substitution; drug withdrawal; gabapentin/ae [Adverse Drug Reaction]; gabapentin/dt [Drug Therapy]; gabapentin/pv [Special Situation for Pharmacovigilance]; gastroenterology; general paediatrics; hypertransaminasemia/si [Side Effect]; irritability; lethargy/si [Side Effect]; muscle twitch; neurodisability; neurologic disease; pain/dt [Drug Therapy]; pregabalin/dt [Drug Therapy]; pregabalin/pv [Special Situation for Pharmacovigilance]; treatment duration; treatment response; vomiting/dt [Drug Therapy]; vomiting/si [Side Effect]
Creator
An entity primarily responsible for making the resource
Collins A; Mannion R; Broderick A; Hussey S; Devins M; Bourke B
Description
An account of the resource
Pain, irritability and feeding intolerance are common symptoms affecting quality of life in children with severe neurological impairment (SNI). We performed a retrospective study to explore the use of gabapentinoid medications for symptom control in children with SNI. Patients attending the palliative care or gastroenterology department being treated with gabapentin for irritability, vomiting or pain of unknown origin were included. Information was gathered retrospectively from medical documentation. Irritability was reduced in 30 of the 42 patients included. Gabapentin was discontinued in 15 children, 12 of whom then received pregabalin. Three children had a good response to pregabalin, six a minimal improvement and three no improvement. These results support the use of gabapentinoids in this patient cohort. Copyright © Author(s) (or their employer(s)) 2019.
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1136/bmjpo-2019-000467" target="_blank" rel="noreferrer noopener">10.1136/bmjpo-2019-000467</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).
2019
alopecia/si [Side Effect]
BMJ Paediatrics Open
Bourke B
Broderick A
Child
Clinical Article
Collins A
Devins M
Disease Severity
Drug Efficacy
Drug Substitution
Drug Withdrawal
gabapentin/ae [Adverse Drug Reaction]
gabapentin/dt [Drug Therapy]
gabapentin/pv [Special Situation for Pharmacovigilance]
Gastroenterology
General Paediatrics
Human
Hussey S
hypertransaminasemia/si [Side Effect]
Irritability
lethargy/si [Side Effect]
Mannion R
muscle twitch
Neurodisability
Neurologic Disease
October 2019 List
Pain
Pain/dt [drug Therapy]
Palliative Care
pregabalin/dt [Drug Therapy]
pregabalin/pv [Special Situation for Pharmacovigilance]
Priority Journal
Retrospective Study
Review
treatment duration
treatment response
vomiting/dt [Drug Therapy]
vomiting/si [Side Effect]
-
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
August 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
August 2019 List
URL Address
<a href="http://doi.org/10.1002/ppul.24073" target="_blank" rel="noreferrer noopener">http://doi.org/10.1002/ppul.24073</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
Exploring knowledge and perceptions of palliative care to inform integration of palliative care education into cystic fibrosis care
Publisher
An entity responsible for making the resource available
Pediatric Pulmonology
Date
A point or period of time associated with an event in the lifecycle of the resource
2019
Subject
The topic of the resource
adolescent; adult; article; female; human; male; palliative therapy; clinical article; palliative care; health care personnel; priority journal; caregiver; parent; perception; communication; semi structured interview; adolescence; attitude to health; cystic fibrosis; health education; lung fibrosis; patient education; qualitative analysis
Creator
An entity primarily responsible for making the resource
Dellon E P; Helms S W; Hailey C E; Shay R; Carney S D; Schmidt H J; Brown D E; Prieur M G
Description
An account of the resource
Background: Individuals with cystic fibrosis (CF) face the challenges of managing a chronic, progressive disease. While palliative care is a standard of care in serious illnesses, there are no guidelines for its incorporation into CF care. Patients with CF, caregivers, and CF care providers may lack knowledge about palliative care and perceive barriers to integrated care. Objective(s): To: 1) explore knowledge and perceptions of palliative care among patients with CF, caregivers, and CF care providers; 2) solicit opinions about incorporating palliative care into routine CF care; and 3) solicit recommendations for CF-specific palliative care education for patients and caregivers. Method(s): We conducted semi-structured interviews with adult patients with CF, parents of adolescents with CF, and CF care providers to assess knowledge and perceptions of palliative care. Discussion included suggestions for palliative care education and integration into CF care. The sample was characterized using summary statistics. Key themes were identified using qualitative content analysis. Result(s): Ten patients with CF, ten parents, and eight CF care providers participated. Many had minimal knowledge of palliative care and endorsed the association with end of life as a barrier to palliative care, but after learning more about palliative care, thought it could be helpful, and should be introduced earlier. Conclusion(s): In this single center study, many patients with CF, caregivers, and providers lacked knowledge about palliative care. These findings warrant replication in a larger, multisite study to inform palliative care educational interventions as a step toward consistent integration of palliative care into routine CF care. Copyright � 2018 Wiley Periodicals, Inc.
