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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.20866" target="_blank" rel="noreferrer noopener">http://doi.org/10.1002/ppul.20866</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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Autonomic Dysregulation in Young Girls With Rett Syndrome During Nighttime in-Home Recordings
Publisher
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Pediatric Pulmonology
Date
A point or period of time associated with an event in the lifecycle of the resource
2008
Subject
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Pediatrics; patterns; autonomic dysregulation; cardiorespiratory dysregulation; disorder; disturbances; dysfunction; hyperventilation; mecp2 mutations; mice; nervous-system; Respiratory System; breathing difficulties; Rett syndrome; trajectory; characteristics; irregular breathing; increased breathing frequency
Creator
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Weese-Mayer D E; Lieske S P; Boothby C M; Kenny A S; Bennett H L; Ramirez J M
Description
An account of the resource
This study was designed to specifically characterize the autonomic phenotype of cardiorespiratory dysregulation during the nighttime in young girls with MECP2 mutation-confirmed Rett Syndrome (RS), studied in their home environment. Computerized breath-to-breath and beat-to-beat characterization of at-home continuously recorded respiratory inductance plethysmography of chest/abdomen and ECG (VivoMetrics, Inc.) was obtained during overnight recordings in 47 girls with MECP2 mutation-confirmed RS and 47 age-, gender-, and ethnicity-matched screened controls (ages 2-7 years). We determined that although the breathing and heart rate appear more regular during the night compared to the day, young girls with RS demonstrate apparent nocturnal irregularities. Comparing daytime versus nighttime, breathing was more irregular, with an increased breathing frequency (and irregularity), mean amplitude of respiratory inductance plethysmography sum (AMP)/T-I, and heart rate and decreased AMP in girls with RS. Comparing girls with RS versus controls during nighttime recording, breathing was more irregular, with an increased breathing frequency (and irregularity), mean AMP/T-I, and heart rate. An increased uncoupling between measures of breathing and heart rate control indicates malregulation in the autonomic nervous system, and is apparent during the day as well as the night. This uncoupling may represent a mechanism that renders the girls with RS more vulnerable to sudden death. Pediatr Pulmonol. 2008;43:1045-1060. (C) 2008 Wiley-Liss, Inc.
Identifier
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<a href="http://doi.org/10.1002/ppul.20866" target="_blank" rel="noreferrer noopener">10.1002/ppul.20866</a>
Rights
Information about rights held in and over the resource
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
2008
autonomic dysregulation
Bennett H L
Boothby C M
breathing difficulties
cardiorespiratory dysregulation
characteristics
Disorder
disturbances
dysfunction
hyperventilation
increased breathing frequency
irregular breathing
Kenny A S
Lieske S P
mecp2 mutations
Mice
nervous-system
Patterns
Pediatric Pulmonology
Pediatrics
Ramirez J M
Respiratory System
Rett syndrome
Trajectory
Weese-Mayer D 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/10.1203/01.pdr.0000238302.84552.d0" target="_blank" rel="noreferrer noopener">http://doi.org/10.1203/01.pdr.0000238302.84552.d0</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 nervous system dysregulation: Breathing and heart rate perturbation during wakefulness in young girls with Rett syndrome
Publisher
An entity responsible for making the resource available
Pediatric Research
Date
A point or period of time associated with an event in the lifecycle of the resource
2006
Subject
The topic of the resource
Pediatrics; patterns; disorder; dysfunction; hyperventilation; explanation; long qt syndrome; mecp2; mutations; sudden-death; breathing difficulties; Rett syndrome; trajectory; characteristics; irregular breathing; increased breathing frequency; increased mean airflow increased hear rate
Creator
An entity primarily responsible for making the resource
Weese-Mayer D E; Lieske S P; Boothby C M; Kenny A S; Bennett H L; Silvestri J M; Ramirez J M
Description
An account of the resource
This study characterizes cardiorespiratory dysregulation in young girls with MECP2 mutation-confirmed Rett syndrome (RS). Respiratory inductance plethysmography of chest/abdomen and ECG was obtained during daytime wakefulness in 47 girls with MECP2 mutation-confirmed RS and 47 age-, gender-, and ethnicity-matched controls (ages 2-7 y). An in-home breath-to-breath and beat-to-beat characterization was conducted and revealed that breathing was more irregular, with an increased breathing frequency, mean airflow. and heart rate in RS versus controls. There was a decreased correlation between normal breathing and heart rate variability, and an exaggerated increase in heart rate response to breathholds in RS versus controls. We conclude that girls with RS have cardiorespiratory dysregulation during breathholds as well as during "normal" breaths and during breaths before and subsequent to breathholds. This dysregulation may offer insight into the mechanisms that render girls With RS more vulnerable to sudden death.
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1203/01.pdr.0000238302.84552.d0" target="_blank" rel="noreferrer noopener">10.1203/01.pdr.0000238302.84552.d0</a>
Rights
Information about rights held in and over the resource
Article information provided for research and reference use only. PedPalASCNET does not hold any rights over the resource listed here. All rights are retained by the journal listed under publisher and/or the creator(s).
2006
Bennett H L
Boothby C M
breathing difficulties
characteristics
Disorder
dysfunction
explanation
hyperventilation
increased breathing frequency
increased mean airflow increased hear rate
irregular breathing
Kenny A S
Lieske S P
long qt syndrome
mecp2
mutations
Patterns
Pediatric Research
Pediatrics
Ramirez J M
Rett syndrome
Silvestri J M
sudden-death
Trajectory
Weese-Mayer D E