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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
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URL Address
<a href="http://doi.org/10.1002/ajmg.1320570223" target="_blank" rel="noreferrer noopener">http://doi.org/10.1002/ajmg.1320570223</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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CIRCADIAN-RHYTHM STUDIES IN NEURONAL CEROID-LIPOFUSCINOSIS (NCL)
Publisher
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American Journal of Medical Genetics
Date
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1995
Subject
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children; Genetics & Heredity; sleep; body temperature; circadian rhythms; cortisol; disturbance; melatonin; mental handicap; motor activity; neuronal ceroid lipofuscinosis; radioimmunoassay; retina; sleep disturbance/disorders; NCL3; trajectory; characteristics
Creator
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Heikkila E; Hatonen T H; Telakivi T; Laakso M L; Heiskala H; Salmi T; Alila A; Santavuori P
Description
An account of the resource
Sleep disorders are common in NCL patients, The patients have problems such as frequent awakenings, difficulties with sleep onset, nightmares, and night terrors, The aim of the study was to examine whether the sleep disturbance in NCL can be explained on the basis of desynchronised circadian rhythms, Therefore we studied diurnal patterns of melatonin, cortisol, body temperature, and motor activity of 14 patients, The group consisted of 8 JNCL patients, 5 INCL children, and one boy with Jansky-Bielschowsky disease of the variant type, There were healthy age- and sex-matched control subjects, The blood samples for serum melatonin and cortisol were collected every 2 hours during 24-hour periods, Body temperature was recorded continuously for a 24 hour period by a polygraph, Diurnal motor activity was measured by wrist actigraphy for 5 days, In most of our patients sleep was fragmented and the sleep phase was irregular, Disturbances in the daily hormonal rhythms occurred only in the minority of the patients and only at an advanced stage of the disease, Although disturbances in the body temperature rhythm were found in about half of the patients, a general failure in the circadian regulatory system does not explain the frequent disturbances of the sleep-wake cycle of the NCL patients. (C) 1995 Wiley-Liss, Inc.
Identifier
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<a href="http://doi.org/10.1002/ajmg.1320570223" target="_blank" rel="noreferrer noopener">10.1002/ajmg.1320570223</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
Alila A
American Journal Of Medical Genetics
body temperature
characteristics
Children
circadian rhythms
cortisol
disturbance
Genetics & Heredity
Hatonen T H
Heikkila E
Heiskala H
Laakso M L
melatonin
mental handicap
Motor Activity
NCL3
neuronal ceroid lipofuscinosis
Radioimmunoassay
retina
Salmi T
Santavuori P
Sleep
sleep disturbance/disorders
Telakivi T
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/s0887-8994(00)00138-7" target="_blank" rel="noreferrer noopener">http://doi.org/10.1016/s0887-8994(00)00138-7</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
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Sleep alterations in juvenile neuronal ceroid-lipofuscinosis
Publisher
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Pediatric Neurology
Date
A point or period of time associated with an event in the lifecycle of the resource
2000
Subject
The topic of the resource
Age Factors; Male; Severity of Illness Index; Case-Control Studies; Child; Humans; Adult; Adolescent; Female; Brain; Genotype; Sleep Stages; Polysomnography; Sleep REM; Sleep Wake Disorders; Neuronal Ceroid-Lipofuscinoses; sleep disturbance/disorders; NCL3; trajectory; characteristics
Creator
An entity primarily responsible for making the resource
Hamalainen M; Partinen M; Larsen A; Salmi T; Telakivi T; Kirveskari E; Santavuori P; Sainio K
Description
An account of the resource
In juvenile neuronal ceroid-lipofuscinosis (JNCL), sleep disorders are common. The purpose of this study was to investigate the sleep structure of 28 patients with JNCL compared with healthy controls subjects and to clarify the pathophysiology underlying the sleep disturbances in these patients. Each of 28 patients with JNCL (age range = 6-27 years), with or without sleep complaints, underwent one night of polysomnography. Electroencephalographic, electro-oculographic, electromyographic, and electrocardiographic findings were recorded. Sleep was scored and analyzed visually. The sleep parameters of the patients were compared with those of healthy control subjects. In most of the patients, the total sleep time, sleep efficiency, and percentages of rapid eye movement (REM) and non-REM (NREM) stage 2 sleep were significantly decreased, and the percentages of NREM stage 1 and slow-wave sleep and the number of nocturnal awakenings significantly increased. The percentage of NREM stage 1 and the number of awakenings increased with age and clinical stage. Paroxysmal epileptiform activity during light sleep (NREM stages 1-2) and high-amplitude delta-wave activity with intermingled sharp waves during slow-wave sleep were characteristic of the recordings. The present study revealed that in patients with JNCL, sleep is consistently altered.
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1016/s0887-8994(00)00138-7" target="_blank" rel="noreferrer noopener">10.1016/s0887-8994(00)00138-7</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).
2000
Adolescent
Adult
Age Factors
Brain
Case-Control Studies
characteristics
Child
Female
Genotype
Hamalainen M
Humans
Kirveskari E
Larsen A
Male
NCL3
Neuronal Ceroid-Lipofuscinoses
Partinen M
Pediatric Neurology
Polysomnography
Sainio K
Salmi T
Santavuori P
Severity Of Illness Index
Sleep REM
sleep disturbance/disorders
Sleep Stages
Sleep Wake Disorders
Telakivi T
Trajectory