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1002/ppul.24073" target="_blank" rel="noreferrer noopener">10.1002/ppul.24073</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).
2019
Adolescence
Adolescent
Adult
Article
Attitude To Health
August 2019 List
Brown D E
Caregiver
Carney S D
Clinical Article
Communication
Cystic Fibrosis
Dellon E P
Female
Hailey C E
Health Care Personnel
Health Education
Helms S W
Human
lung fibrosis
Male
Palliative Care
Palliative Therapy
Parent
Patient Education
Pediatric Pulmonology
Perception
Prieur M G
Priority Journal
Qualitative Analysis
Schmidt H J
Semi Structured Interview
Shay 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
May 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
May 2019 List
URL Address
<a href="http://doi.org/10.1080/17518423.2018.1461946" target="_blank" rel="noreferrer noopener">http://doi.org/10.1080/17518423.2018.1461946</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
Physicians' attitudes when faced with life-threatening events in children with severe neurological disabilities
Publisher
An entity responsible for making the resource available
Developmental Neurorehabilitation
Date
A point or period of time associated with an event in the lifecycle of the resource
2019
Subject
The topic of the resource
human; terminal care; female; male; palliative therapy; emergency ward; major clinical study; resuscitation; article; adult; patient care; intensive care; health care access; priority journal; follow up; risk factor; physician attitude; personal experience; cross-sectional study; tracheostomy; disability; spinal muscular atrophy; Likert scale; clinical decision making; childhood disease/ep [Epidemiology]; neurologic disease/ep [Epidemiology]; noninvasive ventilation; patient comfort; pediatric rehabilitation; profound intellectual and multiple disability; rehabilitation center; risk; Swiss
Creator
An entity primarily responsible for making the resource
Wosinski B; Newman C J
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1080/17518423.2018.1461946" target="_blank" rel="noreferrer noopener">10.1080/17518423.2018.1461946</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).
Description
An account of the resource
Purpose: Children with severe neurological disabilities are at an increased risk of acute, life-threatening events. We assessed physicians' attitudes when making decisions in these situations.
