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40
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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
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Treatment of Symptoms in Children with Q3 Conditions Scoping Review Results
Text
A resource consisting primarily of words for reading. Examples include books, letters, dissertations, poems, newspapers, articles, archives of mailing lists. Note that facsimiles or images of texts are still of the genre Text.
URL Address
<a href="http://doi.org/10.1002/mds.25126" target="_blank" rel="noreferrer noopener">http://doi.org/10.1002/mds.25126</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 randomized trial of oral betamethasone to reduce ataxia symptoms in ataxia telangiectasia
Publisher
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Movement Disorders
Date
A point or period of time associated with an event in the lifecycle of the resource
2012
Subject
The topic of the resource
tone and motor problems; ataxia; ataxia telangectasia; pharmacologic intervention; betamethasone;
Creator
An entity primarily responsible for making the resource
Zannolli R; Buoni S; Betti G; Salvucci S; Plebani A; Soresina A; Pietrogrande M C; Martino S; Leuzzi V; Finocchi A; Micheli R; Rossi L N; Brusco A; Misiani F; Fois A; Hayek J; Kelly C; Chessa L
Description
An account of the resource
No controlled studies exist regarding the pharmaceutical reduction of ataxia symptoms in ataxia telangiectasia (A-T). In a multicenter, double-blind, randomized, placebo-controlled crossover trial, oral betamethasone (BETA) and placebo were compared in terms of their reduction of ataxia symptoms as assessed with the International Cooperative Ataxia Rating Scale (ICARS). In this study of 13 A-T children, betamethasone reduced the ICARS total score by a median of 13 points in the intent-to-treat population and 16 points in the per-protocol population (ie, median percent decreases of ataxia symptoms of 28% and 31%, respectively). In conclusion, Oral betamethasone could be a promising therapy to relieve ataxia symptoms in A-T patients; however, long-term effectiveness and safety must be established. (Current Controlled Trials, number ISRCTN08774933). © 2012 Movement Disorder Society.
Identifier
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<a href="http://doi.org/10.1002/mds.25126" target="_blank" rel="noreferrer noopener">10.1002/mds.25126</a>
Rights
Information about rights held in and over the resource
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
2012
ataxia
ataxia telangectasia
betamethasone
Betti G
Brusco A
Buoni S
Chessa L
Finocchi A
Fois A
Hayek J
Kelly C
Leuzzi V
Martino S
Micheli R
Misiani F
Movement Disorders
pharmacologic intervention
Pietrogrande M C
Plebani A
Rossi L N
Salvucci S
Soresina A
tone and motor problems
Zannolli R
-
Dublin Core
The Dublin Core metadata element set is common to all Omeka records, including items, files, and collections. For more information see, http://dublincore.org/documents/dces/.
Title
A name given to the resource
Treatment of Symptoms in Children with Q3 Conditions Scoping Review Results
Text
A resource consisting primarily of words for reading. Examples include books, letters, dissertations, poems, newspapers, articles, archives of mailing lists. Note that facsimiles or images of texts are still of the genre Text.
URL Address
<a href="http://doi.org/10.1111/dmcn.13156" target="_blank" rel="noreferrer noopener">http://doi.org/10.1111/dmcn.13156</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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Assessment of impaired coordination between respiration and deglutition in children and young adults with ataxia telangiectasia
Publisher
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Developmental Medicine and Child Neurology
Date
A point or period of time associated with an event in the lifecycle of the resource
2016
Subject
The topic of the resource
Pediatrics; dysphagia; patterns; Neurosciences & Neurology; management; cerebral-palsy; disease; dynamics; neurodegeneration; pharyngeal; feeding difficulties; ataxia telangectasia; trajectory; characteristics; expiratory patterns of peri-deglutitive airflow
Creator
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Lefton-Greif M A; Perlman A L; He X M; Lederman H M; Crawford T O
Description
An account of the resource
AimThis cross-sectional investigation aimed to assess the value of non-invasive measures of temporal respiratory-swallow coupling in individuals with ataxic swallowing. MethodTwenty participants (11 males, 9 females; range 9-21y) with ataxia telangiectasia were presented with water and pudding boluses. Their 193 swallows were compared with 2200 swallows from 82 age-matched healthy controls. The two components of airway protection during swallowing that were analyzed were: direction of peri-deglutitive airflow and duration of deglutitive inhibition of respiratory airflow (DIORA). ResultsSafe expiratory patterns of peri-deglutitive airflow occurred significantly less often in participants with ataxia telangiectasia than in age-matched control participants (younger p<0.015 and older p<0.001). The frequency of an expiratory pattern of peri-deglutitive airflow increased with age in participants in the comparison group (p=0.006), but not in those with ataxia telangiectasia (p=0.234). With age, mean duration of DIORA decreased in controls (p<0.001) but was unchanged in participants with ataxia telangiectasia (p=0.164). InterpretationNon-invasive quantitative measures of respiratory-swallow coupling capture temporal relationships that plausibly contribute to airway compromise from dysphagia. Changes in respiratory-swallow coupling observed with advancing age in control participants were not seen in participants with ataxia telangiectasia. Measures of perturbations may herald swallowing problems prior to development of pulmonary and nutritional sequelae.
