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Dublin Core
The Dublin Core metadata element set is common to all Omeka records, including items, files, and collections. For more information see, http://dublincore.org/documents/dces/.
Title
A name given to the resource
Treatment of Symptoms in Children with Q3 Conditions Scoping Review Results
Text
A resource consisting primarily of words for reading. Examples include books, letters, dissertations, poems, newspapers, articles, archives of mailing lists. Note that facsimiles or images of texts are still of the genre Text.
URL Address
<a href="http://doi.org/10.1016/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
A name given to the resource
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>
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).
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
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Dublin Core
The Dublin Core metadata element set is common to all Omeka records, including items, files, and collections. For more information see, http://dublincore.org/documents/dces/.
Title
A name given to the resource
Treatment of Symptoms in Children with Q3 Conditions Scoping Review Results
Text
A resource consisting primarily of words for reading. Examples include books, letters, dissertations, poems, newspapers, articles, archives of mailing lists. Note that facsimiles or images of texts are still of the genre Text.
URL Address
<a href="http://doi.org/10.1016/j.ejpn.2017.09.003" target="_blank" rel="noreferrer noopener">http://doi.org/10.1016/j.ejpn.2017.09.003</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
Intrathecal baclofen treatment an option in X-linked adrenoleukodystrophy
Publisher
An entity responsible for making the resource available
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
An unambiguous reference to the resource within a given context
<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