2019
Adult
Article
childhood disease/ep [Epidemiology]
Clinical Decision Making
Cross-sectional Study
Developmental Neurorehabilitation
Disability
Emergency Ward
Female
Follow Up
Health Care Access
Human
Intensive Care
Likert scale
Major Clinical Study
Male
May 2019 List
neurologic disease/ep [Epidemiology]
Newman C J
Noninvasive Ventilation
Palliative Therapy
Patient Care
Patient Comfort
Pediatric Rehabilitation
Personal Experience
Physician Attitude
Priority Journal
profound intellectual and multiple disability
rehabilitation center
Resuscitation
Risk
risk factor
Spinal Muscular Atrophy
Swiss
Terminal Care
Tracheostomy
Wosinski 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
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/10.1007/s00431-018-3170-6" target="_blank" rel="noreferrer noopener">http://doi.o
rg/10.1007/s00431-018-3170-6</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 timing and circumstances of the implementation of pediatric palliative care in Hungarian pediatric oncology
Publisher
An entity responsible for making the resource available
European Journal of Pediatrics
Date
A point or period of time associated with an event in the lifecycle of the resource
2018
Subject
The topic of the resource
child death; parent; family; terminal care; priority journal; psychologist; doctor patient relation; constructive feedback; childhood cancer; cancer palliative therapy; cohort analysis; oncologist; structured interview; human; article; female; male; adult; exploratory research; time to treatment; ambivalence; data analysis software; Hungarian (citizen); Hungary
Creator
An entity primarily responsible for making the resource
Nyiro J; Zorgo S; Eniko F; Hegedus K; Hauser P
Description
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Despite the continuous improvement of pediatric palliative care, medical professionals still face various barriers regarding its implementation; our aim was to investigate this question in Hungarian pediatric oncology practice. Structured interviews were carried out in person with physicians from the Hungarian Pediatric Oncology Group (n = 22). Codes were generated inductively with the aid of Atlas.ti 6.0 software. Most physicians placed the palliative care discussion at the end of curative treatment (n = 21) and preferred to conduct it in a team setting (n = 18), mainly in the presence of a psychologist. Preparing parents for the child's death can occur during the palliative care discussion (n = 3), in the child's final days/h (n = 6), gradually (n = 10), or never (n = 3). There are words consciously utilized and avoided during this discussion, with the word "death" proving to be the most ambivalent (utilized n = 5, avoided n = 6). Conclusions: There is no widely accepted unified practice among pediatric oncologists concerning the implementation of palliative care in Hungary. Despite the international recommendation, the common practice of timing is still at the end of curative treatment. Physicians rely on multidisciplinary teamwork, where the psychologist's role is the most prominent in this discussion.What is Known:* There is an international consensus that palliative care should commence at the diagnosis of a pediatric malignant disease regardless of illness outcome.* Barriers to the early implementation of palliative care in pediatric oncology involve resource-based and attitudinal factors.What is New:* In Hungary, where pediatric oncologists are sole decision-makers, early implementation of palliative care is rare.* There is a strong preference among physicians for working within a team, while also asserting that presence of team members may decrease the level of intimacy.
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<a href="http://doi.org/%2010.1007/s00431-018-3170-6" target="_blank" rel="noreferrer noopener">10.1007/s00431-018-3170-6</a>
2018
Adult
ambivalence
Article
Cancer Palliative Therapy
Child Death
Childhood Cancer
Cohort Analysis
constructive feedback
Data Analysis Software
Doctor Patient Relation
Eniko F
European Journal of Pediatrics
Exploratory Research
Family
Female
Hauser P
Hegedus K
Human
Hungarian (citizen)
Hungary
Male
Nyiro J
Oncologist
Oncology 2018 List
Parent
Priority Journal
Psychologist
Structured Interview
Terminal Care
time to treatment
Zorgo S
-
Dublin Core
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Title
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February 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
February 2019 List
URL Address
<a href="http://doi.org/10.1038/s41525-018-0049-4" target="_blank" rel="noreferrer noopener"> http://doi.o
rg/10.1038/s41525-018-0049-4</a>
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Rapid whole-genome sequencing decreases infant morbidity and cost of hospitalization
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NLJ Genomic Medicine
Date
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2018
Subject
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infant; childhood mortality; retrospective study; priority journal; cohort analysis; human; article; female; male; controlled study; clinical article; palliative therapy; cost control; hospitalization cost; infant disease/di [Diagnosis]; infant disease/dm [Disease Management]; rapid whole genome sequencing; whole genome sequencing; acutely ill patient; diagnostic test accuracy study; diagnostic value; genetic screening; mortality risk; risk reduction; sensitivity and specificity
Creator
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Farnaes L; Hildreth A; Sweeney NM; Clark MM; Chowdhury S; Nahas S; Cakici JA; Benson W; Kaplan RH; Kronick R; Bainbridge MN; Friedman J; Gold JJ; Ding Y; Veeraraghavan N; Dimmock D; Kingsmore SF
Description
An account of the resource
Genetic disorders are a leading cause of morbidity and mortality in infants. Rapid whole-genome sequencing (rWGS) can diagnose genetic disorders in time to change acute medical or surgical management (clinical utility) and improve outcomes in acutely ill infants. We report a retrospective cohort study of acutely ill inpatient infants in a regional children's hospital from July 2016-March 2017. Forty-two families received rWGS for etiologic diagnosis of genetic disorders. Probands also received standard genetic testing as clinically indicated. Primary end-points were rate of diagnosis, clinical utility, and healthcare utilization. The latter was modelled in six infants by comparing actual utilization with matched historical controls and/or counterfactual utilization had rWGS been performed at different time points. The diagnostic sensitivity of rWGS was 43% (eighteen of 42 infants) and 10% (four of 42 infants) for standard genetic tests (P =.0005). The rate of clinical utility of rWGS (31%, thirteen of 42 infants) was significantly greater than for standard genetic tests (2%, one of 42; P =.0015). Eleven (26%) infants with diagnostic rWGS avoided morbidity, one had a 43% reduction in likelihood of mortality, and one started palliative care. In six of the eleven infants, the changes in management reduced inpatient cost by $800,000-$2,000,000. These findings replicate a prior study of the clinical utility of rWGS in acutely ill inpatient infants, and demonstrate improved outcomes and net healthcare savings. rWGS merits consideration as a first tier test in this setting.