Identifier
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<a href="http://doi.org/10.1111/dmcn.13156" target="_blank" rel="noreferrer noopener">10.1111/dmcn.13156</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
ataxia telangectasia
cerebral-palsy
characteristics
Crawford T O
Developmental Medicine and Child Neurology
Disease
dynamics
Dysphagia
expiratory patterns of peri-deglutitive airflow
feeding difficulties
He X M
Lederman H M
Lefton-Greif M A
Management
neurodegeneration
Neurosciences & Neurology
Patterns
Pediatrics
Perlman A L
pharyngeal
Trajectory
-
Dublin Core
The Dublin Core metadata element set is common to all Omeka records, including items, files, and collections. For more information see, http://dublincore.org/documents/dces/.
Title
A name given to the resource
Treatment of Symptoms in Children with Q3 Conditions Scoping Review Results
Text
A resource consisting primarily of words for reading. Examples include books, letters, dissertations, poems, newspapers, articles, archives of mailing lists. Note that facsimiles or images of texts are still of the genre Text.
URL Address
<a href="http://doi.org/10.1016/s0022-3476(00)70106-5" target="_blank" rel="noreferrer noopener">http://doi.org/10.1016/s0022-3476(00)70106-5</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
Oropharyngeal dysphagia and aspiration in patients with ataxia-telangiectasia
Publisher
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Journal of Pediatrics
Date
A point or period of time associated with an event in the lifecycle of the resource
2000
Subject
The topic of the resource
children; Pediatrics; feeding difficulties; ataxia telangectasia; trajectory; characteristics; dysphagia aspiration; low weight
Creator
An entity primarily responsible for making the resource
Lefton-Greif M A; Crawford T O; Winkelstein J A; Loughlin G M; Koerner C B; Zaburak M; Lederman H M
Description
An account of the resource
Objectives: To determine whether patients with ataxia-telangiectasia exhibit oropharyngeal dysphagia with concomitant aspiration and to examine the relationships among swallowing function, age, and nutritional status. Study design: Seventy patients (mean age, 10.7 years; range,: 1.8 to 30 years) had feeding/swallowing and nutritional evaluations. Fifty-one patients, in whom there were concerns about swallowing safety, were examined with a standardized videofluoroscopic swallow study. Results: Fourteen of the 51 patients (27%) with histories suggestive of dysphagia demonstrated aspiration. Of these, silent aspiration (aspiration without a cough) occurred in 10 (71%) patients. Aspirators were significantly older than non-aspirators (mean age, 16.9 vs 10.8 years; P = .002). Advancing age was the strongest factor associated with aspiration during continuous drinking (P = .01). In patients with ataxia-telangiectasia, weight and weight/height were abnormally low at all ages and most compromised in older patients. Patients who aspirated had significantly lower mean weighs (P < .002) and weight/height z scores (P < .001) than did patients who did not aspirate. Conclusions: Oropharyngeal dysphagia is common and appears to be progressive in patients with ataxia-telangiectasia. Older patients also have a higher incidence of poorer nutritional status. The relationship between dysphagia and nutritional status deserves further investigation.
Identifier
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<a href="http://doi.org/10.1016/s0022-3476(00)70106-5" target="_blank" rel="noreferrer noopener">10.1016/s0022-3476(00)70106-5</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).
2000
ataxia telangectasia
characteristics
Children
Crawford T O
dysphagia aspiration
feeding difficulties
Journal of Pediatrics
Koerner C B
Lederman H M
Lefton-Greif M A
Loughlin G M
low weight
Pediatrics
Trajectory
Winkelstein J A
Zaburak M