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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/ppul.21291" target="_blank" rel="noreferrer noopener">http://doi.org/10.1002/ppul.21291</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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The feasibility and validity of forced spirometry in ataxia telangiectasia
Publisher
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Pediatric Pulmonology
Date
A point or period of time associated with an event in the lifecycle of the resource
2010
Subject
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Adolescent; Ataxia Telangiectasia/di [Diagnosis]; Ataxia Telangiectasia/pp [Physiopathology]; Case-Control Studies; Child; Preschool; Feasibility Studies; Female; Humans; Male; Reproducibility of Results; Spirometry; Young Adult; breathing difficulties; Ataxia Telangiectasia; trajectory; characteristics; lung deterioration
Creator
An entity primarily responsible for making the resource
Vilozni D; Berkun Y; Levi Y; Weiss B; Jacobson J M; Efrati O
Description
An account of the resource
OBJECTIVES: To explore the feasibility and validity of forced spirometry in patients with ataxia telangiectasia (A-T). STUDY DESIGN: Twenty-eight patients (aged 3.7-19.3 years) performed spirometry on 47 occasions. Parameters studied were technical quality and relation to: predicted values, pulmonary illness. RESULTS: Start of test criteria for correct expiratory effort was significantly prolonged (183 +/- 115 ms; P < 0.001). The rise-time to peak flow in children free of respiratory symptoms (Group-FRS; n = 8) increased by 16.2 +/- 12.5 ms/year above recommended and in children having recurrent infections (n = 8) 30.4 +/- 16.1 ms/year, P < 0.01. Expiration-time was significantly shorter than requested (1.21 +/- 0.47 sec) and was ended abruptly in 57% of the patients. FEV(1) could not be established by 8/20 patients. The intra-subject reproducibility met criteria (4.4 +/- 2.7%, 5.2 +/- 2.8%, 2.9 +/- 3.2%, 6.3 +/- 5.3%, for FVC, FEV(0.5), PEF, FEF(25-75), respectively). Group-FRS showed yearly deterioration in FVC of 2.2%, while patients with hyper-reactive airways (Group-HRA; n =12) had a deterioration rate of 3.6%/year. FEV(0.5) deterioration rate was similar in both groups (2.2 and 2.0, respectively), but baseline values in Group-HRA were significantly lower than those of Group-FRS (P = 0.029) in similar young ages, indicating airway obstruction at early ages in Group-HRA. FEV(0.5) values deterioration also correlated with body mass index (P< 0.017). CONCLUSION: Forced spirometry in A-T patients is reproducible and has a distinct pattern, although curves do not meet other recommendations for acceptable criteria. The study insinuates that a rapid deterioration in lung function occurs in A-T patients with recurrent respiratory infection, suggesting that early intervention may prevent further deterioration or improve their lung function. Further studies are needed to confirm our results.Copyright © 2010 Wiley-Liss, Inc.
Identifier
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<a href="http://doi.org/10.1002/ppul.21291" target="_blank" rel="noreferrer noopener">10.1002/ppul.21291</a>
Rights
Information about rights held in and over the resource
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
2010
Adolescent
ataxia telangiectasia
ataxia telangiectasia/di [Diagnosis]
Ataxia Telangiectasia/pp [Physiopathology]
Berkun Y
breathing difficulties
Case-Control Studies
characteristics
Child
Efrati O
Feasibility Studies
Female
Humans
Jacobson J M
Levi Y
lung deterioration
Male
Pediatric Pulmonology
Preschool
Reproducibility of Results
Spirometry
Trajectory
Vilozni D
Weiss B
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/10.1371/journal.pone.0067042" target="_blank" rel="noreferrer noopener">http://doi.org/10.1371/journal.pone.0067042</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
Disorders of Upper Limb Movements in Ataxia-Telangiectasia
Publisher
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PLoS ONE
Date
A point or period of time associated with an event in the lifecycle of the resource
2013
Subject
The topic of the resource
Cross-Sectional Studies; Age Factors; Male; Severity of Illness Index; Young Adult; Child; Humans; Adult; Adolescent; Female; Child Preschool; Periodicity; Ataxia Telangiectasia/pp [Physiopathology]; Motor Activity; Movement; Upper Extremity; Accelerometry; Biomechanical Phenomena; Motor Activity/ph [Physiology]; Movement Disorders/pp [Physiopathology]; Movement/ph [Physiology]; Posture/ph [Physiology]; Tremor/pp [Physiopathology]; Upper Extremity/pp [Physiopathology]; tone and motor problems; ataxia telangiectasia; trajectory; characteristics; abnormal involuntary movements; kinetic tremor; postural tremor; resting tremor
Creator
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Shaikh A G; Zee D S; Mandir A S; Lederman H M; Crawford T O
Description
An account of the resource
Ataxia-telangiectasia is known for cerebellar degeneration, but clinical descriptions of abnormal tone, posture, and movements suggest involvement of the network between cerebellum and basal ganglia. We quantitatively assessed the nature of upper-limb movement disorders in ataxia-telangiectasia. We used a three-axis accelerometer to assess the natural history and severity of abnormal upper-limb movements in 80 ataxia-telangiectasia and 19 healthy subjects. Recordings were made during goal-directed movements of upper limb (kinetic task), while arms were outstretched (postural task), and at rest. Almost all ataxia-telangiectasia subjects (79/80) had abnormal involuntary movements, such as rhythmic oscillations (tremor), slow drifts (dystonia or athetosis), and isolated rapid movements (dystonic jerks or myoclonus). All patients with involuntary movements had both kinetic and postural tremor, while 48 (61%) also had resting tremor. The tremor was present in transient episodes lasting several seconds during two-minute recording sessions of all three conditions. Percent time during which episodic tremor was present was greater for postural and kinetic tasks compared to rest. Resting tremor had higher frequency but smaller amplitude than postural and kinetic tremor. Rapid non-rhythmic movements were minimal during rest, but were triggered during sustained arm postures and goal directed arm movements suggesting they are best considered a form of dystonic jerks or action myoclonus. Advancing age did not correlate with the severity of involuntary limb movements. Abnormal upper-limb movements in ataxia-telangiectasia feature classic cerebellar impairment, but also suggest involvement of the network between the cerebellum and basal ganglia.
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1371/journal.pone.0067042" target="_blank" rel="noreferrer noopener">10.1371/journal.pone.0067042</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
abnormal involuntary movements
accelerometry
Adolescent
Adult
Age Factors
ataxia telangiectasia
Ataxia Telangiectasia/pp [Physiopathology]
Biomechanical Phenomena
characteristics
Child
Child Preschool
Crawford T O
Cross-sectional Studies
Female
Humans
kinetic tremor
Lederman H M
Male
Mandir A S
Motor Activity
Motor Activity/ph [Physiology]
Movement
Movement Disorders/pp [Physiopathology]
Movement/ph [Physiology]
Periodicity
PLoS One
postural tremor
Posture/ph [Physiology]
resting tremor
Severity Of Illness Index
Shaikh A G
tone and motor problems
Trajectory
Tremor/pp [Physiopathology]
Upper Extremity
Upper Extremity/pp [Physiopathology]
Young Adult
Zee D S