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<a href="http://doi.org/10.1038/s41525-018-0049-4" target="_blank" rel="noreferrer noopener">10.1038/s41525-018-0049-4</a>
2018
acutely ill patient
Article
Bainbridge MN
Benson W
Cakici JA
Childhood Mortality
Chowdhury S
Clark MM
Clinical Article
Cohort Analysis
Controlled Study
Cost Control
diagnostic test accuracy study
Diagnostic Value
Dimmock D
Ding Y
Farnaes L
February 2019 List
Female
Friedman J
genetic screening
Gold JJ
Hildreth A
hospitalization cost
Human
Infant
infant disease/di [Diagnosis]
infant disease/dm [Disease Management]
Kaplan RH
Kingsmore SF
Kronick R
Male
mortality risk
Nahas S
NLJ Genomic Medicine
Palliative Therapy
Priority Journal
rapid whole genome sequencing
Retrospective Study
risk reduction
Sensitivity and Specificity
Sweeney NM
Veeraraghavan N
whole genome sequencing
-
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Title
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November 2018 List
Text
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Citation List Month
November 2018 List
URL Address
<a href="http://doi.org/10.1111/apa.14331" target="_blank" rel="noreferrer noopener">http://doi.org/10.1111/apa.14331</a>
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All-cause mortality rates and home deaths decreased in children with life-limiting diagnoses in Denmark between 1994 and 2014
Publisher
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Acta Paediatrica, International Journal of Paediatrics
Date
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2018
Subject
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all cause mortality;childhood mortality;death;home;accident;adolescent;adult;article;automutilation;Epidemiology;Child;chromosome aberration;Epidemiology;congenital malformation;Epidemiology;Denmark;human;infant;infant mortality;International Classification of Diseases;neoplasm;Epidemiology;perinatal death;priority journal
Creator
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Lykke C;Ekholm O;Schmiegelow K;Olsen M;Sjogren P
Description
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Aim: Specialised paediatric palliative care has not previously been a priority in Denmark. The aim of this study was to support its development and organisation, by examining why and where children died using official national data for 1994-2014. Methods: We obtained data on 9462 children who died before the age of 18 from the Danish Register of Causes of Death. The causes of deaths were listed according to the codes in the International Classification of Diseases. Results: The all-cause mortality rate decreased by 52% over the study period, and infants below one year accounted for 61% of all deaths. The decline in infant mortality (26%) primarily reflected fewer deaths due to congenital malformations and chromosomal abnormalities (68%) and perinatal deaths (30%). In children aged one year to 17 years, the substantial decrease (65%) was due to external causes (75%) and neoplasms (57%). The relative proportion of hospital deaths increased, while home deaths decreased. Conclusion: All-cause mortality rate decreased markedly, and the relative proportion of hospital deaths increased. The results may reflect more aggressive and effective treatment attempts to save lives, but some terminally ill children may be deprived of the option of dying at home.
