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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/S0165-5876(01)00417-7" target="_blank" rel="noreferrer noopener">http://doi.org/10.1016/S0165-5876(01)00417-7</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
A name given to the resource
Disordered breathing during sleep in patients with mucopolysaccharidoses
Publisher
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International Journal of Pediatric Otorhinolaryngology
Date
A point or period of time associated with an event in the lifecycle of the resource
2001
Subject
The topic of the resource
breathing difficulties; sleep disturbance; Galactosialidosis; MPSI; MPSII; MPSIII; MPSIIIA; MPSIIIB; MPSVI; MPSVII; trajectory; characteristics; obstructive sleep apnea; OSA; frequent awakenings; poor sleep quality
Creator
An entity primarily responsible for making the resource
Leighton SEJ; Papsin B; Vellodi A; Dinwiddie R; Lane R
Description
An account of the resource
Objective: Obstructive sleep apnoea (OSA) has been reported as a feature of children with mucopolysaccharidoses (MPS). However, the incidence and severity of OSA with respect to disease type is poorly defined. The aim of the present study was to measure objectively the degree of OSA in a group of children with a range of MPS syndromes. Methods: In a cross-sectional study, cardiopulmonary sleep studies were performed during unsedated sleep in 26 children with MPS over a period of 2 years. Scores of OSA severity based upon clinical history and upon objective sleep study data were made in each case and compared. Results: OSA was present in 24/26 patients, and ranged in severity from mild to severe. OSA was most marked in MPS type IH (Hurler syndrome) followed by types IHS (Hurler–Scheie syndrome) and II (Hunter syndrome). Frequent arousals and poor sleep quality, not suspected clinically, were noted in several patients. There was agreement between the clinical and objective scoring systems in only 17/26 patients (65%) with clinical history scores tending to underestimate the most severe cases (5/26 cases) and overestimate the severity in the mild cases (4/26 cases). Conclusions: Obstructive respiratory problems are frequent in MPS patients and there are differences in severity of OSA between the different MPS types. Assessments of the severity of OSA based upon clinical history alone are inadequate. Our results suggest that objective sleep studies are necessary to evaluate these cases, to monitor clinical outcome and to assess the effects of therapeutic intervention. Prospective studies in larger numbers of patients are needed to validate these observations.
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1016/S0165-5876(01)00417-7" target="_blank" rel="noreferrer noopener">10.1016/S0165-5876(01)00417-7</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).
2001
breathing difficulties
characteristics
Dinwiddie R
frequent awakenings
Galactosialidosis
International Journal of Pediatric Otorhinolaryngology
Lane R
Leighton SEJ
MPSI
MPSII
MPSIII
MPSIIIA
MPSIIIB
MPSVI
MPSVII
obstructive sleep apnea
OSA
Papsin B
poor sleep quality
sleep disturbance
Trajectory
Vellodi 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
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/10.1302/0301-620X.79B4.7547" target="_blank" rel="noreferrer noopener">http://doi.org/10.1302/0301-620X.79B4.7547</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
Carpal tunnel syndrome in the mucopolysaccharidoses and mucolipidoses
Publisher
An entity responsible for making the resource available
Journal of Bone and Joint Surgery - Series B
Date
A point or period of time associated with an event in the lifecycle of the resource
1997
Subject
The topic of the resource
tone and motor problems; MLIII; MPSI; MPSII; MPSIII; MPSVI; surgical interventions; physical interventions; surgery; physiotherapy; exercises; simultaneous tendon release
Creator
An entity primarily responsible for making the resource
Haddad F S; Jones D H A; Vellodi A; Kane N; Pitt M C
Description
An account of the resource
Children with a mucopolysaccharidosis or mucolipidosis suffer progressive disability of the hands, particularly in relation to dysfunction of the median nerve. This is an increasing problem because bone-marrow transplantation has dramatically improved survival without apparently changing the musculoskeletal manifestations. We have reviewed 48 children with these syndromes who required carpal tunnel decompression, recording symptoms, signs, radiological, electrophysiological and operative findings, histology and upper-limb function. In these children the carpal tunnel syndrome differs from that seen in adults. Symptoms are rare but signs such as decreased sweating, pulp atrophy, thenar wasting and manual clumsiness are much more common. At operation, the flexor retinaculum was thickened and a mass of white tenosynovium engulfed the flexor tendons. Most patients had some definite nerve constriction with a thickened epineurium. Functional improvement was seen after early decompression, with some benefit from simultaneous tendon release. Regular physiotherapy helped to maintain increased hand movement. We describe our assessment protocol, the physiotherapy and operative regime and the standard functional review which helps to maximise function in the hands and upper limbs of these children.
Identifier
An unambiguous reference to the resource within a given context
<a href="http://doi.org/10.1302/0301-620X.79B4.7547" target="_blank" rel="noreferrer noopener">10.1302/0301-620X.79B4.7547</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).
1997
exercises
Haddad F S
Jones D H A
Journal of Bone and Joint Surgery - Series B
Kane N
MLIII
MPSI
MPSII
MPSIII
MPSVI
physical interventions
Physiotherapy
Pitt M C
simultaneous tendon release
Surgery
surgical interventions
tone and motor problems
Vellodi A