Identifier
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<a href="http://doi.org/10.1111/apa.14331" target="_blank" rel="noreferrer noopener">10.1111/apa.14331</a>
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Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
2018
accident
Acta Paediatrica, International Journal of Paediatrics
Adolescent
Adult
all cause mortality
Article
automutilation
Child
Childhood Mortality
Chromosome Aberration
Congenital Malformation
Death
Denmark
Ekholm O
Epidemiology
Home
Human
Infant
Infant Mortality
International Classification of Diseases
Lykke C
Neoplasm
November 2018 List
Olsen M
Perinatal Death
Priority Journal
Schmiegelow K
Sjogren P
-
Dublin Core
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Title
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November 2018 List
Text
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Citation List Month
November 2018 List
URL Address
<a href="http://doi.org/10.1136/bmjpo-2017-000102" target="_blank" rel="noreferrer noopener">http://doi.org/10.1136/bmjpo-2017-000102</a>
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Title
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Advance care planning for adolescent patients with life-threatening neurological conditions: A survey of Japanese paediatric neurologists
Publisher
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BMJ Paediatrics Open
Date
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2017
Subject
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Advance care planning;neurologic disease;neurologist;pediatrician;Adult;article;artificial ventilation;cross-sectional study;do not resuscitate order;Family;Female;health care survey;hematologist;Human;Japan;living will;Male;Middle Aged;priority journal;prognosis;terminal care
Creator
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Yotani N;Kizawa Y;Shintaku H
Description
An account of the resource
Objective To evaluate current attitudes and barriers to advance care planning for adolescent patients with life-threatening conditions among paediatric neurologists. Design Cross-sectional study. A self-reported questionnaire was administered to assess the practice of advance care planning, advance directives and barriers to advance care planning for adolescent patients with life-threatening conditions. All board-certified paediatric neurologists in Japan were surveyed and those who had experience in taking care of adolescent patients with decision-making capacity were analysed. We compared the results with those of paediatric haematologists reported previously. results In total, 186 paediatric neurologists were analysed. If the patient's prognosis was <3 months, only about 30% of paediatric neurologists reported having discussions with patients, such as 'do not attempt resuscitation' orders (28%) and ventilator use (32%), whereas more than 70% did discuss these topics with patients' families. About half of the paediatric neurologists did not discuss advance directives at the end of life with their patients, whereas over 75% did discuss advance directives with patients' families. Compared with paediatric haematologists, paediatric neurologists had more end-of-life discussions with patients, such as where treatment and care will take place, do not attempt resuscitation orders, and the use of a ventilator, if the patient's prognosis was >>1 year. conclusion About half or less of the paediatric neurologists discussed advance care planning and advance directives with their adolescent patients who had life-threatening conditions, even if the patient's prognosis was <3 months. They tended to discuss advance care planning and advance directives more with families than with patients themselves.
Identifier
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<a href="http://doi.org/10.1136/bmjpo-2017-000102" target="_blank" rel="noreferrer noopener">10.1136/bmjpo-2017-000102</a>
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Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
2017
Adult
Advance Care Planning
Article
Artificial Ventilation
BMJ Paediatrics Open
Cross-sectional Study
do not resuscitate order
Family
Female
Health Care Survey
hematologist
Human
Japan
Kizawa Y
living will
Male
Middle Aged
Neurologic Disease
neurologist
November 2018 List
Pediatrician
Priority Journal
Prognosis
Shintaku H
Terminal Care
Yotani N
-
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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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November 2018 List
Text
A resource consisting primarily of words for reading. Examples include books, letters, dissertations, poems, newspapers, articles, archives of mailing lists. Note that facsimiles or images of texts are still of the genre Text.
Citation List Month
November 2018 List
URL Address
<a href="http://doi.org/10.1111/apa.14394" target="_blank" rel="noreferrer noopener">http://doi.org/10.1111/apa.14394</a>
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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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Low prevalence of palliative care and ethics consultations for children with chronic critical illness
Publisher
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Acta Paediatrica, International Journal of Paediatrics
Date
A point or period of time associated with an event in the lifecycle of the resource
2018
Subject
The topic of the resource
childhood disease th [Therapy];Chronic disease;critical illness;Ethics;Palliative therapy;article;artificial ventilation;consultation;hospital admission;Human;intensive care unit;Length of Stay;prevalence;priority journal;tracheostomy
Creator
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Boss RD;Falck A;Goloff N;Hutton N;Miles A;Shapiro M;Weiss EM;Donohue PK
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<a href="http://doi.org/10.1111/apa.14394" target="_blank" rel="noreferrer noopener">10.1111/apa.14394</a>
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Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
Description
An account of the resource
Medical advances over the past two decades have increased the numbers of children who survive serious conditions. Mortality from premature birth and genetic syndromes has improved such that more clinicians offer, and more families request, interventions to prolong their child's life. While some interventions promise cure, others result in chronic disease states that require ongoing medical care.
Paediatric intensive care units (ICUs) across the United States (U.S.) report increasing numbers of children with prolonged and recurrent hospitalisations and medical complexity 1. We have suggested that this population be considered ‘chronically critically ill (CCI)’ 2. The CCI designation for adult ICU patients signals to families and clinicians alike that the patient's prognosis is poorer than predicted by their primary diagnosis alone.
2018
Acta Paediatrica, International Journal of Paediatrics
Article
Artificial Ventilation
Boss RD
childhood disease th [Therapy]
Chronic Disease
Consultation
Critical Illness
Donohue PK
Ethics
Falck A
Goloff N
Hospital Admission
Human
Hutton N
Intensive Care Unit
Length Of Stay
Miles A
November 2018 List
Palliative Therapy
Prevalence
Priority Journal
Shapiro M
Tracheostomy
Weiss EM
-
Text
A resource consisting primarily of words for reading. Examples include books, letters, dissertations, poems, newspapers, articles, archives of mailing lists. Note that facsimiles or images of texts are still of the genre Text.
Citation List Month
Backlog
URL Address
<a href="http://doi.org/10.1097/NCC.0000000000000146" target="_blank" rel="noreferrer">http://doi.org/10.1097/NCC.0000000000000146</a>
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Facebook Advertisements Recruit Parents of Children With Cancer for an Online Survey of Web-Based Research Preferences
Publisher
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Cancer Nursing
Date
A point or period of time associated with an event in the lifecycle of the resource
2014
Subject
The topic of the resource
Child; Female; Male; Income; adolescent; Human; oncology; Diseases; Sample Size; Population; Article; Child; Caregiver; advertizing; cancer research; Caregiver; childhood cancer; Human; Internet; neoplasm; parent; social media; social network; cancer palliative therapy; cancer patient; cancer recurrence; click; computer; Facebook; health survey; information processing; interpersonal communication; legal guardian; major clinical study; mobile phone; nurse; Only Child; Palliative therapy; parent; parental attitude; participant recruitment; Patient; priority journal; school child; social media; Video Recording; Video Recording
Creator
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Akard TF; Wray S; Gilmer Mary Jo
Description
An account of the resource
BACKGROUND:: Studies involving samples of children with life-threatening illnesses and their families face significant challenges, including inadequate sample sizes and limited diversity. Social media recruitment and Web-based research methods may help address such challenges yet have not been explored in pediatric cancer populations. OBJECTIVE:: This study examined the feasibility of using Facebook advertisements to recruit parent caregivers of children and teenagers with cancer. We also explored the feasibility of Web-based video recording in pediatric palliative care populations by surveying parents of children with cancer regarding (a) their preferences for research methods and (b) technological capabilities of their computers and phones. METHODS:: Facebook's paid advertising program was used to recruit parent caregivers of children currently living with cancer to complete an electronic survey about research preferences and technological capabilities. RESULTS:: The advertising campaign generated 3 897 981 impressions, which resulted in 1050 clicks at a total cost of $1129.88. Of 284 screened individuals, 106 were eligible. Forty-five caregivers of children with cancer completed the entire electronic survey. Parents preferred and had technological capabilities for Web-based and electronic research methods. Participant survey responses are reported. CONCLUSION:: Facebook was a useful, cost-effective method to recruit a diverse sample of parent caregivers of children with cancer. Web-based video recording and data collection may be feasible and desirable in samples of children with cancer and their families. IMPLICATIONS FOR PRACTICE:: Web-based methods (eg, Facebook, Skype) may enhance communication and access between nurses and pediatric oncology patients and their families.
2014-06
Identifier
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<a href="http://doi.org/10.1097/NCC.0000000000000146" target="_blank" rel="noreferrer">10.1097/NCC.0000000000000146</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).
Type
The nature or genre of the resource
Journal Article
2014
Adolescent
advertizing
Akard TF
Article
Backlog
Cancer Nursing
Cancer Palliative Therapy
Cancer Patient
Cancer Recurrence
Cancer Research
Caregiver
Child
Childhood Cancer
click
computer
Diseases
Facebook
Female
Gilmer Mary Jo
Health Survey
Human
Income
Information Processing
Internet
Interpersonal Communication
Journal Article
legal guardian
Major Clinical Study
Male
mobile phone
Neoplasm
Nurse
Oncology
Only Child
Palliative Therapy
Parent
Parental Attitude
participant recruitment
Patient
Population
Priority Journal
Sample Size
School Child
social media
social network
Video Recording
Wray S
-
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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March 2018 List
Text
A resource consisting primarily of words for reading. Examples include books, letters, dissertations, poems, newspapers, articles, archives of mailing lists. Note that facsimiles or images of texts are still of the genre Text.
Citation List Month
March 2018 List
URL Address
<a href="http://doi.org/10.1542/peds.2012-1916" target="_blank" rel="noreferrer">http://doi.org/10.1542/peds.2012-1916</a>
Dublin Core
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Parental perceptions of forgoing artificial nutrition and hydration during end-of-life care
Publisher
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Pediatrics
Date
A point or period of time associated with an event in the lifecycle of the resource
2013
Subject
The topic of the resource
artificial feeding; concept formation; hydration; Terminal Care; Article; Child care; child death; clinical practice; Controlled Study; decision making; Female; Human; Male; parental attitude; personal experience; priority journal; Qualitative Research; quality of life; Religion; satisfaction
Creator
An entity primarily responsible for making the resource
Rapoport A; Shaheed J; Newman C; Rugg M; Steele R
Description
An account of the resource
BACKGROUND AND OBJECTIVE: Forgoing artificial nutrition and hydration (FANH) in children at the end of life (EOL) is a medically, legally, and ethically acceptable practice under speci fi c circumstances. However, most of the evidence on FANH involves dying adults. There is a paucity of pediatric evidence to guide health care providers' and parents' decision-making around this practice. Objectives were (1) to explore the experiences of bereaved parents when a decision had been made to FANH during EOL care for their child and (2) to describe the perceived quality of death in these children, as reported by their parents. METHODS: This was a qualitative study using in-depth interviews with parents whose children died after a decision to FANH. Parental perceptions about the experience and their child's quality of death were explored. Interviews were audiotaped and transcribed, then data were analyzed by using interpretive description methodology. RESULTS: All parents were satisfi ed with their decision to FANH and believed that their child's death was generally peaceful and comfortable. The child's perceived poor quality of life was central to the decision to FANH, with feeding intolerance often contributing to this perception. Despite overall satisfaction, all parents had doubts and questions about the decision and benefited from ongoing assurances from the clinical team. CONCLUSIONS: FANH in children at the EOL is an acceptable form of palliation for some parents and may contribute to a death that is perceived to be peaceful and comfortable. In situations in which FANH may be a reasonable possibility, physicians should be prepared to introduce the option. Copyright _ 2013 by the American Academy of Pediatrics.
2013
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1542/peds.2012-1916" target="_blank" rel="noreferrer">10.1542/peds.2012-1916</a>
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Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
2013
Article
Artificial Feeding
Child Care
Child Death
Clinical Practice
Concept Formation
Controlled Study
Decision Making
Female
Human
Hydration
Male
March 2018 List
Newman C
Parental Attitude
Pediatrics
Personal Experience
Priority Journal
Qualitative Research
Quality Of Life
Rapoport A
Religion
Rugg M
Satisfaction
Shaheed J
Steele R
Terminal Care